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Diabetic polyneuropathy (DPN) can be classified based on fiber diameter into three subtypes: small fiber neuropathy (SFN), large fiber neuropathy (LFN), and mixed fiber neuropathy (MFN). We examined the effect of different diagnostic models on the frequency of polyneuropathy subtypes in type 2 diabetes patients with DPN. This study was based on patients from the Danish Center for Strategic Research in Type 2 Diabetes cohort. We defined DPN as probable or definite DPN according to the Toronto Consensus Criteria. DPN was then subtyped according to four distinct diagnostic models. A total of 277 diabetes patients (214 with DPN and 63 with no DPN) were included in the study. We found a considerable variation in polyneuropathy subtypes by applying different diagnostic models independent of the degree of certainty of DPN diagnosis. For probable and definite DPN, the frequency of subtypes across diagnostic models varied from: 1.4% to 13.1% for SFN, 9.3% to 21.5% for LFN, 51.4% to 83.2% for MFN, and 0.5% to 14.5% for non-classifiable neuropathy (NCN). For the definite DPN group, the frequency of subtypes varied from: 1.6% to 13.5% for SFN, 5.6% to 20.6% for LFN, 61.9% to 89.7% for MFN, and 0.0% to 6.3% for NCN. The frequency of polyneuropathy subtypes depends on the type and number of criteria applied in a diagnostic model. Future consensus criteria should clearly define sensory functions to be tested, methods of testing, and how findings should be interpreted for both clinical practice and research purpose., (© 2020 Peripheral Nerve Society.)

Objectives: Chronic inflammatory axonal polyneuropathy (CIAP) is defined on the basis of the clinical, electrophysiological and nerve biopsy findings and therapeutic responses of 'immunotherapy responding chronic axonal polyneuropathy (IR-CAP)'., Methods: The diagnosis of IR-CAP was made when all of three of the following mandatory criterion were met: (1) acquired, chronic progressive or relapsing symmetrical or asymmetrical polyneuropathy with duration of progression >2 months; (2) electrophysiological evidence of axonal neuropathy in at least two nerves without any evidence of 'strict criteria of demyelination'; and (3) definite responsiveness to immunotherapy., Results: Thirty-three patients with IR-CAP showed similar clinical features of chronic inflammatory demyelinating polyneuropathy (CIDP) except 'motor neuropathy subtype'. High spinal fluid protein was found in 27/32 (78%) cases. 'Inflammatory axonal neuropathy' was proven in 14 (45%) of 31 sural nerve biopsies., Discussions: IR-CAP could well be 'axonal CIDP' in view of clinical similarity, but not proven as yet. Thus, IR-CAP is best described as CIAP, a distinct entity that deserves its recognition in view of responsiveness to immunotherapy., Conclusion: Diagnosis of CIAP can be made by additional documentation of 'inflammation' by high spinal fluid protein or nerve biopsy in addition to the first two diagnostic criteria of IR-CAP., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

ICU-acquired weakness (ICUAW), including critical illness polyneuropathy, critical illness myopathy, and critical illness polyneuropathy and myopathy, is a frequent disabling disorder in ICU subjects. Research has predominantly been performed by intensivists, whose efforts have permitted the diagnosis of ICUAW early during an ICU stay and understanding of several of the pathophysiological and clinical aspects of this disorder. Despite important progress, the therapeutic strategies are unsatisfactory and issues such as functional outcomes and long-term recovery remain unclear. Studies involving multiple specialists should be planned to better differentiate the ICUAW types and provide proper functional outcome measures and follow-up. A more strict collaboration among specialists interested in ICUAW, in particular physiatrists, is desirable to plan proper care pathways after ICU discharge and to better meet the health needs of subjects with ICUAW.

The aim of this paper was to define an unexplained non-classified polyneuropathy condition as a new neurological disease. This new diagnosis of occupation related polyneuropathy has been named as "WORKING HAND SYNDROME (WHS)."This study collected and compared clinic and electrophysiological analyze data from healthy controls, WHS patients, carpal tunnel syndrome (CTS) patients and polyneuropathy patients. The WHS patients presented to the clinic with pain, numbness, tingling, and burning sensations in their hands that increased significantly during rest and nighttime. However, there was no weakness in the muscles, and the deep tendon reflexes were normal in this disease. The patients had all been working in physically demanding jobs requiring the use of their hands/arms for at least 1 year, but no vibrating tools were used by the patients. All of the cases were men. I supposed that overload caused by an action repeated chronically by the hand/arm may impair the sensory nerves in mentioned hand/arm. In patients with these complaints, for a definitive diagnosis, similar diseases must be excluded. Nonetheless, the specific electrophysiological finding that the sural nerves are normal on the lower sides, as well as the occurrence of sensory axonal polyneuropathy in the sensory nerves without a significant effect on velocity and latency in the work-ups of the upper extremity are enough to make a diagnosis.In conclusion, WHS has been defined as a polyneuropathy and occupational disease. Patients with WHS present with pain, numbness, tingling, and burning sensations in their hands that increases significantly during rest and nighttime. They also use their arms/hands for jobs that require heavy labor. The neurological examinations of patients with WHS are normal. Only the sensory nerves in the upper extremities are affected. This article is suggested to serve as a resource for patients, health care professionals, and members of the neurology community at large.

Introduction: The aim of this study was to determine the diagnostic usefulness of skin punch biopsies with emphasis on visualization and quantification of T-cells and macrophages in patients with polyneuropathies., Methods: We quantified inflammatory cells in skin samples (lower leg, upper thigh) in 187 patients and compared data with counts in their sural nerve biopsies and with skin biopsies from 32 healthy volunteers., Results: Vessel-bound T-cells and macrophages were increased in proximal and distal skin samples of neuropathy patients compared with controls (P < 0.001 in both). Patients with vasculitic neuropathy had increased T-cell and macrophage counts in distal skin compared with controls (P < 0.01; for scattered macrophages/mm 2 diagnostic sensitivity 71% and specificity 79%). In patients with vasculitic neuropathy, distal skin perivascular inflammatory cell counts also correlated with those in sural nerve biopsies (P < 0.001)., Conclusion: Neuropathy per se may lead to skin inflammation. In cases of possible vasculitic neuropathy, skin biopsy may be an additional tool to support the diagnosis. Muscle Nerve 55: 884-893, 2017., (© 2016 Wiley Periodicals, Inc.)

Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and - arguably - neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard definitions, resulting in inconsistencies between studies. We propose definitions and provide an up-to-date differential diagnosis of multifocal neuropathy. Available evidence suggests that NSVN and neuropathy-predominant systemic vasculitis might be controlled better by treatment with corticosteroids and an immunosuppressive agent than with corticosteroids alone. Treated NSVN rarely spreads to other organs, but 30% of patients experience a relapse. Long-term neurological outcome is favourable, but chronic pain is common.

Objective: Ultrasound differentiation of neuropathies is a great challenge. We, therefore, suggest a standardized score to operationalize differentiation between several acute and subacute onset neuropathies., Method: We retrospectively analyzed the ultrasound data of 61 patients with acute or subacute neuropathies, e.g. chronic immune-mediated neuropathies, Guillain-Barré syndrome (GBS), and axonal/vasculitic neuropathies. We compared these data to 28 healthy controls. Based on these results an ultrasound pattern sum score (UPSS) with three sub-scores (UPS-A for the sensorimotor nerves, UPS-B for the cervical roots and the vagal nerve and UPS-C for the sural nerve) was developed. Afterwards, the applicability of the score was prospectively validated in 10 patients with chronic neuropathies and in 14 patients with unknown acute and subacute PNP before performing additional tests., Results: UPS-A and UPSS were significantly higher in CIDP than in other neuropathies and controls (p<0.001). UPS-B was significantly more often pathologic in GBS than in CIDP and other neuropathies (p<0.001). Using receiver operation characteristics curve analysis boundary values for each score were defined. Positive predictive value (PPV) of these scores for CIDP and GBS was >85%. Vasculitic neuropathies showed an intermediate type of UPSS compared to other axonal neuropathies (p<0.001). In the prospective application the pattern score could be used with good accuracy in several types of neuropathy., Conclusion: UPS-A and UPSS operationalize to diagnose acute and subacute-onset CIDP and its variants with high sensitivity, specificity, and PPV. An increased UPS-B with normal UPSS and other sub scores may point to the diagnosis of GBS with high PPV and enables the differentiation from CIDP., Significance: Using the UPSS and its sub-scores gives a new diagnostic power to the method of the peripheral nerve ultrasound., (Copyright © 2015 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

Natura non facit saltus (Latin for "nature does not make jumps") is a maxim expressing the idea that natural things and properties change gradually, in a continuum, rather than suddenly. In biomedical sciences, for taxonomic purposes, we make jumps that emphasize differences more than similarities. Among the dysimmune neuropathies, 2 disorders, characterized by the presence of antibodies to gangliosides GM1 and GD1a and a peculiar, exclusive motor involvement, have been identified: acute motor axonal neuropathy (AMAN) and multifocal motor neuropathy (MMN). However, anti-GM1 or -GD1a antibodies are also associated with acute motor and sensory axonal motor neuropathy (AMSAN). We review the results of recent clinical and experimental studies showing that AMAN and MMN are not exclusively motor. We discuss the possible explanations for the greater resistance of sensory fibers to antibody attack to finally suggest that AMAN, AMSAN, and MMN belong to a continuous spectrum with a common pathophysiological mechanism., (Copyright © 2013 Wiley Periodicals, Inc.)

Chronic acquired demyelinating neuropathies (CADP) constitute an important group of immune neuromuscular disorders affecting myelin. This article discusses CADP with emphasis on multifocal motor neuropathy, multifocal acquired demyelinating sensory and motor neuropathy, distal acquired demyelinating symmetric neuropathy, and less common variants. Although each of these entities has distinctive laboratory and electrodiagnostic features that aid in their diagnosis, clinical characteristics are of paramount importance in diagnosing specific conditions and determining the most appropriate therapies. Knowledge regarding pathogenesis, diagnosis, and management of these disorders continues to expand, resulting in improved opportunities for identification and treatment., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Hereditary neuropathies belong to the most common neurogenetic disorders. They appear mostly as sensory and motor neuropathies but there are also pure sensory, pure motor as well as sensory and autonomic hereditary neuropathies. In clinical practice, knowledge of hereditary neuropathies is important in order to recognize them among polyneuropathies and achieve a successful genetic diagnosis. The molecular genetics of hereditary neuropathies are very heterogeneous with currently more than 40 known disease-causing genes. The 4 most common genes account for almost 90% of the genetically diagnosed hereditary neuropathies. In this review article we provide an overview of the currently known genes and propose a rational genetic work-up protocol of the most common genes.

Introduction: Little is known about what constitutes appropriate diagnostic testing in patients with distal symmetric polyneuropathy (DSP)., Methods: Utilizing an ICD-9 screening method and medical record abstraction, we determined the number of new cases of DSP within community neurology practices in Nueces County, Texas. We then compared 2 case capture methods (ICD-9 vs. all-case review screening)., Results: The ICD-9 case capture method identified 52 cases over a 3-month period. Comparing case capture methods, the ICD-9 method identified 16 of 17 cases identified by the all-case review method (94%). The ICD-9 method required screening of 84% fewer charts compared with the all-case review., Conclusions: Many new cases of DSP occur each month within Nueces County. The ICD-9 screening technique combined with medical abstraction is an efficient method to identify new DSP cases in this community. These findings are critical for future epidemiological investigations into patients with DSP., (Copyright © 2012 Wiley Periodicals, Inc.)

