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Objective: This study aimed to analyze the prevalence and risk factors of neuromuscular complications after lung transplantation (LT), as well as the association between neuromuscular complications and extracorporeal membrane oxygenation (ECMO) support. Methods: We retrospectively included 201 patients who underwent LT between 2013 and 2020. Patients were classified into three groups based on the presence and the pattern of postoperative leg weakness: no weakness group, asymmetric weakness group, and symmetric weakness group. Comorbidities, duration of ECMO therapy, and postoperative complications were compared between the three groups. Results: Of the 201 recipients, 16 (8.0%) and 29 (14.4%) patients developed asymmetric and symmetric leg weakness, respectively. Foot drop was the main complaint in patients with asymmetric weakness. The presumed site of nerve injury in the asymmetric weakness group was the lumbosacral plexus in 8 (50%), peroneal nerve in 4 (25%), sciatic nerve in 2 (12.5%), and femoral nerve in 2 (12.5%) patients. In multivariate analysis, the use of preoperative ECMO was found to be independently associated with asymmetric weakness (OR, 3.590; 95% CI [1.227–10.502]). Symmetric leg weakness was associated with age at LT (1.062 [1.002–1.125]), diabetes mellitus (2.873 [1.037–7.965]), myositis (13.250 [2.179–80.584]), postoperative continuous renal replacement therapy (4.858 [1.538–15.350]), and duration of stay in the intensive care unit (1.052 [1.015–1.090]). Conclusion: More than 20% of patients developed leg weakness after LT. Early suspicion for peripheral neuropathy is required in patients after LT who used ECMO preoperatively, and who suffered from medical complications after LT. [ABSTRACT FROM AUTHOR]

Infantile nephropathic cystinosis (INC) is a rare lysosomal storage disease caused by biallelic mutations in the cystinosin gene, leading to cystine accumulation in various organs. The aim of this cross‐sectional study was to investigate neuromuscular complications in a cohort of 55 patients (aged 2.8‐41.3 years, median 18.5 years) with INC. Clinical examination, jumping mechanography, clinical neurophysiology, and muscle/nerve ultrasound were performed. Physical performance, measured by mechanography, was below average in all patients. However, this reduction in physical performance was not always detected by conventional muscle power assessment. Twenty‐eight percent of patients had mostly mild axial weakness of the neck flexors and/or of the abdominal rectus muscles, the latter often presenting during childhood. One adult patient had generalized muscle weakness. Two patients had evidence of specific neuromuscular conditions, which may not have been directly related to cystinosis. 30% of patients presented with mild, 7% with moderate, and 5% with severe weakness of the intrinsic muscles of the hand. Muscle wasting was more pronounced in the older cystinosis patients with multiple organ complications. Sonographic increase in muscle echogenicity corresponded only with severe weakness. Electromyography of the intrinsic hand muscles, performed in selected patients, showed myopathic, neurogenic, or mixed myopathic‐neurogenic abnormalities. A particularly important finding of this study is that the neuromuscular complications were largely independent from both the age of initiation of pharmacological cystine‐depleting therapy and from adherence to treatment. Significant correlation was observed between better physical performance in jumping and cysteine levels in leukocytes. [ABSTRACT FROM AUTHOR]

Abstract Background Obesity is a major global health problem. Kuwait has a very high prevalence of obesity, and consequently, the number of bariatric surgeries is rising. Objectives The aim of this study is to analyze the clinical presentation and electrodiagnostic features of peripheral nerve complications following bariatric surgery. Subjects and methods We retrospectively involved a convenience sample of patients presenting at a tertiary referral center and analyzed the patterns and frequency of peripheral nerve involvement, correlations with operative techniques, perioperative complications, nutritional status, possible risk factors, and functional impairment. Results Among the 58 cases, 23 presented with chronic distal symmetrical sensorimotor neuropathy, 10 suffered from small fiber neuropathy, 22 had mononeuropathies, 2 patients had acute axonal sensorimotor neuropathy, and only 1 patient had lumbar plexopathy. In 22 patients, we observed mononeuropathies (10 cases of carpal tunnel syndrome, 7 cases of peroneal compression at the knee, 4 cases of ulnar neuropathies at the elbow, and 1 case of meralgia paresthetica). Rapid weight loss and protracted postoperative vomiting tended to correlate with generalized neuropathies, while focal compression with loss of the protective subcutaneous tissue pad was associated with mononeuropathies. All patients suffered from a deficiency of at least 1 micronutrient. Compliance with supplementary therapy was poor. Some post-bariatric neuropathies interfere severely with patients’ functional status. Conclusion Prevention by close follow-up, nutritional intervention, and patient education to avoid habitual postures related to nerve compression is appropriate.

