Hypothesis: Bacteria benefiting from electromagnetic field in peripheral neuropathy.

Recent research has already been shown widespread locations of bacteria in various tissues and organs of a healthy host organism. These bacteria (hereinafter referred to as persistent microbiota, PM) cause neither noticeable destruction nor toxins production - no immune response can be noticed either. The role of the PM is unknown. The host nervous system is not an exception and can also be inhabited by the PM. We found that various bacteria were capable of benefiting from the electromagnetic field (EMF). The main advantage of these bacteria, apparently, lies in the increasing supply of ionic forms of compounds into the cells. Since microorganisms use the energy of electrical impulses, their possible colonization of the host's nerve circuit will weaken the nerve signals. The presented hypothesis aims to draw attention to the following points: i) microbial colonization of the host nervous system will lead to the weakening of nerve signals, ii) the sensitivity of bacteria to EMF permits to affect on their activity with electromagnetic treatment.

Functional Outcome of Early, Selective Surgical Nerve Decompression in Leprous Neuropathy.

Background: Leprous neuropathy is a significant, yet preventable, cause of disability worldwide. Decompressive surgery and oral steroids have been used along with Multi Drug Therapy (MDT) for treating leprous neuropathy with varied success as reported in literature. Methods: We prospectively studied 16 peripheral nerves in 10 patients with leprous neuropathy of less than a year duration and not responding to steroid therapy in 3 weeks. The patients were divided into 2 groups: Group-A (decompressive nerve surgery was done within 12 weeks of onset of neurological deficit), and Group-B (nerve decompression was performed after 12 weeks from onset of neurological deficit). Post-operatively patients were assessed for regression of deformity, sensory, motor, vasomotor recovery and neuropathic pain. Results: Median age of patients was 32 years (range; 18 years to 46 years). Mean motor score and mean grip strength was significantly better for group A patients at 2 years follow-up (p < 0.05). Mean sensory score improved significantly in both the groups (p < 0.05). Similarly, mean VAS score for neuropathic pain improved significantly in both the groups (p < 0.05). Recovery of autonomic function was observed in 3 nerves in group A and 1 in group B. Conclusions: The cases who underwent nerve decompression surgery within 12 weeks had better functional outcomes, especially in terms of motor recovery, than those who were operated after that. Studies involving larger number of patients are required to draw firm conclusions.

Appropriately Selected Nerve in Suspected Leprous Neuropathy Yields High Positive Results for Mycobacterium leprae DNA by Polymerase Chain Reaction Method.

Identification of Mycobacterium leprae DNA by polymerase chain reaction (PCR) is a reliable and an affordable method to confirm leprosy. DNA from 87 nerve samples (61 from paraffin blocks and 26 fresh samples) was extracted. Mycobacterium leprae DNA was amplified by PCR from 80/87 (92%) specimens. Patients were seen over a period of 11 years (2007-2019), and leprosy was diagnosed based on clinical and characteristic histopathology findings. The clinical diagnostic possibilities were as follows: leprous neuropathy in 73/80 (91.3%), mononeuritis multiplex of unknown etiology in four (5.0%), vasculitic neuropathy in two (2.5%), and distal symmetric sensory motor neuropathy in one (1.3%). The biopsied nerves were as follows: superficial radial = 34 (42.6%), dorsal cutaneous branch of ulnar = 19 (23.8%), sural = 18 (22.5%), and superficial peroneal = 9 (11.3%), and corresponding neurological deficits were recorded in 77 (96.3%) cases. The histopathological diagnoses in total group were as follows: (borderline tuberculoid (BT) = 52, tuberculoid (TT) = 8, borderline lepromatous (BL) = 8, borderline borderline (BB) = 3, nonspecific inflammation = 3, healed/fibrosed = 4, and axonopathy = 2). Acid fast bacilli (AFB) was demonstrated in 11 (13.7%) samples. For comparison, 31 clinically and histopathologically defined non-leprous disease control nerves (inherited neuropathy = 20, vasculitis = 8, and nutritional neuropathy = 3) subjected to PCR were negative for M. leprae DNA. In most instances, there are multiple thickened peripheral nerves in suspected cases of leprosy, but neurological deficits pertaining to the thickened nerve are not as widespread. The current findings emphasize the importance of selecting the most appropriate nerve for biopsy to obtain a positive PCR result. We infer that clinical, histopathological, and PCR tests complement each other to help achieve a definitive diagnosis of leprosy particularly in pure neuritic leprosy and in leprous neuropathy with negative skin smears/biopsy.

Epidemiological aspects of leprosy.

