Acute Motor Neuropathy Research Articles

The Association of Acute Motor Axonal Neuropathy (Guillain-Barre' Syndrome Variant) with Coronavirus (SARS-Cov-2) in a Child: A Case Report.

Various reports of neurological manifestations of SARS-COV-2 infection after the virus outbreak are available, including anosmia, seizures, acute flaccid myelitis, Guillain-Barré syndrome (GBS), and encephalitis. Most of the literature has focused on the respiratory manifestation of SARS-CoV-2 infection in adults, but recent evidence showed that it is not confined to the respiratory tract. This report is about a rare variant of GBS acute motor axonal neuropathy (AMAN) in a child due to COVID-19 infection An 11 years old boy was referred to the hospital with a history of three-day lasting mild fever, and gastroenteritis, two weeks before starting symptoms. He was presented with progressive ascending weakness, paresthesia, and areflexia in four limbs four days ago. Nasopharyngeal swab polymerase chain reaction (PCR) was positive for SARS-CoV-2. The electrodiagnostic finding was compatible with acute generalized axonal motor neuropathy, and imaging revealed thoracolumbar syrinx and nerve root enhancement in lumbosacral MRI. Other lab tests were normal. GBS and its variant are one of the manifestations of SARS-CoV-2 in children. Children with an unexplained neurological process should be tested for SARS-CoV-2.

Are Miller Fisher syndrome and CANDA due to a paranodopathy?

ObjectiveTo study the pathological characteristics of acute and chronic ataxic peripheral neuropathy at the level of the node of Ranvier. Study design and settingWe performed the pathological study (nerve biopsy of a sural nerve) of two patients, one with an acute form of ataxic peripheral neuropathy called ‘Miller Fisher syndrome’ (MFS), the other one with a chronic form called ‘chronic ataxic neuropathies with disialosyl antibodies’ (CANDA). ResultsA dysimmune process involving peripheral nerves commences in myelin, at the internodal area or/and in the paranodal and nodal regions. Our electron microscopic observations suggest that both patients present lesions in favor of a paranodopathy. ConclusionMany of the immune-mediated peripheral neuropathies are now classified as nodoparanodopathies. This subtype of auto-immune neuropathy may present various clinical phenotypes such as ‘Acute Motor Axonal Neuropathy’ (AMAN), ‘Acute Motor and Sensory Neuropathy’ (AMSAN) or ‘chronic inflammatory demyelinating polyradiculoneuropathy’ (CIDP), and are associated with anti-disialosyl antibodies. In our two cases, some paranodes seem to be associated with macrophages and we hypothesized that these lesions are in favor of a complement-mediated dysfunction/disruption of the nodal region due to disialosyl antibodies against gangliosides which are mainly located at the level of the axolemma of the paranode.

P042 Spontaneous gallbladder perforation: a rare complication of eosinophilic granulomatosis with polyangiitis

