American Journal of Neuroradiology, Vol 13, Issue 1 387-393, Copyright © 1992 by American Society of Neuroradiology
ARTICLES |
Partial albinism with immunodeficiency: a rare syndrome with prominent posterior fossa white matter changes
J Brismar and HA Harfi
Department of Diagnostic Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
PURPOSE: To describe our experience in infants with partial albinism and immunodeficiency (PAID), a rare, recently recognized, probably autosomal recessive disorder. PATIENTS AND METHODS: Five infants suffering from this disease were examined with CT of the brain and four of these patients also underwent MR. Four of the five children also underwent follow-up CT or MR exams. RESULTS: Three of the patients followed with serial examinations demonstrated a rapid progress of white matter changes together with a loss of brain tissue over a few months. In all four patients subjected to follow-up, the posterior fossa white matter structures were severely involved during the course of the disease. CONCLUSIONS: This syndrome should be added to the list of demyelinating diseases, and should be kept in mind when white matter changes are prominent in the posterior fossa.
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