Leprosy is a bacterial infection causing severe disfigurement of the affected individual. It is considered as an ancient disease affecting humanity since thousands of years and also has tremendous stigma associated with it. It is known as a neglected tropical disease. In spite of all the efforts, the disease remains a major healthcare distress in many underdeveloped and developing countries like India and Brazil. Thus, to understand the disease and implement various strategies successfully, one need to understand the epidemiological aspect of the disease along with various operational factors influencing the epidemiological data. Thus, the present paper describes the various epidemiological facts and figures of leprosy along with the suggestions and measures to tackle this global ailment.

Herpes zoster complicated by phrenic nerve palsy and respiratory compromise.

BACKGROUND: Herpes zoster can be associated with severe neurological complications. CASE PRESENTATION: In this article, we describe the case of a 54-year-old man with herpes zoster affecting his right upper chest and neck region complicated by phrenic nerve palsy and respiratory compromise. The diagnosis of herpes zoster was made based on the classic appearance of the rash and associated neuropathic-type pain. The diagnosis of phrenic nerve palsy was made by chest x-ray and ultrasound. CONCLUSION: Clinicians should be aware of the possibility of phrenic nerve palsy occurring in patients who have herpes zoster affecting the region of C3,4,5 dermatomes. Although symptoms of unilateral diaphragmatic paresis are usually mild, in patients with obesity or comorbid lung disease, new onset phrenic nerve palsy can lead to significant respiratory compromise.

Molecular, immunological and neurophysiological evaluations for early diagnosis of neural impairment in seropositive leprosy household contacts.

BACKGROUND: Household contacts constitute the highest risk group for leprosy development, and despite significant progress in the disease control, early diagnosis remains the primary goals for leprosy management programs. METHODS: We have recruited 175 seropositive and 35 seronegative household contacts from 2014 to 2016, who were subjected to an extensive protocol that included clinical, molecular (peripheral blood qPCR, slit-skin smear qPCR, skin biopsy qPCR) and electroneuromyographic evaluations. RESULTS/PRINCIPAL FINDINGS: The positivity of peripheral blood qPCR of seropositive contacts was 40.6% (71/175) whereas only 8.6% (3/35) were qPCR positive in seronegative contacts (p = 0.0003). For the slit-skin smear, only 4% (7/175) of seropositive contacts presented positive bacilloscopy, whereas the qPCR detected 47.4% (83/175) positivity in this group compared with only 17.1% (6/35) in seronegative contacts (p = 0.0009). In the ENMG evaluation of contacts, 31.4% (55/175) of seropositives presented some neural impairment, and 13.3% (4/35) in seronegatives (p = 0.0163). The presence of neural thickening conferred a 2.94-fold higher chance of ENMG abnormality (p = 0.0031). Seropositive contacts presented a 4.04-fold higher chance of neural impairment (p = 0.0206). The peripheral blood qPCR positivity presented odds 2.08-fold higher towards neural impairment (OR, 2.08; p = 0.028). Contrarily, the presence of at least one BCG vaccine scar demonstrated 2.44-fold greater protection against neural impairment (OR = 0.41; p = 0.044). CONCLUSIONS/SIGNIFICANCE: ELISA anti-PGL-I is the most important test in determining the increased chance of neural impairment in asymptomatic leprosy household contacts. The combination of the two assays (ELISA anti-PGL-I and peripheral blood qPCR) and the presence of BCG scar may identify individuals with higher chances of developing leprosy neuropathy, corroborating with the early diagnosis and treatment.

Infections of the Nervous System.

Microorganisms can affect the entire neuraxis, producing a variety of neurologic complications that frequently entail prolonged hospitalizations and complicated treatment regimens. The spread of pathogens to new regions and the reemergence of opportunistic organisms in immunocompromised patients pose increasing challenges to health care professionals. Because rapid diagnosis and treatment may prevent long-term neurologic sequelae, providers should approach these diseases with a structured, neuroanatomic framework, incorporating a thorough history, examination, laboratory analysis, and neuroimaging in their clinical reasoning and decision-making.

Early detection of neuropathy in leprosy: a comparison of five tests for field settings.

