Diagnostic value of F wave changes in patients with Charcot-Marie-Tooth1A and chronic inflammatory demyelinating polyneuropathy / 北京大学学报(医学版)

OBJECTIVE@#To analyze and compare the characteristics and causes of F wave changes in patients with Charcot-Marie-Tooth1A (CMT1A) and chronic inflammatory demyelinating polyneuropathy (CIDP).@*METHODS@#Thirty patients with CMT1A and 30 patients with CIDP were enrolled in Peking University Third Hospital from January 2012 to December 2018. Their clinical data, electrophysiological data(nerve conduction velocity, F wave and H reflex) and neurological function scores were recorded. Some patients underwent magnetic resonance imaging of brachial plexus and lumbar plexus, and the results were analyzed and compared.@*RESULTS@#The average motor conduction velocity (MCV) of median nerve was (21.10±10.60) m/s in CMT1A and (31.52±12.46) m/s in CIDP. There was a significant difference between the two groups (t=-6.75, P < 0.001). About 43.3% (13/30) of the patients with CMT1A did not elicit F wave in ulnar nerve, which was significantly higher than that of the patients with CIDP (4/30, 13.3%), χ2=6.65, P=0.010. Among the patients who could elicit F wave, the latency of F wave in CMT1A group was (52.40±17.56) ms and that in CIDP group was (42.20±12.73) ms. There was a significant difference between the two groups (t=2.96, P=0.006). The occurrence rate of F wave in CMT1A group was 34.6%±39%, and that in CIDP group was 70.7%±15.2%. There was a significant difference between the two groups (t=-5.13, P < 0.001). The MCV of median nerve in a patient with anti neurofascin 155 (NF155) was 23.22 m/s, the latency of F wave was 62.9-70.7 ms, and the occurrence rate was 85%-95%. The proportion of brachial plexus and lumbar plexus thickening in CMT1A was 83.3% (5/6) and 85.7% (6/7), respectively. The proportion of brachial plexus and lumbar plexus thickening in the CIDP patients was only 25.0% (1/4, 2/8). The nerve roots of brachial plexus and lumbar plexus were significantly thickened in a patient with anti NF155 antibody.@*CONCLUSION@#The prolonged latency of F wave in patients with CMT1A reflects the homogenous changes in both proximal and distal peripheral nerves, which can be used as a method to differentiate the CIDP patients characterized by focal demyelinating pathology. Moreover, attention should be paid to differentiate it from the peripheral neuropathy caused by anti NF155 CIDP. Although F wave is often used as an indicator of proximal nerve injury, motor neuron excitability, anterior horn cells, and motor nerve myelin sheath lesions can affect its latency and occurrence rate. F wave abnormalities need to be comprehensively analyzed in combination with the etiology, other electrophysiological results, and MRI imaging.

Controversias en neuroinmunología: esclerosis múltiple, vacunación, SARS-CoV-2 y otros dilemas / Controversies in neuroimmunology: multiple sclerosis, vaccination, SARS-CoV-2 and other dilemas

La neuroinmunología es una disciplina que cada vez amplía más sus horizontes en la comprensión de las enfermedades neurológicas. Contemporáneamente, y a la luz de los nexos fisiopatológicos de las enfermedades neurológicas y la inmunología, se han planteado enfoques diagnósticos y terapéuticos específicos. A pesar de los importantes avances de esta disciplina, existen múltiples dilemas que le conciernen y se filtran en la práctica clínica. En esta revisión, se presentan y discuten 15 controversias, las cuales se construyen con la información clínica disponible más actualizada. Los temas incluidos son: disminución de esteroides en recaídas de esclerosis múltiple; recomendaciones terapéuticas en esclerosis múltiple a la luz de la pandemia por el SARS-CoV-2; evidencia de vacunación en esclerosis múltiple y en otras enfermedades desmielinizantes; panorama actual del síndrome clínico y radiológico aislado; y fallas terapéuticas en esclerosis múltiple; además, criterios para suspender las terapias modificadoras de la enfermedad; evidencia del manejo en recaídas leves; recomendaciones para la profilaxis contra Strongyloides stercolaris; utilidad de un segundo ciclo de inmunoglobulina en el síndrome de Guillain-Barré; criterios para diferenciar una polineuropatía crónica desmielinizante inflamatoria de inicio agudo de un síndrome de Guillain-Barré y, utilidad de la enzima convertidora de angiotensina en neurosarcoidosis. En cada una de las controversias, se presenta la problemática general y se ofrecen recomendaciones específicas que pueden adoptarse en la práctica clínica diaria.

