[Early onset of Huntington disease]

The case of a childhood form of Huntington's disease in a 12-years-old girl is reported. The patient suffered from severe psychosis, progressive difficulties in speaking, and mental deterioration. Her father committed suicide due to Huntington's disease when the patient was eight years old. Neurological examination revealed nothing but speaking difficulties. She had no signs or symptoms of involuntary movements, muscular rigidity or ataxia. SPECT-scanning showed a picture similar to that seen in adult patients with Huntington's disease. The difficulties in diagnosing Huntington's disease in children are discussed, and the necessity of the child psychiatric expertise is emphasized.