BACKGROUND: Early detection and treatment of neuropathy in leprosy is important to prevent disabilities. A recent study showed that the Nerve Conduction Studies (NCS) and Warm Detection Thresholds (WDT) tests can detect leprosy neuropathy the earliest. These two tests are not practical under field conditions, however, because they require climate-controlled rooms and highly trained staff and are expensive. We assessed the usefulness of alternative test methods and their sensitivity and specificity to detect neuropathy at an early stage. METHODS: Through a literature search we identified five alternative devices that appeared user-friendly, more affordable, portable and/or battery-operated: the Neuropad®, Vibratip™, NC-Stat®DPNCheck™, NeuroQuick and the Thermal Sensibility Tester (TST), assessing respectively sweat function, vibration sensation, nerve conduction, cold sensation and warm sensation. In leprosy patients in Bangladesh, the posterior tibial and sural nerves that tested normal for the monofilament test and voluntary muscle test were assessed with the NCS and WDT as reference standard tests. The alternative devices were then tested on 94 nerves with abnormal WDT and/or NCS results and on 94 unaffected nerves. Sensitivity and specificity were the main outcomes. RESULTS: The NeuroQuick and the TST showed very good sensitivity and specificity. On the sural nerve, the NeuroQuick had both a sensitivity and a specificity of 86%. The TST had a sensitivity of 83% and a specificity of 82%. Both the NC-Stat®DPNCheck™ and Vibratip™ had a high specificity (88% and 100%), but a low sensitivity (16% and 0%). On the posterior tibial nerve, the NeuroQuick and the TST also showed good sensitivity, but the sensitivity was lower than for the sural nerve. The Neuropad® had a sensitivity of 56% and a specificity of 61%. CONCLUSIONS: The NeuroQuick and TST are good candidates for further field-testing for reliability and reproducibility. The feasibility of production on a larger scale should be examined.

Nerve Damage in Young Patients with Leprosy Diagnosed in an Endemic Area of the Brazilian Amazon: A Cross-Sectional Study.

OBJECTIVE: To describe nerve damage and its association with clinical and epidemiologic characteristics in young patients with leprosy diagnosed in an endemic area of the Brazilian Amazon. STUDY DESIGN: All 45 patients with leprosy younger than 15 years of age and diagnosed at a health referral unit in northern Brazil were invited to participate in a cross-sectional, descriptive, analytical study. Subjects were submitted to a templated simple neurologic examination of the peripheral nerves and answered a structured questionnaire. RESULTS: Of 41 cases, referral was the mode of detection in 33 participants (80.5%); 19 (46.3%) had been seen by 3 or more physicians to obtain a diagnosis, and 26 (63.4%) had received other diagnoses. The interval between the onset of symptoms and diagnosis was more than 1 year in 30 cases (73.2%). Borderline leprosy was the predominant clinical form (48.8%); 63.4% of the participants had multibacillary leprosy, 31.7% had nerve damage, and 17.1% exhibited disabilities. The following variables showed a statistically significant association (P???.05) with nerve damage at diagnosis: home visit by the community health worker, number of doctors seen, number of skin lesions (>5), and lesions along the path of nerve trunks. CONCLUSION: Centralized healthcare, a low frequency of home visits by community health workers, and the difficulty in diagnosing leprosy in children are factors that contribute to late treatment initiation and an increased risk of peripheral nerve damage. In addition, multiple skin lesions and lesions along the path of nerve trunks require rigorous monitoring.

Minocycline in leprosy patients with recent onset clinical nerve function impairment.

Nerve function impairment (NFI) in leprosy may occur and progress despite multidrug therapy alone or in combination with corticosteroids. We observed improvement in neuritis when minocycline was administered in patients with type 2 lepra reaction. This prompted us to investigate the role of minocycline in recent onset NFI, especially in corticosteroid unresponsive leprosy patients. Leprosy patients with recent onset clinical NFI (<6 months), as determined by Monofilament Test (MFT) and Voluntary Muscle Test (VMT), were recruited. Minocycline 100mg/day was given for 3 months to these patients. The primary outcome was the proportion of patients with 'restored,' 'improved,' 'stabilized,' or 'deteriorated' NFI. Secondary outcomes included any improvement in nerve tenderness and pain. In this pilot study, 11 patients were recruited. The progression of NFI was halted in all; with 9 out of 11 patients (81.82%) showing ?restored? or ?improved? sensory or motor nerve functions, on assessment with MFT and VMT. No serious adverse effects due to minocycline were observed. Our pilot study demonstrates the efficacy and safety of minocycline in recent onset NFI in leprosy patients. However, larger and long term comparative trials are needed to validate the efficacy of minocycline in leprosy neuropathy.

[Pure neural leprosy. Diagnostic aspects of a clinical case]. / Lepra neural pura. Aspectos diagnosticos en un caso clinico.

INTRODUCTION: Leprosy is an infectious disease caused by the bacteria Mycobacterium leprae. It is particularly prone to affect the skin and the nerve trunks and, in fact, both are compromised in most infected patients. It is transmitted by exposure to those with the disease and sometimes by reactivation. One uncommon possibility is pure neural leprosy, which is characterised by neuropathy, but without skin lesions. We report the case of a patient with pure neural leprosy and review the diagnostic aspects. CASE REPORT: A 40-year-old male, an immigrant who was diagnosed and treated for leprosy 20 earlier. The patient visited due to painful paraesthesias and dysesthesias in the hands and legs without the presence of any skin lesions. Acute multiple mononeuritis with mainly ulnar involvement was observed. The disease, typified as paucibacillary/tuberculoid, was treated and in a few weeks there was a clear improvement. CONCLUSIONS: In this case of pure neural leprosy due to reactivation, early diagnosis allowed timely treatment to be established. Evaluation of neuropathy together with clinical, electrophysiological and ultrasound criteria is recommended. By so doing, a high degree of sensitivity is achieved as well as allowing early diagnosis and treatment, and therefore a better functional recovery.

