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120 Movement Disorders

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Cerebral Palsy

  • Lifelong physical disability caused by damage to the developing brain; it is non-progressive.
  • Typically becomes evident within the first 12 to 18 months of life.
  • Etiology: Often idiopathic, but some identified risk factors include:

Risk Factors

  • About 50% of CP patients are born premature.
  • Low birth weight.
  • Maternal infection during early pregnancy.
  • Prolonged loss of oxygen during pregnancy or the birthing process.

Clinical Presentation

  • Speech and Language Issues
  • Dysarthria: Difficulty articulating words.
  • Language delay: Inability to speak simple sentences by 24 months.
  • Musculoskeletal Symptoms
  • Low muscle tone.
  • Difficulty holding the head up.
  • 75% of patients present with spasticity and contractures, often unilateral.
  • Poor muscle coordination.
  • Delay in walking development by 12 to 18 months.
  • Cognitive and Developmental Delays
  • Feeding or Swallowing Difficulties

Labs, Studies, and Physical Exam Findings

  • Physical Exam:
  • Slow motor development.
  • Abnormal muscle tone: either hypertonic or hypotonic.
  • Presence of contractures and spasticity.
  • Missed developmental milestones.
  • Imaging:
  • CT or MRI may reveal brain abnormalities or damage.

Treatment

  • No definitive cure exists.
  • Preventative Measures
  • Ensure baby is carried to term.
  • Magnesium sulfate administration to prevent preterm labor.
  • Medical Treatment
  • Benzodiazepines for muscle spasms, e.g., diazepam.
  • Botulinum toxin type A injections for spastic muscles.
  • Antiepileptic medications to control seizures.
  • Surgical Interventions
  • Fasciotomy.
  • Muscle lengthening procedures.
  • Joint replacements.
  • Rehabilitative Therapies
  • Physical and Occupational Therapy.
  • Massage Therapy.
  • Educational and Psychosocial Support
  • Special education plans.
  • Behavioral therapy and counseling.

Differential Diagnosis

  • Muscular Dystrophy
  • Spina Bifida
  • Childhood Stroke
  • Other metabolic or genetic disorders

Multiple Sclerosis (MS)

  • Demyelinating disease affecting the central nervous system (CNS); exact etiology remains uncertain.
  • Autoimmune destruction of myelin.
  • Failure of myelinating cells.
  • Generally a progressive disease with various clinical courses, most commonly relapsing-remitting.

Risk Factors

  • Geographic location: More prevalent in individuals living far from the equator and in Northern European regions. Risk is retained if the person moves after age 15.
  • Genetic predisposition.
  • Certain viral infections, such as Epstein-Barr virus.
  • Smoking.

Clinical Presentation

  • Relapsing and Remitting Symptoms: Vary from one area of the body to another during each exacerbation.
  • Visual Disturbances: Double vision, nystagmus, partial or total blindness, blurry vision.
  • Motor Dysfunction: Focal weakness, gait difficulties, ataxia.
  • Sensory Changes: Numbness, tingling.
  • General Symptoms: Fatigue, dizziness, vertigo, pain.
  • Emotional Changes: Anxiety, depression.
  • Other Symptoms: Bladder dysfunction, tremor, headache, seizures.

Labs, Studies, and Physical Exam Findings

  • Physical Exam: Neurological assessment.
  • Lumbar Puncture: May indicate chronic CNS inflammation, elevated protein levels, and increased leukocytes.
  • MRI with Gadolinium: Shows areas of demyelination.
  • Evoked Potential Tests: Measures electrical signals in response to stimuli; useful in identifying lesions and nerve damage.
  • Blood Tests: To exclude other conditions mimicking MS symptoms.

Treatment Options

  • Attack Management
  • High-dose corticosteroids: Oral prednisone, IV methylprednisolone (Solu-Medrol).
  • Plasmapheresis for severe attacks unresponsive to steroids.
  • Disease Modifying Therapies
  • Beta interferons.
  • Glatiramer acetate (Copaxone).
  • Fingolimod (Gilenya).
  • Natalizumab (Tysabri).
  • Mitoxantrone.
  • Teriflunomide (Aubagio).
  • Symptom Management
  • Physical therapy: Focused on stretching, strengthening, and adaptive devices.
  • Dalfampridine (Ampyra): Improves walking speed.
  • Baclofen and Tizanidine (Zanaflex): Treat spasticity.
  • Amantadine: Addresses fatigue.
  • Other medications for mood changes, pain, and bladder/bowel dysfunction.

