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Neuro Mx Dz / NMJ
Neurology
| Question | Answer |
|---|---|
| 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
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