RESUMEN
BACKGROUND AND AIMS: Leprosy is a chronic infectious disease caused by Mycobacterium leprae (M. leprae), an intracellular bacillus that systematically invades the peripheral nerves. Diagnosing leprosy neuropathy is still a defying skill, and late diagnosis and treatment are still a reality. Based on the biological characteristics of M. leprae, particularly its preference for invading the Schwann cells localized at the coldest areas of human body, we hypothesized that these areas have focal demyelination that may escape detection through standard nerve conduction studies (NCSs) protocols. METHODS: Twenty-five patients with confirmed multibacillary leprosy and 14 controls were accessed. A multisegmented NCS protocol (MP) was performed, targeting short segments through the coldest areas, to identify focal areas of slowed conduction velocity. The effectiveness of this multisegmented protocol was compared to the standard protocol (SP) to detect abnormalities. RESULTS: All leprosy patients presented an abnormal study with the MP, contrasting to 19 with the SP. The most frequent NCS pattern was an asymmetric neuropathy with focal slowing of conduction velocity, found in 23 out of 25 leprosy patients. Significant differences favoring the proposed method were observed when comparing the MP with the SP. Notably, the MP increased the sensitivity to detect abnormalities by 122%, 133%, and 257% for the median, peroneal, and tibial nerves, respectively. MP also increases sensitivity to detect focal abnormalities in the ulnar nerve. INTERPRETATION: The MP protocol significantly increases the sensitivity of NCSs to detect neurophysiological abnormalities in leprosy neuropathy.
Asunto(s)
Conducción Nerviosa , Humanos , Conducción Nerviosa/fisiología , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Lepra/fisiopatología , Lepra/complicaciones , Adulto Joven , Nervios Periféricos/fisiopatología , Lepra Multibacilar/fisiopatología , Lepra Multibacilar/diagnósticoRESUMEN
OBJECTIVE: To evaluate the prevalence of altered touch perception in the feet of individuals with diabetes mellitus and the associated risk factors. METHOD: Cross-sectional study with 224 individuals with diabetes mellitus conducted in an endocrinology clinic at a public hospital in Campina Grande, Paraíba. The evaluation used touch sensitivity and perception, and a descriptive and multivariate analysis with Poisson regression was performed. RESULTS: We found the prevalence of altered touch perception to be 53.1%. The risk factors that had a significant and joint impact on its occurrence were: female gender; previous ulcer; diabetes mellitus type 2; burning sensation, cracks, fissures, calluses, and Charcot foot. CONCLUSIONS: This study found a high prevalence of altered perception of touch, and this should support the planning of actions aimed at preventing the problem. The study showed the relevance of the phenomenon as a nursing diagnosis that could be included in NANDA-International.
Asunto(s)
Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 2/complicaciones , Neuropatías Diabéticas/epidemiología , Trastornos de la Sensación/epidemiología , Percepción del Tacto , Adulto , Anciano , Anciano de 80 o más Años , Estudios Transversales , Diabetes Mellitus Tipo 1/epidemiología , Diabetes Mellitus Tipo 2/epidemiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Nervios Periféricos/fisiopatología , Prevalencia , Factores de Riesgo , Distribución por SexoRESUMEN
Abstract Background and objectives: Leprosy remains a leading cause of peripheral neuropathy and disability in the world. Primary objective of the study was to determine the incidence of deformities present at a time of diagnosis and new deformities that patients develop over follow up period. Material and methods: An open, retrospective cohort study was performed at a tertiary medical center in western India. Recruitment phase of the study was of 2 years (2009-2010) followed by observation/follow up phase of 7 years till 31st December 2017. New patients with leprosy and released from treatment cases who presented with deformity as defined by WHO disability grade (1998) and subsequently developing new deformities during the follow up period of up to 7 years were included in the study. Results: The study included 200 leprosy patients. Of the total 254 deformities, 168 (66.14%) deformities were noticed at the moment of diagnosis, 20 (7.87%) deformities occurred during the follow up phase. Of all patients, 21.25% had Grade 1 deformity and 6.31% had Grade 2 or more severe deformity. Deformities of hand were most common in 44.48%, followed by feet 39.76%, and face 15.74% respectively. Limitation of study: Mode of inclusion of patient was self-reporting during follow up phase so there is possible under reporting of the disabilities. Conclusion: New deformities continue to develop in certain forms of leprosy even after release from treatment. Long-term & regular follow up of patients who have been released from treatment is required.
