Electrophysiological characteristics in four patients from Brazil with stiff person syndrome

Abstract Stiff person syndrome (SPS) is a rare immune-mediated disorder of the central nervous system characterized by muscle rigidity and episodic muscle spasms. The diagnosis of SPS is based on electrophysiological studies. We analyzed the electrophysiological features in four patients from Brazil...

Full description

Saved in:
Bibliographic Details
Published in:Journal of clinical neuroscience 2012-06, Vol.19 (6), p.889-891
Main Authors: Lorenzoni, Paulo J, Scola, Rosana H, Kay, Cláudia S.K, Teive, Hélio A.G, Coutinho dos Santos, Lúcia H, Werneck, Lineu C
Format: Article
Language:English
Subjects:
Adult
Brazil
Central nervous system
Child
Continuous muscle activity
Cutaneomuscular reflex stiff person syndrome
Electric Stimulation
Electromyography
Electrophysiological recording
Electrophysiological study
Evoked Potentials, Motor - physiology
Female
Humans
Male
Motor units
Muscles
Nervous system
Neurology
Reflexes
Stiff limb syndrome
Stiff-Person Syndrome - physiopathology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Stiff person syndrome (SPS) is a rare immune-mediated disorder of the central nervous system characterized by muscle rigidity and episodic muscle spasms. The diagnosis of SPS is based on electrophysiological studies. We analyzed the electrophysiological features in four patients from Brazil who fulfilled the clinical criteria for SPS. The most common electrophysiological abnormalities were continuous motor unit activity, co-contracting, and the presence of the cutaneomuscular reflex. Despite all patients having clinical characteristics of SPS during the disease, no patient met all the electrophysiological criteria for SPS even after repeat electrophysiological studies. This shows that a diagnosis of SPS should not be restricted to patients with all the classic electrophysiological changes but should be considered in the presence of one or some of those changes.
ISSN:0967-5868
1532-2653
DOI:10.1016/j.jocn.2011.08.034