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Nonabsorptive hydrocephalus associated with nephropathic cystinosis

Departments of Neurology (Dr. Ross), Pediatrics (Dr. Strife), Radiology (Dr. Towbin), and Pathology (Dr. Bove), Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati.

A child with nephropathic cystinosis developed seizures and coma. CT showed prominent sulci and slight ventricular enlargement. Nuclear cisternogram was normal. Despite successful renal transplantation and treatment of hypothyroidism, neurologic recovery was poor. CT and nuclear cisternogram 5 months later showed moderate panventricular and subarachnoid space enlargement and abnormal ventricular isotope retention. Ventriculoperitoneal shunt placement was followed by improved intellectual function, resolution of pyramidal tract signs, and control of seizures. Anisotropic crystals consistent with cystine were demonstrated in biopsy samples of arachnoid and cerebral cortex. Nonabsorptive hydrocephalus may have resulted from deposition of cystine in the meninges.

Address correspondence and reprint requests to Dr. Ross, Division of Child Neurology, Children's Hospital Medical Center, Elland and Bethesda Avenues, Cincinnati, OH 45229.

Supported in part by USPHS grants Nos. RR00123 and RR0055325 from the National Institutes of Health.

Accepted for publication May 13, 1982.

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