Prevention of Adverse Outcomes and Treatment Side Effects in Patients with Neuromuscular Disorders.

In this article, we review prevention of serious adverse clinical outcomes and treatment side effects in patients with neuromuscular disorders including myopathies and myasthenia gravis. While neither of these entities is preventable, their course can often be modified, and severe sequelae may be prevented, with the identification of risk factors and proactive attention toward treatment planning.

Myasthenia gravis and pregnancy.

Myasthenia gravis (MG) is an autoimmune disorder with bimodal age of presentation, occurring in young women of reproductive age and at an older age in men. Occasionally, MG is diagnosed during pregnancy. Management of MG includes symptomatic treatment with cholinesterase inhibitors and immunosuppressive therapy for controlling the disease activity. Treatment of MG in women of reproductive age, who may be contemplating pregnancy, requires discussion regarding the choice of medication as well as the understanding of risks/adverse effects involved with various treatments. During the peripartum period, it is essential to ensure careful monitoring of the disease state along with the well-being of the mother and fetus and to coordinate neonatal monitoring overseen by a multidisciplinary team comprising a high-risk maternal fetal medicine specialist, a neurologist familiar with these complex issues, and a neonatologist.

Tales of a department: how the COVID-19 pandemic transformed Detroit's Henry Ford Hospital, Department of Neurology-part I: the surge.

The COVID-19 pandemic has reshaped the way healthcare systems operate around the world. The major hurdles faced have been availability of personal protective equipment, intensive care unit beds, ventilators, treatments and medical personnel. Detroit, Michigan has been an epidemic 'hotspot' in the USA with Wayne County among the hardest hit counties in the nation. The Department of Neurology at Henry Ford Hospital, in the heart of Detroit, has responded effectively to the pandemic by altering many aspects of its operations. The rapid engagement of the department and enhanced utilisation of teleneurology were two of the pivotal elements in the successful response to the pandemic. In this review, we describe the transformation our department has undergone, as it relates to its infrastructure redesigning, coverage restructuring, redeployment strategies, medical education adaptations and novel research initiatives.

A retrospective analysis of the effect of general anesthetics on the successful detection of interictal epileptiform activity in magnetoencephalography.

BACKGROUND: A magnetoencephalography (MEG) study requires the patient to lie still for a prolonged period of time. In children and uncooperative adults with epilepsy, general anesthesia or sedation may be required to insure a good quality study. As general anesthetics have anticonvulsant and proconvulsant properties, we investigated whether the use of anesthesia reduced the successful detection of interictal epilepsy activity. METHODS: MEG testing was performed on 41 epilepsy patients (10 women, 31 men; 1-48 yr) while anesthetized. To determine the impact of anesthesia on the identification of epileptiform activity, the anesthesia group of patients was compared with all other patients with epilepsy who were recorded in our laboratory without anesthesia, as well as with a subgroup of children with epilepsy who were able to be recorded without the need for anesthesia. RESULTS: Propofol was used in 38 patients, etomidate in two, and one received sevoflurane. Twenty-nine (71%) were found to have interictal epileptiform activity in their MEG results. The percentage of MEG studies with a positive yield for interictal epileptiform activity is comparable with the percentage (63%) found in the patients with epilepsy undergoing MEG without anesthesia. In the 38 children younger than 18 yr, 28 (74%) had interictal epileptiform activity compared with 80% done without anesthesia. CONCLUSION: We conclude that levels of anesthesia needed to provide unconsciousness and immobility during MEG studies do not significantly alter the likelihood of recording interictal epileptiform spike activity with MEG.

Recovery in a patient with locked-in syndrome.

BACKGROUND AND PURPOSE: Sildenafil citrate has been shown to enhance neurogenesis, angiogenesis, synaptogenesis, and neurological outcome by augmentation of cyclic guanosine monophosphate (cGMP) levels in animal models of ischemic stroke. Whether sildenafil citrate may be helpful for recovery in human stroke is unknown at this time. METHODS: A 41-year-old woman with locked-in syndrome due to pontine infarction began receiving 150 mg of oral sildenafil citrate daily on a compassionate use basis in August 2003 and continues treatment at this time. Magneto-encephalography (MEG) was performed at 12 and 17 months after stroke. RESULTS: No serious adverse events have occurred. Significant milestone recoveries including standing, use of both arms, talking, and full return of swallowing have occurred, particularly after nine months of treatment. The MEG showed a significantly increased amplitude in the somatosensory cortex. CONCLUSION: Daily use of high dose sildenafil citrate appears to be safe in this patient with stroke resulting in locked-in syndrome. Further studies will be required to establish safety and efficacy.