Clinical and electrophysiological characteristics of neuropathic pain in leprosy patients: A prospective cross-sectional study

Introduction: Neuropathic pain is a common and disabling late complication of leprosy. We investigated the clinical and electrophysiological characteristics of neuropathic pain in leprosy patients by evaluating nerve conduction, sympathetic skin response (SSR) and A-waves. Methods: Twenty one leprosy patients with neuropathic pain validated by the Douleur Neuropathique en 4 (DN4) Questionnaire were selected for study. Pain intensity was measured by the visual analog scale. Demographic and clinical data were collected for all patients. Clinical data included appraisal of the median, ulnar, radial, tibial and common peroneal nerves, assessment of the sympathetic skin response and conventional electrophysiological recordings. Results: Among all electroneuromyographic presentations, multifocal mononeuropathy was still the most prevalent. Sensory loss was observed more frequently than motor deficits. As most patients presented advanced clinical forms of leprosy and were under treatment, this high mean was found and the ulnar nerve was most frequently affected. The sympathetic skin response was absent in 16 patients. Higher DN4 Questionnaire scores were observed in women and in those receiving corticosteroid therapy. These inferences are possible to be made, but our study's limitations don't allow us to be certain about it. The statistical significance found only permits us to evidence what we related on the textual part of the study. Limitations: The small number of patients studied, the lack of sophisticated diagnostic methods for leprosy, as well as the difficulties in assessing nerve conduction were the main limitations of this study. Conclusion: The neurophysiological and clinical findings in leprous neuropathy were modest despite the conspicuous neuropathic pain. Although electrophysiological studies are a vital tool to verify nerve damage, variations in the clinical presentation of leprosy neuropathic pain render the diagnosis challenging. Further studies are needed to describe the neurophysiological evolution of this disease

Anatomorphometry of the brachial plexus under high-definition system: an experimental study in rats

Purpose: To study the anatomorphometry of the plexus brachialis (PB) of rats under a high-definition video system. Methods: Ten male Wistar rats discarded from other research that did not interfere in the morphology of the animal, respecting the principle of reduction, were used. All animals were submitted to the same protocol. Initially, the cervical region was shaved. The animals were placed in a dorsal position. A single elbow-to-elbow incision was performed and dissection started at the deltopectoral sulcus. The procedures were performed under a video system. To measure the structures, the Image J software was used. Results: All the PB evaluated originated from the C5-T1 spinal nerves. C5 and C6 converged to form the truncus superior, the root of C7 originated the truncus medius, and the confluence of C8 and T1 originated the truncus inferior. It was found the union of C7, C8, and T1 to form truncus inferomedialis instead of separate medial and inferior truncus. C8 (1.31 mm) was the thickest root, the truncus inferior (1.80 mm) and the nerve radialis (1.02 mm), were the thickest. Conclusions: The anatomy of the PB is comparable to humans, admitting variations. The videomagnification system is useful to perform microsurgical dissection.

Anatomical description of the extratemporal facial nerve under high-definition system: a microsurgical study in rats

Purpose: To describe the microsurgical anatomical aspects of the extratemporal facial nerve of Wistar rats under a high-definition video system. Methods: Ten male Wistar rats (12­15 weeks old), without veterinary diseases, weighing 220­280 g, were used in this study. All animals in this study were submitted to the same protocol and by the same surgeon. A 10-mm incision was made below the bony prominence of the right or left ear, and extended towards the angle of the mandible. The dissection was performed and the main branches of the facial nerve were dissected. Results: The main trunk of the facial nerve has a length of 0.88 ± 0.10 mm and a length of 3.81 ± 1.03 mm, measured from its emergence from the stylomastoid foramen to its bifurcation. Seven branches originating from the facial nerve were identified: posterior auricular, posterior cervical, cervical, mandibular, buccal, temporal, and zygomatic. Conclusions: The anatomy of the facial nerve is comparable to that of humans, with some variations. The most observed anatomical division was the distribution in posterior auricular, posterior cervical, cervical, mandibular, buccal, temporal, and zygomatic branches. There is no statistical difference between the thickness and distance of the structures compared to the contralateral side.

Botulinum toxin type A in chronic neuropathic pain in refractory leprosy / Toxina Botulínica Tipo A em Dor Neuropática Crônica em Hanseníase

ABSTRACT Neuropathic pain is a chronic syndrome that is difficult to treat and often affects patients with leprosy. Recommended treatment includes the the use of analgesic drugs, codeine, tricyclic antidepressants, neuroleptics, anticonvulsants and thalidomide, but without consensus on uniform dose and fully satisfactory results. Objective: To analyze botulinum toxin type A (BoNT-A) effectiveness in treatment of chronic neuropathic pain in refractory leprous patients, as well as evaluate and compare the quality of life of patients before and after using the medication. Methods: We used a specific protocol including clinical, demographic, DN4 protocol, analogue scale (VAS), sensory evaluation and evaluation of the WHOQOL-BREF. Therapeutic intervention was performed with BOTOX® BTX-A 100U administered subcutaneously. Fifteen patients were evaluated on days 0, 10 and 60. Results: Patients on VAS showed pain between 5 and 10, in one case there was complete pain relief in 60 days, while others showed improvement in the first week with the return of symptoms with less intensity after this period. WHOQOL-BREF's domains Quality of Life and Physical to have a significant increase in QOL. Conclusion: BoNT-A proved to be a good therapeutic option in relieving pain with improved quality of life for these patients.

