A Case of Adult Neuronal Ceroid-Lipofuscinosis, Proven by Brain Biopsy
Journal of the Korean Neurological Association
;
: 45-48, 2001.
Article
in Korean
| WPRIM
| ID: wpr-176470
ABSTRACT
A 20-year-old woman was admitted because of intractable seizures, myoclonus, gait ataxia, and severe intellectual deterioration with age of onset at 16 years. She had no family history of neurological disease. A thorough laboratory investigation was unremarkable. Brain MRI showed generalized cerebral and cerebellar atrophy. Interictal EEG showed intermittent generalized polyspike and waves, maximum on the bilateral parieto-occipital areas, and MNSEP showed giant cortical SEP. Brain biopsy revealed intraneuronal accumulation of granular osmiophilic deposits (GROD), which is characteristic of electromicroscopic findings of neuronal ceroid lipofuscinosis (NCL). We report biopsy-proven adult NCL, which is one of the rare neurodegenerative diseases. (J Korean Neurol Assoc 19(1)45~48, 2001
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Atrophy
/
Seizures
/
Biopsy
/
Brain
/
Magnetic Resonance Imaging
/
Age of Onset
/
Neurodegenerative Diseases
/
Gait Ataxia
/
Electroencephalography
/
Myoclonus
Limits:
Adult
/
Female
/
Humans
Language:
Korean
Journal:
Journal of the Korean Neurological Association
Year:
2001
Type:
Article
Similar
MEDLINE
...
LILACS
LIS