A cohort study of vincristine-induced peripheral neuropathy in children / 中国当代儿科杂志

OBJECTIVES@#To study the characteristics of vincristine-induced peripheral neuropathy (VIPN) in children with acute lymphoblastic leukemia (ALL) and the factors influencing the development of VIPN.@*METHODS@#The children with ALL, aged 1-18 years, who were treated with CCCG-ALL2015 or CCCG-ALL2020 regimen in the Affiliated Hospital of Guizhou Medical University from January 2018 to February 2022 were enrolled as subjects. According to the influence of age on risk, the children were divided into 1-10 years group with 91 children and >10 years group with 29 children. VIPN was graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events (5th edition), and the incidence rate, severity, and type of VIPN were compared between different groups.@*RESULTS@#A total of 120 children were enrolled in this study, among whom 56 (46.7%) developed VIPN. The >10 years group had a significantly higher incidence rate of VIPN than the 1-10 years group (69% vs 40%, P<0.05). Among the 56 children with VIPN, 12 (21%) had grade 3 VIPN or above, and 44 (79%) had grade 2 VIPN. There were 77 cases of autonomic nerve symptoms (59.7%), 42 cases of peripheral nerve injury (32.5%), and 10 cases of cranial nerve injury (7.8%). There were no significant differences in the severity and type of VIPN between the groups with different ages, sexes, degrees of risk, or treatment regimens (P>0.05). The results of binary logistic regression analysis showed that age is the influencing factor for the occurrence of VIPN (P>0.05).@*CONCLUSIONS@#There is a relatively high incidence rate of VIPN in children with ALL, with the highest incidence rate of autonomic nervous symptoms. The incidence of VIP in children over 10 years old is relatively high.

Evaluación clínica de las disautonomías / Clinical evaluation of dysautonomia

INTRODUCCIÓN: Disfunción del sistema nervioso autonómico ocurre en enfermedades del sistema nervioso central y periférico. Es importante cuantificar el compromiso simpático y parasimpático, diagnosticar la disfunción, monitorizar la evolución y la respuesta a terapias. Las principales pruebas funcionales son las cardiovasculares y sudomotoras. Existen además exámenes para estudiar la disfunción autonómica en distintos órganos y que son específicos de las especialidades médicas respectivas. DESARROLLO: Se describen los síntomas, las pruebas funcionales y métodos de estudio a nivel cardiovascular: simpáticas vasomotoras (noradrenérgicas) y cardiovagales (colinérgicas) y las pruebas para la sudoración: sudomotoras simpáticas (colinérgicas). Se describen los síntomas y exámenes a nivel pupilar, urogenital y gastrointestinal. Se señala la utilidad de las pruebas funcionales autonómicas en el estudio de distintas patologías neurológicas. CONCLUSIONES: la evaluación conjunta de los hallazgos clínicos y de las pruebas funcionales autonómicas permiten determinar el nivel anatómico y el grado de severidad de la disfunción autonómica con un fundamento fisiopatológico.

INTRODUCTION: Autonomic dysfunction occurs in patients with central and peripheral nervous system diseases. It is important to quantify the sympathetic and parasympathetic involvement for the diagnosis of the autonomic failure, for follow up and evaluate the response to a specific treatment. The most important studies are cardiovascular and sudomotor functional tests. There are other tests for the study of autonomic dysfunction in different organs, that are specific to respectively medical specialty. DEVELOPMENT: we describe main symptoms, functional autonomic tests and other methods to study cardiovascular: sympathetic vasomotor (noradrenergic) and cardiovagal (cholinergic) and sudomotor: sympathetic (cholinergic) functions. We describe symptoms and tests for assessment pupillary, genitourinary and gastrointestinal autonomic dysfunction. The indications for autonomic function testing in the different clinical scenarios are reported. CONCLUSIONS: combined evaluation of clinical and tests of autonomic function results allow to obtain the level and severity of autonomic dysfunction based upon pathophysiological support.

Consenso multidisciplinario de diagnóstico y tratamiento del dolor neuropático periférico y localizado en México / Multidisciplinary consensus on diagnosis and treatment of peripheral and localized neuropathic pain in Mexico

Resumen El dolor neuropático es una entidad que provoca discapacidad al paciente y su diagnóstico y tratamiento es un reto para los médicos. En un porcentaje importante de pacientes afectados, el dolor neuropático se presenta circunscrito a un dermatoma o a una región concreta del cuerpo, denominándose en ese caso dolor neuropático localizado. No existen guías clínicas mexicanas que postulen recomendaciones para el diagnóstico y tratamiento del dolor neuropático localizado en nuestra población. En este artículo se exponen las recomendaciones de un consenso multidisciplinario realizado con especialistas de distintas áreas implicadas en el diagnóstico y tratamiento de este tipo de pacientes.

