No abstract

Background: Moyamoya disease (MMD) is a relatively important and common disease, especially in East Asian children. There are few reports about EEG in children with MMD in China till now. This study is aimed to analyze the electroencephalographic features of MMD in pediatric patients in China preliminarily. Methods: Pediatric patients with MMD who were hospitalized in Peking University International Hospital and Beijing Tiantan Hospital from January 2016 to December 2018 were collected. Clinical and electroencephalography (EEG) findings were analyzed retrospectively. Results: A total of 110 pediatric patients with MMD were involved, and 17 (15.5%) cases had a history of seizure or epilepsy. Ischemic stroke was associated with a 1.62-fold relative risk of seizure. A subset of 15 patients with complete EEG data was identified. Indications for EEG in patients with MMD included limb shaking, unilateral weakness, or generalized convulsion. Abnormal EEG was seen in 14 (93.3%) cases, with the most common findings being focal slowing 12 (80.0%), followed by epileptiform discharge 10 (66.7%), and diffuse slowing 9 (60.0%). "Rebuild up" phenomenon on EEG was observed in one patient. Conclusions: Seizure and abnormal background activity or epileptiform discharge on EEG were common in pediatric patients with MMD. EEG may play a role in differential diagnosis among the transient neurological events in MMD such as transient ischemic attack and seizure.

A 43-year-old man with uncontrolled hypertension presented with sudden headache and right homonymous hemianopsia. Brain CT revealed intracerebral hemorrhage and MRI demonstrated a hyperintense lesion on T2-weighted sequence in the splenium of the corpus callosum (Figure). Brain CTA and MRV were unremarkable. Etiological work-up in the blood and CSF were negative. Sixteen days later, the hemorrhage was smaller and the splenial lesion disappeared completely (Figure). Reversible splenial lesion syndrome (RESLES) usually results from antiseizure medication withdrawal, infection (particularly influenza virus), severe hypoglycemia, and hypernatremia [1] .