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 of HSP in a Fijian indian family. Method: A psychiatrist and a general physician examined the affected members of the family on five occasions over three years. Results: There are three affected individuals in a sibship of seven. The parents are without symptoms and the marriage is non-consanguineous. The course of the disease has been remarkably similar. All subjects were healthy and performing well in the early years of school. In two, symptoms of cognitive loss preceded difficulty with 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 possible that HSP is more commonly associated with pre-senile dementia than is currently recognised. HSP with dementia is a very rare cause of failing school performance. Physical examination of the patient and other family members is indicated if this diagnosis is being considered.