Case Report: Diagnostic Pitfalls in an Atypical Case of Primary Neuritic Leprosy.

Primary neuritic leprosy is a form of leprosy clinically limited to the peripheral nerves without obvious skin lesions. Diagnosing leprosy in the absence of typical dermatological features is challenging and often causes a delay in diagnosis. We describe a case of primary neuritic leprosy with atypical features and the roles that histological confirmation using nerve biopsy of an unenlarged nerve and newer techniques, such as polymerase chain reaction and high-resolution ultrasonography, play in improving the diagnosis.

Clinical and pathological features of different types of leprosy.

OBJECTIVE: This study aimed to investigate the clinical and pathological features of leprosy bacillus-related neuropathy in different clinical stages. PATIENTS AND METHODS: Four patients confirmed to have leprosy between 2012 and 2020 were chosen as the primary study subjects. The patients' clinical data were retrospectively analyzed to investigate the clinical and pathological features of the different clinical types of leprosy. RESULTS: Each patient had a different degree of deformity. For instance, in Case 1, upon dermatological examination, the eyebrows were not falling off, and the face was slightly invaded. The great auricular nerve was thickened, and there were plaques on the back of the right hand with ill-defined edges but no itching. No atrophy in the thenar or hypothenar was observed, though pain and a warm sensation were noted. The swelling of the right leg was accompanied by sensory abnormalities. Moreover, the bilateral ulnar nerves were swollen, and demyelination changes were observed upon nerve biopsy. A small number of granulomas were noted in the nerve interstitium, and the acid-fast staining was positive. Additionally, in Case 2, on dermatological examination, scars were observed on the front of the tibia, and large, reddish, symmetrically distributed patches were seen on the back. Satellite foci were visible as well. Contracture was observed in the little finger, and the ulnar nerve was damaged. Skin and nerve biopsy revealed leprosy bacillus via acid-fast staining. CONCLUSIONS: Early detection and treatment are important methods of preventing and reducing damage to peripheral nerve function in patients with leprosy.

Endoneural abscess of common popliteal nerve as first clinical manifestation of leprosy: the first reported case in history in a low-incidence country.

A migrant from Palestine came to our attention for weakness of dorsiflexion of the left foot and hypoesthesia of the homolateral common peroneal nerve territory. Skin biopsies from skin lesions in the hypoesthetic area were not diagnostic. Radiological investigation showed focal nerve enlargement with a possible focal lesion. At this time, and given the uncertainty of the diagnosis, we had to choose between medical therapy with steroid and a surgical exploration of the nerve. We decided for the latter option. Intraoperatively, we found a focal round enlargement of the nerve. Epineurotomy was performed at that level, revealing a round caseous granulomatous mass that was excised. Microbiological examination revealed presence of Mycobacterium Leprae allowing diagnosis of leprosy. Medical therapy was then started, leading to resolution of clinical symptoms. Endoneural lepromatous abscesses are uncommon lesions that should be suspected in patients presenting with peripheral nerve dysfunction with anamnesis of travel in leprosy endemic regions or contacts with people from endemic regions with or even without skin lesions. Detection of endoneural abscesses is of critical importance because prompt surgical excision in conjunction with medical therapy leads to improvement of symptoms and permits correct diagnosis. In times of large human migrations from leprosy endemic areas, knowledge of this uncommon presentation of leprosy and its management will help lead to the best management of these patients.

Sarcoidosis with cutaneous perineural granulomas and neurological manifestations: A potential mimicker of leprosy.

Sarcoidosis is a granulomatous condition with diverse clinical presentations, including neurological findings. It was previously hypothesized that perineural sarcoidal granulomas in the skin may be an explanation of small-fiber neuropathy. Herein, we present a case of a 55 year old female with anesthetic cutaneous lesions mimicking leprosy clinically and histopathologically and discuss the importance of this differential diagnosis.

Evaluation of nerve growth factor serum level for early detection of leprosy disability.