Serum antibodies to different gangliosides have been identified in some Guillain-Barré (GBS) subtypes and variants. In the January issue of Experimental Neurology Susuki and colleagues (2012) showed that in experimental neuropathies associated with antibodies to GM1, GD1a and GD1b the common mechanism is a complement mediated dysfunction and disruption of the nodes of Ranvier which causes a pathophysiological continuum from early reversible conduction failure to axonal degeneration. These observations, correlated and integrated with electrophysiological and pathological findings in humans indicate that the GBS subtypes acute motor conduction block neuropathy, acute motor axonal neuropathy, acute motor and sensory neuropathy and acute sensory neuropathy and possibly also a chronic disorder as multifocal motor neuropathy represent a spectrum of the same immunopathologic process. Being the nodal axolemma and the paranode the focus of the nerve injury, these immune mediated neuropathies could be more properly classified as nodo-paranodopathies., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: The diagnostic potential of ultrasonography (US) in polyneuropathy has been studied rarely, with complex measurement/correction techniques. Whether US may be useful in clinical practice remains uncertain., Materials and Methods: We aimed to ascertain the value of US, as performed routinely at our institution, in differentiating neuropathy sub-types. We prospectively studied 14 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and 14 patients with sensory axonal neuropathy (SAN). Median nerves were studied bilaterally at wrist and forearm by a radiologist blinded to the neuropathy sub-type. Nerve width (medial to lateral diameter), thickness (anterior to posterior diameter) and cross-sectional area were compared in between patient groups and anatomical sites. Optimal cut-off values were determined using receiver operating characteristic (ROC) curves., Results: Largest measured median nerve thickness was significantly greater in patients with CIDP (P = 0.014), and ROC curve analysis indicated a cut-off offering a sensitivity of 57.1% for CIDP and specificity of 92.9% vs SAN. Nerves were wider and had larger cross-sectional areas, but were not thicker, at wrist compared to forearm in both patient groups. There was an equal prevalence in both patients with CIDP and SAN, of increased median nerve wrist-to-forearm area ratio, compatible with sub-clinical carpal tunnel syndrome., Conclusion: This prospective, blinded, pilot study is the first to indicate the diagnostic potential of US, as performed routinely, in distinguishing between acquired demyelinating and axonal neuropathy. These findings now require confirmation in larger, adequately designed studies, evaluating other nerves/nerve sites, assessing healthy controls and taking into account interrater and equipment variabilities., (© 2011 John Wiley & Sons A/S.)

Frequency and nosological attribution of demyelinating polyneuropathies in patients with diabetes mellitus and alcoholism were determined. Eighty-six inpatients with alcoholic (n=46) and diabetic (n=40) polyneuropathy were examined clinically and using electroneuromyography (ENMG). A demyelinating pathogenetic variant was identified by clinical and ENMG data in 27 (31%) patients. Nine patients (33%) had dysimmune polyneuropathies (acute and chronic inflammatory demyelinating polyneuropathy). Polyneuropathies were specified as toxic/metabolic with the prevalence of a demyelinating component within the main disease in 18 (67%) patients. Clinical and ENMG-signs of the demyelinating variant of alcoholic and diabetic neuropathy are presented. The efficacy of the antioxidant berlition was shown for toxic/metabolic polyneuropathies while the addition of immune modulators was needed for treatment of dysimmune polyneuropathy.

Separate discussion of immune-mediated neuropathies from other neuropathies is justified by the serious consequences of the natural course of these diseases, like disability and sometimes even life threatening conditions. On the other hand nowadays effective treatments already exist, and with timely and correct diagnosis an appropriately chosen treatment may result in significant improvement of quality of life, occasionally even complete recovery. These are rare diseases, and the increasing number of different variants makes it more difficult to recognize them. Their diagnosis is based on the precise knowledge of clinical signs and symptoms, and it is verified by the help of neurophysiologic and laboratory, first of all CSF examinations. Description of clinical features of the classic acute immune-mediated neuropathy, characterized by ascending paresis and demyelination is followed by a summary of characteristics of newly recognized axonal, regional and functional variants. Chronic immune-mediated demyelinating polyneuropathies are not diagnosed in due number even today. This paper does not only present the classic form but it also introduces the ever increasing special variants, like distal acquired demyelinating sensory neuropathy, Lewis-Sumner syndrome, multifocal motor neuropathy and paraproteinemic neuropathies. Vasculitic neuropathies can be divided into two groups: systemic and non-systemic ones. The first sign of a vasculitic neuropathy is a progressive, painful mononeuropathy; the classic clinical presentation is the mononeuritis multiplex. It is characterized by general signs like fever, loss of weight, fatigue. In systemic vasculitis organ specific symptoms are also present. From the paraneoplastic diseases the subacute sensory neuropathy and the sensory neuronopathy are members of the immune-mediated neuropathies, being most frequently associated with small cell lung cancer.

Neurologist S. Weir Mitchell first described "causalgia" following wartime nerve injury, with its persistent distal limb burning pain, swelling, and abnormal skin color, temperature, and sweating. Similar post-traumatic symptoms were later identified in patients without overt nerve injuries after trauma. This was labeled reflex sympathetic dystrophy (RSD; now complex regional pain syndrome type I [CRPS-I]). The pathophysiology of symptoms is unknown and treatment options are limited. We propose that persistent RSD/CRPS-I is a post-traumatic neuralgia associated with distal degeneration of small-diameter peripheral axons. Small-fiber lesions are easily missed on examination and are undetected by standard electrophysiological testing. Most CRPS features-spreading pain and skin hypersensitivity, vasomotor instability, osteopenia, edema, and abnormal sweating-are explicable by small-fiber dysfunction. Small fibers sense pain and temperature but also regulate tissue function through neuroeffector actions. Indeed, small-fiber-predominant polyneuropathies cause CRPS-like abnormalities, and pathological studies of nerves from chronic CRPS-I patients confirm small-fiber-predominant pathology. Small distal nerve injuries in rodents reproduce many CRPS features, further supporting this hypothesis. CRPS symptoms likely reflect combined effects of axonal degeneration and plasticity, inappropriate firing and neurosecretion by residual axons, and denervation supersensitivity. The resulting tissue edema, hypoxia, and secondary central nervous system changes can exacerbate symptoms and perpetuate pathology. Restoring the interest of neurologists in RSD/CRPS should improve patient care and broaden our knowledge of small-fiber functions.

The term polyneuropathy (PNP) is used to describe a group of entities affecting the peripheral nerves, due to external trauma or internal pathology. The prevalence of PNP in the elderly is between 5 and 10%. Despite the multiplicity of causes, the most common etiological factor is diabetes. PNP is characterized by a wide variety of symptoms, due to the multiple functions of nerves. Clinical manifestations range from sensory or motor deficit to inability to maintain gait and stability. Diagnosis is difficult in the elderly, and can be a challenge to the geriatrician in patients with functional impairment. The gold standard for diagnosis is electrophysiology testing. The present article describes the main PNP in the elderly based on the physiopathology of these diseases and provides a practical proposal for clinical classification.

