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A case of myoclonic status treated with plasmapheresis in a patient of 63 years of age who was admitted to a Spanish intensive care unit is reported. The patient showed clinical and radiological evidence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection; molecular tests did not verify this.

Neurological involvement, mostly in the form of meningoencephalitis or encephalitis, represents the leading cause of death in patients with infectious mononucleosis (EF). Central nervous system involvement usually occurs after the first 1 to 3 weeks of disease, although patients presenting with neurological signs and symptoms have been rarely reported. In this case presentation, our aim was to examine the association between acute Ebstein barr virus (EBV) infection and refractory myoclonic convulsions in a patient presenting with myoclonic status.

Objective: To systematically examine the electro-clinical characteristics of post anoxic myoclonus (PAM) and their prognostic implications in comatose cardiac arrest (CA) survivors.Methods: Fifty-nine CA survivors who developed myoclonus within 72 h of arrest and underwent continuous EEG monitoring were included in the study. Retrospective chart review was performed for all relevant clinical variables including time of PAM onset ("early onset" when within 24 h) and semiology (multi-focal, facial/ocular, whole body and limbs only). EEG findings including background, reactivity, epileptiform patterns and EEG correlate to myoclonus were reviewed at 6, 12, 24, 48 and 72 h after the return of spontaneous circulation (ROSC). Outcome was categorized as either with recovery of consciousness (Cerebral Performance Category (CPC) 1-3) or without recovery of consciousness (CPC 4-5) at the time of discharge.Results: Seven of the 59 patients (11.9%) regained consciousness, including 6/51 (11.8%) with early onset PAM. Patients with recovery of consciousness had shorter time to ROSC, and were more likely to have preserved brainstem reflexes and normal voltage background at all times. No patient with suppression burst or low voltage background (N = 52) at any point regained consciousness. In the subset where precise electro-clinical correlation was possible, all (5/5) those with recovery of consciousness had multi-focal myoclonus and most (4/5) had midline-maximal spikes over a continuous background. No patient with any other semiology (N = 21) regained consciousness.Conclusions: Early onset PAM is not always associated with lack of recovery of consciousness. EEG can help discriminate between patients who may or may not regain consciousness by the time of hospital discharge. [ABSTRACT FROM AUTHOR]

Neurological involvement, mostly in the form of meningoencephalitis or encephalitis, represents the leading cause of death in patients with infectious mononucleosis (EF). Central nervous system involvement usually occurs after the first 1 to 3 weeks of disease, although patients presenting with neurological signs and symptoms have been rarely reported. In this case presentation, our aim was to examine the association between acute Ebstein barr virus (EBV) infection and refractory myoclonic convulsions in a patient presenting with myoclonic status. [ABSTRACT FROM AUTHOR]

Introduction: Despite decades of research into the prognostic significance of post anoxic myoclonic status (MS), no consistent definition has been used to describe its clinical appearance. We set out to characterize the clinical features of MS and hypothesized that there are distinct clinical subtypes that may have prognostic implications.Methods: Video EEG reports from 2008 to 2016 were searched to identify adult patients with post anoxic MS defined as persistent myoclonus for >30min beginning within 3days of cardiac arrest in a comatose patient. Forty-three patients met inclusion and exclusion criteria. To generate definitions of the clinical features of MS, we reviewed videos of 23 cases and characterized 3 distinct clinical semiologies. An additional 20 cases were independently reviewed and categorized by 3 raters to evaluate inter-rater agreement (IRA). All 43 patients were assigned to a group based on consensus review for the first 23 patients and majority agreement for IRA patients. We also examined the relationship between semiology and outcome.Results: Three distinct clinical semiologies of MS were identified: Type 1: distal, asynchronous, variable; type 2: axial or axial and distal, asynchronous, variable; and type 3: axial, synchronous, stereotyped. For IRA, Gwet's kappa was 0.64 indicating substantial agreement. Two of 3 type 1 patients (66.6%) and 7.4% of type 2 followed commands whereas none of type 3 followed commands (p=0.03).Conclusion: We defined and validated a classification system of post anoxic MS based on clinical semiology. This classification may be a useful bedside prognostication tool. [ABSTRACT FROM AUTHOR]

