Varicella zoster virus associated-polyradiculoneuritis in an elderly woman: A new subtype of varicella zoster virus neuropathy.

An 82-year-old Japanese woman without underlying disease was admitted to our hospital 3 days after she noticed lower-limb weakness. At presentation, she had lower-leg motor paralysis with mild upper-limb paresis and left Ramsay Hunt syndrome. Cerebrospinal fluid (CSF) findings revealed moderate pleocytosis. A polymerase chain reaction for varicella zoster virus (VZV) DNA in CSF was positive. MRI using 3D Nerve-VIEW (Philips) and contrast T1 images showed high-intensity lesions on the L2-5 and S1-2 spinal roots. A new subtype of VZV-associated polyradiculoneuritis was diagnosed in this patient. We provide the case details and compare three similar reported cases.

[Herpes zoster duplex associated with Ramsay Hunt syndrome and cervical zoster paresis. A case report].

A 63-year-old Japanese female in an immunocompetent state developed right Ramsay Hunt syndrome and left shoulder pain, and left upper limb motor paresis with herpes zoster (HZ) duplex in the right auricle and left shoulder regions. With her Ramsay Hunt syndrome, neural deafness, tinnitus and vestibular symptoms were observed, and she lacked facial nerve palsy. Cerebrospinal fluid (CSF) findings revealed an increase in lymphocytes (21 cells/µl) and protein content (29 mg/dl), and polymerase chain reaction for varicella-zoster virus DNA in CSF was negative. Cervical root MRI using 3D Nerve VIEW (Philips) imaging showed high-intensity lesions on the C5-C8 spinal roots with contrast enhancements. No abnormalities were observed in the median or ulnar motor sensory nerve conduction velocity conduction studies including the F wave. PubMed search revealed no report of a patient with this profile, and to the best of our knowledge HZ duplex with concomitant neurological impairments has not been reported. We compare our present case with several similar cases from the literature.

Polyradiculoneuropathy in dourine-affected horses.

Dourine is an equine protozoan disease caused by Trypanosoma equiperdum. Dourine-afflicted animals die after developing neurological clinical signs, such as unilateral paresis. The disease has been a problem for many years; however, the pathogenesis regarding the neurological clinical signs of dourine has been unclear. In the present study, we conducted a histopathological examination in order to investigate the mechanisms by which dourine-afflicted horses develop the accompanying neurological clinical signs. Four dourine-afflicted horses in Mongolia were evaluated. An apparently healthy horse exhibited multifocal neuritis without axonal or myelin degeneration. The other horses, which had obvious neurological clinical signs, also exhibited multifocal neuritis. In particular, the nerves that innervated areas associated with neurological clinical signs exhibited neuritis with demyelination in the latter horses. Inflamed, non-demyelinating nerves were infiltrated with B lymphocytes and T lymphocytes; while inflamed, demyelinating nerves were infiltrated with mononuclear phagocytes. Our observations revealed lesion progression in the nerves, such that polyradiculoneuropathy could explain the accompanying neurological clinical signs of dourine. To our knowledge, this is the first report to describe a pathogenic mechanism for the development of the neurological clinical signs found in dourine-afflicted horses.

Diffuse large B-cell lymphoma involving peripheral nervous system with IgM antibodies against GM1 and GD1b: A case report.

RATIONALE: The occurrence of peripheral neuropathy associated with non-Hodgkin's lymphoma (NHL) is uncommon. And autoimmunity may play an important role. We report a case of the patient with NHL, has sensorimotor demyelinating polyneuropathy. PATIENT CONCERNS: The patient presented with a 1-month history of progressive numbness at the distal extremities and motor weakness of the lower limbs. Meanwhile, patient also endorsed a painful lump on her right cheek. And then the enlarged cervical and supra clavicular lymph nodes were observed on admission. Biopsy of the lymph nodes showed NHL. Serum IgM antibodies against GM1 and GD1b were also positive. DIAGNOSIS: Biopsy of the lymph nodes showed NHL. Serum IgM antibodies against GM1 and GD1b were also positive. Thus, the patience was diagnosed with lymphoma and sensorimotor polyneuropathy. INTERVENTIONS: Patient refused the further treatment. OUTCOMES: After 11-month follow-up, the weakness of bilateral lower limbs worsens. LESSONS: We have presented a case of NHL involving peripheral polyneuropathy with IgM antibodies against GM1 and GD1b. Patients may initially present with peripheral nerve complications or develop them during the course of lymphoma, even when in remission. This could complicate the diagnosis of peripheral polyneuropathy secondary to NHL.

