ABSTRACT
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
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
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
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...