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CSI - LEC 4 - ALS/MS
Terms in this set (76)
What is the typical presentation of MS?
Middle-aged woman presents to office with rapid loss of vision in eye & resolved spontaneously, with weakness in legs, & oligoclonal bands in CSF
What are the visual symptoms of MS?
-internuclear ophthalmoplegia (INO)
What are central symptoms of MS?
What are speech & throat symptoms with MS?
Dysarthria (slurred speech) & dysphagia (difficulty swallowing)
What are musculoskeletal symptoms of MS?
Weakness, spasms, ataxia
What are sensation symptoms of MS?
Pain, hypoesthesia, paraesthesia
What are bowel symptoms of MS?
Incontinence, diarrhea or constipation
What are urinary symptoms of MS?
Incontinence frequency or retention
What is the epidemiology of MS?
Most common inflammatory demyelinating disease of CNS
What is the cause of MS?
Unknown initiating cause, but includes auto-immune medicated inflamm demyelination & axonal injury (no viral etiology found)
What geographical pop is most susceptible to MS?
Pops in northern latitudes
What gender is most affected in MS?
What is the typical age range for onset of MS?
Onset b/t 15 - 50 y.o. (patient = usually middle-aged)
What are the pathophysiological events in MS?
1) breakdown of BBB
2) ***periventricular mononuclear infiltrates
3) circumscribed areas of myelin breakdown
What is the cause of myelin breakdown in MS?
-B lymphocytes & plasma cells surround small CNS blood vessels
-T lymphocytes & monocytes infiltrate CNS parenchyma
What may account for brain atrophy & permanent damage seen in advanced disease?
Injury to CNS neuronal axons
What is the differential diagnosis for MS (what diseases present similarly)?
Vasculitis, paraneoplastic disorders, neurosarcoidosis, SLE, Behcet disease, lymphoma, infections (HIV, Lyme)
What are the most COMMON symptoms seen in MS?
1. Sensory loss in limbs
2. Visual loss
3. Polysymptomatic onset
What reflex signs are seen with MS?
Hyperreflexia, Babinski sign, absent abdominal reflexes
What imaging technique can confirm MS?
What is seen in MRI of MS patient?
Multifocal lesions, appear hyperintense on T2 in periventricular white matter, corpus callous, cerebellum, cerebellar peduncles, brainstem, & spinal cord
What enhances acute lesion on T1 images?
What lab technique can confirm MS?
-increased IgG oligoclonal bands & moderate lymphocytic bands in CSF
-moderate lymphocytic pleocytosis (seen in spinal tap)
What is moderate lymphatic pleocytosis?
< 50 mononuclear cells/microliter
What are the different types of MS?
1. Relapsing-remitting MS
2. Secondary progressive MS
3. Primary progressive MS
4. Progressive-relapsing MS
What is the most common type of MS?
***Relapsing-remitting MS (can eventually become progressive)
What is relapsing-remitting MS?
Get better and disability returns to baseline, then relapse and get worse again (after each relapse, the baseline worsens/ disability worsens over time)
What is the treatment for MS with acute exacerbations/ "flaring of weakness"?
3-5 courses of high dose IV methylprednisolone (1.0 g daily)
What is treatment for severe or steroid unresponsive exacerbations?
Plasma exchange (filtration of O-bands)
What treatment decreases relapse rate & intensity of MS?
IFN-beta-1b, IFN-beta-1a, Glatiramer acetate
What is the treatment for very resistant MS?
What is the initial treatment approach for clinically isolated syndrome of MS?
"Safety; tried & true" approach (injection therapy w/ IFN's or glatiramer)
What is treatment approach for relapsing-remitting MS?
1st, "tried & true" approach, then "convenience" approach (oral therapies) and "efficacy" approach (natalizumab)
What drug treats trigeminal-neuralgia & flexor spasms in MS?
What drugs treat just spasticity in MS?
Baclofen & tizanidine
What drugs treat fatigue in MS?
What are favorable age & gender predictors for a MS prognosis?
Age < 40 yo at time of diagnosis, female
What symptoms seen first in MS give a better prognosis?
Vision or sensory symptoms as first symptoms (& infrequent attacks)
What are poor age & gender predictors for a MS prognosis?
Age < 40 yo, male
What symptoms seen first in MS give a poor prognosis?
