MS, ALS. Peripheral neuropathy, Guillain Barre Flashcards

1
Q

MS is more common in

A

white women who live in colder climate

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2
Q

MS - presenation

A
  • 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
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3
Q

Multiple sclerosis - Charcot classic triad

A

MNEMONIC –> SIN

  1. Scanning speech (dysarthria)
  2. Intention tremor (also Internuclear opthalmoplegia and Incontinence)
  3. Nystagmus
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4
Q

Internuclear opthalmoplegia

A

inability to adduct one eye with nystagmus in the other

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5
Q

MS - best initial test / most accurate test

A

MRI is both the best initial and the most accurate test

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6
Q

MS - LP

A

CSF with a mild elevation in protein and fewer than 50 to 100 WBCs
oligoclonal bands in 85% (not specific to MS)

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7
Q

MS treatment for acute exacerbation

A

high-dose steroids –> they shorten the duration

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8
Q

Multiple sclerosis - drugs that prevent relapse and progression

A
  1. Glatiramer (copolymer 1) 2. b-INF 3. Natalizumab
  2. Azathyoprine 5. Cyclophosphamide
  3. Mitoxantrone 7. Fingolimob (oral)
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9
Q

problem with the use of drugs that prevent relapse and progression

A

neurological deficits with the use of a chronic suppressive medication. The MRI shows new, multiple white matter hypodense lesions

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10
Q

Multiple sclerosis - drugs that prevent relapse and progression - best first choice

A

Glatiramer (copolymer 1) + b-INF

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11
Q

ALS - the most serious presentation

A

difficulty in chewing and swallowing and decrease in gag reflex –> pooling saliva in the pharynx and frequent episodes of aspiration –> poor prognosis

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12
Q

ALS mcc of death

A

Resp failure

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13
Q

ALS - diagnosis

A
  • loss of neural innervation in multiple muscle groups

- CPK levels are elevated

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14
Q

ALS -treatment

A
  • 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
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15
Q

babinski - UMN vs LMN lesion

claso knife paralysis - UMN vs LMN lesion

A

both in UMN

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16
Q

Charcot-Marie Tooth - definition and manifestation

A

genetic disorder with loss of both motor and sensory innervation leading to

  1. Distal weakness and sensory los
  2. wasting in the legs
  3. decreased deep tendon reflexes
  4. tremor
  5. foot deformity with HIGH ARCH (pes cavus)
17
Q

Charcot-Marie Tooth - most accurate test / treatment

A

most accurate test: electromyography

NO TREATMENT

18
Q

acute inflammatory polyneuropathy (Guillain Barre Syndrome) - result in (symptoms)

A
  1. symmetric/paralysis (beginning in lower extremities)
  2. facial paralysis (in 50%)
  3. autonomic regulation (eg. cardiac irregulation, hypertension, hypertension)
  4. sensory abnormalities
19
Q

Guillain Barre Syndrome - most specific diagnostic test

A

nerve conduction studies, electromyography –> decrease in propagation of electrical impulses along the nerves (BUT IT TAKES 1-2 WEEKS TO BECOME ABNORMAL)

20
Q

Guillain Barre Syndrome - CSF

A

increased protein (causes papilledema) with NORMAL count

21
Q

Guillain Barre Syndrome - pulmonary test when diaphragm is involved

A
  • 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
22
Q

Guillain Barre Syndrome - treatment

A
  1. Respiratory support (critical until recovery)
  2. plasmapheresis
  3. IV immunoglobins
    (plasmapheresis and IVIG are equal in efficacy, combining them is wrong)
    NO ROLE FOR STEROIDS
23
Q

The MCC of peripheral neuropathy, other causes

A

DM

other causes: uremia, alcoholism, paraproteinemias (like MGUS)

24
Q

peripheral neuropathy - treatment (and which is the best initial

A

best initial: pregabalin or gabapentin

TCA and most seizure medications are effective

25
Q

peripheral neuropathy - median nerve - precipitating event and manifestations

A
  • typists carpenters, working with hands

- thenar wasting, pain/numbness in first 3 fingers

26
Q

peripheral neuropathy - peronial nerve - precipitating event and manifestations

A
  • high boots, pressure on back of knee

- week foot with dorsiflexion and eversion

27
Q

peripheral neuropathy -tarsal tunnel (Tibial nerve) - precipitating event and manifestations

A
  • worsens with walking

- pain and numbness in ankle and sole of foot

28
Q

peripheral neuropathy - lateral cutaneous nerve of thigh - precipitating event and manifestations

A
  • obesity, pregnancy, sitting with crossed leg

- pain/numbness of outer aspect of on thigh

29
Q

peripheral neuropathy - ulnar - precipitating event and manifestations

A
  • biker, pressure on palms of hand, trauma to the medial side of elbow
  • wasting hypothenar eminence, pain in 4th and 4th fingers
30
Q

peripheral neuropathy - radial - precipitating event and manifestations

A

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

31
Q

MCC of Bell palsy

A

idiopathic

32
Q

2 additional features of bell palsy

A
  1. 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
  2. taste disturbances> anterior 2/3 of the tongue
33
Q

Bell palsy - tests to diagnose

A

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

34
Q

Bell palsy - treatment

A

60% full recovery without treatment
best initial: prednisone
acyclovir not clearly help