Polineuropatía por amiloidosis por transtiretina de inicio tardío. Caso clínico / Late-onset hereditary transthyretin amyloidosis with polyneuropathy. Report of one case

Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.

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.

Polineuropatia hipotireóidea em um paciente com síndrome poliglandular autoimune tipo 2: relato de caso / Hypothyroid polyneuropathy in a patient with autoimmune polyglandular syndrome type 2: case report

A incidência de polineuropatia em indivíduos com hipotireoidismo não é precisamente conhecida, mas alguns estudos relatam que cerca de 25% a 42% dos pacientes podem apresentar sinais clínicos neuropáticos. A seguir, relataremos um caso de síndrome poliglandular autoimune tipo 2 (SPA-2), cuja apresentação inicial foi uma polineuropatia hipotireóidea. Homem de 41 anos com queixas de parestesias e fraqueza lentamente progressiva acometendo os quatro membros associadas a sonolência frequente, astenia, intolerância ao frio, vertigens, náuseas e avidez por sal. O exame físico geral evidenciava hiperpigmentação de pele e mucosas, além de hipotensão. O exame neurológico demonstrou apenas hiporreflexia profunda global e simétrica com discretos sinais de hipoestesia superficial em extremidades dos membros. O estudo eletroneuromiográfico (ENMG), juntamente com a avaliação laboratorial, confirmou a suspeita de tireoidite de Hashimoto associada à doença de Addison, caracterizando o quadro de SPA-2. O paciente foi tratado com fludrocortisona 0,05 mg/dia e levotiroxina 100 mcg/dia e apresentou resolução gradual e completa das queixas e das alterações encontradas nos exames físico geral e neurológico. O ENMG, repetido após seis meses, evidenciou resolução completa do quadro neuropático. Este relato mostra um caso raro de SPA-2 apresentando-se como uma polineuropatia hipotireóidea e reforça a relevância da dosagem de hormônios tireoideanos em síndromes polineuropáticas. A reposição de levotiroxina mostrou-se efetiva em reverter o quadro clínico e eletrofisiológico da neuropatia. .

The incidence of polyneuropathy in patients with hypothyroidism is not precisely known, but some studies report that about 25% to 42% of patients may show neuropathic clinical signs. We report a case of autoimmune poliglandular syndrome type 2 (APS-2), whose initial presentation was hypothyroid polyneuropathy. A 41-year-old man complained of slowly progressive paresthesias and weakness affecting all four limbs, and associated with frequent drowsiness, weakness, cold intolerance, dizziness, nausea, and craving for salt. General physical examination showed hyperpigmentation of skin and mucous membranes, and hypotension. Neurological examination showed global, deep, and symmetrical hyporeflexia with slight signs of superficial hypoesthesia in the limbs. Electrodiagnostic studies (ENMG) together with laboratory tests, confirmed the suspicion of Hashimoto’s thyroiditis associated with Addison’s disease featuring the picture of APS-2. The patient was treated with fludrocortisone 0.05 mg/day and levothyroxine 100 mcg/day, and showed gradual and complete resolution of complaints. Changes were found in general physical and neurological examinations. ENMG repeated six months later showed complete resolution of neuropathy. This report shows a rare case of APS-2 presented as polyneuropathy hypothyroidism, and reinforces the importance of dosing thyroid hormone in polyneuropathy syndromes. Levothyroxine replacement was shown to be effective in reversing clinical and electrophysiologic neuropathy.

Polineuropatia sensitiva induzida por talidomida e estudo eletrofisiológico do nervo sural como forma de screening diagnóstico: relato de caso / Thalidomide-induced sensory polyneuropathy and electrophysiological study of the sural nerve as a screening diagnosis: a case report

Polineuropatia é uma condição clínica freqüente com sintomatologia debilitante e o tratamento depende fundamentalmente da etiologia. Inúmeras são as causas possíveis deste tipo de distúrbio e o diagnóstico etiológico nem sempre é fácil. Neste relato de caso descrevemos um caso de um paciente com mieloma múltiplo que evoluiu com polineuropatia puramente sensitiva, comprovadapor estudo eletrofisiológico, induzida por talidomida.

Polyneuropathy is a common clinical condition with debilitating symptoms whose treatment dependson etiology. There are numerous possible causes of this type of disorder and the etiologicaldiagnosis is not always easy. In this case report we describe a case of a patient with multiple myeloma who developed purely sensory polyneuropathy, confirmed by electrophysiological study, induced by thalidomide.