The aim of this study was to evaluate the causes, prevalences, clinical manifestations of hospital-referred polyneuropathies, and evaluate neurophysiological findings in idiopathic polyneuropathy. From 2000 to 2005, 226 patients with polyneuropathy were examined. Polyneuropathy was diagnosed when symptoms, clinical- and neurophysiological findings were compatible with affection of at least two peripheral nerves. They were classified in symptomatic and idiopathic polyneuropathy after investigation. Clinical manifestations were evaluated for diabetes- (DPN), inflammatory- (INPN), hereditary- (HPN) and idiopathic polyneuropathy (IDPN). Neurophysiological findings were investigated in IDPN. 72% had a symptomatic polyneuropathy. Most frequent causes were diabetes mellitus (18%), inflammation, (16%) and hereditary (14%). Most common prevalences per 100,000 were as follows: IDPN, 21; DPN, 13 and HPN, 11. Predominating clinical manifestations were: sensory and motor in INPN, HPN and IPN; sensory in DPN. Pain was more present in IDPN and DPN than in others. In IDPN axonal demyelinating affection was present in 20%. Symptomatic polyneuropathy was common and diabetes mellitus, inflammation and hereditary were frequent causes. In IDPN, DPN, HPN and INPN different clinical patterns were found. Additionally, in IDPN axonal demyelinating affection was more frequent than previously reported.

Only a few studies have been reported describing polyneuropathies in a series of children. To study the clinical and neurophysiological spectrum of polyneuropathies in a large series of children and obtain an overview of their etiologies, this retrospective study reevaluated all electromyograms and electrophysiologic studies performed between 1995 and 2004 in children under 17 years of age at the Radboud University Nijmegen Medical Center, a tertiary neuromuscular reference center. Electromyograms revealing polyneuropathy were selected for further analysis (n = 118), and the medical records were reviewed to supplement electromyographic findings with the clinical diagnosis. Hereditary polyneuropathies made up 68% of the total, and 54% of these were isolated polyneuropathies; in the remaining 46%, polyneuropathy was part of a more complex disorder. The acquired polyneuropathies were primarily inflammatory. Nerve biopsies had been performed in 22 of the 118 cases (19%) and led to a diagnosis in 4 cases. Despite sophisticated investigation, 11 cases (9%) remained unclassified for underlying cause. Hereditary motor and sensory neuropathies are the most common type of polyneuropathy in childhood, followed by polyneuropathies as part of an inborn error of metabolism and inflammatory polyneuropathies (in patients in whom electromyography was used to diagnose the neuropathy). In the full series of patients, nerve biopsy did not play a prominent role in the diagnostic work-up of childhood polyneuropathies, due to the increasing availability of other laboratory (genetic and metabolic) diagnostic tools. Nerve biopsy nonetheless proved to have an important diagnostic yield in selected, complex cases.

Background: Chronic immune-mediated demyelinating polyneuropathy (CIP) represents a heterogeneous pool of motor, sensory, sensorimotor, symmetric, or asymmetric syndromes., Objective: To evaluate published diagnostic classifications and characterize predictors of treatment response., Methods: One hundred two of 158 patients with a working diagnosis of CIP were included and clinically characterized because they had electrophysiologic and/or histologic evidence of demyelination. The biostatistical profile of patients with symmetric clinical manifestation was analyzed using three proposed classifications (American Academy of Neurology [AAN] criteria, modified AAN criteria, European Federation of Neurological Societies/Peripheral Nerve Society [EFNS/PNS] criteria). Treatment responses to IV immunoglobulins (IVIg) and their positive predictors were investigated., Results: Sensitivities (0.52 [AAN] vs 0.83 [modified AAN] vs 0.95 [EFNS/PNS]) and negative predictive values (0.68 vs 0.85 vs 0.92) differed markedly, whereas specificities (0.94 vs 0.90 vs 0.96) and positive predictive values (0.89 vs 0.89 vs 0.97) were similar. In CIP patients treated with IVIg, a positive response was found in 62 of 76 (82%). Patients with a monophasic or relapsing-remitting course or a more than twofold CSF protein increase had the highest probability to respond to IVIg, most evident when using the modified AAN criteria., Conclusions: The European Federation of Neurological Societies/Peripheral Nerve Society criteria for chronic inflammatory demyelinating polyneuropathy improve treatment of patients with chronic immune-mediated demyelinating polyneuropathy, particularly with respect to diagnostic issues. To predict IV immunoglobulin treatment response, the modified American Academy of Neurology criteria are the most valuable classification provided an increased CSF protein level.