Abstract Background/Aims Gallbladder (GB) perforation is a serious clinical condition, caused by cholecystitis with or without cholelithiasis. Although there have been reports of bowel perforation in many cases of vasculitis, we report to the best of our knowledge, the first case of spontaneous gallbladder perforation following a diagnosis of EGPA. This alerts the clinician to a unique complication of EGPA. Methods A 48-year-old man presented initially to haematology with weight loss and marked eosinophilia, investigations (bone marrow aspirate and trephine, CT chest, abdomen and pelvis) at the time showed no evidence of haematological causes. Subsequently, he developed worsening weakness and paraesthesia of the upper and lower limbs, ongoing for 4 months. He had a background of asthma, and hay fever, which were well-controlled. On examination, he had progressive quadriparesis with extensive muscle wasting. His abdomen was soft non-tender, and he had no skin changes, although he later revealed a photograph of a vasculitic rash affecting the limbs when the symptoms initially presented. Results Laboratory findings revealed raised white blood cells of 46.9 (4-10 x109/L), raised eosinophils of 29.4 (0.01-0.5 x109/L), thrombocytosis of 757 (150-410 x109/L), raised CRP of 172 (0-10mg/L), raised IgE 179(<107 kU/L), and IgG 20.8(6-16 g/L). An extensive infectious screen was performed including HIV, and hepatitis (A-E), which were all negative. Vasculitic screen was negative for PR3 and MPO ANCA. However, nerve conduction studies showed evidence of confluent mononeuritis mimicking acute demyelinating sensory motor neuropathy. Following this, a CT scan was requested to exclude underlying neoplasms and infection which revealed a GB perforation with defect in the distended GB wall. He had no typical symptoms of cholecystitis, favouring an underlying gallbladder necrotizing vasculitis as the cause of GB perforation. The patient was commenced on IV cyclophosphamide and maintained on prednisolone, and azathioprine. A percutaneous cholecystostomy was inserted and immunosuppression continued with antibiotic cover. He had excellent response to therapy and his mobility improved following physiotherapy and rehabilitation. However, he continues to have significant residual weakness of the small muscles of his hand (median and ulnar nerve palsies). An MRCP was later requested, this revealed a contracted GB with no evidence of cholelithiasis or ongoing inflammation. He remained asymptomatic of GB perforation, and as a result, surgeons decided not to proceed with a cholecystectomy. Conclusion Gallbladder vasculitis (GV) can occur as a single organ vasculitis or as part of a systemic vasculitis. In the rare occasion of its occurrence, it mainly presents as symptoms of cholecystitis. Spontaneous GB perforation as a complication of GV has been noted in patients with other vasculitides like Henoch-Schonlein Purpura. However, this is the first report of a spontaneous gallbladder perforation in a patient with EGPA. Disclosure J. Ochogwu: None. A. Madathil: None. H. Mazin: None. D. Makkuni: None.

Çocuklarda nadir görülen bir Guillan Barre sendromu alt türü: Akut aksonal motor nöropati

Guillan Barre Sendromu en sık görülen akut, immün aracılı demiyelinizan poliradikulonöropatidir. Guillan Barre Sendromu klinik ve elektrofizyolojik bulgularına göre alt türlere ayrılır. Bu alt türlerden birisi olan akut aksonal motor nöropati çocuklarda çok nadir görülür. Bu çalışmada ayaklarında ve ellerinde uyuşma, gözlerini tam olarak kapatamama, peltek konuşma, ıslık çalamama şikâyeti ile başvuran ve fizik muayenesinde hiperrefleksi saptanan akut aksonal motor nöropati tanılı on altı yaşındaki erkek olguyu sunuyoruz.

Acute onset generalized weakness: A rare variant of multifocal motor neuropathy

Multifocal motor neuropathy (MMN) is a slowly progressive asymmetrical focal demyelinating neuropathy. We present a patient consistent with acute multifocal motor neuropathy with conduction blocks (AMMNCB).

Axonal-Variant Guillian-Barre Syndrome Temporally Associated With mRNA-Based Moderna SARS-CoV-2 Vaccine

We present a case of an 86-year-old woman who presented with a progressive quadriparesis two days after her second dose of Moderna SARS-CoV-2 vaccine, with cerebrospinal fluid (CSF) evidence of cytoalbuminocytological dissociation and electromyogram/nerve conduction studies (EMG/NCS) findings suggestive of acute axonal motor neuropathy. Her clinical symptoms did not improve with plasmapheresis.There appears to be a potential temporal association between the inoculation of mRNA-based SARS-CoV-2 vaccines and the development of Guillain-Barre Syndrome (GBS). Despite this possible association, infection prevention using highly effective mRNA-based vaccines remains highly recommended. Large epidemiological studies of SARS-CoV-2 vaccine-related adverse events are needed. Physicians should be aware of this possible temporal association since the prompt diagnosis and treatment of GBS can drastically improve outcomes.The aim is to report a case of axonal-variant GBS that was temporally associated with an mRNA-based SARS-CoV-2 vaccine.