INTRODUCTION: this research aimed to analyze nerve growth factor (NGF) contents as diagnostic tools for early disability in leprosy patients and the cut-off point value. METHODS: research samples consisted of 79 leprosy patients with disability grade 0 or 1 who met the clinically approved inclusion criteria. The age of patients ranged from 14 to 50 years. For both sample groups, blood serum was collected to determine NGF concentration. NGF level was analyzed by enzyme-linked immunosorbent assay (ELISA) according to the manual guide of the kit insert from Cussabio®. Statistical analysis used SPSS 17 software for Windows. A comparison was performed with the Student's t-test and the NGF concentration cut-off point was determined using a receiver operating characteristic (ROC) curve. RESULTS: the research result demonstrated that NGF concentration in multibacillary leprosy with disability grade 0 was higher than in grade 1. Leprosy with disability grade 0 had an NGF content reaching 100.46 pg/mL, while those with grade 1 had a lower concentration of NGF at 30.56 pg/mL. The higher disability grade indicated a lower NGF concentration in the blood serum. Based on the ROC analysis result, the NGF cut-off was shown to be 81.43 pg/mL. This result indicated that low NGF in nerve and skin lesions of leprosy patients contributes to early peripheral nerve malfunction due to Mycobacterium leprae infection. CONCLUSION: these results prove that NGF can be used as a marker of early disability in leprosy, with the cut-off value at 81.43 pg/mL.

Fibrosis: a distinguishing feature in the pathology of neural leprosy.

BACKGROUND: Fibrosis in the peripheral nerve is the end stage of leprous neuropathy and the cause of the resulting permanent neural function impairments. Preventive measures to avoid this irreversible pathological state are a relief strategy for leprosy sufferers. OBJECTIVES: The present study describes the frequency of fibrosis along with its characterisation and pathogenic development. METHODS: Six-hundred-and-thirteen nerve samples were sorted from 278 neural leprosy (NL) and 335 non-leprosy neuropathy patients (ON). The total number of samples was histologically examined by routine staining methods (haematoxylin-eosin, Wade staining and Gomori's trichrome) and fibrosis was evaluated via semi-quantitative estimation. FINDINGS: Fibrosis was most frequent in the NL group (33% against 0.4% in ON) while fibrosis in association with endoneurial microfasciculation was found in 38 (41.3%) of the NL samples in the examination of semithin sections. Pericytic activation in the perivascular environment was confirmed to be the source of the fibroblasts and perineurial cells delimiting microfascicles. End-stage fibrosis in leprosy displays an arrangement of microfascicles devoid of neural components (i.e., Schwann cells and axons) lined by an intermediate phenotype of fibroblastic-perineurial cells filled with bundles of collagen fibres. MAIN CONCLUSIONS: The present study underscores that fibrosis is frequently the severe end stage of neural leprosy NL pathogeny after analysing the notably distinct development of fibrosis within the neural environment.

Mycobacterium leprae-induced nerve damage: direct and indirect mechanisms.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae. This disease is characterized by skin and peripheral nerve trunk damage. The mechanisms responsible for the observed nerve damage in leprosy could be directly related to the ability of M. leprae to infect Schwann cells, leading to triggering of signaling events. Therefore, we hypothesize that in response to M. leprae infection, activation of the Notch signaling pathway in Schwann cells could play a crucial role in glial cell dedifferentiation. On the other hand, nerve damage evidenced in this disease may be additionally explained by indirect mechanisms such as the immune response and genetic susceptibility of the host. The understanding of the mechanisms leading to nerve damage induced by M. leprae infection will allow us to generate valuable tools for the early detection of leprosy as well as for the mitigation of the effects of this disabling disease.

