Module 4: Weakness 4

Question 0

Chronic disease of anterior horn cells? Clinical features? Treatment?

Answer 0

Amyotrophic lateral sclerosis

#Insidious onset with distal muscle weakness and fasciculations
#Affects both LMN and UMN
#All patients eventually develop diaphragmatic and pharyngeal weakness

Riluxole
PEG before VC dips under 50%

Question 1

Acute disease of anterior horn cells? Clinical features? Treatment?

Answer 1

Poliomyelitis

#Aseptic  meningitis with fever, headache, stiff neck
#Asymmetric lower motor neuron weakness

Prevention with Vaccine

Question 2

Congenital disease of anterior horn cells? Worst type? Clinical features? Treatment?

Answer 2

Spinal muscular atrophy

Werdnig Hoffman disease (most severe type ) presents with neonatal hypotonia

No treatment

Question 3

Diabetes – type of neuropathies?

Answer 3

#Mononeuritis multiplex
#Distal, symmetrical polyneuropathy
#Cranial neuropathy (CN3 palsy)

Question 4

Nutritional deficiency – type of neuropathy? Most important vitamins?

Answer 4

Distilled, symmetrical polyneuropathy

B1, B6, folate

Question 5

GBS – weakness must peak when? CSF demonstrates?

Answer 5

Within four weeks; cytoalbuminologic dissociation (high-protein, no cells)

Question 6

Chronic inflammatory demyelinating polyneuropathy? Treatment?

Answer 6

GBS that progresses after four weeks

Steroids

Question 7

Chemotherapeutic agents usually cause what type of neuropathy? Most common agents?

Answer 7

Distal, symmetric polyneuropathy

#Vinhristine
#Cisplatin
#Taxol

Question 8

Antibiotics most commonly caused what type of neuropathy? Most common agents?

Answer 8

Distal, symmetric polyneuropathy

#Isoniazid (if given without B6)
#Metronidazole
#Nucleoside antagonists

Question 9

Diphtheria toxin mimics what neurological disorder?

Answer 9

GBS

Question 10

Lead poisoning causes what type of neuropathy?

Answer 10

Mononeuritis multiplex especially wrist drop

Question 11

Neuropathy that involves ears and nose most likely related to?

Answer 11

Leprosy

Question 12

All patients with no clear cause of neuropathy should receive what tests?

Answer 12

SPEP for Multiple myeloma and MGUS

Question 13

Distal, symmetrical polyneuropathy caused what type of metabolic/endocrine abnormalities?

Answer 13

Uremia, hepatic disease, hypothyroidism, porphyria

Question 14

Charcot Marie Tooth disease – genetics? Symptoms? Presents when?

Answer 14

Autosomal dominant on chromosomes 17

Slowly progressive demyelinating neuropathy which presents in 20s

Question 15

Hereditary neuropathy with liability to pressure palsies – mechanism?

Answer 15

Causes damage to nerves at multiple points so insignificant injuries can cause a mononeuropathy

Question 16

Myasthenia gravis – antibody against? Cardinal feature? Diagnosis? Treatment?

Answer 16

Acetylcholine receptor; fatigability

1. Edrophonium
2. Acetylcholine receptor antibody serologies
3. EMG
4. Thymoma

#Cholinesterase inhibitors (pyridostigmine)
#Thymectomy
#immunosuppression via steroids or azathioprine/cyclosporine

Question 17

Botulism – pathogenesis? Clinical features? Diagnosed with? Treatment?

Answer 17

Prevents release of acetylcholine from presynaptic neuron

#Descending paralysis
#Diplopia
#Dysarthria/dysphasia
#Respiratory difficulty
#Limb weakness

Evidence of toxin in stool or vomit

Equine antitoxin

Question 18

Lambert-Eaton – pathogenesis? Clinic features? Diagnosis? Treatment?

Answer 18

Paraneoplastic disease from antibodies to presynaptic calcium channels (small cell lung cancer)

#Proximal weakness
#Ptosis/diplopia
#Depressed reflexes
#Autonomic dysfunction (dry mouth, impotence)

1. Antibody testing
2. Repetitive stimulation increases amplitude

Treatment of underlying cancer

Question 19

Polymyositis – pathogenesis? Clinic features? Diagnosis? Treatment?

Answer 19

Autoimmune attack against muscle

#insidious progressive course
#Proximal muscles involved first (difficulty arising from seated position)
#Dysphasia
#ECG changes

1. Elevated CPK
2. EMG shows abnormal muscle activity
3. Muscle biopsy shows perivascular lymphocytic filtration

Prednisone 1-2 mg/kg/day

Question 20

Dermatomyositis – pathogenesis? Clinic features? Diagnosis? Treatment?

Answer 20

Polymyositis we heliotrope rash/shawl sign/Bouchard’s nodules

Auto immune attack against muscle with skin manifestations

1. Elevated CPK
2. EMG shows abnormal muscle activity
3. Muscle biopsy shows very fascicular atrophy and inflammation

#Prednisone
#if age over 40, malignancies screen

Question 21

Inclusion body Myositis – pathogenesis? Clinic features? Diagnosis? Treatment? Prognosis?

Answer 21

Insidious, progressive weakness involving finger flexors and leg extensors

Autoimmune attack against muscle

1. Elevated CPK
2. EMG shows abnormal muscle activity
3. Muscle biopsy shows interstitial infiltration and “rimmed vacuoles”