Neuromuscular Flashcards
AMAN antibodies
GM1 antibody
Miller Fischer triad
Ataxia
Areflexia
Ophthalmoplegia (6th nerve palsy)
Indications for MV in AIDP/GBS
FEV<20 ml/kg, MIP<30 mmHg, MEP 40 OR decrease by 30% from baseline.
Sural sparing is a characteristic of what neuropathy
Inflammatory neuropathy (GBS)
Antibody in Mille Fischer
GQ1b antibody
Difference between therapy in AIDP and CIDP /variants
AIDP: IVIG or PLEX (not responsive to steroids)
CIDP: steroids (oral or IV), steroid sparing agents, or IVIG/ PLEX in refractory
MMN: IVIG, or cyclophosphamide (no response to PLEX or steroids)
Antibody in MMN and AMAN
GM1 antibody
Most commonly affected location in infectious neuropathy caused by VZV
Mono-radiculitis of thoracic dorsal root ganglia
Others: facial nerve, ophthalmic division of trigeminal (causing vasculitis> MCA stroke)
Most sensitive and most specific in diagnosis of MG
Most specific: AchR- binding antibodies
Most sensitive: single fiber EMG > increased jitters
What is the treatment of lambert Eaton
Amifampridine/diaminopyridine DAP
Effective therapy in MG (anti musk)
PLEX or steroids, good reponse to rituximab.
poor response to IVIG and AZA
worsenes with achEI
Most common inherited neuromuscular disease in adults + genetics
Classic myotonic dystrophy type 1- CTG repeat in DMPK gene
Most common type of limb girdle myopathy disease
Calpainopathy (LGM 2A)
Delayed walking, toe walking, calf hypertrophy, winging of scapula.
Highest CK
Central core disease genetics (continental myopathy)
AD, ryanodine receptor gene (RYR1)
-malignant hyperthermia
-Delayed walking 3-4y
-Congenital dislocation of hip
Pompe disease (type 2 glycogenosis- acid Maltese deficiency) genetics
Alpha 1,4 glucosdiase, AR
Most common inherited myopathy/ muscular disease
Duschenne muscular dystrophy DMD
Most common acquired inflammatory myopathy in >50 yo
INCLUSION BODY MYOSITIS
Clinical: asymmetrical progressive painless proximal muscle weakness and atrophy (forearm, wrist, finger flexors. Knee extensors, ankle dorsiflexion, facial weakness), deltoid sparing
Dx: anti NT5CIA (cytosol 5 neucleotidase 1A antibodies.)(cN1A), RIMMED VACUOLES
Muscle Histopathology in corticosteroid myopathy
Type 2 fiber atrophy, fast twitch type 2B fibers