Subject(s)
Humans , Infant , Child, Preschool , Child , Polyneuropathies/diagnosis , Vision Disorders/diagnosis , Epilepsies, Partial/diagnosis , Xanthogranuloma, Juvenile/diagnosis , Neurofibromatoses/complications , Neurofibromatoses/diagnosis , Neurofibromatoses/diagnostic imaging , Meningioma/diagnosis , Intellectual Disability/diagnosis , Neurilemmoma/diagnosis , Diagnosis, DifferentialABSTRACT
Colorectal cancer is one of the most common oncological diseases. Chemotherapy is usually recommended as an adjuvant treatment for stage-II, -III, and -IV tumors. Approximately 10% of the patients develop neuropathic pain after chemotherapy, and they may remain refractory despite the administration of drugs that are commonly used to treat neuropathic pain. Spinal cord stimulation is a good treatment option for neuropathic pain of the lower limbs, and it should be trialed in patients with chemotherapy-induced peripheral neuropathy. We report the case of a patient with oxaliplatin-induced neuropathy and neuropathic pain refractory to oral medication who was successfully treated by spinal cord stimulation.
Subject(s)
Humans , Female , Middle Aged , Polyneuropathies/surgery , Polyneuropathies/diagnosis , Polyneuropathies/chemically induced , Spinal Cord Stimulation/methods , Colorectal Neoplasms, Hereditary Nonpolyposis/epidemiology , Chemotherapy, Adjuvant , Peripheral Nervous System Diseases/therapy , Cancer PainABSTRACT
A vasculopatia livedoide é uma doença rara caracterizada pela oclusão da microvasculatura da derme, originando lesões maculosas que, posteriormente, podem evoluir para úlceras e cicatrizes atróficas. Como um fenômeno vaso-oclusivo, o tratamento geralmente é realizado com antiplaquetários e fibrinolíticos. O presente relato descreve o caso de uma paciente refratária à terapia convencional, que obteve regressão da doença utilizando a rivaroxabana, um fármaco inibidor seletivo do fator Xa. (AU)
Livedoid vasculopathy is a rare disease characterized by occlusion of the dermis microvasculature, leading to spotted lesions that can later develop into ulcers and atrophic scars. As a vaso- occlusive phenomenon, treatment is usually performed with antiplatelet and fibrinolytic agents. The present report describes the case of a female patient refractory to conventional therapy who presented disease remission using rivaroxaban, a selective factor Xa inhibitor drug. (AU)
Subject(s)
Humans , Female , Middle Aged , Thrombosis/drug therapy , Skin Diseases, Vascular/drug therapy , Thrombotic Microangiopathies/drug therapy , Rivaroxaban/therapeutic use , Livedoid Vasculopathy , Paresthesia , Pentoxifylline/therapeutic use , Polyneuropathies/diagnosis , Thrombosis/complications , Vasodilator Agents/therapeutic use , Biopsy , Platelet Aggregation Inhibitors/therapeutic use , Nifedipine/therapeutic use , Fibromyalgia , Skin Diseases, Vascular/complications , Skin Diseases, Vascular/diagnosis , Connective Tissue Diseases/complications , Lower Extremity/injuries , Electromyography , Thrombotic Microangiopathies/complications , Factor Xa Inhibitors/therapeutic use , Foot/pathology , Diverticular Diseases , Smokers , Gabapentin/therapeutic use , Analgesics/therapeutic useABSTRACT
ABSTRACT Objective: To determine the sensitivity and specificity of peripheral and respiratory muscle strength tests in diagnosing critical illness polyneuromyopathy (CIPNM), compared with an electrophysiological examination. Methods: Fifty septic patients who required mechanical ventilation for at least five days, and without a previous history of muscle weakness, were included. Peripheral muscle strength was assessed using the Medical Research Council (MRC) score, handgrip strength by dynamometry, and respiratory muscle strength with maximum respiratory pressures. Diagnosis of CIPNM was either confirmed or rejected by an electrophysiological examination. Receiver operating characteristic curve analysis was performed to determine the cut-off values with the best sensitivity (SN) and specificity (SP) of the studied variables in the presence or absence of CIPNM. Results: Patients with CIPNM were older, more critical (APACHE IV/SAPS 3), had a longer hospitalization, required mechanical ventilation for longer, and had a higher rate of intensive care unit readmission. Cutoff values identified CIPNM patients using MRC scores, dynamometry according to sex, maximal expiratory and inspiratory pressures, as well as being confirmed by the electrophysiological examination, with good sensitivity and specificity: < 40 (SN: 0.893; SP: 0.955); < 7 kg (SN: 1; SP: 0.909) for men, < 4 kg (SN: 0.882; SP: 1) for women; < 34 cmH2O (SN: 0.808; SP: 0.909) and > −40 cmH2O (SN: 0.846; SP: 0.909), respectively. Conclusion: The MRC score, dynamometry or maximum respiratory pressures can be used to identify patients with CIPNM at the intensive care bedside assessment. The healthcare professional can choose any of the methods studied to evaluate the patient, based on his experience and the resource available.
