Desde la pérdida de la fuerza hasta el compromiso sistémico: serie de casos del síndrome POEMS / From limb weakness to a systemic involvement: Poems syndrome case two report and review of the literature

El síndrome POEMS es un trastorno paraneoplásico raro y poco frecuente, que se presenta principalmente en la sexta década de la vida, caracterizado por el compromiso multisistémico con predominio de neuropatía desmielinizante. Abarca diversas y heterogéneas manifestaciones clínicas y su diagnóstico requiere un alto índice de sospecha. Se presentan dos casos de pacientes que consultaron por cuadros poco frecuentes en los que la pérdida de la fuerza orientó al acercamiento de una afectación multisistémica que concluyó con el diagnóstico de esta enfermedad(AU)

POEMS syndrome is a rare and infrequent paraneoplastic syndrome, which occurs mainly in the sixth decade of life, characterized by multisystem involvement with a predominance of demyelinating neuropathy, which encompasses diverse and heterogeneous clinical manifestations and whose diagnosis requires a high index of suspicion. We present two cases of patients who consulted due to unusual symptoms and whose loss of strength led to an approach due to multisystem involvement that concluded with the diagnosis of this disease(AU)

Peripheral polyneuropathy in severely obese patients with metabolic syndrome but without diabetes: Association with low HDL-cholesterol / Polineuropatia periférica em pacientes obesos graves com síndrome metabólica sem diabetes: associação com baixo HDL-colesterol

Summary Introduction: The purpose of this study was to evaluate the prevalence of peripheral polyneuropathy (PPN) in subjects with grade II and III obesity (Ob-II,III) and metabolic syndrome (MetS) but without diabetes and to investigate possible associated factors. Method: A cross-sectional study was performed in non-diabetic Ob-II,III,MetS patients using the Michigan Neuropathy Screening Instrument (MNSI) to assess the presence of PPN. Results: A total of 24 of 218 non-diabetic Ob-II,III,MetS patients had PPN. Based on univariate analysis, serum levels of LDL-cholesterol (p=0.046) were significantly associated with PPN, while serum triglycerides (p=0.118) and low HDL-cholesterol (p=0.057) showed a tendency toward this association. On a Poisson regression analysis, when the three possible associations were included, low HDL-cholesterol (p=0.047) remained independently associated. Conclusion: In non-diabetic Ob-II,III,MetS patients, PPN defined by the MNSI showed a high prevalence and was associated with low levels of HDL-cholesterol. In order to diagnose that complication, neurological evaluation should be performed in these patients.

Resumo Objetivo: Avaliar a prevalência da polineuropatia periférica (PNP) em indivíduos obesos graus II e III com síndrome metabólica (Ob-II,III,SM) sem diabetes e buscar possíveis fatores associados. Método: Em um estudo transversal, realizado em indivíduos Ob-II,III,SM e sem diagnóstico de diabetes, o Instrumento de Screening de Michigan (MNSI) foi utilizado para avaliar a presença de PNP. Resultados: Um total de 24 de 218 pacientes Ob-II,III,SM e sem diabetes tinham PNP. Quando observamos as associações com PNP em uma análise univariada, níveis séricos de LDL-colesterol (p=0.046) estiveram significativamente associados e houve também uma tendência à associação com níveis séricos de triglicerídeos (p=0.118) e baixo HDL-colesterol (p=0.057). Em uma análise de regressão de Poisson, quando as três possíveis associações foram incluídas, baixo HDL-colesterol (p=0.047) manteve-se independentemente associado. Conclusão: Em pacientes Ob-II,III,SM, mas sem diabetes, a PNP definida pelo MNSI tem uma prevalência elevada e está associada a baixos níveis de HDL-colesterol. Para diagnóstico dessa complicação, recomenda-se realizar o exame neurológico desses pacientes.

Heterogeneidade clínica e coexistência das neuropatias diabéticas: diferenças e semelhanças entre diabetes melito tipos 1 e 2 / Clinical heterogeneity and coexistence of diabetic neuropathies: difference and similarities between types 1 and 2 diabetes mellitus