Neuroimmunology is a discipline that increasingly broadens its horizons in the understanding of neurological diseases. At the same time, and in front of the pathophysiological links of neurological diseases and immunology, specific diagnostic and therapeutic approaches have been proposed. Despite the important advances in this discipline, there are multiple dilemmas that concern and filter into clinical practice. This article presents 15 controversies and a discussion about them, which are built with the most up-to-date evidence available. The topics included in this review are: steroid decline in relapses of multiple sclerosis; therapeutic recommendations in MS in light of the SARS-CoV-2 pandemic; evidence of vaccination in multiple sclerosis and other demyelinating diseases; overview current situation of isolated clinical and radiological syndrome; therapeutic failure in multiple sclerosis, as well as criteria for suspension of disease-modifying therapies; evidence of the management of mild relapses in multiple sclerosis; recommendations for prophylaxis against Strongyloides stercolaris; usefulness of a second course of immunoglobulin in the Guillain-Barré syndrome; criteria to differentiate an acute-onset inflammatory demyelinating chronic polyneuropathy versus Guillain-Barré syndrome; and, the utility of angiotensin-converting enzyme in neurosarcoidosis. In each of the controversies, the general problem is presented, and specific recommendations are offered that can be adopted in daily clinical practice.

Eficacia de la movilización de células madre en pacientes con Polineuropatía Desmielinizante Inflamatoria Crónica refractaria / Efficacy of mobilization of stem cells in patients with Refractory Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Introducción: La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad desmielinizante e inflamatoria de mediación autoinmune. El tratamiento convencional es basado en la inmunomodulación e inmunosupresión. El uso de células madre es una terapia novedosa en los trastornos autoinmune, siendo incluida como terapia. Objetivo: Determinar la eficacia de la movilización de células madre mediante la aplicación del factor estimulador de colonias granulocíticas (F-ECG) en pacientes con CIDP que han recibido otras líneas de tratamiento. Material y Métodos: Se realizó un estudio aleatorizado, doble ciego sobre una cohorte de 45 pacientes con CIDP, donde se administró el (F-ECG) en 25 pacientes y 20 continuaron con el tratamiento habitual, tratados anteriormente con otras variantes terapéuticas por más de tres años, sin respuesta satisfactoria. Resultados: Predominio de los hombres para 64,4 por ciento, la Diabetes Mellitus tuvo mayor asociación y la medicación más usada fueron los esteroides. Los síntomas y signos clínicos mejoraron significativamente tras el tratamiento. Los valores de la puntuación del TCSS al mes y 3 meses después del tratamiento disminuyeron significativamente; pero este decremento no se mantuvo al final del estudio. La velocidad de conducción y el potencial de acción de los nervios sensoriales y motores mejoraron considerablemente después del tratamiento. Conclusiones: La efectividad de la aplicación del (F-ECG) para la mejoría de los síntomas clínicos y resultados de estudios neurofisiológicos evolutivamente son mayores que otras variantes terapéuticas en los primeros meses, con buena seguridad y tolerabilidad, por lo que se puede incluir en la terapéutica convencional para la CIDP(AU)