RESUMO Objetivo: Determinar a sensibilidade (SN) e especificidade (SP) dos testes de força muscular periférica e respiratória no diagnóstico da Polineuromiopatia do Doente Crítico (PNDC) em comparação com o estudo eletrofisiológico. Métodos: Foram incluídos 50 pacientes sépticos, em ventilação mecânica (VM) durante pelo menos cinco dias e sem história prévia de fraqueza muscular. A força muscular foi avaliada utilizando o escore Medical Research Council (MRC), a força de preensão palmar e as pressões respiratórias máximas. O diagnóstico de PNDC foi confirmado ou excluído pelo estudo eletrofisiológico. A análise da curva ROC foi realizada para determinar os valores de corte com a melhor SN e SP. Resultados: Os pacientes com PNDC eram mais velhos, mais graves, tiveram hospitalização mais longa, necessitaram de VM por mais tempo e apresentaram maior taxa de readmissão na Unidade de Terapia Intensiva. Os valores de corte identificaram os pacientes com PNDC usando o MRC, a dinamometria de acordo com o sexo, as pressões expiratórias e inspiratórias máximas, também confirmado pelo estudo eletrofisiológico, com boa sensibilidade e especificidade: < 40 (SN: 0.893; SP: 0.955), < 7 kg (SN: 1; SP: 0,909) para homens, < 4 kg (SN: 0,882; SP: 1) para mulheres, <34 cmH2O (SN: 0,808; SP: 0,909) e > −40 cmH2O (SN: 0,846; SP: 0,909), respectivamente. Conclusão: Tanto o MRC, a dinamometria quanto as pressões respiratórias máximas podem ser usadas para identificar pacientes com PNDC na avaliação à beira do leito, podendo o profissional de saúde escolher qualquer um dos métodos baseado em sua experiência e no recurso disponível.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Polyneuropathies/diagnosis , Polyneuropathies/physiopathology , Critical Illness , Sepsis/physiopathology , Reference Values , Respiratory Muscles/physiopathology , Prospective Studies , Reproducibility of Results , Sensitivity and Specificity , Statistics, Nonparametric , APACHE , Electromyography/methods , Muscle Strength/physiology , Muscle Strength Dynamometer , Point-of-Care Testing , Maximal Respiratory Pressures , Intensive Care UnitsABSTRACT
A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)
POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)
Subject(s)
Humans , Male , Middle Aged , POEMS Syndrome/diagnosis , Osteosclerosis/etiology , Paraproteinemias/etiology , Polyneuropathies/diagnosis , Splenomegaly/diagnostic imaging , Thyroid Diseases/diagnostic imaging , Dexamethasone/therapeutic use , Castleman Disease , POEMS Syndrome/complications , POEMS Syndrome/drug therapy , Adrenal Cortex Hormones/therapeutic use , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Angiogenesis Inhibitors/therapeutic use , Cyclophosphamide/therapeutic use , Diagnosis, Differential , Electromyography , Immunosuppressive Agents/therapeutic use , Antibiotics, Antineoplastic/therapeutic use , Multiple Myeloma , Antineoplastic Agents/therapeutic useABSTRACT
ABSTRACT Objective This cross sectional study intended to evaluate two bedside tests (Neuropad and VibraTip) as screening tools for distal symmetrical polyneuropathy (DSPN) in Latin American patients with type 2 diabetes mellitus (T2D). Subjects and methods Ninety-three Colombian patients diagnosed with T2D were recruited. Anthropometric variables, glycemic control parameters, lipid profile and renal function were assessed for each patient. DSPN was defined by a Michigan Neuropathy Screening Instrument (MNSI) clinical score greater than 2. Both Neuropad and Vibratip tests were applied to each patient. Contingency analyses were performed to evaluate the diagnostic power of both tools. Results The prevalence of DSPN determined clinically by MNSI was 25.8%. DSPN in these patients was associated with age, worsening renal function, and insulin treatment. The sensitivity and specificity of the Neuropad test for DSPN was 66.6% and 63% respectively. Its negative predictive value (NPV) was 84.6%. The VibraTip test exhibited a sensitivity of 54.1% and specificity of 91.3%, with a NPV of 85.1%. Conclusion Neuropad and VibraTip are reliable screening tools for DSPN in Latin American population. VibraTip presents a considerable diagnostic power for DSPN in this population. Further studies regarding the cost-effectiveness of these tools in clinical practice are needed.
