IgG4-related disease: neurologic manifestations
Nov. 29, 2022
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During hyperventilation (first arrow), the patient stopped responding. There were mild horizontal head movements associated with similar leg movements, ending with a tonic spasm of the adductor muscles bringing the legs close together. The concomitant EEG abnormality was characterized by generalized spike-wave discharges of higher amplitude frontal regions. The episode lasted about 8 seconds (second arrow). Also during hyperventilation in another episode, a single eyelid jerk and a single upper limb myoclonic jerk were part of the described myoclonic absence seizure. Perinatal and past history were noncontributory. Neurologic examination, neuroimaging, and relevant investigations were normal. The electroclinical events responded to sodium valproate, and he had remained free of seizures and EEG discharges for 3 years when last seen. (Contributed by Dr. Athanasios Covanis.)