Typical Clinical Findings Should Prompt Investigation for Juvenile Huntington Disease

An 11-year-old boy presented for neurological evaluation with a 5-year history of global developmental regression, abnormal involuntary movements, and worsening behavioral problems. He was adopted at 14 months of age and there was limited prenatal or family history. In early childhood, he had appropriate gross motor, fine motor, and language development, but “shakiness” was noted by his adoptive parents. In first grade, he was able to ride a bicycle, participate in gym class, dress himself, use utensils, write in cursive, and read at grade level. Since that time, a slow regression of fine motor, gross motor, and language skills was noted. His handwriting became illegible and he was having difficulty dressing himself. He walked on the edges of his feet and was no longer able to ride a bicycle or run without difficulty. His speech became unintelligible to strangers at the age of 9 years and he had significant difficulty with impulse control and memory. There was no known history of seizures. He had multiple involuntary movements of his arms and hands, such as hand-curling or arm-swinging, and had received the diagnosis of Tourette syndrome. He was under treatment with risperidone, citalopram, and clonidine for his involuntary movements without significant benefit.
Source: Pediatric Neurology - Category: Neurology Authors: Tags: Images in Child Neurology Source Type: research