Motor Neuron Disease Clinical case presentation

Uh, I'll be presenting a case whose name is XYZ. He's a 72-year-old male, a retired teacher from Darw. Admitted on the 27th of September and examined on the 18th of October. He's a right-handed person, and the informant is the patient himself and his wife, who are reliable.

Presenting complaints: He came with a history of left lower limb weakness since 1 year, right lower limb weakness since 8 months, and complaints of bilateral upper limb weakness since 4 months, and difficulty in speech since 1 month, and fever and breathlessness since 5 days.

History of presenting illness: Patient was apparently all right before one year. Later, he developed weakness of the left lower limb, which was insidious in onset and gradually progressive in nature. Patient initially noticed that he was tripping while walking, with three to four episodes of tripping in a 10-day duration. Later, after 15 days, he noticed foot weakness in the left lower limb, and he was not able to grip his slippers, and there was a slippage of slippers while walking, in spite of the patient being aware of that. The weakness gradually progressed over the next month. Within one month, he had developed difficulty in getting up from a squatting position, difficulty in climbing stairs. During the initial period, he was able to walk with the help of a walking stick, but later, 3 months later, he required the help of crutches to walk.

He also came with a history of weakness in the right lower limb since 8 months, which was also insidious in onset and gradually progressive. He initially noticed that he was not able to grip slippers and slippage of slippers while walking, in spite of being aware of it. He was initially using an Indian commode. Then later, he had to switch to the western commode because he was not able to get up from the squatting position. Since the past 5 months, he's been bedridden and is not able to lift his leg. There is also a history of difficulty to roll over in the bed since the past 5 months.

Also, complaints of bilateral upper limb weakness since the past 4 months, which was insidious in onset and gradual progression. Weakness started as the patient initially was not able to mix his food, not able to button and unbutton his shirt, not able to hold a glass of water. Later, it progressed to that extent that he is not even able to comb his hair, lift his arm above his head, and had a difficulty in wearing a shirt since the past one month.

Patient's wife noticed fine involuntary twitching movements over the arm, leg, and fingers, which aggravated on touch and movement, and flickering of water. And patient's wife also noticed there was a thinning of both upper and lower limbs.

Patient also complained of difficulty in speech since the past one month, which was insidious in onset, in such a manner that he had a difficulty to initiate the speech, and he has to strain while speaking. But the patient was able to understand the words and respond back to the commands. No history of change in voice quality or nasal regurgitation.

Fever since one week, sudden onset, high-grade, associated with chills and rigors, continuous type, and subsided slightly on taking paracetamol tablets. Also, a complaint of breathlessness since which is sudden in onset, gradually progressive, increased on exertion, and delayed on rest. No history of chest pain, excessive sweating, cough, palpitation, orthopnea, PND, or V. No history of backache. No history of bowel or bladder disturbances. No history of headache, vomiting, or altered sensorium. No history of diurnal variation. No history suggestive of higher mental function involvement. No history suggestive of other cranial nerve involvement. No history suggestive of sensory system involvement or cerebellar and autonomic system involvement.

Past history: Patient is a known case of hypertension since the past 4 years and is on Telmisartan 40 mg, compliant with the medication. No history of pulmonary tuberculosis, seizure, or diabetes in the past.

Personal history: Appetite and sleep are normal. Bowel and bladder habits are normal. Non-alcoholic, non-smoker.

Family history: Nothing contributory. Born of non-consanguineous marriage.

Course in the hospital: There's no improvement in his neurological status since he got admitted. But when he came, he had respiratory distress, in view of which he was intubated and he was later tracheostomized. And now he's on tracheostomy with oxygen support. Presently, he is better done and is dependent on his wife and son for all his daily routine activities.

Summary: Elderly male hypertensive patient presented with slowly progressive pure motor symmetrical quadriparesis, lower limb more than upper limb, with trunkal muscle weakness with wasting, with fasciculation, dysarthria, and without sensory, cerebellar, bowel, and bladder dysfunction.

Okay, we'll start analyzing the symptoms now, one by one.

No. Yes, ma'am. You told he is a right-handed person. No. Yes. How did you decide, ma'am? Ma'am, uh, how did you decide, ma'am, a right-handed person? When we ask him to catch something, he is right-handed, ma'am. He writes with his right hand, and whenever we ask him to catch something, when we give something, he was bringing his right hand forward, ma'am.