NEUROPATIA AXONAL MOTORA AGUDA COM PIORA AO TRATAMENTO COM IMUNOGLOBULINA: RELATO DE CASO

Neuropatias são condições que acometem o sistema nervoso periférico, podendo estar relacionadas a doenças sistêmicas. A síndrome de Guillain-Barré é o principal exemplo quando se fala em polirradiculoneuropatia aguda, geralmente seu quadro é associado a uma infecção prévia de Campylobacter jejuni sendo a neuropatia axonal motora aguda uma de suas variantes motoras puras e tem sua incidência variante de acordo com o espaço geográfico estudado entre 30 a 65% dos quadros totais de Guillain-Barré. As manifestações dessa variação são paralisia das extremidades de caráter simétrico e ascendente, arreflexia, com acometimento da musculatura respiratória, ocular e facial. Descrevemos um caso no qual o paciente apresentou, após uma infecção de vias aéreas superiores, parestesia de mãos e pés associada a fraqueza de caráter progressiva e ascendente, diplopia e disfagia, evoluindo para insuficiência respiratória aguda, após exames comprobatórios foi iniciado protocolo de tratamento com Hiperimunoglobulina Humana Endovenosa para o tratamento de Síndrome de Guillain-Barré. O paciente evoluiu com tetraplegia flácida e necessidade de ventilação mecânica após isso, no exame de eletroneuromiografia evidenciou-se Neuropatia Axonal Motora Aguda. Por fim novo ciclo de imunoglobulina humana intravenosa obtendo resultado. O paciente recebeu alta hospitalar com sequelas motoras, mantendo acompanhamento domiciliar multiprofissional após 63 dias de internamento em unidade de terapia intensiva.

Nerve conduction study as a diagnostic tool in atypical presentation of accidental organophosphorus poisoning

Poisoning with organophosphorus (OP) compounds is a worldwide phenomenon. However, atypical presentation of OP toxicity, particularly when there is a paucity of muscarinic manifestations, can be a diagnostic challenge. We report a patient with atypical symptoms where exposure to OP poison was not clearly determined. Motor nerve conduction studies done to rule out acute motor neuropathy revealed that a single supramaximal stimulus-evoked repetitive compound motor action potentials. As this finding is the earliest and most sensitive indicator of acetylcholine esterase inhibition, OP poisoning was suspected. Decreased plasma acetylcholinesterase levels confirmed the diagnosis. The patient recovered completely with treatment. Although the diagnostic hallmark of OP poisoning is a reduction in serum and red blood cell cholinesterase activity, clinicians should be alert to recognize evidence of neuromuscular transmission failure seen on nerve conduction study to diagnose atypical or concealed cases of OP poisoning.

Acute Peripheral Motor Neuropathy Induced by Oxaliplatin-Correlated Hypokalaemia.

Neurotoxicity is one of the most common side effects of oxaliplatin-based therapy. Most patients who receive at least 3–4 months of treatment suffer from peripheral sensory neurotoxicity (PSN), characterised by the loss or impairment of tactile and proprioceptive sensory function. Motor impairment, such as muscle weakness or palsy, has been rarely described, and the physiopathology of PSN, as well as the motor symptoms due to oxaliplatin-based treatment, are not adequately understood. Here we report the case of a patient who experienced severe acute peripheral motor neuropathy as a side effect of oxaliplatin-based treatment. We also review other cases of PSN published in the literature and suggest a novel hypothesis on the physiopathology of this particular event. Take-away lessons: Not all of the neurological symptoms observed during oxaliplatin-based treatment can be traced back directly to the oxaliplatin itself, and other factors, such as electrolyte imbalances, may contribute to the symptoms. Patients with gastro-intestinal malignancies are the patients most affected by neurotoxicity due to the side effects of chemotherapy and the disease itself.Electronic Supplementary MaterialThe online version of this article (10.1007/s40487-019-00102-3) contains supplementary material, which is available to authorized users.

S40. Acute and subacute motor neuropathy in type II diabetic patients

Introduction Diabetic neuropathy is the most common complication of diabetes mellitus (DM), affecting as many as 50% of patients with type 1 and type 2 DM. There are four main types of diabetic neuropathy; Peripheral neuropathy, autonomic neuropathy, radiculo-plexus neuropathy (diabetic-amyotrophy) and mononeuropathy. The aim of the study was to describe the clinical presentation, electrophysiological investigations and in the future the immunological profile of such patients. Methods Seven type II diabetic patients were selected for the study. Ethical approval and patients’ informed consent have been obtained. They are all presented with acute and subacute onset of predominantly lower limb distal muscle weakness, on occasion proximal. The onset of this weakness was not preceded by upper respiratory tract infection, diarrhea or febrile illness. Results On examination there was symmetrical weakness of the distal foot flexors and extensors (power ranges 0–3), lower limb reflexes varies from diminished to absent. Tone was normal in the majority and flaccid in some of the patients. All modalities of sensation are intact except for disturbance of light touch in two patients. All patients were investigated for other causes of neuropathies. Neurophysiological investigations revealed signs of predominant demyelination with axonal degeneration. Patients showed very good response to immunoglobulin infusion (i.e. recovery of weakness after one to three months). Immunological studies are undergoing. Conclusion This was a preliminary report of a very uncommon presentation of acute motor neuropathy in diabetic patients. The cause possibly might be immune-mediated nerve damage but this has to be confirmed when we recruit more patients with detailed immune-profile studies.