Myoclonic status in non-progressive encephalopathy (MSNPE) is characterized by the recurrence of long-lasting atypical status epilepticus associated with attention impairment and continuous polymorphous jerks, mixed with other complex abnormal movements, in infants suffering from a non-progressive encephalopathy. The ketogenic diet (KD) has been used as an alternative to antiepileptic drugs (AEDs) for patients with refractory epileptic encephalopathies.Purpose: In this study we assess the efficacy and tolerability of the KD in patients with MSNPE.Methods: Between March 1, 1980 and August 31, 2013, 99 patients who met the diagnostic criteria of MSNPE were seen (58 patients in Verona and 41 patients in Buenos Aires). Six of these 99 patients were placed on the KD using the Hopkins protocol and followed for a minimum period of 24 months.Results: Twelve months after initiating the diet, three patients had a 75%-99% decrease in seizures, two had a 50%-74% decrease in seizures, and the remaining child had a less than 50% seizure reduction. In five patients with a seizure reduction of more than 50%, the myoclonic status epilepticus disappeared within 6 months after starting the diet. All patients had very good tolerability and no adverse events were identified. In most of the patients AEDs were reduced.Conclusion: The KD is a promising therapy for MSNPE, with most of our patients showing a more than 50% seizure reduction. In patients that responded well to the diet cognitive performance and quality of life also improved. [ABSTRACT FROM AUTHOR]

Propriospinal myoclonus is a rare motor disorder consisting of involuntary spinal‐generated muscular jerks. Here, we report a severe and uncommon case of propriospinal myoclonus that appeared after a vertebral fracture of T11. In this patient, the polysomnographic investigation showed continuing focal myoclonic activity in axial muscles during stable sleep and an acute progression into a myoclonic "status" associated with respiratory failure and loss of consciousness. It is necessary to consider this severe progression and support it with prompt emergency maneuvers and adequate pharmacotherapy. © 2005 Movement Disorder Society [ABSTRACT FROM AUTHOR]

Abstract: Myoclonic jerks and myoclonic status (MS) are sometimes difficult to distinguish clinically from movement disorders such as hand stereotypies, tremor, and dystonia in Rett syndrome. We describe a rare and complete video-polygraphic study of a girl with Rett syndrome (MECP2 mutation) and MS misdiagnosed as movement disorders and disclosed after video-polygraphic recordings. Corresponding to closely recurring activity of diffuse spike and polyspikes–wave-type paroxysms, rhythmic and, especially, arrhythmic myoclonias, usually asymmetrical and asynchronous, involving mainly right muscle deltoid and rarely followed by an inhibitory phenomenon, appeared. The MS improved and, most importantly, disappeared after the use of levetiracetam, with an evident antimyoclonic efficacy and a marked improvement of daily life for the patient and her caregivers. The difficulty in differentiating some typical nonepileptic behavioral features and movement disorders of patients with Rett syndrome from seizures was overcome using prolonged video-polygraphic recordings in our case. [Copyright &y& Elsevier]

The risk of aggravation of juvenile myoclonic epilepsy (JME) among 40 of 170 consecutive patients who had received carbamazepine (CBZ) or phenytoin (PHT) was determined at the Centre Saint Paul, Marseille, and the Clinique Neurologique, Hopital Pasteur, Nice, France.

It has been suggested that early myoclonic status after cardiorespiratory arrest is an agonal event. Here we describe three cases who developed early myoclonic status during a coma after cardiorespiratory arrest due to acute asthma. As consciousness improved, each patient developed Lance-Adams type multifocal myoclonus, but the eventual outcome was satisfactory. Only one patient needed assistance to walk, and all three were self caring. One patient had persistent dyscalculia. Early myoclonic status is not necessarily an agonal event, particularly when it follows arrest due to acute asthma or asphyxia. [ABSTRACT FROM AUTHOR]