Okay, we have done this exercise many times. Still, it is important in some situations. So, would you like to tell me what all methods you are supposed to use? A teacher, he would have written with the right hand only. So, what all methods you are supposed to apply when you do a scientific presentation? Because in some situations, it has got relevance. Hundredness. No. Yes. So, what all questions you are expected to do? This is only revision. We have done it before. Tell me, ma'am. What all things will help you to know it is right-handedness? You said that he writes with the right hand and he was bringing his right hand when he offered some object. Okay, agreed. Tell me, with that, we cannot go ahead with an epilepsy surgery or tumor surgery on the left side and say that you will not get aphasia. No. Yes, ma'am. So, what all things you are supposed to do to know the real handedness? One, you will look at eyedness. I have told it in previous sessions. Highness, because right normal, that is about 90% of the population is right-handed. Right ear, left footed. The dominant foot is left. Yes. Dominant ear is left. Dominant eye is right, and dominant hand is right. So, handedness, we shift by our culture. And he is 72 years old. At that time, the people will shift. They have a tendency to think that left-handedness is wrong. So, that era is over now. Still, so he is 72. So, you have to look at the eyedness. Yes, ma'am. So, you tell the patient to focus on an object with your illuminoscope, which you use to do transillumination in swellings. No, illuminoscope or something like that, kaleidoscope, something you give, and then which eye he uses. That's the simplest bedside test to know handedness. So, eyedness and handedness go together. Second, you can hold both hands like this. No, the dominant hand, dominant thumb will be down. Okay, reflexively, consciously, you should not do. Reflexively, dominant thumb will be down. Or if you put your hands behind, the dominant hand will be up. So, those are things which you can modify if you are a medical person. Otherwise, eyedness, then you apply next level. Apply what is that inventory called? Edinburgh's Handedness Inventory. Inventory, yes. Two inventories are there. One is 10-point, another one is 21-point. The 10-point is for routine purpose, simple situations. 21-point is pre-operative. No. Yes. Of which 80% of the questions point to one side, that is the dominant side. Yes. Next, you can go for the EEG. Yes. EEG will show less well-formed alpha rhythm on the dominant side. Alpha is a rhythm of relaxation. So, well-formed alpha means dominant side or non-dominant side? Poorly formed, poorly modulated alpha is non-dominant. That is easy. These are non-invasive, simple tools you can do. And olden days, people used to do intracarotid Amytal test. You do the carotid angiography and inject short-acting phenobarbital into the carotid artery. Patient will be counting and stretching both his hands. And when he is, when it is injected into one side, the opposite hand will come down. That means you have injected into the carotid only, not into the soft tissue. And when that hand is coming down, if the language stops, we are telling the patient to count. If the speaking stops, that is the dominant hemisphere. So, when you are injecting into the right carotid, if the patient's left hand is coming down, it means you are correctly in the carotid artery. And if counting also stops, that means that side's language is on the right side. Yes, that is the intracarotid test. Uh, it is not now. Nowadays, it is not done. You can do the PET scan. So, higher thing, you can do functional imaging. When the patient is given some task like communication, which part of the brain gets activated, that will be the dominant. So, these are the methods by which then you can see the angular gyrus. The simple MR voxel-based morphometry, the volume of angular gyrus is larger on the dominant side. So, that is non-invasive and less if less costly. Voxel-based morphometry, we call it that way. You can check. Or if you want to do PET scan, if you are in that situation, you can do a task-based PET scan and see whether which side is activated during language activity. So, these are the methods at the bedside. You should say eyedness. Not that he writes with the right hand. Or you need not commit handedness if that case it is not relevant. But if you are telling right-handed, you should tell, I checked the eyedness, he is checking with the right eye, so I think it is right-handed. So, that is the first thing. Second, you told that he was tripping. The first thing we started was tripping, no? Yes, ma'am. So, tripping means what does it mean to you when somebody's telling tripping, used to fall? It means that he is injuring his toes. Yes, ma'am. The word tripping is used when a person is falling due to injury to the toes. That means he has a foot drop. Yes. Dorsiflexion weakness. You understand? Somebody is tripping means he's injuring his toes while walking. Normally, we don't do that because we start our gait with the heel. All of us start our walking with a heel, not with a toe. You can watch your walking tomorrow. When we start walking, it is a heel that comes to the floor first. The gait starts with the heel strike. So, this person is tripping means instead of his heel strike, it is the toe strike. So, there is an alteration in his walking pattern. Normal walking pattern starts with the heel strike, whereas my patient's walking has shifted to toe first. That means he has developed a foot drop. He's unable to dorsiflex and put the heel on the floor. So, first thing you will see, my patient is tripping. So, gait semiology has changed from the heel-first gait to toe-first gait. That means there is a foot drop. Okay. So, the first symptom indicates there is a foot drop. So, now you want to know whether the foot drop is due to. So, that is how you will expand this symptom. Instead of saying gradually progressive, you analyze that symptom. Is that toe-first gait with a flail dangling toe, or is it a stiff circumducting toe, or was it a continuously twisting? Because you can have element weakness for foot drop. You can have pyramidal foot drop. You can have dystonic foot drop, and you can have foot drops due to contracture. That is not applicable in this patient because it is not there before and it came now. So, contractures are not applicable now. So, it's an element to first or toe-first, or is it a dystonic foot drop? Yes. So, when you tell that symptom, my patient started tripping, you should tell that I asked him, he said that his feet was flying and it was hanging loose, because of that his injuries were getting, toes were getting injured. Then you know that it is a lower motor neuron. Yes, ma'am. If he said that his limb was slightly stiff and he could not lift his toe, then you know that it is an upper motor neuron. Yes. And if he said that when he tried to put his foot forward, the leg was continuously twisting, it indicates that there was a foot dystonia. So, these are the three situations where the people change their semiology from a heel-first gait to toe-first gait, which makes them trip. So, that information you have to gather in a history like this, then we will know whether it is element or dystonic. If it is, um, generally pyramidal tract due to pyramidal foot drop or extrapyramidal tract, that is dystonia. Whereas if the person says that my leg was flail, I was landing on my toe and falling, and I had sensory loss. I felt that my leg had become now numb. Then you know that it is either in the sciatic nerve, common peroneal nerve, S1 root. Amen. So, all these are lower motor neuron. So, lower motor neuron can be in the nerve. It can be in the muscle. It can be in the anterior horn cell. It can be in the neuromuscular junction. All these things. Neuromuscular junction, very unusual because it will not start distally. Yes. So, we are not affected. You said at the end. So, it is very unlikely to be sciatic, common, or S1 roots. So, if it was a root or a nerve, it will be sensory-motor. So, sensory is not there. So, if it was a flail one, then you are left with motor nerves, motor roots, and anterior horn cells. These are the three things in the element because you did not tell me whether it was flail or stiff. We will have to take it as either way. We do not know. Okay. So, let us imagine it is flail. We have cut off the nerves. We have cut off the roots. You are left with motor radiculopathies, or it may be a motor nerve, or it can be anterior horn cells, or muscle disease also. Out of neuromuscular junction also out. Among the purely motor syndromes, because neuromuscular junction disorders involve the proximal and the bulbar muscles. They do not start in the distal muscles. So, you will not consider neuromuscular junction at all. You will not consider muscle disease because muscle diseases are symmetrical. They do not start asymmetrically, and primary muscle diseases do not become manifest in the seventh decade. They are manifest in the first four decades. They do not become symptomatic in the seventh decade. So, neuromuscular junction and muscle disease are excluded for obvious reasons. So, whether it is a motor or nerve. So, is it a motor nerve involving the motor part of the sciatic nerve or common peroneal? Then you know that these nerves cannot be involved separately as motor because all the fibers are in one bundle. So, sciatic nerve, common peroneal nerve cannot manifest as purely motor. So, it cannot be a motor neuropathy. Motor neuropathies are common in some situations where the sensory is less dominant, and they are bilateral. Pro- this is motor neuropathy with multifocal conduction block, affects middle-aged males and starts in the upper limb. So, motor neuropathies affect upper limbs, young males, usually fourth to fifth decade. So, that and very unusual in sciatic nerve and common peroneal. So, it is less likely. No. Then we will go to what are roots. So, cauda equina. So, cauda equina roots. Last class, I told that you have got a dissociated relationship between the vertebra and the spinal roots at various levels because you are having eight cervical segments in the cervical cord, and your cord stops at L1 lower down, but you have up to coccygeal segments. Cord stops at L1, but you have sacral, coccygeal, all those segments are there. And the cervical cord, you have got eight roots and seven vertebrae. For this reason, there is a dissociated relationship. That is more obvious in the cauda equina region because the cord stops at L1, and you have got so many vertebrae below that. So, each root in the cauda equina region to reach its corresponding foramen remains a longer distance free, whereas cervical, up to five, four or five, they immediately enter. Then upper thoracic enter a little higher. But when you come down, they travel long distance because the cord has already stopped. From the, like the cluster of the horsehair, all the roots are hanging, and depending on whichever is the root number, they travel varying distances before they join the intervertebral foramen. What, what is the second unique feature? The other regions, the motor and the sensory roots join in the intervertebral foramen. Here, because they travel a longer distance, they stay separate throughout that distance. So, in the cauda equina region, you can have purely motor, you can have purely sensory. So, radiculopathy, you cannot rule out. So, so, we will keep it as a possibility because dorsiflexion means L5. So, L5 is already in the cauda equina region. Motor radiculopathy is possible because it travels a long distance from L1 to enter the L5 foramen, and after entering that foramen only, it joins with the sensory root. So, it is very much possible. It may be asymmetrical. So, we typically describe a cauda equina as asymmetrical, areflexic, with late bladder. Asymmetrical, areflexic, late bladder is typical cauda equina. So, it is asymmetrical. We don't know element or we have not got the history appropriately, and it is L5 apparently because it is a foot drop and pure motor. And pure sensory is possible in the cauda equina region, so it is possible. So, L5 root is possible. So, we'll keep it there. The next will be dorsiflexors, yes, anterior horn cells. That is L5 anterior horn cell. I said that it is not possible in the narrow level and horn cell. So, now, how are you going to differentiate L5 motor root from L5 anterior horn cell? That is the question to you, which you will have to answer. Sensory-motor roots means you will say root pains are there. Yeah. Got sensory loss. So, it's a root. So, if it is a sensory-motor or root, sensations and presence of root pains will help you to understand that it is a root. But if it is only motor root, you cannot apply that point. So, now tell me, how to differentiate L5 motor root from L5 anterior horn cell, and how