Greater Number of Plasma Exchanges Does Not Improve Outcome in Myasthenic Crisis.

Objectives: To determine the relationship between the number of plasma exchanges and clinical outcome in patients experiencing myasthenic crisis.

Methods: We retrospectively reviewed all episodes of myasthenia gravis exacerbation/crisis who received plasmapheresis in patients admitted to a single-center tertiary care referral center from July 2008 to July 2017. We performed statistical analyses to determine whether the increased number of plasma exchanges improves the primary outcome (hospital length of stay) and the secondary outcome (disposition to home, skilled nursing facility, long-term acute care hospital, or death).

Evidence-based guideline: Neuromuscular ultrasound for the diagnosis of ulnar neuropathy at the elbow.

Introduction/aims: The purpose of this literature review is to develop an evidence-based guideline for the use of neuromuscular ultrasound in the diagnosis of ulnar neuropathy at the elbow (UNE). The proposed research question was: "In patients with suspected UNE, does ulnar nerve enlargement as measured with ultrasound accurately identify those patients with UNE?"

Methods: A systematic review and meta-analysis was performed, and studies were classified according to American Academy of Neurology criteria for rating articles for diagnostic accuracy.

Results: Based on Class I evidence in four studies, it is probable that neuromuscular ultrasound measurement of the ulnar nerve at the elbow, either of diameter or cross-sectional area (CSA), is accurate for the diagnosis of UNE.

The Incidence of Amyotrophic Lateral Sclerosis in Ohio 2016-2018: The Ohio Population-Based ALS Registry.

Introduction: Amyotrophic lateral sclerosis (ALS) is a fatal, neuromuscular disease with no cure. ALS incidence rates have not been assessed specifically in Ohio, yet the state contains both metropolitan and rural areas with a variety of environmental factors that could contribute to disease etiology. We report the incidence of ALS in Ohio residents diagnosed from October 2016 through September 2018.

Neuropathy associated with IgA monoclonal Gammopathy. A harbinger of AL amyloidosis.

Peripheral neuropathy is seen in 15% of patients with IgA monoclonal gammopathy. Treatment and prognosis of dysproteinemic neuropathy is usually guided by the underlying plasma cell disorders, which could be either benign or malignant. The true incidence of hematologic malignancy in patients with neuropathy associated with IgA monoclonal gammopathy is not known.

Spinal cord injury level and Phrenic Nerve Conduction Studies do not predict diaphragm pacing success or failure- all patients should undergo diagnostic laparoscopy.

Background: Diaphragm Pacing(DP) demonstrates benefits over mechanical ventilation(MV) for spinal cord injured(SCI) patients. The hypothesis of this report is that phrenic nerve conduction study(PNCS) results cannot differentiate success or failure in selection of patients for DP. Direct surgical evaluation of the diaphragm should be performed.

Risk factors for amyotrophic lateral sclerosis: A regional United States case-control study.

Most amyotrophic lateral sclerosis (ALS) cases are considered sporadic, without a known genetic basis, and environmental exposures are thought to play a causal role. To learn more about sporadic ALS etiology, we recruited n = 188 ALS patients from northern New England and Ohio and matched controls 2:1 from the general population of the same regions. Questionnaires evaluated the association between a variety of lifestyle, behavioral (ie, hobbies and activities), and occupational factors and the risk of ALS, including the duration of time between exposure and ALS onset, and exposure frequency.

Minimal manifestation status and prednisone withdrawal in the MGTX trial.

Objective: To examine whether sustained minimal manifestation status (MMS) with complete withdrawal of prednisone is better achieved in thymectomized patients with myasthenia gravis (MG).

Methods: This study is a post hoc analysis of data from a randomized trial of thymectomy in MG (Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone Therapy [MGTX]). MGTX was a multicenter, randomized, rater-blinded 3-year trial that was followed by a voluntary 2-year extension for patients with acetylcholine receptor (AChR) antibody-positive MG without thymoma.

Autoimmune musicogenic epilepsy associated with anti-glutamic acid decarboxylase antibodies and Stiff-person syndrome.

Epilepsy should be suspected in patients with Stiff-person syndrome and new onset paroxysmal episodes. Musicogenic epilepsy may be a manifestation of anti-GAD-Ab spectrum, supporting an autoimmune workup in these patients. Appropriate treatment is not well established, and immunotherapy should be considered in patients with only partial response to antiepileptic drugs.

Peripheral nerve hyperexcitability.

Neuromyotonic and myokymic discharges are abnormal electrical muscular discharges caused by ectopic discharges from motor axons and represent the hallmarks of peripheral nerve hyperexcitability. Neuromyotonic discharges are specific for peripheral nerve hyperexcitability syndromes, whereas myokymic discharges may occur either focally or in a more generalized fashion in many other peripheral nerve disorders. Isaacs syndrome and Morvan syndrome are rare acquired peripheral nerve hyperexcitability disorders that share common clinical features and are often associated with elevated voltage-gated potassium channel-complex antibodies.

