A 7-year-old right-handed girl presented to the pediatric neurology outpatient clinic after 5 episodes of headache over the previous 3 months. Her family history was positive for migraine in the mother and maternal grandmother and for febrile seizures in the older sister. The neurologic examination and cognitive profile were normal. Five seconds after the end of hyperventilation, video-EEG showed high-amplitude delta waves predominantly over the left hemisphere with concomitant acute aphasia and right-sided weakness. After the event, which self-resolved over 8 minutes, the girl showed intact recall. A second instance of hyperventilation evoked the appearance of pseudo-rhythmic slow activity localized to the right hemisphere, associated with left-sided weakness, 20 seconds after the end of the test. This event spontaneously resolved in 3 minutes and was followed by headache.An exaggerated physiologic response to hyperventilation, the possible epileptic nature of the events, and a migraine variant were all considered in the differential. Nonetheless, the EEG slowing is shorter in duration and generalized in physiologic and paraphysiological conditions. A clear ictal morphology and evolution of the EEG activity were lacking in this case, and migraine attacks induced by hyperpnea have not been reported to date. Instead, EEG alterations similar to that observed in our patient are described in association with vascular abnormalities. We report the clinical presentation and diagnostic workup of a rare cerebrovascular disorder, highlighting the key features in the differential. Our case emphasizes the clinical value of the EEG rebuild-up phenomenon, which can help the clinician in achieving a prompt diagnosis.
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Neuroimage
December 2024
INSERM U1114, Cognitive Neuropsychology and Pathophysiology of Schizophrenia, 1 place de l'Hôpital, 67091 Strasbourg Cedex, France; INSERM U1329, team Psychiatry of STEP (Strasbourg Translational nEuroscience and Psychiatry), 1 place de l'Hôpital, 67091 Strasbourg Cedex, France. Electronic address:
Time prediction is pervasive, and it is unclear whether it is supra-modal or task-specific. This study aimed to investigate the role of motor temporal prediction in preparing to stop a movement following a sensory stimulus. Participants performed a straight-line movement with their finger until a target signal, which occurred after a short or long foreperiod.
View Article and Find Full Text PDFBrain Commun
December 2024
CNNP Lab, Interdisciplinary Computing and Complex BioSystems Group, School of Computing, Newcastle University, NE4 5DG Newcastle upon Tyne, UK.
EEG slowing is reported in various neurological disorders including Alzheimer's, Parkinson's and Epilepsy. Here, we investigate alpha rhythm slowing in individuals with refractory temporal lobe epilepsy compared with healthy controls, using scalp EEG and magnetoencephalography. We retrospectively analysed data from 17 (46) healthy controls and 22 (24) individuals with temporal lobe epilepsy who underwent scalp EEG and magnetoencephalography recordings as part of presurgical evaluation.
View Article and Find Full Text PDFMol Genet Metab
November 2024
Scott-Ritchey Research Center, College of Veterinary Medicine, Auburn University, AL 36849, United States of America; Department of Anatomy, Physiology & Pharmacology, College of Veterinary Medicine, Auburn University, AL 36849, United States of America. Electronic address:
Though it has no catalytic activity toward GM2 ganglioside, the GM2 activator protein (GM2A) is essential for ganglioside hydrolysis by facilitating the action of lysosomal ß-N-acetylhexosaminidase. GM2A deficiency results in death in early childhood due to rapid central nervous system deterioration similar to the related GM2 gangliosidoses, Tay-Sachs disease and Sandhoff disease. This manuscript further characterizes a feline model of GM2A deficiency with a focus on clinical and biochemical parameters that may be useful as benchmarks for translational therapeutic research.
View Article and Find Full Text PDFNeuroimage
November 2024
Department of Otolaryngology, Head and Neck, University of Tübingen, Tübingen 72076, Germany. Electronic address:
The slowing and reduction of auditory responses in the brain are recognized side effects of increased pure tone thresholds, impaired speech recognition, and aging. However, it remains controversial whether central slowing is primarily linked to brain processes as atrophy, or is also associated with the slowing of temporal neural processing from the periphery. Here we analyzed electroencephalogram (EEG) responses that most likely reflect medial geniculate body (MGB) responses to passive listening of phonemes in 80 subjects ranging in age from 18 to 76 years, in whom the peripheral auditory responses had been analyzed in detail (Schirmer et al.
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