Subject(s)
Humans , Male , Female , Aged , Polyneuropathies/diagnosis , Point-of-Care Systems , Diabetes Mellitus, Type 2 , Diabetic Neuropathies/diagnosis , Diagnostic Techniques, Neurological/instrumentation , Cross-Sectional Studies , Predictive Value of Tests , Sensitivity and Specificity , ColombiaABSTRACT
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.
Subject(s)
Humans , Polyneuropathies/physiopathology , Arthritis, Rheumatoid/physiopathology , Foot Deformities , Health Status , Polyneuropathies/complications , Polyneuropathies/diagnosis , Arthritis, Rheumatoid/complications , Severity of Illness Index , Activities of Daily Living , Foot/innervation , Neural Conduction/physiologyABSTRACT
Entecavir (Baraclude®) is an oral antiviral drug used for the treatment of HBV. Entecavir is a reverse transcriptase inhibitor which prevents the HBV from multiplying. Most common adverse reactions caused by entecavir are headache, fatigue, dizziness, and nausea. Until now, there has been no report of peripheral neuropathy as a side effect associated with entecavir treatment. Herein, we report a case of peripheral neuropathy which probably occurred after treatment with entecavir in a hepatitis B patient. The possibility of the occurrence of this side effect should be carefully taken into consideration when a patient takes a high dose of entecavir for a long period of time or has risk factors for neuropathy at the time of initiating entecavir therapy.
Subject(s)
Humans , Male , Middle Aged , Administration, Oral , Antiviral Agents/adverse effects , Brain/diagnostic imaging , Drug Therapy, Combination , Duloxetine Hydrochloride/therapeutic use , Glucocorticoids/therapeutic use , Guanine/adverse effects , Hepatitis B, Chronic/drug therapy , Polyneuropathies/diagnosis , Prednisolone/therapeutic use , Pregabalin/therapeutic use , Tomography, X-Ray ComputedABSTRACT
Os inibidores da 3-hidroxi-3-metilglutaryl coenzima-A (HMG-CoA)redutase têm eficácia comprovada em reduzir os níveis de colesterole prevenir a inflamação do endotélio coronariano, cerebral e periférico.Os efeitos adversos devem ser conhecidos, pois sua suspensão pode levar à completa reversibilidade dos sintomas. São descritas complicações musculares, entre elas, mialgia, miosite e rabdomiólise, além de complicações hepáticas, neuropatias e outras. Foram revistos 1 estudo experimental, 6 estudos populacionais, 25 relatos de casos e 2 revisões sobre o tema, a maioria apontando para a real existência dessa complicação. A neuropatia induzida por estatinas tem incidência aproximada de 12 por 100.000 pessoas-ano. Apresenta-se mais comumente como polineuropatia sensitivo-motora axonal de predomínio sensitivo. Em alguns casos, agravam neuropatias periféricas preexistentes. A fisiopatologia parece convergir para o comprometimento da cadeia respiratória mitocondrial. O diagnóstico baseia-se na relação temporal entre o uso ou suspensão da droga e o surgimento ou melhora dos sinais e sintomas. Os exames laboratoriais são fundamentais para excluir causas de neuropatias periféricas bem estabelecidas. O prognóstico relaciona-se com o momento de suspensão da droga, com relatos desde melhora completa até irreversibilidade.
