Progressive myoclonic epilepsy (PME) is characterized by prominent myoclonus and generalized or focal seizures. A recently described novel KCNC1 mutation is associated with a specific phenotype of progressive myoclonic epilepsy, which has been defined as myoclonic epilepsy and ataxia due to potassium channel mutation (MEAK). Our case illustrates a typical presentation of this disease and the potential for misdiagnosis as idiopathic generalized epilepsy during the early phase of the disease. Unique findings that may suggest an alternative diagnosis are a progressive myoclonus, prominent ataxia/dysmetria on examination, and abnormally high amplitude in the sensory evoked potential recording. We also report a brief review of the existing literature on MEAK. Early and accurate diagnosis with genetic testing may significantly help in counseling patients and families.

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BACKGROUND While many institutions have mock simulations for a code blue or rapid response, there is no standard practice for stroke alert simulations to train neurology residents. This lack of training means residents feel less confident when responding to stroke alerts and when giving tPA early in their training.

When caring for neurosurgical patients, many will either be started on a new antiepileptic medication or will be continued on a regimen that had been started prior to hospitalization. Because of this, it is important for a hospitalist to be familiar with the potential risks and benefits of these medications, even though they may be initiated by a neurosurgeon or neurologist. This chapter discusses several common antiepileptic drugs and their uses in the inpatient setting. This is not intended to be an exhaustive discussion; as of this writing, there are at least 27 unique antiepileptic medications available in the United States alone, with more being studied and produced.

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