Clinical features of neuroleptic malignant syndrome in basal ganglia disease. Spontaneous presentation in a patient with Hallervorden-Spatz disease in the absence of neuroleptic drugs.

Abstract

Hallervorden-Spatz disease is a rare autosomal recessive disorder in which dopaminergic deficiency in the substantia nigra and its nigrostriatal projection has been identified. It is characterised by a slow but progressive course culminating in death. This case report describes a 13-year-old male, with a clinical diagnosis of Hallervorden-Spatz disease, who developed recurrent episodes of an acute illness, the features of which closely resembled those of the neuroleptic malignant syndrome. However, in this patient there had been no exposure to neuroleptic medication. The clinical events in this case suggest that dopaminergic hypoactivity, which is characteristic of Hallervorden-Spatz disease, can trigger episodes of neuroleptic malignant syndrome.