Sea-blue histiocytes, lymphocytic cytosomes, movement disorder and59fe-uptake in basal ganglia: Hallervorden-spatz disease or ceroid storage disease with abnormal isotope scan?

Kenneth F. Swaiman, Stephen A. Smith, Gary L. Trock, Aslam R. Siddiqui

Research output: Contribution to journalArticlepeer-review

55 Scopus citations

Abstract

We studied two siblings with Hallervorden-Spatz syndrome.59Fe-uptake and disappearance studies of the brain utilizing a narrow-field-of-view collimator indicated iron retention in the basal ganglia. Ferrokinetic studies utilizing31Cr or59Fe are within normal limits. Hematologic abnormalities heretofore unreported in Hallervorden-Spatz syndrome included sea-blue histiocytes in bone marrow and cytoplasmic inclusions in circulating lymphocytes, consisting of vacuoles, fingerprint profiles, multilaminar profiles, granular osmiophilic deposits, curvilinear bodies, and increased numbers of tubular arrays. These findings demonstrate extracerebral manifestations of Hallervorden-Spatz syndrome that may aid diagnosis and shed light on the etiology.

Original languageEnglish (US)
Pages (from-to)301-305
Number of pages5
JournalNeurology
Volume33
Issue number3
DOIs
StatePublished - Mar 1983

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