TUBEROUS SCLEROSIS

Seizures

Focal seizures are seen, with features referable to the location of the cortical tuber from which they arise. If these do not have motor features, they may be difficult to detect in infants and young children.

Focal to bilateral tonic-clonic seizures can occur, these may have focal features referable to the location of the cortical tuber from which they arise if this information is carefully sought.

Epileptic spasms may be seen and may be the presenting seizure type; these may have focal features that are referable to location of the cortical tuber from which they arise.

Status epilepticus, including focal status epilepticus, can occur.

CAUTION patients with tuberous sclerosis, who have earlier age of seizure onset, may have a change in their seizure types over time, with the emergence of epileptic spasms or generalized seizure types, such as atypical absences, atonic and tonic seizures. Developmental and cognitive impairments, and psychiatric disorders are more common in this group. If these seizure types are seen, achieving full seizure control is important, if this is not achieved with medication, epilepsy surgery should be considered.