Encefalopatía de Wernicke y polineuropatía asociada a déficit de complejo B después de una cirugía bariátrica / Wernicke´s encephalopathy and polyneuropathy associated with vitamin B complex deficiency after a bariatric surgery

La cirugía bariátrica es un tratamiento que garantiza una pérdida de peso sustancial y duradera, y beneficios tangibles respecto a condiciones médicas asociadas a la obesidad. El aumento del número de cirugías bariátricas ha llevado también a un aumento de las complicaciones relacionadas con ella, incluyendo la encefalopatía de Wernicke y la polineuropatía por deficiencia de vitaminas del complejo B. En este artículo se reporta un caso de encefalopatía de Wernicke siete semanas después de la cirugía, enfatizando en la importancia de reconocer el espectro de la sintomatología para hacer un diagnóstico temprano, que permita intervenir en la fase reversible de esta enfermedad potencialmente letal.

Bariatric surgery is a treatment that guarantees a substantial and lasting weight loss in addition to the tangible benefits relating to obesity-associated medical conditions. The increasing number of bariatric surgeries has revealed an increasing number of complications related to this procedure, including Wernicke´s encephalopathy and vitamin B deficiency polyneuropathies. Herein, a 7-week post-surgery case of Wernicke´s encephalopathy is presented that emphasizes the importance of an early recognition of these symptoms so as to initiate intervention during the reversible phase of these potentially lethal pathologies.

Evolución neurológica y funcional en pacientes con polineuropatía de paciente crítico: revisión de literatura y reporte de casos / Neurological and functional evolution in critical illness polyneuropathy: literature review and report of cases

Patients with prolonged ICU stay may have a critical illness polyneuropathy (CIP) which corresponds to an acute sensory-motor axonal neuropathy that occurs in severely ill patients between 60 to 100 percent of cases. While most patients have good recovery, reports suggest that between 30 and 40 percent of patients with critical illness polyneuropathy have long-term sequelae, which generate motor limitations that could impact its functionality. The functional outcome of neurological pathology depends on clinical factors, familiy-social factors and access to rehabilitation. Knowing the functional prognosis of the disease is key to organizing the rehabilitation plans. The current literature only reports of neurological recovery as a factor associated with better function in patients with critical illness polyneuropathy. This article reviews clinical and pathogenic aspects of the CIP, neurological and functional changes described in the literature and presents 6 cases of patients admitted to rehabilitation last year.

Acute demyelinating encephalomyelitis with polyneuropathy caused by acute brucella infection: a case report

Brucellosis is a zoonotic infectious illness. It is the commonest zoonotic illness in the world. Our patient, a forty-year-old Saudi lady had fever, diminished level of consciousness, bilateral optic atrophy, bilateral sensorineural deafness and upper motor neurone type paraparesis with bladder involvement. This is an uncommon and rare combination of clinical presentation in acute brucellosis. Brain magnetic resonance imaging [MRI] showed features of acute demyelinating encephalomyelitis [ADEM]. Electrophysiological studies of the extremities revealed motor sensory neuropathy with axonal involvement. To the best of our knowledge, a case of acute brucellosis with such a combination involving both the central and peripheral systems simultaneously in a patient has not been reported before. After appropriate diagnosis our patient responded remarkably well to the treatment

Prednisolone as a new approach for treatment of HTLV-1 associated polyneuropathy

We have previously shown the implication of HTLV-1 in polyneuropathy in a HTLV-1 endemic area in Mashhad. Treatment with corticosteroids [prednisolone and methylprednisolone] have been recommended for HTLV-1 associated diseases. In the current study we attempted to evaluate the efficiency of prednisolone in HTLV-1 associated polyneuropathy. All recognized cases of HTLV-1 polyneuropathy admitted to Imam Reza Hospital [between 1999-2004] were selected for the study. The other common causes of polyneuropathy were excluded using biochemical, hematological, and neurologic examination. Anti-HTLV1 IgG assays were carried out and positive anti-HTLV-1 results were then confirmed by Western Blot Analyzing. All of the HTLV-1 associated polyneuropathy patients were given prednisolone, 1mg/ kg and tapering 5mg/ per week, for three month. Muscle force and paresthesia of all cases were returned to normal pattern. This therapeutic regimen led to reduction of disease severity. Muscle force increased to V/V and paresthesia of all cases were severely decreased. The results of present study suggest that immunosuppressive treatment is very effective for HTLV-1 associated polyneuropathy patients

Cryoglobulinemia presenting as acute polyneuropathy.

A 48 years male presented with acute axonal neuropathy and palpable purpura over bilateral lower limb, RA factor, and cryoglobulins were present in the serum. Nerve biopsy revealed myelinated fibre loss, axonal degeneration and necrotizing vasculitis of epineural vessels.

