SUBDURAL HEMORRHAGE AS AN INITIAL SIGN OF GLUTARIC ACIDURIA TYPE-1 - A DIAGNOSTIC PITFALL

The case of a 9-month-old girl with glutaric aciduria type 1 (GA 1) is reported. On initial presentation at 6 months of age, the patient dem onstrated bilateral subdural hemorrhages and widening of the basal cis terns. After neurosurgical intervention the subdural effusions regress ed; their etiology remained unclear. At the age of 9 months the patien t presented again because of progressive loss of psychomotor abilities and a dystonic movement disorder. Cerebral MRI revealed regressive su bdural hematoma, but marked frontotemporal atrophy as well. Because of a suspected metabolic disorder, urinary analysis of organic acids was performed. This repeatedly showed marked excretion of glutaric acid, 3-hydroxyglutaric acid and glutaconic acid, indicating a diagnosis of GA 1. Considering our patient's history, we recommend the inclusion of GA 1 in the differential diagnosis of patients with unexplained subdu ral hematoma and neurological deficits.