Demyelinating polyneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a most common chronic immune-mediated demyelinating neuropathy, and includes a number of clinical subtypes. The major phenotype is "typical CIDP", which is characterized by symmetric polyneuropathy and "proximal and distal" muscle weakness. During the historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (MAG) neuropathy, and autoimmune nodopathy have been excluded. Currently CIDP is considered as a syndrome including typical CIDP and CIDP variant such as distal CIDP and multifocal CIDP...

POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes) refers to a rare paraneoplastic entity linked to a plasma cell disorder, characterized by multiple systemic manifestations that must be studied together to establish a timely diagnosis. We report a case of a 60-year-old female who had been initially classified to have Guillain Barré syndrome for one year and seven months, receiving three cycles of immunoglobulin without a positive response...

Miller Fisher syndrome is a rare and atypical variation of Guillain-Barré syndrome, which includes the clinical triad of areflexia, ataxia, and ophthalmoplegia. Miller Fisher syndrome is commonly associated with the involvement of the lower cranial and facial nerves. Miller Fisher syndrome is one of the types of Guillain-Barré syndrome. Guillain-Barré syndrome has been defined to be the foremost incapacitating form of neurological disease following the disease polio. Guillain-Barré syndrome is a broad category that encompasses several types of acute immune-mediated polyneuropathies, the most common of which is acute inflammatory demyelinating polyradiculoneuropathy...

BACKGROUND: Brachial plexus magnetic resonance imaging (MRI) is an important noninvasive supplementary diagnostic method of chronic immune peripheral neuropathies, but few MRI studies on the preganglionic nerves have been conducted. This retrospective cross-sectional study aimed to establish a reliable assessment for brachial plexus preganglionic nerve thickness and to use this method to assess and compare nerve characteristics in various types of peripheral neuropathies. METHODS: Hospitalized patients diagnosed as positive for anti-neurofascin-155 (NF155)-positive autoimmune nodopathy (AN) (NF155+ ), chronic inflammatory demyelinating polyneuropathy (CIDP), or multifocal motor neuropathy (MMN) at Huashan Hospital of Fudan University in Shanghai, China, who underwent brachial plexus MRI between October 2011 and August 2023 were consecutively recruited for this study...

Recent advances in genetic and antibody testing have limited pathological examination of peripheral nerve specimens. However, when examining peripheral neuropathological findings from a modern perspective, there is often an opportunity to comprehend previously unnoticed observations upon re-examining the same specimen. For example, electron microscopy studies have suggested that the components that distinguish between nodal regions and internodes play a pivotal role in the behavior of macrophages that initiate myelin phagocytosis in the demyelinating form of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP)...

BACKGROUND AND PURPOSE: We previously reported that nerve enlargement assessment by nerve ultrasonography of the intermediate upper limb is applicable for distinguishing demyelinating Charcot-Marie-Tooth disease (CMT) from chronic inflammatory demyelinating polyneuropathy (CIDP). However, differences in the severity and distribution patterns of lower extremity nerve enlargement have not been established for either disease. Therefore, we examined the utility of lower extremity nerve ultrasonography for differentiating between CMT and CIDP...

A 63-year-old man with type 2 diabetes mellitus, alcohol consumption in moderation, and three episodes of hepatic encephalopathy presented with symmetrical lower limb distal weakness, sensory ataxia, thickened palpable nerves, mood disturbances for seven years, and a family history of schizophreniform disorders. Nerve conduction studies showed demyelinating sensorimotor polyradiculoneuropathy. CSF analysis showed mild albumino-cytological dissociation. MRI brain and lumbosacral plexus showed thickened fifth cranial nerves and lumbosacral roots...

Metachromatic leukodystrophy (MLD) is a neuro-metabolic disorder due to arylsulfatase A deficiency, causing demyelination of the central and peripheral nervous system. Hematopoietic cell transplantation (HCT) can provide a symptomatic and survival benefit for pre-symptomatic and early symptomatic patients by stabilizing CNS disease. This case series, however, illustrates the occurrence of severely progressive polyneuropathy shortly after HCT in two patients with late-infantile, one with late-juvenile, and one with adult MLD, leading to the inability to walk or sit without support...

Guillain-Barré syndrome is a polyneuropathy that can be caused by an autoimmune condition or a bacterial infection. In typical GBS cases, there is hypo- or areflexia, symmetrical limb weakness that worsens within four weeks of the symptoms. The facial nerve is involved in this situation, which results in weak facial muscles, which, in turn, affect facial emotions and movements. In this case study, a 21-year-old athlete who suffered from unexpected weakness that resulted in quadriplegia had goal-oriented physical therapy treatment designed for the patient, who recovered quickly...

BACKGROUND AND PURPOSE: Antiganglioside antibodies (AGAs) might be involved in the etiopathogenesis of many neurological diseases, such as Miller-Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS). Available comprehensive reference data regarding AGA positivity rates and cross-responsiveness among AGAs (where one line immunoblot is positive for ≥1 AGA) during routine clinical care are scant. METHODS: In this 10-year monocentric retrospective study, 3560 immunoglobulin (Ig) G and IgM line blots (GA Generic Assays' Anti-Ganglioside Dot kit) obtained using cerebrospinal fluid (CSF) and serum samples from 1342 patients were analyzed for AGA positivity in terms of 14 diagnosis categories and AGA cross-responsiveness...