Unlabelled: The aim of the study was to assess severity of injury of the peripheral nervous system of chronic renal failure patients on continuous ambulatory peritoneal dialysis (CAPD) or on maintenance hemodialysis (HD) diagnosed based on the electrophysiological testing as well as to establish the effect of adequate dialysis dose on peripheral nerve functioning. The study was performed in 53 patients (21 males, 32 females) on CAPD for mean 16.9 +/- 14.4 months and 68 patients (46 males, 22 females) on HD for mean 47.5 +/- 46.5 months. Both groups were age matched (48.9 +/- 12.4 vs. 50.6 +/- 11.9 yrs; p= NS). Patients with co-existing diseases that could lead to disturbances in nerve conduction were excluded from the study group., Methods: Electrophysiological testing was performed using Medelec Shaphire 2ME equipment. Nerve conductivity testing was performed in motor and sensory fibres of right ulnar nerve, motor fibres of right fibular nerve and left tibial nerve as well as in sensory fibres of right calf nerve. In the sensory nerves there were assessed: amplitude of the evoked sensory response, latency of the response and conduction velocity. In the motor nerves there were analyzed amplitude of the compound muscle action potential, latency of the evoked response, F-wave minimal latency and conduction velocity. Polyneuropathy was diagnosed if abnormal results obtained in electrophysiological examination were found in at least two out of four tested nerves. To assess dialysis adequacy in the CAPD group weekly Kt/V (wKt/ V) and weekly creatinine clearance (WCrCI) were used and in the HD patients the following indices were measured: Kt/V, PRU, TAC and TAD., Results: Polyneuropathy diagnosed based on the above described criteria, was found in 59 (86.8%) HD patients and in 41 (77.4%) CAPD patients (p= NS). The results of motor conductivity testing showed significantly longer distal latency in HD patients in comparison to the CAPD patients in fibular nerve (4.9 +/- 0.9 vs. 4.5 +/- 0.8 ms; p< 0.05) and F-wave latency in ulnar nerve (29.3 +/- 3.1 vs. 27.3 +/- 2.5 ms; p< 0.05), in fibular nerve (52.4 +/- 6.4 vs. 48.5 +/- 7.94 ms; p< 0.05), in tibial nerve (54.8 +/- 7.8 vs. 50.6 +/- 7.12 ms; p< 0.05) and also significantly lower conduction velocity in ulnar nerve (51.9 +/- 5.9 vs. 55.6 +/- 6.99 m/s; p< 0.05), in fibular nerve (41.2 +/- 5.9 vs. 44.5 +/- 5.5 m/s; p< 0.05), in tibial nerve (40.1 +/- 5.81 vs. 42.7 +/- 4.6 m/s; p< 0.05). Mean value of sensory response amplitude evoked in ulnar nerve was significantly higher in the CAPD treated patients than in HD patients (21.2 +/- 14.8 vs. 15.1 +/- 11.4 microV; p< 0.05) and distal latency in calf nerve was significantly shorter (2.1 +/- 1.0 vs. 2.4 +/- 0.6 ms; p< 0.05). Analysing the effect of dialysis on peripheral nerve functioning in the group of CAPD patients a statistically significant relationship between the severity of peripheral nerve injury and WCrCl was demonstrated. Mean value of WCrCl in patients without features of neuropathy was significantly higher (83.3 +/- 28.98 l/week/ 1.73m2) in comparison with those with diagnosed polyneuropathy (59.9 +/- 15.9 l/week/1.73m2) (p< 0.01). Analyzing effect of WCrCl on conduction velocity in the tested nerves there was demonstrated that in patients with WCrCl value at least 60 l/week/1.73m2, mean velocity values were higher, and the statistically significant difference was found for sensory (p< 0.05) and motor (p< 0.01) fibers of ulnar nerve. Mean value of residual renal clearance in the CAPD treated patients without features of sensory and motor nerve injury was significantly higher (2.9 +/- 1.8 ml/min) when compared to the patients with diagnosed polyneuropathy (1.4 +/- 1.3 ml/min) (p= 0.006). In the hemodialyzed group no statistically significant relationship was found between conduction velocity in the tested nerves and indices of dialysis efficiency (Kt/V, PRU) as well as degree of exposure to uremic toxemia described by TAC and TAD., Conclusions: Polyneuropathy is a common complication in patients with chronic renal failure independently of a kind of the therapy. Well preserved residual renal function in CAPD patients plays an important role in improving effectiveness of the dialytic treatment, in consequence influencing preservation of proper peripheral nerve function.

Restless legs syndrome (RLS), diagnosed according to the International RLS Study Group criteria, was investigated in 97 consecutive patients with polyneuropathy and found in 29 patients. RLS patients were more often women (22 of 29 vs. 33 of 68; P = 0.015), mainly with sensory neuropathy of small fiber type (15 of 29 vs. 16 of 68; P = 0.009). Changes of sensory action potentials were significantly less severe in RLS patients. In the RLS group, acquired neuropathies, and in particular dysimmune neuropathies, were significantly more frequent (27/29 vs. 46/68; P = 0.009). Thus, RLS is frequent in acquired polyneuropathy of sensory type and mild entity, mainly in women., ((c) 2006 Movement Disorder Society)

In elderly patients, peripheral neuropathies are common and may lead to disability. In order to investigate the relative incidence of different polyneuropathies in the elderly focusing on the contribution of nerve biopsy to their diagnosis, the authors studied 74 patients over 65 years of age with clinical, laboratory, electrophysiological, and sural nerve biopsy findings of different types of polyneuropathy. Vasculitic polyneuropathy seemed to be the most common cause of disabling neuropathy in the elderly, followed by paraneoplasia and diabetes. The possible diagnosis of idiopathic axonal neuropathy in the nine cases with neuropathy of unknown origin is discussed.

Vasculitic neuropathy usually presents with painful mononeuropathies or an asymmetric polyneuropathy of acute or subacute onset. The disorder should be classified as being systemic or non-systemic. Systemic vasculitis should be further classified into one of the primary and secondary forms. Although specific treatment regimens vary among neurologists, basic principles can be applied. Corticosteroids and cytotoxic drugs have been the mainstay of treatment for most forms of vasculitic neuropathy. Here we discuss dosing, potential side-effects, and management recommendations of conventional treatments. New treatments showing promise include intravenous immunoglobulin and biological agents and trials of the newest treatments are being reviewed. Future trials should compare commonly used treatment regimens and better establish the efficacy of newer, potentially safer, treatments.

POEMS syndrome is a unique clinical entity. Although it's a diagnosis of exclusion, it was previously described by the presence of several typical characteristics as paraproteinemia, polyneuropathy, organomegaly, endocrinopathy, and skin changes. Recently, new criteria were proposed, and the presence of two major and one minor criterion was claimed to suffice for a diagnosis. Both methods considered other important characteristics germane to the syndrome unessential for diagnosis. Retrospective evaluation of patients with lymphoproliferative disease was carried out to reveal the presence of the syndrome according to these different methods. Patients' clinical progression during follow-up will be used to validate the criteria's sensitivity and specificity. Six hundred twenty-nine consecutive files of patients with paraproteinemia who were followed-up at a tertiary medical center were reviewed. Of 12 patients who fulfilled the new criteria for diagnosis of POEMS, 3 remain stable during long-term follow-up and only 5 finally developed the full-blown syndrome. Four patients developed other diseases that accounted for their clinical findings. Patients presenting with neuronal vasculitic changes on biopsy, kappa light-chain monoclonal gammopathy, and cryoglobulinemia were unlikely to develop POEMS syndrome, even though they fulfilled the newly suggested criteria. Although they are not in the criteria, sclerotic bone lesions were found only in patients who eventually developed the full syndrome. The diagnosis of POEMS syndrome according to the newly suggested criteria should not be definitive in the presence of atypical clinical features of the syndrome., (Copyright (c) 2005 Wiley-Liss, Inc.)

