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Neuro Mx Dz / NMJ

Neurology

QuestionAnswer
ALS: Dx UMN & LMN sx in 3 regions; UMN: clinical; LMN: EMG; CK nml or high
ALS Tx Riluzole; cough assist device, chest PT, BiPAP; PEG feeding tube;
PLS: path degeneration of lateral corticospinal tract
PLS clin findings usu legs before arms; leg weak/stiff; spasticity; ave duration >8 yrs; many pts develop LMN sx & transition to ALS
PLS: dx EMG, MRI (br & spcord), LP, evoked potls; B12, Lyme, RPR, long chain fatty acids
PMA pure LMN dz; slower progress than ALS; often spares bulbar mx; CK very high
Kennedy dz X-linked rec, TNR; mutation in androgen receptor; sx onset (wide age range); facial fasciculations, weakness mouth/tongue; dysphagia, limb weakness; gynecomastia, DM, oligospermia
Disorders of neuromuscular transmission (NMJ): MG (most common), LEMS, botulism
MG: pathology Acquired autoimmune: Ach receptor insufficiency & Abs to Ach receptor protein; possibly also thymus hyperplasia
MG S/S Insidious; fluctuating mx weakness (worse w/reps); asymmetric ptosis; tired face, difficulty chewing/swallowing; SOB. Normal DTRs. F 20-30 yo, M 70-80
MG Dx Tensilon test (or neostigmine); Ach receptor & MuSK Abs; Repetitive nerve stim; Single fiber EMG; CT Chest to exclude thymoma
MG Tx Symptomatic (Cholinesterase inhibitors, eg pyridostigmine & neostigmine); Firstline: steroids (alt immunosupp: CellCept, Cyclosporine); thymectomy
MG exacerbations d/t infxn, PG, meds; may worsen resp sx; tx w/ plasmapheresis or IVIg, supportive respiratory care
MG: drugs to avoid NM blockers; quinine; macrolides, FQ; botox; beta/CCB; IV contrast
LEMS usu paraneoplastic (autoimm rare); proximal weakness / autonomic sx (dry mouth); hypo/absent reflexes; voltage gated Ca channel Abs; facilitation w/ RNS
Botulism MOA irreversible blockade of Ach release
Botulism S/S Diplopia, ptosis, dilated pupils; facial / resp weakness; descending paralysis; Autonomic dysfunction
Botulism Tx supportive; horse serum antitoxin? (months to recover)
MD classified by: distribution, inheritance and clinical features
MD: labs/studies ultrastructural protein abnormalities; mx bx: mx fiber necrosis
Duchenne onset at 3-5 yrs; calf pseudohypertrophy; loss of ambulation by teens; cardiomyopathy; death in 20s
Duchenne/Becker: Dx Mx bx; genetic testing (need complete sequencing); CK sometimes >10,000; FH
Duchenne/Becker Tx Prednisone (age 5) for Duchenne; Supportive (orthotics, resp, cardiac, PT/OT); cardiac TP in Becker
FSHD auto dominant; asymmetric; eye or mouth closure weakness: may sleep w/ eyes open; pectoral & biceps/triceps atrophy (but normal deltoid); also scap winging; no fx on life exp; orthotics & counseling
FSHD Dx genetic testing (95%); FH; mx bx rarely helpful
Emery Dreifuss Humeroperoneal / scapuloperoneal weakness w/ early contractures (ankle neck elbow); teen onset (very thin); scap winging/deltoid sparing; x linked or auto dom; cardiac conduction block/arrhythmia (need pacemaker)
Most common MD’s Duchenne/Becker; myotonic; FSHD
Myotonic dystrophy auto dom; any age; TNR
Myotonic dystrophy S/S tenting upper lip; distal weakness; frontal bald; cataracts; conduction block; DM, infertile; cognitive impairment; percussion myotonia
MG tx: pyridostigmine dose 15-90 mg Q6h and 180 mg of long acting version HS
MG tx: neostigmine dose 15 mg before excessive physical activity
Young kid with difficulty standing from seated position. Calf muscle wasting Muscular dystrophy (weakness begins at pelvic girdle)
fluctuating weakness/fatigability of voluntary mx (diplopia, blurry vision, ptosis, difficulty swallowing); resp difficulty, limb weakness (worsened w/activity); bulbar sxs (dysarthria, dysphagia, fatigable chewing) Myasthenia gravis
MG pathophys Abs vs acetylcholine receptors
LEMS pathophys: defective release of Ach in response to nerve impulse; may be assoc with small cell ca
MG vs LEMS: S/S LEMS: power increases w/sustained contraction; MG: fatigability
Kennedy dz genetics X-linked rec, TNR; mutation in androgen receptor
Kennedy dz S/S facial fasciculations, weakness mouth/tongue; dysphagia, limb weakness; gynecomastia, DM, oligospermia
Disorders of neuromx transmission (NMJ): MG, LEMS, botulism
MG: pathophys Acquired autoimmune: Ach receptor insufficiency
MG S/S fluctuating mx weakness (worse w/reps); asymmetric ptosis; tired face, difficulty chewing/swallowing; SOB
LEMS S/S usu paraneoplastic (autoimm rare); proximal weakness / autonomic sx (dry mouth); hypo/absent reflexes; voltage gated Ca channel Abs; facilitation w/ RNS
Botulism MOA irreversible blockade of Ach release
Duchenne MD onset at 3-5 yrs; calf pseudohypertrophy; loss of ambulation by teens; cardiomyopathy; death in 20s
Emery Dreifuss Humeroperoneal / scapuloperoneal weakness w/ early contractures (ankle neck elbow); teen onset (very thin); scap winging/deltoid sparing; x linked or auto dom; cardiac conduction block/arrhythmia (need pacemaker)
Most common MD’s Duchenne/Becker; myotonic; Facioscapulohumeral dystrophy
Myotonic dystrophy auto dom; any age; TNR
Myotonic dystrophy S/S tenting upper lip; distal weakness; frontal bald; cataracts; conduction block; DM, infertile; cognitive impairment; percussion myotonia
Created by: Abarnard