Neuro 2 Flashcards
(28 cards)
Causes of ophistiotonus
Severe hyperextension and spasticity. head, neck and spinal column enters a complete arching position. Due to extrapyramidal effect–> spasms of axial muscles along spinal column
causes: meningitis, teatnus, tetani, kernicterus, SAH, lithum intox
def of CP
nonprogressive disorder of movement and posture that result from a lesion of the developing brain (before 2 YOL), it is a chronic disorder that continues throughout life.
top 5 causes of CP
periventricular leucomalacia, intrapartum asphyxia, cerebral dysgenesis, intracranial hemorrhage, vascular infarction
Postnatal causes of CP
stroke, trauma, kernicterus, infection of nervous system
prenatal factors/causes of CP
intrauterine infection, prematurity, placental hemorrhage, multiple gestations
clinical features of neuromuscular disorder
weakness, floppiness, delayed motor milestones, abnomrla gait, fatiguability, muscle cramps.
gowers sign: normal range
Normal until age 3
Spinal muscular atrophy
AR mutation in SNR gene causing degeneration of ant. horn cells –> progressive weakness of wasting of skeletal muscles. 2nd most common neuromuscular disease, after duchenne. 4 ypes
SMA type 1: werdnig- hoffmann
most sever form
becomes evident in early infancy (0-6 months), with generalized hypotonia and weakness, absent DTR, intercostal rescession, fasciculation of tonuge. death from resp resp failure within 12 months
SMA type 2: dubowitz
can sit but never walk independently., pseduohypertrophy of gastrocnemius. 6-18 months, resp failure from 2 yrs-3 decade
type 3 SMA
Kugelberg-Welander: presenting >18 months, can stand and walk alone but have trouble with complex motor skills. near normal life expectancy
diagnosis of SMA
molecular markers in blood (SMN gene), EMG, muscle biposy
poliomyelitis
ASYMMETRICAL,
clinical, 10 % symptomatic: 5 % minor illness: fever headache, sore throat, vomiting, 2 % CNS involvement with meningitis , 1 % paralytic polio.
hereditary motor sensosry neuropathies
group of disorder typically leading to symmetrical progressive muscular wasting, which is DISTAL. eg type 1 charcot-marie-tooth disease (MC ) AD
charcot-marie-tooth disease
AD
affected nerves may be hypertrophic due to demyleination followed by attemps of remyelination: ONION BULB FORMATION on biopsy.
onset: first decade, distal atrophy: legs>arms –> inverted champange bottle of legs. initial symptoms: foot drop and hammer toe. pes cavus
Guillain-Barre syndrome
def
rapid onset muscle weakness as a result of damage to peripheral nervous system( autoimmune mediated demyelination of peripheral nerves). present typically 2-3 w after an URTI or campylobacter gastorenteritis.
clinical manifestations of Guillain -BS
Ascending symmetrical weakness, loss of reflexes and autonomic involvement (inc BP and HR), can involve bulbar muscles leading to difficulty with eating and swallowing –> inc risk of aspiration. resp depression may require mechanical ventilation.
diagnosis and managment of Guillan BS
Dx: nerve conduction veolcity are reduced, inc protein in CSF , normal WBC
managment; supportive, Ig infusion, plasma exchange. full recovery i 95 % after 2 yrs
Duchenne muscular dystrophy def + presentation
XLR–>lack of dystrophin. muscle fiber friability –> breakdown –> necrosis and regeneration.
presentation: proximal weakness, and gowers sign, waddling gate and toe walking by age 3. loss of reflexes and pseudohypertrophy. wheelchair by age 10. daath by 20s due to resp failure or HF. average age of diagnosis: 5 yrs
Congenital Myasthenia gravis
12 % of newborn born to MG mothers. auto Ach-ab cross placenta. Present: ophtalmopleia, limb weakness, weak sucking/cry, resp depression (may need ventilation), lasting days to weeks. treatment: neostigmine, prednisolon, plasmapheresis.
Duchenne muscular dystrophy
high CK-MM and lactase dehydogenase. EMG/biopsy
treatment: ventilation support: physiotherapy, b2 agonist, prednisolon, digoxin for HF.
Backer muscular dystrophy
XLR mutation in gene producing dystrophun. milder form of duchenne, with some functional dystrophin still produced. Milder, later and slow progression, average age of presentation i 11 yrs, wheelchair by 20s, death 40s. managment: prednisolon, IVIG
diff diagnosis of facial nerve palsy
Bells´s: post infetious (HSV, swelling in ear canal), lyme (usually bilateral), Ramsyhunt syndrome 2 (shingels), melkersson tosenthal syndrome (reccuring facial paralysis, swelling of lip and development of fold in tongue.
Acute cerebellar ataxia
MCC of ataxia: due to medication, post viral (vzv, coxsackie), medulloblastoma. clinical: vomitingm dysarthria, nystagmus, ataxia.
