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Idiopathic peripheral neuropathy

Kristin Samuelsson, Ayman A M Osman, Maria Angeria, Mårten Risling, Simin Mohseni, Rayomand Press
Twenty-five percent of polyneuropathies are idiopathic. Microangiopathy has been suggested to be a possible pathogenic cause of chronic idiopathic axonal polyneuropathy (CIAP). Dysfunction of the autophagy pathway has been implicated as a marker of neurodegeneration in the central nervous system, but the autophagy process is not explored in the peripheral nervous system. In the current study, we examined the presence of microangiopathy and autophagy-related structures in sural nerve biopsies of 10 patients with CIAP, 11 controls with inflammatory neuropathy and 10 controls without sensory polyneuropathy...
2016: PloS One
Nathaniel M Robbins, Vinil Shah, Nancy Benedetti, Jason F Talbott, Cynthia T Chin, Vanja C Douglas
Magnetic resonance neurography (MRN) is an important tool to detect abnormalities of peripheral nerves. This pictorial review demonstrates the MRN features of a variety of neuropathies affecting the lumbosacral plexus (LSP) and lower extremity nerves, drawn from over 1200 MRNs from our institution and supplemented by the literature. Abnormalities can be due to spinal compression, extraspinal compression, malignancy, musculoskeletal disease, iatrogenesis, inflammation, infection, and idiopathic disorders. We discuss indications and limitations of MRN in diagnosing LSP neuropathies...
July 11, 2016: Clinical Imaging
Bianca T A de Greef, Margot Geerts, Janneke G J Hoeijmakers, Catharina G Faber, Ingemar S J Merkies
BACKGROUND: Small fiber neuropathy is the most common cause of neuropathic pain in peripheral neuropathies, with a minimum prevalence of 53/100,000. Patients experience excruciating pain, and currently available anti-neuropathic and other pain drugs do not relieve the pain substantially. Several open-label studies have suggested an immunological basis in small fiber neuropathy and have reported efficacy of treatment with intravenous immunoglobulin. Therefore, immunological mechanisms conceivably may play a role in small fiber neuropathy...
2016: Trials
Valeria Katchan, Paula David, Yehuda Shoenfeld
Concurrent association of idiopathic thrombocytopenic purpura (ITP) and peripheral neuropathy is a rare condition. There are only few case reports published concerning peripheral neuropathy with ITP. One of the etiopathogenetic mechanisms proposed is intraneural hemorrhage, but the pathogenesis is not fully understood. Autoimmune nature with common antibodies to the platelets and the nerve´s myelin sheath should also be considered. Here we describe a 47-year-old woman, with a family history of autoimmune diseases...
July 18, 2016: Immunologic Research
E Hachulla, O Benveniste, M Hamidou, L Mouthon, N Schleinitz, P Lozeron, J M Léger, C Vial, K Viala
BACKGROUND: In patients with autoimmune diseases who still derive benefit from high dose intravenous immunoglobulin (IVIg) treatment, some physicians resort to subcutaneous (SC) Ig as a replacement therapy. OBJECTIVE: To collect quality of life (QoL) and tolerance data on SCIg in patients for whom the switch from IVIg to SCIg is essential to maintain treatment. METHODS: This observational study included patients with either idiopathic inflammatory myopathies (IIM) or chronic dysimmune peripheral neuropathies (CDPN) treated with IVIg, who had been switched to SCIg administration for at least three months...
July 14, 2016: International Journal of Neuroscience
A M Mahon
This report documents an unusual case of distal symmetrical peripheral neuropathy (DSPN) in an otherwise healthy patient without diabetes mellitus (DM) presenting to a podiatric wound care clinic. The development of gas gangrene coupled with Charcot neuroarthropathic changes ultimately resulted in a potentially life-saving transmetatarsal (TMT) amputation. Causation of, or at least a contributor to, the DSPN was likely phenytoin usage for epileptic seizures. Long-term use of phenytoin can lead to axonal shrinkage and random clusters of nerve demyelination [1]...
June 24, 2016: Journal of Tissue Viability
Hisao Shimizu, Koji Haratani, Masayuki Miyazaki, Yoshiaki Kakehi, Shuhei Nagami, Yuichi Katanami, Hiroki Kawabata, Nobuyuki Takahashi
A 38-year-old man visited our hospital because of hemifacial paresis that developed 2 months after being bit by a tick. We diagnosed idiopathic peripheral facial palsy and gave the patient oral prednisolone and valacyclovir. Although the symptoms completely resolved in about 2 weeks, there was a risk of Lyme neuroborreliosis. The patient therefore received doxycycline (100 mg twice daily) and amoxicillin (1,000 mg 3 times daily) for 14 days. Two months later, he had symptoms of meningitis such as headache and fever accompanied by lymphocytic cerebrospinal fluid pleocytosis...