Differential Diagnosis

  • Lupus
  • Sjögren’s Syndrome
  • Vitamin B12 Deficiency
  • Lyme Disease
  • Neuromyelitis Optica

Myasthenia Gravis

  • Autoimmune neuromuscular disorder affecting the interaction between acetylcholine and its receptors at the neuromuscular junction.
  • Acetylcholine usually mediates excitatory signals for muscle contraction.

Clinical Presentation

  • Fatigability: Increased muscle weakness that improves with rest.
  • Ocular Symptoms: Ptosis (drooping eyelids), diplopia (double vision).
  • Oral and Pharyngeal Symptoms: Difficulty in chewing, swallowing, and change in voice quality.
  • Respiratory Issues: Difficulty breathing, potentially progressing to respiratory failure (known as myasthenic crisis).

Labs, Studies, and Physical Exam Findings

  • Physical and Neurologic Exam
  • Often unremarkable but may show easy fatigability.
  • Weakness that improves after rest and worsens on repeat exertion.
  • Intact reflexes and sensation.
  • Blood Tests
  • Acetylcholine receptor antibody test.
  • Ice Test: Improvement in strength following the application of ice.
  • Edrophonium Test: Short-acting anticholinesterase aiding in diagnosis.
  • Imaging: CXR, CT, MRI to check for thymoma or other tumors.
  • Nerve Conduction Studies: To evaluate nerve-to-muscle signal strength.
  • Electromyography (EMG): Shows unresponsive muscle fibers to stimuli.
  • Pulmonary Function Tests: To assess respiratory function.
  • Muscle Biopsy: Rarely used but may offer additional insights.

Treatment

  • Pharmacotherapy
  • Acetylcholinesterase Inhibitors: Neostigmine and pyridostigmine prolong the action of acetylcholine.
  • Immunosuppressive Agents: Prednisone, cyclosporine, mycophenolate, and azathioprine often used in conjunction with acetylcholinesterase inhibitors.
  • Emergent Therapies
  • Plasmapheresis and IVIG to remove circulating antibodies.
  • Surgical Treatment
  • Thymectomy: Particularly in the presence of a thymoma.

Differential Diagnosis

  • Lambert-Eaton Myasthenic Syndrome (LEMS)
  • Ocular Myositis
  • Drug-induced Myopathy
  • Guillain-Barré Syndrome

Key Distinctions

  • Cerebral Palsy mainly affects motor skills and muscle tone, and it is not progressive.
  • Multiple Sclerosis involves an autoimmune attack on the CNS and presents with a wide range of neurological symptoms.
  • Myasthenia Gravis focuses on muscle fatigability due to impaired neuromuscular transmission and usually starts with eye symptoms

Full Transcript (click to expand)

Hello and welcome to the Physician Assistant Exam Review Podcast, episode 120. I’ve got something really exciting for you today, something I just finished putting together this morning. In fact, what it is, is it’s an active guide to today’s episode. What I mean by that is I talk a lot about active versus passive studying, and listening to this show tends to be more passive, right? You listen to me teach the material. So what I did is I went ahead and put together what would be a fantastic active review of the material we covered today. And what I put in there was, if you’re already on the email list, you’ll recognize them.

I’ve been writing stories about the different diagnoses because stories stick. Lists don’t stick. Stories, images, those kind of things stick in your brain. So I have three stories about today’s topics. I also put in there PANCE type review questions so that you can walk through how they would look about these topics. It’s not about getting them right or wrong. It’s about seeing the structure of them as you move through the questions that I put in there.

And then I have some discussion topics, meaning if you can go through the ideas that I put in that guide and you can speak the answers to them out loud, whether that’s to a friend or a classmate or a cat or a dog or whatever, you will find a tremendous increase in what you retain from today’s episodes. You can find that at physicianassistantexamreview.com/120. There you can find the notes for today’s show as well as that active study guide. I think it’s going to make a huge, huge difference for you.