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune demyelinating disease. Conventional treatment is based on immunomodulation and immunosuppression. The use of stem cells is a novel therapy in autoimmune disorders, so it is included as therapy. Objective: To determine the efficacy of mobilization of stem cells by applying granulocyte colony-stimulating factor (G-CSF) in patients with CIDP who have followed other lines of treatment. Material and Methods: A randomized, double-blind study was carried out on a cohort of 45 patients with CIDP. G-CSF was administered to 25 patients and 20 of them continued with the usual treatment. These patients were previously treated with other therapeutic variants for more than three years without satisfactory response. Results: There was a prevalence of men (64.4 percent), Diabetes Mellitus had a greater association, and the most used medications were steroids. Clinical symptoms and signs improved significantly after treatment. TCSS scores significantly decreased at one and three months after treatment, but this decrease was not maintained at the end of the study. The conduction velocity and action potential of sensory and motor nerves improved considerably after treatment. Conclusions: The effectiveness of the use of G-CSF shows an improvement of clinical symptoms. The results of neurophysiological studies have a better course than other therapeutic variants during the first months, with good safety and tolerability, so it can be included in the conventional therapy for the CIDP(AU)

Diagnostic challenges in systemic amyloidosis: a case report with clinical and laboratorial pitfalls

Currently, there is growing evidence in the literature warning of misdiagnosis involving amyloidosis and chronic inflammatory demyelinating polyneuropathy (CIDP). Although inducing clinical manifestations outside the peripheral nervous system, light chain and transthyretin amyloidosis may initially present with peripheral neuropathy, which can be indistinguishable from CIDP, leading to a delay in the correct diagnosis. Besides, the precise identification of the amyloid subtype is often challenging. This case report exemplifies clinical and laboratory pitfalls in diagnosing amyloidosis and subtyping amyloid, exposing the patient to potentially harmful procedures.

A comparison of IL-17 and IL-34 concentrations in the cerebrospinal fluid of patients with acute inflammatory demyelinating neuropathy and chronic inflammatory demyelinating polyneuropathy

SUMMARY OBJECTIVE: The role of interleukins, such as IL-17 and IL-34, in the pathogenesis of autoimmune diseases has been established in the literature. In the current study, we aimed to identify the concentrations of IL-17 (IL-17A, IL-17F) and IL-34 in the cerebrospinal fluid (CSF) of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and acute inflammatory demyelinating neuropathy (AIDN). METHODS: We included in this study 8 patients with CIDP (none of them receiving immunomodulatory or immunosuppressant therapy), 7 patients with Guillain-Barre syndrome (GBS, AIDN), and 7 control subjects. The CIDP and AIDN diagnoses were made by clinical evaluation and electrophysiological investigations according to international criteria. CSF samples were obtained appropriately, and the levels of IL-17A, IL-17F, and IL-34 were measured by ELISA kits. RESULTS: The concentrations of IL-17A, IL-17F, and IL-34 were higher in those with CIDP and AIDN compared to the controls (p=0.005, p=0.01, and p=0.001, respectively). While IL-34 levels were significantly higher in AIDN patients than in CIDP patients (p=0.04), there were no significant differences between the AIDN and CIDP groups with regard to the levels of IL-17A and IL-17F (p=0.4 and p=0.2, respectively) CONCLUSION: Our results indicate that IL-17A, IL-17F, and IL-34 levels may have a role in CIDP and AIDN. Furthermore, the difference in the IL-34 levels of patients with AIDN and CIDP may indicate an important difference between the pathogenesis of these two sets of the disease.

