Non-length dependent small fibre neuropathy/ganglionopathy

Gorson, Kenneth C.; Herrmann, David N.; Thiagarajan, Ramu; Brannagan, Thomas H.; Chin, Russell L.; Kinsella, Laurence J.; Ropper, Allan H.

doi:10.1136/jnnp.2007.128801

Cited by 148 publications

(161 citation statements)

References 33 publications

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“…In general, the symptoms of SFN include sensory disorders resulting from Aδ fiber damage (e.g., pain and feelings of numbness) as well as autonomic nervous disorders induced by C fiber damage (e.g., digestive symptoms, orthostatic ataxia, dysuria and hot flashes). The distribution of neurological symptoms has been reported to be non-length-dependent in cases of sarcoidosis-induced SFN (12), while generalized SFN sensory pain may present with a "stocking and glove" distribution depending on the nerve length (length-dependent SFN) or be distributed around the face, torso or ganglia (non-length-dependent SFN) (13,14). Lacomis et al (6) proposed the following criteria for diagnosing SFN: (1) symptoms of peripheral dysesthesia (typically pain), (2) an electrophysiologically normal nerve conduction velocity and needle electromyography results and (3) pathological findings (reduced intraepidermal nerve fiber density).…”

Section: Discussionmentioning

confidence: 99%

Neurological Symptoms of Sarcoidosis-induced Small Fiber Neuropathy Effectively Relieved with High-dose Steroid Pulse Therapy

et al. 2015

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A 59-year-old woman was admitted to our hospital for an evaluation of a 10-day history of progressive pain and hypoesthesia of the right lower back associated with fever and constipation. Sarcoidosis was confirmed on mediastinal lymph node and skin biopsies. Although the neurological symptoms were suspected due to sarcoidosis-induced nerve dysfunction, nerve conduction studies and other routine examinations did not show any abnormalities. The intraepidermal nerve fiber density assessed on a skin biopsy was significantly reduced, suggesting small-fiber neuropathy (SFN). The patient was finally diagnosed with sarcoidosisinduced SFN, and her neurological symptoms were effectively relieved with high-dose steroid therapy.

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Section: Discussionmentioning

confidence: 99%

Neurological Symptoms of Sarcoidosis-induced Small Fiber Neuropathy Effectively Relieved with High-dose Steroid Pulse Therapy

et al. 2015

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“…The characteristic temporal (acute/fulminant versus chronic) and spatial (distal versus patchy/proximal) patterns characteristic of polyneuropathies were all identified. 6 With ubiquitous dysautonomic symptoms, signs, and test abnormalities, juvenile SFPN integrates autonomic as well as neuropathic pain components. Unexpectedly, 63% of patients suffered chronic headaches.…”

Section: Discussionmentioning

confidence: 99%

“…Occasional patients develop patchy or predominantly proximal symptoms attributed to proximal attack on the neuronal cell bodies (ganglionopathy/neuronopathy; Fig 2A). 6 Because small fibers also densely innervate and regulate the tone of microvessels, abnormal appearance (eg, edema, color changes) is common in affected areas. 7 Smallfibers were formerly dichotomized as somatic versus autonomic, a distinction blurred by recent discoveries that somatic/nociceptive axons have efferent and trophic effects formerly considered autonomic, 8 and that axons innervating sweat glands and blood vessels express the TRPV 1 /capsaicin pain receptor.…”

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confidence: 99%

Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes

2013

PEDIATRICS

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WHAT'S KNOWN ON THIS SUBJECT: Acquired widespread pain syndromes of youth are prevalent, disabling, usually unexplained, and untreatable. Small-fiber polyneuropathy causes widespread pain and multisystem complaints in older adults. Some causes are treatable. Neurodiagnostic skin biopsy, autonomic function testing, and nerve biopsy permit objective diagnosis.WHAT THIS STUDY ADDS: It identifies definite (in 59%) and probable (in 17%) small-fiber polyneuropathy among 41 young patients with otherwise-unexplained, childhood-onset widespread pain. It characterizes this new disease' s clinical features, diagnostic, and treatment options. Some cases appeared immune mediated and responded to immunomodulatory therapies. abstract OBJECTIVE: We tested the hypothesis that acquired small-fiber polyneuropathy (SFPN), previously uncharacterized in children, contributes to unexplained pediatric widespread pain syndromes.METHODS: Forty-one consecutive patients evaluated for unexplained widespread pain beginning before age 21 had medical records comprehensively analyzed regarding objective diagnostic testing for SFPN (neurodiagnostic skin biopsy, nerve biopsy, and autonomic function testing), plus histories, symptoms, signs, other tests, and treatments. Healthy, demographically matched volunteers provided normal controls for SFPN tests.RESULTS: Age at illness onset averaged 12.3 6 5.7 years; 73% among this poly-ethnic sample were female (P = .001). Sixty-eight percent were chronically disabled, and 68% had hospitalizations. Objective testing diagnosed definite SFPN in 59%, probable SFPN in 17%, and possible SFPN in 22%. Only 1 of 41 had entirely normal SFPN test results. Ninetyeight percent of patients had other somatic complaints consistent with SFPN dysautonomia (90% cardiovascular, 82% gastrointestinal, and 34% urologic), 83% reported chronic fatigue, and 63% had chronic headache. Neurologic examinations identified reduced sensation in 68% and vasomotor abnormalities in 55%, including 23% with erythromelalgia. Exhaustive investigations for SFPN causality identified only history of autoimmune illnesses in 33% and serologic markers of disordered immunity in 89%. Treatment with corticosteroids and/or intravenous immune globulin objectively and subjectively benefited 80% of patients (12/15). CONCLUSIONS:More than half among a large series of patients with childhood-onset, unexplained chronic widespread pain met rigorous, multitest, diagnostic criteria for SFPN, which extends the age range of acquired SFPN into early childhood. Some cases appeared immunemediated and improved with immunomodulatory therapies. Pediatrics 2013;131:e1091-e1100

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“…20 A two-sided approach was chosen (testing both right and left extremities), as symptoms may be asymmetric. 21,22 Hence, a total of 26 combinations (excluding the combination of the original testing protocol, see last row of boxes) were predefined for comparison purposes. After using this flowchart for analysis, the onesided (right side and left side examined separately) findings of all combinations (adding temperature testing protocol 7…”

Section: Ienfd Was Abnormal Ifmentioning

confidence: 99%