Sustained effort network for treatment of status epilepticus/European academy of neurology registry on adult refractory status epilepticus (SENSE-II/AROUSE).

Background: Status Epilepticus (SE) is a common neurological emergency associated with a high rate of functional decline and mortality. Large randomized trials have addressed the early phases of treatment for convulsive SE. However, evidence regarding third-line anesthetic treatment and the treatment of nonconvulsive status epilepticus (NCSE) is scarce.

Feasibility of non-invasive neuromonitoring in general intensive care patients using a multi-parameter transcranial Doppler approach.

Purpose: To assess the feasibility of Transcranial Doppler ultrasonography (TCD) neuromonitoring in a general intensive care environment, in the prognosis and outcome prediction of patients who are in coma due to a variety of critical conditions.

Methods: The prospective trial was performed between March 2017 and March 2019 Addenbrooke's Hospital, Cambridge, UK. Forty adult patients who failed to awake appropriately after resuscitation from cardiac arrest or were in coma due to conditions such as meningitis, seizures, sepsis, metabolic encephalopathies, overdose, multiorgan failure or transplant were eligible for inclusion.

Neuromuscular monitoring devices - where to go next?

Purpose Of Review: This paper reviews the clinical applications, technology, and evidence supporting the use of telemedicine devices and telehealth in neuromuscular disease.

Recent Findings: The COVID-19 pandemic interrupted standard multidisciplinary care of patients with neuromuscular disease and created a need to adapt to remote care. Telemedicine applications were rapidly introduced and have rapidly proved an important tool in maintaining specialist care.

The clinical management of neuromuscular disorders in intensive care.

Life-threatening neuromuscular disorders affect a small, but growing group of patients in the intensive care unit who present special management problems, as well as great therapeutic opportunities. In inflammatory conditions, a cure is often possible, and for chronic, genetic or degenerative conditions, achieving the previous level of function is the target. Neuromuscular experts and intensivists need to cooperate closely to achieve the best possible outcomes.

Trismus caused by paraneoplastic brainstem encephalitis.

We discuss the assessment and differential diagnoses of a middle-aged man who presented with trismus, double vision and behavioural problems. MRI scan of the brain was initially normal, but a month later showed high signal in the hippocampal region on fluid attenuated inversion recovery sequence (FLAIR) imaging. We suspected a paraneoplastic brainstem encephalitis because of his smoking history, rapidly progressive symptoms and abnormal brainstem signs.

Neuromuscular problems in the ICU.

Purpose Of Review: Patients with acute life-threatening neuromuscular disease require close cooperation between neuromuscular and intensive care specialists to achieve the best possible outcomes. The problems encountered by these patients are different from those in traditional neuromuscular practice, and neurologists consulting in the ICU need a specific skill set to provide useful guidance. However, outcomes can be very good if treatment is instituted effectively.

Practice guideline summary: Reducing brain injury following cardiopulmonary resuscitation: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

Objective: To assess the evidence and make evidence-based recommendations for acute interventions to reduce brain injury in adult patients who are comatose after successful cardiopulmonary resuscitation.

Methods: Published literature from 1966 to August 29, 2016, was reviewed with evidence-based classification of relevant articles.

Results And Recommendations: For patients who are comatose in whom the initial cardiac rhythm is either pulseless ventricular tachycardia (VT) or ventricular fibrillation (VF) after out-of-hospital cardiac arrest (OHCA), therapeutic hypothermia (TH; 32-34°C for 24 hours) is highly likely to be effective in improving functional neurologic outcome and survival compared with non-TH and should be offered (Level A).

Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein-Barr Viral Infection.

Background: Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe variant of antiphospholipid syndrome with a high mortality rate. We report a unique case of CAPS secondary to Epstein-Barr viral (EBV) infection complicated by pulmonary and intracerebral hemorrhage. A review of the CAPS literature relevant to intensive care practice is used to outline a rational approach to diagnosis and management.

