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Abstract Background Studies were carried out with the objective of evaluating the quality of life (QoL) of patients affected by chronic inflammatory demyelinating polyradiculopathy (CIDP). However, the cognitive issue is still little addressed. Objectives To assess the QoL and cognitive impairment of patients with CIDP and to analyze whether there is a correlation between these parameters. Methods Seven patients with CIDP and seven paired controls were subjected to: mini-mental state examination (MMSE); Montreal cognitive assessment (MoCA); digit symbol replacement/symbol copy test (DSST); fatigue severity scale (FSS); Beck depressive inventory-I (BDI-I), and a short-form of health survey (SF-36). Results The mean age of the participants was 50 years (71.4% male). The MMSE and MoCA had no statistical difference between the groups. Patients showed superior results in the memory domain in the MoCA (5 vs. 2, p = 0.013). In the DSST, we observed a tendency for patients to be slower. There was a strong negative correlation between fatigue levels and vitality domain (SF-36). There was no significant correlation between depression levels and QoL, and there was no correlation between depression and the results obtained in the cognitive tests. The patients presented higher levels of depression (15.28 vs. 3.42, p < 0.001). A total of 57% had severe fatigue, 28.8% self-reported pain, and 57.1% complained of cramps. Conclusion There was no cognitive impairment in these patients. However, there was a tendency of slower processing speed. To better evaluate the alterations found, a study with a larger number of individuals would be necessary. Chronic inflammatory demyelinating polyradiculopathy affects the QoL of patients in different ways.
Resumo Antecedentes Estudos foram realizados com o objetivo de avaliar a qualidade de vida (QV) de pacientes acometidos pela polirradiculopatia desmielinizante inflamatória crônica (PDIC). No entanto, a questão cognitiva ainda é pouco abordada. Objetivos Avaliar a QV e o comprometimento cognitivo em pacientes com PDIC bem como se existe correlação entre esses parâmetros. Métodos Sete pacientes com PDIC e sete controles pareados foram submetidos a: miniexame do estado mental (MEEM); avaliação cognitiva de Montreal (MoCA); teste de substituição de símbolo de dígito/cópia de símbolo (DSST); escala de gravidade da fadiga (FSS);Beck depressive inventory-I (BDI-I) e um short-form of health survey (SF-36). Resultados A média de idade dos pacientes foi de 50 anos (71,4% do sexo masculino). O MMSE e o MoCA não apresentaram diferença estatística entre os grupos. Os pacientes apresentaram resultados superiores no domínio memória do MoCA (5 vs. 2, p = 0,013). No DSST, observamos uma tendência de os pacientes serem mais lentos. Houve forte correlação negativa entre os níveis de fadiga e o domínio vitalidade (SF-36). Não houve correlação significativa entre níveis de depressão e QV. Não houve correlação entre depressão e os resultados obtidos nos testes cognitivos. Níveis elevados de depressão foram observados nos pacientes (15,28 vs. 3,42, p < 0,001). Um total de 57% apresentou fadiga intensa, 28,8% dor autorreferida, e 57,1% queixam-se de câimbras. Conclusão Não há comprometimento cognitivo nos pacientes estudados. Observamos somente uma tendência de lentificação na velocidade de processamento. Para melhor avaliar as alterações encontradas, será necessário estudo com um número maior de indivíduos. A PDIC afeta de diferentes formas o nível de QV de seus portadores.