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.

Erythropoietin reduces nerve demyelination, neuropathic pain behavior and microglial MAPKs activation through erythropoietin receptors on Schwann cells in a rat model of peripheral neuropathy.

Neuroprotective effects of erythropoietin (EPO) on peripheral nerve injury remain uncertain. This study investigated the efficacy of EPO in attenuating median nerve chronic constriction injury (CCI)-induced neuropathy. Animals received an intraneural injection of EPO at doses of 1,000, 3,000, or 5,000 units/kg 15 min before median nerve CCI. Afterwards, the behavioral and electrophysiological tests were conducted. Immunohistochemistry and immunoblotting were used for qualitative and quantitative analysis of microglial and mitogen-activated protein kinases (MAPKs), including p38, JNK, and ERK, activation. Enzyme-linked immunosorbent assay and microdialysis were applied to measure pro-inflammatory cytokine and glutamate responses, respectively. EPO pre-treatment dose-dependently ameliorated neuropathic pain behavior, decreased microglial and MAPKs activation, and diminished the release of pro-inflammatory cytokines and glutamate in the ipsilateral cuneate nucleus after CCI. Moreover, EPO pre-treatment preserved myelination of the injured median nerve on morphological investigation and suppressed injury-induced discharges. We also observed that EPO receptor (EPOR) expression was up-regulated in the injured nerve after CCI. Double immunofluorescence showed that EPOR was localized to Schwann cells. Furthermore, siRNA-mediated knockdown of EPOR expression eliminated the therapeutic effects of EPO on attenuating the microglial and MAPKs activation, pro-inflammatory cytokine responses, injury discharges, and neuropathic pain behavior in CCI rats. In conclusion, binding of EPO to its receptors on Schwann cells maintains myelin integrity and blocks ectopic discharges in the injured median nerve, that in the end contribute to attenuation of neuropathic pain via reducing glutamate release from primary afferents and inhibiting activation of microglial MAPKs and production of pro-inflammatory cytokines.

Myelitis and Polyradiculoneuropathy With Severe Pain: Unusual Neurological Manifestations as Presenting Symptoms of Brucellosis.

Brucellosis, an endemic zoonosis in Portugal, is a multisystem disease, presenting with neurological manifestations in up to 25% of cases. Neurobrucellosis diagnostic criteria include evidence of central nervous system invasion, either by documenting increased blood-brain barrier permeability that normalizes after treatment or by Brucella isolation. We report 2 patients with systemic brucellosis presenting with neurological symptoms: A 28-year-old female with progressive hemiparesis associated with severe refractory thoracic and lumbar pain, whose spinal magnetic resonance imaging identified longitudinally extensive myelitis. Brucella agglutination test was positive in blood; however, cerebrospinal fluid cytochemical, serological testing, and cultures were negative. A 58-year-old male with intermittent fever in the evening, associated with severe refractory cervical and lumbar spinal and radicular pain. Blood workup identified leukocytosis, elevated inflammatory markers and positive Brucella agglutination test. Cerebrospinal fluid presented mild protein increase and negative serological testing and cultures. Electromyogram revealed demyelinating polyradiculoneuropathy. In both cases, antibiotic therapy induced symptom resolution. Despite the neurological presentation, no evidence of direct nervous system infection was found. An indirect mechanism appears to be involved, such as a parainfectious syndrome or circulating endotoxins release by the bacteria. Brucellosis should be considered in patients presenting with inflammatory neurological symptoms in endemic regions. Prompt diagnosis and treatment are important as chronic infection has significant morbidity.

Immunohistochemistry and electrophysiological findings in swine abattoir workers with immune-mediated polyradiculoneuropathy.