As dores do eu na dor neuropática hansênica: uma leitura psicanalítica do fenômeno subjetivo / / The us pain in hansenic neuropathic pain: a psychoanalytic reading of the subjective phenomenon

O presente trabalho elucida o caso clínico de uma paciente em tratamento na Divisão de Reabilitação do Instituto “Lauro de Souza Lima” por consequência da dor neuropática hansênica em ambos os pés. Essa circunstância a remeteu a condições emocionais e psíquicas de difícil compreensão, a manifestação da dor no corpo passou a apresentar correspondências subjetivas e inconscientes, fazendo emergir com isso sofrimentos psíquicos frente á dor. Sendo assim, tivemos como objetivo geral investigar e compreender as manifestações emocionais presentes no seu discurso, e como específico analisar qual a relação estabelecida entre os processos subjetivos e a presença da dor neuropática. Nossa proposta foi embasada na metodologia de estudo de caso, apoiada no referencial teórico psicanalítico em que se evidenciou uma construção de elementos para uma investigação analítica. Concluímos nesse contexto que ocorreram rupturas nos investimentos narcísicos, no âmbito das relações afetivas, que intensificam a relação conflitiva entre o eu-corpo-dor. E quando esses conteúdos emergem à consciência, proporcionam elaboração psíquica e recursos para lidar com as dores do eu

The present study elucidates the clinical case of a patient undergoing treatment in the Rehabilitation Division of the "Lauro de Souza Lima" Institute due to leprosy neuropathic pain in both feet. This circumstance referred to emotional and psychic conditions of difficult comprehension, the manifestation of pain in the body began to present subjective and unconscious correspondences, thus causing psychic sufferings in the face of pain. Thus, we had as general objective to investigate and understand the emotional manifestations present in his discourse, and how specific to analyze the relationship established between the subjective processes and the presence of neuropathic pain. Our proposal was based on the methodology of a case study, based on the theoretical psychoanalytical framework in which a construction of elements for an analytical investigation was evidenced. We conclude in this context that there are ruptures in narcissistic investments, in the ambit of affective relations that intensify the conflicting relationship between the body-pain-self. And when these contents are presented to the conscious light, they provide psychic elaboration and resources to deal with the pains of the self

Avaliação para o manejo da úlcera neuropática associada à hanseníase: produção e validação de vídeo educativo / Evaluation for the management of leprosy-associated neuropathic ulcer: production and validation of educational video

A hanseníase é uma das maiores causas de complicações neuropáticas no mundo. Dentre estas, estão as úlceras neuropáticas, consideradas uma das causas mais comuns de amputações, morbidade e mortalidade, consequência de uma assistência à saúde embasada em procedimentos fragmentados com baixa resolutividade. Estudo de delineamento metodológico, com abordagem quali-quantitativa dos dados, que objetivou desenvolver um vídeo para intervenção educativa sobre avaliação para manejo da úlcera neuropática associada à hanseníase, a ser disponibilizado a enfermeiros. A pesquisa foi aprovada pelo Comitê de Ética em Pesquisa e foi realizada em 4 etapas: 1: Produção do roteiro e storyboard; 2:Validação do roteiro e storyboard; 3: Edição do vídeo e 4: Estudo piloto. A produção do conteúdo do roteiro e storyboard seguiu as recomendações do Ministério da Saúde e da Wound, Ostomy and Continence Nurses. A validação de conteúdo e aparência foi realizada por um comitê de especialistas. O instrumento apresentou os seguintes quesitos: objetivo; conteúdo; relevância; ambiente; funcionalidade, usabilidade e eficiência, os quais foram considerados validados a partir da concordância de 78% entre os avaliadores, calculada por meio do Índice de Validade de Conteúdo. Entre os especialistas na temática, houve maior prevalência do sexo feminino (88,9%), com idade entre 29 e 62 anos (média de 42,3 anos e desvio padrão de 11,0 anos), sendo a maior titulação acadêmica o doutorado (55,6%), e tempo de atuação profissional entre seis e 30 anos (média de 14,9 anos e desvio padrão de 8,2 anos). Dos especialistas em vídeo, a maioria era do sexo feminino (62,5%), com idade entre 30 e 46 anos (média de 37,5 anos e desvio padrão de 6,4 anos), com maior número de mestres (50%) e tempo de atuação profissional entre 2 e 24 anos (média de 10,8 anos e desvio padrão de 7,3 anos). Para todos os quesitos avaliados, obteve-se um Índice de Validade de Conteúdo superior ao mínimo preconizado. Após a adequação do roteiro e storyboard em face da validação, o vídeo foi gravado e, a partir de uma préedição, um estudo piloto (validação semântica) foi realizado por meio da técnica de grupo focal, com a participação de nove enfermeiros que atuavam na assistência à pessoa com hanseníase. Houve prevalência do sexo feminino (88,9%), com idade entre 29 e 50 anos e maior titulação acadêmica em nível de mestrado (55,6%), sendo que a maioria (55,6%) atuava na Estratégia Saúde da Família. A partir da análise de conteúdo do grupo focal, emergiram as categorias: dialogar sobre os objetivos propostos; identificar a aplicabilidade do vídeo na prática clínica do enfermeiro e valorizar a consulta de enfermagem na avaliação de pessoas com úlcera neuropática associada à hanseníase. Acredita-se que o vídeo educativo validado poderá auxiliar na realização de uma avaliação de enfermagem eficaz, segura e com qualidade à pessoa com úlcera neuropática decorrente da hanseníase

