Intracranial chordoma with a novel symptom.

Carbamazepine in the treatment of generalised tonic clonic seizures in juvenile myoclonic epilepsy Juvenile myoclonic epilepsy is characterised by bilateral myoclonic jerks on wakening, and generalised tonic clonic seizures with onset in the mid-teens, which may be preceded by typical absences in 33% of patients.' Findings on EEG include bilateral spikes or multiple spike-slow wave discharges at 4-6 Hz with a normal background; about 30% of these patients show photosensitivity or focal abnormalities, or both.' A particularly good long-term prognosis exists if compliance with valproate, the drug of choice for the treatment of juvenile myoclonic epilepsy, continues.'" In the 8-20% of patients in whom full control cannot be achieved with valproate treatment alone, low doses of the second-line anticonvulsant drugs clonazepam, cloba-zam, or phenobarbitone may be needed.'-3 Carbamazepine is not indicated for juvenile myoclonic epilepsy, with some reports suggesting that it may actually exacerbate seizures.34 In view of this consensus it was of particular interest to us that in two patients with juvenile myoclonic epilepsy, generalised tonic clonic seizures but not absences or myoclonic jerks were controlled only after carbamazepine was added to val-proate treatment. CASE 1 This woman, aged 32 years, whose mother also has generalised tonic clonic seizures, has late onset congenital adrenal hyper-plasia and juvenile myoclonic epilepsy with photosensitivity. Her first generalised tonic clonic seizure at the age of 14 years was induced by television; all others occurred on wakening. Generalised tonic clonic seizures were well controlled by carbamazepine (400 mg/day, plasma concentration 34 pmol/l), only six having occurred in 13 years, but violent myoclonic jerks on wakening and frequent, inconspicuous typical absences were resistant. Neurological examination was normal and EEG findings were consistent with juvenile myoclonic epilepsy and photosensitivity, but showed some inconsistent focal abnormalities. During her first pregnancy at the age of 29 years, the myoclonic jerks disappeared. Within a few days of the birth of a healthy son, they returned every morning after wakening, and were violent enough to make her drop her baby. Six months later she had her first gen-eralised tonic clonic seizure in three years. Juvenile myoclonic epilepsy was diagnosed and valproate (1 g/day) added. This fully controlled the myoclonic jerks and absences, and carbamazepine was withdrawn completely over two months. Three weeks later she had a second generalised tonic clonic seizure, associated with sleep deprivation and stress. Valproate was increased to 1-5 g/day but after two weeks a further three …