All right, let’s go ahead and get started. My name is Brian Wallace. I’m the host and creator here at Physician Assistant Exam Review. And today we’re going to be covering movement disorders as we make our way through neurology. We’re going to cover just three topics today. Cerebral palsy, myasthenia gravis, and multiple sclerosis. Multiple sclerosis is easy for me to say.

Those are going to be our three topics for today as we go through this.

All right, let’s hop right into our priming questions. What are the common risk factors for cerebral palsy? What are the common risk factors for cerebral palsy? What is the key term describing symptoms of MS? What would you be looking for? What would you hope to see in your questions if they were looking for the diagnosis of MS? What are the crucial lab tests and imaging studies for diagnosing MS?

What is the clinical finding you’d most expect to see with myasthenia gravis? Again, what would you want to see in that question stem? What would you hope to see? Number five, name two acetylcholinesterase inhibitors.

All right. I am really excited to get started on this particular show. I’ve been putting together the registration for the 33 Days to start to pass the PANCE program, the November cohort. Really excited about that group. Really excited to be getting off the ground with them this week. And I’ve been doing a couple interviews over the past couple shows. So this is back to the content stuff, how I built the program.

So I’m really excited to be back today behind the microphone doing the content review.

So let’s go ahead and get started with cerebral palsy. Cerebral palsy is a lifelong physical disability and is caused by the damage to the developing brain. It is not progressive. This is something that happens at birth, right around birth. And it typically becomes evident within the first 12 to 18 months.

Okay. So a lot of times this is idiopathic. Not sure why Siri’s chiming in there. A lot of times this is idiopathic, but there are some risk factors that go along here. So again, just so you have it in your head, this is a lifelong disability. There is no cure for this, which we’ll talk about when we get to treatments, but it’s not progressive. The risk factors are that about 50% of CP patients are born premature.

They tend to have a low birth weight. They may also have a maternal infection during early pregnancy or a prolonged loss of oxygen during pregnancy or the birthing process. So those are your risk factors. Clinical presentation. They can have speech and language problems with difficulty articulating words or language delay. Again, it’s in that first, they may not be able to speak or have trouble making complete sentences by age two. Musculoskeletal symptoms, they can have low muscle tone, difficulty holding up their heads.

They will often, and most of them, 75% or more will have spasticity and contractures, often unilateral. My nephew has this and his right side is, he has a right-sided contracture on his wrist. We’ll talk about that a little bit more as we go. They tend to have poor muscle coordination. And just like the delay in speech, they have a delay in walking. They can have cognitive development delays and feeding and swallowing difficulties. Again, it all depends on where the damage or issues are.

On physical exam, again, they have slow motor development, abnormal muscle tone, either hypertonic or hypotonic. Like I said, one side is contractured, other side is weak. And again, missing those developmental milestones should be a red flag for this because it may not be evident at birth. It may take a little bit longer. And a CT or MRI may reveal brain abnormalities or damage. Treatment, there’s no definitive cure here. What we’re trying to do is get full gestation, right?

So we’re trying to carry the baby to term. So there are some things we can do, although we can’t cure it. There are a lot of things we can do to improve functionality, improve day-to-day activities. So you can use benzodiazepines for muscle spasms. Not great long-term, but it can work. Botulinum toxin type A injections for spastic muscles. That can certainly work to help break that muscle tissue up.

Anti-epileptic medications if they have seizures. Physical and occupational therapy is going to be really important for these individuals. Massage therapy, again, depending on those contractures and how their muscles play in. They’re going to need help in school. So behavioral therapy and counseling, education programs. And then there are surgical interventions, but this is, again, not curative. This is to help with like some of those contractures.

So, for example, fasci… fasciotomies, muscle lengthening procedures, joint replacements down the road if they have problems. So, it’s not like we’re treating the CP with surgical interventions. But what we’re doing is, like again, my nephew who has his wrist in a contracture, he had surgery to straighten out his wrist and fuse it out straight. This may sound like not a great plan, but he can now use, he has some function in that hand that he never had before. Your differential diagnosis here are things like muscular dystrophy, spina bifida, childhood stroke, and some other genetic or metabolic issues. We’re not going to go down that path too far today.

