Neuromuscular Flashcards
Anterior interosseus nerve
All C7, C8. Comes off AFTER pronator teres. Innervates pronator quadratus - Flexor Dig Profundus (Digits 2+3) - Flexor Pollicis Longus
Posterior interosseus nerve
All C7, C8. ABductor Pollicis Longus (APL) (fake APB) Ext Carpi Ulnaris Ext Digitorum Ext Poll Brevis Ext Poll Longus
Serratus anterior
inn. long thoracic nerve. C5-7.
Comes off C5-6 before the upper trunk.
Inversion of foot
Tibialis posterior. L4-S3. Tibial nerve.
Eversion of foot
Peroneus longus. L5-S1. Superficial peroneal nerve.
Extension of foot
Tibialis anterior. L4, L5. Deep peroneal nerve.
Extension of great toe
EHL. L5, S1. Deep peroneal nerve.
Hypokalemic periodic paralysis
AD. Calcium channels (DHP channels) paralysis after sleep + REST POST-exercise POST CARB PARALYSIS Can also occur after sleep
Hyperkalemic periodic paralysis
AD.
Sodium channels.
Paralysis with INCREASE K+, COLD, EXERCISE.
Rx = glucose or diamox/thiazide
Myotonia congenita
Thomsen disease.
Autosomal dominant
Chloride channels
Myotonia w INACTIVITY - improve with exercise
Paramyotonia congenita
AD,
Sodium channels
Myotonia with COLD, EXERCISE, LOW K+
Central core disease
AR ryanodine Ca channel mutation \+/- Mal hyperthermia lucent core (NADH diaphorase stain) non-progressive weakness
Centronuclear myopathy
MYF6 (AD), BIN1 (AR), myotubularin(XR)
“central myonuclei (H&E stain)
stellate sarcoplasmic reticulum (NADH diaphorase stain)”
Emery Dreifuss
Emerin (XR), lamin A/C (AD+AR)
Cardiomyopathy
Biceps+ triceps weakness then spreading to legs
Facioscapulohumeral dystrophy (FSHD)
AD; D4Z4 on chromosome 4q; encodes polyA binding P Facial weakness + drooping shoulders SN Hearing LOSS MR, seizures
Fukuyama muscular dystrophy
AR Fukutin Contractures Polymicrogyria + Cobblestone lissencephaly Mental retardation SEIZURES
Hirayama
Aka monomelic amyotrophy
“Japan/Indian/Hawaiian origin
single-limb or UE-only ALS type picture”
Nemaline rod myopathy
Mutations in:
alpha-actin (MC) + troponin
Path = EM with rods parallel to myofibers
Polymyositis
endomysial + perimysial +perivascular inflammation
association with MALIGNANCY (not as much as DM)
responds to steroids
Dermatomyositis
Characteristic rash, assoc w malignancy
path = peri-fascicular atrophy + periVASCULAR infl.
Anti Jo1 associated with ILD (do not not use MTX)
pediatric myasthenic syndromes
Neonatal transitory = passive Xfer of AB’s
Congenital MG = AR mutations in ACH (R) epsilon subunit
familial infantile = chromosome 17 polymorphism
HNPP
deletion in PMP-22 gene (also impl. in CMT1A)
Lewis Sumner
aka MADSAM - basically asymmetric CIDP