OBJETIVO: Estudar a heterogeneidade e a coexistência das neuropatias no diabetes melito tipos 1 (DMT1) e 2 (DMT2). MÉTODOS: Foram avaliados 74 DMT2 e 20 DMT1 em relação à idade (anos), tempo de diagnóstico do DM (TDDM, em anos), índice de massa corpórea (IMC, kg/m²), HbA1c e tipo de neuropatia (critérios da American Diabetes Association). RESULTADOS: DMT1 era mais jovem (32,7 ± 11 versus 56,9 ± 10,3; p = 0,0001), com maior TDDM (17,1 ± 9,7 versus 10,4 ± 6,8; p = 0,003) e menor IMC (23,6 ± 3,8 versus 28,4 ± 5,3; p = 0,0005). A neuropatia autonômica cardiovascular (NAC) (60 por cento versus 32,4 por cento; p = 0,02) e a coexistência desta com polineuropatia (PND) (62,5 por cento versus 33,3 por cento; p = 0,03) foram mais prevalentes no DMT1; a PND dolorosa crônica (PNDDC) (60,8 por cento versus 30,0 por cento; p = 0,009) o foi no DMT2. A HbA1c (p = 0,04) foi preditiva de PND em ambos os grupos. O TDDM (p = 0,03) e a PNDDC (p = 0,003) foram preditivos de NAC no DMT1. A idade (p = 0,0004) teve valor preditivo para PNDDC no DMT2. CONCLUSÕES: As neuropatias apresentam distribuição heterogênea no DMT1 e no DMT2. Com exceção do controle glicêmico, os fatores relacionados a essa complicação diferem de acordo com o tipo de diabetes.

OBJECTIVE: To evaluate the heterogeneity and the coexistence of diabetic neuropathy (DNP) in type 1 (T1DM) and 2 (T2DM) diabetes mellitus. METHODS: 74 T2DM and 20 T1DM patients were evaluated according to age (years), time from diagnosis of diabetes (TDD, years), body mass index (BMI, kg/m²), HbA1c and DNP type (American Diabetes Association criteria). RESULTS: T1DM was younger (32.7 ± 11.0 versus 56.9 ± 10.3; p = 0.0001), leaner (BMI: 23.6 ± 3.85 versus 28.4 ± 5.3; p = 0.0005) and they had longer TDD (17.1 ± 9.7 versus 10.4 ± 6.8; p = 0.003). Cardiovascular autonomic neuropathy (CAN) (60 percent versus 32.4 percent; p = 0.02) and its coexistence with polyneuropathy (PN) (62.5 percent versus 33.3 percent; p = 0.03) were more common in T1DM. Chronic painful polyneuropathy (CPP) was more prevalent in T2DM (60.8 percent versus 30.0 percent; p = 0.009). Logistic regression showed HbA1c as an independent variable related to PN (p = 0.04) in both groups. TDD (p = 0.03) and CPP (p = 0.003) were related to CAN in T1DM. Age (p = 0.0004) was related to CPP in T2DM. CONCLUSIONS: The DNP have shown a heterogeneity distribution in type 1 and type 2 diabetes mellitus. The related factors to different phenotypes of this complication, apart from hyperglycemia, may be variable between these two types of diabetes mellitus.

[Evaluation of some prevalent causes of polyneuropathy in patients referring to the neurologic outpatient clinic of Bahman 22[nd] hospital during the years of 2007-2008]

Polyneuropathy is defined as a diffused peripheral nerve disorder that presents with sensory, motor or autonomic dysfunctions. This study aims at determining the causes of polyneuropathy among patients who referred to Bahman 22[nd] Hospital in Mashhad. This is a descriptive study that was carried out on 31 patients suspected to polyneuropathy referring to Bahman 22[nd] Hospital in Mashhad during the years of 2007-2008. The study collected data on the patients' past medical history, drug history and family history of manifesting similar symptoms. Also, the research undertook neurological examinations and neurophysiologic studies on all the participating patients. Polyneuropathy was confirmed using electrophysiologic studies. Supplementary laboratory exams were conducted with patients' consent to diagnose polyneuropathy. Statistical analysis was then done on the collected data using SPSS software. The most frequent cause of polyneuropathy was found to be CIDP. The other causes were determined as diabetes mellitus in 16.5% of the participants, GBS in 16.5% as well as leprosy, toxic and hereditary causes each in 9.7% of the participants. Also Vitamin B[12] deficiency was determined to be the cause in 6.5% of the cases. We didn't find any determinative cause of the disease in 9.7% of the cases; so, they were classified as cryptogenic. Among the predominating clinical manifestation was sensory complains [66.7%]. Pain and temperature disorders were the most common presentations [87.1%]. It was also found that muscle tonicity affected mostly the distal area of lower extremities. Muscle strength was found to be diminished in the distal areas of both lower and upper extremities. Moreover, Achilles Reflex function was reduced in 87.1% of the patients in this study. The most frequent causes of polyneuropathy were inflammation and metabolic disorders. Muscle tonicity, muscle strength and deep tendon reflexes were reduced in the distal areas of extremities. Therefore, in neurological examinations, detailed evaluation of distal areas is essential

