MS, ALS. Peripheral neuropathy, Guillain Barre Flashcards
MS is more common in
white women who live in colder climate
MS - presenation
- MC presentation: focal sensory symptoms with gait and balance problems
- visual disturbances are no longer as common as the first presentation
- after optic neuritis, the MC abnormalities are motor and sensory
- the least common: cognitive and dementia
- sexual function remains intact
Multiple sclerosis - Charcot classic triad
MNEMONIC –> SIN
- Scanning speech (dysarthria)
- Intention tremor (also Internuclear opthalmoplegia and Incontinence)
- Nystagmus
Internuclear opthalmoplegia
inability to adduct one eye with nystagmus in the other
MS - best initial test / most accurate test
MRI is both the best initial and the most accurate test
MS - LP
CSF with a mild elevation in protein and fewer than 50 to 100 WBCs
oligoclonal bands in 85% (not specific to MS)
MS treatment for acute exacerbation
high-dose steroids –> they shorten the duration
Multiple sclerosis - drugs that prevent relapse and progression
- Glatiramer (copolymer 1) 2. b-INF 3. Natalizumab
- Azathyoprine 5. Cyclophosphamide
- Mitoxantrone 7. Fingolimob (oral)
problem with the use of drugs that prevent relapse and progression
neurological deficits with the use of a chronic suppressive medication. The MRI shows new, multiple white matter hypodense lesions
Multiple sclerosis - drugs that prevent relapse and progression - best first choice
Glatiramer (copolymer 1) + b-INF
ALS - the most serious presentation
difficulty in chewing and swallowing and decrease in gag reflex –> pooling saliva in the pharynx and frequent episodes of aspiration –> poor prognosis
ALS mcc of death
Resp failure
ALS - diagnosis
- loss of neural innervation in multiple muscle groups
- CPK levels are elevated
ALS -treatment
- Riluzole: reduce glutamate buildup in neurons and may prevent progression of disease
- Baclofen treats spasticity
CPAP and BiPAP help with respiratory difficulties - tracheostomy and maintenance on a ventilator is often necessay wjen the disease advances
babinski - UMN vs LMN lesion
claso knife paralysis - UMN vs LMN lesion
both in UMN
Charcot-Marie Tooth - definition and manifestation
genetic disorder with loss of both motor and sensory innervation leading to
- Distal weakness and sensory los
- wasting in the legs
- decreased deep tendon reflexes
- tremor
- foot deformity with HIGH ARCH (pes cavus)
Charcot-Marie Tooth - most accurate test / treatment
most accurate test: electromyography
NO TREATMENT
acute inflammatory polyneuropathy (Guillain Barre Syndrome) - result in (symptoms)
- symmetric/paralysis (beginning in lower extremities)
- facial paralysis (in 50%)
- autonomic regulation (eg. cardiac irregulation, hypertension, hypertension)
- sensory abnormalities
Guillain Barre Syndrome - most specific diagnostic test
nerve conduction studies, electromyography –> decrease in propagation of electrical impulses along the nerves (BUT IT TAKES 1-2 WEEKS TO BECOME ABNORMAL)
Guillain Barre Syndrome - CSF
increased protein (causes papilledema) with NORMAL count
Guillain Barre Syndrome - pulmonary test when diaphragm is involved
- decreases in force VC and peak inspiratory pressure
- inspiration is the active part of the breathing and the patient loses the strength to inhale
- PFT tell who might die fro GBS
Guillain Barre Syndrome - treatment
- Respiratory support (critical until recovery)
- plasmapheresis
- IV immunoglobins
(plasmapheresis and IVIG are equal in efficacy, combining them is wrong)
NO ROLE FOR STEROIDS
The MCC of peripheral neuropathy, other causes
DM
other causes: uremia, alcoholism, paraproteinemias (like MGUS)
peripheral neuropathy - treatment (and which is the best initial
best initial: pregabalin or gabapentin
TCA and most seizure medications are effective
