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3-46.0 - Neuro Large Group 3 - Neuropathy/MG/ALS/MS
Terms in this set (50)
Does case 1 appear focal, multifocal, or diffuse?
Focal, the right quad is weaker than the rest
Does case 1 appear to be a central or peripheral disease?
Does case 1 appear to be a peripheral mononeuropathy or polyneuropathy? (1 nerve or many nerves affected)
Mononeuropathy (a single nerve is affected)
DDx of case 1
Peripheral mononeuropathy (amyotrophy)
Mass lesion leading to entrapment neuropathy
Autoimmune diseases (sudden onset)
What tests would you order for case 1 and what are you looking for?
EMG (look for active denervation)
Blood glucose levels
What is the Dx of case 1?
Peripheral mononeuropathy (amyotrophy)
Case 2 eye symptoms (vertical diplopia, hyperdeviation, head tilt) resemble a ___ nerve palsy
4th nerve palsy
A lesion affecting the PERIPHERAL 4th CN would result in (contralateral/ipsilateral) symptoms, while a lesion affecting the 4th CN in the brainstem would result in (contralateral/ipsilateral) symptoms
DDx of Case 2
Oculomotor-pharyngeal muscular dystrophy
What in the history of case 2 indicates it could NOT be botulism?
Timeframe (botulism is more of a short term, 1 day or hours situation)
What in the symptoms of case 2 indicates it could NOT be Horner's syndrome?
The pt has bilateral ptosis, and Horner's is predominately UNILATERAL
Even though Lambert-Eaton spares the CN muscles, why would you still test for it in case 2 with ocular muscle abnormalities (CN 4 palsy)?
LEMS could accompany a malignancy that could cause ocular muscle abnormalities
What aspects of case 2 presentation point you towards MG?
Older male, eye symptoms (90% of MG pts will have eye Sx), dysphagia, dysarthria (hoarseness), dyspnea (respiratory weakness, ptosis gets better with rest (and with MG, fatigue of muscles improves with rest and worsens with sustained activity)
What is the mechanism behind MG?
Autoimmune disease affecting the postsynaptic ACh receptors, preventing Ach from binding with full force resulting in muscle weakness
What tests could you run for case 2 (specifically, to test for MG)?
Ice pack test
EMG - repetitive stimulation
What would you see in an EMG with a pt with MG? What do you see in a normal pt?
Pt with MG - Decremental response (muscles are weak and get weaker with repeated stimulation)
Normal pt - incremental response
What key test would you run to test for MG? What are you looking for? What is a downfall for this?
Tensilon = Achase inhibitor
Looking for increased muscle activity (degree of ptosis in this case) by measuring the time the muscle can be active without fatiguing
What test is a good, less invasive substitute for the Tensilon test? What is the basis behind this?
Ice pack test
Put ice for 2 minutes on the muscles suspected with MG, and see if muscle activity increases because the AChase activity slows down in the cold
Why do these autoimmune diseases that target synaptic junctions tend to target those in the eye? Shouldn't they be ubiquitous and be non-selective?
The sensitivity of the eye muscles make it become symptomatic more often
Once you confirm the Dx of MG, what other test would you run and why?
MRI/CT of the chest, looking for a thymoma
There is a correlation between MG and thymomas
What therapeutics would you use to treat a pt with MG?
Prednisone - steroid (immunosuppressant)
Neo-/pyridostigme - Achase inhibitors
IVIG or plasma exchange
When would IVIG or plasma exchange be a good treatment for MG?
In an ACUTE case
For routine management of MG, what therapeutics would you use?
Probably an Achase inhibitor
In case 3, a steppage gait is a compensatory mechanism for what?
Foot drop (if foot can't dorsiflex, you hike the hip in compensation for it)
In case 3, are the symptoms from lesions of UMNs, LMNs, or both? List some symptoms and classify them
UMNs - spasticity, clumsiness, hyperreflexia, fasciculations
LMNs - atrophy, weakness
What are some ways you can test for hyperreflexia?
Adductor reflexes (crossed adductors)
What is a Hoffman sign? Is this an UMN or LMN sign?
Click the distal phalynx and the hand will contract
DDx of Case 3
PLS (predominately UMN symptoms)
Progressive Supranuclear Palsy
MS usually presents with UMN or LMN signs?
What testing would we order to rule in or out the DDx?
Blood work (looking for Creatine kinase, parathyroid hormone, both elevated in ALS)
EMG of LE
Treatment options for ALS (which one is the only FDA approved treatment, what are you using which drug for what)?
Riluzole: glutamate transmission modulator
* the only FDA approved Tx
* Not a great tx, only prolongs survival and delays progression by 2 months
Dextromethorphan + Quinidine for pseudobulbar affect
Supportive of symptoms
AGAIN: What IS a pseudobulbar affect?
Inappropriate emotions (laughing, crying)
In a majority of ALS pts, what mutation is responsible for aggregation of dismutase protein? What does this result in?
Superoxide dismutase mutation
Results in an inability to remove glutamate properly and causing oxidative injury to neurons
TEST: What other neurologic disease can occur with ALS?
Why do we get an increased expansion of the ventricles in the brain with frontotemporal dementia?
Atrophy results in less mass of the brain compressing the ventricles, allowing them to expand resulting in secondary hydrocephalus
What are some symptoms presented in case 4?
Hemiataxia, nystagmus, vertigo, dysmetria, dysdiadochokinesia
What does dysmetria refer to?
Inability to do point-to-point
What does dysdiadochokinesia refer to?
Inability to do rapid alternating movements
Dysmetria and dysdiadochokinesia are ______ symptoms
DDx of case 4
Acute disseminate encephalomyelitis
What tests would you do and in what order?
MRI (strokes, tumors, MS)
- cervical spine
Bloodwork (CBC, CMP)
Lumbar puncture - look for oligoclonal bands
CTs are good for what?
What is the MRI contrast that you would use?
On MRI, what do you see?
Periventricular sclerosing lesions
Lesion in the cerebellar peduncle
Therapeutically, what would you give for pts with acute MS symptoms? Would you give them/start with prednisone? Why or why not?
High dose steroids (1000 mg IV solumedrol)
Do NOT give them prednisone, because studies showed that it made the symptoms worse initially
TEST: What is the goal of long term therapy for MS? What is this called?
Decrease the number of relapses that occur
This is called disease modifying therapy
What are some choices of long term therapy for MS?
What would you use to treat RELAPSES of MS with significant debilitating symptoms?
High dose steroids
How do you determine if treatment for MS was effective or not?
No further relapses
AGAIN: What was the phenomenon called when elevated body temperature (say from hot baths) unmasks prior MS deficits?
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