Cerebellar/pyramidal tract findings as first symptoms (& frequent attacks in first 2 years + incomplete remission, progressive & many lesions on MRI)
What is the typical presentation of ALS?
50 yo man presents with "clumsiness", worsening over time, has fasciculations & hyperreflexia, & denies recent infections
What is ALS the most commons cause of?
Anterior horn cell disorder
What gender is most affected by ALS?
What is the cause of ALS?
Unknown - most cases are sporadic
What is the theory for a genetic cause of ALS?
-Genetic locus at copper-zinc superoxide dismutase gene (SOD1) on xsome 21
-Recessive locus on some 2 linked to GTPase regulation
What is the toxicity theory of the cause of ALS?
Possible evidence of glutamate toxicity & protein nitration involved in development of ALS
What type of mutations are seen in ALS?
Wide variety of missense mutation, w/ A4V most common
What is the hallmark sign of ALS?
***Simultaneous UMN & LMN disease (see both symptoms' i.e. positive Babinski w/ tongue fasciculations)
Where is the site of loss with LMN loss?
Neurons in anterior horn of spinal cord
Where is the site of loss with UMN loss?
Neurons projecting into corticospinal tracts
What two musculoskeletal symptoms is ALS characterized by?
Neuronal muscle atrophy (amytrophy) & hyperreflexia
What is the differential diagnosis commonly seen with ALS?
Multifocal motor neuropathy, polio, spinal muscle atrophy, West Nile Virus encephalitis, demyelinating diseases, stroke, heavy metal poisoning, B12 deficiency, syphilis, endocrinopathies
What are early symptoms of ALS?
Lower extremity weakness, frequent falls, asymmetric weakness of hands, difficulty with fine motor skills, foot drop, muscle cramping, spasticity, dysarthria & dysphagia
What is NOT a symptom of ALS?
***Cognitive dysfunction is NOT a feature of ALS (memory is not affected!)
What are late symptoms of ALS?
Fasciculations, recurrent bouts of pulmonary infection, resp failure
What typically causes death in ALS?
What is the ALSFRS-R?
ALS functional rating scale
What are some specific UMN bulbar signs of ALS?
Increased jaw & facial reflex, poor palatal elevation, slow tongue movement
What are some specific LMN bulbar signs of ALS?
Weak masseter, can't keep jaw closed, facial diparesis, muscle atrophy & fasciculations
What are some specific UMN bulbar symptoms of ALS?
Dysphagia, laryngospasm, drooling
What are some specific LMN bulbar symptoms of ALS?
Incomplete eye closure, dysarthria, hoarseness
What are some specific LMN respiratory signs/symptoms of ALS?
Tachypnea, altered speech, use of accessory muscles, low speech volume, weak cough, confusion, hallucinations
What are some specific UMN axial signs/symptoms of ALS?
Absent abdominal reflexes, stiffness & imbalance
What are some specific LMN axial signs/symptoms of ALS?
Neck extension weakness, abdomen protuberance, cramps
What are some specific UMN limb signs/symptoms of ALS?
increased reflexes, gait disorder, spontaneous flexor spasms,
What are some specific LMN limb signs/symptoms of ALS?
Weakness of limbs, reduced reflexes, **
**positive Babinski reflex (toes go up)
What imaging is done to confirm ALS diagnosis?
- Brain & spinal MRI to rule out other conditions that mimic early ALS
-PET & fMRI being investigated
What can be used if MRI is a contraindication?
MR spectroscopy & CT with myelography
What do EMGs look for?
Fibrillations and fasciculation potentials
What levels are examined with EMG?
1. most involved limb first
2. distal muscles
3. bulbar muscles
What common causes of ALS symptoms are ruled out by lab tests?
B12/folate, HIV status, Lyme serology, thread function test, CPK
Is CSF used to diagnose ALS?
No - generally not helpful
What test is used in severe disease?
Pulmonary function test
What overall treatment is used in ALS?
Respiratory, nutritional, mobility, & communication support
What specific drug is used to treat ALS?
What is the prognosis of ALS?
Very poor - progressive, involves upper & lower extremities, truncal & bulbar musculature, terminal within 3-5 years after diagnosis
THIS SET IS OFTEN IN FOLDERS WITH...
CSI - LEC 1 - DELIRIUM
CSI - LEC 2 - TOXIDROMES
CSI - Lecture 11 - ABNORMAL BEHAVIOR
CSI - LEC 6 - DIABETES
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