Juvenile Huntington disease: CT and MR features

To describe the clinical and radiologic manifestations of juvenile Huntington disease and to determine whether adult imaging criteria for Huntington disease are helpful for pediatric patients. Six patients (3 to 18 years of age; mean age, 9.8 +/- 5.6 years; 3 female, 3 male) with juvenile Huntington...

Full description

Saved in:
Bibliographic Details
Published in:American journal of neuroradiology : AJNR 1995-08, Vol.16 (7), p.1405-1412
Main Authors: Ho, VB, Chuang, HS, Rovira, MJ, Koo, B
Format: Article
Language:English
Subjects:
Adolescent
Atrophy
Biological and medical sciences
Brain - pathology
Caudate Nucleus - pathology
Child
Child, Preschool
Diagnosis, Differential
Dominance, Cerebral - physiology
Female
Frontal Lobe - pathology
Humans
Huntington Disease - diagnosis
Huntington Disease - genetics
Investigative techniques, diagnostic techniques (general aspects)
Magnetic Resonance Imaging
Male
Medical sciences
Nervous system
Phenotype
Putamen - pathology
Radiodiagnosis. Nmr imagery. Nmr spectrometry
Reference Values
Tomography, X-Ray Computed
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:To describe the clinical and radiologic manifestations of juvenile Huntington disease and to determine whether adult imaging criteria for Huntington disease are helpful for pediatric patients. Six patients (3 to 18 years of age; mean age, 9.8 +/- 5.6 years; 3 female, 3 male) with juvenile Huntington disease were studied with CT (n = 6) and/or MR (n = 3). CT and MR studies were evaluated for frontal horn distance/intercaudate distance and bicaudate ratios, which were compared with those of 24 age-matched healthy children and 12 age-matched patients with Leigh (n = 9) or Wilson (n = 3) disease. Atrophy of the caudate nuclei was identified in all Huntington patients. The frontal horn distance/intercaudate distance (1.64 +/- 0.39) and bicaudate (0.205 +/- 0.060) ratios of the patients with juvenile Huntington disease were found to be significantly different from those of healthy children and that of those patients with Leigh/Wilson disease. The 3 patients with Huntington disease who underwent MR evaluation were noted to have increased proton density- and T2-weighted signal in the caudate nuclei and putamina. As in adult patients, the use of frontal horn distance/intercaudate distance and bicaudate ratios are helpful for the diagnosis of Huntington disease in pediatric patients. On MR, increased proton density- and T2-weighted signal in the atrophic caudate nuclei and putamina are additional features of juvenile Huntington disease.
ISSN:0195-6108
1936-959X