Abstract Neuropathic pain is an entity that causes patient disability and its diagnosis and treatment is a challenge for physicians. In a significant percentage of patients with neuropathic pain, it is restricted to one dermatome or to a particular region of the body; in this case, it is referred to as localized neuropathic pain. There are no Mexican clinical guidelines proposing recommendations for the diagnosis and treatment of localized neuropathic pain in our population. This article presents the recommendations of a multidisciplinary consensus of specialists from different areas involved in the diagnosis and treatment of this type of patients.

Misdiagnosis and diagnostic delay in non-paraneoplastic sensory neuronopathies / Erro e atraso diagnóstico nas neuronopatias sensitivas não paraneoplásicas

ABSTRACT Sensory neuronopathies (SN) are a group of peripheral nerve disorders characterized by multifocal non-length-dependent sensory deficits and sensory ataxia. Its recognition is essential not only for proper management but also to guide the etiological investigation. The uncommon SN clinical picture and its rarity set the conditions for the misdiagnosis and the diagnostic delay, especially in non-paraneoplastic SN. Therefore, our objectives were to characterize the diagnostic odyssey for non-paraneoplastic SN patients, as well as to identify possible associated factors. Methods We consecutively enrolled 48 non-paraneoplastic SN patients followed in a tertiary neuromuscular clinic at the University of Campinas (Brazil). All patients were instructed to retrieve their previous medical records, and we collected the data regarding demographics, disease onset, previous incorrect diagnoses made and the recommended treatments. Results There were 34 women, with a mean age at the diagnosis of 45.9 ± 12.2 years, and 28/48 (58%) of the patients were idiopathic. Negative sensory symptoms were the heralding symptoms in 25/48 (52%); these were asymmetric in 36/48 (75%) and followed a chronic course in 35/48 (73%). On average, it took 5.4 ± 5.3 years for SN to be diagnosed; patients had an average of 3.4 ± 1.5 incorrect diagnoses. A disease onset before the age of 40 was associated to shorter diagnosis delay (3.7 ± 3.4 vs. 7.8 ± 6.7 years, p = 0.01). Conclusions These results suggest that diagnostic delay and misdiagnosis are frequent in non-paraneoplastic SN patients. As in other rare conditions, increased awareness in all the healthcare system levels is paramount to ensure accurate diagnosis and to improve care of these patients.

RESUMO As neuronopatias sensitivas (NS) representam um grupo de doenças caracterizadas por ataxia sensitiva e déficits sensitivos multifocais e não-comprimento dependentes. O seu reconhecimento é fundamental para o tratamento apropriado e para a investigação de doenças associadas. O quadro clínico pouco frequente aliado à baixa prevalência, especialmente das formas não-paraneoplásicas (NSnp), colaboram para o atraso e erro no diagnóstico. Os objetivos desse trabalho são descrever a odisseia diagnóstica dos pacientes com NSnp e tentar identificar possíveis fatores associados. Métodos Foram incluídos consecutivamente 48 pacientes com NSnp acompanhados no ambulatório de doenças neuromusculares da Universidade Estadual de Campinas (Brasil). Dados demográficos e sobre o início da NS (incluindo diagnósticos que lhes foram dados e tratamentos prescritos) foram coletados. Resultados Na coorte descrita havia 34 mulheres e a idade ao diagnóstico era de 45,9 ± 12,2 anos. Os sintomas inaugurais eram sensitivos deficitários em 25/48 (52%) dos pacientes, sendo assimétricos em 36/48 (75%) e de evolução crônica em 35/48 (73%). Para 28/48 (58%) dos pacientes a NS era idiopática. Em média, os pacientes com NSnp tiveram um atraso diagnóstico de 5,4 ± 5,3 anos com uma média de 3,4 ± 1,5 diagnósticos incorretos. Pacientes com início antes dos 40 anos tiveram diagnóstico mais precoce que aqueles com início tardio (3,7 ± 3,4 vs. 7,8 ± 6,7 anos, p = 0,01). Conclusão Os dados ora apresentados sugerem que o erro e o atraso diagnóstico são frequentes e impactam os pacientes com NS. A importância do diagnóstico das NS deve ser constante em todos os níveis do sistema de saúde para o diagnóstico correto e a consequente melhora no cuidado a esses pacientes.

