Desmin body myofibrillar myopathy
Jun. 28, 2023
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Video-EEG recording of myoclonic-atonic seizures in a 3-year-old boy consisting of a massive jerk followed by a loss of tone. Before the seizure, the patient was standing and speaking with his mother. Suddenly, he fell to the ï¬oor onto his buttocks. The EEG documented a high-voltage generalized spikes and polyspikes discharge associated with massive myoclonia followed by a slow wave associated with loss of tone of the same muscles. Polygraphic channels documented the main involvement of proximal limb muscles (both the extensor and the ï¬exor muscles) associated with muscle atonia. (Contributed by Dr. Nicola Specchio.)