Objective: To present criteria for pathophysiological interpretation of motor and sensory nerve conduction studies and for pathophysiological classification of polyneuropathies suggested by a group of European neurophysiologists., Methods: Since 1992 seven neurophysiologists from six European countries have collected random samples of their electrodiagnostic examinations for peer review medical audit in the ESTEEM (European Standardized Telematic tool to Evaluate Electrodiagnostic Methods) project. Based on existing criteria in the literature, the experience with a patient material of 572 peer reviewed electrodiagnostic examinations, and productive discussions between the physicians at workshops, the collaboration has produced a set of criteria now routinely used at the centres involved in the project., Results: The first part of the paper considers pathophysiology of individual nerve segments. For interpretation of motor and sensory nerve conduction studies, figures showing change in amplitude versus change in conduction velocity/distal latency and change in F-wave frequency versus change in F-wave latency are presented. The suggested boundaries delimit areas corresponding to normal, axonal, demyelinated, or neuropathic nerve segments. Criteria for motor conduction block in upper and lower extremities are schematically depicted using the parameters CMAP amplitude and CMAP duration. The second part of the paper suggests criteria for classification of polyneuropathies into axonal, demyelinating, or mixed using the above-mentioned criteria., Conclusions: The suggested criteria are developed during many years of collaboration of different centres and may be useful for standardization in clinical neurophysiology., Significance: Consistent interpretation of nerve conduction studies is an important step in optimising diagnosis and treatment of nerve disorders.

Diabetes mellitus is associated with a wide spectrum of neuropathy syndromes, ranging from a mild asymptomatic distal sensory neuropathy to a severe disabling radiculoplexus neuropathy. As the pathophysiology of these separate conditions is better understood, classification of the various phenotypes becomes important because of treatment implications. Here we provide a short summary of the history of the classification of diabetic neuropathies and try to describe the most common forms classified according to their presumed pathophysiology. We have tried to include epidemiological data where available, as well as histopathology of nerve in several diabetic neuropathies.

The objective of this report was to develop a case definition of distal symmetric polyneuropathy to standardize and facilitate clinical research and epidemiologic studies. A formalized consensus process was employed to reach agreement after a systematic review and classification of evidence from the literature. The literature indicates that symptoms alone have relatively poor diagnostic accuracy in predicting the presence of polyneuropathy; signs are better predictors of polyneuropathy than symptoms; and single abnormalities on examination are less sensitive than multiple abnormalities in predicting the presence of polyneuropathy. The combination of neuropathic symptoms, signs, and electrodiagnostic findings provides the most accurate diagnosis of distal symmetric polyneuropathy. A set of case definitions was rank ordered by likelihood of disease. The highest likelihood of polyneuropathy (useful for clinical trials) occurs with a combination of multiple symptoms, multiple signs, and abnormal electrodiagnostic studies. A modest likelihood of polyneuropathy (useful for field or epidemiologic studies) occurs with a combination of multiple symptoms and multiple signs when the results of electrodiagnostic studies are not available. A lower likelihood of polyneuropathy occurs when electrodiagnostic studies and signs are discordant. For research purposes, the best approach to defining distal symmetric polyneuropathy is a set of case definitions rank ordered by estimated likelihood of disease. The inclusion of this formalized case definition in clinical and epidemiologic research studies will ensure greater consistency of case selection.

Polyneuropathies are common disorders of the peripheral nervous system. Early diagnosis and therapy enables to stop the progression of the polyneuropathy and to ameliorate polyneuropathic symptoms in most cases. Clinical examination is sufficient to diagnose polyneuropathy. However, to reveal the etiology of a polyneuropathy additional diagnostic procedures are necessary. The general practitioner should recognize the signs and symptoms of a polyneuropathy and start necessary investigations. If the etiology of the polyneuropathy is revealed specific therapy can be started. Furthermore, polyneuropathic symptoms can be ameliorated independently of the underlying cause.

Vasculitic neuropathies are immune mediated diseases of the peripheral nervous system, in which inflammation of the blood vessels causes damage to the nerves. We distinguish neuropathies associated with primary and secondary systemic vasculitis, with rheumatic diseases, with malignant disorders, drug-induced vasculitis and the non-systemic vasculitic neuropathies (NSVN). The typical clinical picture consists in an asymmetric or multifocal, painful sensorimotor neuropathy with an acute, subacute or chronic course and acute relapses. Neurophysiology reveals an active, asymmetric, axonal sensorimotor neuropathy. The disorders usually respond to immunosuppressive treatment. A diagnosis of definite vasculitis can be made with evidence of vasculitis in a biopsy specimen. The absence of positive morphological evidence, however, does not exclude the diagnosis. There is no single laboratory test that can prove or exclude vasculitis, in NSVN even an elaborate panel of blood tests can show normal findings. Systemic vasculitis has an incidence of 4/100,000 per year and, untreated, has a poor prognosis, which is greatly improved by the use of immunosuppressive treatment. The prognosis of NSVN is generally better, although many patients need long term immunosuppression. Current treatment recommendations for vasculitic neuropathies are presented.

Objective: Considerable debate still exists regarding the classification of polyneuropathies (PNPs) into predominantly demyelinating, predominantly axonal loss, mixed or unclassified. This study was designed to determine the variation among physicians in the classification of PNPs by using the European Standardized Telematic tool to Evaluate Electromyography knowledge-based systems and Methods (ESTEEM) multicenter database., Methods: Seven physicians from 6 laboratories in Europe sent a total of 156 prospectively collected cases of PNP with electromyography (EMG) data including diagnosis (examination diagnosis) to the database. Each physician interpreted the electrophysiological data from all cases (interpretation diagnosis) and a final diagnosis was given at the consensus meetings of the group (consensus diagnosis)., Results: Comparison of each physician's examination diagnosis with his/her interpretation diagnosis, i.e. intra-physician variation, showed a change towards less classified PNPs (P < 0.05). Interpretation diagnoses showed large inter-physician variation in the classification of PNPs. The consensus group was more cautious than individual physicians in classifying PNPs as mixed and axonal. The probability of the consensus diagnosis increased with increasing number of abnormal motor and sensory segments tested., Conclusions: Recognition of variation in classification of PNP as shown in this study and suggesting standards of good clinical practice developed by a consensus group may increase the quality of EMG practice.

