DEVELOPMENT OF GANGLIONOPATHY AND TABETIC VISCERAL CRISES ON THE BACKGROUND OF POLYRADICULONEUROPATHY ASSOCIATED WITH MONOCLONAL GAMMOPATHY (CASE REPORT).

The article presents an analysis of the clinical occurrence of development of chronic polyradiculoneuropathy associated with monoclonal IgG/k (kappa) gammopathy of the undetermined significance. The peculiarity of this occurrence is the uniqueness of the development of the symptoms which are characteristic of tabes dorsalis in this pathology with episodic severe visceral crises and also with ganglionopathy. The example describes the clinical polymorphism of the course of visceral crises, the problems of their diagnosis and as a consequence of inadequate treatment with the development of severe social maladaptation. The importance of timely diagnosis and treatment of such conditions is discussed.

Immunotherapy-responsive allodynia due to distal acquired demyelinating symmetric (DADS) neuropathy.

INTRODUCTION: Distal acquired demyelinating symmetric (DADS) neuropathy is a distal variant of chronic inflammatory demyelinating polyradiculoneuropathy. It is characterized by chronic distal symmetric sensory or sensorimotor deficits. Sensory ataxia is a common clinical presentation. Nerve conduction studies typically show markedly prolonged distal motor latencies. METHODS: We report 2 patients with chronic progressive generalized pain and fatigue, with normal neurological examinations except for allodynia. RESULTS: Nerve conduction studies were typical of DADS neuropathy. Monoclonal protein studies were negative. Cerebrospinal fluid protein levels were elevated. Sural nerve biopsies revealed segmental demyelination and remyelination. One biopsy had marked endoneurial and epineurial lymphocytic infiltration. Immunomodulatory therapy alleviated the pain and fatigue and markedly improved distal motor latencies in both patients. CONCLUSIONS: DADS neuropathy can present with pain and a normal neurological examination apart from allodynia. Nerve conduction studies are necessary for diagnosis. These patients respond to immunotherapy better than typical DADS neuropathy patients with sensory ataxia. Muscle Nerve 54: 973-977, 2016.

Repeated Acute Exacerbations of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Accompanied by Pain and Swelling in Distal Extremities.

An 81-year-old man experienced acute progression of weakness in the extremities accompanied by a fever, tenderness, and swelling in distal parts of the extremities. He had flaccid tetraparesis with fasciculations and general hyporeflexia. Nerve conduction studies indicated demyelinating sensorimotor neuropathy. A cerebrospinal fluid examination revealed elevated proteins without pleocytosis. Immunological treatments were effective, but his symptoms exhibited repeated relapse and remission phases. He was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with an acute onset. The highlight of this case is pain with inflammatory reaction recognized as red flags of CIDP, with the clinical course and electrophysiological findings compatible with CIDP.

Evidence-based review of therapeutic plasma exchange in neurological disorders.

Several neurologic disorders have been treated with therapeutic plasma exchange (TPE). Case reports, case series, and clinical trials have published results regarding the outcomes in such patients. The data gathered have been used to formulate evidence-based guidelines, which can be used to guide therapy in patients with these neurological disorders. Adequately designed and powered randomized controlled trials have proven the efficacy of TPE in some disease entities, while other diseases are lacking such data. In the latter, decisions for the use of TPE must be made using the limited published data available. In this review, we discuss the published evidence regarding the use of TPE in neurological disorders, focusing on the most recent guidelines published by the American Society of Apheresis in 2010 and the American Academy of Neurology in 2011.

Clinical course of acute canine polyradiculoneuritis following treatment with human IV immunoglobulin.

Treatment of dogs with acute canine polyradiculoneuritis (ACP) is restricted to physical rehabilitation and supportive care. In humans with Guillain-Barré syndrome, the counterpart of ACP, randomized trials show that IV immunoglobulin (IVIg) speeds recovery. The authors of the current study hypothesized that dogs with ACP would tolerate IVIg well and recover faster than dogs managed with supportive treatment only. Sixteen client-owned dogs with ACP were treated with IVIg, and 14 client-owned dogs served as a retrospective control group. Diagnosis was confirmed using clinical features, electrodiagnostics, cerebrospinal fluid analysis, and muscle/nerve biopsies. The duration of the initial progressive phase, the time from IVIg administration until the dogs were ambulating without assistance, and the duration of the complete episode were evaluated. Adverse reactions (anaphylaxis, mild hematuria) were observed in two dogs. Dogs treated with IVIg were ambulating without assistance after a median of 27.5 days (range, 15-127 days) from onset of clinical signs. The control group was ambulatory without assistance at a median of 75.5 days (range, 5-220 days). Even though this result is not statistically significant, there is a clear trend toward faster recovery in dogs treated with IVIg.