Polineuropatía en una persona viviendo con vih - sida / Polyneuropathy in a person living with HIV - AIDS

Se expone el caso clínico de un paciente de 31 años de edad, con virus de inmunodeficiencia humana desde hacía 4 años, que había comenzado a presentar fiebre de 39°C, dolor lumbar, calambre con dificultad para la marcha y sepsis urinaria. Los exámenes realizados confirmaron una lesión axonomielínica en fibras sensitivas dístales bilaterales, atribuibles a una polineuropatía axonal desmielinizante subaguda sensitiva. Se administraron 43 g/L de ozono por vía rectal en 20 sesiones, al término de las cuales disminuyó el dolor, desapareció la parestesia y mejoró la marcha, lo cual corroboró la efectividad del ozono como potente inmunomodulador y antimicrobiano, particularmente en el tratamiento de la mencionada enfermedad.

The clinical case of a 31 year-old patient with human immunodeficiency virus for 4 years is reported, who had begun to present with fever of 39°C, lumbar pain, cramps with difficulty for walking and urinary sepsis. Examinations confirmed an axonomyelinic lesion in bilateral distal sensitive fibers attributable to a subacute sensitive demyelinating axonal polyneuropathy. 43 g/L ozone was administered via the rectal route in 20 sessions, at the end of which pain was relieved, paresthesia disappeared and walking improved, thus proving the effectiveness of the ozone as a strong immunomodulator and antimicrobial, particularly in the treatment of this condition.

Polineuropatia periférica dolorosa após cirurgia bariátrica: Relato de casos / Painful peripheral polyneuropathy after bariatric surgery: Case reports

JUSTIFICATIVA E OBJETIVOS: O número de pacientes submetidos à cirurgia bariátrica aumenta a cada ano com a ocorrência, cada vez mais freqüente, de complicações relacionadas com esse procedimento. As principais complicações relatadas são as complicações nutricionais, metabólicas, neurológicas e psicológicas/psiquiátricas. Dentre as complicações neurológicas destacam-se pela sua incidência as neuropatias periféricas. O objetivo desses relatos é chamar a atenção para esse tipo de complicação, que pode ter como manifestação inicial dor tipo neuropática, com posterior ou concomitante envolvimento motor e cujo prognóstico depende de diagnóstico e tratamento precoces. RELATO DOS CASOS: Foram relatados os casos de três pacientes, dois do sexo feminino e um do sexo masculino, submetidos à cirurgia bariátrica (método restritivo - bypass gástrico em Y-de-Roux) que evoluíram, no pós-operatório, com quadro de neuropatia periférica cujo sintoma inicial foi dor neuropática, com posterior ou concomitante envolvimento motor. As eletroneuromiografias demonstraram, nos três casos, uma polineuropatia periférica sensitivo-motora de padrão axonal. Em dois pacientes houve comprometimento intenso dos nervos fibulares. Todos tiveram em comum, antes da manifestação dos sintomas neurológicos, importante perda ponderal num curto período de tempo. CONCLUSÕES: As complicações neurológicas são uma das mais temíveis complicações da cirurgia bariátrica. Nenhum outro fator é mais importante na sua patogenia do que as deficiências nutricionais decorrentes da cirurgia. A vigilância nutricional, evitando-se perdas ponderais acentuadas e rápidas, e a reposição de suplementos são fundamentais para evitar as complicações, sobretudo os quadros neurológicos.

BACKGROUND AND OBJECTIVES: The number of patients undergoing bariatric surgery increases every year, and the frequency of complications associated with this procedure has been increasing. The main complications reported are nutritional, metabolic, neurological, and psychological/psychiatric. Among the neurological complications, peripheral neuropathies are important due to their high incidence. The objective of this report was to stress the importance of this type of complication, in which the initial manifestation might be neuropathic pain followed or accompanied by motor involvement, and whose prognosis depends on early diagnosis and treatment. CASE REPORTS: The case of three patients were reported, two females and one male, who underwent bariatric surgery (restrictive method - Roux-en-Y gastric bypass) and developed, postoperatively, peripheral neuropathy with neuropathic pain as the initial symptom, with posterior or concomitant motor involvement. Electroneuromyography demonstrated in all three cases a sensitive-motor axonal peripheral neuropathy. In two patients, the fibular nerves were severely affected. In common, they all had an important weight loss in a short period of time before developing neurological symptoms. CONCLUSIONS: Neurological complications are one of the most feared complications of bariatric surgeries. Nutritional deficiencies secondary to the surgery are the most important factors in its pathogeny. Nutritional surveillance, avoidance of severe and fast weight loss, and nutritional supplements are fundamental to avoid complications, especially neurological complications.