Asunto(s)
Humanos , Masculino , Femenino , Evaluación de la Discapacidad , Lepra/fisiopatología , Lepra/patología , Nervios Periféricos/fisiopatología , Factores de Tiempo , Índice de Severidad de la Enfermedad , Deformidades Adquiridas del Pie/fisiopatología , Deformidades Adquiridas del Pie/patología , Deformidades Adquiridas de la Mano/fisiopatología , Deformidades Adquiridas de la Mano/patología , Registros Médicos , Estudios Transversales , Estudios Retrospectivos , Estudios de Seguimiento , Progresión de la Enfermedad , Cara/anomalías , IndiaRESUMEN
BACKGROUND AND OBJECTIVES: Leprosy remains a leading cause of peripheral neuropathy and disability in the world. Primary objective of the study was to determine the incidence of deformities present at a time of diagnosis and new deformities that patients develop over follow up period. MATERIAL AND METHODS: An open, retrospective cohort study was performed at a tertiary medical center in western India. Recruitment phase of the study was of 2 years (2009-2010) followed by observation/follow up phase of 7 years till 31st December 2017. New patients with leprosy and released from treatment cases who presented with deformity as defined by WHO disability grade (1998) and subsequently developing new deformities during the follow up period of up to 7 years were included in the study. RESULTS: The study included 200 leprosy patients. Of the total 254 deformities, 168 (66.14%) deformities were noticed at the moment of diagnosis, 20 (7.87%) deformities occurred during the follow up phase. Of all patients, 21.25% had Grade 1 deformity and 6.31% had Grade 2 or more severe deformity. Deformities of hand were most common in 44.48%, followed by feet 39.76%, and face 15.74% respectively. LIMITATION OF STUDY: Mode of inclusion of patient was self-reporting during follow up phase so there is possible under reporting of the disabilities. CONCLUSION: New deformities continue to develop in certain forms of leprosy even after release from treatment. Long-term & regular follow up of patients who have been released from treatment is required.
Asunto(s)
Evaluación de la Discapacidad , Lepra/patología , Lepra/fisiopatología , Estudios Transversales , Progresión de la Enfermedad , Cara/anomalías , Femenino , Estudios de Seguimiento , Deformidades Adquiridas del Pie/patología , Deformidades Adquiridas del Pie/fisiopatología , Deformidades Adquiridas de la Mano/patología , Deformidades Adquiridas de la Mano/fisiopatología , Humanos , India , Masculino , Registros Médicos , Nervios Periféricos/fisiopatología , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de TiempoRESUMEN
ABSTRACT Objective: To evaluate the prevalence of altered touch perception in the feet of individuals with diabetes mellitus and the associated risk factors. Method: Cross-sectional study with 224 individuals with diabetes mellitus conducted in an endocrinology clinic at a public hospital in Campina Grande, Paraíba. The evaluation used touch sensitivity and perception, and a descriptive and multivariate analysis with Poisson regression was performed. Results: We found the prevalence of altered touch perception to be 53.1%. The risk factors that had a significant and joint impact on its occurrence were: female gender; previous ulcer; diabetes mellitus type 2; burning sensation, cracks, fissures, calluses, and Charcot foot. Conclusions: This study found a high prevalence of altered perception of touch, and this should support the planning of actions aimed at preventing the problem. The study showed the relevance of the phenomenon as a nursing diagnosis that could be included in NANDA-International.
RESUMEN Objetivo: Evaluar la prevalencia de la percepción alterada del tacto en los pies de individuos con diabetes mellitus y los factores de riesgo asociados. Método: Estudio transversal con 224 individuos con diabetes mellitus conducido en ambulatorio de endocrinología del hospital público de Campina Grande, Paraíba. Testes de sensibilidad y percepción del tacto han sido empleados en la evaluación; y ha sido realizado análisis descriptivo y multivariado con regresión de Poisson. Resultados: Ha sido encontrado prevalencia de la percepción alterada del tacto de 53,1%. Los factores de riesgo que tuvieron impacto de forma significativa y conjunta en su ocurrencia han sido: sexo femenino; úlcera previa; diabetes mellitus tipo 2; irritación, rajaduras, fisuras, callosidades y pies de Charcot. Conclusiones: Alta prevalencia de la percepción alterada del tacto ha sido encontrada, y esta debe subsidiar el planeamiento de acciones vueltas para la prevención del problema. El estudio evidenció la relevancia del fenómeno en cuanto un diagnóstico de enfermaría pasible de inclusión en la NANDA International.
