Treatment of Drop Attacks in Coffin-Lowry Syndrome With the Use of Sodium Oxybate

Coffin-Lowry syndrome is a well-defined clinical entity classically associated with moderate to severe mental retardation, characteristic facial features, skeletal deformities, and tapering fingers. A characteristic paroxysmal disorder was described in up to 10% patients with Coffin-Lowry syndrome,...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric neurology 2007-11, Vol.37 (5), p.373-374
Main Authors: Havaligi, Navasuma, MD, Matadeen-Ali, Chandra, MD, Khurana, Divya S., MD, Marks, Harold, MD, Kothare, Sanjeev V., MD
Format: Article
Language:English
Subjects:
Adult
Anesthetics, Intravenous - therapeutic use
Biological and medical sciences
Coffin-Lowry Syndrome - complications
Humans
Immunomodulators
Male
Malformations of the nervous system
Medical sciences
Neurology
Pediatrics
Pharmacology. Drug treatments
Sodium Oxybate - therapeutic use
Syncope - drug therapy
Syncope - etiology
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:Coffin-Lowry syndrome is a well-defined clinical entity classically associated with moderate to severe mental retardation, characteristic facial features, skeletal deformities, and tapering fingers. A characteristic paroxysmal disorder was described in up to 10% patients with Coffin-Lowry syndrome, characterized by sudden loss of muscle tone induced by unexpected tactile or auditory stimuli. These events were given several names, including cataplexy, nonepileptic collapses with atonia, exaggerated startle responses, hyperekplexia, and stimulus-induced drop episodes. Various therapies were undertaken for these drop attacks, including clonazepam, tiagabine, felbamate, selective serotonin reuptake inhibitors, and tricyclics, with variable improvement. We report on a 22-year-old man with Coffin-Lowry syndrome with stimulus-induced drop episodes, who failed therapy with clonazepam, several antiepileptic drugs, and escitalopram, and who was given a trial of sodium oxybate with complete resolution of the drop attacks.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2007.06.025