Inhibitors of coenzyme A 3-hydroxy-3-metilglutaryl (HMG-CoA) reductaseinhibitors have proven to reduce cholesterol levels and prevent inflammation of the coronary, cerebral and peripheral endothelium. Adverse effects should be known, for its suspension can lead to complete reversibility of symptoms. Muscle complications are described, among them, myalgia, myositis and rhabdomyolysis, besides hepatic, neuropathies, and others. One experimental study and 6 population studies, 25 cases reports, and other 2 reviews were reviewed, most pointing to the actual existence of this complication. Statin induced neuropathy has an approximate incidence of 12 per 100,000 persons-year. It most commonly is presented as a sensorimotor axonal polyneuropathy predominantly sensory. In some cases it aggravates pre-existing peripheral neuropathies. The pathophysiology seems to converge to impairment of the mitochondrial respiratory chain. The diagnosis is based on the temporal relationship between the use or discontinuation of the drug and the emergence or improvement of signs and symptoms. Laboratory tests are essential to exclude well established causes of peripheral neuropathies. The prognosis is related to the moment of drug suspension, with reports from complete recovery to irreversibility.
Subject(s)
Humans , Adult , Middle Aged , Aged , Young Adult , Polyneuropathies/diagnosis , Polyneuropathies/chemically induced , Peripheral Nervous System Diseases , Hydroxymethylglutaryl-CoA Reductase Inhibitors/adverse effects , Comorbidity , Risk Factors , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Hypercholesterolemia/chemically inducedABSTRACT
The objective of this study was to evaluate if the ratio of ulnar sensory nerve action potential (SNAP) over compound muscle action potential (CMAP) amplitudes (USMAR) would help in the distinction between ganglionopathy (GNP) and polyneuropathy (PNP). Methods We reviewed the nerve conductions studies and electromyography (EMG) of 18 GNP patients, 33 diabetic PNP patients and 56 controls. GNP was defined by simultaneous nerve conduction studies (NCS) and magnetic resonance imaging (MRI) abnormalities. PNP was defined by usual clinical and NCS criteria. We used ANOVA with post-hoc Tukey test and ROC curve analysis to compare ulnar SNAP and CMAP, as well as USMAR in the groups. Results Ulnar CMAP amplitudes were similar between GNP x PNP x Controls (p=0.253), but ulnar SNAP amplitudes (1.6±3.2 x 11.9±9.1 × 45.7±24.7) and USMAR values (0.3±0.3 × 1.5±0.9 × 4.6±2.2) were significantly different. A USMAR threshold of 0.71 was able to differentiate GNP and PNP (94.4% sensitivity and 90.9% specificity). Conclusions USMAR is a practical and reliable tool for the differentiation between GNP and PNP. .
O objetivo deste estudo foi avaliar se a razão entre as amplitudes dos potenciais de ação sensitivo (SNAP) e motor (CMAP) do nervo ulnar (USMAR) auxiliaria na distinção entre ganglionopatia (GNP) e polineuropatia (PNP). Métodos Revisamos os estudos de neurocondução e eletromiografia de 18 pacientes com GNP, 33 com PNP diabética e 56 controles. GNP foi definida pela presença simultânea de anormalidades na neurocondução e na ressonância magnética cervical. PNP foi definida por critérios clínicos e neurofisiológicos usuais. Usamos o teste ANOVA com Tukey post-hoc e análise da curva ROC para comparar o SNAP e CMAP ulnares, assim como o USMAR entre os grupos. Resultados As amplitudes dos CMAPs ulnares foram similares entre GNP × PNP × Controles (p=0,253), mas as amplitudes dos SNAPs ulnares (1,6±3,2 × 11,9±9,1 × 45,7±24,7) e os valores de USMAR (0,3±0,3 × 1,5±0,9 × 4,6±2,2) foram significativamente diferentes. Um corte de 0,71 para a USMAR foi capaz de diferenciar GNP de PNP (sensibilidade de 94,4% e especificidade de 90,9%). Conclusões A USMAR é um parâmetro útil e confiável para o diagnóstico diferencial entre GNP e PNP. .
