morvan syndrome

Thymoma is often associated with paraneoplastic neurologic diseases. Neural autoantibody testing is an important tool aiding diagnosis of thymoma and its autoimmune neurologic complications. Autoantibodies specific for muscle striational antigens and ion channels of the ligand-gated nicotinic acetylcholine receptor superfamily are the most prevalent biomarkers. The autoimmune neurologic disorders associating most commonly with thymoma are myasthenia gravis (MG), peripheral nerve hyperexcitability (neuromyotonia and Morvan syndrome), dysautonomia, and encephalitis...

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In autoimmune neurological diseases, the autonomic nervous system can be the primary target of autoimmunity (e.g. autoimmune autonomic ganglionopathy), or, more frequently, be damaged together with other areas of the nervous system (e.g. Guillain-Barré syndrome). Patients with autoimmune encephalitis and paraneoplastic neurological syndromes (PNS) often develop dysautonomia; however, the frequency and spectrum of autonomic signs and symptoms remain ill defined except for those scenarios in which dysautonomia is a core feature of the disease...

A man in his mid-60s presented with a 3-month history of progressive muscle twitching, agitation, cognitive impairment, insomnia, hyperhidrosis and lower limb pain. He had fasciculations, myokymia, myoclonus, exaggerated startle response and significant postural hypotension. Electrophysiological studies showed evidence of peripheral nerve hyperexcitability with neuromyotonia. Contactin-associated protein-like 2 antibodies (CASPR2) were strongly positive. A diagnosis of Morvan syndrome was made. CT of the chest, abdomen and pelvis was undertaken to identify any occult malignancy, and a large bowel carcinoma in situ was identified and resected...

INTRODUCTION: Anti-contactin-associated protein-like 2 (CASPR2) is classically associated with limbic encephalitis (LE), Morvan syndrome and peripheral nerve hyperexcitability (PNH). Additional clinical features have been previously recognized. OBJECTIVE: To describe a cohort of patients with anti-CASPR2-associated neurological syndromes from a tertiary referral centre. METHODS: Retrospective analysis of patients with positive serum anti-CASPR2 antibodies in the period between 2014 and 2021...

Motor symptoms are common, and sometimes predominant, in almost all nonparaneoplastic CNS disorders associated with neural antibodies. These CNS disorders can be classified into five groups: (1) Autoimmune encephalitis with antibodies against synaptic receptors, (2) cerebellar ataxias associated with neuronal antibodies that mostly target intracellular antigens. (3) Stiff-person syndrome and progressive encephalomyelitis with rigidity and myoclonus which have antibodies against glutamic acid decarboxylase and glycine receptor, respectively...

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Autoantibodies against LGI1 and Caspr2 complexed with voltage-gated potassium channels (VGKC) cause limbic encephalitis. Anti-LGI1 encephalitis progresses in a subacute course with memory impairment, disorientation, and focal epileptic seizures. Anti-LGI1 encephalitis is preceded by faciobrachial dystonic seizures (FBDS), which are specific involuntary movements and frequently complicated by hyponatremia due to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Neutralization of LGI1 by anti-LGI1 antibodies reduces AMPA receptors and induces epileptic seizures and memory impairment...

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OBJECTIVES: Autoimmune encephalitis arising from autoantibodies against leucine-rich glioma-inactivated protein 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) are rare and with high clinical heterogeneity. They are easily misdiagnosed and missing diagnosed. This study aims to explore the clinical characteristics, auxiliary examinations, therapies and prognosis of anti-LGI1 and anti-CASPR2 encephalitis. METHODS: Seventeen anti-LGI1 and 11 anti-CASPR2 encephalitis patients who were admitted to the Department of Neurology, Xiangya Hospital, Central South University between January 2018 and January 2021 were collected and retrospectively analyzed...

Contactin-associated protein-like 2 autoimmunity is an uncommon disorder resulting in peripheral nerve hyperexcitability or encephalitis. In a fifth of cases, onset may be provoked by thymoma, but other associations are largely unknown. We report a patient with anti-contactin-associated protein-like 2-related peripheral nerve hyperexcitability arising in the setting of Charcot-Marie-Tooth type 4F and discuss potential mechanisms underlying the association.