Leprosy is one of the major causes of neuropathic complications in the world. Among the complications most commonly found are neuropathic ulcers, considered one of the most common causes of amputations, morbidity and mortality in the world, as a consequence of health care based on fragmented procedures with low resolution. This is a methodological design study with qualitative and quantitative data, aiming to develop a video for educational intervention on evaluation for the management of neuropathic ulcer associated with leprosy, to be made available to nurses. The research was approved by the Research Ethics Committee and was carried out in 4 stages: 1: Production of the script and storyboard; 2: Script and storyboard validation; 3: Video editing and 4: Pilot study. The production of script content and storyboard followed the recommendations of the Ministry of Health and Wound, Ostomy and Continence Nurses. Validation of content and appearance was performed by a committee of experts. The instrument presented the following requirements: objective; content; relevance; environment; functionality, usability and efficiency, which were considered validated from the agreement of 78% between the evaluators, calculated through the Content Validity Index Among the specialists in the subject, there was a higher prevalence of female (88.9%), aged between 29 and 62 years (mean of 42.3 years and standard deviation of 11.0 years), the highest academic qualification being the doctoral degree (55.6%), and professional performance time between six and 30 years (mean of 14.9 years and standard deviation of 8.2 years). Of the video specialists, the majority were female (62.5%), aged between 30 and 46 years (mean of 37.5 years and standard deviation of 6.4 years), with a greater number of masters (50%) and time of professional activity between 2 and 24 years (mean of 10.8 years and standard deviation of 7.3 years). For all evaluated items, a Content Validity Index higher than the recommended minimum was obtained. After the adaptation of the script and storyboard in the face of validation, the video was recorded and, from a pre-edition, a pilot study (semantic validation) was performed through the focal group technique, with the participation of nine nurses who assisted the person with leprosy. There was a prevalence of females (88.9%), aged between 29 and 50 years, and a higher academic degree at the master's level (55.6%), with the majority (55.6%) working in the Family Health Strategy. From the content analysis of the focus group, the following categories emerged: dialogue about the proposed objectives; to identify the applicability of the video in the clinical practice of the nurse and to value the nursing consultation in the evaluation of people with neuropathic ulcer associated with leprosy. It is believed that the validated educational video can help in the performance of an effective, safe and quality nursing assessment to the person with neuropathic ulcer due to leprosy

Revisiting primary neural leprosy: Clinical, serological, molecular, and neurophysiological aspects.