RESUMO A dor neuropática é uma síndrome crônica que é difícil de tratar e freqüentemente afeta pacientes com hanseníase. O tratamento recomendado inclui o uso de drogas analgésicas, codeína, antidepressivos tricíclicos, neurolépticos, anticonvulsivantes e talidomida, mas sem consenso sobre dose uniforme e resultados plenamente satisfatórios. Objetivo: Busca-se analisar a efetividade da toxina botulínica tipo A no tratamento da dor neuropática crônica hansênica refratária. Método: Estudo de intervenção do tipo ensaio clínico em portadores de dor neuropática crônica hansênica. Foram coletados dados epidemiológicos, protocolo DN4, escala analógica da dor (EVA), avaliação sensitiva, motora a avaliação do WHOQOL-Bref. Realizado intervenção terapêutica com toxina botulínica tipo A 100U. Os pacientes foram avaliados nos dias de 0, 10 e 60. A dor neuropática foi mais frequente no sexo masculino, na faixa etária de 40 à 49 anos. Resultados: Da forma Dimorfa, multibacilar com baciloscopia positiva e incapacidades presentes. Os escores EVA variam entre 5 e 10, todos os pacientes apresentaram alterações sensoriais. O WHOQOL-Bref apresentou melhora após o tratamento com TxBA. A TxBA foi bem tolerada o único efeito adverso notável foi dor leve. E com apenas uma única aplicação de TxBA promoveu efeitos analgésicos a longo prazo em pacientes com dor associada à alodinia, sugerindo que a analgesia observada pode ser causada por um efeito periférico da TxBA em terminações nociceptivas. Conclusão: O estudo sugere que a TxBA é uma boa opção para os casos de dor neuropática crônica hansênica, no entanto, novos estudos são necessários para confirmar estes resultados.

Does the type of electrode affect the electromyoneurographic parameters in rats?

Abstract Purpose: To evaluate if the type of electrode (needle vs. surface) affects the electromyoneurography parameters in rats. Methods: Twenty male rats were anesthetized, then compound muscle action potential were recorded using a Neuropack S1 MEB- 9400©. All animals were submitted to two electroneuromyography analysis: first with surface electrode and then by needle electrode. We evaluated the latency, amplitude, duration and area of the negative peak of the gastrocnemius and cranial tibial muscles. Results: There were no significant differences between the groups in the mean of duration, latency, amplitude or area of the negative peak in gastrocnemius and cranial tibial muscles. Conclusion: The type of electrode does not affect the electroneuromyography parameters.

X-linked spinal and bulbar muscular atrophy (Kennedy's disease): the first case described in the Brazilian Amazon / Atrofia muscular bulboespinhal ligada ao cromossomo X (doença de Kennedy): o primeiro caso descrito na Amazônia brasileira

ABSTRACT The X-linked spinal and bulbar muscular atrophy (Kennedy's disease) is a rare X-linked, recessive, lower motor neuron disease, characterized by weakness, atrophy, and fasciculations of the appendicular and bulbar muscle. The disease is caused by an expansion of the CAG repetition in the androgen receptor gene. Patients with Kennedy's disease have more than 39 CAG repetitions. We report a case of 57-year-old man, resident of Monte Dourado (PA, Brazil) who complained of brachiocrural paresis evolving for 3 years along with fasciculations and tremors of extremities. In addition, he also developed dysarthria, dysphagia, and sexual dysfunction. The patient clinical picture included gait impairment, global hyporeflexia, proximal muscle atrophy of upper limbs, deviation of the uvula to right during phonation and tongue atrophy with fasciculations. The patient reported that about 30 years ago he had undergone gynecomastia surgery. His electroneuromyography suggested spinal muscular atrophy, and nuclear magnetic resonance imaging showed tapering of the cervical and thoracic spinal cord. Patient's creatine kinase level was elevated. In view of the findings, an exam was requested to investigate Kennedy's disease. The exam identified 46 CAG repetitions in the androgen receptor gene, which confirmed the diagnostic suspicion. This was the first case of Kennedy's disease diagnosed and described in the Brazilian Amazon. To our knowledge only other four papers were published on this disease in Brazilian patients. A brief review is also provided on etiopathogenic, clinical and diagnostic aspects.

