HEREDITARY SPASTIC PARAPLEGIA WITH DEMENTIA

Objective: Hereditary spastic paraplegia (HSP) with dementia is a very rare condition. The aim of the paper is to present the first report o f HSP in a Fijian Indian family. Method: A psychiatrist and a general physician examined the affected members of the family on five occasion s over three years. Results: There are three affected individuals in a sibship of seven, The parents are without symptoms and the marriage i s non-consanguineous. The course of the disease has been remarkably si milar. All subjects were healthy and performing well in the early year s of school. In two, symptoms of cognitive loss preceded difficulty wi th ambulation and in the third, these symptoms appeared concurrently. All subjects had both symptoms by 13 years of age; they were unable to ambulate independently by the mid to late teens, at which time there was dysarthria spastic paraplegia and dementia. One subject suffered a three month episode of hypomanic behaviour. Over the three-year study period deterioration was slight but noticeable. Conclusions: It is po ssible that HSP is more commonly associated with pre-senile dementia t han is currently recognised. HSP with dementia is a very rare cause of failing school performance, Physical examination of the patient and o ther family members is indicated if this diagnosis is being considered .