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Computed tomography in Hallervorden-Spatz disease

Charles S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology Laboratories, Massachusetts General Hospital (Drs. Dooling and Richardson), and the Departments of Neurology-Neuropathology (Drs. Dooling and Richardson), Pathology (Dr. Richardson), and Radiology-Neuroradiology (Dr. Davis), Massachusetts General Hospital, Harvard Medical School, Boston, MA.

Computed tomography (CT) in a 34-year-old woman with Hallervorden-Spatz disease (established on the basis of clinicopathologic findings in a sister, and a typical clinical course) showed severe diffuse cerebral atrophy and ventricular enlargement. Ratios between the intercaudate distance and the width of the frontal horns (FH:CC) or the outer tables of the skull (CC:OT_cc) exceeded those found in patients with Huntington disease. CT may be helpful diagnostically in progressive cerebral disorders of late childhood or adolescence.

Address correspondence and reprint requests to Dr. Dooling, Pediatric Neurology Unit, Harvard Medical School, Massachusetts General Hospital, Boston, MA 02114.

Accepted for publication January 3, 1980.

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