RESUMO Objetivo: Avaliar a prevalência da percepção do tato alterada nos pés de indivíduos com diabetes mellitus e os fatores de risco associados. Método: Estudo transversal com 224 indivíduos com diabetes mellitus conduzido em ambulatório de endocrinologia de hospital público de Campina Grande, Paraíba. Testes de sensibilidade e percepção do tato foram empregados na avaliação; e foi realizada análise descritiva e multivariada com regressão de Poisson. Resultados: Encontrou-se prevalência da percepção do tato alterada de 53,1%. Os fatores de risco que tiveram impacto de forma significativa e conjunta na sua ocorrência foram: sexo feminino; úlcera prévia; diabetes mellitus tipo 2; queimação, rachaduras, fissuras, calosidades e pés de Charcot. Conclusões: Alta prevalência da percepção do tato alterada foi encontrada, e esta deve subsidiar o planejamento de ações voltadas para a prevenção do problema. O estudo evidenciou a relevância do fenômeno enquanto um diagnóstico de enfermagem passível de inclusão na NANDA-International.
Asunto(s)
Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Trastornos de la Sensación/epidemiología , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 2/complicaciones , Neuropatías Diabéticas/epidemiología , Percepción del Tacto , Nervios Periféricos/fisiopatología , Prevalencia , Estudios Transversales , Factores de Riesgo , Distribución por Sexo , Diabetes Mellitus Tipo 1/epidemiología , Diabetes Mellitus Tipo 2/epidemiologíaRESUMEN
KEY POINTS: Hypoxaemia evokes a repertoire of homeostatic adjustments that maintain oxygen supply to organs and tissues including the brain and skeletal muscles. Because hypertensive patients have impaired endothelial-dependent vasodilatation and an increased sympathetic response to arterial oxygen desaturation, we investigated whether hypertension impairs isocapnic hypoxia-induced cerebral and skeletal muscle hyperaemia to an extent that limits oxygen supply. In middle-aged hypertensive men, vertebral and femoral artery blood flow do not increase in response to isocapnic hypoxia, limiting brain and peripheral hyperaemia and oxygen supply. Increased chemoreflex-induced sympathetic activation impairs skeletal muscle perfusion and oxygen supply, whereas an attenuation of local vasodilatory signalling in the posterior cerebrovasculature reduced brain hyperperfusion of hypertensive middle-aged men in response to isocapnic hypoxia. ABSTRACT: The present study investigated whether hypertension impairs isocapnic hypoxia (IH)-induced cerebral and skeletal muscle hyperaemia to an extent that limits oxygen supply. Oxygen saturation (oxymetry), mean arterial pressure (photoplethysmography) and muscle sympathetic nerve activity (MSNA; microneugraphy), as well as femoral artery (FA), internal carotid artery and vertebral artery (VA) blood flow (BF; Doppler ultrasound), were quantified in nine normotensive (NT) (aged 40 ± 11 years, systolic pressure 119 ± 7 mmHg and diastolic pressure 73 ± 6 mmHg) and nine hypertensive men (HT) (aged 44 ± 12 years, systolic pressure 152 ± 11 mmHg and diastolic pressure 90 ± 9 mmHg) during 5 min of normoxia (21% O2 ) and IH (10% O2 ). Total cerebral blood flow (tCBF), brain (CDO2 ) and leg (LDO2 ) oxygen delivery were estimated. IH provoked similar oxygen desaturation without changing mean arterial pressure. Internal carotid artery perfusion increased in both groups during IH. However, VA and FA BF only increased in NT. Thus, IH-induced increase in tCBF was smaller in HT. CDO2 only increased in NT and LDO2 decreased in HT. Furthermore, IH evoked a greater increase in HT MSNA. Changes in MSNA were inversely related to FA BF, LDO2 and end-tidal oxygen tension. In conclusion, hypertension disturbs regional and total cerebrovascular and peripheral responses to IH and consequently limits oxygen supply to the brain and skeletal muscle. Although increased chemoreflex-induced sympathetic activation may explain impaired peripheral perfusion, attenuated vasodilatory signalling in the posterior cerebrovasculature appears to be responsible for the small increase in tCBF when HT were exposed to IH.
