Abstract
An 8-year-old boy with idiopathic basal ganglia calcification was reported. He was brought to our clinic on July 12th, 1978 because of gait disturbance and intension tremor of the left hand. The family history was not particular. His gestational and neonatal history was unremarkable. He had generalized convulsions at the ages of 20 days and 27 days. A slight delay in intellectual milestones was recognized but he did well until 10 months before admission. On admission neurological examination revealed horizontal nystagmus at lateral gaze, slurred speech, and intention tremor, dysmetria, dysdiadochokinesia of the left hand. Equilibrium reaction was slightly disturbed on the left side. Laboratory studies were within normal range including complete blood count, urinalysis and blood chemistry. The analysis of cerebrospinal fluid was normal. The endcrinological studies revealed normal secretion of GH, TSH, FSH, LH, and cortisol. Ellsworth-Howard test gave a normal result. An electroencephalogram showed slight slow dysrhythmga in the background activities especially on the right occipital region but did not show any abnormal paroxysmal activities. Roentgenography and computed tomography of the skull disclosed bilaterally symmetric calcification in the basal ganglia and dentate nuclei. Angiographies of both caroteid and the right vertebral artery were normal.
Our case did not show any clinical features and laboratory data of the following disorderes; hypoparathyroidism, pseudohypoparathyroidism, pseudopseudohypoparathyroidism, hyperthyroidism, toxoplasmosis, tuberous sclerosis, Cockayne's syndrome. From these findings, this patient was considered to be a case of idiopathic basal ganglia calcification.
