Objective: To report a novel phenotype of negative myoclonus in acute post-anoxic brain injury (PABI).
Methods: We performed a retrospective analysis of 18-channel video-EEG and surface-EMG (sEMG) recordings of three patients with PABI. sEMG electrodes were placed on the neck, bulbar and arm muscles.
Results: All three patients had whole body tonic posturing with intermittent brief relaxation. In patients #1 and #2, a generalized EEG burst-suppression was present. Repetitive silent periods (SPs) were noted in the sEMG channels, time-locked to EEG bursts. The bursts preceded the SPs by 135 ms and 124 ms, respectively. The average SP duration was 910 ms and 852 ms in patients #1 and 2, respectively. Patient #3 had a generalized background suppression pattern and average SP duration of 272.5 ms. The SP recruitment pattern in patient #1 was rostro-caudal whereas patient #3 had a variable recruitment pattern.
Conclusion: Acute post-anoxic negative myoclonus can be detected in comatose patients with sEMG electrodes. The muscle SPs produce intermittent relaxation of the tonic posturing. The putative generator can be cortical or reticular, similar to Lance-Adams syndrome.
Significance: We describe a novel phenotype of negative myoclonus in acute PABI. We also describe the EEG and sEMG characteristics and the localization of the putative generator.
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http://dx.doi.org/10.1016/j.clinph.2024.11.010 | DOI Listing |
BMJ Case Rep
December 2024
Internal Medicine, Hirslanden Klinik Sankt Anna, Luzern, Switzerland
A patient with reactivated varicella zoster virus (VZV) manifesting in the left-sided dermatome L3 and S2-S4 developed tonic spasms which morphed into myoclonic jerks, paresis, rigidity and hypoesthesia of the left leg. Later, stimuli-sensitive myoclonus progressed to affect the upper body and was accompanied by fever surges with high-frequency myoclonus, hypertensive derailment, dysphagia and other features of the brainstem with autonomic dysfunction. Cerebrospinal fluid tested positive for VZV, MRI showed no signs of myelitis and EEG was negative for epilepsy.
View Article and Find Full Text PDFSeizure
November 2024
Department of Neurology, Royal Victoria Infirmary, Queen Victoria Rd, Newcastle-Upon-Tyne NE1 4LP, United Kingdom; Translational and Clinical Research Institute, Henry Wellcome Building, Framlington Place, Newcastle-Upon-Tyne NE2 4HH, United Kingdom. Electronic address:
Purpose: Mutations in NUS1 cause a neurological congenital glycosylation disorder which encompasses a spectrum from developmental encephalopathy to musculoskeletal, hearing, and visual abnormalities. Pathogenic variants include both point mutations and genomic deletions. We report an adult phenotype of progressive myoclonus epilepsy (PME) and a review of cases with a complete or partial deletion of NUS1.
View Article and Find Full Text PDFClin Neurophysiol
January 2025
Epilepsy Center, Neurological Institute, University Hospitals Cleveland Medical Center, Cleveland, OH, USA; Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH, USA. Electronic address:
Neurohospitalist
October 2024
Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
We describe the case of a 36-year-old woman with a past medical history of low grade right frontal lobe glioma and focal epilepsy presenting with subacute, progressive, multifocal myoclonus and neck and back pain. Unlike her typical seizures, the myoclonus exhibited a distinct semiology, involving both positive and negative muscle jerks affecting multiple limb muscles while sparing the face. In addition, neurological examination revealed low-amplitude, arrhythmic movements of the hands and fingers, resembling minipolymyoclonus.
View Article and Find Full Text PDFNeurohospitalist
September 2024
Department of Neurology, University Hospital "12 de Octubre", Madrid, Spain.
Background: Scrub typhus is an acute febrile infectious disease highly prevalent in the Asia Pacific region, often referred to as the "tsutsugamushi triangle." This mite-borne rickettsial zoonosis is caused by , an intracellular Gram-negative organism that primarily targets endothelial cells. The resulting vasculitis leads to multisystem involvement.
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