Asunto(s)
Circulación Cerebrovascular , Hipertensión/etiología , Hipoxia/fisiopatología , Oxígeno/administración & dosificación , Flujo Sanguíneo Regional , Vasodilatación , Adulto , Estudios de Casos y Controles , Femenino , Arteria Femoral/fisiopatología , Hemodinámica , Humanos , Hipertensión/patología , Masculino , Persona de Mediana Edad , Consumo de Oxígeno , Nervios Periféricos/fisiopatología , Arteria Vertebral/fisiopatologíaRESUMEN
Axonal Charcot-Marie-Tooth disease (CMT) represents an expanding group of inherited motor and sensory neuropathies in clinical practice. SACS-gene related disorders have been associated with complex neurological phenotypes of early-onset cerebellar ataxia, spastic-ataxia, spastic paraplegia, demyelinating neuropathy and variable ophthalmological, cognitive and psychiatric disturbances, but never related to pure axonal neuropathy phenotypes. Two unrelated Brazilian men with early-onset axonal CMT-like presentations associated with SACS gene mutations are presented. Both patients presented with pure sensorimotor axonal neuropathy without cerebellar ataxia, spastic paraplegia or other systemic and neurological involvement. Classical neuroimaging findings observed in other sacsinopathies were observed in both cases. Homozygous pathogenic mutations were found in SACS gene in both patients. SACS gene mutations can be associated with pure axonal sensorimotor neuropathy without other neurological features, but with typical neuroimaging features of other sacsinopathies, disclosing the importance of performing neuroimaging studies in patients with suspected axonal CMT.
Asunto(s)
Enfermedad de Charcot-Marie-Tooth/genética , Proteínas de Choque Térmico/genética , Mutación , Adulto , Encéfalo/diagnóstico por imagen , Enfermedad de Charcot-Marie-Tooth/diagnóstico por imagen , Enfermedad de Charcot-Marie-Tooth/patología , Enfermedad de Charcot-Marie-Tooth/fisiopatología , Diagnóstico Diferencial , Humanos , Masculino , Nervios Periféricos/patología , Nervios Periféricos/fisiopatología , Fenotipo , Adulto JovenRESUMEN
OBJECTIVE: To describe the etiologies and frequency of traumatic peripheral nerve injury (TPNI) seen in the electrodiagnostic laboratory of a tertiary care hospital in Puerto Rico. METHODS: The charts of patients who underwent an electrodiagnostic study for a TPNI were revised. The main outcome measure was the frequency of each injury by anatomic location, specific nerve or nerves affected, injury mechanism, and injury severity. RESULTS: One hundred forty-six charts were included, and in them were listed a total of 163 nerve injuries; 109 (74.7%) cases were men and 37 (25.3%) were women. The mean age was 33.6 years. The facial nerve, the brachial plexus, and the ulnar nerve were more frequently injured than any other nerve or nerve bundle. The ulnar, sciatic, median, and radial nerves and the lumbosacral plexus were more commonly injured as a result of gunshot wounds than of any other mechanism of injury. The brachial plexus was most frequently injured in motor vehicle accidents and the facial nerve injuries most commonly had an iatrogenic cause. In terms of injury severity, 84.2% were incomplete and 15.8% were complete. CONCLUSION: TPNIs are common in young individuals and potentially can lead to significant disability. Further studies are needed to assess the socioeconomic impact of these injuries on our population.
Asunto(s)
Electrodiagnóstico/métodos , Traumatismos de los Nervios Periféricos/epidemiología , Nervios Periféricos/fisiopatología , Accidentes de Tránsito/estadística & datos numéricos , Adolescente , Adulto , Anciano , Femenino , Humanos , Enfermedad Iatrogénica/epidemiología , Masculino , Persona de Mediana Edad , Traumatismos de los Nervios Periféricos/diagnóstico , Traumatismos de los Nervios Periféricos/etiología , Puerto Rico , Estudios Retrospectivos , Centros de Atención Terciaria , Índices de Gravedad del Trauma , Heridas por Arma de Fuego/complicaciones , Heridas por Arma de Fuego/epidemiología , Adulto JovenRESUMEN
Peripheral neuropathy is frequent in spinocerebellar ataxia type 2 (SCA2), but the pattern and characteristics of nerve involvement are still an unsettled issue. This study aimed to evaluate the prevalence, extent, and distribution of nerve involvement in SCA2 patients through neurophysiological studies. Thirty-one SCA2 patients and 20 control subjects were enrolled in this study. All subjects were prospectively evaluated through electromyography, including nerve conduction, needle electromyography in proximal and distal muscles of the upper and lower limbs, and sural radial amplitude ratio (SRAR). We aimed to differentiate distal axonopathy from diffuse nerve commitment, characterizing neuronopathy. Nerve involvement was observed in 83.6 % (26 individuals) of SCA2 patients. Among these, 19 had diffuse sensory abnormalities on nerve conduction predominantly on the upper limbs, with diffuse chronic denervation on needle electromyography and elevated SRAR values. Four individuals had only diffuse sensory involvement, and 2 had only motor involvement on needle evaluation and normal nerve conduction. These were interpreted as neuronopathy due to the diffuse distribution of the involvement. One individual had distal sensory axonopathy, with lower limb predominance. In this study, we found neuronopathy as the main pattern of nerve involvement in SCA2 patients and that motor involvement is a frequent feature. This information brings new insights into the understanding of the pathophysiology of nerve involvement in SCA2 and sets some key points about the phenotype, which is relevant to guide the genetic/molecular diagnosis.