RESUMO OBJETIVO: O papel das interleucinas, como IL-17 e IL-34, na patogênese da doença auto-imune foi estabelecido na literatura. No presente estudo, objetivamos identificar as concentrações de IL-17 (IL-17A, IL-17F) e IL-34 no líquido cefalorraquidiano (LCR) de pacientes com polineuropatia desmielinizante inflamatória crônica (CIDP) e neuropatia desmielinizante inflamatória aguda (AIDN). MÉTODOS: incluímos neste estudo 8 pacientes com CIDP (nenhum deles recebendo terapia imunomoduladora ou imunossupressora), 7 pacientes com síndrome de Guillain-Barre (GBS, AIDN) e 7 indivíduos controle. Os diagnósticos CIDP e AIDN foram feitos por avaliação clínica e investigações eletrofisiológicas de acordo com critérios internacionais. As amostras de LCR foram obtidas adequadamente e os níveis de IL-17A, IL-17F e IL-34 foram medidos através de kits ELISA. RESULTADOS: As concentrações de IL-17A, IL-17F e IL-34 foram maiores naqueles com CIDP e AIDN em comparação aos controles (p = 0,005, p = 0,01 ep = 0,001, respectivamente). Enquanto os níveis de IL-34 foram significativamente mais altos nos pacientes com AIDN do que nos pacientes com CIDP (p = 0,04), não houve diferenças significativas entre os grupos com AIDN e CIDP em relação aos níveis de IL-17A e IL-17F (p = 0,4 ep = 0,2, respectivamente) CONCLUSÃO: Nossos resultados indicam que os níveis de IL-17A, IL-17F e IL-34 podem ter um papel no CIDP e no AIDN. Além disso, a diferença nos níveis de IL-34 de pacientes com AIDN e CIDP pode indicar uma diferença importante entre a patogênese desses dois conjuntos de doenças.

Factores asociados a respuesta favorable de tratamiento corticoidal en pacientes con polirradiculoneuropatía desmielinizante inflamatoria crónica / Variables associated with a good response to steroids in chronic inflammatory demyelinating polyradiculoneuropathy

Background: The treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on corticosteroids, immunoglobulin and plasmapheresis. In our Health System, corticosteroids are commonly used as first line therapy for economic reasons and accessibility. However, the factors associated with a good response are not well known. Aim: To assess the association of demographic, clinical and laboratory variables with a favorable response to corticosteroid therapy in patients with CIDP. Material and Methods: Observational, descriptive, longitudinal and retrospective study of 33 patients with a diagnosis of typical, definitive or probable CIDP, treated with corticosteroids for at least six months. Results: Twenty-three patients had a good clinical response to corticosteroid treatment and 10 were non-responders. The variables significantly associated with a good response to steroids were a disease lasting less than 1 year prior to the start of treatment, the absence of axonal damage in electromyography a relapsing-recurrent course and a favorable response within two months of treatment. Conclusions: Most of these patients with CIDP had good response to corticosteroid therapy.

Different distributions of nerve demyelination in chronic acquired multifocal polyneuropathies / 中华医学杂志(英文版)

BACKGROUND@#Multifocal motor neuropathy (MMN), Lewis-Sumner syndrome (LSS), and many chronic inflammatory demyelinating polyradiculoneuropathies (CIDPs) are representative of acquired multifocal polyneuropathy and are characterized by conduction block (CB). This retrospective study aimed to investigate the demyelinating distribution and the selective vulnerability of MMN, LSS, and CIDP with CB (CIDP-CB) in nerves.@*METHODS@#Fifteen LSS subjects (107 nerves), 24 MMN subjects (176 nerves), and 17 CIDP-CB subjects (110 nerves) were included. Their clinical information was recorded, blood and cerebrospinal fluid tests were conducted, and nerve conductions of the median, ulnar, radial, peroneal, and tibial nerves were evaluated. CB, temporal dispersion, distal motor latency (DML), and F-wave latency were recorded, and nerve conduction velocity, terminal latency index, and modified F-wave ratio were calculated.@*RESULTS@#CB was more likely to occur around the elbow in CIDP-CB than in MMN (78.6% vs. 6.8%, P < 0.01) but less likely to occur between the wrist and the elbow than in LSS (10.7% vs. 39.3%, P < 0.05). Tibial nerve CB was most frequently observed in MMN (47.4%, P < 0.05). CIDP-CB was characterized by a prolonged DML in all nerves, and slow motor nerve velocity of the upper limb was significant when CB nerves were excluded (P < 0.05).@*CONCLUSIONS@#We report the different distributions of segmental and diffuse demyelination of the ulnar and tibial nerves in LSS, MMN, and CIDP-CB. These distinct distributions could help in differentiating among these conditions.

