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Clinical and Electrophysiologic Responses to Acetylcholinesterase Inhibitors in MuSK-Antibody-Positive Myasthenia Gravis: Evidence for Cholinergic Neuromuscular Hyperactivity
Journal of Clinical Neurology ; : 119-124, 2014.
Artículo en Inglés | WPRIM | ID: wpr-84613
ABSTRACT
BACKGROUND AND

PURPOSE:

Patients with muscle-specific tyrosine kinase (MuSK) antibody (MuSK-Ab)-positive myasthenia gravis (MG) show distinct responses to acetylcholinesterase inhibitors (AChEIs). Although clinical responses to AChEIs in MuSK-Ab MG are reasonably well known, little is known about the electrophysiologic responses to AChEIs. We therefore investigated the clinical and electrophysiologic responses to AChEIs in MuSK-Ab-positive MG patients.

METHODS:

We retrospectively reviewed the medical records and electrodiagnostic findings of 17 MG patients (10 MuSK-Ab-positive and 7 MuSK-Ab-negative patients) who underwent electrodiagnostic testing before and after a neostigmine test (NT).

RESULTS:

The frequency of intolerance to pyridostigmine bromide (PB) was higher in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (50% vs. 0%, respectively; p=0.044), while the maximum tolerable dose of PB was lower in the former (90 mg/day vs. 480 mg/day, p=0.023). The frequency of positive NT results was significantly lower in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (40% vs. 100%, p=0.035), while the nicotinic side effects of neostigmine were more frequent in the former (80% vs. 14.3%, p=0.015). Repetitive compound muscle action potentials (R-CMAPs) developed more frequently after NT in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (90% vs. 14.3%, p=0.004). The frequency of a high-frequency-stimulation-induced decrement-increment pattern (DIP) was higher in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (100% vs. 17.7%, p=0.003).

CONCLUSIONS:

These results suggest that MuSK-Ab-positive MG patients exhibit unique and hyperactive responses to AChEIs. Furthermore, R-CMAP and DIP development on a standard AChEI dose may be a distinct neurophysiologic feature indicative of MuSK-Ab-positive MG.
Asunto(s)

Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Bromuro de Piridostigmina / Proteínas Tirosina Quinasas / Potenciales de Acción / Registros Médicos / Inhibidores de la Colinesterasa / Estudios Retrospectivos / Miastenia Gravis / Neostigmina Tipo de estudio: Estudio observacional Límite: Humanos Idioma: Inglés Revista: Journal of Clinical Neurology Año: 2014 Tipo del documento: Artículo

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Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Bromuro de Piridostigmina / Proteínas Tirosina Quinasas / Potenciales de Acción / Registros Médicos / Inhibidores de la Colinesterasa / Estudios Retrospectivos / Miastenia Gravis / Neostigmina Tipo de estudio: Estudio observacional Límite: Humanos Idioma: Inglés Revista: Journal of Clinical Neurology Año: 2014 Tipo del documento: Artículo