Introduction/aims: We aimed to describe the clinical phenotype, histopathological findings and overall survival (OS) of the immune-mediated neuromuscular complications of graft-versus-host disease (GVHD).Methods: We conducted a retrospective chart review of adult patients presenting with immune-mediated neuromuscular complications of GVHD to Mayo Clinic, between April 2013 and July 2018.We collected clinical and laboratory characteristics, histopathological findings, response to treatment and survival data.Results: We identified 20 patients with a mean age at presentation of 55 y. Mean time from transplant to neurological presentation was 14 mo. Myositis was the most common complication seen in 17 patients, manifesting with predominantly axial and/or proximal weakness. Eleven patients had a muscle biopsy showing diffuse perimysial, predominantly macrophagic infiltration in 10, 3 of them with perimysial perivascular lymphocytic collections, and endomysial and perimysial lymphocytic infiltration in 1. Only two patients had a neuropathic complication: one each with acute inflammatory demyelinating polyradiculoneuropathy and neuralgic amyotrophy. A single patient had a myasthenic syndrome presenting with fluctuating foot drop. Nineteen patients were treated and all responded to immunosuppressive agents; however, 11 had further GVHD flares requiring escalation of therapy. After a median follow-up of 83 mo, seven (35%) patients died: five from progressive GVHD and two from infections. The 5-y OS from time of transplant was 68%.Discussion: Myositis is the most common immune-mediated neuromuscular complication of GVHD while peripheral neuropathy and myasthenic syndromes appear less common. The macrophage-predominant infiltration on muscle biopsy deserves further study to better clarify the role of macrophages in GVHD pathogenesis. [ABSTRACT FROM AUTHOR]

Introduction: In the last years, many new drugs have been developed targeting different oncology pathways, overall improving both quality of life and survival in several malignancies. However, the increase of those therapies is associated with novel toxicities, mainly immune-related adverse events (irAEs), never observed before. Different irAEs are now well characterized, and, among them, neuromuscular complications, following immune checkpoint inhibitor (ICPi) therapy, are increasingly studied and described. However, there are also neurological complications related to the use of other targeted therapies, less known and probably underestimated. Herein we describe two oncological patients who developed neuromuscular diseases after administration of targeted therapies, different from ICPi.Case Reports: The first patient was treated with the combination of Vemurafenib and Cobimetinib, BRAF and MEK inhibitors, respectively, for a cutaneous melanoma. One year after the beginning of the combined treatment, she developed a sub-acute motor neuropathy with predominant cranial nerve involvement. She was successfully treated with methylprednisolone. The second patient received therapy with Imatinib, tyrosine kinase inhibitor and precursor of the targeted therapy, for a gastrointestinal stromal tumour. Few days after the first administration, he developed generalized myasthenia gravis with respiratory failure. Clinical remission was obtained with plasma-exchange, intravenous immunoglobulins and steroids. DISCUSSION AND CONCLUSION: We strengthen the relevance of neuromuscular complications which may occur long after treatment start or in patients receiving not only the latest ICPi but also "older" and apparently better-known targeted therapies. Also in the latter cases, an immune-mediated "off-target" pathogenic mechanism can be hypothesized, and consequences can be life threatening, if not promptly diagnosed and appropriately managed. [ABSTRACT FROM AUTHOR]

Background: Camptocormia is a complication in which the spine bends forward while walking or standing. This axial postural deformity is common in Parkinson's disease (PD), with prevalence ranging from 3 to 18 percent; it is generally associated with a more severe disease and longer duration of symptoms. Camptocormia in PD typically responds poorly to levodopa. Other treatment options are limited and are often not effective. Case Presentation: We describe an unusual case of PD presenting with camptocormia that only emerged during the "off" state of PD. The patient was treated with classical dopaminergic anti-Parkinson's therapy plus a new formulation of palmitoylethanolamide co-ultramicronized with luteolin (Lut) termed um-PEALut. We observed that the addition of um-PEALut to acute treatment with carbidopa/levodopa resulted in improved dyskinesia and reduced camptocormia. The patient continued treatment for four months, resulting in a complete resolution of leg and trunk dyskinesia and a marked reduction in the onset of camptocormia during the "off" states. Conclusion: um-PEALut shows potential as an efficacious adjuvant therapy for patients with PD receiving carbidopa/levodopa to treat both dyskinesia and camptocormia in acute and chronic fashion. [ABSTRACT FROM AUTHOR]

Different mechanisms of neuromuscular complications, their clinical presentation, methods of diagnosis, prognosis and treatment options arepresented. The material is presented according to the level of nervous system damage, with inclusion of different clinical entities. Authors revieweda wide range of disease – disease of the spinal cord, peripheral nerves and plexus, neuromuscular junction and muscle – as a result of chemotherapy and radiation therapy. Systemic effects of drugs on peripheral nervous system, various departments of which can be simultaneously involved in the pathological process, were also highlighted.