IMPORTANCE: Workers exposed to aerosolized brain in a swine-processing plant developed immune-mediated polyradiculoneuropathy (IP) possibly triggered by an immune response. OBJECTIVE: Immunohistochemistry results were correlated with electrophysiological variables to examine the immunopathogenesis of this disorder. DESIGN/SETTING: Laboratory studies used normal nerve tissue that was exposed to sera from 12 IP patients; 10 exposed controls; and 10 unexposed controls. Clinical and electrophysiological data from IP patients were obtained from medical record reviews. MAIN OUTCOME MEASURES: Analysis included electromyography results of IP patients and nerve conduction studies examining CMAP amplitude, distal motor latency, motor conduction velocity, F-wave latency, sensory nerve action potential amplitude, and sensory nerve conduction velocity. Case and control results were compared relative to distance from exposure. RESULTS: Electrodiagnostic findings revealed prolongation of the distal and f-wave latencies suggestive of demyelination at the level of the nerve root and distal nerve terminals. Immunohistochemical results identified an antibody to the peripheral nerve, with staining at the level of the axolemma. Thus, IP may be a primary axonopathy with secondary paranodal demyelination causing the conduction changes. Staining of the distal and proximal portions of the nerve appears consistent with easier access through the blood-nerve barrier. CONCLUSIONS AND RELEVANCE: IP is an immune-mediated neuropathy related to antibodies to an axon-based antigen on peripheral nerves. Secondary paranodal demyelination is likely. Further studies to identify the primary axonal antigenic target would be useful.

[Chronic demyelinating polyradiculoneuropathy revealing a solitary plasmacytoma of the right iliac bone]. / Polyradiculonévrite chronique révélant un plasmocytome solitaire de l'os iliaque droit.

Solitary bone plasmacytoma (pso) is a rare tumor, made of malignant plasma cells observed in a single bone, and without systemic proliferation. We report the case of a 33 year-old man who was admitted with chronic demyelinating sensorimotor polyneuropathy. Immunoelectrophoresis with immunofixation of serum proteins showed a monoclonal peak igg with lambda light chain and the workup was in favour of right hipbone secreting solitary plasmacytoma. The patient was treated with radiotherapy and steroids, and the evolution has been marked by a partial recovery of the motor deficit. An acute demyelinating sensorimotor polyneuropathy has often been described in association with pso. However, subacute and chronic demyelinating polyradiculoneuropathies have rarely been described in such patients with pso.the diagnosis should differentiate pso, poems and multiple myeloma.

Le plasmocytome solitaire osseux (pso) est une tumeur rare, faite de plasmocytes malins qu'on observe dans un seul os, sans atteinte plasmocytaire proliférative systémique. Nous rapportons l'observation clinique d'un patient de 33 ans qui a été admis pour une polyradiculonévrite chronique sensitivo-motrice démyélinisante. L'immunoélectrophorèse avec immunofixation des protéines sériques a révélé un pic monoclonal igg à chaîne légère de type lambda et le bilan étiologique a mis en évidence un plasmocytome solitaire sécrétant de l'os iliaque droit. Le patient a été traité par radiothérapie et corticothérapie, et l'évolution a été marquée par une récupération partielle du déficit moteur. C'est la polyneuropathie sensitivo-motrice démyélinisante aiguë qui a été le plus souvent décrite en association avec le pso alors que les polyradiculoneuropathies démyélinisantes subaiguës ou chroniques ont été rarement rapportées. Le diagnostic différentiel, parfois difficile, doit se faire entre un pso, un poems et un myélome multiple.

Varied immuno-related adverse events induced by immune-check point inhibitors - Nivolumab-associated psoriasiform dermatitis related with increased serum level of interleukin-6.