to differentiate L5 motor root from L5? Anyone wants to answer? Answer. Anterior horn cells will be having fasciculations. Okay. Fasciculations may be there. Yes, ma'am. And the wasting will be prominent. Mhm. In L5 root, wasting will not be prominent. Yeah. Both you see root. What are all the muscles that are supplied by the L5 root? Uh, dorsiflexion to the toes. Name the muscles. Name the muscles supplied by the L5 root. Uh, tibialis anterior. Tibialis anterior is for okay. So, extensor hallucis longus, extensor digitorum longus, extensor digitorum brevis. Gluteus medius, gluteus minimus. That is the L5 root supplied muscles. Yes. So, L5 root supplies extensor hallucis longus, extensor digitorum longus, extensor digitorum brevis, gluteus medius, and gluteus minimus. Yes, you see. So, these are the muscles. So, that if there is an L5 radiculopathy, even though the patient may be showing the symptom maximum in the extensor hallucis longus, that is ultimately we say that extensor hallucis longus will show maximum symptoms, but when you examine, if you carefully look for, you will find that the person is having slight waddling on that side, and hip abductors are slightly weak on that side compared to the normal side. That will say it's a clear radicular pattern. Nobody can imitate because one is proximal and one is distal. The muscles are separately supplied proximally and distally. So, L5 radiculopathy, psychogenic foot drop, patient cannot. So, the abduction, he will not know. So, you will not imitate it. So, L5 root means extensor hallucis longus gets maximum symptomatic, but extensor digitorum longus, extensor digitorum brevis, gluteus medius, and minimus. So, hip abduction will also be slightly affected. Whereas, what is anterior horn cell pattern? Is it all groups of cells? You know, in the ventral horn, in the spinal cord, there is a peculiar arrangement. You see, you know, the ventral horn has got medial fibers, central fibers, and lateral fibers. Lateral fibers go to the appendicular muscles. Medial fibers go to the head and neck, and intermediate goes to the central structures. And in the limb, that is outermost, anterior horn cells are supplying the limb muscles. And cervical limb, in the cervical cord, lumbar in the lumbar. Lumbar anterior horn cells to the leg muscles. There, there is something called motor unit. What is a motor unit? A single anterior horn cell with its axon and the muscle that it supplies is called a motor unit. A single anterior horn cell with its axon supplying a muscle fiber is the motor unit. So, in the ventral horn, it is not like a root. Root is already organized for a leg and it supplies a group of muscles. Whereas in the ventral horn, there is a motor unit. So, that is called as a motor unit pattern. That means it is having units within the muscle. Even within the extensor hallucis longus, all the fibers will not be wasted. Even within the gluteus medius, all the fibers will not be wasted. So, anterior horn cell pattern is motor unit pattern. So, we call it as a selection within your territory. So, let us imagine it is L5 radiculopathy, and you are examining, you find that the EHL is slightly weak, EDB and EDL are normal, gluteus medius is slightly weak, gluteus minimus is normal. So, that is you think it's a root. But there are selected motor units. We call it as finger drop in the upper limb. When there is an anterior horn cell pattern, even though all the fingers are supplied by C7 root in the upper limb, only one finger will drop like that. So, that is very, very unique. So, it is selection within a radicular territory, which is otherwise called as motor unit pattern. So, you first decide, okay, my patient has got a flaccid foot drop with no sensory involvement. So, it may be a motor root in the cauda equina, or it may be anterior horn cells. And start examining EHL, EDB, EDL, and you are surprised that only EHL is slightly weak, or the second toe is slightly weak, other toes are all right, or other toes are weak, and EHL is all right, and hip abductors are all right. That is called motor unit pattern. So, to differentiate between anterior horn cells and motor root, the most important thing will be the pattern. Motor roots will supply all the muscles that are supplied by that root. Whereas anterior horn cell is a motor unit pattern. So, it will select some fibers within a muscle or within a motor territory. That is one. Second, fasciculations, of course, I agree. But in the distal muscles, in the small muscles of the foot, it is very difficult. You don't generally find. You find it in the bulky muscles, pectoralis, deltoid, infraspinatus, facial muscles, tongue muscles. You see them, but very, very uncommon. I have not seen fasciculations or fibrillation in the muscles of the leg so far. It's very difficult to see clinically. Electrophysiologically, you will be able to pick. But clinically, it is very difficult. So, fasciculations will not get. Second point, you will not be able to demonstrate. Then, third point will be because it is only some, some motor units. Reflexes are not altered. Even if that root perceives a reflex, whereas L5, S1 root, S1 root, hip extensor will go, plantar flexion is going, ankle jerk will be lost in that territory also. Patient will have normal reflex because it is only motor unit. Single, single units are destroyed in the early stage. The reflexes are not suppressed. So, first point is a motor unit pattern. Second point is reflexes remain retained till very late, and in certain distributions, you may be able to demonstrate fasciculations, not everywhere. You may show some tremors, especially in the upper limb. In the lower limbs, a tremor has to be demonstrated by making the patient lie down and the foot hanging, and you may see tremors. That is an indirect evidence of fasciculations. So, tremors, slight rumbling may be seen. So, you understood what is anterior horn cell pattern and an anterior root pattern. Anterior root means all the muscles supplied by that root will be affected. Anterior horn cell pattern means some of the muscles will be normal, some of the muscles will be affected, and even inside that affected muscle, some groups will be severely affected, and the rest will be normal. Yes, that is the reason you get toe drop, finger drop in anterior horn cell disease. So, now, when you examine, your differential diagnosis is going to be between anterior root and anterior horn cell. So, you will specifically look for this motor unit pattern. Yes, ma'am. Then, is there any dystonia? You did not tell that the limb is going on twisting. If it is dystonia, continuous twisting is very typical. So, usually when the patient starts walking, it will be action-induced aggravation. When he starts walking, there will be some twisting, and when he starts progressing with walking, the limb will twist very badly, and it is stiff and remains like that. He has to stand, relax the floor, that it will go. So, dynamic, dynamic change in the foot drop with continuous twisting, which increases with the task. If it is there, it will indicate a foot dystonia. So, you will go to the basal ganglia. Then, if it is a stiff foot drop without continuous twisting, you may think of a corticospinal tract. So, these are the possibilities we will keep for a purely motor foot drop. So, tripping indicates you had a foot drop. Whether it is element, you could have determined if the history was available because it is not there. We are left with whether it is a motor root, is it an anterior horn cell, or motor unit pattern, or is it a dystonia? Obviously not there. Or is it an pyramidal pattern? We don't know. We will keep it like that. Yes, ma'am. The next, you said that you have difficulty in climbing, getting up from sitting posture. No, squatting position. Yes, ma'am. What was the difficulty? I have discussed before also. You have to tell them what is the semiology. So, first, you say my patient is unable to get up from the sitting posture. All of us have. You may have knee pain, you may have hip pain, you may have back pain. So, he is not troubled by. He's a 72-year-old person. He can have asked you arthritis. No. Yes. So, you should tell that. So, I have told it before in subsequent classes. You should apply that and improve the history. No. Yes. So, you should say my patient is unable to get up from the sitting posture, but it is not due to pain. He has to, he has, his leg feels stiff, so he needs somebody to lift him up. So, if the patient says that I do not sit at all, and if I am forced to sit on the floor, my leg feels stiff, and somebody has to lift me up, then it is an upper motor neuron. Upper motor neuron patient cannot flex his legs and bring it under the thighs and support his trunk with the hands and get up. It is not possible. So, so, it is painless. So, it's not osteoarthritis. He needs help, then it is plastic. So, if the patient says I am able to flex my leg, even though I am tripping, I am having some distal weakness, but I am able to flex my leg and bring it under the thighs, but when I try to get up, I have to plant my hands on the floor and support myself to get up, that means if you have a hip extensor weakness. So, as I have told in the previous sessions, getting up from the sitting posture is by spine extensor, hip extensor, knee extensor, and dorsiflexor at the ankle. They are the agonists. Spine extensor, hip extensor, knee extensor, and dorsiflexor at the ankle are the agonists. Any one of them can be affected. He already has dorsiflexor at the ankle affected. So, how will it manifest? When I am trying to get up from the floor, I have to fix my ankle, otherwise my ankle wobbles, and I sprain my ankle when I get up. I always sprain my ankle. Already he has got dorsiflexor, which is an agonist in getting up, is affected. So, if that is the culprit, patient will be able to tell. You should ask. Yes, he's a teacher, he will certainly be able to tell. So, patient will tell, my ankle is unstable, I twist my ankle. So, I sometimes hold my ankle with my hand to prevent it wobbling. Then I am all right. That means it is the dorsiflexor at the ankle which is already affected. That is the cause for the getting up from the sitting posture difficulty. Instead, if the patient says, no, no, I am able to get up, but when I try to stand, I am buckling and falling. That means knee extensor. So, getting up is dorsiflexor at the ankle, knee extensor, hip extensor, and spine extensor. So, if he says, when I get up, I am buckling and falling, then you know, okay, my patient is already having dorsiflexor at the ankle weakness, now he's developing quadriceps weakness. So, that is the second thing. That is why you should elicit this. Now, we have now gone some way into our sessions. No, you should apply it in subsequent classes. So, useful, you will feel excited that you have an MRA which is superior to the 1.5 or 3 Tesla MR outside available. No, you can prove it yourself any number of times. So, next, okay, these parts are all right. I'm not buckling. But when I try to get up, I feel that I have to bend my trunk to elevate my hip. That means it is the hip extensor weakness. If the hip extensor is weak, he will not buckle. He will not wobble at the ankle. Instead, when he tries to get up, he will bend his trunk forward. While he's bending his, why he's bending his trunk forward? Because the hip extensor is weak. He's using the spine extensor to pull the hip up. You know that erector spinae has got an origin from the iliac crest. So, it can lift the hip. Erector spinae origin from the iliac crest can help the hip to be lifted. So, it is using that. You have been told to walk, sir. So, whatever possibilities there used to lift your hip. So, the brain will bring out that erector spinae is there. It is having origin to the iliac. So, let me use that. Yes. So, so, why you are bending? You see, now I am here. I cannot be in my hospital now. You see, similarly, now I am here. I cannot be with my mother now. So, like that, when the erector spinae has to erect the hip, it cannot erect the spine. Because it cannot do both work together. So, erection of the spine done by the erector spinae is switched off, and all the force in the erector spinae is directed towards the iliac to lift the hip. Okay. So, patient becomes bent. Understood? Is it clear? Is there any doubt? You ask me. Yes. Next class, you should apply all this, and you, I should not be discussing anything. The diagnosis will be obvious. He will sit on the diagnosis. Yes. That is what I tell. You will know the structures involved very clearly. Yes. So, then slowly, what will the patient do? He will, the erector spinae will lift the hip. Then, now you have to erect the spine. Then only you can walk. So, you will lock your leg with the cruciate ligaments. You got semilunar cartilages, anterior cruciate ligament, and posterior cruciate ligament. Using these things, you