Uremic neuropathy: an overview of the current literature

SUMMARY INTRODUCTION: Peripheral neuropathy is a disorder that affects the cell body, axon or myelin of motor or peripheral sensory neurons and occurs in 60-100% of patients who are submitted to dialysis due to chronic kidney disease. Uremic neuropathy is attributed to the accumulation of organic waste, evident in patients with reduced glomerular filtration rate. Objectives: This review aims to make clinical characteristics of uremic neuropathy evident enabling early diagnosis and treatment. Methods: This is a literature review of articles published on PubMed over the last 10 years using "Uremic Neuropathy" as "Title/Abstract". Results: A total of nine articles that met the inclusion criteria were included. UN is a distal symmetric sensorimotor polyneuropathy that occurs due to the accumulation of uremic toxins associated with an oxidative stress-related free radical activity. Hyperkalemia is thought to play an important role in its pathophysiology. Diagnosis depends on nerve conduction studies, and treatment includes dialysis or renal transplant. Conclusion: Clinical presentations of UN are broad and non-specific; nonetheless, it is important to detect early changes in order to avoid its progression. The earlier UN is diagnosed and treated, the more successful are the clinical outcomes.

RESUMO INTRODUÇÃO: A neuropatia periférica (NU) é um distúrbio que afeta o corpo celular, o axônio ou a mielina do motor ou neurônios sensoriais periféricos e ocorre em 60%-100% dos pacientes que são submetidos à diálise por doença renal crônica. A neuropatia urêmica é atribuída à acumulação de resíduos orgânicos, evidente em pacientes com taxa de filtração glomerular reduzida. Objetivo: O objetivo desta revisão é fazer com que as características clínicas da neuropatia urêmica sejam evidenciadas, permitindo o diagnóstico e tratamento precoce. Método: Esta é uma revisão da literatura de artigos publicados no PubMed nos últimos dez anos usando "Neuropatia Urêmica" como "Título/Resumo". Resultados: No total, foram incluídos nove artigos que atendem aos critérios de inclusão. A NU é uma polineuropatia sensório-motora simétrica distal que ocorre devido ao acúmulo de toxinas urêmicas associadas à atividade de radicais livres relacionados ao estresse oxidativo. A hipercalemia tem um papel importante na sua fisiopatologia. O diagnóstico depende de estudos de condução nervosa e o tratamento inclui diálise ou transplante renal. Conclusão: As apresentações clínicas das NU são amplas e não específicas; no entanto, é importante detectar mudanças iniciais para evitar sua progressão. Quanto mais precoce for a detecção e tratamento da NU, melhor será o resultado clínico.

Compromiso neuropático y autonómico en Enfermedad de Fabry: presentación de casos clínicos / Neuropathy and Fabry's disease: report of five cases

Fabry's disease is an X-linked multisistemic lisosomal storage disorder caused by deficiency or absence in α-Galatosidase A. Symptoms develop early in childhood with small fiber neuropathy, autonomic disorders and skin lesions (angiokeratomas). More severe in males, patients develop over years heart disease (hypertrophic cardiomyopathy, bradycardia), proteinuria, renal failure, transient ischemic attacks and stroke, associated with decreased life expectancy. We report five patients with Fabry's disease aged between 21 to 56 years and with family history. Neuropathic symptoms are described and neurophysiological testing findings of nerve conduction studies, quantitative sensory testing, autonomic testing and sympathetic skin response are presented.

Síndrome de Davidenkow: Una neuropatía periférica rara / Davidenkow syndrome. A rare peripheral neuropathy

En 1939 Davidenkow describió un tipo de atrofia diferente y rara con un patrón predominante en distribución escápulo-peroneal. Algunos investigadores caracterizaron el síndrome como una variante de la enfermedad de Charcot-Marie-Tooth; sin embargo, Davidenkow percibió que las manifestaciones clínicas y de laboratorio no corroboraban exactamente esta hipótesis. Describimos el caso de una mujer de 39 años, con cuadro clínico semejante al síndrome descrito por Davidenkow, presentando atrofia escápulo-peroneal. Sus primeros síntomas comenzaron cuando tenía 24 años, inicialmente con debilidad motora proximal en los miembros superiores. No tenía historia familiar de miopatía o neuropatía y se excluyeron otros síndromes que se podrían incluir entre los diagnósticos diferenciales mediante la realización de pruebas de mutación genética, además del examen físico y electromiografía. El amplio espectro de enfermedades neuromusculares a veces dificulta su diagnóstico y debe ser siempre considerado en el diagnóstico diferencial.

A different and rare type of atrophy with a predominant pattern in scapulo-peroneal distribution was described by Davidenkow in 1939. The syndrome was characterized by some researchers as a variant of Charcot-Marie-Tooth disease, however Davidenkow noticed that clinical and laboratorial manifestations did not corroborate exactly with this hypothesis. We describe a case of a female patient, 39 years-old, clinical picture similar to the syndrome described by Davidenkow, presenting scapulo-peroneal atrophy. Her first symptoms had appeared when she was 24, initially with proximal motor weakness in the upper limbs. This patient did not have family history of myopathy or neuropathy. Several tests were performed to exclude other syndromes that could be included in the differential diagnosis, by testing gene mutation, in addition to the physical examination and electromyography. The large spectrum of neuromuscular diseases makes difficult the diagnosis of Davidenkow’s syndrome which always should be considered in the differential diagnosis.