Neurophysiological profile of peripheral neuropathy associated with childhood mitochondrial disease

IntroductionPeripheral nerve involvement is common in mitochondrial disease but often unrecognised due to the prominent central nervous system features. Identification of the underlying neuropathy may assist syndrome classification, targeted genetic testing and rehabilitative interventions. MethodsClinical data and the results of nerve conduction studies were obtained retrospectively from the records of four tertiary children's hospital metabolic disease, neuromuscular or neurophysiology services. Nerve conductions studies were also performed prospectively on children attending a tertiary metabolic disease service. Results were classified and analysed according to the underlying genetic cause. ResultsNerve conduction studies from 27 children with mitochondrial disease were included in the study (mitochondrial DNA (mtDNA) – 7, POLG – 7, SURF1 – 10, PDHc deficiency – 3). Four children with mtDNA mutations had a normal study while three had mild abnormalities in the form of an axonal sensorimotor neuropathy when not acutely unwell. One child with MELAS had a severe acute axonal motor neuropathy during an acute stroke-like episode that resolved over 12months. Five children with POLG mutations and disease onset beyond infancy had a sensory ataxic neuropathy with an onset in the second decade of life, while the two infants with POLG mutations had a demyelinating neuropathy. Seven of the 10 children with SURF1 mutations had a demyelinating neuropathy. All three children with PDHc deficiency had an axonal sensorimotor neuropathy. Unlike CMT, the neuropathy associated with mitochondrial disease was not length-dependent. ConclusionsThis is the largest study to date of peripheral neuropathy in genetically- classified childhood mitochondrial disease. Characterising the underlying neuropathy may assist with the diagnosis of the mitochondrial syndrome and should be an integral part of the assessment of children with suspected mitochondrial disease.

Clinical Phenotypes in Guillain-Barré Syndrome

Guillain-Barré syndrome can be classified into several phenotypes according to the type of predominantly disturbed nerve fibers, distribution of muscular weakness, and electrophysiological and pathological findings. Although several regional variants including Fisher syndrome and pharyngeal-cervical-brachial weakness were initially reported in Western researchers, many labor-intensive studies by Japanese researchers have significantly contributed to defining and highlighting these variants. This review summarizes the several regional variants of Guillain-Barré syndrome while highlighting the substantial contributions made by Japanese investigators. Furthermore, a new regional variant named "distal limb weakness" is proposed, in which regional weakness of hands and feet is observed throughout the disease course. It is considered a mild phenotype of acute axonal motor neuropathy after Campylobacter jejuni enteritis.

Target Molecule for a Demyelinating Type of Guillain-Barré Syndrome, Acute Inflammatory Demyelinating Polyneuropathy

Guillain-Barré syndrome is classified into demyelinating type, acute inflammatory demyelinating polyneuropathy (AIDP) and axonal form, acute axonal motor neuropathy (AMAN). It has been clearly established that the target molecule for the former is a ganglioside. In contrast, despite years of effort, the target molecule for the latter has not been identified. Recently, molecules around the nodes of Ranvier have entered the spotlight, and "moesin" was reported to be a target molecule for cytomegalovirus associated-AIDP.

Acute and subacute motor neuropathy in diabetic patients case reports

Acute and subacute motor neuropathy in diabetic patients case reports

Acute motor neuropathy after intrathecal treatment with methotrexate: A case report

Intrathecal therapy with methotrexate and cytosine-arabinoside is a therapeutic strategy for maintaining remission in patients with acute lymphoblastic leukemia, that is associated with neurological side effects are described. We report a 4-year-old girl who became tetraplegic after the last dose of intrathecal therapy. Cerebrospinal fluid showed elevated protein level and nerve conduction study revealed a motor neuronopathy. Spine magnetic resonance imaging revealed gadolinium enhancement of the anterior roots of the cauda equina. We describe a myelopathy confined to the anterior horns and anterior spinal nerve roots without sensory involvement caused by intrathecal methotrexate. The pathogenesis of this selective involvement remains uncertain.