Asunto(s)
Nervios Periféricos/fisiopatología , Ataxias Espinocerebelosas/fisiopatología , Adulto , Electromiografía , Femenino , Humanos , Extremidad Inferior/fisiopatología , Masculino , Músculo Esquelético/fisiopatología , Conducción Nerviosa , Neurofisiología , Fenotipo , Estudios Prospectivos , Trastornos de la Sensación/etiología , Trastornos de la Sensación/fisiopatología , Ataxias Espinocerebelosas/complicaciones , Extremidad Superior/fisiopatologíaRESUMEN
Objective: Patients with Wilson’s disease (WD) may develop a wide variety of neuropsychiatric symptoms, but there are few reports of autonomic dysfunction. Here, we described evidence of small fiber and/or autonomic dysfunction in 4 patients with WD and levodopa-responsive parkinsonism. Method: We reviewed the charts of 4 patients with WD who underwent evaluation for the presence of neuromuscular dysfunction and water-induced skin wrinkling test (SWT). Results: Two men and 2 women (33±3.5 years) with WD were evaluated. They all had parkinsonism at some point during their disease course. Parkinsonism on patient 4 almost completely subsided with treatment of WD. Two patients had significant sensory and 2 significant autonomic complaints, including syncopal spells. NCS/EMG was normal in all but SWT was abnormal in half of them (mean 4-digit wrinkling of 0.25 and 1). Discussion: A subset of patients with WD exhibit evidence of abnormal skin wrinkling test (small fiber neuropathy). .
Objetivo: Pacientes com doença de Wilson (DW) podem desenvolver uma ampla variedade de sintomas neuropsiquiátricos, mas existem poucos relatos de disfunção autonômica. Aqui, nós descrevemos evidência de disfunção de fibras finas/autonômica em 4 pacientes com DW e parkinsonismo responsivo à levodopa. Método: Nós revisamos os prontuários de 4 pacientes com DW que foram submetidos a avaliação neuromuscular e ao teste de quantificação do enrugamento cutâneo (TEC). Resultados: Dois homens e 2 mulheres (33±3,5 anos) com DW foram avaliados. Todos apresentaram parkinsonismo durante o curso de sua doença. Parkinsonismo no paciente 4 quase completamente desapareceu com tratamento da DW. Dois pacientes apresentaram queixas sensitivas e 2 apresentaram queixas autonômicas significativas incluindo episódios de síncope. Eletroneuromiografia foi normal em todos e TEC foi anormal em metade deles (score do TEC nos 4 dedos de 0,25 e 1). Discussão: Um subgrupo de pacientes com DW apresenta evidência de TEC anormal (neuropatia de fibras finas). .
Asunto(s)
Adulto , Femenino , Humanos , Masculino , Adulto Joven , Degeneración Hepatolenticular/fisiopatología , Fibras Nerviosas/fisiología , Conducción Nerviosa/fisiología , Nervios Periféricos/fisiopatología , ElectromiografíaRESUMEN
OBJECTIVE: Patients with Wilson's disease (WD) may develop a wide variety of neuropsychiatric symptoms, but there are few reports of autonomic dysfunction. Here, we described evidence of small fiber and/or autonomic dysfunction in 4 patients with WD and levodopa-responsive parkinsonism. METHOD: We reviewed the charts of 4 patients with WD who underwent evaluation for the presence of neuromuscular dysfunction and water-induced skin wrinkling test (SWT). RESULTS: Two men and 2 women (33±3.5 years) with WD were evaluated. They all had parkinsonism at some point during their disease course. Parkinsonism on patient 4 almost completely subsided with treatment of WD. Two patients had significant sensory and 2 significant autonomic complaints, including syncopal spells. NCS/EMG was normal in all but SWT was abnormal in half of them (mean 4-digit wrinkling of 0.25 and 1). DISCUSSION: A subset of patients with WD exhibit evidence of abnormal skin wrinkling test (small fiber neuropathy).