Objectives: To study a representative group of patients with chronic acquired symmetric demyelinating polyneuropathies, and to evaluate classification by pattern of weakness and by presence of immunoglobulin monoclonal protein (M protein)., Methods: In Vest-Agder County, Norway, an unselected population of patients with chronic symmetric polyneuropathies who fulfill electrodiagnostic criteria for demyelination are registered in a database and followed up prospectively. Data were taken from the database on April 2, 2001. Patients with proximal as well as distal weakness were classified as having chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and patients with only distal symptoms as having distal acquired demyelinating symmetric polyneuropathy (DADS)., Results: A total of 29 patients had chronic acquired symmetric demyelinating polyneuropathy; 15 had CIDP and 14 had DADS. The 2 categories differed regarding spinal protein level (mean +/- SD, 0.102 +/- 0.060 g/dL in CIDP vs 0.065 +/- 0.029 g/dL in DADS; P =.05); clinical course (remitting in 6 of 13 patients with CIDP vs 0 of 14 with DADS; P =.02); disability score at diagnosis (mean +/- SD, 3.3 +/- 1.0 in CIDP vs 1.9 +/- 0.6 in DADS; P<.001) and at peak of symptoms (mean +/- SD, 3.6 +/- 1.1 in CIDP vs 2.3 +/- 0.6 in DADS; P<.001); and response to immunosuppressive treatment (11 of 12 patients with CIDP vs 2 of 7 with DADS; P =.01). An M protein was detected in 8 patients (3 with CIDP and 5 with DADS). Patients with polyneuropathy with and without M protein were similar in clinical features, course, disability, and treatment response., Conclusion: Classification by presence or absence of proximal weakness separates patients with chronic acquired symmetric demyelinating polyneuropathy into groups that are different in clinical course, disability, and treatment response.

Background: In the World Health Organisation (WHO) International Classification of Impairments, Disabilities, and Handicaps (ICIDH), it is suggested that various levels of outcome are associated with one another. However, the ICIDH has been criticised on the grounds that it only represents a general, non-specific relation between its entities., Objective: To examine the significance of the ICIDH in immune mediated polyneuropathies., Methods: Four impairment measures (fatigue severity scale, MRC sum score, "INCAT" sensory sum score, grip strength with the Vigorimeter), five disability scales (nine hole peg test, 10 metres walking test, an overall disability sum score (ODSS), Hughes functional grading scale, Rankin scale), and a handicap scale (Rotterdam nine items handicap scale (RIHS9)) were assessed in 113 clinically stable patients (83 with Guillain-Barré syndrome, 22 with chronic inflammatory demyelinating polyneuropathy, eight with a gammopathy related polyneuropathy). Regression analyses with backward and forward stepwise strategies were undertaken to determine the correlation between the various levels of outcome (impairment on disability, impairment on handicap, disability leading to handicap, and impairment plus disability on handicap)., Results: Impairment measures explained a substantial part of disability (R(2) = 0.64) and about half of the variance in handicap (R(2) = 0.52). Disability measures showed a stronger association with handicap (R(2) = 0.76). Combining impairment and disability scales accounted for 77% of the variance in handicap (RIHS9) scores., Conclusions: In contrast to some suggestions, support for the ICIDH model is found in the current study because significant associations were shown between its various levels in patients with immune mediated polyneuropathies. Further studies are required to examine other possible contributors to deficits in daily life and social functioning in these conditions.

We studied: (1) the sensitivity of various neurophysiologic parameters in the diagnosis of uremic polyneuropathy, (2) the relationship between subjective symptoms and neurophysiologic parameters, and (3) the effect of a single hemodialysis on the neurophysiologic parameters in 21 patients undergoing hemodialysis. The following parameters were studied: sensory and motor nerve conduction, including F-wave parameters; vibration detection thresholds; and thermal thresholds. The clinical findings and subjective symptoms were studied using a standardized questionnaire. The most sensitive parameters in the diagnosis of uremic neuropathy were F-wave parameters from lower limbs, vibration detection thresholds from the feet, and the sural nerve sensory action potential amplitude. The nerves from the upper extremities on the side of the fistula should not be used in the diagnosis of uremic polyneuropathy due to numerous mild local nerve lesions. The positive neuropathic symptoms correlated with quantitative vibratory detection thresholds and sensory nerve conduction studies, especially the amplitude of the sensory nerve action potential in the sural nerve. We found no significant change in any of the neurophysiologic parameters following a single hemodialysis session., (Copyright 2002 Wiley Periodicals, Inc.)

Objectives: To determine the validity, reliability, and responsiveness of a new overall disability sum score in immune mediated polyneuropathies., Methods: Three impairment measures (MRC sum score, sensory sum score, grip strength (Vigorimeter)) and three disability scales (an overall disability sum score (ODSS), Hughes' functional scale (f score), Rankin scale) were assessed in a cross sectional group of 113 clinically stable patients (83 with Guillain-Barré syndrome, 22 with chronic inflammatory demyelinating polyneuropathy (CIDP), eight with a gammopathy related polyneuropathy). The ODSS was also used serially in 20 patients with recently diagnosed Guillain-Barré syndrome (n = 7) or CIDP (n = 13) and changing clinical conditions. Multiple regression studies were performed to compare the impact of impairment disturbances (independent variables) on the various disability scales (dependent variable)., Results: Moderate to good construct validity (stable group: Spearman's rank test (absolute values), r = 0.41-0.79; longitudinal group: multiple correlation coefficient, R = 0.69-0.89; p < 0.006 for all associations) and reliability (intraclass correlation coefficient, R = 0.90-0.95; p < 0.0001) were demonstrated for the ODSS. Its SRM values were high (> 0.8), indicating good responsiveness. Impairment measures accounted for a higher variance proportion of the ODSS compared with the f score and Rankin (R = 0.64 v 0.56 and 0.45, respectively)., Conclusions: All clinimetric requirements were met by the overall (arm and leg) disability sum score in immune mediated polyneuropathies. Its use is therefore suggested in evaluating immune mediated polyneuropathies.

