A family with hereditary spastic paraparesis and epilepsy.
OBJECTIVE
We describe a family with hereditary spastic paraparesis (HSP) in which 4 of 6 affected members also have epilepsy.


METHODS
All family members were examined by 2 neurologists. Four affected and 3 unaffected family members had EEG recordings. Four affected members were investigated for other causes of spastic paraparesis and epilepsy.


RESULTS
Epileptic symptoms varied among family members: 1 had complex partial seizures, another had focal myoclonic epilepsy, and 2 had simple partial seizures secondarily generalized. All 4 had clinical or EEG evidence to support a focal origin for the epilepsy, and 2 had photoparoxysal responses on EEG. Symptoms were more severe and occurred earlier in the younger generation, suggesting genetic anticipation in this family. The onset of epilepsy developed simultaneously with, or < or = 18 years before, onset of gait disturbance. Three unaffected family members had normal EEGs.


CONCLUSIONS
The association of HSP and epilepsy should no longer be assumed to be fortuitous.
Webb
S
S
Department of Neurology, St. Vincent's Hospital, Elm Park, Dublin, Ireland.
Flanagan
N
N
Callaghan
N
N
Hutchinson
M
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D004827:Epilepsy / Q000453:epidemiology / Q000235:genetics*; D005190:Family; D005260:Female; D006801:Humans; D007494:Ireland / Q000453:epidemiology; D008297:Male; D010375:Pedigree; D010641:Phenotype; D015419:Spastic Paraplegia, Hereditary / Q000453:epidemiology / Q000235:genetics*