Primary progressive aphasia with focal periodic sharp wave complexes: An unusual manifestation of Creutzfeldt-Jakob disease.

Background: Creutzfeldt-Jakob disease (CJD) is a devastating degenerative brain disorder caused by an abnormal isoform of a cellular glycoprotein which is known as the prion protein. A diagnosis of CJD is usually based on specific clinical signs, EEG and MRI findings, as well as the presence of the 14-3-3 protein in the cerebrospinal fluid. Although end-stage CJD usually has a typical clinical presentation, early symptoms may be variable.

Sleep-related hypermotor epilepsy in a patient with mucopolysaccharidosis type III.

Both non-epileptic sleep disturbances and epilepsy are common in patients with mucopolysaccharidoses (MPS), so diagnosis of sleep-related hypermotor epilepsy in these patients is a tackling issue. We present a case of an adult patient with MPS IIIB (Sanfilippo syndrome), who presented with numerous nocturnal events of sudden awakening and hypermotor behavior, which had been previously regarded as parasomnias. Overnight video-EEG captured numerous stereotypical seizures with ictal pattern in the frontal regions, which led the diagnosis of SHE.

Bitemporal independent 3-Hz spike-and-waves in adult patient with idiopathic generalized epilepsy and Graves disease.

Objective: We present a case of idiopathic generalized epilepsy (IGE) with seizures manifesting in the context of Graves disease and unusual interictal EEG pattern of bilateral independent 3 Hz spike and wave activity.

Case Report: A 33-year old man with three generalized tonic-clonic seizures (GTCS) in history admitted for overnight video-EEG. At the age of 28, he had his first seizure soon after being diagnosed with Graves' disease.