Asunto(s)
Degeneración Hepatolenticular/fisiopatología , Fibras Nerviosas/fisiología , Conducción Nerviosa/fisiología , Nervios Periféricos/fisiopatología , Adulto , Electromiografía , Femenino , Humanos , Masculino , Adulto JovenRESUMEN
O diagnóstico da hanseníase neural pura baseia-se em dados clínicos e laboratoriais do paciente, incluindo a histopatologia de espécimes de biópsia de nervo e detecção de DNA de Mycobacterium leprae (M. leprae) pelo PCR. Como o exame histopatológico e a técnica PCR podem não ser suficientes para confirmar o diagnóstico, a imunomarcação de lipoarabinomanana (LAM) e/ou Glicolipídio fenólico 1 (PGL1) - componentes de parede celular de M. leprae foi utilizada na primeira etapa deste estudo, na tentativa de detectar qualquer presença vestigial do M. leprae em amostras de nervo sem bacilos. Além disso, sabe-se que a lesão do nervo na hanseníase pode diretamente ser induzida pelo M. leprae nos estágios iniciais da infecção, no entanto, os mecanismos imunomediados adicionam severidade ao comprometimento da função neural em períodos sintomáticos da doença. Este estudo investigou também a expressão imuno-histoquímica de marcadores envolvidos nos mecanismos de patogenicidade do dano ao nervo na hanseníase. Os imunomarcadores selecionados foram: quimiocinas CXCL10, CCL2, CD3, CD4, CD8, CD45RA, CD45RO, CD68, HLA-DR, e metaloproteinases 2 e 9. O estudo foi desenvolvido em espécimes de biópsias congeladas de nervo coletados de pacientes com HNP (n=23 / 6 BAAR+ e 17 BAAR - PCR +) e pacientes diagnosticados com outras neuropatias (n=5) utilizados como controle. Todas as amostras foram criosseccionadas e submetidas à imunoperoxidase. Os resultados iniciais demonstraram que as 6 amostras de nervos BAAR+ são LAM+/PGL1+. Já entre as 17 amostras de nervos BAAR-, 8 são LAM+ e/ou PGL1+. Nas 17 amostras de nervos BAAR-PCR+, apenas 7 tiveram resultados LAM+ e/ou PGL1+. A detecção de imunorreatividade para LAM e PGL1 nas amostras de nervo do grupo HNP contribuiu para a maior eficiência diagnóstica na ausência recursos a diagnósticos moleculares...
The diagnosis of pure neural leprosy (PNL) is based on clinical and laboratory data, including the histopathology of nerve biopsy specimens and detection of M. leprae DNA by polymerase chain reaction (PCR). Given that histopathological examination and PCR methods may not be sufficient to confirm diagnosis, immunolabeling of lipoarabinomanan (LAM) and/or phenolic glycolipid 1 (PGL1) M. leprae wall components were utilized in the first step of this investigation in an attempt to detect any vestigial presence of M. leprae in AFB- nerve samples. Furthermore, it´s well known that nerve damage in leprosy can be directly induced by Mycobacterium leprae in the early stages of infection; however, immunomediated mechanisms add gravity to the impairment of neural function in symptomatic periods of the disease. Therefore, this study also investigated the immunohistochemical expression of immunomarkers involved in the pathogenic mechanisms of leprosy nerve damage. These markers selected were CXCL10, CCL2 chemokines and CD3, CD4, CD8, CD45RA, CD45RO, CD68, HLA-DR, metalloproteinases 2 and 9 in nerve biopsy specimens collected from leprosy (23) and nonleprosy patients (5) suffering peripheral neuropathy. Twenty-three PNL nerve samples (6 AFB+ and 17 AFB-PCR+) were cryosectioned and submitted to LAM and PGL1 immunohistochemical staining by immunoperoxidase; 5 nonleprosy nerve samples were used as controls. The 6 AFB-positive samples showed LAM/PGL1 immunoreactivity. Among the 17 AFB- samples, only 8 revealed LAM and/or PGL1 immunoreactivity. In 17 AFB-PCR+ patients, just 7 had LAM and/or PGL1-positive nerve results. In the PNL cases, the detection of immunolabeled LAM and PGL1 in the nerve samples would have contributed to enhanced diagnostic efficiency in the absence of molecular diagnostic facilities...
Asunto(s)
Humanos , Lepra/diagnóstico , Mycobacterium leprae/patogenicidad , Lepra/patología , Biomarcadores/análisis , Mycobacterium leprae/crecimiento & desarrollo , Nervios Periféricos/fisiopatología , Reacción en Cadena de la Polimerasa , Piel/inervación , Índice de Severidad de la EnfermedadRESUMEN
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.