Anesthesitic management for insulinoma resection in a patient having chronic inflammatory demyelinating neuropathy / Manejo anestésico para resección de insulinoma en un paciente portador de neuropatía desmielinizante crónica

Chronic Inflammatory Demyelinating Polyneuropathy associated with hypoglycemia 2 to insulinoma is unusual, and to our knowledge, very few patients have been reported in literature. Despite varying presentations in these patients, the clinical characteristics are usually the same. The syndrome usually occurs after several episodes of protracted hypoglycemia. The neuropathy is nearly always symmetrical. We report anesthetic management for a young female patient presenting with CIDP & repeated hypoglycemic episodes during a 2-year period scheduled for insulinoma enucleation.

La polineuropatía desmielinizante inflamatoria crónica asociada con hipoglicemia secundaria a insulinoma es inusual y, hasta donde sabemos, muy pocos pacientes han sido reportados en la literatura. A pesar de las diferentes presentaciones en estos pacientes, las características clínicas suelen ser las mismas. El síndrome generalmente ocurre después de varios episodios de hipoglicemia prolongada. La neuropatía es casi siempre simétrica. Presentamos el manejo anestésico para una paciente joven que se presenta con polineuropatía desmielinizante inflamatoria crónica y episodios repetidos de hipoglicemia durante un período de 2 años programado para la enucleación de insulinoma.

Interpretation of Electrodiagnostic Tests in Chronic Inflammatory Demyelinating Polyneuropathy: Classification Using Nerve Conduction Study / 대한신경근육질환학회지

Electrodiagnostic tests (EDX) is essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). EDX could provide information about demyelinating pathology in the peripheral nerves. According to phenotypes, CIDP could be classified several phenotypes, which has different clinical manifestations, EDX could present a different distribution pattern of demyelinating lesions. In addition, EDX could be useful markers for predicting treatment response of prognosis of CIDP.

Síndrome POEMS: um diagnóstico desafiador / POEMS Syndrome: a challenging diagnosis

A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)

POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)

Polineuropatía desmielinizante inflamatoria crónica en paciente diabética con sífilis / Chronic inflammatory demyelinating polyneuropathy in a diabetic patient with syphilis