ABSTRACT
Introducción: Dentro de los fenotipos de polineuropatía desmielinizante inflamatoria crónica (CIDP) existe uno cuyo tiempo de evolución es menor de ocho semanas desde el inicio de los síntomas, denominado de inicio agudo (A-CIDP). Esta entidad puede confundirse con el síndrome de Guillain-Barré, variedad desmielinizante inflamatoria aguda (AIDP), lo que retrasa el inicio del tratamiento. Objetivo: Analizar las diferencias clínicas y electrofisiológicas entre A-CIDP, CIDP clásica y AIDP, con el fin de identificar factores que auxilien al diagnóstico diferencial de forma temprana. Pacientes y métodos: Se realizó un estudio transversal con pacientes atendidos en la clínica de enfermedades neuromusculares del Instituto Nacional de Neurología y Neurocirugía con diagnóstico de CIDP según criterios de la European Federation of Neurological Societies and Peripheral Nerve Society. Los pacientes con CIDP <8 semanas se catalogaron como A-CIDP y fueron comparados con pacientes diagnosticados con CIDP clásica y AIDP. Se obtuvieron y analizaron variables clínicas, paraclínicas y electrofisiológicas. Resultados: Se observaron diferencias significativas en antecedente de infección, afección de nervios del cráneo y disautonomías entre la A-CIDP y la AIDP. Los registros electrofisiológicos describieron diferencias significativas en velocidad de conducción de los nervios motores y en los registros del nervio sural, que fueron menores en el grupo de A-CIDP. Conclusión: El antecedente de infección, la afección de nervios del cráneo y las disautonomías son parámetros importantes que se debe tener en cuenta para el diagnóstico diferencial de estas entidades. El análisis electrofisiológico es similar entre la A-CIDP y la CIDP. El diagnóstico diferencial entre estos tipos de polineuropatía desmielinizante debe basarse en el juicio clínico.(AU)
Introduction: The phenotypes of chronic inflammatory demyelinating polyneuropathy (CIDP) include an acute-onset phenotype (A-CIDP) with an evolution time of less than eight weeks from the onset of symptoms. This entity can be confused with Guillain-Barré syndrome of the acute inflammatory demyelinating variety (AIDP), delaying the start of treatment. Objective: To analyze the clinical and electrophysiological differences between A-CIDP, classic CIDP and AIDP, in order to identify factors that may help in the early differential diagnosis. Patients and methods: A cross-sectional study was carried out with patients seen at the neuromuscular disease clinic of the National Institute of Neurology and Neurosurgery with a diagnosis of CIDP according to the criteria of the European Federation of Neurological Societies and Peripheral Nerve Society. Patients with CIDP <8 weeks were categorized as A-CIDP and were compared with patients diagnosed with classic CIDP and AIDP. Clinical, paraclinical and electrophysiological variables were obtained and analyzed. Results: Significant differences in history of infection, cranial nerve involvement and dysautonomia were observed between A-CIDP and AIDP. Electrophysiological recordings reported significant differences in motor nerve conduction velocity and sural nerve recordings, being lower in the A-CIDP group. Conclusion: A history of infection, cranial nerve involvement and dysautonomia are important parameters to take into account for the differential diagnosis of these entities. Electrophysiological analysis is similar between A-CIDP and CIDP. The differential diagnosis between these types of demyelinating polyneuropathy must be based on clinical assessment.(AU)
Subject(s)
Guillain-Barre Syndrome , Neurophysiology , Neuromuscular Diseases , Prognosis , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Neurology , Nervous System Diseases , Cross-Sectional StudiesABSTRACT
Abstract Background There is a lack of evidence of cognitive involvement in chronic inflammatory demyelinating polyneuropathy (CIDP) and, the reports about the involvement of the brain and central nervous system (CNS) are few and controversial. The Five Digit Test (FDT) evaluates processing speed (PS) and executive functions orally. Objective To evaluate the performance on the FDT of CIDP patients with and without CNS (brain/cerebellum) alterations observed on brain Magnetic Resonance Imaging (MRI) scans. Methods The Hospital Anxiety and Depression Scale (HADS, to assess neuropsychiatry symptoms), the Rasch-built Overall Disability Scale (R-ODS; to assess disability), and the FDT (to assess cognition) were applied to 14 CIDP patients and 24 age-matched healthy control subjects. The patients were submitted to routine brain MRI and, according to the results, they were divided into two groups: those with abnormalities on the MRI (CIDPabnl) and those with normal parameters on the MRI (CIDPnl). The FDT data of five CIDPnl patients and nine CIDPabnl subjects were analyzed. Comparisons between the groups were performed for each task of the FDT. Results We found statistical differences for both groups of CIDP patients in terms of PS, for the patients spent more time performing the PS tasks than the controls. The PS measures were negatively associated with disability scores (reading: r = −0.47; p = 0.003; counting: r = −0.53; p = 0.001). Conclusions Our data suggested the presence of PS impairment in CIDP patients. Disability was associated with slow PS.