Asunto(s)
Lepra , Enfermedades del Sistema Nervioso Periférico , Diagnóstico Diferencial , Humanos , Lepra/diagnóstico , Lepra/fisiopatología , Nervios Periféricos/fisiopatología , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Enfermedades del Sistema Nervioso Periférico/fisiopatologíaRESUMEN
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.
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Humanos , Lepra , Enfermedades del Sistema Nervioso Periférico , Diagnóstico Diferencial , Lepra/diagnóstico , Lepra/fisiopatología , Nervios Periféricos/fisiopatología , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Enfermedades del Sistema Nervioso Periférico/fisiopatologíaRESUMEN
INTRODUCTION: Focal peripheral neuropathy of the median nerve is mainly caused by a traumatic event or pressure, but it may also be produced by systemic illnesses. Among the latter, leprosy is a rare cause. METHODS: Six cases of isolated median neuropathy as the first sign of leprosy were selected from patients with an exclusively neurological complaint as the initial symptom. The patients, evaluated at the National Leprosy Reference Center in Rio de Janeiro, Brazil, followed routine and specialized procedures. RESULTS: Three of the patients had pure neural leprosy, and 3 had skin lesions. Clinical median nerve function impairment was confirmed by neurophysiological testing and histopathology. Both mononeuritis and mononeuritis multiplex were observed. CONCLUSIONS: This case series demonstrates an additional form of presentation of leprosy, which, if not diagnosed and treated in time, may lead to permanent disability.
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Lepra/fisiopatología , Neuropatía Mediana/patología , Neuropatía Mediana/fisiopatología , Adulto , Electromiografía , Potenciales Evocados Motores/fisiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Dimensión del Dolor , Nervios Periféricos/patología , Nervios Periféricos/fisiopatología , Piel/patología , Muñeca/inervación , Adulto JovenRESUMEN
UNLABELLED: Diabetic polyneuropathy can be confirmed by nerve conduction studies. The data can be analyzed in the form of a combined index instead of individual parameters. METHODS: The combined index included five parameters of nerve conduction studies commonly used for evaluation of polyneuropathies. We evaluated sensitivity in 100 diabetic patients with suspected polyneuropathy, and specificity in 200 non-diabetic patients with suspected lumbosacral radiculopathy. All results were expressed in number of standard deviations (SD). RESULTS: The sensitivity of the combined index was 81 or 74%, and specificity was 97 or 98%, using respectively -2.0 or -2.5 SD as cutoff. The range of sensitivity of the other parameters was 57-65% or 48-56%, and specificity range was 96-98% or 98-100%, using the same criteria. DISCUSSION: The combined index had higher sensitivity and equivalent specificity compared to isolated parameters.
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Neuropatías Diabéticas/diagnóstico , Conducción Nerviosa/fisiología , Nervios Periféricos/fisiopatología , Adulto , Anciano , Estudios de Casos y Controles , Neuropatías Diabéticas/fisiopatología , Electrodiagnóstico/instrumentación , Electrodiagnóstico/métodos , Electrofisiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Diabetic polyneuropathy can be confirmed by nerve conduction studies. The data can be analyzed in the form of a combined index instead of individual parameters. METHODS: The combined index included five parameters of nerve conduction studies commonly used for evaluation of polyneuropathies. We evaluated sensitivity in 100 diabetic patients with suspected polyneuropathy, and specificity in 200 non-diabetic patients with suspected lumbosacral radiculopathy. All results were expressed in number of standard deviations (SD). RESULTS: The sensitivity of the combined index was 81 or 74%, and specificity was 97 or 98%, using respectively -2.0 or -2.5 SD as cutoff. The range of sensitivity of the other parameters was 57-65% or 48-56%, and specificity range was 96-98% or 98-100%, using the same criteria. DISCUSSION: The combined index had higher sensitivity and equivalent specificity compared to isolated parameters.
A polineuropatia diabética pode ser confirmada por estudos de condução nervosa. Os dados podem ser analisados por um índice combinado ao invés de parâmetros isolados. MÉTODOS: O índice combinado incluiu cinco parâmetros usados rotineiramente na avaliação de polineuropatias. Avaliamos a sensibilidade em 100 pacientes diabéticos com suspeita de polineuropatia e a especificidade em 200 pacientes não diabéticos com suspeita de radiculopatia lombossacral. Todos os resultados foram expressos em número de desvios-padrão (DP). RESULTADOS: A sensibilidade do índice combinado foi 81 ou 74%, e a especificidade foi 97 ou 98%, usando respectivamente -2,0 ou -2,5 DP como ponto de corte. A sensibilidade dos parâmetros isolados oscilou entre 57-65% ou 48-56%, e a especificidade foi 96-98% ou 98-100%, usando os mesmos critérios. DISCUSSÃO: O índice combinado apresentou melhor sensibilidade e especificidade equivalente aos parâmetros isolados.