La polineuropatía desmielinizante inflamatoria crónica (PDIC) se presenta generalmente con síntomas motores, debilidad tanto en los músculos proximales como en los distales con reflejos globalmente disminuidos o ausentes. La neuritis insulínica es un trastorno caracterizado por dolor agudo de las extremidades, y daño de los nervios periféricos con afectación predominante de las fibras pequeñas, en los pacientes diabéticos sometidos a un rápido control glucémico. El dolor es raro en la PDIC clásica. Describimos el caso de una mujer de 54 años con diabetes mellitus (DB) tipo II, en tratamiento reciente con insulina, que consultó por un cuadro de debilidad e hiperalgesia de los cuatro miembros de dos meses de evolución. Al examen físico presentaba dolor de intensidad 10/10 y alodinia en los cuatro miembros, a niveles proximal y distal, con fuerza muscular reducida de los músculos proximales y arreflexia patelar y aquilea bilateral. Se realizó un estudio electrofisiológico, el cual mostró una polineuropatía sensitiva y motora desmielinizante. Se indicó tratamiento con inmunoglobina humana recombinante, con total remisión del cuadro. Estudios realizados posteriormente demostraron positividad débil de los anticuerpos GM1, GD1a, GD1b y anti-asialo GM1. Previo al alta hospitalaria se recibieron los resultados de VDRL sérica positiva, y FTA-Abs. VDRL en líquido cefalorraquídeo fue negativa por lo que se descartó neurosífilis, indicándose tratamiento con penicilina benzatínica.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder characterized by motor symptoms such as weakness in both proximal and distal muscles with globally diminished or absent reflexes. Insulin neuritis is referred as an acute pain in the extremities, due to the damage of peripheral nerves affecting mainly small fibers, in diabetic patients treated with insulin who achieved rapid glycemic control. Pain is unusual in classic CIDP. We report the case of a 54-year-old female patient with type II diabetes mellitus, and a recent onset of insulin therapy, who presented at the emergency room with a 2-month history of weakness and hyperalgesia of extremities. Physical examination showed marked pain and proximal and distal allodynia in the 4 limbs, with reduced muscle strength of the proximal muscles and patellar and achillear areflexia. Electrophysiological study showed sensory and motor polyneuropathy with a demyelinating predominance. Treatment with recombinant human immunoglobin was started, and the patient presented a total remission of the condition. Complementary studies confirmed weak serum positivity of GM1, GD1a, GD1b and anti-asialo GM1. Prior to hospital discharge, results of positive serum VDRL and FTA-Abs were received. VDRL in cerebrospinal fluid was negative, so neurosyphilis was ruled out, and treatment with benzathine penicillin was indicated.

Intravenous Immunoglobulin in the Treatment of Neurological Diseases / 대한소아신경학회지

Intravenous immunoglobulin (IVIG) is used in treating many cases of autoimmune and inflammatory conditions thanks to its multiple anti-inflammatory and immunomodulatory properties. The clinical use of IVIG has been for the patients with primary immunodeficiencies, but lately it is expanding its usage to the realms of treating patients with neurological conditions. Both the efficacy and safety of IVIG treatment in chronic inflammatory demyelinating polyradiculoneuropathy and Guillain–Barré syndrome have been studied successfully. However, the use of IVIG treatment in other neurological conditions still remains investigational despite several successful reports. Considerable numbers of mechanisms have been suggested in order to explain the effects of IVIG, but the exact mechanisms are not understood yet. This review covers the new developments in clinical fields and the possible ways in which IVIG could help in the future.

Role of short-latency somatosensory evoked potential in the diagnosis of chronic inflammatory demyelinating polyneuropathy / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the role of short-latency somatosensory evoked potential (SSEP) in the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).</p><p><b>METHODS</b>A total of 48 children with a confirmed or suspected CIDP and 40 healthy children were enrolled. Nerve electrophysiological examination and/or SSEP examination was performed (the children in the healthy control group only underwent SSEP examination). Four-lead electromyography was used for nerve electrophysiological examination, including at least 4 motor nerves and 2 sensory nerves. N6 (elbow potential), N13 (cervical cord potential), and N20 (cortex potential) of the median nerve and N8 (popliteal fossa potential), N22 (lumbar cord potential), and P39 (cortex potential) of the tibial nerve were observed by SSEP examination.</p><p><b>RESULTS</b>Among the 48 children with CIDP, 35 had demyelination in both motor and sensory nerves, 8 had demyelination in sensory nerves, and 5 had axonal degeneration. SSEP examination showed that 7 had conduction abnormality in the trunk of the brachial plexus and/or the posterior root and 33 had damage in the lumbosacral plexus and/or the posterior root. The 40 children with abnormal findings of SSEP examination included 8 children with affected sensory nerves and 5 children with secondary axonal degeneration who did not meet the electrophysiological diagnostic criteria for CIDP. Compared with the healthy control group, the CIDP group had significantly prolonged latency periods of N13 and N22 (P<0.05).</p><p><b>CONCLUSIONS</b>SSEP can be used for the auxiliary diagnosis of CIDP, especially in CIDP children with affected sensory nerves or secondary axonal degeneration.</p>

Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Membranous Glomerulonephritis and Tendinitis

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated progressive or relapsing demyelinating peripheral neuropathy. Other autoimmune diseases may be associated with CIDP. A 38-year-old man developed CIDP, which was subsequently associated with membranous glomerulonephritis (MGN) and tendinitis. He was treated with intravenous immunoglobulin, rituximab, and prednisone, which resulted in improvement of the clinical symptoms. This is a case report of CIDP associated with MGN and tendinitis.

