Síndrome de poems: a proposito de un caso, Hospital Escuela Universitario / Poems Syndrome: a case, Hospital Escuela Universitario

El síndrome de POEMS se caracteriza por polineuropatía, organomegalia, endocrinopatia, proteína M, y alteraciones cutáneas, también incluye papiledema, sobrecarga de volumen extravascular, trombocitosis/eritrocitosis, niveles elevados de factor de crecimiento endotelial vascular, una predisposición hacia la trombosis y está incorporado en el grupo de las gammapatias monoclonales. Se diagnostica en base a una combinación de clínica y de hallazgos laboratoriales. Objetivo: describir un síndrome clínico poco frecuente, las dificultades diagnósticas, así como diferenciarlo de otras polineuropatías crónicas. Presentación del caso clínico: femenina de 52 años, con hiperpigmentacion de piel, disminución de agudeza visual bilateral y debilidad distal progresiva con arreflexia y síntomas sensitivos asociados, organomegalia, hipotiroidismo; se comprobó polineuropatía crónica en estudios de neuroconducción, trastornos hematológicos, pérdida de peso y niveles bajos de vitamina B12, cumpliendo la mayoría de los criterios del síndrome, aunque no se encontró trastorno monoclonal, la literatura resalta normal en un 25% de los casos la electroforesis de proteínas. Conclusión: Es una enfermedad poco frecuente, para su diagnóstico se requiere una alta sospecha clínica, como hallazgos insulares edema de papila y niveles bajos de vitamina B12, descritos en la literatura. Debido a la diversidad en la forma de presentación del síndrome es necesario estar alerta sobre las múltiples manifestaciones y la búsqueda clínica, analítica y radiológica debe ser dirigida...(AU)

Complicações neurológicas pós-cirurgia bariátrica: uma revisão de literatura / Neurological complications post-bariatric surgery: a review of the literature

A obesidade é um grave problema de saúde em todo o mundo. Apresenta alta prevalência e grande impacto sobre a mortalidade. A cirurgia bariátrica é cada vez mais utilizada no tratamento de obesidade mórbida por se mostrar o mais eficaz tratamento mantendo a perda de peso sustentada e diminuindo a incidência das comorbidades associadas. Complicações neurológicas agudas e crônicas têm sido relatadas após este procedimento, e podem resultar principalmente por deficiência nutricional. Objetivo: Destacar as complicações neurológicas comuns e raras que podem ocorrer após cirurgia bariátrica. Metodologia: Revisão narrativa da literatura. Resultados: Complicações neurológicas pós-cirurgia bariátrica podem ocorrer em qualquer nível do neuroeixo ou em músculos. As mais comuns são por deficiências nutricionais, mas outros mecanismos mais raros podem ocorrer como inflamatórios mecânicos. Conclusão: Com o aumento de incidência da obesidade, a cirurgia bariátrica tem se tornado cada vez mais frequente para perda de peso. É importante avaliar corretamente a indicação desse procedimento uma vez que ele não é isento de complicações. Embora a maioria das complicações do sistema nervoso central, periférico e musculoesquelético após a cirurgia bariátrica seja devido a deficiências nutricionais, existem outras neuropatias associadas com envolvimento inflamatório do nervo periférico. Um processo autoimune tem sido aceito como fisiopatologia subjacente. (AU)

Obesity is a serious health problem throughout the world. It has high prevalence and significant impact on mortality. Bariatric surgery is increasingly used in the treatment of morbid obesity out to be the most effective treatment maintaining sustained weight loss and decreasing the incidence of comorbidities. Acute and chronic neurological complications have been reported after this procedure, and may result primarily by nutritional deficiency. Objective: To emphasize the common and rare neurological complications that may occur after bariatric surgery. Methodology: Narrative review of the literature. Results: Neurological complications after bariatric surgery can occur at any neuraxial level or muscle. The most common are by nutritional deficiency, but other rarer mechanisms may occur, like mechanical or inflammatory. Conclusion: With increasing incidence of obesity, bariatric surgery has become increasingly common for weight loss. It is important to properly evaluate the indication for this procedure since it is not without complications. Although most of the central and peripheral nervous system and musculoskeletal complications after bariatric surgery are due to nutritional deficiencies, there are other neuropathies associated with inflammatory involvement of the peripheral nerve. An autoimmune process has been accepted as an underlying pathophysiology. (AU)

Sensory disturbance and polyneuropathy in rheumatoid arthritis patients with foot deformity / Perturbações sensitivas e polineuropatia em pacientes com artrite reumatoide com deformidade do pé