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Ganglia/physiopathology , Polyneuropathies/diagnosis , Ulnar Nerve/physiopathology , Analysis of Variance , Action Potentials/physiology , Case-Control Studies , Diagnosis, Differential , Diabetic Neuropathies/diagnosis , Electromyography , Neural Conduction/physiology , Reproducibility of Results , ROC CurveABSTRACT
Guillain-Barre Syndrome [GBS] is the most common disease resulting in acute diffuse flaccid paralysis. It is an autoimmune disease that can occur at any age. The clinical course is characterized by weakness in the arms and legs, areflexia and the progression of muscle weakness from the lower limbs to the upper limbs. The most common causes of GBS include infections, vaccinations, surgery and some medicines. We present the case of a 48 years old male patient, who developed GBS after undergoing surgery for renal calculus, under spinal anaesthesia. In this case report, we presented a rather rare case of GBS occurring following spinal anaesthesia
Subject(s)
Humans , Male , Anesthesia, Spinal/adverse effects , Polyneuropathies/diagnosis , Spinal Cord Injuries/complications , Muscle Weakness/etiology , ElectromyographyABSTRACT
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.
Subject(s)
Adult , Female , Humans , Gastric Bypass , Polyneuropathies/etiology , Postoperative Complications/etiology , Vitamin B Deficiency/etiology , Wernicke Encephalopathy/etiology , Anxiety Disorders/diagnosis , Brain Damage, Chronic/diagnosis , Brain Damage, Chronic/etiology , Coma/diagnosis , Coma/etiology , Diagnostic Errors , Factitious Disorders/diagnosis , Hypothyroidism/complications , Mental Disorders/complications , Obesity, Morbid/complications , Obesity, Morbid/surgery , Prognosis , Polyneuropathies/diagnosis , Postoperative Complications/diagnosis , Postoperative Nausea and Vomiting/complications , Risk Factors , Urinary Tract Infections/complications , Vitamin B Complex/pharmacokinetics , Vitamin B Deficiency/diagnosis , Wernicke Encephalopathy/diagnosis , Wernicke Encephalopathy/drug therapy , Wernicke Encephalopathy/physiopathologyABSTRACT
Myopathy is an important complication of thyrotoxicosis. Neuropathy is a less commonly reported complication, and is often subclinical. Here we report a patient of throtoxic myopathy with sub-clinical entrapment neuropathy. This case is reported to emphasise the importance of NCV to look for neuropathy.
Subject(s)
Female , Humans , Middle Aged , Peripheral Nerves/pathology , Peripheral Nerves/physiopathology , Polyneuropathies/diagnosis , Polyneuropathies/physiopathology , Thyrotoxicosis/complications , Thyrotoxicosis/physiopathologyABSTRACT
Acute sensorimotor polyneuropathy that resembles Guillain-Barre syndrome (GBS) is rarely accompanied with nephrotic syndrome, and its underlying immunological mechanisms are unclear. A 56-year-old man presented with simultaneous acute progressive symmetric sensorimotor polyneuropathy and proteinuria. A kidney biopsy revealed focal segmental glomerulosclerosis. Serial electrophysiologic studies showed only a transient proximal conduction block in the median nerve, stimulated somatosensory evoked potential and prolonged terminal latencies of the median and peroneal nerves. The patient's neurologic deficits and kidney dysfunction recovered with corticosteroid treatment. Our case showed that somatosensory evoked potential study can be an important objective tool in the diagnosis of acute polyneuropathy with normal distal nerve conduction and that corticosteroids should be considered in the initial treatment of GBS-resembling polyneuropathy associated with nephrotic syndrome.