Peripheral neurppathy electrophysiological screening in children with celiac disease / Triagem eletrofisiológica de neuropatia periférica em crianças com doença celíaca

Background The involvement of the peripheral nervous system in children with celiac disease is particularly rare. Objective The aim of this study was to assess the need for neurophysiological testing in celiac disease patients without neurological symptoms in order to detect early subclinical neuropathy and its possible correlations with clinical and demographic characteristics. Methods Two hundred and twenty consecutive children with celiac disease were screened for neurological symptoms and signs, and those without symptoms or signs were included. Also, patients with comorbidities associated with peripheral neuropathy or a history of neurological disease were excluded. The remaining 167 asymptomatic patients as well as 100 control cases were tested electro-physiologically for peripheral nervous system diseases. Motor nerve conduction studies, including F-waves, were performed for the median, ulnar, peroneal, and tibial nerves, and sensory nerve conduction studies were performed for the median, ulnar, and sural nerves with H reflex of the soleus muscle unilaterally. All studies were carried out using surface recording electrodes. Normative values established in our laboratory were used. Results Evidence for subclinical neuropathy was not determined with electrophysiological studies in any of the participants. Conclusion In this highly selective celiac disease group without any signs, symptoms as well as the predisposing factors for polyneuropathy, we did not determine any cases with neuropathy. With these results we can conclude that in asymptomatic cases with celiac disease electrophysiological studies are not necessary. However, larger studies with the electrophysiological studies performed at different stages of disease at follow-ups are warranted. .

Contexto O envolvimento do sistema nervoso periférico em crianças com doença celíaca é particularmente raro. Objetivo O objetivo do presente estudo foi avaliar a necessidade de testes neurofisiológicos em pacientes com doença celíaca sem sintomas neurológicos, a fim de detectar neuropatia subclínica precoce e suas possíveis correlações com características clínicas e demográficas. Métodos Duzentos e vinte crianças consecutivas com doença celíaca foram pesquisadas para os sinais e sintomas neurológicos, e foram incluídos somente aqueles sem sintomas ou sinais. Além disso, os portadores de comorbidades associadas à neuropatia periférica ou história de doença neurológica também foram excluídos. Os 167 pacientes assintomáticos, bem como 100 casos controles foram testados electrofisiologicamente para doenças do sistema nervoso periférico. Estudos de condução nervosa motora, incluindo ondas F foram realizados para os nervos medianos, ulnar, fibular e tibiais; realizaram-se estudos de condução sensorial para o nervo mediano, ulnar, e nervos surais com reflexo H do músculo sóleo unilateralmente. Todos os estudos foram realizados utilizando gravação por eletrodos de superfície. Foram usados valores normativos estabelecidos em nosso laboratório. Resultados Não foi determinada evidência de neuropatia subclínica com estudos eletrofisiológicos em qualquer um dos participantes. Conclusão Neste grupo altamente seletivo de pacientes com doença celíaca sem quaisquer sinais, sintomas, bem como os fatores predisponentes para a polineuropatia, não se determinou qualquer caso com neuropatia. Com estes resultados, pode-se concluir que, em casos assintomáticos com doença celíaca os estudos eletrofisiológicos não são necessários. No entanto, são necessários maiores estudos eletrofisiológicos realizados em diferentes fases da doença. .

Escores de neuropatia periférica em diabéticos / Peripheral neuropathy scores in diabetic

OBJETIVO: Diabetes mellitus é uma doença crônica prevalente, associada a inúmeras complicações. A neuropatia periférica diabética é a mais comum, acometendo 50% dos diabéticos, mas muitas vezes não é diagnosticada. Por cursar com insensibilidade distal e alterações arquiteturais dos pés, predispõe a úlceras, podendo culminar no pé diabético com risco de amputação. O diabetes mellitus é responsável por 70% das amputações de membros, que poderiam ser prevenidas com o diagnóstico precoce da neuropatia periférica diabética. Sugere-se avaliar o grau de neuropatia em diabéticos por meio de escores, visando homogeneizar o diagnóstico, quantificar a prevalência e promover medidas preventivas. MÉTODOS: Realizou-se entrevista, exame físico e coleta de dados de diabéticos atendidos ambulatorialmente, para pontuação e qualificação no Escore de Sintomas Neuropáticos e no Escore de Comprometimento Neuropático, validados na língua portuguesa para avaliar neuropatia periférica diabética, além de análise das características clínicas e epidemiológicas associadas. RESULTADOS: Foram incluídos 116 pacientes, constatando-se neuropatia periférica diabética em 31,9%. Houve correlação significativa de neuropatia periférica diabética coma idade dos pacientes, mas não com as demais variáveis clínicas e laboratoriais. Os pacientes avaliados apresentaram médias de idade de 55±15 anos e tempo de diabetes de 14,8±10,9 anos, sendo predominantemente caucasianos, mulheres e portadores de diabetes mellitus tipo 2. Eram hipertensos 67,2% e 42,2%, obesos. CONCLUSÃO: A prevalência encontrada corrobora a literatura, embora poucos estudos tenham utilizado critérios similares para diagnosticar neuropatia periférica diabética. Empregando os escores padronizados, de baixo custo e fácil aplicação possibilitamos o diagnóstico precoce e embasado dessa entidade, sendo possível, com isso, reduzir a prevalência de graves complicações do pé diabético e disseminar informações a respeito.