Nivolumab is a standard recombinant antibody treatment for patients with malignant melanoma (MM), which functions as an immune checkpoint inhibitor by blocking the programmed cell death-1 (PD-1) pathway in T cells. However, it leads to various immune-related adverse events (irAEs), and also exacerbates underlying autoimmune diseases. Herein we report cases of MM with irAE. Case 1: A 69-year-old woman with MM developed destructive thyroiditis resulting in hypothyroidism after 3 doses of nivolumab, and had been treated with thyroid gland auxiliary therapy. Case 2: A 80-year-old man with MM developed an acute onset of hyperthyroidism after 4 doses of nivolumab. Case 3: A 85-year-old woman with MM developed polyradiculoneuropathy resulting in somatosensory disorder and muscle weakness after 2 doses of nivolumab, and had been treated with intravenous immunoglobulin and oral predonisolone (40 mg/day). Case 4: A 77-year-old man with MM developed psoriasiform dermatitis after local injections of IFN-ß and 11 doses of nivolumab. Case 5: Case 2 also developed psoriasiform dermatitis. We analyzed serum levels of inflammatory cytokines in MM patients before/after treatments with nivolumab. All six patients who developed psoriasiform dermatitis with/without anamnesis of psoriasis after treatment with nivolumab, and all seven patients with other irAE exhibited increased serum IL-6 levels after nivolumab treatment, while decreased serum levels of IL-6 were observed in 5 of 7 non-afflicted MM patients. In addition, MM patients who achieved good responses to nivolumab significantly exhibited decreased serum TNF-α levels after nivolumab treatment compared to progressive MM patients.

Therapy with eculizumab for patients with CD59 p.Cys89Tyr mutation.

OBJECTIVE: The objective of this work was to report on the outcome of eculizumab treatment in pediatric patients with recurrent acute predominantly motor, demyelinating neuropathy with conduction block, and chronic hemolysis attributed to p.Cys89Tyr mutation in the CD59 gene. METHODS: Four patients were recruited from our new registry of patients with homozygosity for the p.Cys89Tyr mutation on CD59. Participants received repeated intravenous eculizumab. In this 24-month open-label phase IIa study, we aimed to determine whether eculizumab reduces chronic hemolysis, and cumulative doses of steroids and intravenous immunoglobulin (IVIG), and ameliorates neurological deficits, compared to pretreatment status. Treatment response was evaluated every 2 to 4 weeks over 104 weeks and included examination with gross motor scoring by American Spinal Injury Association Impairment Scale and Inflammatory Neuropathy Cause and Treatment disability score, laboratory examination, well-being [12-item Short Form Health Survey; SF-12]). Neurological relapses and cumulative dose of IVIGs and/or corticosteroids before and after treatment were documented. Red blood cells (RBCs) and neutrophils were stained to evaluate C5b-9 deposition. ClinicalTrials.gov: NCT01579838. RESULTS: Dramatic and significant neurological amelioration in the upper limbs and trunk with more-modest amelioration in the lower limbs was observed in all patients. Corticosteroid and IVIG treatment was completely stopped. No patient relapsed during treatment despite infections, and there were no hospital admissions. Decreased C3bi and C5b-9 deposition on RBCs and neutrophils was documented (p < 0.0001). The SF-12 health questionnaires indicated significant improvement (p < 0.003). INTERPRETATION: Eculizumab was safely administered to these patients. Marked clinical improvement suggests that eculizumab may be a life-saving treatment for patients with acute predominantly motor, demyelinating neuropathy with conduction block, and secondary axonal damage attributed to primary p.Cys89Tyr mutation in the CD59 gene. Ann Neurol 2016;80:708-717.

[Refractory encephalitis because of seronegative celiac disease: a case report]. / Encefalitis refractaria debido a enfermedad celíaca seronegativa: caso clínico.

INTRODUCTION: The celiac disease is an autoimmune bowel disease trigger by gliadine proteins, that can has systemic involvement with varied neurological manifestation since polineuropathy, ataxia, epilepsy, migraine to encephalitis. The aim of the current work is to report a case of possible refractory encephalitis due to celiac disease. CASE REPORT: A 45 years-old man with histopathologic diagnosis of celiac disease but negative celiac antibodies developed encephalic, brainstem and periphery nervous system manifestations: cognitive declaim and seizures, diplopia and ataxia, polyneuropathy and polyradiculopathy respectively; inflammatory cebrospinal fluid and cortico-subcortical and multiple brainstem lesions with mild contrast enhancement; the neurologic deficits progress in spite of gluten a free diet and immuno-suppressive treatment. During the illness process some pathologies with nervous system and intestinal involvement were discarded: sarcoidosis, systemic lupus; Sjogren syndrome, paraneoplastic syndromes, Whipple disease, AIDS and B12 vitamin deficit. CONCLUSION: The encephalitis is an infrequent clinical manifestation of the celiac disease despite the high prevalence of the celiac disease in the general population. It presents with numerous differential diagnosis, with requires high suspicious diagnosis.