will lock your knee. By locking the knee, patients can't stand, they cannot walk. So, the body will use that apparatus and slowly lift the trunk and, what it will do? One hand is kept on the floor, and one hand is put on the knee. That is called tripod sign. So, first, the patient was bent, standing with both hands down. Now, he's trying to stabilize his weak leg using the one hand, putting it on the knee to stabilize the cruciate ligament, and the opposite hand is on the floor. That is called tripod sign. Then, the tripod is stable. So, the person will know, I can move up. So, he will put that hand also on the opposite knee. That is called the Gower's sign. So, Gower's sign indicates gluteus maximus is weak. So, he will then slowly, slowly erect the spine. Now, the erector spinae is not erecting the hip. The hip is fixed in a standing posture by the ligaments, and the erector spinae is erecting the spine. Yes. So, if the person says like that, then you know that he's getting up from sitting posture is due to gluteus maximus. And if it is due to trunk muscle involvement, patient cannot erect himself. It is like the upper motor neuron. Somebody will have to pull him, and when he is pulled up also, he will stand bending only. So, that is obviously not there. So, erector spinae is not the culprit. It could be dorsiflexor at the ankle. If you have elicited the history, and patient said, my ankle was twisting and wobbling, that was the difficulty. Yes. Otherwise, buckling, he was not buckling. No, no, he was not buckling. I suppose initially, you know, was he buckling at the knee initially? He was buckling it. No, he didn't have. So, whatever was the history that will help. It's not erector spinae because he was not bent. So, it could be any one of these muscles or all of them together. That depends on what were the functional disability that patient had while getting up from the sitting posture. Okay. If you had elicited that, we will know the demarcation. Then, you said that you had climbing. That also I had discussed before. So, climbing, your hip flexors will help you to put one leg on the step above. So, if the hip flexor is weak, patient will tell, I am not able to do alternate climbing. Instead, I have to lift my one leg with one hand, put it on the step above, and then the next leg is flexed and kept. So, if it is iliopsoas, I saw the hip flexor weakness, patient will tell, I cannot do alternate climbing, and when I am climbing to put one leg on the step above, I have to lift my leg and put it on the step above. So, you should elicit that history. You should not stop by translating what the patient is telling. You should elaborate. Yes, ma'am. Yes, you know the anatomy. Patient does not know. No. Yes. So, he says, okay, my patient. Say, yes, madam, you can tell that if he is telling, no, no, no, no, I can put my leg above. I can put my, lift my foot, leg, and put it on the step above, no problem. But once I'm trying to bear my weight on that leg, that hip slightly comes down, so I feel that I am going to fall, but I manage by holding on to the side railing. If he says that, it is the waddling muscles, gluteus medius and minimus. I am able to put my leg above, but when I put my weight on the right leg and try to elevate my left leg, my hip is sort of waddling, and I feel I may fall backwards or sideways, so I hold on to the side. That's clearly gluteus medius and minimus. And if he says that I am not dropping my hip, I'm not using my hand to lift my leg and put it on the step above, but when I try to move up, I have to slightly bend. That is again the hip extensor. Okay. So, when climbing, you have iliopsoas first, then you stabilize using the gluteus medius and minimus, then lift your pelvis using the gluteus maximus, and then ascend through the erector spinae. So, all that can be elicited, or more than one level of involvement is there, that will clearly tell you what is the cause for his climbing up. So, climbing up is more difficult than climbing down means element disease. But if climbing up and climbing down are equally difficult, it is pyramidal disease because in pyramidal disease, it is a stiff limb. Now, we are differentiating between element and motor. So, if it is motor, patient will say, because it is a kinetic melody in upper motor neuron, it is the, no muscle paralysis. So, this muscle pattern which we discussed will not be there. It is a movement pattern. We call it as kinetic melody. It is the melody of the movement that gets affected in upper motor neuron. So, everything will be difficult. Climbing up is also difficult. Climbing down is also difficult. My leg is stiff. If you are saying that it is upper motor neuron, whereas lower motor neuron, muscle by muscle, just like I analyzed now. Next class, you should elaborate your history like that. Okay, ma'am. Okay, ma'am. If the person says climbing up was all right, but down is difficult. Down is more difficult. We call it as lumbar canal stenosis. We are even thinking of motor radiculopathy in the cauda equina. So, that will be a very useful point. My patient is, you are suspecting whether motor root or anterior horn cell. That is where we left with the first symptom of tripping, no? So, if the person is saying, madam, even I am tripping, otherwise I can climb up little better, but when I am climbing down, I become very weak. That means it is root, lumbar canal stenosis, where the nerve roots are jammed because when you climb down, we naturally bend our trunk, then the roots become stretched. So, climbing down is more difficult means it is favoring a stenosis. Yes. So, we are keeping two DDs. So, as we improve with each symptom analysis, we should be improving in our localization, provided the history has been properly taken. Understand, ma'am. Okay. So, if you asked, or you are, are you lifting your leg with your hand? Are you able to do alternate walking? What is the problem? Up, down, everything is difficult. All those histories if you have elaborated, we will clearly know element or pyramidal. So, next class, anybody presenting a similar case, you should analyze the symptoms which we have already discussed, should be incorporated into subsequent cases, so that we will go upper and upper in the neuraxis slowly. Understood, ma'am. Yes, ma'am. So, now, we do not know what is his problem in climbing up. So, climbing up is difficult. Okay. So, what the patient tells should be elaborated with your knowledge. Yes. So, we become wiser at the end of our history only. So, now we'll say, okay, it's difficult because semiology is not there. We'll keep it like that. It could be any of these things. The next, you told that this rapidly deteriorated and patient developed the right leg incidence gradually. Was he, was he not able to grip slippers, slip slippers? Similar to left. Okay. So, slipping of the thing with the knowledge is indicating what muscle sensation was intact. Yes. Which muscle then, indashi, indashi, which muscle will make slippage of slipper with the knowledge? Indashi? Yes. So, initially, it is the dorsiflexors which made the patient trip. Now, we have got hindrashi involvement. Yes, sir. Here, would you like to tell me how to differentiate? Em can also slip. No. So, hemiplegic patient, slipper will slip. No. It is. Yes. How do you, what will be the symptom? Patient will have difficulty in putting his feet into the chapel because, you just remember, upper motor neuron movement paralysis. Lower motor neuron muscle paralysis. In upper motor neuron, why is there movement paralysis? Movement has got a pattern. Supposing I want to speak something. So, to bring out the correct sound, what all muscles in the vocal cord, what all muscles in my lung, what all muscles in my lip should be put into action so that the correct word is pronounced, that is the motor schema. So, that motor schema fails. As I told, motor schema has agonists and antagonists, fixators. In the lower motor system, they are properly coordinated. So, in upper motor neuron movement paralysis, due to failure of formation of the proper motor schema, there is no muscle paralysis. Whereas in element, it is muscle. So, if I say indashi, it is a muscle. No. So, if the indashi is weak, only the indashi-mediated activity will be paralyzed. So, indashi is for reduction. So, patient will not be able to hold the chapel and walk. But he can put his feet into the chapel because indashi is giving space. It can be easily incorporated. You can remove the feet out of the chapel easily. That is also possible. You cannot hold on and walk. Whereas in upper motor neuron, what happens? Schema, everything needs the schema. So, he cannot put his feet into the chapel properly. The toes somewhere, the toes will be antagonist activated. Somewhere it will be agonist activated. So, one toe will be medial, one toe will be lateral. So, patient will tell, I will have to use my hands, separate my toes and put my feet into the chapel. So, putting on the chapel is weak. Holding on and walking is weak, and removing is also weak. All three things are weak. Means it is upper motor neuron. It is not muscle. It is the motor schema. Motor schema to carry out the movement that has been formulated wrongly because of the upper motor neuron problem. So, all faculties are affected means upper motor neuron. Yes, ma'am. Is only holding on and walking means lower motor neuron. Yes. So, if you think it is lower motor neuron, it is hindering. And if it is tripping due to lower motor neuron, it is EHL and EDL. Then climbing up weak without the patient lifting his leg and putting on the step, elbow means it may be hip abductor or hip extensor because it's not bent. We are not thinking of spine extensor, probably if it is stiffly, it may be also that is not there. So, if slipping is also finished, then unable to get up. Okay. Next month. So, next was five months, he has found that his upper, he has become bedbound. Yes, ma'am. He has become bedbound because both legs have been affected. Is it so? Yes, ma'am. Okay. He had a difficulty rolling over. Roll over in a bed. Yes. What are the muscles which are helping you to roll over? Abdominal muscles. Uh, so, rolling over means turning from left side to, if you are lying on the left side, you want to twist your trunk towards the left side. If you are lying on the right side, you want to shift your trunk towards the right side. So, what are the primary muscles involved? It is the obliques. No, you have got external oblique and internal oblique muscles. So, the right external oblique and right internal oblique help you to tilt your trunk towards the left side. Left side. So, right-sided obliques will help you to tilt your trunk towards the left side. Yes. If the left-sided obliques will help you to tilt your trunk towards the right side. So, if he's not able to tilt to the left side, not able to tilt to the right side, it indicates that the oblique muscles on both sides are affected. Affected. Okay. Oblique muscles on both sides. No. So, that means the oblique muscle. Was he stiff while turning? No. Yes. Was he stiff? No. No. Was he feeling stiff while turning? The patient says that my body is stiff like a log of wood. Then it is upper motor neuron. He was not feeling stiff. No. No, ma'am. No, ma'am. In that case, in that case, it is okay. It is oblique muscles on both sides. Oblique muscles on both sides are affected. Now, the upper limb, you told that mixing of the food. So, mixing of the food is like this. So, mixing of the food is mainly an action of the lumbricals. Mixing of the food is lumbrical. So, mixing is difficult and buttoning is normal. Means buttoning is done by the opponent's muscle. Now, buttoning is affected. I'm just teaching you the anatomy. No, you said mixing is weak. Mixing is weak means it may be lumbrical. If it is in the lower motor neuron, even upper motor neuron patient cannot mix, but it will not just mixing. He will not be able to hold the pen, he will not be able to do extension. All movements are affected means upper motor neuron. Only mixing of the food means it may be lumbrical. So, he has mixing difficulty. If it is in the element, it is lumbrical. He has buttoning difficulty, it is the opponents. This opponent policies and the finger extensors, they are helping in buttoning. Buttoning is helped by opponent group of small muscles and flexor group of long muscles. Then he is not able to hold the glass of water. That's a long flexor. Long flexor. So, if it is element in the upper limb, it is lumbricals. It is opponents, and the long flexors because of unable to mix the food, unable to button, and unable to hold a glass of water. But if it is upper motor neuron, everything will be affected, but the worst affected will be the finest movement. Yes. Holding pen and mixing food will be worst affected, whereas holding a cup will be much better because kinetic melody is most important for the finest.