July 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
Wiebke Schrempf, Istvan Katona, Imis Dogan, Verena V Felbert, Miriam Wienecke, Julia Heller, Andrea Maier, Andreas Hermann, Katharina Linse, Moritz D Brandt, Heinz Reichmann, Jörg B Schulz, Johannes Schiefer, Wolfgang H Oertel, Alexander Storch, Joachim Weis, Kathrin Reetz
BACKGROUND: Idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD) has been increasingly acknowledged to be an initial specific manifestation of alpha-synucleinopathies such as Parkinson's disease (PD), multiple system atrophy (MSA) and dementia with Lewy bodies (DLB). Recent findings suggest that cutaneous abnormalities like small fiber neuropathy and alpha-synuclein deposition might reflect brain pathology and might function as early biomarkers in PD. This is the first study to elucidate whether iRBD patients already suffer from distinctive cutaneous features...
August 2016: Parkinsonism & related Disorders
Bulent Yardimci, Rumeyza Kazancioglu
INTRODUCTION: Idiopathic systemic capillary leak syndrome (ISCLS) is rarely seen, and presents with recurrent episodes of hypotension, shock, hemoconcentration, and hypoproteinemia. The main pathology is the dysfunction of the vascular endothelium, and it is characterized by an increase of capillary permeability that is accompanied by the loss of intravascular fluid and protein. CASE PRESENTATION: We present a 58-year-old female who presented with peripheral edema, leg pain, and syncope at the emergency department...
February 2016: Iranian Red Crescent Medical Journal
A Jeandel, J L Thibaud, S Blot
OBJECTIVES: The aim of this study was to describe the signalment, clinical presentation, diagnostic findings and long-term follow-up in dogs with concomitant facial and vestibular neuropathy of unknown origin. METHODS: Appropriate cases were located through medical record searches. Inclusion criteria comprised dogs that had: clinical signs of facial paralysis with concomitant peripheral vestibular syndrome, thyroid function tests, no abnormalities on magnetic resonance imaging of the brain and tympanic bullae, and cerebrospinal fluid analysis...
February 2016: Journal of Small Animal Practice
Romana Vieira, José Miguel Bernardes, José Araújo Pinto, Lúcia Costa
We present a rare association of idiopathic livedoid vasculopathy (LV) with peripheral sensory neuropathy successfully treated with intravenous immunoglobulin and warfarin and, after its failure, with rituximab. Although LV aetiology remains incompletely understood, the excellent clinical response observed in our patient suggests that B cells may be key players in its pathogenesis.
January 7, 2016: Acta Reumatológica Portuguesa
Mohammad A Khoshnoodi, Shaun Truelove, Ahmet Burakgazi, Ahmet Hoke, Andrew L Mammen, Michael Polydefkis
IMPORTANCE: Few data are available on the natural history of small fiber neuropathy (SNF). Peripheral neuropathy typically follows a length-dependent pattern, leading us to hypothesize that patients with SFN would lose intraepidermal nerve fibers at the distal leg more quickly than at more proximal thigh sites. OBJECTIVE: To compare the longitudinal rate and pattern of intraepidermal nerve fiber density (IENFD) change in idiopathic SFN (iSFN), impaired glucose tolerance-associated SFN (IGT-SFN), and diabetes mellitus-associated SFN (DM-SFN)...
June 1, 2016: JAMA Neurology
Hafiz Abdul Moiz Fakih, Emmanuel Elueze, Rajiv Vij
BACKGROUND: Cryoglobulinemia is a cold-reactive autoimmune disease. It is of distinctive importance in cardiac surgery because of the use of hypothermic cardiopulmonary bypass (CPB). Cryoglobulins, which activate at variable levels of hypothermia, can cause precipitation during surgery leading to possibly severe leukocytoclastic or necrotizing vasculitis, clinically manifested as ischemic events, such as cutaneous ulcerations, glomerulonephritis, arthritis, or peripheral neuropathies among the most reported associated comorbidities...