Resumo Antecedentes Faltam evidências de envolvimento cognitivo na polineuropatia inflamatória desmielinizante crônica (PIDC), e há poucos e controversos estudos que tratam do envolvimento cerebral e do sistema nervoso central (SNC). O Teste dos Cinco Dígitos (Five Digit Test, FDT, em inglês) avalia a velocidade de processamento (VP) e as funções executivas oralmente. Objetivo Avaliar o desempenho no FDT de pacientes com PIDC com e sem alterações no SNC (cérebro/cerebelo) de acordo com o exame de imagem cerebral por ressonância magnética (RM). Métodos Ao todo, 14 pacientes e 24 controles saudáveis pareados por idade responderam a Escala Hospitalar de Ansiedade e Depressão (que avalia sintomas neuropsiquiátricos), a Escala de Incapacidade Geral elaborada pelo método Rasch (que avalia a incapacidade) e o FDT (que avalia a cognição). Os pacientes foram submetidos a RM cerebral e, de acordo com os resultados, divididos em dois grupos: aqueles com anormalidades (PIDCabnl) e aqueles sem alterações (PIDCnl) na RM. Cinco pacientes PIDCnl e nove PIDCabnl tiveram os dados analisados. Comparações entre os grupos foram realizadas para cada parte do FDT. Resultados Os dois grupos de pacientes foram estatisticamente mais lentos nas tarefas de VP comparados ao grupo controle. As medidas de VP foram negativamente associadas às pontuações de incapacidade (leitura: r = −0,47; p = 0,003; contagem: r = −0,53; p = 0,001). Conclusões Os dados indicaram a presença de prejuízo na VP em pacientes com PIDC. A incapacidade foi associada à lentidão na VP.
ABSTRACT
INTRODUCTION: According to most studies, the incidence of Guillain-Barré syndrome increases with age, with a peak incidence occurring between 70 and 80 years of age. The objective of this study is to describe the incidence (overall and by sex and age group) and clinical characteristics of Guillain-Barré syndrome in Osona (Barcelona, Spain). METHODS: We performed a retrospective, descriptive, population-based study covering the period 2003 to 2016. RESULTS: The global incidence of Guillain-Barré syndrome is 2.07 cases per 100000 person-years. Incidence increases with age, except for small peaks during childhood and between 40 and 50 years, and reaches a maximum of 6.26 cases per 100000 person-years above the age of 80. The incidences of the different variants were: AIDP, 72.1%; AMAN, 16.3%; ANSAN, 4.7%; and Miller Fisher syndrome, 4.7%. A total of 41.9% of patients had a history of respiratory tract infections, and 20.9% had a history of gastrointestinal infections. Protein in the cerebrospinal fluid was found in 76.7%. EMG findings suggested demyelination in 73.7% of the patients and axonal degeneration in 26.3%. A total of 20.9% of patients needed ventilatory support. Six-month mortality was 9.3%. Variables associated with worse prognosis were age over 80 years, delay in admission, previous gastrointestinal infection, and AMAN variant. CONCLUSIONS: The incidence observed in our study is in the upper range of estimated incidence rates reported in European and North American studies. The syndrome may be underdiagnosed in elderly patients; physicians must be vigilant to the possibility of the disease, which is associated with a high mortality rate if it is not treated early.