Prevalence and characteristics of diabetic polyneuropathy in Passo Fundo, South of Brazil

PURPOSE: The aim of the study was to investigate the prevalence of peripheral diabetic neuropathy (PDN) and associated characteristics among type 2 diabetic mellitus (DM2) patients in Passo Fundo, a city on Southern Brazil. BASIC PROCEDURES: A cross-sectional study was conducted with 340 patients with type 2 diabetes mellitus. Tests were performed to evaluate vibration (tuning fork), light touch (10-g monofilament), temperature, and pain (pinprick) sensations, as well as ankle reflexes and heel walking. The condition was classified as peripheral diabetic neuropathy when results in at least three of these tests were negative. The electrically induced Hoffmann reflex test was performed in a group of patients to define the criterion standard used in the assessment of clinical examination sensibility in the diagnosis of neuropathy. Sensitivity was 83 percent, specificity, 91 percent, positive predictive value, 63 percent, and negative predictive value, 90 percent. The monofilament, pinprick and deep tendon reflex were the most accurate tests in the diagnosis of neuropathy. MAIN FINDINGS: Seventy-five patients (22.1 percent) had peripheral diabetic neuropathy, nephropathy in 29.5 percent and retinopathy in 28.8 percent. CONCLUSIONS: Logistic regression revealed that only duration of diabetes, creatinine and glycated hemoglobin concentrations were significantly associated with neuropathy.

PROPOSITO: O objetivo deste estudo foi investigar a prevalência da neuropatia diabética periférica (NDP) e características associadas entre pacientes com diabetes melito tipo 2 (DM2) na cidade de Passo Fundo, sul do Brasil. PROCEDIMENTOS BASICOS: Um estudo de corte foi realizado com 340 pacientes portadores de DM2. Testes foram conduzidos a fim de avaliar sensações de vibração (diapasão), de toque leve (monofilamento 10 g), de temperatura e de dor (agulha), bem como reflexos do tornozelo e caminhar com os calcanhares. A condição foi classificada como NDP quando os resultados em pelo menos três desses testes foram negativos. O teste reflexo de Hoffman induzido eletricamente foi conduzido em um grupo de pacientes a fim de determinar o critério padrão usado na avaliação da sensibilidade do exame clínico no diagnóstico de neuropatia. A sensibilidade foi de 83 por cento, especificidade, 91 por cento, valor preditivo positivo, 63 por cento e valor preditivo negativo, 90 por cento. O teste do monofilamento, a dor e o reflexo do tendão foram os mais precisos no diagnóstico de neuropatia. PRINCIPAIS ACHADO: Setenta e cinco pacientes (22,1 por cento) foram diagnosticados com NDP, nefropatia em 29,5 por cento e retinopatia em 28,8 por cento. CONCLUSÕES: Regressão logística revelou que somente a duração do diabetes e as concentrações de creatinina e hemoglobina glicada foram significativamente associados à neuropatia.

Clinical and electrophysiologic evaluation of peripheral neuropathy in a group of HIV-infected patients in Thailand.

OBJECTIVE: To study characteristics of peripheral neuropathy in a group of Thai HIV-infected patients by clinical and electrophysiologic evaluation. MATERIAL AND METHOD: Patients with HIV infection from HIV and neurology clinic were recruited during June and October 2005. Neurological examination and nerve conduction study were done in all patients to establish the presence and pattern of peripheral neuropathy. Clinical data were compared between the groups with and without HIV-related neuropathy. RESULTS: Forty-eight HIV-infected patients were recruited but complete data were obtained in 34 patients. There were 11 males and 23 females with average age of 36.3 +/- 7.3 years. Among these, 17 (50.0%) patients received a diagnosis of HIV-related neuropathy. Distal symmetrical polyneuropathy (DSP) accounted for 64.7%, mononeuropathy multiplex (MM) for 17.6%, acute inflammatory demyelinating polyneuropathy (AIDP) for 11.8%, and progressive polyradiculopathy (PP) for 5.9% of cases with HIV-related neuropathy. The presence of neuropathy was not correlated with age, sex, body mass index, and duration of HIV infection. However, patients with HIV-related neuropathy had significantly lower nadir CD4 cell counts than patients without HIV-related neuropathy (p < 0.05). When taking antiretroviral therapy in to account, we did not find correlation of any drugs with the presence of DSP except for stavudine, which had shown a statistical trend. CONCLUSION: The incidence of HIV-associated neuropathy in this group of Thai patients was higher than previous report. The most common pattern was distal symmetrical polyneuropathy, which was associated with low nadir CD4 cell counts.