Inmunoglobulina subcutánea: Tratamiento en polineuropatía desmielinizante inflamatoria crónica / Subcutaneous immunoglobulin. Treatment in chronic inflammatory demyelinating polyradiculo-neuropathy

La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad adquirida que puede afectar a raíces, plexos y nervios periféricos. A pesar de su baja incidencia, su diagnóstico cobra especial relevancia dado que actualmente existen tratamientos efectivos para la misma. La gammaglobulina humana endovenosa (IVIgG) es, junto con los esteroides y la plasmaféresis, uno de los tratamientos de primera elección. La vía de administración subcutánea se ha propuesto como una alternativa novedosa frente a la administración endovenosa con una eficacia similar. Presentamos tres casos de CIDP definitiva, clasificados según los criterios de la European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) en los cuales se utilizó tratamiento crónico con inmunoglobulina subcutánea (IgSC). Todos ellos habían recibido tratamiento previo con IVIgG. Se obtuvo mejoría de la fuerza evaluada por Overall Neuropathy Limitations Scale (ONLS) y los tres pacientes manifestaron una mejor adaptación a sus actividades de la vida diaria.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disease that may affect nerve roots and peripheral nerves. Despite its low incidence, diagnosis is particularly important because there are different effective treatments. Human immunoglobulin is one of the mainstays of the treatment. Although there are few studies up to date, subcutaneous immunoglobulin (IgSC) has been proposed as an alternative to intravenous administration with similar efficacy. We present three cases with definite CIDP, classified according to the European Federation of Neurological Societies / Peripheral Nerve, Society (EFNS /PNS) criteria in which was used SCIgG as a treatment after success with the intravenous route. The Overall Neuropathy Limitations Scale (ONLS) was used to estimate the changes in the muscular strength before and after treatment.

Polineuropatía asociada a infección por VIH. Revisión del tema ypresentación de un caso / Polyneuropathy associated to VIH infection. Review of the topic and case report

La infección por el virus de inmunodeficiencia humana (VIH) constituye un problema de salud pública. Laafectación neurológica en los pacientes infectados por el VIH es frecuente, involucrando tanto al sistema nerviosocentral como al periférico, y en algunos casos puede ser la primera manifestación de la infección. Entrelas afecciones neurológicas, las neuropatías periféricas pueden observarse en el 100% de las autopsias. Lasmismas pueden adoptar diferentes formas, que por lo general dependen de la fase de la enfermedad en la quese encuentre el paciente. Las neuropatías autoinmunes como el síndrome de Guillain-Barré y la polineuropatíadesmielinizante inflamatoria crónica (CIDP) aparecen en los estadios iniciales de la infección, cuando el conteode CD4 está ligeramente disminuido. La CIDP tiene criterios clínicos y electrofisiológicos bien definidos quela diferencian de otras formas de neuropatías periféricas, responde bien al tratamiento inmunomodulador,pero su diagnóstico puede ser difícil de realizar debido a su forma insidiosa de comienzo. Se realiza una breverevisión de las neuropatías periféricas que pueden asociarse a la infección por VIH y se presenta un caso deasociación de esta infección con CIDP.

