Multifocal motor neuropathy

Combined central and peripheral demyelination (CCPD) is a rare autoimmune neurologic disease, characterized by immune-mediated damage of myelin sheath at central and peripheral levels of the nervous system. The current knowledge about this disorder is only limited, mainly due to the low incidence of the disease. According to previous studies, CCPD has a very heterogeneous course, insufficient therapeutic response, and an unfavorable prognosis. We report on the 37-year-old patient with a coincidence of demyelinating lesions in the brain fulfilling current McDonald's diagnostic criteria for multiple sclerosis, as well as the presence of an atypical variant of chronic inflammatory demyelinating polyneuropathy (CIDP) - multifocal acquired demyelinating sensory-motor neuropathy (MADSAM), as a subtype of combined central and peripheral demyelination (CCPD)...

Neurological involvement in sarcoidosis is termed as neurosarcoidosis. It usually leads to cranial neuropathies, although it can involve any part of the neuroaxis. Although sarcoidosis is a proinflammatory state, there is an associated anergic state demonstrable by a feeble tuberculin response. Lymphocytic sequestration in granulomas can be associated with peripheral CD4 lymphocytopenia (40% of patients with sarcoidosis) predisposing to opportunistic infections. Here we have described a young, otherwise immunocompetent male presenting with subacute onset right hemiparesis with motor aphasia, who was diagnosed to have progressive multifocal leukoencephalopathy (PML) secondary to pulmonary sarcoidosis...

PURPOSE: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) developing in the postvaccination period was distinctly unusual and its course was rarely well described. We aimed to clearly depict the clinical features of acute-onset multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) caused by mRNA-1273 COVID-19 vaccination. CASE REPORT: A 74-year-old man noticed weakness of hands 2-3 days after he accepted the second dose of mRNA-1273 COVID-19 vaccine...

The perineurium surrounds each fascicle in peripheral nerves, forming part of the blood-nerve barrier. We describe its normal anatomy and function. "Perineuritis" refers to both a nonspecific histopathological finding and more specific clinicopathological entity, primary perineuritis (PP). Patients with PP are often assumed to have nonsystemic vasculitic neuropathy until nerve biopsy is performed. We systematically reviewed the literature on PP and developed a differential diagnosis for histopathologically defined perineuritis...

Autoantibodies against contactin-associated protein 2 (CASPR2) are usually associated with autoimmune encephalitis and neuromyotonia. Their association with inflammatory neuropathies has been described in case reports albeit all with distal symmetric manifestation. Here, we report a patient who developed distal arm paresis, dominantly of the right arm, over the course of 1 year. Electroneurography showed a conduction block of motor nerve conduction, nerve ultrasonography a swelling of the right median and ulnar nerve and flow cytometry an increase in natural killer (NK cells) in the blood and natural killer T (NKT) cells in the cerebrospinal fluid (CSF), therefore indicating a multifocal motor neuropathy-like (MMN-like) phenotype...

The autoimmune peripheral neuropathies with prominent motor manifestations are a diverse collection of unusual peripheral neuropathies that are appreciated in vast clinical settings. This chapter highlights the most common immune-mediated, motor predominant neuropathies excluding acute, and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP, respectively). Other acquired demyelinating neuropathies such as distal CIDP and multifocal motor neuropathy will be covered. Additionally, the radiculoplexus neuropathies, resulting from microvasculitis-induced injury to nerve roots, plexuses, and nerves, including diabetic and nondiabetic lumbosacral radiculoplexus neuropathy and neuralgic amyotrophy (i...

BACKGROUND AND AIMS: Multifocal motor neuropathy (MMN) is a peripheral nerve disorder characterized by slow progressive distal asymmetric weakness with minimal or no sensory impairment. Currently, a vast evidence supports a direct pathogenic role of IgM anti-GM1 antibodies on disease pathogenesis. Patients with MMN seropositive for GM1-specific IgM antibodies have significantly more weakness, disability and axon loss than patients without these antibodies. During the screening for IgM anti-GM1 antibodies in a cohort of patients with neuropathy we noticed an absence or significant reduction of natural IgM anti-GM1 autoreactivity in some patients with MMN, suggesting a mechanism of self-control of autoreactivity...

Intravenous immune globulin (IVIG) is an immune-modulating biologic therapy that is increasingly being used in neuromuscular disorders despite the paucity of high-quality evidence for various specific diseases. To address this, the AANEM created the 2009 consensus statement to provide guidance on the use of IVIG in neuromuscular disorders. Since then, there have been several randomized controlled trials for IVIG, a new FDA-approved indication for dermatomyositis and a revised classification system for myositis, prompting the AANEM to convene an ad hoc panel to update the existing guidelines...

