1,721,163 research outputs found

    Anti-ganglioside complex IgM antibodies in multifocal motor neuropathy and chronic immune-mediated neuropathies.

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    Anti-ganglioside complexes (GSCs) IgG antibodies have been reported in patients with Guillain-Barré (GBS) or Fisher syndrome but little is known on their presence in multifocal motor neuropathy (MMN) or other chronic immune-mediated neuropathies. We examined 24 patients with MMN, 34 with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 23 with neuropathy associated with IgM monoclonal gammopathy (PN+IgM), 13 with GBS, 34 with motor neuron disease (MND), 24 with other neuropathies and 20 normal subjects. Patients' sera were tested by ELISA for IgM reactivity to GM1, GM2, GD1a, GD1b and GT1b and with GSCs made by any combination of two of these gangliosides. In all GM1 positive patients with MMN (11), PN+IgM (1), CIDP (1) and POEMS (1), binding to GM1 was abolished or consistently reduced when tested in GSCs also containing GD1a or other gangliosides. This only occurred in one of the three GM1 positive MND patients. In a patient with PN-IgM and anti-GM2 and GD1a IgM, both reactivities were reduced when tested in GSCs also containing GM1. New reactivities were found in a patient with CIDP and anti-GD1b IgM who presented an additional reactivity to GT1b/GM1 and GT1b/GM2 GSCs, and in one with PN-IgM who had reactivity to GM2/GD1b but not to individual gangliosides. Testing for IgM antibodies to GSCs rarely permitted to identify new reactivities in chronic immune neuropathies. IgM binding to gangliosides was however often modified in GSCs suggesting that these reactivities may be affected by contiguous gangliosides possibly influencing their pathogenicity

    Acute and chronic inflammatory neuropathies and COVID-19 vaccines: Practical recommendations from the task force of the Italian Peripheral Nervous System Association (ASNP)

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    Background and aims: To develop recommendations for vaccination for coronavirus-19 (COVID-19) in patients with inflammatory neuropathies. Methods: Key questions were formulated in order to perform a literature review on the safety and efficacy of vaccines in patients with inflammatory neuropathies. Based on the best evidence and expert opinion, a list of recommendations was formulated to inform decision on vaccination for COVID-19 in patients with inflammatory neuropathies and increase adherence to vaccination programmes. Results: Recommendations addressing safety and efficacy of vaccination in patients with inflammatory neuropathies were formulated. No data are currently available on the safety and efficacy of COVID-19 vaccines in patients with inflammatory neuropathies or other immune-mediated conditions. There is only sparse data on the safety of previous available vaccines in patients with inflammatory neuropathies, but studies on other autoimmune disorders indicate that these are safe and mostly efficacious. Patients with inflammatory neuropathies might be at increased risk for severe illness from COVID-19. Interpretation: Patients with inflammatory neuropathies should be encouraged to adhere to the vaccination campaign for COVID-19. These recommendations provide guidance on the management of vaccinations for COVID-19 in patients with inflammatory neuropathies. More research is needed regarding the safety and efficacy of vaccination in patients with inflammatory neuropathies and other immune conditions

    PROMM in Italy: Clinical and biomolecular findings

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    Proximal myotonic myopathy PROMM is a multisystem disorder, only similar to myotonic dystrophy. The genetic lesion is unknown but clinical and genetic heterogeneity exist. We describe 50 patients from 10 unrelated families in Italy to better define the clinical spectrum of the syndrome. The clinical and biomolecular findings of these patients fits the core features delineated recently at ENMC International Workshop

    Chronic immune sensory polyradiculopathy (CISP): First juvenile case description

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    In its typical presentation, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) occurs more often in old males as a progressive/recurrent motor and sensory nerve dysfunction with tendon areflexia. However, CIDP has also atypical clinical presentations, including pure sensory neuropathies, among which chronic immune sensory polyradiculopathy (CISP) accounts for only 0.5% of all CIDP, with no juvenile cases reported as yet. A 17-year-old girl presented for a progressive sensory ataxia and hands clumsiness. Diffuse tendon areflexia and hypokinaesthesia were observed. Motor and sensory nerve conduction studies were normal. F-waves were normal in median nerves and elongated in tibial nerves. H-reflex and somatosensory evoked potentials (SSEP) were absent. CSF normal cellularity with hyperproteinorrachia was found. Paraneoplastic, metabolic, and paraproteinemic neuropathies were excluded. A diagnosis of CISP has been made based on the presence of pure sensory symptoms in a polyneuropathic distribution, normal peripheral nerve conduction studies, and two supportive criteria (SSEP and CSF). Our paper describes the first CISP case in the pediatric age. We confirm SSEP and CSF as useful complementary tests for this diagnosis also at this age and suggest that clinicians should consider CISP in the spectrum of sporadic sensory ataxias of the pediatric age. We also suggest that in the presence of normal F-wave and peripheral motor nerve conduction, an absent H-reflex can further substantiate SSEPs in the diagnosis of CISP. Intravenous immunoglobulins were rapidly effective and safe

    Low levels of LTB4 in cerebrospinal fluid of AIDS patients with cryptococcal meningitis

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    In this study we evaluated the release of leukotriene B4 (LTB4) in the cerebrospinal fluid (CSF) of 12 patients with Acquired Immunodeficiency Syndrome (AIDS), which disease was complicated by Cryptococcal Meninigitis and in CSF of 12 control subjects with inflammatory and degenerative pathologies of the Central Nervous System (CNS). We obtained low levels of LTB4 in all the AIDS patients (mean 60.5 pg/ml), while the HIV negative subjects with degenerative and inflammatory pathologies of CNS showed a mean of 91.5 pg/ml. The finding of low levels of this inflammatory reaction mediator agrees with the limited clinical symptoms of cryptococcal meningoencephalitis in patients affected by AIDS
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