OBJECTIVE: Diabetes mellitus is a prevalent chronic disease, associated with numerous complications. Diabetic peripheral neuropathy is the most common, affecting 50% of diabetics, although is often not diagnosed. Presenting with distal numbness and architectural alterations of the feet, it predisposes ulcers and may culminate in diabetic foot at risk for amputation. Diabetes mellitus is responsible for 70% of limb amputations, which could be prevented with early diagnosis of diabetic peripheral neuropathy. This study aims to evaluate the degree of neuropathy in diabetics through validated scores, in order to standardize the diagnosis, quantify the prevalence and promote preventive actions. METHODS: We performed an interview, physical examination and data collection of diabetic outpatients, for rating in the Neuropathy Symptom Score and the Neuropathy Disability Score, validated in Portuguese, to assess diabetic peripheral neuropathy, in addition to analysis of clinical and epidemiological associated characteristics. RESULTS: We included 116 patients and diabetic peripheral neuropathy was found in 31.9%. There was significant correlation diabetic peripheral neuropathy with age, but not with other clinical and laboratory variables. The mean age was 55±15 years, diabetes duration was 14.8±10.9 years and patients were predominantly Caucasian, women and had type 2 diabetes mellitus. Of the patients, 67.2 % were hypertensive and 42.2% obese. CONCLUSION: The prevalence found is supported by previous data, although few studies have used similar criteria to diagnose diabetic peripheral neuropathy. Employing the standard scores, of low cost and easy implementation, we enable early and accurate diagnosis of this condition, allowing to reduce the prevalence of severe diabetic foot complications and spread information about it.

Benign Solitary Schwannoma of Right Ulnar Nerve – A Case Report.

Introduction: Benign peripheral nerve schwannomas are uncommon tumours. Extra cranial schwannomas have also been reported from uncommon and unusual sites including breast, pancreas, and gastrointestinal system. Peripheral nerve schwannomas may pose a problem in clinical diagnosis, however an appropriate diagnostic work-up including thorough history and clinical examination, Ultrasonography, magnetic resonance imaging, fi ne needle aspiration cytology, nerve conduction velocity and electromyography study may all help reaching the correct preoperative diagnosis. Th e important clinical diff erential diagnoses include traumatic neuroma, neurfi bromas, lipoma, cold abscess and muscle hernia. Th e defi nitive treatment of benign peripheral nerve schwannoma is complete enucleation of the tumour mass without damaging the intact nerve fascicles followed by confi rmatory histopathological examination. When there is a doubt on histopathology, a positive Immunohistochemical staining with S100 is helpful in confi rming a diagnosis of schwannoma. Case presentation: We had 40 years female who had a slow growing swelling over the inner aspect of her right elbow for the last one year; this was followed by pain, tingling and numbness over inner one and half fi ngers of her right hand for six months. Tinnels sign was positive over the swelling. Her subsequent clinical examination and investigations including a magnetic resonance imaging was suggestive of a benign growth in her right ulnar nerve in the elbow region. Complete enucleation of the swelling was done from the right ulnar nerve in the elbow region and subsequent histopathological examination confi rmed it to be a benign cellular schwannoma. Patient recovered successfully after the surgery and paresthesia in the distribution of her right ulnar nerve also improved six weeks after surgery. At her last follow-up six months after surgery, the patient was completely asymptomatic and highly satisfi ed with the results of surgery. Conclusion: A correct preoperative diagnosis of peripheral nerve schwannomas is possible, and it can be successfully managed with complete enucleation of tumour mass with satisfactory patient outcomes.