Anti-MAG neuropathy: From biology to clinical management.

The acquired chronic demyelinating neuropathies include a growing number of disease entities that have characteristic, often overlapping, clinical presentations, mediated by distinct immune mechanisms, and responding to different therapies. After the discovery in the early 1980s, that the myelin associated glycoprotein (MAG) is a target antigen in an autoimmune demyelinating neuropathy, assays to measure the presence of anti-MAG antibodies were used as the basis to diagnose the anti-MAG neuropathy. The route was open for describing the clinical characteristics of this new entity as a chronic distal large fiber sensorimotor neuropathy, for studying its pathogenesis and devising specific treatment strategies. The initial use of chemotherapeutic agents was replaced by the introduction in the late 1990s of rituximab, a monoclonal antibody against CD20+ B-cells. Since then, other anti-B cells agents have been introduced. Recently a novel antigen-specific immunotherapy neutralizing the anti-MAG antibodies with a carbohydrate-based ligand mimicking the natural HNK-1 glycoepitope has been described.

European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision.

The aim of this guideline is to update the 2006 EFNS/PNS guideline on management of patients with a demyelinating neuropathy and a paraprotein (paraproteinemic demyelinating neuropathy [PDN]) by review of evidence and expert consensus. In the absence of adequate evidence, the panel agreed on good practice points: (1) patients with PDN should be investigated for a malignant plasma cell dyscrasia; (2) a monoclonal gammopathy of undetermined significance is more likely to be causing the neuropathy if it is immunoglobulin (Ig)M, anti-neural antibodies are present, and the clinical phenotype is chronic distal sensory neuropathy; (3) patients with IgM PDN usually have predominantly distal sensory impairment, prolonged distal motor latencies, and often anti-myelin-associated glycoprotein antibodies; (4) IgM PDN may respond to immunomodulatory therapies. Their potential benefit should be balanced against possible side effects and the usually slow disease progression; (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy; and (6) Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes syndrome is a multi-system malignant PDN.

[A case of polyradiculoneuritis associated with disseminated herpes zoster].

A 34-year-old man developed right-dominant lower limb paraplegia, and then upper limb paresis with radicular pain following disseminated herpes zoster (HZ) in his right forehead, back of the trunk, and lumbar and right lower limb regions. Cerebrospinal fluid (CSF) findings revealed an increase in lymphocytes (32 cells/µl) and protein content (50 mg/dl), and polymerase chain reaction (PCR) for varicella-zoster virus (VZV) DNA was negative in CSF, but VZV antigen was positive in the patient's vesicle smear. Lumbar root MRI using 3D Nerve VIEW (Philips) imaging showed high-intensity lesions on the L2-L5 spinal roots with contrast enhancements, and cervical MRI showed similar findings on both sides at the C4-Th1. Peripheral nerve conduction study revealed prolonged distal latency to 4.9 ms, decreased MCV to 38 m/s, and complete loss of F-wave was seen in the right peroneal nerve study. Minimal F-wave latency was prolonged in the right tibial nerve. Thus, the patient was diagnosed with VZV polyradiculoneuritis caused by disseminated HZ. Regarding the possible pathogenesis of polyradiculoneuritis in this patient with disseminated HZ, we speculate that VZV reached by retrograde transmission from the involved peripheral nerves to the spinal ganglia, which, then, produced polyradiculoneuritis.

Diagnosis and treatment in inflammatory neuropathies.