Abstract Introduction Our aim in this study was to present the results of sensory evaluation tests and electrophysiological evaluations in rheumatoid arthritis (RA) patients with foot deformity and to determine their relation with general health status and lower extremity functionality. Materials and methods Fifty-one patients with RA diagnosis and foot deformity were included in the study. Demographic and disease characteristics of the patients were recorded, and a detailed neurological examination was performed. Superficial sensation, pain, heat, vibration, and two-point discrimination sensation were evaluated in each foot, and their sum was used to determine the sensory deficits index (SDI) of 0–10. The presence of polyneuropathy was evaluated with electrophysiological methods. The Health Assessment Questionnaire and mobility and walking subscales of the Arthritis Impact Measurement Scales-2 were used to assess general health status and lower extremity functionality, respectively. According to the sensory examination and electromyography results, patients were compared in terms of their general health status and lower extremity functional status. Results Sensory disturbance was detected in 39 patients (74%) during the examination; however, 27 patients (52.9%) had polyneuropathy determined electrophysiologically. In patients with sensory deficits, statistically significant deterioration was detected in general health and foot functionality, including mobility and walking, when compared to patients with a normal sensory evaluation. Conclusions Even in the presence of normal electrophysiological tests, sensory dysfunction alone seems to be associated with severe disability in general health status and foot functionality when compared to patients with a normal sensory examination.

Resumo Introdução O objetivo deste estudo foi apresentar os resultados dos testes de avaliação sensitiva e do exame eletrofisiológico em pacientes com artrite reumatoide (AR) com deformidade do pé e determinar a sua relação com o estado geral de saúde e o aspecto funcional dos membros inferiores. Materiais e métodos Foram incluídos no estudo 51 pacientes com diagnóstico de AR e deformidade do pé. Foram registradas as características demográficas e da doença de cada indivíduo e foi feito um exame neurológico detalhado. Foi avaliada a sensibilidade superficial, dolorosa, térmica e vibratória e aplicado o teste de discriminação de dois pontos em cada um dos pés. A soma dos escores foi usada para determinar o índice de déficits sensitivos (IDS) de 0‐10. A presença de polineuropatia foi avaliada com métodos eletrofisiológicos. Foram usados o Health Assessment Questionnaire e as subescalas mobilidade e deambulação da Arthritis Impact Measurement Scales‐2 para avaliar o estado geral de saúde e o aspecto funcional de membros inferiores, respectivamente. De acordo com os resultados dos exames de eletromiografia e de sensibilidade, os pacientes foram comparados em relação ao seu estado geral de saúde e estado funcional de membros inferiores. Resultados Foram detectados distúrbios sensitivos em 39 pacientes (74%) durante o exame; contudo, 27 (52,9%) tinham polineuropatia determinada eletrofisiologicamente. Em pacientes com déficits sensitivos, foi detectada deterioração estatisticamente significativa no estado geral de saúde e no aspecto funcional do pé, inclusive na mobilidade e deambulação, quando comparados com os pacientes com uma avaliação sensitiva normal. Conclusão Mesmo na presença de testes eletrofisiológicos normais, a disfunção sensitiva isolada parece estar associada a incapacidade grave no estado geral de saúde e ao aspecto funcional do pé em comparação com pacientes com um exame sensitivo normal.

Síndrome de Poems: reporte de un caso clínico / Poems syndrome: a case report

El Síndrome de POEMS (polineuropatía, organomegalia, endocrinopatía, pico gamma monoclonal ycambios en la piel) es una discrasia de células plasmáticas que se manifiesta con un característicoconjunto de trastornos para neoplásicos. Los cambios en la piel, como hiperpigmentación cutáneageneralizada, son debidos al aumento en la secreción del factor de crecimiento del endotelio vascular.Se reporta el caso de una mujer de 67 años de edad que se presentó con astenia, adinamia y uncuadro importante de anemia, asociados a diabetes mellitus, hipotiroidismo y polineuropatía motora ysensitiva de moderada intensidad. Sus cambios en la piel consistieron en hiperpigmentación, manchasvioláceas en los labios y acentuación de los pliegues de las manos. Los estudios subsecuentesevidenciaron el pico gamma monoclonal y la polineuropatía axonal sensitivo-motora en elelectromiograma. Se brindó el tratamiento quimioterapéutico correspondiente pero sufriócomplicaciones mielosupresoras por esta razón, desarrollo una neumonía intrahospitalaria y falleció aconsecuencia.

The POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes)is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. The skinchanges, as generalized skin hyperpigmentation, are related with elevated levels of vascularendothelial growth factor. We report a clinical case of 67 years old woman who presented withasthenia, adynamia and important anemia, associated to diabetes mellitus, hypothyroidism and amoderated intensity motor-sensitive polyneuropathy. The skin changes presented by this patientwere hyperpigmentation, violet spots in the lips and accentuation of the lines in the palm of the hand.Subsequent analysis showed the M–protein and the electromyography showed a motor-sensitivepolyneuropathy in both legs. Adequate chemotherapy was given but mielosuppressive effects wereshown, she acquired nosocomial pneumonia and died as consequence.

