Home About us Contact | |||
Fiber Neuropathy (fiber + neuropathy)
Kinds of Fiber Neuropathy Selected AbstractsEffect of intravenous immunoglobulin on cerebellar ataxia and neuropathic pain associated with celiac diseaseEUROPEAN JOURNAL OF NEUROLOGY, Issue 12 2008N. Souayah Background:, Cerebellar syndrome and small fiber neuropathy may complicate celiac disease (CD) and may be resistant to a strict gluten-free diet. Methods:, Case series. Results:, We report three patients with biopsy-proven CD who developed cerebellar ataxia and neuropathic pain despite strict adherence to a gluten-free diet. A small fiber neuropathy was suggested by skin biopsy findings in two patients. All patients' symptoms, including small fiber neuropathy symptoms, responded to treatment with intravenous immunoglobulin (IVIG). Discontinuation of IVIG in two patients resulted in worsened ataxia that reversed after resumption of IVIG. Conclusion:, Intravenous immunoglobulin may be effective in treating cerebellar ataxia and small fiber neuropathy associated with CD, suggesting an immune pathogenesis. Further prospective, controlled studies are necessary to determine the long-term response to IVIG or other immunomodulation therapy. [source] Dermal sheet preparations in the evaluation of dermal innervation in Parkinson's disease and multiple system atrophyJOURNAL OF CUTANEOUS PATHOLOGY, Issue 3 2009Peter Novak Background:, Evaluation of dermal nerve fibers in conventional vertical sections is difficult because of the small number of fibers available for examination. In this study, we evaluated dermal sheet mounts for fibers in which the majority of fibers can be visualized. Methods:, We compared the dermal small fiber density in six Parkinson's disease (PD) and six multiple system atrophy (MSA) patients using dermal sheet preparations (DSP). DSP are based on epidermal-dermal separations and immunostaining of the entire dermis by the nerve growth factor receptor p75 antibody that stains both autonomic and sensory fibers. Results:, The small fiber density was reduced in PD compared with MSA (p < 0.0001), suggesting the presence of small fiber neuropathy in PD. Conclusions:, DSP offer a unique method of evaluation of dermal nerve fibers. This method can be used to evaluate small nerve fibers in many neurological disorders such as MSA and PD. [source] European Federation of Neurological Societies/Peripheral Nerve Society Guideline on the use of skin biopsy in the diagnosis of small fiber neuropathy.JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2010Report of a joint task force of the European Federation of Neurological Societies, the Peripheral Nerve Society Revision of the guidelines on the use of skin biopsy in the diagnosis of peripheral neuropathy, published in 2005, has become appropriate due to publication of more relevant papers. Most of the new studies focused on small fiber neuropathy (SFN), a subtype of neuropathy for which the diagnosis was first developed through skin biopsy examination. This revision focuses on the use of this technique to diagnose SFN. Task force members searched the Medline database from 2005, the year of the publication of the first EFNS guideline, to June 30th, 2009. All pertinent papers were rated according to the EFNS and PNS guidance. After a consensus meeting, the task force members created a manuscript that was subsequently revised by two experts (JML and JVS) in the field of peripheral neuropathy and clinical neurophysiology, who were not previously involved in the use of skin biopsy. Distal leg skin biopsy with quantification of the linear density of intraepidermal nerve fibers (IENF), using generally agreed upon counting rules, is a reliable and efficient technique to assess the diagnosis of SFN (level A recommendation). Normative reference values are available for bright-field immunohistochemistry (level A recommendation) but not yet for confocal immunofluorescence or the blister technique. The morphometric analysis of IENF density, either performed with bright-field or immunofluorescence microscopy, should always refer to normative values matched for age (level A recommendation). Newly established laboratories should undergo adequate training in a well established skin biopsy laboratory and provide their own stratified age and gender-matched normative values, intra- and interobserver reliability, and interlaboratory agreement. Quality control of the procedure at all levels is mandatory (Good Practice Point). Procedures to quantify subepidermal nerve fibers and autonomic innervated structures, including erector pili muscles, and skin