Infection by Human Immune deficiency (VIH) is a public health problem. Neurological affection in those patients is frequent, it involve central and peripheral nervous system. In some cases neurological involvement is the first sign of the infection. Peripheral neuropathies are the most common of neurological illness associated to VIH infection; it could be observed in 100 % of autopsy. Autoimmune neuropathies like Guillain Barré and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) appear at initial phase of infection, when CD4 count is slight diminish. CIDP has defined clinic and electrophysiological criteria to differentiate it from other types of neuropathies, CIDP has a good response to immunomodulation treatment, it has an insidious start, which could difficult the diagnostic. We show a brief revision of peripheral neuropathic associated to VIH infection; we show a case with VIH infection and CIDP.

Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses / 소아과

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-Barre syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy Mimicking Miller-Fisher Syndrome

A 77-year-old man developed diplopia, gait ataxia, and paresthesia. A clinical examination also revealed ophthalmoplegia, facial palsy, ataxia of the limbs and trunk, and reduced deep tender reflexes. Laboratory and electrophysiological studies revealed albuminocytological dissociation and demyelination. He was diagnosed as Miller-Fisher syndrome and received intravenous immunoglobulin therapy. His clinical symptoms deteriorated at 12 weeks after onset. We diagnosed acute-onset chronic inflammatory demyelinating polyradiculoneuropathy, and which the patient recovered from following corticosteroid therapy.

Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy Mimicking Miller-Fisher Syndrome

A 77-year-old man developed diplopia, gait ataxia, and paresthesia. A clinical examination also revealed ophthalmoplegia, facial palsy, ataxia of the limbs and trunk, and reduced deep tender reflexes. Laboratory and electrophysiological studies revealed albuminocytological dissociation and demyelination. He was diagnosed as Miller-Fisher syndrome and received intravenous immunoglobulin therapy. His clinical symptoms deteriorated at 12 weeks after onset. We diagnosed acute-onset chronic inflammatory demyelinating polyradiculoneuropathy, and which the patient recovered from following corticosteroid therapy.

Chronic inflammatory demyelinating polyneuropathy: quality of life, sociodemographic profile and physical complaints / Polineuropatia inflamatoria desmielinizante cronica: qualidade de vida, perfil sociodemografico e queixas fisicas

Whereas an evaluation of quality of life and possible impacts on the mental state of a patient may help to evaluate the evolution of chronic inflammatory demyelinating polyneuropathy (CIDP), the aim of this study was to study the psychological profile of patients, and evaluate quality of life associated with the disease. Method 41 patients were evaluated using a Mini-Mental State Examination (MMSE) and a Short-Form Health Survey (SF-36). Results The mean age of the patients was 50.6 years, 63.4% men. Of the participants, 65.9% had other health problems, 39% reported needing help with activities of daily living, 49% slept less than 8 hours per night, and 34.1% complained of some memory deficit. The average MMSE score was 26. Impairment of functional capacity and pain were the more important altered health states. Conclusion CIDP has important social and economic impacts, owing to functional impairments that can lead to professional and personal limitations. .

A avaliação da qualidade de vida (QV) e dos possíveis impactos dos déficits funcionais sobre o estado mental de pacientes com polirradiculoneuropatia inflamatória desmielinizante crônica (PIDC) pode contribuir para a melhor compreensão de aspectos evolutivos da doença. A presente investigação teve como objetivo estudar as atividades da vida diária depacientes com PIDC e avaliar a sua QV. Método Foram avaliados 41 pacientes através do Mini Exame do Estado Mental (MEEM) e do inventário de saúde SF-36®. Resultados A média de idade dos participantes foi 50,6 anos, 63,4% homens. Problemas adicionais de saúde foram referidos por 65,9%: 39% relataram necessitar de ajuda para atividades de vida diária, 49% dormiam menos de 8 horas por noite e 34,1% referiam alguma dificuldade de memória. A média do MEEM foi 26. Através do SF-36 foi verificado maior prejuízo na capacidade funcional; a referência a dor foi proeminente. Conclusão A PIDC pode ter importante impacto social e econômico em decorrência dos prejuízos funcionais primários e secundários que podem levar ao afastamento do trabalho. .