Different combinations of at least three of six distinctive interictal EEG patterns and their long-lasting (> or =6-month) persistence seem to be the hallmarks of patients with BECTS at risk for neuropsychological impairments.

Ictal bradycardia is a rare, probably underestimated, manifestation of epileptic seizures whose pathophysiology is still debated. Autonomic modifications may result either from a sympathetic inhibition or from a parasympathetic activation probably due to the ictal discharge arising from or spreading to the structures of the central autonomic network. We review 60 cases of ictal bradycardia from the available literature and present three additional cases associated with left temporal lobe seizures studied by autonomic polygraphic ictal monitoring. Only 47 of the 63 reported cases were documented by simultaneous EEG and ECG recordings during an attack. About 76% of patients in whom well-localized ictal discharges were recorded had temporal or frontotemporal lobe seizures. Forty-five cases included information allowing confident localization of the side of ictal onset, and a 26 : 19 ratio of the left versus right side was evident. Simultaneous monitoring of ECG and other autonomic parameters during EEG recording in partial seizures should be performed to gain more insight into ictal semiology. Correlation of the symptoms referred to by patients with changes in autonomic parameters could avoid erroneous diagnosis of non-epileptic attacks and disclose a potentially lethal condition. Our cases confirm the preferential role of the left hemisphere in the genesis of ictal bradycardia and shed light on the relationship between suprabulbar control of autonomic function and partial epileptic seizures.

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We examined whether the anti-saccade task (AST) performance after the first methylphenidate (MPH) dose could be associated with subsequent clinical outcome in adults with attention-deficit/hyperactivity disorder (ADHD).Methods: Ninety-seven drug-naive DSM-5 ADHD adults participated in this study. The AST parameters were measured at baseline, after the first MPH-dose (10 mg orally), and 6 months after chronic MPH treatment. Results were compared with those of 50 healthy control (HC) subjects.Results: At baseline, ADHDs showed longer saccadic reaction times and more direction errors than HCs (both p < 0.00001). Acute and chronic MPH administration resulted in normalization of the AST performances. Multivariate regression analysis after adjusting for age, sex, weight, and severity of symptoms at baseline, revealed that a low percentage of direction errors after the first MPHdose (i.e., ≤10%) could predict remission at month 6 (OR: 5.84; 95% CI: 2.00-17.11; p = 0.001). Conclusions:Our findings indicate that: (1) impairments of motor planning and response inhibition in adults with ADHD are improved with MPH, and (2) a low direction error percentage after the first MPH-dose may be an independent predictor of remission. ClinicalTrials.gov identifier: NCT03411434

A woman had severe psychomotor retardation, epilepsy, rigidity, and chorioretinitis. Magnetic resonance imaging showed cerebellar and cerebral atrophy and hypointensities in T2‐weighted images of the thalami and basal ganglia. Muscle biopsy documented size variations in rounded muscle fibers, fibrosis, and minicores on electron microscopy. Merosin staining was normal. These hitherto unreported features do not permit classification of our patient within the current types of encephalomyopathy and congenital muscular dystrophies. Ann Neurol 2000;47:395–399