Myoclonus is a sudden brief involuntary activity and is either epileptic or non-epileptic. Myoclonic seizures are common in juvenile myoclonic epilepsy and in the much rarer childhood epilepsies, such as Dravet syndrome and Doose syndrome. However, they also occur at any age in adults. Myoclonic seizures may occur in cortical stroke, Alzheimer's disease, Lance-Adams syndrome, autoimmune limbic encephalitis and toxic-metabolic disorders. Clarithromycin may also cause myoclonic status epilepticus. We report a patient with myoclonic status epilepticus induced by clarithromycin.
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http://dx.doi.org/10.1136/pn-2024-004464 | DOI Listing |
BMJ Neurol Open
February 2025
Mater Misericordiae Health Services Brisbane Ltd, South Brisbane, Queensland, Australia.
Background: A broad range of inflammatory and neurodegenerative conditions manifest with progressive cognitive and behavioural changes. A diagnostic challenge is the differentiation of limbic encephalitis (LE) from Creutzfeldt-Jakob disease (CJD). LE and CJD are distinct neurological conditions with distinct variations in their clinical course, with overlapping clinical presentations.
View Article and Find Full Text PDFNeurol Genet
April 2025
AP-HP, Service de Neuropédiatrie, CRMR LEUKOFRANCE, Hôpital Robert Debré, Paris, France; and.
Background And Objectives: Spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) due to acid ceramidase deficiency is a rare disorder, allelic with Farber disease, resulting from recessive variants. Patients present in early childhood with muscle weakness due to anterior horn degeneration and/or progressive drug-resistant myoclonic epilepsy. Death usually results from respiratory complications or status epilepticus during adolescence.
View Article and Find Full Text PDFEpilepsy Behav Rep
March 2025
Neurology and Stroke Unit, San Francesco Hospital, via Mannironi 08100 Nuoro, Italy.
We report cases of two elderly women with dementia who presented with a new-onset seizure disorder characterized by subtle, rhythmic muscular contractions involving the buccolingual region and the four limbs, persistent jaw opening, and abnormal cervical posture that mimicked myoclonus-dystonia syndrome and oromandibular dystonia. The symptoms lasted several minutes to a few hours. Video-polygraphic recordings revealed an electromyographic (EMG) pattern of brief, shock-like muscular contractions consistent with myoclonus that correlated with a high-amplitude (70-90 µV), 11-14 Hertz, bilaterally symmetric electroencephalographic (EEG) rhythm over the frontocentral regions.
View Article and Find Full Text PDFPract Neurol
February 2025
Neurology, Uşak Training and Research Hospital, Uşak, Turkey
Myoclonus is a sudden brief involuntary activity and is either epileptic or non-epileptic. Myoclonic seizures are common in juvenile myoclonic epilepsy and in the much rarer childhood epilepsies, such as Dravet syndrome and Doose syndrome. However, they also occur at any age in adults.
View Article and Find Full Text PDFEpilepsia
February 2025
Department of Neurology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.
Objective: Juvenile myoclonic epilepsy (JME) is a prevalent genetic generalized epilepsy with linked abnormalities in cognition, behavior, and brain structure. Well recognized is the potential for advancing understanding of the epigenetic contributions to the neurobehavioral complications of JME, but to date there has been no examination of the role of socioeconomic disadvantage in regard to the cognitive and brain health of JME, which is the focus of this investigation.
Methods: Seventy-seven patients with JME and 44 unrelated controls underwent neuropsychological assessment, structural neuroimaging, and clinical interview to delineate epilepsy history and aspects of family status.
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