Our next one is multiple sclerosis. MS is a demyelinating disease affecting the central nervous system. We’re not really sure 100% why this happens, but it tends to be an autoimmune destruction of myelin and failure of the myelinating cells. Generally, it’s a progressive disease with various clinical courses, most commonly relapsing and remitting. That’s our key term for MS, right? Is that relapsing and remitting. Symptoms come and then they go.

Again, unlike cerebral palsy, and this is what I want you to be doing is think about how do these compare to the other things we’re talking about? Unlike cerebral palsy, MS is progressive. Risk factors for MS, interestingly enough, geographic location, more prevalent in individuals living far from the equator and in northern European regions. Risk is retained if the person moves after age 15. Not sure that’s going to come up on your exam, but I thought it was interesting. There is a genetic predisposition. There are some links to viral infections like Epstein-Barr, not definitive by any stretch.

Smoking is an increased risk factor. Your clinical description here should be something that sticks in your head for MS. Again, it’s those relapsing and remitting symptoms, so they come and they go. So they can have double vision, nystagmus, blindness, partial or complete. They can have blurry vision. And again, it comes and goes. Motor dysfunction.

So they can have weaknesses in certain areas. They can have trouble with gait and balance. They can get numbness and tingling, fatigue, dizziness, vertigo, tremors, headaches, all sorts of things. But the key here is that they come and they go. That’s really going to be the thing on your test that you’re going to hopefully be able to see that’s going to help you there. Lab studies and physical exam findings. Physical exam, you’re going to do a neurological assessment, of course.

An MRI with gadolinium will show areas of demyelination. At some point, you’re going to order a lumbar puncture, which may indicate inflammation with elevated proteins and increased leukocytes. Evoked potential tests which measure electrical signals in response to stimulus useful in identifying lesions and nerve damage. Then you’re going to get some blood work, but really this is to exclude any other possibilities here. So treatment is going to be a couple different ideas for MS. It’s going to be if you have an acute phase. So when they come in with those symptoms versus trying to keep those symptoms away.

So if someone comes in with symptoms, you’re going to give them high dose corticosteroids, oral prednisone or methylprednisolone, which is also Solu-Medrol. And then you can use plasmapheresis also for severe attacks unresponsive to steroids. That’s pretty significant, but it is possible. Disease modifying therapies, so beta interferons can be helpful here. Glatiramer acetate, fingolimod. These are all ideas. I’m not sure how deep you’re going to get into these, but I listed a bunch here for you.

Excuse me. Also things like physical therapy. Focus on stretching, strengthening, gait training. It’s going to be important for these patients.

Now, these are going to be difficult to diagnose initially because, again, the symptoms come and go. So the patients may not even come in and present initially, or they may present very strange ways. So this is a tough one if you’re in the office. But those visual disturbances, that weakness that comes and goes is really going to be what you’re looking for. So some of the things in the differential would be like lupus, Sjogren’s, a B12 deficiency, Lyme maybe.

All right, and then our last one for the day is myasthenia gravis. This is an autoimmune disease where it affects the neuromuscular junctions at the acetylcholine receptors. And acetylcholine usually mediates excitatory signals for muscle contraction, right? So, these patients will come in with fatigability, increased muscle weakness, but here’s the key. It improves with rest. So, they get muscle tired, but it improves with rest. So, again, go back to what we were just talking about.

Compare that to CP or MS. MS is relapsing or remitting, but doesn’t get better with rest. Cerebral palsy is just consistent. They may get ptosis or drooping of the eyelids, double vision. They may have difficulty swallowing or chewing. A change in voice. Again, we’re talking about things where muscles are affected at that neuromuscular junction.

They can have difficulty breathing. Again, if it’s a respiratory, muscles involved. Lab studies and physical exam findings. You may not see much because of the nature of this disease, right? If you think about how someone may present, oh, I’m tired, oh, I feel weak. But you’re looking for that weakness that improves after rest and worsens when they repeat activity. But everything else is going to be kind of normal unless they have something like the eye issues where they have drooping eyelids or double vision or things like that, which again may point you toward MS, but think about that improved with rest, not just cyclical.

You can do an acetylcholine receptor antibody test for this. Interestingly enough is the ice test, which is the patients will improve in strength following the application of ice. There’s an edrophonium test, short-acting anticholinesterase aiding in diagnosis. So they give the person a short-acting anticholinesterase, and then they get better, right? So anticholinesterase is what they are. They’re enzymes that break down acetylcholine. And if we’re having trouble with the receptor sites for acetylcholine, if we can keep acetylcholine in those junctions longer with a higher concentration, the patient’s symptoms will be lessened.

