Differences in clinical features between small fiber and sensitive large fiber neuropathies in Sj ögren's syndrome.

CONCLUSIONS: An association of non-length-dependent pattern and dysautonomia seems to predict the absence of LFN in SS and encourages the search for SFN. In contrary, patients with length-dependent involvement and without dysautonomia should be prioritized for EMG. PMID: 32471733 [PubMed - as supplied by publisher]
Source: European Journal of Internal Medicine - Category: Internal Medicine Authors: Tags: Eur J Intern Med Source Type: research

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We read with interest the study “Retinal and corneal neurodegeneration and its association to systemic signs of peripheral neuropathy in type 2 diabetes” by Hafner and associates.1 The authors have evaluated macular and peripapillary retinal nerve fiber layer in patients with various grades of diabetic retinopathy (DR) using o ptical coherence tomography (OCT), and have correlated these changes to corneal nerve length/density measured with confocal microscopy, clinical diabetic peripheral neuropathy (DPN) scores, and intraepidermal nerve fiber density (IENFD) measured with skin punch biopsy scores from the leg.
Source: American Journal of Ophthalmology - Category: Opthalmology Authors: Tags: Correspondence Source Type: research
Janszky J, Komoly S Abstract Small fiber neuropathy develops due to the selective damage of the thin fibers of peripheral nerves. Many common diseases can cause this condition, including diabetes, infections, autoimmune and endocrine disorders, but it can occur due to genetic alterations, as well. Eighty-five skin biopsy-proven small-fiber neuropathy cases were analyzed. Forty-one (48%) cases were idiopathic; among secondary types, hypothyreosis (9.4%), diabetes mellitus (7%), cryoglobulinemia (7%), monoclonal gammopathy with unproved significance (4.7%), Sjögren's disease (3%), and paraneoplastic neuropathy ...
Source: Behavioural Neurology - Category: Neurology Authors: Tags: Behav Neurol Source Type: research
Conclusions: The results expand the molecular spectrum of GSN-linked systemic amyloidosis. The novel p.Met544Arg pathogenic variant is predicted to affect gelsolin function, presumably by impairing a potential calcium-sensitive, actin-binding region. PMID: 32368002 [PubMed - in process]
Source: Molecular Vision - Category: Molecular Biology Tags: Mol Vis Source Type: research
Two ethnic Chinese men with clinico-radiologic features of Fragile X-associated tremor-ataxia syndrome (FXTAS) were found on genetic testing to have neuronal intranuclear inclusion disease (NIID), highlighting that NIID should be considered in the differential diagnosis of FXTAS. NIID may also be much more common than FXTAS in certain Asian populations.Neuronal intranuclear inclusion disease (NIID) was considered a rare juvenile-onset disease; however, the characterization of skin biopsy and neuroimaging abnormalities led to many more cases being diagnosed, including patients with adult-onset movement disorders (cerebellar...
Source: Parkinsonism and Related Disorders - Category: Neurology Authors: Tags: Correspondence Source Type: research
Abstract Consistent associations between the severity of neuropathic pain and cutaneous innervation have not been described. We collected demographic and clinical data, McGill Pain Questionnaires (MPQ) and skin biopsies processed for PGP9.5 and CGRP immunohistochemistry from patients with bortezomib-induced peripheral neuropathy (BiPN; n=22), painful diabetic neuropathy (PDN; n=16), chronic idiopathic axonal polyneuropathy (CIAP; n=16) and 17 age-matched healthy volunteers. Duration of neuropathic symptoms was significantly shorter in patients with BiPN in comparison with PDN and CIAP patients. BiPN was characteri...
Source: Brain Research - Category: Neurology Authors: Tags: Brain Res Source Type: research
ConclusionsPolyneuropathy in polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome can present as a pseudosensory level.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Conclusions Our findings confirm previous data on the tight clinico-serological correlation between antibodies to nodal/paranodal proteins and CIDP. Despite the low prevalence, testing for their presence and isotype could ultimately be part of the diagnostic workup in suspected inflammatory demyelinating neuropathy to improve diagnostic accuracy and guide treatment. Classification of evidence This study provides Class III evidence that antibodies to nodal/paranodal proteins identify patients with CIDP (sensitivity 6%, specificity 100%).
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, All Neuromuscular Disease, Peripheral neuropathy, Chronic inflammatory demyelinating polyneuropathy Article Source Type: research
This article presents a stepwise approach to the evaluation and workup of peripheral neuropathy, which starts with a detailed history of symptoms, family and occupational history, and a neurological as well as general physical exam. Pattern recognition of various neuropathies can help to build a differential diagnosis based on the presentation. Such patterns include acute versus chronic, primary demyelinating versus axonal, hereditary versus acquired, asymmetric versus symmetric, presence of facial palsies, sensory or motor predominant, and presence of prominent autonomic symptoms. Early categorization of the type of neuro...
Source: Seminars in Neurology - Category: Neurology Authors: Tags: Review Article Source Type: research
CONCLUSIONS: We report two novel ATP13A2 pathogenic mutations, further expanding the phenotype of Kufor-Rakeb syndrome with the unusual features of ataxia and polyneuropathy. We thoroughly describe ultrastructural findings and document a meaningful response to levodopa. PMID: 31588715 [PubMed - in process]
Source: Folia Neuropathologica - Category: Neurology Tags: Folia Neuropathol Source Type: research
Objective Reported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN. Methods Data of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Associations with other o...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Vasculitis, All Medical/Systemic disease, Peripheral neuropathy, Prevalence studies Article Source Type: research
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