Prospective Study on Noninvasive Assessment of Intracranial Pressure in Traumatic Brain-Injured Patients: Comparison of Four Methods.

Elevation of intracranial pressure (ICP) may occur in many diseases, and therefore the ability to measure it noninvasively would be useful. Flow velocity signals from transcranial Doppler (TCD) have been used to estimate ICP; however, the relative accuracy of these methods is unclear. This study aimed to compare four previously described TCD-based methods with directly measured ICP in a prospective cohort of traumatic brain-injured patients.

The role of neurosciences intensive care in neurological conditions.

The neurosciences intensive care unit provides specialized medical and nursing care to both the neurosurgical and neurological patient. This second of two articles describes the role it plays in the management of patients with neurological conditions.

The effect of secular trends and specialist neurocritical care on mortality for patients with intracerebral haemorrhage, myasthenia gravis and Guillain-Barré syndrome admitted to critical care : an analysis of the Intensive Care National Audit & Research Centre (ICNARC) national United Kingdom database.

Purpose: To analyse mortality for spontaneous intracerebral haemorrhage (ICH), myasthenia gravis (MG) and Guillain-Barré syndrome (GBS) from 1996 to 2009 in UK intensive care units (ICUs).

Methods: We used the Intensive Care National Audit & Research Centre (ICNARC) database. We identified specialised neurosciences critical care units (NCCUs) (n = 16), general ICUs with full neurological support (n = 48) and general ICUs with limited neurological support (n = 138) and undertook descriptive analyses for each condition.

Abdominal wall closure in neonates after congenital diaphragmatic hernia repair.

Purpose: Repair of Congenital Diaphragmatic Hernia (CDH) abruptly increases intra-abdominal pressure. This study sought to characterize the incidence and significance of ACS and delayed fascial closure (DFC) after CDH repair.

Methods: We reviewed the CAPSNet database from 2006 to 2011, identifying the subset of patients that developed ACS or required DFC.

Hereditary myopathy with early respiratory failure: occurrence in various populations.

Objective: Several families with characteristic features of hereditary myopathy with early respiratory failure (HMERF) have remained without genetic cause. This international study was initiated to clarify epidemiology and the genetic underlying cause in these families, and to characterise the phenotype in our large cohort.

Methods: DNA samples of all currently known families with HMERF without molecular genetic cause were obtained from 12 families in seven different countries.

Clinical and myopathological characteristics of desminopathy caused by a mutation in desmin tail domain.

Background: Most of the previously described pathogenic mutations in desmin are located in highly conserved α-helical domains that play an important role in intermediate filament assembly. The role of the C-terminus non-α-helical 'tail' domain is much less investigated and until recently mutations in this domain have been implicated in only a few patients. The majority of reported desminopathy cases caused by the tail mutations were sporadic, creating a representation bias regarding the disease frequency and phenotypic characteristics.

Clinical and genetic findings in a large cohort of patients with ryanodine receptor 1 gene-associated myopathies.

Ryanodine receptor 1 (RYR1) mutations are a common cause of congenital myopathies associated with both dominant and recessive inheritance. Histopathological findings frequently feature central cores or multi-minicores, more rarely, type 1 predominance/uniformity, fiber-type disproportion, increased internal nucleation, and fatty and connective tissue. We describe 71 families, 35 associated with dominant RYR1 mutations and 36 with recessive inheritance.

Quality of life after laparoscopic cholecystectomy for biliary dyskinesia in the pediatric population: a pilot study.

This pilot study examined symptom relief and quality of life in pediatric patients who received laparoscopic cholecystectomy surgery at our institution for biliary dyskinesia. We used two validated questionnaires: the Child Health Questionnaire (CHQ-PF28), to assess general well-being, and the Gastrointestinal Quality of Life Index (GIQLI), to measure gastrointestinal-related health. After Institutional Review Board approval, all patients under the age of 18 years who underwent laparoscopic cholecystectomy for biliary dyskinesia between November 2006 and May 2010 received mailed questionnaires.