Prevalence of neuropathy in patients with chronic obstructive pulmonary disease

Chronic obstructive pulmonary disease is a disease that reduces the expiratory flow, thus resulting in hypoxemia in variable severity. Twenty-one chronic obstructive pulmonary disease patients and 43 age-matched control subjects were included in the study. Nerve conduction study, pulmonary function tests and oxygen saturation were evaluated. The sensory conduction velocities of all the examined nerves were reduced significantly in the patient's group. The motor conduction velocity is reduced but not to a significant level. All the parameters of pulmonary function tests and oxygen saturation were reduced in the chronic obstructive pulmonary disease patients. A clear relationship between the degree of airway obstruction and posterior tibial sensory neuropathy was noticed. The abnormality in the nerve conduction velocity is predominantly of sensory type. Low oxygen concentration appears to be the causative agent

Comportamiento de la polineuropatía epidémica en un municipio / Development of epidemic polyneuropathy in a municility

Resumen: Se realiza un estudio de carácter explorativo descriptivo en el período del 1ro. de enero de 1993 al 1ro. de enero de 1997 (4 años) en la población correspondiente al municipio Lajas de la provincia Cienfuegos de 81 pacientes afectados por polineuropatía epidémica, los cuales se mantuvieron bajo seguimiento por el médico y la enfermera de la familia. Los enfermos fueron entrevistados, examinados, y se aplicó un formulario para la obtención de datos de interés como sexo, edad, raza, forma clínica, hábitos tóxicos, síntomas, signos y secuelas. Se comprueba el predominio del sexo femenino (66,6 porciento) y los grupos etáreos más afectados fueron del 25 a 44 y el de 45 a 64 años, el 44,5 porciento presentó forma clínica periférica y el 32 porciento óptica. El estrés fue un factor asociado en el 64,2 porciento, la debilidad de miembros inferiores y la disminución de la agudeza visual fueron los síntomas más frecuentes y los trastornos de sensibilidad, el signo más señalado; predominó la evolución favorable y los enfermos con secuelas se relacionaron con tener hábitos tóxicos. Se realiza test de significación estadística chi cuadrado y los principales resultados se expresan en tablas

Evolución clínica y electrofisiológica de pacientes con diagnóstico de parálisis flácida en Nicaragua durante el período del 1ro de Enero al 31 de diciembre de 1993 / Clinical evolution and electrofisiológica of patient with diagnosis of flabby paralysis in Nicaragua during the period of January 1ro at December 31 1993

Se estudiaron 46 pacientes que fueron notificados con parálisis flácida aguda en el período entre el 01 de enero y el 31 de diembre de 1993. Al finalizar el período de vigilancia de estos pacientes (90 días), todos los casos fueron descartados como poliomielitis, 20 casos fueron clasificados como polineuropatía tipo Síndrome de Guillan Barré y 4 correspondieron a otras neuropatias perifericas. De los 46 pacientes, ocho fallecieron durante el período de vigilancia, tres de ellos clasificados como SGB. DE 24 casos preseleccionados con diagnostico correspondiente o sugestivo de afeccion de la neurona motora inferior, sólo fue posible localizar para las evaluaciones a 19 pacientes, de estos se excluyeron 3 casos que cumplieron con los criterios previamente estabelcidos para la realización del estudio electrodiagnostico, quedando finalmente un total de 16 pacientes para ser sometidos a dicho procedimiento y sobre los cuales se basan las conclusiones y resultados que se presentan en este trabajo. En ningún paciente captado por el programa de vigilancia epidemiologica de parasilisis flácida aguda en Nicaragua fue confirmado caso de poliomielitis y en ninguno de los pacientes evaluados clínicamente entre los 9 y 17 meses después de sufrida la parálisis, se encontraron secuelas paralítcas del tipo descrito y ampliamente reconocido por dicha enfermedad. El 75 porciento de los casos aún presenta algún grado de secuelas motoras; (62.5 porciento secuelas moderadas, 12.5 porciento secuelas leves, y ninguno secuelas graves), éstos son de predominio distal, simétricas y con poca atrofia muscular, contrario a lo esperado para Polio, ésto es de predominio proximal, asimetricas y con marcada atrofia muscular. El esudio electrofisiológico fue anormal en el 100 porciento de los pacientes estudiados, en el 93.8 porcientoe (15 de 16) la alteración correspondió a afección del nerivio periférico (68.8 porciento mixta, axonomielínica y 25.0 porciento desmielinizante). En un caso (6.2 porciento) los hallazgos electrofisiológicos fueron indicativos de afección primaria de la fibra muscular...