Subject(s)
Humans , Male , Middle Aged , Evoked Potentials, Somatosensory/physiology , Nephrotic Syndrome/diagnosis , Polyneuropathies/diagnosisABSTRACT
Four children with vincristine (VCR)-induced neuropathy are being reported. All cases were followed with the diagnosis of acute lymphoblastic leukemia. Two were boys aged between 2 and 13 year. Electromyographic examination consisted of sensoriomotor polyneuropathy with axonal involvement in three patients. In another patient, it consisted of motor axonal polyneuropathy. In all patients, pyridoxine and pyridostigmine were successfully used in the treatment of VCR-induced neuropathy. They recovered completely with this drug combination. Recovering period of symptoms was between 1-2 week.
Subject(s)
Adolescent , Antineoplastic Agents, Phytogenic/administration & dosage , Antineoplastic Agents, Phytogenic/adverse effects , Child , Child, Preschool , Cholinesterase Inhibitors/therapeutic use , Drug Therapy, Combination , Female , Humans , Male , Polyneuropathies/chemically induced , Polyneuropathies/diagnosis , Polyneuropathies/drug therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Pyridostigmine Bromide/therapeutic use , Pyridoxine/therapeutic use , Treatment Outcome , Vincristine/administration & dosage , Vincristine/adverse effects , Vitamin B Complex/therapeutic useABSTRACT
Introdução: A polineuropatia periférica é uma das 19 síndromes neuropsiquiátricas do lúpus eritematoso sistêmico, segundo os critérios de classificação propostos pelo Colégio Americano de Reumatologia (ACR) para síndromes neuropsiquiátricas. No entanto, há descrições raras dessa manifestação, particularmente em populações de lúpus eritematoso sistêmico juvenil (LESJ). Métodos: De 1983 a 2007, 5.079 pacientes foram acompanhados na Unidade de Reumatologia Pediátrica do ICrûHCûFMUSP, e o diagnóstico de LESJ segundo os critérios do ACR foi estabelecido em 228 casos (4,5%). Polineuropatia periférica foi diagnosticada de acordo com as síndromes neuropsiquiátricas do ACR. Resultados: Dos 228 pacientes com LESJ, cinco (2,2%) desenvolveram polineuropatia periférica e foram retrospectivamente descritos. O diagnóstico foi confirmado por eletroneuromiografia, que evidenciou polineuropatia periférica distal, sensitiva e/ou motora, envolvendo quatro membros em dois pacientes e membros inferiores nos demais. Três eram pacientes do sexo feminino e todos tiveram a polineuropatia periférica após o diagnóstico de LESJ. A mediana de idade de início da doença foi de 14 anos, e a mediana de tempo entre o início de LESJ e o diagnóstico da polineuropatia periférica foi de 23 meses. As apresentações clínicas mais comuns foram fraqueza muscular e hiporreflexia. Todos os pacientes apresentavam anticorpos antifosfolípides. O tratamento foi realizado com corticosteroides em todos os pacientes, associado com ciclofosfamida endovenosa em três. Um paciente evoluiu com incapacitação funcional, presença de paresia de membros inferiores e necessidade de cadeira de rodas. Uma paciente faleceu por sepse grave. Conclusões: A polineuropatia periférica é uma manifestação rara no LESJ, grave, por vezes incapacitante e habitualmente associada a anticorpos antifosfolípides.
INTRODUCTION: Peripheral polyneuropathy is one of 19 neuropsychiatric syndromes seen in systemic lupus erythematosus, according to the classification criteria proposed by the American College of Rheumatology (ACR) for neuropsychiatric syndromes. However, this manifestation has not been reported very often, especially in patients with juvenile systemic lupus erythematosus (JSLE). PATIENTS AND METHODS: From 1983 to 2007, 5,079 patients were seen at the Pediatric Rheumatology Unit of the ICr-HC-FMUSP; 228 (4.5%) patients were diagnosed with JSLE according to the criteria of the ACR. Peripheral polyneuropathy was diagnosed according to the criteria for neuropsychiatric syndromes of the ACR. RESULTS: Five (2.2%) out of 228 patients with JSLE developed peripheral polyneuropathy and were described retrospectively. The diagnosis was confirmed by electroneuromyography, which showed the presence of distal peripheral polyneuropathy, sensorial and/or motor, involving all four limbs, in two patients, and the lower limbs, in three patients. Three of those patients were females, and peripheral neuropathy developed after the diagnosis of JSLE. The mean age of onset of the disease was 14 years, and the mean time between the onset of JSLE and the diagnosis of peripheral polyneuropathy was 23 months. The most common clinical presentations included muscular weakness and hyporeflexia. Antiphospholipid antibodies were present in all patients. Treatment consisted of corticosteroids in all patients, associated with intravenous cyclophosphamide in three patients. One patient evolved to functional disability and paresis of the lower limbs, requiring a wheelchair. One female patient died of severe sepsis. CONCLUSIONS: Peripheral polyneuropathy is a rare, severe, and occasionally incapacitating manifestation of JSLE, commonly associated with the presence of antiphospholipid antibodies.