A Rare Case of Acute Axonal Motor Neuropathy in a Puerperal Woman

ABSTRACT We report the case of a puerperal woman who presented to us with sepsis, multiorgan dysfunction and motor weakness of both lower limbs. On detailed evaluation, patient was found to have axonal neuropathy establishing the diagnosis of critical illness polyneuropathy (CIP). A high index of suspicion is required to arrive at the diagnosis as this condition is not only associated with high mortality and morbidity rates but also can affect the quality of life of the individual in the long-term. This case has been reported to highlight the importance of recognition of this common, but rarely diagnosed condition as it can help us to portend the prognosis. How to cite this article Nayak A, Sundari N, Swarup A, Girish J. A Rare Case of Acute Axonal Motor Neuropathy in a Puerperal Woman. J South Asian Feder Obst Gynae 2015; 7(1):48-49.

A Rare Case of Acute Axonal Motor Neuropathy in a Puerperal Woman

ABSTRACT We report the case of a puerperal woman who presented to us with sepsis with multiorgan dysfunction and motor weakness of both lower limbs. On detailed evaluation, patient was found to have axonal neuropathy establishing the diagnosis of critical illness polyneuropathy. A high index of suspicion is required to arrive at the diagnosis as this condition is not only associated with high mortality and morbidity rates but also can affect the quality of life of the individual in the long term. This case has been reported to highlight the importance of recognition of this common, but rarely diagnosed condition as it can help us to portend the prognosis. How to cite this article Nayak A, Sundari N, Swaroop A, Girish J. A Rare Case of Acute Axonal Motor Neuropathy in a Puerperal Woman. J South Asian Feder Obst Gynae 2015;7(2):93-94.

Rare Association of Gullain-Barré Syndrome with Non-Hodgkin’s Lymphoma: A Case Report

Guillain-Barré Syndrome (GBS) is a very common cause of acute motor neuropathy in medical ward, though its association with lymphoma is not very common. Here we present a case of acute motor neuropathy which was clinically diagnosed as a case of GBS. CSF (Cerebrospinal fluid) study and electrophysiological study supported the clinical diagnosis. The patient also had generalized lymphadenopathy & histopathological examination of lymph node revealed high grade Non-Hodgkin’s Lymphoma (NHL). A staging bone marrow study was performed which revealed lymphoreticular malignancy. The case was finally diagnosed as GBS with stage IV Non-Hodgkin’s Lymphoma. DOI: http://dx.doi.org/10.3329/jbcps.v31i3.20986 J Bangladesh Coll Phys Surg 2013; 31: 168-173

P214: Study of sympathetic skin response in diabetic patients with soft tissue rheumatism of the hand

P214: Study of sympathetic skin response in diabetic patients with soft tissue rheumatism of the hand

Acute motor and sensory polyganglioradiculoneuritis in a cat: clinical and histopathological findings.

Polyneuropathies can have a variety of clinical presentations and tend to be rare in cats. In this report we describe a 6-year-old domestic shorthair cat with an acute and rapidly progressive onset of lower motor neuron and sensory signs affecting the spinal and cranial nerves. Histopathological examination revealed moderate-to-severe multifocal inflammatory infiltrates at the ventral and dorsal nerve roots, and dorsal spinal ganglia at the level of the L4 and cauda equina. The type and severity of inflammation varied between nerve roots, being composed of mainly neutrophils in some and mainly lymphocytes and macrophages in others. Immunohistochemistry showed a combination of neutrophils, macrophages and lymphocytes infiltrating the nerve roots and ganglia. The majority of the lymphocytes were T lymphocytes; only a few B lymphocytes were seen. Neurons within the affected ganglia showed central chromatolysis and necrosis. Wallerian-like degeneration and demyelination were observed in the nerve roots. A sensory and motor polyganglioradiculoneuritis was diagnosed. An autoimmune process similar to the acute motor and sensory neuropathy subtype of Guillain-Barré syndrome in humans or an infection by an unidentified agent were considered most likely.