Neuropatía diabética periférica: eficacia clínica de la evaluación semiológica / Diabetic peripheral neuropathy: semiológica clinical efficacy of assessment

El diagnóstico de la Neuropatía Diabética Periférica es tardío. Identificar maniobras semiológicas que permitan el diagnóstico precoz de la neuropatía diabética. Estudio de casos, analítico, transversal y operacional: personas sanas, prediabéticos, diabéticos de reciente diagnóstico y diabéticos de más de 5 años de diagnóstico. Se realizaron 2 evaluaciones: la primera por dos investigadores a ciegas que evaluaron: sensibilidad mecánica, reflejos osteotendinosos y palestesia. También se evaluación de la córnea con Rosa de Bengala y se aplicó el Cuestionario DN4. Segunda Evaluación: von Frey. Biopsia de Piel: será tratada con inmunohistoquímica de campo claro. Muestra de 25 personas. El DN4, obtuvo 14 personas con dolor neuropático. La tinción con Rosa de Bengala obtuvo 7 pacientes con ojo seco y una diabética con más de 5 años de diagnóstico con alteración corneal. En la evaluación con von Frey hubo 3 pacientes con zonas sin respuesta al microfilamento de 10 g. La inmunohistoquímica demostró que el número y densidad de fibras nerviosas tuvo un promedio de 7 fibras/campo en sanos y a partir de los prediabéticos disminuyó desde 4,4 fibras/campo. El ojo seco justifica la evaluación periódica del internista. La evaluación de la sensibilidad con los filamentos de von Frey señala que el monofilamento utilizado individualmente tiene menor eficiencia diagnóstica. La biopsia demostró una capacidad diagnóstica precoz de esta, aún en ausencia de síntomas. La biopsia de piel con cuantificación del número y densidad de fibras, es útil en la identificación temprana de lesión de fibras C y se comporta como método de pesquisa.

The diagnosis of Diabetic Peripheral Neuropathy is made lately. To identify semiological maneuvres that allow early diagnosis of diabetic neuropathy. Case studie, analitic, transversal and operational, without therapeutic intervention in healthy, prediabetic, diabetic and newly diagnosed diabetes over 5 years of diagnosis. The First Assessment was: conducted by two blinded researchers measuring mechanical sensitivity, tendon reflexes, and palesthesia. Von Frey 3) Skin biopsy: the cornea Bengal Rose and DN4 Questionnaire. The second assessment was done with brightfield immunohistochemistry. The sample consisted of 25 persons. The DN4 had 14 people with neuropathic pain. Staining with Rose Bengal scored 7 persons. The second Assessment was done in patients with dry eye and over 5 years of diagnosis of corneal disorder. The evaluation with von Frey 3 patients with no response areas were obtained at 10 g microfilament. Immunohistochemistry showed that the number and density of nerve fibers had an average of 7 fibers in healthy and from prediabetic decreased to 4.4 fibers. The Dry eye justifies the periodic evaluation by the internist. The evaluation of sensitivity with von Frey hairs used indicate that the monofilament has a lower diagnostic efficiency individually. The biopsy revealed an early diagnostic capacity in this condition in the absence of symptoms. Skin biopsy with quantification of the number and density of nerve fibers is useful in early identification of C fiber damage and behaves like screening method.

Leprosy neuropathy: clinical presentations / Neuropatia da lepra: apresentacoes clinicas

Leprosy is a chronic infectious peripheral neuropathy caused by Mycobacterium leprae. The different clinical presentations of the disease are determined by the quality of the host immune response. Early detection of leprosy and treatment by multidrug therapy are the most important steps in preventing deformity and disability. Thus the early recognition of the clinical leprosy presentation is essential. Mononeuritis, mononeuritis multiplex (MM), polyneuritis (MM summation) are the most frequent. The frequent anesthetic skin lesions are absent in the pure neuritic leprosy presentation form. Isolated peripheral nerve involvement is common, including the cranial ones. Arthritic presentation is occasionally seen, usually misdiagnosed as rheumatoid arthritis. Attention should be given to autonomic dysfunctions in leprosy. There are clinical presentations with severe neuropathic pain - painful small-fiber neuropathy. Leprous late-onset neuropathy (LLON) clinical presentation should be considered facing a patient who develop an inflammatory neuropathy many years after a previous skin leprosy treatment.

A hanseníase é uma neuropatia periférica infecciosa, crônica, causada pelo Mycobacterium leprae. As diferentes apresentações clínicas são determinadas pela qualidade da resposta imune do hospedeiro. O diagnóstico precoce e a multi-droga terapia são os passos mais importantes na prevenção de deformidades e incapacidades. Dessa forma, o reconhecimento precoce da apresentação clínica da hanseníase é essencial. Mononeurites, mononeurites múltipla (MM), polineurite (superposição de MM) são as mais frequentes. As frequentes lesões anestésicas de pele estão ausentes na forma neurítica pura. Comprometimento de nervo isolado é comum, inclusive os cranianos. Apresentação com artrite é ocasionalmente vista, erroneamente diagnosticada como artrite reumatóide. Atenção deve ser dada às disfunções autonômicas na hanseníase. Há apresentações clínicas com dor neuropática grave - neuropatia dolorosa de pequenas fibras. Neuropatia de início tardio (LLON) é apresentação clínica que deve ser considerada frente a paciente que desenvolve neuropatia inflamatória muitos anos depois de tratamento prévio da lepra cutânea.