Rescue treatment in patients with poorly responsive Guillain-Barre syndrome.

Objectives: To evaluate the effectiveness of rescue treatment (intravenous immunoglobulin or plasma exchange) in patients with Guillain-Barre syndrome who did not respond or deteriorated after the initial management with intravenous immunoglobulin.

Methods: We performed a retrospective review of the medical records of patients who responded poorly or did not respond to intravenous immunoglobulin treatment. The disability parameters of those who received second-line treatment with intravenous immunoglobulin or plasma exchange (20 patients) were compared with those who did not receive second-line treatment (19 patients).

Chronic immune sensorimotor polyradiculopathy: Report of a case series.

Introduction: Chronic immune sensorimotor polyradiculopathy (CISMP) is a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant affecting both sensory and motor nerve roots without evidence of peripheral nerve demyelination.

Methods: We report a case series of 9 patients with CISMP, identified from 2 tertiary centers. Clinical, electrodiagnostic, and neuroradiologic features, and treatment responses, were retrospectively reviewed.

Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial.

Background: The Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone (MGTX) showed that thymectomy combined with prednisone was superior to prednisone alone in improving clinical status as measured by the Quantitative Myasthenia Gravis (QMG) score in patients with generalised non-thymomatous myasthenia gravis at 3 years. We investigated the long-term effects of thymectomy up to 5 years on clinical status, medication requirements, and adverse events.

Methods: We did a rater-blinded 2-year extension study at 36 centres in 15 countries for all patients who completed the randomised controlled MGTX and were willing to participate.

Long-term experience with diaphragm pacing for traumatic spinal cord injury: Early implantation should be considered.

Background: Cervical spinal cord injury can result in catastrophic respiratory failure requiring mechanical ventilation with high morbidity, mortality, and cost. Diaphragm pacing was developed to replace/decrease mechanical ventilation. We report the largest long-term results in traumatic cervical spinal cord injury.

Autoimmune Neuromuscular Diseases Induced by Immunomodulating Drugs.

Immunomodulating drugs are widely used in autoimmune, transplant, and cancer patients. However, these drugs are associated with various autoimmune neuromuscular diseases such as demyelinating polyneuropathy, myasthenia gravis, and myositis. Early recognition of these complications and immediately terminating these drugs are very essential since some are life-threatening conditions.

Unihemispheric central nervous system vasculitis.

Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria.

Guillain-Barré Syndrome Triggered by Immune Checkpoint Inhibitors: A Case Report and Literature Review.

Immune checkpoint inhibitors, used as immunotherapy in the treatment of melanoma and refractory cancer, may trigger autoimmune disorders. We describe a case of Guillain-Barré syndrome (GBS) after the treatment with ipilimumab and nivolumab and review 4 other reported cases. We confirmed the diagnosis of GBS by electrodiagnostic and cerebrospinal fluid studies.

Peripheral Nerve Hyperexcitability Syndromes.

Purpose Of Review: This article provides a review of the clinical phenotypes and evaluation of peripheral nerve hyperexcitability syndromes. These rare diagnoses include cramp-fasciculation syndrome, Isaacs syndrome, and Morvan syndrome. Recent investigations have led to an understanding of the autoimmune underpinnings of these conditions and their specific associated antibodies.

Diagnostic challenges in POEMS syndrome presenting with polyneuropathy: A case series.

Objective: To analyze the clinical characteristics of patients with POEMS presenting with polyneuropathy and discuss associated diagnostic challenges.

Method: Retrospective analysis of 6 patients from 2 tertiary-care institutions.

Results: Six patients presented with progressive sensorimotor deficits predominantly or exclusively in the lower extremities and were diagnosed with POEMS syndrome.

Concurrent Paraspinous Myopathy and Myasthenia Gravis.

Paraspinous myopathy is a rare neuromuscular disorder characterized by selective involvement of the cervical, thoracic, or lumbar muscles. Leading clinical features include a bent spine or dropped head (antecollis). In myasthenia gravis (MG), patients may have camptocormia secondary to neuromuscular junction dysfunction of the paraspinal muscles, and this condition usually responds to acetylcholinesterase inhibitors or immunosuppressive treatments.

Abducens nerve palsy due to inferior petrosal sinus thrombosis.

Isolated unilateral abducens nerve palsy is usually due to ischemia, trauma or neoplasm. Dorello's canal is the space between the petrous apex and superolateral portion of the clivus, bound superiorly by Gruber's ligament. The abducens nerve travels with inferior petrosal sinus (IPS) though the Dorello's canal before entering the cavernous sinus.