Journal of Clinical Neuromuscular Disease This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Case Study of an Elderly Patient With New Onset Weakness
We present a patient presented with new onset progressive proximal weakness. On examination noted to have proximal weakness on upper and lower limbs, with preserved reflexes, without sensory involvement. Blood work revealed to have elevated creatine kinase. On electromyography testing shows myopathic features and also noted to have myotonic discharges. Muscle biopsy was obtained next which showed many vacuolization, marked increase in all fat content noted. These findings led us to checking carnitine levels which were noted to be significantly reduced with elevated carnitine palmitoyltransferase levels. These findings high...
Source: Journal of Clinical Neuromuscular Disease - February 22, 2022 Category: Neurology Tags: Case Review Source Type: research

Progressive Multiple Mononeuropathy in a Patient With Familial Transthyretin Amyloidosis After Liver Transplantation
We report a patient with homozygous V122I mutation who presented with asymmetrical, upper limb predominant neuropathy rather early in his disease course, which progressed for a period of 5 years after liver transplantation before stabilization with the initiation of patisiran. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2022 Category: Neurology Tags: Case Review Source Type: research

A Case of Probable Multifocal Motor Neuropathy With Clinical Stability for Ten Years After a Single Treatment of Rituximab
We describe a case of anti-ganglioside GM1 IgM-positive multifocal motor neuropathy with typical incomplete and diminishing response to intravenous immunoglobulin over time. Sixteen years after symptom onset, rituximab was administered at 2 g/m2 over 2 weeks. No significant progression of disease has occurred over the following 10 years despite no additional treatments, including intravenous immunoglobulin, being given. Only case reports and small, mostly uncontrolled studies have reported the use of rituximab in multifocal motor neuropathy with mixed results. However, given its potential benefits and lack of an establishe...
Source: Journal of Clinical Neuromuscular Disease - February 22, 2022 Category: Neurology Tags: Case Review Source Type: research

Multiple Cranial Nerve Enhancement in Guillain–Barre Syndrome With Clinicoradiological Dissociation
Guillain-Barre Syndrome (GBS) is an acute inflammatory polyradiculoneuropathy which can lead to rapid neuromuscular respiratory failure, with an estimated annual incidence of 1-2 per 100,000 person-years. Even though cranial nerve involvement is known to occur in GBS, radiological correlation on neuroimaging studies are less frequently reported in pediatric population. We hereby report the case of a 14-year-old boy with acute motor axonal neuropathy variant of GBS, who had extensive contrast enhancement of multiple cranial nerves on Magnetic Resonance Imaging brain, associated with clinicoradiological dissociation on prese...
Source: Journal of Clinical Neuromuscular Disease - February 22, 2022 Category: Neurology Tags: Review Article Source Type: research

What Is in the Literature
This issue of What Is in the Literature focuses on articles on amyotrophic lateral sclerosis over the past year. Amyotrophic lateral sclerosis remains a challenging disorder with progression to death. Within the past year, a phase 2 trial of a drug combination showed slowing in the rate of progression. While awaiting a phase 3 trial or approval by the Food and Drug Administration, selected articles that aid the diagnosis, contribute to care, or add to general knowledge about the disease are reviewed. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2022 Category: Neurology Tags: Review Article Source Type: research

Neck Flexion Strength as a Predictor of Need for Intubation in Guillain–Barre Syndrome
Conclusions: Significant weakness of neck flexion (Medical Research Council grade ≤3) at the time of admission correlates with poor respiratory status as measured by the need for intubation in patients with GBS. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2022 Category: Neurology Tags: Original Article Source Type: research

Atypical Case of POEMS Presented as Demyelinating Polyneuropathy With Motor Conduction Block
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Letters to the Editor Source Type: research

Clinical Variant of Guillain–Barre Syndrome With Prominent Facial Diplegia After AstraZeneca Coronavirus Disease 2019 Vaccine
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Letters to the Editor Source Type: research

Miller Fisher Syndrome Presenting After a Second Dose of Pfizer-BioNTech Vaccination in a Patient With Resolved COVID-19: A Case Report
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Letters to the Editor Source Type: research

Retrospective Analysis of Mycophenolate Mofetil in Generalized Myasthenia Gravis
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Letters to the Editor Source Type: research

Determinants of Outcome in Chronic Inflammatory Demyelinating Polyneuropathy: A Longitudinal Study
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Letters to the Editor Source Type: research

Severe Sensorimotor Axonal Large Polyneuropathy Associated With Atypical P-ANCA
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Letters to the Editor Source Type: research

Ice Pack Test in Myasthenic Ptosis as an Important Tool for Clinical Practice
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Letters to the Editor Source Type: research

Atypical Clinical and Neuroradiological Findings in a Child With Bifacial Weakness With Paresthesias
Conclusions: This atypical case underlines the potential role of MRI in contributing to refining the nosological classification of GBS spectrum and optimizing individual treatment, especially in children where unusual manifestations are not infrequent and neurological examination is more challenging. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Short Report Source Type: research

Anti-TIF-1α/γ Antibody-Positive Dermatomyositis Associated With Metastatic Prostatic Adenocarcinoma
We describe a case of dermatomyositis with unusually severe oropharyngeal dysphagia and respiratory muscle weakness on presentation, who was found to have underlying metastatic prostate cancer. Prostate cancer is uncommonly associated with DM. The patient tested positive for antitranscription intermediate family-1 (anti-TIF-1, also known as anti-p155/410) antibodies, which are linked to malignancy-associated DM in adults and are associated with dysphagia and more severe cutaneous findings. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Case Review Source Type: research