moment. A hemoplegic patient might have recovered completely from his paralysis. You have thrombolized in time and everything happened perfectly, but the patient is a computer engineer or somebody like that. He says, "Okay, I have recovered, but now I am trying to type on the keyboard. I am unable to do that." This will be a surprise for him because that's the finest moment, so finest movement, piano playing moment, playing on the vi, typing the typewriter or computer. Those finest movements become first affected. They need more coordination. They are small motor units, more coordination. So if the patient says, "The finest movement is very, very difficult. I cannot mix my food at all, but somehow I can manage and hold the glass of water," then you know it's a kinetic melody finest.

If it is in the lower motor neuron muscle pattern, lumbricles for mixing and holding the cup of water is long flexor opponents for buttoning. In your case, all are affected. So if it is lower motor, all muscles are affected. If the involvement is uniform, it is favoring an element. If the patient says the fine movement was worst affected, probably because we need more data like stiffness and all those things have to be obtained. Then, then you said that later he developed abduction of the elevating the hand above the head. Yes, ma'am. And was affected. Which is the muscle used for elevating the hand above the head? Uh, deltoid, ma'am. Yes. Deltoid. Supraspinatus. Deltoid and trapezius. Yes, ma'am. First 30° is supraspinatus. Then 30° to 90° is deltoid. Above 90° is trapezius. So all three, all three faces are they affected? No, ma'am. He was able to lift, ma'am, like, uh, above head. He was not able to lift, ma'am. That means probably supraspinatus is not that much affected. Probably it is the deltoid and the plus minus trapezius. So approximately, first part is able to lift. So distally, the small muscles for muscles and proximally, probably delta plus minus trapezius. Yes.