The inflammatory neuropathies are a large diverse group of immune-mediated neuropathies that are amenable to treatment and may be reversible. Their accurate diagnosis is essential for informing the patient of the likely course and prognosis of the disease, informing the treating physician of the appropriate therapy and informing the scientific community of the results of well-targeted, designed and performed clinical trials. With the advent of biological therapies able to manipulate the immune response more specifically, an understanding of the pathogenesis of these conditions is increasingly important. This review presents a broad overview of the pathogenesis, diagnosis and therapy of inflammatory neuropathies, concentrating on the most commonly encountered conditions.

[Inflammatory neuropathies and multineuritis]. / Neuropathies inflammatoires et mononévrites.

Inflammatory neuropathies include those neuropathies in which the diagnosis, outcome and type of treatment are badly known, the reason of this review. They are expressed as diffuse (such as CIDP and ganglionopathies), multifocal (vasculitic neuropathy) or focal (MMN; plexopathies; immune reconstitution inflammatory syndrome). These forms of neuropathies are important to be known because the beneficial therapeutic possibilities of immunosuppression.

Wernicke encephalopathy concurrent with polyradiculoneuropathy in a young man after bariatric surgery: A case report.

RATIONALE: Bariatric surgery is the recommended treatment for morbid obesity because of its rapid and sustained body weight loss effect. Nutrient deficiency-related neurological complications after bariatric surgery are often disabling. Thus, early recognition of these complications is important. Neurological complications involving the central and peripheral nerve system after bariatric surgery were reported. However, the report on the clinical course of the concurrent involvement of central and peripheral nervous system is limited. We present a rare case of a patient who developed Wernicke encephalopathy concurrent with polyradiculoneuropathy after receiving bariatric surgery. PATIENT CONCERNS: A 22-year-old man with a history of morbid obesity presented progressive bilateral lower limbs weakness, blurred vision, and gait disturbance 2 months after receiving laparoscopic sleeve gastrectomy. Bilateral lower limb numbness and cognition impairment were also noted. DIAGNOSIS: Brain magnetic resonance imaging and electrophysiologic studies confirmed the diagnosis of Wernicke encephalopathy concurrent with acute polyradiculoneuropathy. INTERVENTIONS: Vitamin B and folic acid were given since admission. He also received regular intensive rehabilitation program. OUTCOMES: The subject's cognitive impairment and diplopia improved 1 week after admission under medical treatments, yet lower limb weakness and gait disturbance were still noted. After a month of intensive inpatient rehabilitation, he was able to ambulate with a walker for 30 munder supervision. LESSONS: Nutrient deficiency-related neurological complications after bariatric surgery are often disabling and even fatal. Prevention of neurological complications can be improved through close postsurgical follow-up of the nutritional status. Recognizing the signs and symptoms and evaluating the medical history are critical to the early diagnosis and treatment of this potentially serious yet treatable condition.

Outcome measures in immune-mediated neuropathies: the need to standardize their use and to understand the clinimetric essentials.

Peripheral neurological disorders like neuropathies may cause impairments (such as weakness and sensory deficits), which may lead to problems in daily life and social functioning with a possible decrement in quality of life expectations. Choosing the proper outcome measure to evaluate the therapeutic efficacy of an intervention at one of these levels of outcome should therefore be considered as fundamental to the design of randomized trials in peripheral neurological disorders. However, these choices are dependent not only on the proposed research purposes but also, and perhaps more importantly, on the fulfillment of the scientific needs of these measures. With an increasing demand for accuracy, a thorough and comprehensive evaluation of an outcome measure is needed to determine its simplicity, communicability, validity, reliability, and responsiveness before being clinically applicable, techniques that are being captured by the science of clinimetrics. Most neurologists are still unfamiliar with these rigorous methodological essentials or overlook some of them in their trial preparations because these are considered time consuming and mind numbing. This review will highlight, against the background of the international classification framework and clinimetric needs for outcome measures, the selected scales applied in published randomized controlled trials in patients with Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and gammopathy-related neuropathies. The need for comparison responsiveness studies between equally valid and reliable measures and to standardize their use is emphasized in these conditions. Finally, specific recommendations are given to move from classic to modern clinimetric approach when constructing, evaluating, and selecting outcome measures using new methods like Rasch analysis, accentuating the need of shifting toward a more modern era.

[Dysimmune neuropathies: current diagnosis and therapy]. / Neuropathies dysimmunitaires.