Subject(s)
Guillain-Barre Syndrome , Miller Fisher Syndrome , Adult , Aged , Aged, 80 and over , Guillain-Barre Syndrome/epidemiology , Humans , Incidence , Middle Aged , Retrospective Studies , Spain/epidemiologyABSTRACT
Introducción: La mayoría de los estudios muestran que la incidencia del síndrome de Guillain-Barré aumenta con la edad, con un máximo entre los 70 y los 80 años y un descenso posterior. El objetivo del estudio es describir la incidencia global y específica por sexo y grupos de edad y las características clínicas del síndrome de Guillain-Barré en la comarca de Osona (Barcelona, España).MétodosEstudio descriptivo retrospectivo de base poblacional en el periodo 2003-2016.ResultadosLa incidencia global es de 2,07/100.000 habitantes-año. La incidencia aumenta con la edad, salvo un pequeño pico en la infancia y entre los 40 y los 50 años, alcanzando la máxima de 6,26/100.000 habitantes-año pasados los 80 años. Los porcentajes de las variantes fueron: AIDP (72,1%), AMAN (16,3%), ANSAN (4,7%) y síndrome de Miller-Fisher (4,7%). Presentaron infección previa de vías respiratorias el 41,9% e infección gastrointestinal el 20,9%. Se halló proteinorraquia en el 76,7%. El EMG mostraba un predominio desmielinizante en el 73,7% y axonal en el 26,3%. Necesitaron soporte ventilatorio el 20,9%. La mortalidad a los 6 meses fue del 9,3%. Las variables que se asociaron a un peor pronóstico fueron la edad superior a los 80 años, la demora en el ingreso, presentar infección gastrointestinal previa y la variante AMAN.ConclusionesLa incidencia descrita en nuestro estudio se encuentra en el rango más alto de las estimadas en Europa y Estados Unidos. En ancianos pudiera estar infradiagnosticado y se requeriría de una mayor alerta ante una enfermedad con alta mortalidad si no se trata de forma precoz. (AU)
Introduction: According to most studies, the incidence of Guillain-Barré syndrome increases with age, with a peak incidence occurring between 70 and 80 years of age. The objective of this study is to describe the incidence (overall and by sex and age group) and clinical characteristics of Guillain-Barré syndrome in Osona (Barcelona, Spain).MethodsWe performed a retrospective, descriptive, population-based study covering the period 2003 to 2016.ResultsThe global incidence of Guillain-Barré syndrome is 2.07 cases per 100,000 person-years. Incidence increases with age, except for small peaks during childhood and between 40 and 50 years, and reaches a maximum of 6.26 cases per 100,000 person-years above the age of 80. The incidences of the different variants were: AIDP, 72.1%; AMAN, 16.3%; ANSAN, 4.7%; and Miller Fisher syndrome, 4.7%. A total of 41.9% of patients had a history of respiratory tract infections, and 20.9% had a history of gastrointestinal infections. Protein in the cerebrospinal fluid was found in 76.7%. EMG findings suggested demyelination in 73.7% of the patients and axonal degeneration in 26.3%. A total of 20.9% of patients needed ventilatory support. Six-month mortality was 9.3%. Variables associated with worse prognosis were age over 80 years, delay in admission, previous gastrointestinal infection, and AMAN variant.ConclusionsThe incidence observed in our study is in the upper range of estimated incidence rates reported in European and North American studies. The syndrome may be underdiagnosed in elderly patients; physicians must be vigilant to the possibility of the disease, which is associated with a high mortality rate if it is not treated early. (AU)
Subject(s)
Humans , Guillain-Barre Syndrome/epidemiology , Incidence , Miller Fisher Syndrome , Spain/epidemiology , Retrospective StudiesABSTRACT
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.
Subject(s)
Humans , Female , Adult , Pancreatic Neoplasms/surgery , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complications , Insulinoma/surgery , Anesthetics/administration & dosage , HypoglycemiaABSTRACT
Objetivos: Describir las características clínicas y paraclínicas de pacientes con síndrome de Guillain-Barré en el Hospital Regional Lambayeque entre los años 2011 y 2015. Material y métodos: Se revisaron las historias clínicas de pacientes con síndrome de Guillain-Barré atendidos en el Hospital Regional Lambayeque en la ciudad de Chiclayo-Perú, Resultados: Encontramos 16 pacientes con Síndrome de Guillain-Barré, 56% varones y 44% mujeres; la enfermedad se presentó mayormente en adultos de 20 a 60 años (44%). La mayor proporción de casos se dio durante las estaciones de invierno y primavera. El subtipo axonal fue el más común (62,5%). En cinco de seis pacientes en los que se practicó punción lumbar para efectuar tests de líquido cefalo-raquídeo se encontró disociación albuminocitológica. Cinco de los pacientes requirieron ventilación mecánica y tres de ellos fallecieron. Conclusiones: Este primer reporte del Síndrome de Guillain-Barré realizado en un hospital del norte del Perú, demuestra diferencias en las características clínicas y paraclínicas de nuestros pacientes.
