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
peripheral neuropathy - median nerve - precipitating event and manifestations
- typists carpenters, working with hands
- thenar wasting, pain/numbness in first 3 fingers
peripheral neuropathy - peronial nerve - precipitating event and manifestations
- high boots, pressure on back of knee
- week foot with dorsiflexion and eversion
peripheral neuropathy -tarsal tunnel (Tibial nerve) - precipitating event and manifestations
- worsens with walking
- pain and numbness in ankle and sole of foot
peripheral neuropathy - lateral cutaneous nerve of thigh - precipitating event and manifestations
- obesity, pregnancy, sitting with crossed leg
- pain/numbness of outer aspect of on thigh
peripheral neuropathy - ulnar - precipitating event and manifestations
- biker, pressure on palms of hand, trauma to the medial side of elbow
- wasting hypothenar eminence, pain in 4th and 4th fingers
peripheral neuropathy - radial - precipitating event and manifestations
pressure of inner, upper arm, falling asleep with arm over back of chair (Saturday night palsy), using crutches and pressure in the axilla
wrist drop
MCC of Bell palsy
idiopathic
2 additional features of bell palsy
- hyperacusis: sounds are extra loud because 7th cranial nerve normally supplies the stapedius miscle, which acts as a shock absorber on the ossicles of the middle ear
- taste disturbances> anterior 2/3 of the tongue
Bell palsy - tests to diagnose
No test is usually done because of the characteristic presentation of the paralysis of half of the face. The MOST ACCURATE is electromyography and nerve conduction studies
Bell palsy - treatment
60% full recovery without treatment
best initial: prednisone
acyclovir not clearly help