2016: Journal of Community Hospital Internal Medicine Perspectives
Brian Cox, John R Zuniga, Neeraj Panchal, Jonathan Cheng, Avneesh Chhabra
OBJECTIVE: This tertiary care experience examines the utility of magnetic resonance neurography (MRN) in the management of peripheral trigeminal neuropathies. MATERIALS AND METHODS: Seventeen patients with clinically suspected peripheral trigeminal neuropathies (inferior alveolar nerve and lingual nerve) were imaged uniformly with 1.5-T examinations. MRN results were correlated with clinical and surgical findings in operated patients and the impact on clinical management was assessed...
October 2016: European Radiology
Rens Hanewinckel, Marieke van Oijen, M Arfan Ikram, Pieter A van Doorn
Polyneuropathy is a disabling condition of the peripheral nerves, characterized by symmetrical distal numbness and paresthesia, often accompanied with pain and weakness. Although the disease is often encountered in neurological clinics and is well known by physicians, incidence and prevalence rates are not well known. We searched EMBASE, Medline, Web-of-science, Cochrane, PubMed Publisher, and Google Scholar, for population-based studies investigating the prevalence of polyneuropathy and its risk factors. Out of 5119 papers, we identified 29 eligible studies, consisting of 11 door-to-door survey studies, 7 case-control studies and 11 cohort/database studies...
January 2016: European Journal of Epidemiology
Ronan Niall McGinty, Brian McNamara, Helena Moore
A 52-year-old man with idiopathic Parkinson's disease and severe rheumatoid arthritis presented with a 1-year history of progressively worsening limb paraesthesia. Examination showed sensory loss in a glove and stocking distribution, absent reflexes and unsteady tandem gait. Nerve conduction studies suggested an acquired peripheral neuropathy with distal demyelination, which-together with the clinical phenotype-was consistent with a Distal Acquired Demyelinating Symmetric (DADS) neuropathy pattern. This was attributed to therapy with adalimumab, an antitumor necrosis factor (TNF)-α agent, which the patient had been taking for 2 years for rheumatoid arthritis...
2015: BMJ Case Reports
Anne Louise Oaklander
The best-known peripheral neuropathies are those affecting the large, myelinated motor and sensory fibers. These have well-established immunological causes and therapies. Far less is known about the somatic and autonomic "small fibers"; the unmyelinated C-fibers, thinly myelinated A-deltas, and postganglionic sympathetics. The small fibers sense pain and itch, innervate internal organs and tissues, and modulate the inflammatory and immune responses. Symptoms of small-fiber neuropathy include chronic pain and itch, sensory impairment, edema, and skin color, temperature, and sweating changes...
January 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Paz Zuberbuhler, Pablo Young, Luciana V León Cejas, Bárbara C Finn, Julio E Bruetman, Cristian R Calandra, Ernesto Fulgenzi, Manuel Pérez Akly, Alejandro Rodríguez, Ana Pardal, Ricardo Reisin
Sensory neuronopathies or ganglionopathies, or dorsal root ganglion disorders, represent a subgroup of peripheral nervous system diseases, frequently associated with dysinmune or neoplastic disorders and with toxic agents. A degeneration of both central and peripheral sensory proyections is present. Patients typically show early ataxia, loss of deep tendon reflexes and positive sensory symptoms present both in proximal and distal sites of the body. We retrospectively studied 10 cases with a final diagnosis of sensory neuronopathy...
2015: Medicina
Karine Viala
BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare disease and the diagnosis is complicated by heterogeneity of the variant forms. Underdiagnosis is undesirable as effective treatments exist. Conversely, overdiagnosis can lead to inappropriate and expensive treatment and delay the initiation of appropriate treatment. SUMMARY: The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria are used widely in clinical trials and clinical practice...
September 2016: International Journal of Neuroscience
Katharina A Schindlbeck, Arne Mehl, Sarah Geffe, Steffen Benik, Serdar Tütüncü, Fabian Klostermann, Frank Marzinzik
Parkinson's disease (PD) is a neurodegenerative condition presenting with motor and non-motor symptoms including somatosensory disturbances. As neuropathic syndromes in advanced PD patients are supposed to be due to antiparkinsonian medication, we studied the presence of somatosensory symptoms and peripheral nerve function in drug naïve patients with PD as well as age-matched healthy controls. Somatosensory symptoms and signs were investigated in 39 de novo PD patients and 32 age-matched healthy controls using the modified Toronto Clinical Neuropathy Scale...
March 2016: Journal of Neural Transmission
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