Autoantibodies against nodal and paranodal proteins, such as neurofascin 140/186, neurofascin 155, contactin 1, and contactin-associated protein 1, have been identified in subsets of patients with chronic inflammatory demyelinating polyneuropathy. Their distinctive characteristics including poor response to immunoglobulin led to the establishment of a new disease entity called "autoimmune nodopathies." IgM monoclonal antibodies against myelin-associated glycoproteins cause intractable sensory-dominant demyelinating polyneuropathy...

OBJECTIVE: Antibody- and complement-mediated peripheral nerve inflammation are central in the pathogenesis of MMN. Here, we studied innate immune responses to endotoxin in patients with MMN and controls to further our understanding of MMN risk factors and disease modifiers. METHODS: We stimulated whole blood of 52 patients with MMN and 24 controls with endotoxin and collected plasma. With a multiplex assay, we determined levels of the immunoregulating proteins IL-1RA, IL-1β, IL-6, IL-10, IL-21, TNF-α, IL-8 and CD40L in unstimulated and LPS-stimulated plasma...

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No abstract text is available yet for this article.

No abstract text is available yet for this article.

OBJECTIVE: A growing body of literature recognises the importance of peripheral nerve ultrasound in neuromuscular disorders. Several attempts have been made to differentiate amyotrophic lateral sclerosis (ALS) from multifocal motor neuropathy (MMN) using peripheral nerve ultrasound. A much-debated question is whether the cross-sectional area (CSA) of peripheral nerve in ALS patients is significantly smaller compared to healthy controls. This study aims to determine the CSA of peripheral nerves in patients with ALS...

The spectrum of immune-mediated neuropathies is broad and the different subtypes are still being researched. With the numerous subtypes of immune-mediated neuropathies, establishing the appropriate diagnosis in normal clinical practice is challenging. The treatment of these disorders is also troublesome. The authors have undertaken a literature review of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Guillain-Barre syndrome (GBS) and multifocal motor neuropathy (MMN). The molecular, electrophysiological and ultrasound features of these autoimmune polyneuropathies are analyzed, highlighting the differences in diagnosis and ultimately treatment...

RATIONALE: Ischemic monomelic neuropathy (IMN) is a disease that occurs after acute arterial occlusion or steal phenomenon in an extremity that results in single or multiple axonal mononeuropathies in the distal limb without the classical features of limb ischemia, including a skin color change, limb swelling, and ischemic claudication. IMN can easily be misdiagnosed as any other neuropathic disorder. Here we present a case of IMN that was misdiagnosed as spinal epidural hematoma. PATIENT CONCERNS: A 77-year-old man presented with sudden motor weakness and pain in his left foot and calf 5 days after a bilateral L4 to 5 posterior decompression for lumbar spinal stenosis...

Dysfunction of the immune system can result in damage of the peripheral nervous system. The immunological mechanisms, which include macrophage infiltration, inflammation and proliferation of Schwann cells, result in variable degrees of demyelination and axonal degeneration. Aetiology is diverse and, in some cases, may be precipitated by infection. Various animal models have contributed and helped to elucidate the pathophysiological mechanisms in acute and chronic inflammatory polyradiculoneuropathies (Guillain-Barre Syndrome and chronic inflammatory demyelinating polyradiculoneuropathy, respectively)...

BACKGROUND: The aim of this study was to assess the phenotype of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) in quantitative MR neurography. METHODS: In this prospective study, 22 patients with ALS, 8 patients with MMN, and 10 healthy volunteers were examined with 3T MR neurography, using a high-resolution fat-saturated T2-weighted sequence, diffusion-tensor imaging (DTI), and a multi-echo T2-relaxometry sequence. The quantitative biomarkers fractional anisotropy (FA), radial and axial diffusivity (RD, AD), mean diffusivity (MD), cross-sectional area (CSA), T2-relaxation time, and proton spin density (PSD) were measured in the tibial nerve at the thigh and calf, and in the median, radial, and ulnar nerves at the mid-upper arm...

Primary lymphomas of peripheral nerves (PLPNs) are extremely rare and most commonly reported in lumbar nerves and have been found in only five cases in the upper extremities. We describe two patterns of presentation focusing on clinical, radiological, and pathological findings of two patients affected by primary multifocal lymphoma of the ulnar nerve without systemic involvement or other medical conditions. We report a case of extraneural lymphoma in a 72-years-old (patient #1) and a case of intraneural lymphoma in a 45-years old woman (Patient #2)...

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) are inflammatory neuropathies that can lead to considerable limitations in daily activities and in social participation. However, systematic evaluation of these self-reported limitations is lacking in the currently available studies. Understanding the impact of these diagnoses on patients' life is important to optimize management strategies. AIM: To systematically assess the self-reported limitations in activities and participation and determine associated factors...