Then what else? You said that he is unable to speak? You told me that he has some problem in speaking. No. Yes. Next thing is speaking or teaching. Uh, no, speak. Speaking difficulty. Speaking. So you told that speaking is effortful. Yes, ma'am. He used to strain while speaking and he could comprehend very well. He can read. He can understand what is spoken to him. But only one component is affected, that is production of sound. So it is a speech disorder or a language disorder? What disorder? How do you differentiate speech from language? Would you like to tell? You're hearing now? Yeah, now I, I can hear. Speech means, you see, language, we say that language is ability to convert thoughts into comprehensible modes of communication. Yes, ma'am. So language means thought is there and you want to convert it into various modes of communication. That involves writing, speaking, uh, listening to spoken words, and reading and repeating. So if it is language, all components will be affected. So thought converting thoughts into comprehensible modes of communication, that is language. Whereas speech is converting language into audible sounds, only one sound. So you said that your patient is able to comprehend. He's probably able to read. He's probably able to not able to write, maybe because he's already having an element problem. But only sound production is altered. Yes, ma'am. So it is not language, it is speech. Speech. Only sound production is affected. So it is not a language problem, it is a speech problem. No. Yes, ma'am. So that is called as dysarthria. So this is not a dysphasia. This is a dysarthria. What are all the types of dysarthria? You know of? Bulbar dysarthria, pseudobulbar dysarthria, cerebellar dysarthria. So dysarthria is classified into flaccid and ataxic, dyskinetic and cortical. Yes. Flaccid, ataxic, dyskinetic, and cortical. Flaccid is element. Flaccid can be due to tongue, lips, and palatal muscles. If it is tongue, tongue is important for producing sounds which need 'y'. So 'lily', 'la', no, those kind of letters are coming. Those words are very difficult. So that is a flaccid speech. So flaccid speech is characterized by dysarthria for specific letters. So labial means 'p', 'b'. Dentals, 'person', 'd', 't'. Gutal, 'ng', 'g'. So if the person is telling that I am having difficulty in speaking out, all other compounds are all right, and this is worst when I am mentioning 'papadam' or 'papa' or something like that, then you know something has happened to his oricularis oris. So it's a labial type of flaccid disorder. If the 'l' is affected or 'd' is affected, it is lingual. 'dot', 'log', 'lily'. No, then you know maybe my patient's tongue is affected. And if he says that when I am telling 'bring', 'sing', everybody is telling I am speaking through my nose, then you know it is the gutal. So flaccid dysarthria can be due to labial, it could be due to lingual, or it could be due to gutals. So all this will produce specific defects. So that is flaccid dysarthria only. Specific defects mean it is flaccid. Whereas spastic dysarthria, patient will not have this kind of specific letter-based defect. But again, the kinetic melody is affected. So he will strain and skew out his words because the whole body is stiff. So it will appear that he is skewing out his words. When we skew out, what happens? The syllables will run into one another. When you are excusing, you cannot split anything. It runs into one another. Just like your um, those, what is that? When you make excuse, the 'm', it comes in a continuum. The 'mu' which has been put inside that will come in a continuum. So the letters come in a continuum. That is called the, the syllables are not separated. So it will be effectual speech and syllables run into one another. Okay. So it is mixing of syllables in a continuous way without breaking it. That is fast. So you said that it is effortful. So whether there is an element of spasticity in his speech, that's what I thought from the way you said. So that is spastic. Then you come to ataxic. Could be scanning, or it could be, uh, scanning speech when it is split into syllables. When you say 'chakrai' and you say that, so that is a typical scanning speech of cerebellar disease. Cerebellar speech can also produce an explosive speech. So what is there? So if you say 'soak', 'some', 'point', suddenly you make an explosion where it is really not needed. So these are the 'ste' speech and the scanning speech. The 'ste' speech is where you have got an explosive character coming in between where it is really not warranted. That is 'stetto' speech. So ataxic may be scanning or 'stetto' when there is an explosive component. Scanning when the syllables are split into their subcomponents. Then you go to the dyskinetic. Dyskinetic may be hypokinetic or hyperkinetic. Hypokinetic is Parkinson's disease. So like the bradykinesia and movement, movement, hypokinetic speech. Patient will start his talk very loud. As he keeps on speaking, the voice volume will progressively go down. It is like micrographia. Yes. Progressive smallness of letters. Progressive decrease in the voice volume. So that is Parkinsonian speech, hypokinetic. When he speaks, he starts, it is little louder, and as speech progresses, it becomes lower and lower voice volume. That is hypokinetic. Hyperkinetic is of the tongue, tongue tremor, tongue dystonia. So here what happens? Tongue tremor. So when he's speaking, the words which contains the 'l' also, it will become shaky. The letters which words which need the lingual become shaky. Yes, that is tongue tremor. Yes. Voice tremor is different from tongue tremor. Voice tremor is seen in old people, they have a shaky voice. Whereas tongue tremor is, it is a lingual switch which shakes. That is tongue tremor. Tongue dystonia. Dystonia, when like any other choreic movement, rheumatic chorea can manifest in the tongue. When the choreic movement comes, suddenly the patient will stop the speech, and when that chorea has disappeared, you will start. So sudden unwarranted stops during communication is very typical of choreic speech. Sudden unwarranted stops, choreic speech. Then what about dystonia? When the patient says, "When I am speaking, suddenly my tongue twists and I am forced to stop." That is dystonia. That is hyperkinetic. Under hyperkinetic speeches, you have got tongue tremor, you have got chorea, you got dystonia. So then there is something called cortical dysarthria. Cortical dysarthria is very peculiar. It is like a childish speech. All the words are there, grammar is there, everything is there. But you know, you might have seen mentally abnormal children, even when they grow up into adults, they will speak like children. So it is due to some disorganization in the motor input into the Broca's area, commonly seen as an inherited condition in a childhood developmental condition where people are m and their speech does not mature into the adult type of word word handling. Instead, it has got a childish. Sometimes in an acquired lesion to the Broca's area which keeps the Broca's area intact but connections to the Broca's area are partly affected, they get that childish type of speech in later age group as an acute condition. That is called cortical dysarthria. It's an exclusion diagnosis. Sometimes you get Broca's also has got a dysarthric element, but that is an aphasia, not a dysarthria. No. Yes. So you understood? So your patient's speech appears to be probably because you said it is effortful. So now, if you consider the whole thing is purely motor, agreed, asymmetrical to start with, and it picked up distal muscles first. Yes. And probably it picked up the EHL dorsiflex the ankle. Then what other muscles are involved in between? We do not know, but probably picked up the hip extensors and the spine extensors, the obliques, then the upper limb distal muscles and the proximal muscles, and now the person has got probably some upper motor neuron involvement evidenced by the speech. Yes. Then you said that he has so this is the, uh, structures involved in short. These are the structures involved. If it is in the lower motor neuron, we do not have any evidence from the history which you have provided to say that there is upper motor neuron, except the spastic, probably it is spastic speech. I have to hear that speech, then I will be able to clarify it for you. But if the speech is spastic, it is some upper motor neuron element. Element is there. Then you said that he's having some twitching moment. Where did you see? Patient said or you said? You asked what areas? Patient's wife noticed, ma'am. Over the arms. Yes, ma'am. Over the arms, thighs, and, uh, when, uh, later phase, they also noticed over the tongue. So it is diffused. Yes, ma'am. Both upper limb, upper limb, lower limb, and tongue. Yes. Did the patient say or the wife only found out? Why? So it is objective, not subjective. So widespread fasciculations which are objective, not subjective, in muscles which are already weak and weak, that is pathological. You see, you can have physiological fasciculations. Pathological fasciculations are objective. They are seen in weak muscles and they are widespread. Yes. Whereas physiological fasciculations happen when you are tired or when you are under stress, and especially we call third-year syndrome when we suddenly go into third year and see a very severely damaged patient, gets frightened and start teaching. No. So this will be subjective teaching. Objectively to demonstrate it is very difficult. Patient will tell, "When I am in my room and I am studying medicine, I am getting twitching over my deltoid." No, okay. You show me. Patient will say, "Now it is not there." So that is more subjective. So subjective twitching and very rarely objective, that is one point for physiological or psychological. Second, the muscle is having normal bulk, normal power. And the third, it is usually in one site, not widespread. So subjective, localized, and normal muscle, that is more physiological or psychological. Electrophysiologically also, their unique characters are there that we will not go into now. That will you will study when you become DM neurology. Okay. So now there is a widespread movement which is objective, seen in wasted, weak muscles, and it is widespread. So it could be anything like fibrillation, fasciculations, myokymia, cramps, contractions, so many things. So last class I discussed something about cramps and contractions, I think, or the class before, I don't know. So now you said it is a fasciculation. Why did you think it's a fasciculation, ma? In other words, you can get fibrillation, you can have fasciculations, you can have myokymia, which are painless movements. Whereas cramps and contractions are painful movements. So this is painless. So it could be fibrillation, it could be fasciculation, or it could be myokymia. How do you differentiate? Fasciculations can be seen with naked eye, whereas fibrillation difficult or it's not able to see. Agreed. So fibrillations are not visible to the human eye except in the tongue, whereas fibr, fasciculations are visible. So in this patient, it is not in the tongue. In the tongue also it was there, but it is in the whole body. So when the whole body, it is visible, it is less likely to be fibrillation. So you thought it is fasciculation. What else more? How to differentiate from myokymia? Myokymia will happen in motor radiculopathies. Motor neuropathies. So motor radicular and neuropathies, it will produce myokymia. So how do you differentiate fasciculation from myokymia? Myokymia, there also involuntary muscular movements, ma'am. Can be. That's why it becomes a differential diagnosis for this case. Yeah, like has a vermicular contri movements. So it will be a shifting movement, verified by tapping the muscle. Thereby it indicates it is not due to myotatic irritability. Fasciculations, there is increase. So it will not shift. It involves one motor unit. So it will be in that place only. So fasciculations are not showing the vermiform shifting movement. They are in that place only, and they can be aggravated by hyperventilation and they can be aggravated by tapping the muscles. By hyperventilation, what happens? There is alkalosis. Ionic calcium comes down. Irritability of the muscle becomes more. So it becomes visible. By tapping also, you are producing the phenomena called myotatic irritability. Yes. Whereas myokymia is ephaptic conduction between different motor groups, muscle groups. So by conduction of the muscle membrane, they become activated. So it's a vermiform movement. It does not become aggravated by hyperventilation. It does not become aggravated by tapping. Yes. So that is how you differentiate. So vermiform movement was not there, and you could not aggravate it by tapping. You could not aggravate it by making the patient hyperventilate. So probably it is fasciculation. So theory shape sake, I told that myokymia is ephaptic conduction from adjoining muscle groups. Adjoining muscle groups following acute denervation. Acute denervation or chronic. Whereas fasciculations and fibrillation are chronic denervation. Yes, myokymia is acute. Following acute denervation, neighborhood muscle fibers become ephaptically activated, resulting in a vermiform movement. That is myokymia. So Guillain-Barré syndrome, acute radiculopathies of any cause, no, you get that. Whereas fasciculation or fibrillations are chronic. So what is the mechanism in fibrillation? Pathogenesis of fibrillation. Uh, why there are? There are visible fine, ma'am, fast movements like, uh, because of contractions of motor unit. Ma'am, fibrillation is contraction of, not motor unit. Fibrillation is spontaneous contraction of a single muscle fiber. Yes. Single muscle