Guillain-Barré Syndrome following ChAdOx1-S/nCoV-19 Vaccine.

As of April 22, 2021, around 1.5 million individuals in three districts of Kerala, India had been vaccinated with COVID-19 vaccines. Over 80% of these individuals (1.2 million) received the ChAdOx1-S/nCoV-19 vaccine. In this population, during this period of 4 weeks (mid-March to mid-April 2021), we observed seven cases of Guillain-Barre syndrome (GBS) that occurred within 2 weeks of the first dose of vaccination. All seven patients developed severe GBS. The frequency of GBS was 1.4- to 10-fold higher than that expected in this period for a population of this magnitude. In addition, the frequency of bilateral facial weakness, which typically occurs in <20% of GBS cases, suggests a pattern associated with the vaccination. While the benefits of vaccination substantially outweigh the risk of this relatively rare outcome (5.8 per million), clinicians should be alert to this possible adverse event, as six out of seven patients progressed to areflexic quadriplegia and required mechanical ventilatory support. ANN NEUROL 2021;90:312-314.

Understanding and Managing Autonomic Disorders in the Neurocritical Care Unit: A Comprehensive review.

Background: For a favorable outcome, patients admitted to critical care units require continuous monitoring and swift decision-making ability regarding management. One of the biggest challenges in neurocritical care units is the identification and management of autonomic dysfunction and in the worst-case scenario, autonomic storms. Objective: Most of the literature available focuses mainly on autonomic storms following traumatic brain injury. However, due to the myriad neurological presentations in a critical care setting, it is particularly important for physicians and intensivists to suspect and manage autonomic dysfunction in various neurological scenarios. Methods: Understanding the mechanism of paroxysmal sympathetic hyperactivity (PSH) is essential for early recognition and treatment. PSH-AM is an assessment measurement scale to diagnose and assess the severity of PSH in traumatic brain injury. However, this is not yet standardized across all neurological settings. Conclusion: We present a comprehensive report on understanding the mechanism of autonomic storms across various neurological disorders and outline the management.

Epilepsy with myoclonic absences in siblings.

BACKGROUND: Epilepsy with myoclonic absences (EMAs) is a distinct form of childhood epilepsy characterized by a peculiar seizure type that identifies this condition. PURPOSE: To describe the clinical, electroencephalographic features, treatment strategies and outcome in this first case series of two siblings with normal intelligence presenting with EMAs. MATERIALS AND METHODS: Both siblings underwent video-polygraphic investigations (simultaneous recording of electroencephalogram [EEG] and electromyogram [EMG] from deltoids), high-resolution magnetic resonance imaging (MRI), karyotyping, neuropsychological evaluation and language assessment. RESULTS: Both the children had a mean age of onset of prototype seizures by 3.5 years. Myoclonic absences (MAs) were characterized by rhythmic, bilateral, synchronous, symmetric 3-Hz spike-wave discharges, associated with EMG myoclonic bursts at 3 Hz, superimposed on a progressively increasing tonic muscle contraction. The interictal EEG showed a normal background activity with bursts of generalized spike and waves (SWs) as well as rare focal SWs independently over bilateral temporal and frontal regions. Increase in the seizure frequency from 5 to 100/day was observed due to use of carbamazepine and phenobarbitone which decreased with its withdrawal and introduction of valproate. Though lamotrigine was given as an add on to valproate, it did not benefit them and was therefore replaced by topiramate at 3.5 mg/kg/day which has maintained them on remission at one year follow up. CONCLUSIONS: Recognition of this ictal pattern allows identification and differentiation of EMAs from other seizure types. Idiopathic and symptomatic EMAs need to be differentiated from childhood absence epilepsy with myoclonia. MAs are worsened by drugs like carbamazepine while valproate either alone or in combination with topiramate (preferred to lamotrigine) gives excellent outcome.