Comparative Analysis of Electrophysiological Parameters of Median Nerve in Normal and Diabetic Subjects.

Peripheral neuropathy is a common complication in diabetic patients. Nerve conduction study is widely used for the assessment of diabetic polyneuropathy. This study was designed to characterize motor nerve conduction abnormalities in subjects having relatively shorter duration (2.28 ± 1.51 years) of type 2 diabetes mellitus. The median motor nerve conduction study was carried out on 40 male type 2 diabetic patients attending diabetic clinic in the department of medicine and 40 healthy male volunteers who served as control. Motor Distal latency (MDL), Amplitude (Amp) and Conduction Velocity (CV) were measured. On comparing the parameters of median nerves of both the groups, it was found that motor distal latency of (right and left) median nerves was higher in diabetics than in non diabetics with statistically significant difference. Results also showed decreased amplitude and conduction velocities of median nerve of both sides in diabetics (statistically significant). All the parameters were found correlated with blood sugar levels in diabetics.

Ultrassonografia no diagnóstico da tendinite e eletroneuromiografia no diagnóstico da neuropatia periférica e da radiculopatia do membro superior: visão do reumatologista / Ultrasonography for the diagnosis of tendinitis and electromyography for the diagnosis of peripheral neuropathy and upper limb radiculopathy: rheumatologists' perspectives

OBJETIVO: Averiguar o valor que os reumatologistas brasileiros conferem ao exame de ultrassonografia para o diagnóstico de tendinite e ao exame de eletroneuromiografia para o diagnóstico da neuropatia periférica e da radiculopatia dos membros superiores. MATERIAL E MÉTODOS: No total, 165 reumatologistas responderam a uma pesquisa de opinião anônima (enviada pela internet), sobre diversas situações relativas aos dois exames, no que diz respeito aos seguintes questionamentos: confiabilidade, precisão no diagnóstico, importância e necessidade desses exames para confirmação diagnóstica e credibilidade e treinamento dos profissionais que executam os exames. RESULTADOS: O estudo revelou que a maioria dos reumatologistas reconhece que esses exames são operador-dependentes, que não confia integralmente nos resultados observados, que tais exames não são imperativos para os diagnósticos elencados, e que os profissionais que executam esses exames deveriam ser mais bem treinados para fornecer resultados mais confiantes. CONCLUSÃO: Para os reumatologistas brasileiros, os resultados desses exames devem ser interpretados com cautela e não são definitivos para o diagnóstico; a ultrassonografia musculoesquelética e a eletroneuromiografia devem ser realizadas por profissionais capacitados; deve haver melhor preparo dos executores desses exames em nosso meio.

OBJECTIVE: To ascertain the value ascribed by Brazilian rheumatologists to ultrasonography (US) for diagnosing tendinitis and to electromyography (EMG) for diagnosing peripheral neuropathy and upper limb radiculopathy. MATERIAL AND METHODS: In total, 165 rheumatologists answered an anonymous survey (sent via the internet) concerning the two exams, with respect to the following characteristics: reliability, diagnostic accuracy, the importance and necessity of these tests for diagnostic RESULTS: The study revealed that most of the rheumatologists recognised that these exams are operator-dependent, that clinicians do not rely entirely on the results, that these exams are not mandatory for the diagnoses listed, and that professionals who perform these exams should be better trained to provide reliable results. CONCLUSIONS: The Brazilian rheumatologists believe the following: the results of these exams should be interpreted with caution and are not definitive for diagnosis; musculoskeletal US and EMG should be performed by trained professionals; and there must be better preparation of the professionals who perform these exams.

Ischemic monomelic neuropathy: a rare complication after vascular access formation

No abstract available.

Vasculitis del nervio periférico: revisión clínico-patológica de 13 casos / Peripheral nerve vasculitis: clinicopathological review of 13 cases