Cancer is becoming a treatable and even often curable disease. The neuromuscular system can be affected by direct tumor invasion or metastasis, neuroendocrine, metabolic, dysimmune/inflammatory, infections and toxic as well as paraneoplastic conditions. Due to the nature of cancer treatment, which frequently is based on a DNA damaging mechanism, treatment related toxic side effects are frequent and the correct identification of the causative mechanism is necessary to initiate the proper treatment. The peripheral nervous system is conventionally divided into nerve roots, the proximal nerves and plexus, the peripheral nerves (mono- and polyneuropathies), the site of neuromuscular transmission and muscle. This review is based on the anatomic distribution of the peripheral nervous system, divided into cranial nerves (CN), motor neuron (MND), nerve roots, plexus, peripheral nerve, the neuromuscular junction and muscle. The various etiologies of neuromuscular complications – neoplastic, surgical and mechanic, toxic, metabolic, endocrine, and paraneoplastic/immune – are discussed separately for each part of the peripheral nervous system. [ABSTRACT FROM AUTHOR]

Neuromuscular diseases such as polymyositis, dermatomyositis, peripheral neuropathy, and disorders of neuromuscular transmission are reported to be complications of hematopoietic stem cell transplantation (HSCT). Although cases have been reported with allogeneic HSCT in the setting of chronic graft versus host disease, they are also known to occur without evidence thereof and even occur in the setting of autologous HSCT. The 2005 National Institutes of Health Consensus Criteria classify polymyositis and dermatomyositis as "distinctive" features, and neuropathy and MG as "other" features. These neuromuscular complications present very similarly to the idiopathic autoimmune disorders and respond to similar treatment modalities. [ABSTRACT FROM AUTHOR]

Background: A rising number of bariatric surgeries (BS) are associated with a rise in medical complications including protein malnutrition and neuromuscular complications (NM). Although BS were minimized during the COVID-19 pandemic, the number of admissions due to complications continued to increase. Proper outpatient follow-up was negatively affected during the pandemic. We aim to address the rising rate of post-BS admissions during COVID-19 pandemic compared to the previous six years' admissions with a similar diagnosis. Methods: This is a retrospective observational study of 33 patients admitted with malnutrition and/or NM complications post-BS at Hamad General Hospital, Qatar, from 1st Jan 2014-30th Aug 2020. Patients' data were retrieved from the electronic medical records and bariatric patients' database. Malnutrition was evaluated using serum albumin, total protein, minerals, and vitamins. Nerve conduction study/electromyography confirmed NM complications. Risk factors addressed were interval between BS and admission, gastrointestinal symptoms, total weight loss (TWL %), excess weight loss (EWL %), and irregular multivitamins intake. Results: The study included 21 patients, admitted from 1st Jan 2014-31th Dec 2019, compared to 12 patients during the period 1st Jan-30th August 2020. The patients' mean age was 26.90±9.81 years, and females were 18 (59%). The mean pre-operative weight, Body Mass Index (BMI), post-operative weight, BMI were: 123.48 kg, 44.37 kg/m² and 84.61 kg - 30.67 kg/m², respectively. The mean weight loss, EWL% and TWL% was 38.04 kg, 73.26%, and 30.57%, respectively. The time interval between BS and admission was 7.18±8.99 months. Seventeen patients (51.5%) had malnutrition, while 16 (58.5%) had NM complications, 87.9% were off multivitamins, and 66% had gastrointestinal symptoms. All patients showed minerals and vitamins deficiencies, especially for vitamin D (81.8%) and potassium (30.3%). Conclusion: Despite a reduction in the number of BS during the COVID-19 pandemic, an increase in the rate of hospital admissions with medical complications after BS was observed. [ABSTRACT FROM AUTHOR]

This article considers a practical approach to patients presenting with neurological signs and symptoms and highlights possible endocrine causes. It notes that behavioural and cognitive changes are associated with both hyper- and hypothyroidism. It recommends monitoring and therapy in a high dependency environment for those suffering from reduced consciousness due to severe hyperthyroidism. The clinical features of Hashimoto's encephalopathy include coma, seizures, neuropsychiatric changes and/or focal neurological deficits. It points out the importance of decompressive surgery in patients with ophthalmopathy secondary to Graves' disease.