So we’ll get to that in a minute when we talk about treatments. But what we’re doing here is we’re just doing a short term of getting more acetylcholine into those junctions. And if the patient improves, that’s an indicator that we’re dealing with myasthenia gravis. Thymoma, you can do imaging for chest x-ray, CT, or MRI. Nerve conduction studies to about nerve to muscle strength. So that’s like an EMG. Muscle biopsies could be done, but it’s pretty intensive and pretty rare.

Treatments. So like I just talked about, you can use acetylcholinesterase inhibitors. Neostigmine and pyridostigmine prolong the action of acetylcholine, so they keep that acetylcholine out there longer in higher concentrations. And then again, immunosuppressive agents can be helpful, so like prednisone, something like steroids in general. And then they’re looking into plasmapheresis and IVIG to remove circulating antibodies. Interesting. Patient may require a thymectomy, I’m saying that correctly, if they have a thymoma, which can exacerbate this problem.

Our differential diagnosis here is a drug-induced myopathy, Guillain-Barre syndrome. We’re going to get into Guillain-Barre. I don’t know if it’s going to be next week, but it’s going to be coming up soon. Wow, you would not believe. I just looked at the clock on this episode, and I am stunned at how quickly I can get through this material with how long it takes me to put that together to isolate key factors and go through it with you. So this is going to be a short episode.

Let’s talk a little bit about our study tip for today since we’re just wrapped up. I’m just going to cover those three for today. Our study tip for today is going to be what I talked about earlier, which is that active studying, that progression from just listening to me talk to going back through and saying, okay, how do I compare and contrast these three things? At what ages would these probably come in? When would there be an issue with, let’s say, patient comes in with fatigue, at what point would I start to work them up for these things? Patient comes in with muscle weakness, at what point would I start to work them up for these things? And what would that workup look like?

And to point out that things, like I said, I know I’m repeating myself, but things like that cerebral palsy is consistent. Myasthenia gravis is they get tired and then with rest they improve. It’s important to note those things because the questions are going to come up and have these on the answer choices. You’re going to have to tell the difference between them. So it’s going to be a great way for you to do that is if you practice doing that as you’re listening to the show. If you practice doing that as you are studying your notes. So it’s that active, active engagement.

It’s not just reading and recopying. It’s not just reading and highlighting.

All right, let’s answer our priming questions. What are the common risk factors for cerebral palsy? What are the common risk factors for cerebral palsy? Well, if you remember, there were premature birth, low birth weight, and an anoxic brain issue during pregnancy. So we’re trying to get those. To reduce risk, we’re trying to get patients to carry to term. There’s also the issue where possible infection, right?

What is the key term describing symptoms of MS? That should be pretty easy, that relapsing and remitting of symptoms. What are the crucial lab tests and imaging studies for diagnosing MS? An MRI of the brain and spinal cord. We’re looking for plaques characteristic of MS. What is the clinical finding you’d most expect to see with myasthenia gravis?

All right, so we’ve talked about this several times. It’s that fatigue that improves with rest. And lastly, name two acetylcholinesterase, acetylcholinesterase inhibitors, because those you probably should be able to pick out on your exam. Neostigmine and pyridostigmine.

All right, that takes us out for today. That wraps up one more section of neuro. Again, go over to the website physicianassistantexamreview.com/120 to get the notes for today’s section, but also even more importantly is the active study guide for today’s section. I’m really excited about that. I’d love to know when, once you get it, email me, let me know what you think about it. Let me see if there are ways I could improve it, but I’m really excited about it. I think it’s going to make a big difference.

All right. So that’s going to be it for today. I hope you have a great week and looking forward to people taking their exams in November and let me know how you do and definitely keep in touch. So I’ll talk to you soon. Take care. Bye. Thank you.

More Neuro for the PANCE

132 Neuro: Neoplasms & how to be a better student

131 Neuro: Spinal Cord issues

130B PANCE Prep for Infectious Neuro Disorders

130 Encephalitis Review for the PANCE

See all Neuro episodes →

Get 19 Neurology questions straight from The Final Step.

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