vessels are under development but need to be confirmed by further studies. Sweat gland innervation can be examined using an unbiased stereologic technique recently proposed (level B recommendation). A reduced IENF density is associated with the risk of developing neuropathic pain (level B recommendation), but it does not correlate with its intensity. Serial skin biopsies might be useful for detecting early changes of IENF density, which predict the progression of neuropathy, and to assess degeneration and regeneration of IENF (level C recommendation). However, further studies are warranted to confirm the potential usefulness of skin biopsy with measurement of IENF density as an outcome measure in clinical practice and research. Skin biopsy has not so far been useful for identifying the etiology of SFN. Finally, we emphasize that 3-mm skin biopsy at the ankle is a safe procedure based on the experience of 10 laboratories reporting absence of serious side effects in approximately 35,000 biopsies and a mere 0.19% incidence of non-serious side effects in about 15 years of practice (Good Practice Point). [source] Phrenic nerve diabetic neuropathy in rats: unmyelinated fibers morphometryJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2009Valéria Paula S. Fazan Abstract We have demonstrated that phrenic nerves' large myelinated fibers in streptozotocin (STZ)-induced diabetic rats show axonal atrophy, which is reversed by insulin treatment. However, studies on structural abnormalities of the small myelinated and the unmyelinated fibers in the STZ-model of neuropathy are limited. Also, structural changes in the endoneural vasculature are not clearly described in this model and require detailed study. We have undertaken morphometric studies of the phrenic nerve in insulin-treated and untreated STZ-diabetic rats and non-diabetic control animals over a 12-week period. The presence of neuropathy was assessed by means of transmission electron microscopy, and morphometry of the unmyelinated fibers was performed. The most striking finding was the morphological evidence of small myelinated fiber neuropathy due to the STZ injection, which was not protected or reversed by conventional insulin treatment. This neuropathy was clearly associated with severe damage of the endoneural vessels present on both STZ groups, besides the insulin treatment. The STZ-diabetes model is widely used to investigate experimental diabetic neuropathies, but few studies have performed a detailed assessment of either unmyelinated fibers or capillary morphology in this animal model. The present study adds useful information for further investigations on the ultrastructural basis of nerve function in diabetes. [source] Clinical and electromyographic deep tendon reflexes in polyneuropathy: diagnostic value and prevalence,ACTA NEUROLOGICA SCANDINAVICA, Issue 4 2009K. R. Sharma Background,,, Evidence is accumulating that patients with polyneuropathy may present with normal clinical deep tendon reflexes (C-DTR). There are few studies that assessed the diagnostic utility of electromyographically recorded DTR (Er-DTR) in patients with polyneuropathy. Objectives,,, The objectives of this study were twofold: (i) to evaluate the prevalence of preserved C-DTR in polyneuropathy; (ii) diagnostic value of Er-DTR latency measurement in patients with polyneuropathy. Methods,,, We prospectively studied 38 controls and 185 patients with polyneuropathy. All subjects had evaluation of C-DTR, Er-DTR obtained from right biceps brachii (BR), right patellar (PR) and bilateral ankle reflexes (AR). Results,,, Of these 185 patients, 118 (63.8%) had chronic axonal neuropathy (CAN), 49 (26.5%) demyelinating polyradiculoneuropathy (DPN) and 18 (9.7%) small fiber neuropathy (SFN). The C-DTR were normal in 65 patients whereas 39 of these 65 (60%) patients had abnormalities of Er-DTR at one or more sites. Er-DTR latencies in patients with polyneuropathies were prolonged at all sites compared with controls (P < 0.01). Among patients with various types of polyneuropathies the Er-DTR, mean latencies at all the sites and latency indicative of demyelination (>150% of the normal mean) were higher in patients with DPN than that of CAN or SFN (P < 0.01). Conclusions,,, We conclude that C-DTR are preserved in 35.1% of the patients with polyneuropathies and Er-DTR should be performed in such patients in order to provide electrophysiological evidence of a polyneuropathy. Er-DTR are useful in distinguishing axonal from demyelinating disorders of peripheral nerve, and detection of subclinical involvement of large fibers in SFN. [source] |