Objective: To describe the various patterns of neurophysiological abnormalities which may complicate prolonged critical illness and identify possible aetiological factors.Design: Prospective case series of neurophysiological studies, severity of illness scores, organ failures, drug therapy and hospital outcome. Some patients also had muscle biopsies.Setting: General intensive care unit (ICU) in a University Hospital.Patients: Forty-four patients requiring intensive care unit stay of more than 7 days. The median age was 60 (range 27-84 years), APACHE II score 19 (range 8-33), organ failures 3 (range 1-6), and mortality was 23%.Results: Seven patients had normal neurophysiology (group I), 4 had a predominantly sensory axonal neuropathy (group II), 11 had motor syndromes characterised by markedly reduced compound muscle action potentials and sensory action potentials in the normal range (group III) and 19 had combinations of motor and sensory abnormalities (group IV). Three patients had abnormal studies but could not be classified into the above groups (group V). All patients had normal nerve conduction velocities. Electromyography revealed evidence of denervation in five patients in group III and five in group IV. There was no obvious relationship between the pattern of neurophysiological abnormality and the APACHE II score, organ failure score, the presence of sepsis or the administration of muscle relaxants and steroids. A wide range of histological abnormalities was seen in the 24 patients who had a muscle biopsy; there was no clear relationship between these changes and the neurophysiological abnormalities, although histologically normal muscle was only found in patients with normal neurophysiology. Only three of the eight patients from group III in whom muscle biopsy was performed had histological changes compatible with myopathy.Conclusions: Neurophysiological abnormalities complicating critical illness can be broadly divided into three types -- sensory abnormalities alone, a pure motor syndrome and a mixed motor and sensory disturbance. The motor syndrome could be explained by an abnormality in the most distal portion of the motor axon, at the neuromuscular junction or the motor end plate and, in some cases, by inexcitable muscle membranes or extreme loss of muscle bulk. The mixed motor and sensory disturbance which is characteristic of 'critical illness polyneuropathy' could be explained by a combination of the pure motor syndrome and the mild sensory neuropathy. More precise identification of the various neurophysiological abnormalities and aetiological factors may lead to further insights into the causes of neuromuscular weakness in the critically ill and ultimately to measures for their prevention and treatment. [ABSTRACT FROM AUTHOR]

The purpose of this study is to evaluate nerve and muscle physiology and histopathology in a murine lupus model. Muscle strength, compound muscle action potentials (distal latency and amplitude), proximal limb muscle, sciatic nerve and joint specimens were studied in MRL/lpr (lupus model) and MRL/++ (control) mice. MRL/lpr mice showed decreased muscle strength (P<10-6 Wilcoxon rank sum), lower compound muscle action potential mean amplitude and prolonged distal latency (P = 0.005 and 0.042, Mann-Whitney U-test), and muscle and nerve inflammation (P = 0.002 and P = 0.037, Fisher's exact test) compared with MRL/++ mice. The MRL/lpr strain evaluated in this study demonstrated muscle weakness, abnormal motor nerve conduction studies and inflammation of both muscle and nerve. These features make it an excellent model for studying the neuromuscular complications of lupus. [ABSTRACT FROM PUBLISHER]

Background: Obesity is a major global health problem. Kuwait has a very high prevalence of obesity, and consequently, the number of bariatric surgeries is rising.Objectives: The aim of this study is to analyze the clinical presentation and electrodiagnostic features of peripheral nerve complications following bariatric surgery.Subjects and methods: We retrospectively involved a convenience sample of patients presenting at a tertiary referral center and analyzed the patterns and frequency of peripheral nerve involvement, correlations with operative techniques, perioperative complications, nutritional status, possible risk factors, and functional impairment.Results: Among the 58 cases, 23 presented with chronic distal symmetrical sensorimotor neuropathy, 10 suffered from small fiber neuropathy, 22 had mononeuropathies, 2 patients had acute axonal sensorimotor neuropathy, and only 1 patient had lumbar plexopathy. In 22 patients, we observed mononeuropathies (10 cases of carpal tunnel syndrome, 7 cases of peroneal compression at the knee, 4 cases of ulnar neuropathies at the elbow, and 1 case of meralgia paresthetica). Rapid weight loss and protracted postoperative vomiting tended to correlate with generalized neuropathies, while focal compression with loss of the protective subcutaneous tissue pad was associated with mononeuropathies. All patients suffered from a deficiency of at least 1 micronutrient. Compliance with supplementary therapy was poor. Some post-bariatric neuropathies interfere severely with patients' functional status.Conclusion: Prevention by close follow-up, nutritional intervention, and patient education to avoid habitual postures related to nerve compression is appropriate. [ABSTRACT FROM AUTHOR]

A wide array of neurologic complications can occur in relation to cardiac surgical procedures, most of which are transient and do not result in permanent sequelae. Specific neurologic insults can occur depending on the type of cardiac procedure and are an important cause of morbidity and mortality. Neurologists practicing in the hospital setting as well as outpatient neurologists should be familiar with the cardiac surgical procedures currently available. Prompt identification of neurologic deficits is important in order to plan an appropriate systematic evaluation and initiate possible treatments in a timely manner. This chapter provides a comprehensive overview of all facets of neurologic complications after cardiac surgical procedures., (© 2014 Elsevier B.V. All rights reserved.)