JUSTIFICATIVA Y OBJETIVOS: El número de pacientes sometidos a la cirugía bariátrica aumenta a cada año con el surgimiento cada vez más frecuente, de complicaciones relacionadas con este procedimiento. Las principales complicaciones relatadas son las complicaciones nutricionales, metabólicas, neurológicas y psicológicas/psiquiátricas. Entre las complicaciones neurológicas se destacan por su incidencia las neuropatías periféricas. El objetivo de estos relatos es destacar este tipo de complicación, que puede tener como manifestación inicial, un dolor tipo neuropático, con posterior o concomitante envolvimiento motor, y cuyo pronóstico depende del diagnóstico y del tratamiento precoces. RELATO DE LOS CASOS: Relatamos los casos de tres pacientes, de los del sexo femenino y uno del sexo masculino, sometidos a cirugía bariátrica (método restrictivo - bypass gástrico en Y de Roux), que evolucionaron en el postoperatorio con un cuadro de neuropatía periférica cuyo síntoma inicial fue dolor neuropático, con posterior o concomitante envolvimiento motor. Las electro neuromiografías demostraron en los tres casos, una Polineuropatía periférica sensitivo-motora de estándar axonal. En de los pacientes hubo un comprometimiento intenso de los nervios fibulares. Todos tuvieron en común, antes de la manifestación de los síntomas neurológicos, una importante pérdida ponderal en un corto período de tiempo. CONCLUSIONES: Las complicaciones neurológicas son una de las más temibles complicaciones de la cirugía bariátrica. Ningún otro factor es más importante en su patogenia que las deficiencias nutricionales provenientes de la cirugía. La vigilancia nutricional, evitando las pérdidas ponderales acentuadas y rápidas, y la reposición de suplementos son fundamentales para evitar las complicaciones, particularmente los cuadros neurológicos.

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.

Early nerve damage in leprosy: an electrophysiological study of ulnar and median nerves in patients with and without clinical neural deficits.

BACKGROUND: Peripheral nerve trunk involvement in leprosy is very common. However, by the time it becomes clinically manifest, the damage is quite advanced. If the preclinical nerve damage can be detected early, the deformities and disabilities can be prevented to a large extent. AIMS: To assess the electrophysiological functions of the ulnar and median nerve trunks in cases of clinically manifest leprosy with and without manifest nerve damage at different durations of nerve damage. MATERIALS AND METHODS: Electrophysiological functions of ulnar and median nerves were studied in leprosy patients, both normal and at different stages of disease and damage. PB cases, having disease for six months or less, without neurological symptoms and clinically normal appearing nerve. STATISTICAL METHODS: Mean was taken of different values. The changes in values of different parameters were expressed as percentage change with reference to the control values (increase or decrease). RESULTS: Reduced nerve conduction velocities and changes in latency and amplitude were observed. Changes in sensory nerve conduction were more pronounced. Sensory latencies and amplitude changes were more severe than motor latencies and amplitudes in cases with manifest muscle palsies. Changes in MB cases were less marked. CONCLUSIONS: Further studies are needed to identify parameters likely to be helpful in the diagnosis of early nerve damage.

peripheral nervous system complications of HIV infection

More than half of HIV-infected persons develop symptomatic neurological disease. The nervous system is extensively involved with no part of the neuraxis being immune from the virus. HIV-associated neuropathies have become the most frequent neurological disorder associated with HIV infection. The most common forms of HIV-associated neuropathies are the distal sensory polyneuropathy [DSP] and antiretroviral toxic neuropathy. Other forms include acute or chronic inflammatory polyneuropathies. Mononeuritis multiplex or radiculopathies may occur in late stages and are mostly associated with opportunistic infections. Furthermore, HIV-related muscle involvement is an uncommon but important complication. This may be due to polymyositis, HIV-associated wasting syndrome or may be aggravated by drugs. The aim of this review is to evaluate the available data on clinical manifestations, pathogenesis, investigations and the therapeutic implication for peripheral nervous system [PNS] neuropathies complications of HIV/AIDS. This review summarises those issues that are likely to confront clinicians, including those who do not routinely treat people infected with HIV

Four cases of polyneuropathy due to HTLV[1] infection in Imam Reza Hospital: North East of Iran

HTLV[1] is the first retrovirus which is known to infect human. HTLV[1] infection has two important manifestations including neurologic and hematologic disorders. We report four cases of HTLV[1] polyneuropathy managed between 1999 and 2004 at Imam Reza Hospital Mashad, [north east of Iran]. In all the four patients the disease started with paresthesia followed by muscle weakness. All patients had areflexia and most of them suffered from distal hypoesthesia

Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery / Polineuropatia axonal aguda com acometimento proximal predominante: manifestação neurológica incomum de cirurgia bariátrica

Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involvement. After treatment with immunoglobulin and vitamin supplementation, rapid clinical and neurophysiologic recovery was observed. We describe the clinical and electroneuromyographic features of this case, stressing the difficulty of initial diagnosis, particularly in the differential diagnosis with Guillain-Barré syndrome. We discuss the importance of nutritional follow-up and the eventual indication of routine vitamin supplementation in these patients.