Actividad espontánea de nociceptores cutáneos en pacientes con neuropatía de fibras delgadas / Spontaneous activity of cutaneous nociceptors in patients with painful polyneuropathy. Report of three patients

Background: Painful polyneuropathy may result from selective impairment of small diameter nerve fibers, while tactile and motor functions are preserved. In these patients clinical and electrophysiological assessment is usually unrevealing. We report three patients with a pure painful polyneuropathy. One of them had neurogenic pruritus additionally. Quantitative sensory analysis disclosed a slight warm hypoesthesia (3/3) and paradoxical hot sensation (2/3) in the feet. Intraneural recordings from the peroneal nerve demonstrated abnormal spontaneous activity in 8 of 17 nociceptive afferents. One of them displayed double firing reflecting impulse multiplication. These results support the notion that patients with pain or pruritus with a distal distribution similar to a polyneuropathy, could have small diameter afferent fiber damage, despite normal function of large diameter fibers.

Carpal Tunnel Syndrome and Peripheral Polyneuropathy in Patients with End Stage Kidney Disease

This study was designed to identify the causes of the development of carpal tunnel syndrome (CTS) associated with end stage kidney disease (ESKD). A total of 112 patients with ESKD, 64 on hemodialysis (HD) and 48 on peritoneal dialysis (PD), were enrolled. The duration of ESKD and dialysis, the site of the arteriovenous (A-V) fistula for HD, laboratory data such as blood urea nitrogen, creatinine, and beta-2-microglobulin were determined. Clinical evaluation of CTS and electrophysiological studies for the diagnosis of CTS and peripheral neuropathy were performed. The electrophysiological studies showed that the frequency of CTS was not different in the HD and PD groups (P = 0.823) and the frequency of CTS was not different in the limb with the A-V fistula compared to the contralateral limb (P = 0.816). The frequency of HD and PD were not related to beta-2-microglobulin levels, an indicator of amyloidosis. The frequency of CTS did not increase as the severity of the peripheral neuropathy and the duration of ESKD and dialysis increased (P = 0.307). The results of this study do not support that microglobulin induced amyloidosis or placement of an A-V fistula are associated with an increase in CTS.

The role of sensory nerve conduction study of the palmar cutaneous nerve in the diagnosis of carpal tunnel syndrome in patients with polyneuropathy.

BACKGROUND: Conventional methods in the diagnosis of carpal tunnel syndrome (CTS) in patients with polyneuropathy (PNP) are insufficient. AIMS: We suggest that the comparison of the conduction of the median nerve with that of the neighboring peripheral nerves may be more beneficial in the diagnosis of entrapment neuropathy. SETTING AND DESIGN: The median nerve sensory conduction in healthy volunteers, in cases of CTS, PNP cases without CTS and in cases of PNP in whom clinical findings point to CTS, were compared by palmar cutaneous nerve (PCN) sensory conduction. MATERIALS AND METHODS: Comparative parameters were difference of PCN-1st digits' nerve conduction velocities (NCV), PCN/1st digit NCVs ratio, difference of 5th-2nd digits' NCVs and 5th/2nd digits' NCVs ratio. STATISTICAL ANALYSIS: The statistical analysis was performed by the SPSS package for statistics. Student t test and receiver operating characteristic were used. RESULTS: Although the ratio of PCN-1st digit did not differ significantly between the control group and the polyneuropathy group, there was a significant difference between CTS and PNP+CTS groups and the control group ( P < 0.001 and P < 0.001, respectively). The ratio of PCN-1st digit nerve conduction velocity was also significantly different between polyneuropathy and PNP+CTS groups ( P < 0.001). CONCLUSION: To diagnose CTS on a background of polyneuropathy in mild cases in which sensory conduction is preserved, the ratio of sensory nerve conduction velocities of the palmar cutaneous nerve and the median nerve 1st digit-wrist segment may be a criterion.

Critical illness polyneuropathy: how often do we diagnose it?

Neuromuscular weakness in critically ill patients is a diagnostic challenge. Critical illness polyneuropathy, an important cause of failure to wean from assisted ventilation is often missed due to lack of suspicion and initiative to undertake regular bedside neurological and electrophysiological examinations in critically ill patients. We report two cases who developed motor weakness while receiving mechanical ventilation in whom axonal neuropathy was diagnosed on electrophysiological studies, establishing a diagnosis of critical illness polyneuropathy. Both patients had evidence of sepsis and multiorgan failure. One case could be successfully weaned off and weakness improved while other succumbed to the underlying illness.

Asymmetrical polyneuropathy associated with Churg-Strauss syndrome.

A 26-year-old man who presented with fever, leg edema, weakness and numbness of both hands and feet and painless palpable purpura was reported. He had had a history of asthma and arthalgia before this admission. Physical examination revealed distal asymmetrical polyneuropathy. Electrodiagnostic study was consistent with polyneuropathy. Skin biopsy revealed vasculitis with prominent eosinophilic infiltration. His motor power was markedly improved by prednisolone treatment.