Objetivo: Realizar una revisión de las biopsias de nervio y músculo de pacientes con sospecha clínica de neuropatía vasculítica y correlacionarlas con los datos clínicos, de laboratorio y electrofisiológicos. Materiales y métodos: Fueron revisadas retrospectivamente las historias clínicas de pacientes sometidos a biopsia de nervio/músculo debido a sospecha clínica de neuropatía vasculítica en el Hospital General de Agudos Juan A. Fernández de la Ciudad Autónoma de Buenos Aires entre los años 1999 y 2011. Resultados: Se incluyeron 13 pacientes, 8 (61,54 por ciento) de sexo femenino y 5 (38,46 por ciento) de sexo masculino; la edad media fue de 58,85 +/- 15,02 años. Dos tercios de los casos presentaron mononeuropatía múltiple al diagnóstico y en 9 de 12 casos el patrón electromiográfico fue axonal. La anatomía patológica del nervio mostró vasculitis definida en 6 casos (46,15 por ciento) y probable sólo en uno (7,69 por ciento), mientras que la biopsia de músculo evidenció vasculitis en el 90 por ciento de los casos (p=0,077). En el subgrupo de pacientes con diagnóstico definitivo de vasculitis sistémica primaria, el 100 por ciento de las biopsias de músculo y el 62,5 por ciento de las biopsias de nervio resultaron positivas para neuropatía vasculítica (NPV). Conclusiones: La biopsia de nervio es el único procedimiento aceptado actualmente para el diagnóstico definitivo de neuropatía vasculítica. La biopsia combinada de nervio y músculo mostró una clara tendencia, aunque no significativa, hacia una mayor utilidad diagnóstica de neuropatía vasculítica que la biopsia de nervio aislada. Consideramos que el estudio de un mayor número de casos contribuirá a aclarar esta duda.

Objective: To review the nerve and the muscle biopsies from patients with clinical suspicion of vasculitic neuropathy and their correlation with clinical, laboratory and electrophysiologic studies. Materials and methods: We retrospectively reviewed the medical records of patients undergoing nerve/muscle biopsy due to clinical suspicion of vasculitic neuropathy at the Juan A. Fernandez General Hospital in the city of Buenos Aires between 1999 and 2011. Results: Thirteen patients, 8 (61.54 percent) female and 5 (38.46 percent) male, mean age 58.85 +/- 15.02 years, were included. Two thirds of the patients had multiple mononeuropathy at diagnosis, and 9 of 12 cases had axonal pattern in the electromyogram. The histopathology of the nerve showed definite vasculitis in 6 cases (46.15 percent) and probable vasculitis in only one (7.69 percent), whereas muscle biopsy showed vasculitis in 90 percent of cases (p=0.077). In the subgroup of patients with definite diagnosis of primary systemic vasculitis, 100 percent of muscle biopsies and 62.5 percent of nerve biopsies were diagnostic of vasculitis neuropathy. Conclusion: Nerve biopsy in the only currently accepted procedure for definitive diagnosis of vasculitic neuropathy. The combined nerve and muscle biopsy showed a clear trend, but not statistically significant toward increased the diagnostic yield of vasculitis neuropathy that isolated nerve biopsy. A large number of cases will clarify this issue.

Validation of a Brazilian quantitative sensory testing (QST) device for the diagnosis of small fiber neuropathies / Validação de um aparelho brasileiro de teste de quantificação sensitiva brasileiro para o diagnóstico de neuropatia de fibras finas

Quantitative sensory testing (QST) is defined as the determination of thresholds for sensory perception under controlled stimulus. Our aim was to validate a new QST device for Brazilian sample. In 20 healthy adults, thermoalgesic thresholds were assessed using a QST prototype (Heat Pain Stimulator-1.1.10; Brazil). A 30 × 30 mm² thermode with a 1°C/s stimulus change rate were applied. Thresholds of three consecutive stimuli were averaged in two different sessions separated by at least two weeks. Additionally long thermal heat pain stimulus was performed. To evaluate the consistency of our method we also analyzed 11 patients with small fiber neuropathy. Results showed good reproducibility of thermal perception thresholds in normal individuals and plausible abnormal thresholds in patients. We conclude that our QST device is reliable when analyzing the nociceptive pathway in controls and patients.

Teste de quantificação sensitiva (TQS) significa determinação de limiares de percepção sensitiva frente a um estímulo de intensidade controlada. Nosso objetivo foi validar um novo equipamento de TQS adaptado à população brasileira. Em 20 adultos saudáveis, limiares termoalgésicos foram avaliados, utilizando um aparelho protótipo do TQS (Heat Pain Stimulator-1.1.10; Brazil). Foi utilizado um termodo de 30 × 30 mm², com estímulo térmico de 1°C/s. A média dos limiares de três estímulos consecutivos foi obtida em duas sessões diferentes, separadas por pelo menos 2 semanas. Adicionalmente, foram aplicados estímulos térmicos dolorosos de longa duração. Para avaliar a consistência do nosso método, foram também analisados 11 pacientes com neuropatia de fibras finas. Os resultados mostraram boa reprodutibilidade dos limiares de percepção nos indivíduos saudáveis, assim como limiares anormais nos pacientes. Em conclusão, nosso aparelho de TQS apresentou boa confiabilidade ao analisar a via nociceptiva de controles e pacientes.