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Sudden unexpected death in epilepsy (SUDEP) is considered cause of many deaths in young people with epilepsy.We describe a 14-year-old male who experienced a prolonged tonic-clonic seizure, followed by respiratory insufficiency, and therefore requiring transfer to the intensive care unit.Magnetic resonance imaging (MRI) obtained 48 hours after the seizure revealed multiple and bilateral T2-signal hyperintensities. The followup MRIs (8 months) showed a partial resolution of the MRI abnormalities. As a “near miss” patient at the first seizure, this case offers several points of discussion.

We investigated the neurochemical pattern in patients with Benign Adult Familial Myoclonic Epilepsy (BAFME/FAME), an inherited form of myoclonic epilepsy, by proton MR spectroscopy (1H-MRS).Methods: eleven BAFME patients from three families showing linkage to 2p11.1-q12.2 were compared with eleven age-matched healthy control subjects. Results: patients with BAFME displayed elevated Choline/creatine ratio in the cerebellum cortex (p = 0.01). No 1H-MRS values in the frontal and occipital cortex significantly differed in the patients compared with the healthy controls. No correlation was detected between 1H-MRS values and disease duration (ρ = -0,35) as well as myoclonus severity (ρ = -0,48).Conclusions: our findings confirm that cerebellum is the prominent site of dysfunction in BAFME. 1H-MRS was able to detect brain changes also in patients with recent disease onset and may be a useful tool supporting the diagnosis based on familial and electrophysiologic data.

Epileptic seizures occur more frequently in multiple sclerosis (MS) patients than in the general population. We evaluated clinical, electroencephalographic (EEG) and magnetic resonance imaging (MRI) findings, as well as EEG-MRI correlations and the response to antiepileptic drugs (AEDs) in 270 consecutive patients with definite MS referred to our Department from 1995 to 2002. Thirteen (4.8%) subjects experienced epileptic seizures. In four cases, seizures manifested within 1-2 years ("early-onset"), and in six cases within 8-23 years ("late-onset") of MS diagnosis. Seizures were usually partial with secondary generalization. Thus, acute symptomatic seizures occurred in three cases. Epilepsy usually appeared late in the course of disease, although a single episode or a cluster of seizures can represent the onset symptom or a relapse of MS. Prognosis of epilepsy during the course of MS is usually good but the choice of AEDs remains a matter of debate.