BACKGROUND: Leprosy neuropathy is considered the most common peripheral neuropathy of infectious etiology worldwide, representing a public health problem. Clinical diagnosis of primary neural leprosy (PNL) is challenging, since no skin lesions are found and the slit skin smear bacilloscopy is negative. However, there are still controversial concepts regarding the primary-neural versus pure-neural leprosy definition, which will be explored by using multiple clinical-laboratory analyses in this study. METHODOLOGY/PRINCIPAL FINDINGS: Seventy patients diagnosed with primary neural leprosy from 2014 to 2016 underwent clinical, laboratorial and neurophysiological evaluation. All patients presented an asymmetric neural impairment, with nerve thickening in 58.6%. Electroneuromyography showed a pattern of mononeuropathy in 51.4%. Positivity for ELISA anti-PGL1 was 52.9%, while the qPCR of slit skin smear was 78.6%. The qPCR of nerve biopsies was positive in 60.8%. Patients with multiple mononeuropathy patterns showed lower levels of anti-PGL-1 (p = 0.0006), and higher frequency of neural thickening (p = 0.0008) and sensory symptoms (p = 0.01) than those with mononeuropathy. CONCLUSIONS/SIGNIFICANCE: PNL is not a synonym of pure neural leprosy, as this condition may include a generalized immune response and also a skin involvement, documented by molecular findings. Immunological, molecular, and neurophysiological tools must be implemented for diagnosing primary neural leprosy to achieve effective treatment and reduction of its resultant disabilities that still represent a public health problem in several developing nations. Finally, we propose a algorithm and recommendations for the diagnosis of primary neural leprosy based on the combination of the three clinical-laboratorial tools.

Evaluation of polymerase chain reaction in nerve biopsy specimens of patients with Hansen's disease.

BACKGROUND: Pure neuritic variety of leprosy (PNL) presents as peripheral neuropathy with absent skin lesions and negative skin smears. Diagnosing PNL is an uphill task as most of these patients have nonspecific changes on nerve biopsy. In such circumstances, additional molecular diagnostic tools like polymerase chain reaction (PCR) has proven to be useful in diagnosing leprosy. The present study was planned to evaluate the role of PCR in nerve biopsy specimens of patients with PNL. METHODS: Patients attending the neuromuscular clinic from January 2013 to June 2014 with mononeuropathy multiplex underwent detailed diagnostic evaluation to ascertain the cause of neuropathy. Patients where this evaluation failed to establish an etiology underwent a nerve biopsy. RESULTS: Nerve biopsy was done in 52 patients, of which 35 were diagnosed as pure neuritic leprosy. Definite leprosy with positive wade fite staining for lepra bacilli was seen in 13 patients and 22 biopsies revealed a probable leprosy without lepra bacilli being identified. PCR for M. leprae was positive in 22 patients (62%). 12 of the 13 cases with definite leprosy on histopathology were PCR positive while in the AFB negative group, PCR was positive in 10 cases. PCR had a sensitivity of 92.3%, specificity of 54.5%. The positive and negative predictive value of PCR was 54.5% and 92.3% respectively. CONCLUSIONS: PCR helps in diagnosing PNL in doubtful cases. A positive PCR increases the sensitivity of detection of M. leprae especially in cases of probable PNL group where AFB cannot be demonstrated on histopathology.

Perineural and intraneural cutaneous granulomas in granulomatous mycosis fungoides mimicking tuberculoid leprosy.

BACKGROUND: Histological similarities between granulomas and granulomatous mycosis fungoides (GMF) may lead to misdiagnoses of sarcoidosis or leprosy. METHODS: This report presents four patients with GMF in whom skin biopsies showed perineural and intraneural granulomas that were confused with tuberculoid leprosy granulomas. RESULTS: Patient 1 presented with erythematous plaques and bulky nodules. Biopsy findings suggested cutaneous sarcoidosis. Tumor resection showed granulomatous infiltrate extending to the fascia and skeletal muscle. Clinicopathological correlations permitted a diagnosis of GMF. Patient 2 presented with erythematous plaques. Skin biopsies had indicated sarcoidosis. Resection of a thigh nodule excluded leprosy, and GMF was diagnosed. Patient 3 presented with scaly, hyperpigmented plaques. Biopsy showed diffuse granulomatous inflammation with epithelioid and giant cells, abundant lymphocytes, and some eosinophils, and indicated GMF. Patient 4 presented with pruritic, erythematous plaques. Biopsy of an indurated mammary plaque initially indicated sarcoid granulomatous inflammation. Biopsy review suggested GMF. CONCLUSIONS: This study highlights both the diagnosis of GMF, and granulomatous cutaneous nerve injury in GMF and its possible confusion with leprosy granulomas. The histological diagnosis of GMF includes: (i) a granulomatous infiltrate rich in giant cells, emperipolesis, histiocytic cells, and scattered eosinophils, which may reach the fascia and muscle; (ii) the absence of elastic fibers or their phagocytosis by giant cells; and (iii) lymphocytes that may show atypia and epidermotropism. Deep biopsies reveal GMF diagnostic changes and, in conjunction with clinicopathological correlations, exclude a diagnosis of leprosy and support one of GMF, thus facilitating its appropriate management.

