Neuromuscular Flashcards

1
Q

AMAN antibodies

A

GM1 antibody

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2
Q

Miller Fischer triad

A

Ataxia
Areflexia
Ophthalmoplegia (6th nerve palsy)

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3
Q

Indications for MV in AIDP/GBS

A

FEV<20 ml/kg, MIP<30 mmHg, MEP 40 OR decrease by 30% from baseline.

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4
Q

Sural sparing is a characteristic of what neuropathy

A

Inflammatory neuropathy (GBS)

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5
Q

Antibody in Mille Fischer

A

GQ1b antibody

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6
Q

Difference between therapy in AIDP and CIDP /variants

A

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)

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7
Q

Antibody in MMN and AMAN

A

GM1 antibody

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8
Q

Most commonly affected location in infectious neuropathy caused by VZV

A

Mono-radiculitis of thoracic dorsal root ganglia
Others: facial nerve, ophthalmic division of trigeminal (causing vasculitis> MCA stroke)

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9
Q

Most sensitive and most specific in diagnosis of MG

A

Most specific: AchR- binding antibodies
Most sensitive: single fiber EMG > increased jitters

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10
Q

What is the treatment of lambert Eaton

A

Amifampridine/diaminopyridine DAP

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11
Q

Effective therapy in MG (anti musk)

A

PLEX or steroids, good reponse to rituximab.
poor response to IVIG and AZA
worsenes with achEI

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12
Q

Most common inherited neuromuscular disease in adults + genetics

A

Classic myotonic dystrophy type 1- CTG repeat in DMPK gene

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13
Q

Most common type of limb girdle myopathy disease

A

Calpainopathy (LGM 2A)
Delayed walking, toe walking, calf hypertrophy, winging of scapula.
Highest CK

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14
Q

Central core disease genetics (continental myopathy)

A

AD, ryanodine receptor gene (RYR1)
-malignant hyperthermia
-Delayed walking 3-4y
-Congenital dislocation of hip

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15
Q

Pompe disease (type 2 glycogenosis- acid Maltese deficiency) genetics

A

Alpha 1,4 glucosdiase, AR

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16
Q

Most common inherited myopathy/ muscular disease

A

Duschenne muscular dystrophy DMD

17
Q

Most common acquired inflammatory myopathy in >50 yo

A

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

18
Q

Muscle Histopathology in corticosteroid myopathy

A

Type 2 fiber atrophy, fast twitch type 2B fibers