RESUMO A atrofia muscular bulboespinhal ligada ao cromossomo X (doença de Kennedy) é uma rara doença de neurônio motor inferior, recessiva, ligada ao X, e caracterizada por fraqueza, atrofia e fasciculações da musculatura apendicular e bulbar. É causada por uma expansão da repetição CAG no gene do receptor de androgênio. Pacientes com doença de Kennedy apresentam mais de 39 repetições CAG. O paciente deste relato era do sexo masculino, 57 anos, morador de Monte Dourado (PA, Brasil), com queixa de paresia braquiocrural há 3 anos, acompanhada de fasciculações e tremores de extremidades. Em seguida, ele desenvolveu disartria, disfagia e disfunção sexual. Também apresentava comprometimento da marcha, hiporreflexia global, atrofia muscular proximal dos membros superiores, desvio da úvula para direita à fonação e atrofia de língua com fasciculações. Foi realizada cirurgia para tratamento de ginecomastia há 30 anos. A eletroneuromiografia sugeriu quadro de atrofia muscular espinhal. Imagens de ressonância magnética demonstraram afilamento da medula espinhal cervical e torácica. A creatina quinase estava elevada. Diante dos achados, solicitou-se investigação para doença de Kennedy, e foram identificadas 46 repetições CAG no gene do receptor de androgênio, o que confirmou a suspeita diagnóstica. Este foi o primeiro caso de doença de Kennedy diagnosticado e descrito na Amazônia brasileira. Existem, além deste relato, apenas outros quatro trabalhos publicados sobre a doença em pacientes do Brasil. Também realizamos breve revisão de aspectos etiopatogênicos, clínicos e diagnósticos.

Dor neuropática crônica hansenica: estudo de casos com ênfase no diagnóstico e na terapêutica com toxina botulìtica tipo A / Chronic leprosy neuropathic pain: study of cases with emphasis on diagnóstic and therapeutics with botulinum toxin type A

OBJETIVO: descrever pacientes com dor neuropática crônica hansênica, diagnóstico, nervos acometidos, formas clínicas, alterações motoras e sensitivas com ênfase no diagnóstico e na resposta ao tratamento da dor neuropática crônica, utilizando toxina botulínica tipo A (TxBA). MÉTODO: foram descritos 04 pacientes hansenianos, portadores de dor neuropática crônica. Utilizou-se protocolo específico incluindo dados clínicos, demográficos, protocolo DN4, escala analógica da dor (EVA), avaliação sensitiva e motora eeletroneuromiografia. Realizada intervenção terapêutica com toxina botulínica tipo A, 100U da marca comercial BOTOX®, administrada por via sub-cutânea na área de com-prometimento neural. Os pacientes foram avaliados nos período de 0, 15, 45, e 60 dias. RESULTADOS: escores de DN4 variaram entre7 e 9, Escala EVA entre 8 e 10, todos os pacientes apresentaram alterações sensoriais, motoras e eletroneuromiograficas. Em 03 casos observou-se alívio completo da dor no período de 15 dias, e retorno dos sintomas em menor intensidade após este período, 01 caso ocorreu melhora da clínica após 45 dias da aplicação. Não foram ob-servados efeitos adversos à medicação. CONCLUSÃO: os pacientes apresentavam dor neuropática de alta intensidade, contínua, persistente e refratária ao tratamento habitual. O uso de TxBA mostrou-se como boa opção terapêutica no alívio do quadro doloroso, com melhora na qualidade de vida desses pacientes.

OBJECTIVE: to describe patients with chronic neuropathic pain leprosy, its diagnosis, afflicted nerves, clinical for-ms, motor and sensory alterations with emphasis on the treatment of chronic neuropathic pain using botulinum toxin. METHODOLOGY: 04 leprosy patients with chronic neuropathic pain deriving from the Tropical Medicine Nucleus and UEPA Health Center School will be described. Specific protocol was used including clinical, demographic, DN4 protocol, visual analog scale (EVA), sensory and motor assessment and electroneuromyography. It was performed a therapeutic intervention with botulinum toxin type A 100U of BOTOX® trademark, administered subcutaneously in the area of neural commitment. Patients were evaluated in the period of 0, 15, 45, and 60 days after application. RE-SULTS: the DN4 results varied between 7 to 9, EVA between 8 and 10, all patients had sensory changes, motor and electroneuromyography in the territory of the affected nerve. In 03 cases it was observed complete pain relief within 15 days and return of symptoms of lower intensity after this period, 01 case of clinical improvement occurred after 45 days of application. No adverse drug effects were observed. CONCLUSION: the patients presented a high intensity neuropathic pain, it was continuous, persistent and refractory to the usual treatment. The use of TxBA showed up as a good therapeutic option in relieving pain condition, with better quality of life this patients.