[Borderline leprosy as a rare differential diagnosis]. / Borderline-Lepra als seltene Differenzialdiagnose.

History and clinical findings | A 42-year-old migrant from Brazil presented with persistent sensory disturbances, skin discolorations and local alopecia in the upper limbs. Decisive for the presentation in our Tropical Medicine Clinic were new occurrences of severe pain and redness and swelling in the area of the lesions that had already been assessed by a number of medical specialists without a clear diagnosis could be made. Investigations and diagnosis | The histological analysis of skin biopsies showed perivascular, perineural, periadnexial lymphocytic and granulomatous dermatitis. In a direct microbiological preparation individual acid fast bacilli could be detected (Ziehl-Neelsen stain). The electroneurographical examination demonstrated a sensitive peripheral-neurogenic damage with emphasis on the right median nerve and the left ulnar and radial nerves. Thermography revealed an increased heating or cooling threshold. The serological investigation by ELISA for IgM antibodies against the phenolic glycolipid (PGL-1) was positive (titer 1 : 1200). In summary, the diagnosis of borderline leprosy (infection with Mycobacterium leprae) with transition to multibacillary leprosy (according to WHO) and leprosy reaction type 1 was made. Treatment and course | We initiated an oral antimycobacterial therapy (multidrug therapy, MDT) with rifampin, clofazimine and dapsone for 12 months (WHO regimen for multibacillary leprosy). Leprosy reaction type 1 was treated with prednisolone and by increasing the dose of clofazimine. Analgesic therapy on demand was carried out with nonsteroidal anti-inflammatory drugs (ibuprofen). MDT and successful management of leprosy reaction lead to a rapid improvement of symptoms. Conclusions | Leprosy is an infectious disease occurring only rarely in Germany (average incidence of 1-2 cases per year) that is diagnosed almost exclusively among migrants. Main symptoms comprise non-itchy, reddish, touch insensitive skin lesions or nerve deficits. The diagnosis is based primarily on the clinical presentation, supplemented by pathogen detection, histology, neurophysiological findings and serology. Standard therapy is a combination of rifampin, clofazimine and dapsone (WHO scheme) for at least 6 months.

Unsolved matters in leprosy: a descriptive review and call for further research.

Leprosy, a chronic mycobacterial infection caused by Mycobacterium leprae, is an infectious disease that has ravaged human societies throughout millennia. This ancestral pathogen causes disfiguring cutaneous lesions, peripheral nerve injury, ostearticular deformity, limb loss and dysfunction, blindness and stigma. Despite ongoing efforts in interrupting leprosy transmission, large numbers of new cases are persistently identified in many endemic areas. Moreover, at the time of diagnosis, most newly identified cases have considerable neurologic disability. Many challenges remain in our understanding of the epidemiology of leprosy including: (a) the precise mode and route of transmission; (b) the socioeconomic, environmental, and behavioral factors that promote its transmission; and

Leprous neuromyositis: A rare clinical entity and review of the literature.