A cirurgia bariátrica é freqüentemente indicada no tratamento da obesidade mórbida. Complicações previamente não relatadas têm sido associadas a essa cirurgia; dentre estas, as complicações neurológicas têm recebido destaque. Relatamos o caso de um homem de 25 anos de idade submetido a cirurgia gástrica para tratamento de obesidade mórbida que desenvolveu, dois meses após a cirurgia, fraqueza de predomínio proximal nos membros inferiores, de instalação aguda. A eletroneuromiografia demonstrou polineuropatia periférica axonal nos membros inferiores, de predomínio proximal. Após tratamento com imunoglobulina e suplementação vitamínica, apresentou rápida melhora clínica e neurofisiológica. Descrevemos as características clínicas e eletroneuromiográficas desse caso, destacando a dificuldade diagnóstica inicial, particularmente com relação ao diagnóstico diferencial com síndrome de Guillain-Barré. Discutimos a importância de acompanhamento nutricional e a eventual indicação de suplementação vitamínica de rotina nesses pacientes.

Programa de reabilitação fisioterápica para um adolescente com polineuropatia por uso inadvertido de Imizol, confundido com esteróide anabolizante eqüino / Physical therapy rehabilitation program for an adolecent with polyneuropathy by the inadvertent use of Imizol, confounded with equine anabolic steroid

Polineuropatias periféricas são termos que descrevem síndromes surgidas após lesões dos nervos periféricos. Dentre várias formas de polineuropatias, destacam-se as tóxicas por organofosforados, com- postos químicos, utilizados como inseticidas, que ocasionam uma neuropatia crônica de caráter axonal ou desmielinizante. Os principais sintomas localizam-se nas extremidades dos membros e os sinais são motores, sensitivos, autonômicos e mistos. Essa pesquisa caracterizou- se por ser do tipo experimental, com design quasi-experimental. A amostra foi do tipo restritiva, constando de um indivíduo do sexo masculino, 16 anos, residente no interior do RN, com diagnóstico de polineuropatia tóxica axonal e quadro clínico de tetraplegia e paralisia facial periférica. A pesquisa foi realizada na Fisioterapia da Universidade Potiguar e teve como propósito elaborar e implementar um programa de tratamento fisioterápico para um paciente com polineuropatia tóxica por uso de Imizol, verificando seus benefícios. Os resultados dessa pesquisa constataram que a fisioterapia atenuou a gravidade do quadro, pro- moveu sua reabilitação precocemente, e minimizou seqÜelas advindas dessa patologia.

Hepatitis C virus infection, cryoglobulinemia, and peripheral neuropathy: a case report

Hepatitis C virus (HCV) is essentially hepatotropic but its manifestations can extend beyond the liver. It can be associated with autoimmune diseases, such as mixed cryoglobulinemia, membranoproliferative glomerulonephritis, autoimmune thyroiditis, and lymphoproliferative disorders. The mechanisms that trigger these manifestations are not completely understood. We describe a 48-year-old man with chronic HCV infection (circulating HCV RNA and moderate hepatitis as indicated by liver biopsy), cryoglobulinemia, and sensory and motor peripheral neuropathy. The diagnosis of multineuropathy was confirmed by clinical examination and electromyographic tests. A nerve biopsy revealed an inflammatory infiltrate in the perineurial space and signs of demyelination and axonal degeneration. The patient had no improvement of neurological symptoms with the use of analgesics and neuro-modulators. He was then treated with interferon-alpha (3 million units subcutaneously, 3 times per week) and ribavirin (500 mg orally, twice a day) for 48 weeks. Six months after the end of therapy, the patient had sustained viral response (negative HCV RNA) and remission of neurological symptoms, but cryoglobulins remained positive. A review of the literature on the pathogenesis and treatment of neurological manifestations associated with HCV infection is presented. This report underscores the need for a thorough evaluation of HCV-infected patients because of the possibility of extrahepatic manifestations. Antiviral treatment with interferon and ribavirin can be effective and should be considered in patients with neurological complications associated with HCV infection.