TITLE: Encefalitis refractaria debido a enfermedad celiaca seronegativa: caso clinico.Introduccion. La enfermedad celiaca es una enteropatia inducida por el gluten por mecanismos inmunes, que puede presentar afectacion sistemica y producir un amplio espectro de manifestaciones neurologicas, que van desde polineuropatia periferica, ataxia, epilepsia y migraña hasta encefalitis. El objetivo es informar de un caso de posible encefalitis refractaria por enfermedad celiaca. Caso clinico. Varon de 45 años con diagnostico histopatologico de enfermedad celiaca y anticuerpos anticeliaquia negativos que intercurre con encefalitis, manifestaciones del tronco encefalico y del sistema nervioso periferico, entre las que se incluyen deterioro cognitivo y convulsiones, diplopia y ataxia, y polirradiculopatia y polineuropatia, respectivamente; liquido cefalorraquideo inflamatorio y multiples lesiones corticosubcorticales supratentoriales y en el tronco encefalico con leve realce con contraste. Los deficits neurologicos progresan a pesar de una dieta libre de gluten y el tratamiento farmacologico inmunosupresor. Durante el curso de la enfermedad se estudian patologias con afectacion simultanea intestinal y del sistema nervioso, como sarcoidosis, lupus eritematoso sistemico, enfermedad de Sjogren, sindrome paraneoplasico, sida, enfermedad de Whipple y deficit de vitamina B12. Conclusion. La encefalitis es una manifestacion clinica infrecuente en la enfermedad celiaca, con pocos casos notificados a pesar de la alta prevalencia de la enfermedad. Los diagnosticos diferenciales son dificiles y exigen una alta sospecha diagnostica, por lo que es un reto terapeutico.

Acute myeloid leucaemia presenting as a rapidly progressive polyradiculoneuropathy.

Neurological involvement at onset in acute myeloid leucaemia (AML) is rare, with only a few isolated case reports. We present the case of a 46-year-old man with rapidly progressive polyradiculoneuropathy as the presenting feature of AML. The proposed mechanism for this is postulated to be direct intraneural infiltration, although a paraneoplastic, autoimmune-related phenomenon could be possible. Despite chemotherapeutic intervention, the patient died 1 month after initial presentation. Although rare, neurological manifestations of AML do occur and it is important to include haematological malignancies in the differential diagnosis in patients presenting with neurological symptoms.

Primary Neurolymphomatosis Presenting With Polyradiculoneuropathy Affecting One Lower Limb.

OBJECTIVES: We report a case of primary neurolymphomatosis (NL) with unusual presentation and excellent treatment response. METHODS: Chart review. RESULTS: A 64-year-old woman presented with 2 months of progressive pain, weakness, and numbness in her right leg. Nerve conduction study/electromyogram suggested a right lumbosacral radiculoplexus neuropathy with associated acute right peroneal neuropathy at the fibular head. L/S spine and right leg magnetic resonance imaging showed thickening and contrast enhancement of the right S1 nerve root and the right distal sciatic, tibial, and common peroneal nerves, as well as a lobular enhancing lesion of the right superficial peroneal nerve. Whole-body fludeoxyglucose-positron emission tomography scan showed no other lesions. A right superficial peroneal nerve lesion biopsy revealed infiltration of the nerve by diffuse large B-cell lymphoma. The lymphoma cells expressed BCL2 but not CD10, suggesting an origin in peripheral blood not lymph nodes. Despite the expression of BCL2, which is considered as a poor prognosis marker, our patient responded very well to the combined radiotherapy and chemotherapy with the R-MPV (rituximab, MTX, procarbazine, and vincristine) regimen. The patient showed marked clinical improvement and complete resolution of lymphoma lesions on the PET scan. CONCLUSIONS: Our case broadens the clinical spectrum and illustrates the importance of early diagnosis and aggressive treatment of primary NL.