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A family with hereditary spastic paraparesis and epilepsy
S Webb1, N Flanagan, N Callaghan
1Department of Neurology, St. Vincent's Hospital, Elm Park, Dublin, Ireland.
Purpose:
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.