Epileptic disorders : international epilepsy journal with videotape
-
Case Reports
Gaucher disease: successful treatment of myoclonic status epilepticus with levetiracetam.
We present the first reported case of a rapid clinical and electroencephalographic response to intravenous levetiracetam infusion of myoclonic status epilepticus in a patient with progressive myoclonus epilepsy due to Gaucher disease. Under continuous video-EEG monitoring, the clinical myoclonic status and the electrographic ictal discharges resolved within 10 minutes after the infusion was initiated. The patient tolerated the treatment well without any reported side effects. This case suggests that levetiracetam may be a safe, effective, and well tolerated intravenous drug in patients with metabolic myoclonic status epilepticus such as Gaucher disease.
-
Involuntary movements can appear before and after initiation of vitamin B12 treatment. The pathogenesis of involuntary movements in vitamin B12 deficiency and their relationship with cobalamin injection remain unclear due to a lack of video-EEG documentation making the electroclinical correlation difficult to ascertain. Here, we report video-EEG and neuroimaging findings of an 11-month-old girl with vitamin B12 deficiency, who acutely developed involuntary movements a few days after initiation of vitamin B12 treatment with normal vitamin plasmatic levels. Abnormal movements were a combination of tremor and myoclonus involving the face, mouth, and left arm, which disappeared after discontinuation of therapy. [Published with video sequences].
-
This paper reviews the clinical trials in acute repetitive seizures and in tonic-clonic status epilepticus. There are good randomised controlled studies on the use of benzodiazepines in early status epilepticus, but an inadequate trial base in the later stages. Therapy has therefore to be based on open studies, although in the later stages there is also a dearth of open data. ⋯ The lack of information compromises optimal therapy. This paper reviews the reasons for the lack of data and the problems associated with collecting data. It is proposed that, in the first instance, the best way of improving the quality of evidence would be a multinational case registry of existing practice.
-
Gelastic seizures are classically associated with hypothalamic hamartoma. The most effective treatment for gelastic epilepsy is surgery, although confirming that a hypothalamic hamartoma is an epileptic lesion prior to surgical intervention is challenging. Here, we report the case of a patient with a hypothalamic hamartoma who was diagnosed with psychogenic non-epileptic gelastic seizures using video-EEG monitoring. [Published with video sequences].