Mycobacterium leprae, the causative agent of leprosy (Hansen's disease), is a slow growing intracellular acid-fast bacillus that affects the skin, peripheral nerves and respiratory tract. In patients with suppressed cell-mediated immunity, the infiltration of the Bacilli can produce disseminated illness such as leprous neuromyositis. We reported a case of 56-year-old gentleman presenting with pyrexia of unknown origin, asymmetric sensory motor axonal polyneuropathy and was on chronic exogenous steroid therapy. On evaluation, his skin, muscle, nerve and bone marrow biopsy showed numerous globi of acid-fast Bacilli suggestive of leprous neuromyositis, a rare form of disseminated Hansen's disease. We reported this case in view of its rarity, atypical manifestation of a relatively rare disease and literature review on poor electrophysiological correlation in the diagnosis of leprous neuromyositis as compared to the histopathological examination.

¿Cuál es su diagnóstico? Lepra lepromatosa multibacilar / No disponible

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Leprosy: a 'common' and curable cause of peripheral neuropathy with skin lesions.

Leprosy (or Hansen's disease) is a curable chronic infectious disease caused by the acid-fast bacillus Mycobacterium leprae. While leprosy remains one of the most common causes of neuropathy worldwide, its rarity in the UK means that many doctors are unfamiliar with the typical clinical features. This is problematic because early recognition and treatment is vital in order to minimise disease-related complications such as nerve injury. We describe a 75-year-old man who presented with multiple mononeuropathy (mononeuritis multiplex, particularly affecting the ulnar nerves) and typical granulomatous skin lesions, in whom the diagnosis was made on the basis of skin biopsy. We highlight the clinical features, investigations and treatment of the patient, and provide information about the epidemiology and pathogenesis of leprosy.

A practical approach to enlargement of nerves, plexuses and roots.

Detecting enlargement of accessible nerves is very helpful in assessing patients with peripheral nerve disorders, as only a few types of neuropathy lead to nerve thickening. The three leading causes are leprosy, hereditary motor and sensory neuropathies (types 1 and 3) and chronic inflammatory demyelinating neuropathies. MRI, neurography and ultrasonography allow assessment of clinically inaccessible portions of deep-seated nerves, plexuses and roots. As a result, isolated proximal segment thickenings, as found in chronic inflammatory sensory polyradiculopathy, can now be better evaluated and managed. Similarly, focal nerve enlargements due to infection, inflammation, infiltration and neoplasm are being identified and treated effectively. We present a practical approach to the diagnosis and management of patients with enlarged peripheral nerves, plexuses and roots, including cranial nerves.

CXCL10, MCP-1, and other immunologic markers involved in neural leprosy.

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. This study investigated the immunohistochemical expression of immunomarkers involved in the pathogenic mechanisms of leprosy nerve damage. These markers selected were CXCL10, CCL2 chemokines and immunomarkers as CD3, CD4, CD8, CD45RA, CD45RO, CD68, HLA-DR, and metalloproteinases 2 and 9 (MMP2 and MMP9) occurring in nerve biopsy specimens collected from leprosy (23) and nonleprosy patients (5) suffering peripheral neuropathy. CXCL10, CCL2, MMP2, and MMP9 immunoreactivities were found in the leprosy nerves but not in nonleprosy samples. Immunolabeling was predominantly found in recruited macrophages and Schwann cells composing the inflammatory cellular population in the leprosy-affected nerves. The immunohistochemical expression of all the markers, but CXCL10, was associated with fibrosis, however, only CCL2 was, independently from the others, associated with this excessive deposit of extracellular matrix. No difference in the frequency of the immunolabeling was detected between the AFB⁺ and AFB⁻ leprosy subgroups of nerve, exception made to some statistical trend to difference in regard to CD68⁻ and HLA-DR⁺ cells in the AFB⁻ nerves exhibiting epithelioid granuloma. MMP9 expression associated with fibrosis is consistent with previous results of research group. The findings conveys the idea that CCL2 and CXCL10 chemokines at least in advanced stages of leprosy nerve lesions are not determinant for the establishment of AFB⁺ or AFB⁻ leprosy lesions, however, CCL2 is associated with macrophage recruitment and fibrosis.