We illustrate the case of a 71-year-old male who initially presented with sudden onset muscle weakness and ambulation difficulty. Following medication discontinuation and additional clinical studies, he failed to improve and was admitted to the hospital 11 weeks later. He had an associated 20-pound weight loss, sudorrhea, and muscle stiffness only when weight-bearing. A complete connective tissue cascade and a paraneoplastic panel were obtained. Clinical diagnosis of acquired neuromyotonia, or Isaacs syndrome (IS), was made, and he began experiencing significant improvement after intravenous steroid infusion...

Paraneoplastic neurologic syndromes (PNS) are neurologic disorders that can affect any part of the nervous system, occur in association with cancer, and have an immune-mediated mechanism that produces direct damage to the neural tissue. Neurological symptoms frequently precede, in months to years, the symptoms directly attributed to the primary tumor, requiring a high clinical suspicion for adequate investigation. We report the case of a man in his early 80s admitted for an altered level of consciousness, alternating between periods with stupor and drowsiness, short-term waking states and psychomotor agitation, respiratory failure and dysautonomia, resembling a Morvan's syndrome...

BACKGROUND/AIM: Olaparib was approved in 2014 by the European Medicines Agency (EMA) as maintenance treatment for patients with breast cancer gene (BRCA)-mutated platinum-sensitive relapsed high-grade epithelial ovarian cancer (EOC) following the results of the Study 19. We present the results of a national real-world study on the effectiveness of olaparib in relapsed BRCA-mutated EOC patients. PATIENTS AND METHODS: Patients with EOC, peritoneal, and/or fallopian-tube cancer treated with olaparib in a French Center between May 2014 and March 2017 were included...

UNLABELLED: Autoimmune encephalitis (AIE) is an inflammatory brain condition with multiple aetiologies but is mainly associated with paraneoplastic syndromes. Several antibodies described in AIE are being investigated in relation to different cancers, including antibodies against Contactin-associated protein-like 2 (Caspr2), which have been associated with thymoma but very rarely with lung cancer. The authors present the case of a 72-year-old man with cardiovascular risk factors, who presented with a 3-week history of left hemichorea following a first unprovoked seizure the week before, with no other signs or symptoms...

BACKGROUND: Apart from myasthenia gravis (MG), thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes (PNSs). Here, we report on a rare case presenting with four different PNSs, namely, MG, membranous nephropathy, cutaneous amyloidosis, and Morvan's syndrome associated with thymoma. CASE PRESENTATION: A middle-aged man was frequently hospitalized because of nephrotic syndrome (stage I membranous nephropathy), cutaneous amyloidosis, and MG with acetylcholine receptor (AChR) antibody and titin antibody positivity...

OBJECTIVES: Every year, foetal alcohol spectrum disorders impact 1 in 100 live births in France. France is one of the few countries with mandated labelling that must include a pregnancy warning. However, as the regulation passed with minimal specifications regarding the size and colour of the ensuing pictogram, the current pregnancy warning labels (PWLs) is often barely visible. This study investigated the potential influence of the PWL design on women's attention and alcohol product choice...

There are currently no data regarding characteristics of critically ill patients with Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) variant of concern (VOC) 20H/501Y.V2. We therefore aimed to describe changes of characteristics in critically ill patients with Covid-19 between the first and the second wave when viral genome sequencing indicated that VOC was largely dominant in Mayotte Island (Indian Ocean). Consecutive patients with Covid-19 and over 18 years admitted in the unique intensive care unit (ICU) of Mayotte during wave 2 were compared with an historical cohort of patients admitted during wave 1...

Tears in the gluteus medius and minimus tendons are a common cause of greater trochanter pain syndrome (GTPS). Given the non-specific clinical signs and imaging findings, they are often misdiagnosed, with delayed treatment. The lesions can show several aspects: trochanteric bursitis, simple tendinopathy, partial or full-thickness tear, tendon retraction, or fatty degeneration. Non-surgical treatment associates physical rehabilitation and activity modification, oral analgesics, anti-inflammatories and peri-trochanteric injections (corticosteroids, PRP)...

PURPOSE OF REVIEW: Autoimmune neuromyotonia encompasses a group of rare immune-mediated neurological disorders frequently associated with anti-contactin-associated protein-like 2 (CASPR2) antibodies and featuring clinical and electrical signs of peripheral nerve hyperexcitability (PNH). We aim to summarize the current knowledge on immune-mediated neuromyotonia, focusing on clinical presentations, pathophysiology, and management. RECENT FINDINGS: Neuromyotonia is a major feature of several autoimmune neurological syndromes characterized by PNH with or without central neurological system involvement...