fiber, not motor unit, due to hypersensitivity to the chemical environment. You know that muscle fiber is like this, and the end plate is coming and joining that. So if the single muscle fiber is disconnected from that end plate, the acetylcholine released will not be in the myoneural junction. The acetylcholine released from that terminal axon will be smeared over the muscle fiber. So it's a single muscle fiber which suffers denervation supersensitivity to the chemical environment, that is acetylcholine. So a single muscle fiber which is disconnected from the terminal axon will twitch because of the acetylcholine that is smeared over that. That is fibrillation. Yes. It's not motor unit. It is a muscle fiber. Whereas fasciculation is due to motor unit spontaneous contraction of a single motor unit due to ephaptic conduction in the terminal axon. Fibrillation is in the muscle fiber membrane where the acetylcholine is smeared. Whereas terminal axon is there. A group of muscle fibers are there. The terminal axon is connected by a group of muscle fibers. But this terminal axon is unable to go retrograde to the cell body. So whenever an activity is starting in the muscle fiber, it goes, ascends through the axon, suddenly it finds its pathway is blocked, it cannot go up, so it comes back and puts into action the same motor unit. So fasciculation is due to ephaptic conduction in the terminal axon, putting into spontaneous activity a group of muscle fibers. Yes. Whereas fibrillation is sensitivity to the chemical environment of a single muscle fiber, and myokymia is acute denervation producing contiguous muscle fiber activation by coniguity. It is coniguity, nearness of the muscle fibers that makes it contract. That is myokymia. Yes. So you are correct. It may be fibrillation in the tongue. It may be fasciculation in the, uh, limbs. So purely motor, patchy, predominantly element, plus some, and fasciculations. So your closest possibility, you only know. Yes. So what is the possibility? It could be like both and element. Yes. So it may be both upper motor neuron and lower motor neuron. So if you are still thinking of muscle, myoneural junction, no, yes. So purely motor, purely motor will be muscle, myoneural junction, motor roots, no, yes, or upper motor neuron. All are possible. So if you think it is purely motor muscle, then you cannot explain the upper motor neuron involvement. If you are thinking neuromuscular junction, again, you will not get upper motor neuron involvement. No. So because of the involvement of the upper motor neuron, many things become deleted. Yes. You see, motor roots become deleted, muscle become deleted, myoneural junction become deleted. So when there is a combination of upper motor and lower motor, there are only few differential diagnoses. No, okay. That we will keep it there. And here I will tell that even otherwise, I, as I have told, myoneural junction not considered. I'm just telling pure motor syndromes. If that is the situation here, we will not consider myoneural junction. We can start distally, distally, asymmetrically, whereas proximal asymmetrical may be myoneural junction, limb girdle type of myasthenia. Whereas distal is not at all myoneural junction. Motor radiculopathy is a possibility. We considered. But that will not produce upper motor neuron. Yes. Muscle diseases, distal, there are, but they do not start at this age group. At this age group, the muscle disease which manifests is polymyositis. Paraneoplastic polymyositis. If you have a malignancy somewhere and polymyositis has a paraneoplastic manifestation, that involves the bulkiest muscles, neck muscles, esophageal muscles. So it is not likely to be polymyositis. It's not painful. So late onset muscle diseases are paraneoplastic polymyositis or metabolic myopathies like hypocalcemic osteoporosis, osteomalacia related metabolic myopathies, diabetic neuromyopathies. No, so those are the things. They all involve the proximal muscles. So in this age group, muscle disease, myoneural junction, even if there is, if the UMN involvement is not at all possible, muscle diseases of this age group, not at all possible because it started distally. No. Yes, sir. And with the involvement of UMN, you are thinking that differential diagnosis becomes very less. Yes, sir. So bulbar muscles and all four limbs and trunk muscles are involved. There is an element. So the differential diagnosis in this age group for bulbar and limb weakness. If you have not examined the patient, it can be a craniovertebral junction disease where you can have lower cranial nerve palsy which produces the bulbar symptoms. Lower vertebral junction anomaly, high cervical cord compression, lower cranial nerves will be paralyzed. So you'll have bulbar symptoms and you'll have weakness of limbs which are mainly upper motor neuron. So craniovertebral junction problems will produce upper motor neuron type of weakness at the limbs and bulbar muscles. Yes. Second, you can have multiple infarct. This person has got hypertension. You can have multiple infarct that can produce pseudo bulbar palsy. Okay. Yes, ma'am. Vascular pseudo bulbar palsy. There also the weakness is, um, all are mostly it is motor. It can produce bulbar symptoms. It can produce limb symptoms. But that pseudo bulbar of vascular, you'll have to differentiate from degenerative. That I'll come to later. So second possibility in this age group, considering his comorbidity, imagining that the limb is upper motor neuron, will be vascular pseudo bulbar palsy. Third, you can have medial medullary syndrome. That's a medial medullary artery infarct. That will involve bilateral pyramidal weakness and bulbar muscles. So medial medullary artery when it is infarct. Patient will present with weakness of all four limbs and he will have lower cranial palsy. Posterior column will be elicited on examination, and the weakness will be upper motor neuron in the limbs. Yes. So now let us imagine our patient's limb weakness is not upper motor neuron because you did not give us the symptom of spasticity or stiffness or heaviness or anything. So let us imagine my patient's limbs are involved in the lower motor neuron fashion. So if it is involved in the lower motor neuron, distal asymmetrical to start with, with fasciculations, element, and there is only one diagnosis, it could be an anterior horn cell disease. Disease. So anterior horn cell disease at the bedside is classified as purely LMN, purely LMN plus. But in this age group, if it is because we don't have, we are blind, we don't know whether it's UMN or if you think it is all those DDS, you have to consider CVJ anomaly, medial medullary artery syndrome, which is called ventral bulbar syndrome of Duchenne, or then you may have, uh, brain stem tumors, foramen magnum tumors, no, and vascular pseudo bulbar. Okay. Yes, sir. Supposing it is all LMN, then the possibility becomes anterior horn cell disease, which are classified as purely LMN, under which you have got progressive bulbar palsy, progressive muscular atrophy, and purely you are having ALS, primary lateral sclerosis, and LMN plus is classically ALS. Yes. And purely will be primary lateral sclerosis and pseudobulbar palsy. Yes. Pseudo bulbar palsy and primary lateral sclerosis is purely progressive bulbar palsy and progressive muscular atrophy is purely also. If you think that your patient has only LMN and in the speech, then it will qualify for. So now I want you to, considering 72 years before we go into your examination, then that I want you to differentiate vascular pseudo bulbar from degenerative pseudo bulbar. You know, the pseudo bulbar palsy is a type of upper motor neuron type of antonym. No, and you got a vascular pseudo bulbar. How are you going to differentiate both? Vascular will be acute in onset, but this patient had a, so it may be acute and step-like. So one, one, so many episodes came together in a step-like fashion. One came, stabilized, another came, stabilized. So acute onset, step-like progression is a feature of vascular pseudo bulbar. Whereas slow onset, steady progression is a feature of degenerative. Agreed. Second, but sometimes vascular, if it is a small vessel disease, it will behave like a degenerative disease. Small vessel disease, large vessel disease only will show the acute and steplike, whereas small vessel disease will be slow and progressive. So it can closely resemble because prognosis is different. If a vascular pseudo bulbar, still patient can expect something. If it is a degenerative pseudo bulbar, there is nothing to expect. No. Yes, ma'am. So then, uh, next point, associated, like, weakness, fibrillation. You said fibrillation, fasciculation. No. Presence of fibrillation and fasciculation is favoring a degenerative. These are not seen in vascular pseudo bulbar. Yes, ma'am. You see, even degenerative pseudo bulbar can have significant stiffness in the limbs. It does not exclude ALS. Limbs need not be always LMN. It can have stiffness. No. So that will closely resemble, but if there are fibrillation and fasciculation, it is degenerative. In vascular, you don't find it. Third point, pseudo bulbar laughter and crying. Yes, is more common in vascular because vascular is again non-selective. Like I told in the lower motor neuron, you have got a motor unit pattern. Yes, in the LMN, whereas motor root pattern in the root for the root is the motor root pattern, whereas motor unit pattern for anterior horn cell. Similarly, same thing happens in the upper motor neuron also. There is selectivity because of that. Patient will have a speech, there may be nasal stridor. You do the palatal gag reflex, it is brisk. So it is eating pseudo bulbar palsy. But the pseudo bulbar crying and laughter may not be dominant. So dementia may not be dominant. So dementia is not dominant and pseudo bulbar laughter and crying is not dominant in degenerative, but they are equally dominant in vascular. So three points: acute onset and step-like is favoring vascular, steady progression is favoring degeneration. Presence of fibrillation, fasciculation favors degeneration. They are not seen in vascular. And the affective component is more affected in vascular, whereas they are not that much affected in degenerative. Whereas asymmetry is common to both. So that doesn't help you. So now considering all these features, it is closer to degenerative. Study dist, probably it is a from the history. Yes. So you'll go to. But always rule out a treatable cause. Always look for the least possibility of a treatable cause keeping it in mind. That is why I discuss all the differential diagnoses. Okay. Go on. Yes. General physical examination. Patient is conscious, cooperative, oriented, poorly built and nourished. BMI 16 kg per meter square, and no peripheral cyanosis, clubbing or lymphadenopathy or edema. Vital pulse rate was 74 beats per minute. Regular rhythm, normal character, good volume, no vessel wall thickening, no radio-radial or radio-femoral delay. Peripheral pulses are well felt. Blood pressure 130 by 80 in the right arm. Supine position. In left arm 126 by 80. Uh, uh, respiratory rate 25 cycles per minute. Abdominal type of breathing. Saturation is 94%. No peripheral. Abdominal means what, ma'am? What is the normal male pattern respiration? Abdominal, male pattern, male, male pattern, abdominal, thoracic, abdominal, thoracic, male pattern is abdominal. So there is reversal. It has become thoraco-abdominal. That means which muscle is affected? Uh, extra diaphragm, oblique, it is moving better. That means intercostal muscles are better. Yes. So reversal of the respiratory rhythm in the male to thoraco-abdominal indicates that his diaphragm is more affected. That is why the intercostals are expanding the chest, but diaphragm is not expanding the abdomen. So the respiration has become thoracic-abdominal. So diaphragmatic muscles are seem to be more involved in this case. Okay. Then, uh, no peripheral thickening seen, ma'am. No markers of atherosclerosis or cutaneous markers. CNS examination. Uh, mental functions, ma'am. Uh, conscious, oriented to time, place, person. Speech and language, actually could not, uh, like, he was able to speak with the help of the speaking valve which is attached to tracheostomy port, ma'am. So, and memory was not intact, ma'am. No. Normal tongue. Tongue. What about tongue? Tongue, uh, we, we could see there were fibrillations were present. Was it atrophy? Was there any atrophy? Yes, ma'am. Atrophy. Was this spastic or flaccid? It was flaccid, ma'am. Okay. Uh, gag reflex. Did you elicit gag reflex? Yes, ma'am. Gag was present, ma'am. Present. So that means it is even LMN gag can be present because as I told, unlike the other kinds of LMN like myopathy, if it is an upper motor neuron myopathy, you expect the gag to go. Whereas in anterior horn cell disease, that can be present. Even if it is. Did you record the speech? The speech is not there, no, for me to hear and see whether it is. Didn't record. Okay. Next time, some of the things we'll try to demonstrate. Yes, ma'am. So that we will, uh, see whether it is what exactly it is. Okay. Okay. Motor system, attitude, supine position, extension in the both hip and knee joint, plantar flexion at the both ankle joint, and there was a muscle wasting was there, ma'am, at, uh, and bulk was, uh, actually, there was a wasting, ma'am, right more than left, right arm more than left, and, uh, all over the muscles, there was a wasting was there, ma'am, and tone increased in all four limbs, ma'am. And the power, ma'am, shoulder flexion, extension, adduction, abduction, uh, uh, there was 3 minus by five, and elbow and wrist, elbow flexion and extension, 2 by five, wrist flexion and extension, 2 by five, and there was weak hand grip was there. Both