Episodic coma in a new leukodystrophy.
Among the leukodystrophies of a hypomyelinating nature, childhood ataxia with diffuse central nervous system hypomyelination exhibits the unique feature of rapid decrease in mental status after relatively minor head injuries or otherwise noncomplicated febrile illnesses. This article reports the case of a child with progressive spastic quadriparesis in whom unconsciousness developed repeatedly as a result of minor head trauma and required prolonged critical-care nursing. Although cognition is believed to be relatively preserved in this disorder, this child developed progressive cognitive decline. A detailed review of the literature is presented along with discussion of the potential mechanisms of neurologic deterioration.
Espay
Alberto J
AJ
Department of Neurology, Section of Pediatric Neurology, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA.
Bodensteiner
John B
JB
Patel
Hema
H
D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000378:metabolism; D001259:Ataxia / Q000473:pathology; D001921:Brain / Q000473:pathology; D002648:Child; D002794:Choline / Q000378:metabolism; D003128:Coma / Q000473:pathology*; D003401:Creatine / Q000378:metabolism; D003711:Demyelinating Diseases / Q000473:pathology*; D004834:Epilepsy, Post-Traumatic / Q000473:pathology; D016489:Head Injuries, Closed / Q000150:complications / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D009128:Muscle Spasticity / Q000473:pathology; D009460:Neurologic Examination; D011782:Quadriplegia / Q000473:pathology
D001224:Aspartic Acid; C000179:N-acetylaspartate; D003401:Creatine; D002794:Choline