INTRODUCTION: Small cross-sectional studies and case reports observed improvement after administration of second IVIG dose (SID) amongst Guillain-Barré Syndrome (GBS) patients not responsive to initial IVIG cycle. Nevertheless, recent clinical trial and larger observational studies did not find any positive effects of SID. Instead, an increased risk of thromboembolism and mortality was noted. The conclusions of these studies however were not robust as confounding and selection bias were present...

RATIONALE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder that affects the peripheral nerves, leading to weakness and sensory symptoms. CIDP is a rare disease, and few studies have reported on anesthetic management in patients with this condition, especially the peripheral nerve block (PNB). Therefore, a safe and standardized anesthetic approach remains to be established. This case report aims to address this gap in evidence by documenting our experience with PNB in a patient with CIDP undergoing surgery...

Cytomegalovirus (CMV) belongs to the Herpesviridae family and is also known as human herpesvirus type 5. It is a common virus that usually doesn't cause any symptoms in healthy individuals. However, once infected, the virus remains in the host's body for life and can reactivate when the host's immune system weakens. This virus has been linked to several neurological disorders, including Alzheimer's disease, Parkinson's disease, Autism spectrum disorder, Huntington's disease (HD), ataxia, Bell's palsy (BP), and brain tumours, which can cause a wide range of symptoms and challenges for those affected...

Transcutaneous electrical nerve stimulation (TENS) has proven effective in treating pain in many experimental and clinical studies. In addition to the analgesic effect, direct TENS of peripheral nerves had anti-inflammatory and regenerative effects in the treatment of distal polyneuropathy and spinal cord injury. This work demonstrates the experience of using direct TENS in the treatment of a 52-year-old patient with post-COVID-19 Guillain-Barré (GBS) and acute transverse myelitis (ATM) overlap syndrome...

N-Hexane is a solvent widely used in manufacturing as a cleaner, degreaser and component of rubber cement. Chronic exposure to n -hexane either through contact with unprotected skin or inhalation can lead to the development of clinical symptoms and electrophysiological changes similar to those of inflammatory demyelinating polyneuropathy which requires careful differential diagnosis. This article presents three cases of severe predominantly motor polyneuropathy with demyelinating features in 15- and 16-year-old adolescents...

BACKGROUND: Autoimmune nodopathy (AN) has emerged as a novel diagnostic category that is pathologically different from classic chronic inflammatory demyelinating polyneuropathy. Clinical manifestations of AN include sensory or motor neuropathies, sensory ataxia, tremor, and cranial nerve involvement. AN with a serum-positive contactin-1 (CNTN1) antibody usually results in peripheral nerve demyelination. In this study, we reported a rare case of AN with CNTN1 antibodies characterized by the presence of CNTN1 antibodies in both serum and cerebrospinal fluid, which is associated with cerebellar dysarthria...

BACKGROUND AND PURPOSE: Peripheral neuropathy is a frequent complication of brentuximab vedotin (BV), used in CD30+ lymphoma treatment. Classic BV-induced neuropathy (BV-CN) is a mild distal sensory axonal polyneuropathy. Severe BV-induced inflammatory neuropathies (BV-IN) have been described. BV-IN contribute to lymphoma-associated morbidity but might be immunotherapy-responsive. Our primary objective was to evaluate the rate of BV-IN. Our secondary objectives were to determine risk factors and warning signs...

Neuromuscular ultrasound is a painless, radiation-free, high-resolution imaging technique for assessing the peripheral nervous system. It can accurately depict changes in the nerves and muscles of individuals with neuromuscular conditions, and it is therefore a robust diagnostic tool for the assessment of individuals with polyneuropathies. This review will outline the typical ultrasonographic changes found in a wide variety of polyneuropathies. In general, demyelinating conditions result in greater nerve enlargement than axonal conditions, and acquired conditions result in more patchy nerve enlargement compared to diffuse nerve enlargement in hereditary conditions...

Paraneoplastic neurologic syndromes are rarely associated with hematologic malignancies. In their rarity, lymphomas are the diseases with more frequent paraneoplastic neurologic syndrome. High-risk antibodies are absent in most lymphoma-associated paraneoplastic neurologic syndromes, with the exception of antibodies to Tr/DNER in paraneoplastic cerebellar degeneration, mGluR5 in limbic encephalitis, and mGluR1 in some cerebellar ataxias. Peripheral nervous system paraneoplastic neurologic syndromes are rare and heterogeneous, with a prevalence of demyelinating polyradiculoneuropathy in non-Hodgkin lymphoma...

INTRODUCTION: Few studies have pointed to the possible role of infectious diseases in triggering Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP). Given the association of Hepatitis E Virus (HEV) with Guillain Barrè syndrome, we conducted a case-control study to determine the possible association of HEV infection with CIDP, analyzing possible risk factors for acquiring HEV infection in both CIDP patients and controls. MATERIALS AND METHODS: 82 CIDP and 260 from the general population have provided some personal information (demographics, anamnestic data and recognized risk factors for HEV infection) and underwent venipuncture blood sampling for virological assays testing for anti-HEV IgG and IgM with ELISA and RNA-HEV performing RT-PCR...