Objectives: To describe clinical and paraclinical characteristics of patients with Guillain-Barré Syndrome (GBS) admitted to the Regional Hospital of Lambayeque from 2011 to 2015. Material and methods: The charts of patients with GBS treated in the Regional Hospital of Lambayeque (Chiclayo, Peru) were reviewed. Results: We found 16 patients with GBS, 56% male and 44% female; the disease was mainly observed in adults between 20-60 years (44%). The major proportion of cases was seen in the winter and spring seasons. The axonal form was the most common subtype (62.5%). In five of six patients in whom lumbar puncture was performed to test cerebro-spinal fluid, albumin-cytological dissociation was found. Five patients required artificial ventilation and three died. Conclusions: This is the first study of patients with GBS in a hospital in Northern Peru, demonstrating specific characteristics in the patient population hereby studied.
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Se realizó un estudio descriptivo y prospectivo de 25 pacientes con síndrome de Guillain-Barré, atendidos en el Hospital Infantil Docente Sur de Santiago de Cuba, desde enero de 2006 hasta diciembre de 2012, con vistas a identificar sus características clinicoepidemiológicas y determinar la utilidad del tratamiento empleado. Predominaron los niños menores de 5 años (40,0 por ciento), el sexo masculino y la infección respiratoria alta previa (ambas con 60,0 por ciento), así como la debilidad muscular y arreflexia osteotendinosa (76,0 por ciento en cada caso). La polirradiculoneuropatía desmielinizante inflamatoria aguda fue la variante clínica más frecuente (72,0 por ciento). Tanto el tratamiento con inmunoglobulina humana intravenosa como la rehabilitación resultaron beneficiosos y la mortalidad baja (4,0 por ciento)(AU)
A descriptive and prospective study of 25 patients with Guillain-Barré syndrome, assisted in the Teaching Southern Children Hospital in Santiago de Cuba was carried out from January, 2006 to December, 2012, with the aim of identifying their clinical and epidemiological characteristics and to determine the usefulness of the treatment applied. Children younger than 5 years (40.0 percent), male sex and previous upper respiratory infection (both with 60.0 percent), as well as the muscular weakness and osteotendinous areflexia (76.0 percent in each case) prevailed. The inflammatory and acute desmyelinating polyrradiculoneuropathy was the most frequent clinical variant (72.0 percent). Either the treatment with intravenous human immunoglobulin as rehabilitation were beneficial and the mortality was low (4.0 percent)(AU)
Subject(s)
Humans , Male , Female , Child , Guillain-Barre Syndrome , Polyradiculoneuropathy , Immunoglobulins, Intravenous , Secondary Care , Epidemiology, Descriptive , Prospective StudiesABSTRACT
Se realizó un estudio descriptivo y prospectivo de 25 pacientes con síndrome de Guillain-Barré, atendidos en el Hospital Infantil Docente Sur de Santiago de Cuba, desde enero de 2006 hasta diciembre de 2012, con vistas a identificar sus características clinicoepidemiológicas y determinar la utilidad del tratamiento empleado. Predominaron los niños menores de 5 años (40,0 %), el sexo masculino y la infección respiratoria alta previa (ambas con 60,0 %), así como la debilidad muscular y arreflexia osteotendinosa (76,0 % en cada caso). La polirradiculoneuropatía desmielinizante inflamatoria aguda fue la variante clínica más frecuente (72,0 %). Tanto el tratamiento con inmunoglobulina humana intravenosa como la rehabilitación resultaron beneficiosos y la mortalidad baja (4,0 %).
