[Huntington chorea in children].
Mots clés
Abstrait
BACKGROUND
The onset of Huntington's disease, a dominantly inherited degeneration of the basal ganglia, usually occurs in middle age; cases of childhood onset are rare.
METHODS
Case 1. A boy from a family including several cases of Huntington's disease gradually developed behavior disorders from the age of 5 years. He had learning and language disorders at 7 years of age and at 11 years he suffered from generalized seizures and progressive rigidity. The disease led to dementia at 17 years and the patient died a few months later. CT scan showed diffuse cortical atrophy. Case 2. A girl aged 5 years, was first examined for gait disorders. At that time she had dystonia and spasticity. Rigidity gradually developed, and 2 months later several cases of Huntington's disease were identified in her father's family. Case 3. A boy developed gait disorders from the age of 5 years. He gradually developed dystonia, rigidity, learning disorders, and finally mental retardation. The CT scan showed cortical atrophy and atrophy of the caudate nucleus. His father and others members of the paternal family had Huntington's disease that appeared in the adulthood.
CONCLUSIONS
The clinical manifestations of Huntington's disease in childhood are different from those seen in adults. Progressive alteration of mental development and dementia can occur. Diagnosis may be difficult except when a parent has the fully developed disease.