Neuropathies associated with monoclonal gammopathy of undetermined significance (MGUS) occur frequently in the elderly. Data from 42 consecutive elderly patients with a MGUS-associated neuropathy - 12 patients with IgM MGUS, 25 patients with IgG MGUS, 5 patients with IgA MGUS - were evaluated prospectively using quantifiable clinical criteria and quantitative sensory testing. Results consistent with preferential injury to large fibers were found in patients with IgM isotypes (P <.005) but not in patients with IgG isotypes (P <.0005 to.0004). These findings support an immunologic origin for at least some MGUS-associated neuropathies and provide information important for the clinical evaluation of neuropathies in the elderly.

Our recent study showed that the overall prevalence of CIDP was estimated as 2.2 per 100,000 population in Aomori Prefecture, in Northan Honshu of Japan. In our series of more than 80 cases with CIDP, a chronic acquired inflammatory demyelinating polyneuropathy, nearly 30% showed clear laterality of weakness, and electrophysiologic laterality or multifocality was apparent in almost all cases. Nearly 90% of patients were able to walk without walking aids or other assistance. Sixty% showed distal dominant muscular weakness. In 12 patients with age of onset under 15, pes cavus deformity was seen in 5. Two thirds complained numbness in the extremities during progressive phase. Four cases initially showed severe sensory ataxia associated with motor conduction block. It should be, thus, reminded that clinical spectrum of CIDP is enormously wide: chronic acquired demyelinating multiple mononeuropathy showing asymmetric involvement (Lewis-Summer syndrome) should be put on one side of the clinical presentation of CIDP. Multifocal motor neuropathy (MMN) is, on the other hand, an unique syndrome mimicking amyotrophic lateral sclerosis (ALS). There may be, however, true association syndrome of CIDP and ALS presenting both peripheral nerve demyelination and pyramidal sign with progressive bulbar involvement. Recently, several atypical varieties of CIDP showing only one-limb involvement, upper limb weakness rather than lower limb power loss, or proximal weakness, etc ... have been reported in the literature. To realize such clinical variations of chronic acquired demyelinating neuropathy is important for early diagnosis and commencement of treatment of CIDP. Clinical guideline for suspicion of CIDP could be useful for general physicians and neurologists unfamiliar to peripheral neuropathies.

In humans, damage to the nervous system can lead to a pain state referred to as neuropathic pain. Here, we give a short overview of the clinical picture and classification of neuropathic pain and highlight some of the currently known pathophysiological mechanisms involved, with special emphasis on neuropeptide plasticity. In this context, we discuss a specific group of neuropeptides, the melanocortins. These peptides have been demonstrated to play a role in nociception and to functionally interact with the opiate system. Recently, we demonstrated that spinal melanocortin receptors are upregulated in a rat model of neuropathic pain and that blockade of the melanocortin MC(4) receptor has anti-allodynic effects in this condition, suggesting that the melanocortin system plays a role in neuropathic pain. A natural agonist of melanocortin receptors is alpha-melanocyte-stimulating hormone (alpha-MSH), derived from the precursor molecule pro-opiomelanocortin (POMC). Cleavage of this precursor also yields beta-endorphin, which is co-released with alpha-MSH in nociception-associated areas of the spinal cord. We hypothesise that melanocortin receptor blockade attenuates a tonic influence of alpha-MSH on nociception, thus allowing the analgesic effects of beta-endorphin to develop, resulting in the alleviation of allodynia. In this way, treatment with melanocortin receptor antagonists might enhance opioid efficacy in neuropathic pain, which would be of great benefit in clinical practice.

Neuropathic pains refer to a heterogeneous group of pain conditions characterised by lesion or dysfunction of the normal sensory pathways. Clinical characteristics include: delayed onset of pain after nervous system lesion, pain in area of sensory loss, spontaneous and different evoked types of pains. It has so far only been possible to classify these pains on basis of underlying cause or on anatomical location. The mechanisms underlying neuropathic pain are not yet clear, but neuronal hyperexcitability in those neurons that have lost their normal patterned input seems to be a common denominator for many, if not all types, of neuropathic pains. Along these lines, a mechanism-based classification has recently been proposed, which is an attractive approach because it provides a frame for a rationally based therapy of neuropathic pains. The clinical manifestations of neuronal hyperexcitability due to nervous system lesions is described.

Neuromuscular blocking agents are routinely used as an adjunct therapy for critically ill patients. Unlike many neuromuscular blocking agents, vecuronium does not cause significant histamine release, which may lead to bronchoconstriction. Due to a short duration of action and limited accumulation, vecuronium has been widely used. Prolonged ventilatory dependence due to persistent neuromuscular blockade has been reported in patients treated with vecuronium. We report a case of an 8-year-old girl who had a primarily motor axonopathy presenting with weakness after extended vecuronium administration with prolonged course of recovery. This primarily motor neuropathy with axonal features may be a variant of critical illness polyneuropathy, a rarely reported condition in pediatric patients.

Background: Persian Gulf syndrome is a set of symptoms that do not fit into well-understood diagnostic categories. Among these symptoms, there are some that could suggest a generalized neuropathic process., Objective: Correlate neurological symptoms with objective electrodiagnostic findings., Methods: A randomized sample of 176 Persian Gulf veterans (PGV) evaluated at the San Juan Veterans Administration Medical Center was obtained. The subjects completed a questionnaire, and those who met the inclusion criteria underwent electrodiagnostic evaluation., Results: Of the 176 PGV selected, 162 completed the questionnaire. The next step was to perform electrodiagnostic studies on those who described symptoms suggesting peripheral neuropathy and met the inclusion criteria. Twelve individuals met the inclusion criteria for electro-diagnostic studies. All studies were normal except that two subjects were found to have bilateral carpal tunnel syndrome., Conclusions: Although this is a relatively small sample of PGV, the findings are in accordance with other studies in which no definite generalized neuropathic pattern has been described.