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Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Neuropatías Diabéticas/diagnóstico , Conducción Nerviosa/fisiología , Nervios Periféricos/fisiopatología , Estudios de Casos y Controles , Neuropatías Diabéticas/fisiopatología , Electrofisiología , Electrodiagnóstico/instrumentación , Electrodiagnóstico/métodos , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
SETTINGS: Many believe that the regular treatment for multibacillary (MB) leprosy cases could be shortened. A shorter treatment, allowing for uniform treatment for all cases, makes case classification superfluous and therefore simplifies leprosy control. OBJECTIVE: To evaluate the association of the treatment duration with the frequency of reactions among MB patients. METHODS: An open-label randomised clinical trial to compare the present routine treatment with one lasting six months. Patients were recruited between March 2007 and February 2012. We analysed the frequency of first reaction with the Kaplan-Meier method and of recurrent reaction with a Poisson regression, using the treatment group and baciloscopic index level (BI) as independent variables. Logistic regression was used to evaluate the statistical association of different reaction types and the treatment group. RESULTS: Among those with BI < 3, we found a statistical significant difference of reaction frequencies between the treatment groups from 6 to 18 months since the beginning of treatment. This difference disappears at 2 years after the start of treatment. Multiple reactions were associated with the treatment group and with BI > or = 3. No specific types of reactions were associated with treatment duration. CONCLUSION: Although this is the first report of U-MDT/CT-BR, the results presented here support the possibility of use of UMDT in the field.
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Leprostáticos/uso terapéutico , Lepra Multibacilar/tratamiento farmacológico , Mycobacterium leprae/efectos de los fármacos , Adulto , Brasil/epidemiología , Quimioterapia Combinada , Eritema Nudoso/diagnóstico , Eritema Nudoso/epidemiología , Femenino , Humanos , Incidencia , Estimación de Kaplan-Meier , Leprostáticos/efectos adversos , Lepra Multibacilar/diagnóstico , Lepra Multibacilar/epidemiología , Modelos Logísticos , Masculino , Persona de Mediana Edad , Mycobacterium leprae/aislamiento & purificación , Neuritis/diagnóstico , Neuritis/epidemiología , Nervios Periféricos/fisiopatología , Recurrencia , Factores de Tiempo , Resultado del Tratamiento , Organización Mundial de la SaludRESUMEN
OBJETIVO: Determinar, através de dissecção em cadáveres frescos, a anatomia topográfica do nervo tibial e seus ramos ao nível do tornozelo, em relação ao túnel do tarso. MÉTODOS: Foram realizadas dissecções bilaterais em 26 cadáveres frescos e as localizações da bifurcação do nervo tibial e seus ramos aferidas em milímetros, com relação ao eixo maleolar-calcaneal (EMC). Para os ramos calcâneos, a quantidade e seus respectivos nervos de origens também foram analisados. RESULTADOS: A bifurcação do nervo tibial ocorreu sob o túnel em 88% dos casos e proximalmente em 12%. Quanto aos ramos calcâneos, o medial apresentou-se com um (58%), dois (34%) e três (8%) ramos, com a origem mais comum do nervo tibial (90%) e o inferior com ramo único por perna, tendo o nervo plantar lateral como origem mais comum (70%). Nivel de Evidência V, Opinião de especialista.
OBJECTIVE: Determine, through dissection in fresh cadavers, the topographic anatomy of the tibial nerve and its branches at the ankle, in relation to the tarsal tunnel. METHODS: Bilateral dissections were performed on 26 fresh cadavers and the locations of the tibial nerve bifurcation and its branches were measured in millimeters. For the calcaneal branches, the amount and their respective nerves of origin were also analyzed. RESULTS: The tibial nerve bifurcation occurred under the tunnel in 88% of the cases and proximally in 12%. As for the calcaneal branches, the medial presented with one (58%), two (34%) and three (8%) branches, with the most common source occurring in the tibial nerve (90%) and the lower with a single branch per leg and lateral plantar nerve as origin most common (70%). Level of Evidence, V Expert opinion.