Dysimmune neuropathies encompass an acute form, Guillain-Barré syndrome (GBS), and mainly 3 chronic forms: chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with anti-MAG (myelin-associated-glycoprotein) IgM monoclonal gammopathy. Recent concepts have concerned both incidence and mortality rates, and better scoring system for predict outcome in GBS, but new therapeutical strategy is needed for the so-called "benign" forms and for relapsing forms after first-line IVIg therapy. In chronic forms, criteria for diagnosis and guidelines for management have been edited in the recent years, together with recommendations for outcome measures. However, there is still a need for knowing the better outcome measures, and to elaborate new trials, mainly focusing on the long-term management of the patients.

Influence of impaired T- and B-cell compartments on efficacy of IVIg in dysimmune neuropathies.

Autoimmune mechanisms are postulated to play a role in the development and progression of dysimmune neuropathies (DN). We investigated the relation between lymphocyte number and marker expression, and disease activity in 20 patients with DN under intravenous immunoglobulins (IVIg) treatment. B- and T-lymphocyte markers were studied by flow cytometry of the expression of CD5, CD25, CD23 and CD38 markers on B cells and of CD3, CD4 and CD8 markers, respectively. These parameters were compared with those obtained from matched healthy volunteers. The proportions of CD38+ B cells were higher in patients compared with those of controls. Proportions of activated CD4+ and CD8+ T cells were comparable in peripheral blood mononuclear cells of patients and controls, but a significant reduction of the absolute numbers of CD3+, CD4+ and CD8+ cells were observed in DN patients. The percentages of CD25+ memory T cells were instead significantly increased in DN patients. Lastly, T-cell reduction and the CD19/CD38 ratio over total B (CD19+) cells directly correlated with a poor response to IVIg therapy. In DN, whereas T-cell number is reduced, activated T and B cells are increased, thus suggesting an intrinsic defect of the immune response.

Chronic inflammatory demyelinating polyradiculopathy: an atypical pediatric case complicated with phrenic nerve palsy.

An atypical form of chronic inflammatory demyelinating polyneuropathy (CIDP) complicated with phrenic nerve palsy is presented with clinical and electrophysiologic features. A seven-year-old girl had initial presentation mimicking Guillain-Barré syndrome based on electrophysiologic characteristics. Between 7-11 years of age, she had five recurrences of subacute onset of weakness which usually developed over at least 2-4 months and progressed to loss of ambulation and to respiratory insufficiency. Radiologic examinations revealed unilateral phrenic nerve palsy associated with CIDP. Our patient demonstrated the rare association of CIDP and phrenic nerve palsy, resulting in diaphragmatic paralysis and respiratory failure.

[Crohn's disease presenting with recurrent acute polyradiculoneuropathy]. / Polyradiculonévrite à rechutes révélant une maladie de Crohn.

INTRODUCTION: The neurological manifestations of Crohn's disease are rare, dominated by multiple mononeuropathies and the abnormalities of the white matter. Polyradiculoneurities remain exceptional. OBSERVATIONS: We report the case of a 33-year-old patient admitted for an ascending weakness of all four limbs. Eight years earlier he had presented a similar episode which had regressed spontaneously. The neurological examination revealed a tetraparesis with areflexia and hypotonia. These manifestations were concomitant with chronic diarrhea which had been neglected to date. The electrophysiological aspect was compatible with an acute polyradiculoneuritis. The analysis of the cerebrospinal fluid showed an albumino-cytological dissociation. The existence of the diarrhea directed the investigations towards an inflammatory enteropathy, which was attested later on by the endoscopic, radiologic and histological data leading to the diagnosis of active Crohn's disease. The diagnosis of a relapsing polyradiculoneuritis associated with Crohn's disease was retained. The patient was treated by salazopyrine-budesonide with improvement in the digestive and neurological manifestations after 3 years. CONCLUSION: The frequency of neurological features in Crohn's disease is not well documented. The incriminated mechanisms are either directly related to the disease (deficit in B12 vitamin or folic acid and/or by the means of an auto-immune vascularitis) or secondary to long-term treatment with metronidazole. The course of neurological manifestations is largely dependent on the course of the inflammatory disease.