side hip, uh, lower limb, hip flexion, extension, abduction, adduction, there was 1 by five, ma'am. Both side knee flexion, extension, 1 by five. Ankle dorsiflexion and plantar flexion, 1 by five. Extensor hallucis longus, flexion and extension, 1 by five. And the involuntary movements were the fasciculations. And gait, we could not assess, ma'am. Superficial reflexes, corneal reflex was present, and plantar was absent, ma'am. Deep tendon reflexes, ma'am. Plantar is absent because you hardly have any movement in those muscles. So even if it is upper motor, nothing you can elicit. Abdominal reflex is absent. So in this context, what you should do more? You want to know whether it's LMN or UMN, you should elicit the deep abdominal reflex because speech, you told. So you should look for other evidences for upper motor neuron involvement. So LMN weakness, both superficial and deep abdominal reflex will be absent. In UMN weakness, the superficial abdominal reflex will be absent. Deep abdominal reflex will be present. So that will help us to know whether there is an upper motor involvement contributing to the abdominal reflex. So some, some signs of, you can look at the jaw. Gag reflex is not going to help you to differentiate. Even pure LMN gag will be preserved in anterior horn cell disease. So we don't know. But if it is absent, it will help us. If it is present, it's not going to help. Jaw jerk will help, and superficial abdominal and deep abdominal reflexes will help. Plantar will not help because the muscles have no power to extend or flex. So it will not show plantar. Then other reflexes, reflexes, they are all indicating an upper motor neuron component. Yes. Speech and such weak muscle showing three plus reflex is an upper motor neuron component. If you have elicited the jaw as well as you have elicited the deep abdominal reflex, uh, it would have been a very good point for you. Okay. Go next. Sensory system was intact, ma'am. Cerebellar system could not. Meningeal signs, skull and spine was normal, but no deformity or scar at the vertebral level. So after examination, you did not find posterior column involvement. So it is not a ventral bulbar syndrome of Duchenne. You did not find, um, any evidence for restricted neck movement. So you did not find painful restriction of neck activity. So it's less likely to be craniovertebral junction. And in the lower limbs, everywhere it is LMN with retained reflex. Yes. So LMN. So this diagnosis in your case is straightforward, but you should not miss a similar case of other etiology. That is why you have to do symptom. A given case may be easy diagnosis, but another disease masquerading like an MND. You diagnose MND means it is a fatal diagnosis. Yes. Fatal diagnosis should be the last one. No. So you should always remember an elderly person. It can be CVJ, it can be vascular pseudo bulbar, it can be ventral bulbar syndrome. No, all these things you should keep in your mind. In your case, it looks like straightforward because so much LMN with very well elicited reflex. Yes, ma'am. And no bladder, no bowel, and there is no sensation at all, including posterior column. So it looks like a degenerative disease. But, um, um, okay. Next, why indeed he developed acute respiratory distress? Most probably he would have aspirated. Yes, ma'am. Otherwise, even diaphragmatic paralysis, they can manage without. Without diaphragm, will resemble cardiac failure. Your diaphragmatic paralysis will produce paroxysmal nocturnal dyspnea. So you'll think cardiac failure because when you lie down, the diaphragm goes up. So patient will develop nocturnal dyspnea. It can be mistaken like that. That history you did not tell. Uh, but did he have any aspiration? Yes, ma'am. He had aspiration, ma'am. That may be the cause for acute deterioration. Yes. But, um, okay. So you had the diagnosis of MND. Yes, ma'am. Why there is crying and laughing? I will tell you, ma. So you see, why this tracheostomy? Because if you know the diagnosis as MND, the law tells you not to do tracheostomy, not to connect you to the ventilator. You see, because it is torture for the patient to have a hole in the neck and die. Better to die without a hole in the neck. The law protects you. If you know the diagnosis as MND, okay, then you should not ventilate the patient. Yes. Because it is torture. He's a teacher. He will know. He may want to sing Narium and die. You see, the very highly teachers are very great people. They are altruistic and unselfish and they know what to do with their life when it becomes no more useful to the world. So law protects you from doing a tracheostomy or ventilatory support because it is a point of no return. If you do not know the diagnosis and patient presented with acute pneumonia and you are still investigating to know whether he has a CVJ anomaly, you can do tracheostomy. Otherwise, you examine the patient, diagnosis straightforward. I think it is straightforward. In that situation, you should not do. So this is a masterly inactivity. We tell some situation, you have to apply masterly skill to be inactive. You have a stick in the neck is more than death. No. So that should not happen and legally you are protected. You are not supposed to do that. Okay. Doesn't matter. Somebody asked me, what is why there is no crying and laughing in pseudobulbar? You see, in pseudobulbar of vascular nature or whatever nature it is, the corticobulbar fibers which come from the motor cortex to the brain motor nuclei. So motor cortex to the brain motor nuclei alone degenerate in degenerative pseudobulbar in the initial stage, or if you can have some combination FTDs, those syndromes are different. The classical degenerative anterior horn cell disease, it is the corticobulbar fibers from the motor cortex to the bulbar motor cranial nerve that degenerate. So it is only purely motor pseudobulbar. So you have a speech, you got bulbar weakness, and strong, uh, gag reflex. That is the degenerative pseudobulbar. Whereas in the vascular pseudobulbar, it is not just the motor cortex, it is the prefrontal, prefrontal, all those areas. It is ACA territory or the MCA branches which supplies the posterior part of the frontal lobe or multiple small vessels. That is non-selective. It involves the frontal lobe. So not only the motor cortex, frontal lobe to the brainstem structures. What does the frontal lobe do to the other areas? There is frontal monitoring of limbic function. Limbic function means it includes care for the young one, love for the N1, laughter, smiling, everything is a limbic function. So frontal lobe monitors our limbic functions. That is why we don't go and laugh with strangers. We do not cry and share our emotions with people who are cold to us. We share it with someone who will give you a shoulder. So that is called frontal monitoring of limbic function. In vascular pseudobulbar, it is non-selective frontal lobe involvement. So not only the motor cortex to the bulbar functions that are affected. It is a frontal monitoring of the limbic function is also affected. So you're unable to control your crying. You are unable to control your laughter. You cry without stimuli. Laugh without stimuli. Cry with strangers. Laugh with people who laugh at you. You see, that is because in vascular, it is non-selective. It affects the motor cortex, the bulbar motor nuclei, resulting in swallowing difficulty and speech, and prefrontal region controlling the monitoring of the limbic functions. Frontal monitoring of the limbic expression in the social setting is also affected because it is non-selective. So the patient will laugh and cry without concern for the one who is listening to you. Is he your loved one? Will he understand your emotion that you are not knowing? So you'll cry and laugh without appropriate stimuli, without appropriate person. That is why there is laughing and crying unmonitored, unstimulated, with un unacceptive environment in vascular. Whereas in degenerative, those frontal areas are not affected. Only the motor cortex to the bulbar nucleus affected. So there is only speech, swallowing defect. As the disease advances, you know that there is also also degenerate at that end stage, they can have pseudo bulbar laughter and crying also. Understood, ma? Is my answer clear? Yes, ma'am. Any other question? Any other question? No. Is this answer clear? Ma'am, if anybody did not understand, you can ask me. Frontal lobe is not just a motor lobe. Frontal lobe has got a pre-central motor strip, but rest of it is behavior and limbic functions. So frontal monitoring of the limbic function is another function of the frontal lobe. Because of the non-selective involvement in vascular, that also goes. That is why you laugh and cry. Okay. This is a wasted hand, wasted leg. A great show. Anything else is there? Tongue is there. Show the video. Video is there. Yeah, it's there. Then show one second. It's not able to play, ma'am, here. Okay, no problem. Any other doubt, ma'am? So if you want to tell etiology, just one second, ma'am. I got the video. I'll just share it in a minute. Sure, sure. Meanwhile, you can say that cause of these diseases, we say inherited superoxide dismutase happens in young onset MND. Late onset can be paraneoplastic. This person progressed very rapidly. So you'll have to look for paraneoplastic. Sometimes chronic organophosphorus compound poisoning, heavy metal poisoning, then you have got acquired superoxide dismutase deficiency resulting in oxidative stress. Then some other postulates are slow virus diseases and again immune mediated. So these are the, in young people, it is superoxide dismutase deficiency, rapidly progressive and honel. You should always look for paraneoplastic syndrome. Then heavy metals like mercury is incorporated. Slow organophosphorus. Then oxidative stress and immune mediated possibility has been postulated by a postmortem analysis of spinal cord of these patients where people found that there is accumulation of immune complexes. So in some centers, especially popularized by NIMS by Professor Govi Devi, use of cyclophosphamide, but it did not show any long-standing benefit. So it has been removed. That was based on spinal cord postmortem analysis where people found immune complexes. Then people have used antiviral agents because of the slow virus possibility. Now we see that sometimes MND and myasthenia coexist. So the possibility of other immunological like acetylcholine receptor antibody, like antibody mediated possibilities are also being considered. So immunotherapy including IVIG is being tried. So oxidative stress, you treat with antioxidants, and you have got Riluzole, a strong antioxidant. Cyclophosphamide was tried suspecting the possibility of based on the seeing of immune complexes. Then plasmapheresis, IVIG based on the new syndromes of myasthenia and MND coexisting. All those things are there. But as I have told, it is supposed to delay the ventilatory stage of the patient by three months. That is all. And whether it is significant or not, we do not know. And this is one disease where the rule forbids that you intubate or do any massive investig, massive, um, restorative procedures because a conscious patient will struggle with his life. No. Yes. Okay. [Music] It's an atrophic tongue with a central depression and definitely fibrillations are there. It is very much visible. Did you look for any occult malignancy? No. Um, yes, ma'am. Like, no, it was not there. Okay. Yes. Too rapid. You said that within one year he became like this. Yes, ma'am. Indian ALS study says that people live up to 11 years. No. So this is very rapid progression. Some kind of tumulousness is there in the limbs, I think. Yes. Okay. So it's a straightforward, but only thing is too rapid because Indian ALS has got a slower progression than Western ALS and they live much much longer. So what made it very fast? That is the only odd thing. So we'll have to, even though he is end-stage disease, paraneoplastic antibodies, some occult malignancy somewhere, you'll have to rule out because it is too rapid. Indian ALS study done by, uh, some people in NIMS only and that is published. It shows that they live up to 11 to 12 years. No. So this is very rapid. That is the only feature which need to be addressed in this patient. Any other question, ma'am? Any other question? No. Okay. You, these are straightforward cases, but I have, um, repeated some of the points which discussed before, and each symptom you are presenting, I am teaching you how to analyze so that any other case which is a close imitator will not be missed. Yes, that is why that is how I am planning because you know that more number of doctors are developed for manpower development of rural India. That is the aim of the government to develop manpower for rural India. You should not ask that I want five Tesla, seven Tesla, MR everywhere, then only I will diagnose. And MRIs are much inferior computers compared to your computer, provided you consider your life as a submission to almighty. Yes, that is the thing. People will beat us, they will hit us because the society consists of all kind of people. But we cannot degrade ourselves like that. We will remain sustain our contract with almighty so that our jma becomes pun. Yes. Thank you so much. Thanks a lot, ma'am. Thank you. Thank you all. Any other doubt is there? I will answer. Anything? No. No. So should we close now? Yes, ma'am. Ma'am, it was indeed a comprehensive clinical class. Uh, thank you Professor Chandra, ma'am, for the wonderful discussion and your valuable inputs, ma'am. Uh, thank you, Dr. Sachin, for, uh, the amazing presentation. We could learn a lot from it. Uh, thank you all the participants for your active participation. Uh, thank you one and all. Thank you.