Wilson's disease: clinical and radiological features.
Article
in English
| IMSEAR
| ID: sea-90877
ABSTRACT
BACKGROUND:
Wilson's disease is a treatable movement disorder with autosomal recessive inheritance which is associated with severe morbidity and mortality if not treated early. MATERIAL ANDMETHODS:
The clinical and radiological features of 22 cases of Wilson's disease seen during January 1984 to December 1993 were analysed for clinical presentation and common radiological features.RESULTS:
Among all the patients extrapyramidal features were the commonest (19/22 patients), followed closely by impaired higher mental functions (17/22 patients) and cerebellar signs (11/22 patients). In patients with onset of symptoms before 20 years, the common presentations were impaired higher mental functions, speech disturbance, dystonia and choreo-athetosis; whereas in patients with onset after 20 years cerebellar signs were commonest. The commonest CT head abnormality was basal ganglion hypodensity (10 patients) followed by brain stem hypodensity (6 patients).CONCLUSIONS:
The clinical and CT scan features are evaluated and compared with reported series. Hypodensities of brain stem earlier reported a rarity, was seen in 6 out of 22 cases.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Brain
/
Female
/
Humans
/
Male
/
Tomography, X-Ray Computed
/
Child
/
Retrospective Studies
/
Adolescent
/
Adult
/
Brain Diseases, Metabolic, Inborn
Type of study:
Observational study
Language:
English
Year:
1998
Type:
Article
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