A descriptive and prospective study of 25 patients with Guillain-Barré syndrome, assisted in the Teaching Southern Children Hospital in Santiago de Cuba was carried out from January, 2006 to December, 2012, with the aim of identifying their clinical and epidemiological characteristics and to determine the usefulness of the treatment applied. Children younger than 5 years (40.0%), male sex and previous upper respiratory infection (both with 60.0%), as well as the muscular weakness and osteotendinous areflexia (76.0% in each case) prevailed. The inflammatory and acute desmyelinating polyrradiculoneuropathy was the most frequent clinical variant (72.0%). Either the treatment with intravenous human immunoglobulin as rehabilitation were beneficial and the mortality was low (4.0%).
ABSTRACT
La Polirradiculoneuropatía Desmielinizante Inflamatoria Crónica (PDIC) es un desorden adquirido de los nervios periféricos y las raíces nerviosas, que se desarrolla de forma continua durante ocho semanas o más. La forma clásica se caracteriza por compromiso simétrico sensitivo y motor, la debilidad está presente tanto en musculatura proximal como distal y es característico en el estudio electrofisiológico la desmielinización junto a disociación albúmino-citológica en Líquido Cefalorraquídeo (LCR). PRESENTACION DEL CASO. Mujer de 34 años con antecedente de depresión en tratamiento, inicia hace un año aproximadamente debilidad muscular de las cuatro extremidades, ascendente y progresiva, llegando a la incapacidad de la marcha. Ingresó al servicio de urgencia del hospital de Puerto Montt, con paraparesia predominante en extremidades inferiores, hipoestesia y ausencia de reflejos osteotendinosos. Estudio electrofisiológico evidencia severa polineuropatía sensitivamotora desmielinizante con degeneración axonal secundaria.LCR con proteinorraquia de 100 mg/dl y sin celularidad. Inició tratamiento con corticoides y luego plasmaféresis, evolucionando con mejoría significativa de la funcionalidad y marcha independiente. DISCUSIÓN. Las principales modalidades terapéuticas de PDIC son la inmunoglobulina intravenosa, glucocorticoides y plasmaféresis. Todos estos parecen ser igualmente efectivos de forma separada. La elección está influenciada por las preferencias del paciente, efectos adversos, costo, duración y disponibilidad. El uso de inmunoglobulina intravenosa es a veces limitado; los corticoides son económicos, pero con efectos adversos frecuentes y relevantes; y la plasmaféresis es cara, invasiva y solamente disponible en centros especializados. La mayoría de los pacientes, responden inicialmente, pero las recaídas son frecuentes...
INTRODUCTION. Chronic inflammatory demyelinating polyradiculoneuropathy(CIDP) is an acquired disorder of peripheral nerves and nerve roots that run continuously for 8 weeks or more. The classic form is characterized by symmetric sensory and motor involvement, weakness is present in both proximal and distal muscles and is characteristic in the electrophysiological study demyelination with albumin-cytological dissociation in cerebrospinal fluid (CSF). PRESENTATION OF THE CASE.34-year-old woman with a history of depression in treatment, started about a year ago muscle weakness of all limbs, ascending and progressive, leading to the inability of the march. Was admitted with paraparesis, predominantly in lower extremities, hypoesthesia and absence of tendon reflexes. Electrophysiological study evidence severe demyelinating motor sensory polyneuropathy with secondary axonal degeneration. CSF protein concentration of 100 mg / dL and no cellularity. Starts treatment with corticosteroids and plasmapheresis, evolving with significant improvement in functionality and independent walking. DISCUSSION. The main therapeutic modalities in CIDP are intravenous immunoglobulin, corticosteroids and plasmapheresis. All these seem to be equally effective separately. The choice is influenced by patient preference, adverse effects, cost, duration and availability. The use of intravenous immunoglobulinis sometimes limited; corticosteroids are inexpensive, but with frequent and significant side effects; and plasmapheresis is expensive, invasive and only available at specialized centers. Most patients respond initially but relapses are frequent...
