Hallervorden-Spatz syndrome is a rare autosomal recessive hereditary condition characterized by early onset of progressive movement alteration that include dystonia, rigidity and choreoathetosis usually associated with pyramidal signs and mental deterioration. We report two sisters where diagnosis was missed till MRI showed classic imaging findings. Mutation analysis in one, revealed homozygous...

SCN2A mutations are associated with a broad spectrum of neurological phenotypes ranging from neonatal or infantile seizures with normal development to epileptic encephalopathies with severe developmental delay. Recently, movement disorders in children with SCN2A mutations and epileptic encephalopathies including infantile spasms has been described [1,2]. We present a case of a typically develop...

Two patients presented with acute neurologic deficits attributable to contralateral basal ganglia infarction 1 to 3 months after episodes of chickenpox. Both presented with hemiparesis with one patient also demonstrating ipsilateral choreoathetosis. In both patients MR revealed unilateral basal ganglia infarction. Cerebral angiography findings in one patient were normal; in the second, there wa...

A familial syndrome of progressive cerebellar ataxia and choreoathetosis, associated with oculocutaneous telangiectasia and recurrent sinobronchitis in childhood, has recently been described in the American literature. The close similarity of the clinical features observed by Boder and Sedgwick (1957, 1958), Wells and Shy (1957), Biedmond (1957), Centerwall and Miller (1958) and Ford (1960) lea...

Aim: To evaluate the influence of the classification of cerebral palsy (CP) on the caries experience in children and adolescents, and determine the types of teeth most affected by dental caries. Methods: A total of 181 individuals with CP, aged 8.30±4.10 years were examined for dental caries diagnosis in primary, mixed and permanent dentitions. The sample consisted of 96 quadriplegic individual...