American Journal of Neuroradiology, Vol 16, Issue 7 1405-1412, Copyright © 1995 by American Society of Neuroradiology
ARTICLES |
Juvenile Huntington disease: CT and MR features
VB Ho, HS Chuang, MJ Rovira and B Koo
Department of Radiology, Madigan Army Medical Center, Tacoma, WA 98431- 5000, USA.
PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
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