Subject(s)
Humans , Male , Female , Child , Adolescent , Antiphospholipid Syndrome , Autoimmune Diseases , Lupus Erythematosus, Systemic , Polyneuropathies , Polyneuropathies/diagnosisABSTRACT
Six Doberman Pinscher, between six and eight years of age, were presented to the Veterinary Hospital from Faculty of Veterinary Science of The University of Buenos Aires. Neurological examination revealed tetraparesis with inability to walk, decreased muscle tonus and myotatic reflexes in all dogs. Serum cholesterol levels, creatine kinase and alkaline phosphatase activities were mildly to markedly elevated, and tibial motor nerve conduction velocities were slow in all dogs. Basal measurements of free T4 and TSH were determined by radioimmunoassay. Although fT4 values were within normal range, in all dogs TSH values were elevated. Based on this results, hypothyroidism was diagnosed and a supplementation therapy was established with oral levothyroxine (T4). Two weeks after treatment has been started, all patients had an improvement in clinical signs, and within a month gait became normal, as well as muscular tonus and spinal reflexes.
Seis Dobermans Pinscher, entre seis e oito anos de idade, foram encaminhados ao Hospital Veterinário da Faculdade de Ciências Veterinárias da Universidade de Buenos Aires. O exame neurológico revelou tetraparesia com incapacidade para andar, diminuição do tono muscular e de reflexos miotáticos em todos os cães. Os níveis de colesterol, creatina quinase e fosfatase alcalina encontravam-se de moderada a acentuadamente elevados. As velocidades de condução nervosa do nervo tibial estavam diminuídas em todos os cães. Os níveis séricos basais de T4 livre e TSH foram determinados por radioimunoensaio e, embora os valores de T4 livre estivessem dentro dos limites de normalidade, em todos os cães o TSH estava elevado. Baseado nestes resultados, diagnosticou-se hipotireoidismo e iniciou-se uma suplementação oral com levotiroxina (T4). Duas semanas após o início do tratamento todos os pacientes tiveram melhora clínica e, dentro de um mês a locomoção, o tono muscular e os reflexos medulares tornaram-se normais.
Subject(s)
Animals , Dogs , Hypothyroidism/complications , Polyneuropathies/diagnosis , Thyroxine/analysisABSTRACT
JUSTIFICATIVA E OBJETIVOS: O objetivo deste estudo foi apresentar um relato de caso pouco comum. RELATO DO CASO: Paciente do sexo feminino com diagnóstico de polirradiculoneuropatia inflamatória crônica (PDIC) havia cinco anos; há um ano evoluindo com perda progressiva da visão e peso e, há cerca de seis meses, necessitou punções liquóricas de repetição para alívio de sintomas de hipertensão intracraniana, sem etiologia definida. Ao exame físico detectou-se hiperfonese de 2a bulha, hepatoesplenomegalia e hiperpigmentação da pele, principalmente da palma das mãos e planta dos pés. Foi considerada a hipótese de síndrome de POEMS, confirmada após laudo de mielograma, evidenciando predomínio de plasmócitos e presença linhagem monoclonal, com gamopatia por IgA. CONCLUSÃO: Este relato apresentou um caso de síndrome de POEMS rico em manifestações típícas e atípicaso Um dos diagnósticos diferenciais mais importantes é a polineuropatia inflamatória desmielizante crônica. A distinção entre as duas doenças foí fundamental para a adequada escolha do tratamento