1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-3-70-a 10.15844/pedneurbriefs-3-9-10 Metabolic Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Progressive Spastic Cerebral Ataxia http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 09 1989 01 08 2016 3 9 70 70 Copyright: © 1989 The Author(s) 1989 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Idiopathic central diabetes insipidus followed by progressive spastic cerebral ataxia. Report of four cases

A syndrome of diabetes insipidus followed by progressive spastic cerebellar ataxia is reported in four boys from the Departments of Neurology, Pediatrics and Psychiatry, UCLA School of Medicine, Los Angeles, CA.

 Spastic Cerebellar Ataxia Histiocytosis Multiple Hypodense Areas

A syndrome of diabetes insipidus followed by progressive spastic cerebellar ataxia is reported in four boys from the Departments of Neurology, Pediatrics and Psychiatry, UCLA School of Medicine, Los Angeles, CA. In two patients central nervous system histiocytosis was detected. CT scan showed bilateral calcification of the cerebellar dentate nuclei and multiple hypodense areas in the skull; a biopsy confirmed the diagnosis of histiocytosis. A trial of Prednisone was beneficial. [1]

COMMENT. Each of these patients developed idiopathic central diabetes insipidus between the ages of two and six years and all responded to intranasal Desmopressin. Spastic cerebellar ataxia developed eight to ten years later. Histiocytosis accounts for 8-16% of cases of diabetes insipidus in children. Patients with this syndrome may benefit from treatment with corticosteroids.

 Birnbaum DC Shields D Lippe B Perlman S Phillipart M Idiopathic central diabetes insipidus followed by progressive spastic cerebral ataxia. Report of four cases Arch Neurol 1989 Sep 46 9 1001 1003 10.1001/archneur.1989.00520450071022 2775003