Desmin body myofibrillar myopathy
Jun. 28, 2023
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(Top left) EEG of a woman with jaw jerks (arrow) while reading. (Top middle and right) Video EEG of another woman with jaw jerks (bars and arrows) while reading. The EEG shows no detectable abnormality during jaw jerks, and possible changes are obscured by muscle activity. (Bottom) Video EEG of a 24-year-old man with simple focal seizures manifested with alexia (inability to understand written words) and 4 nocturnal generalized tonic-clonic seizures. Interictal EEG during reading showed sharp and slow waves focused in the left temporal regions (midway between middle and posterior temporal electrode). When the patient indicated his inability to understand text (arrow), the EEG showed low-amplitude fast rhythms (around 10 to 11 Hz), which were localized in the left middle temporal regions. This lasted 70 seconds before clinical recovery. MRI and interictal PET scan were normal. The patient was effectively treated with carbamazepine. (Courtesy of CP Panayiotopoulos from: Panayiotopoulos CP. Reflex seizures and related epileptic syndromes: a clinical guide to epileptic syndromes and their treatment. Revised 2nd ed. London: Springer, 2010:497-531.)