TITLE: Lepra neural pura. Aspectos diagnosticos en un caso clinico.Introduccion. La lepra es una enfermedad infecciosa causada por la bacteria Mycobacterium leprae. Presenta especial avidez por la piel y los troncos nerviosos, y, de hecho, ambos se afectan en la mayor parte de los infectados. Se trasmite por exposicion con enfermos y en ocasiones por reactivacion. Una posibilidad inhabitual es la lepra neural pura, caracterizada por neuropatia, pero sin lesiones en la piel. Se describe un paciente con lepra neural pura y se revisan los aspectos diagnosticos. Caso clinico. Varon de 40 años, inmigrante, diagnosticado y tratado de lepra 20 años antes. Acudio por parestesias y disestesias dolorosas en las manos y las piernas sin lesiones en la piel. Se demostro mononeuritis multiple aguda con principal afectacion de cubitales. La enfermedad, tipificada como tuberculoide paucibacilar, se trato y en pocas semanas la mejoria fue evidente. Conclusiones. En este caso de lepra neural pura por reactivacion, el diagnostico temprano permitio un rapido tratamiento. Es recomendable la evaluacion de la neuropatia integrada con criterios clinicos, electrofisiologicos y ecograficos. De este modo se consigue una alta sensibilidad y especialmente una precocidad en el diagnostico y la instauracion del tratamiento, y por consecuencia una mejor recuperacion funcional.

Early predictive factors for lower-extremity motor or sensory deficits and surgical results of patients with spinal tuberculosis: A retrospective study of 329 patients.

Many studies about the characteristics of spinal tuberculosis (STB) have been published, but none has investigated the predictive factors for lower-extremity motor or sensory deficits (LMSD) in patients with STB.The objective of this study was to find early predictive factors for LMSD and evaluate surgical results of patients with STB.From 2001 through 2010, 329 patients with STB were treated in our department and surgical treatment was performed in 274 patients. The factors assessed included age, sex, duration of symptoms, worsening of illness, clinical symptoms, clinical signs, imaging characteristics, kyphotic angle, Oswestry disability index (ODI), and visual analogue scale (VAS) scores.Of the 329 patients studied, 164 presented with LMSD (the LMSD group), of which 93 patients (28.3%) had motor deficits and 177 patients (53.8%) had sensory disturbance. The other 165 patients were included in the control group (the No LMSD group). Using univariate logistic regression analysis, we found that the sex (P = 0.042), age (P = 0.001), worsening of sickness (P = 0.013), location (P = 0.009), and spinal compression (P = 0.035) were the risk factors of LMSD. Furthermore, the multivariate logistic regression analysis indicated that age (OR = 1.761, 95% CI: 1.227-2.526, P = 0.002), worsening of sickness (yes vs no: OR = 1.910, 95% CI: 1.161-3.141, P = 0.011), location (T vs C: OR = 0.204, 95% CI: 0.063-0.662, P = 0.008), and spinal compression (yes vs no: OR = 1.672, 95% CI: 1.020-2.741, P = 0.042) were independent risk factors of LMSD. Surgical treatment was performed in 274 patients. The kyphotic angle improved from 25.8 ±â€Š9.1° preoperatively to 14.0 ±â€Š7.6°, with a mean correction of 11.8 ±â€Š4.0°, and a mean correction loss of 1.5 ±â€Š1.8° at final visit. There were significant differences between the preoperative and the final ODI and VAS scores in both groups (P < 0.001 and P < 0.001, respectively).Spinal tuberculosis with cervical or lumbar vertebra involvement among the elder patients with a history of worsening of illness and spinal compression tended to cause LMSD, such as motor deficits or sensory disturbance. We should implement an appropriate treatment regimen to prevent exacerbation of STB such as operation, which can achieve thoroughness of debridement, adequate spinal stabilization, and better functional recovery.

Renal amyloidosis in leprosy, an infrequent cause of nephrotic syndrome in Europe.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae The main clinical manifestations involve the skin and the peripheral nervous system. Several types of nephropathy have been described in leprosy. One frequent form of renal involvement is amyloidosis, especially in patients with lepromatous leprosy. In these patients, end-stage renal disease is an important contributor to morbidity and mortality. Here, we present the case of a patient with nephrotic syndrome caused by secondary amyloidosis, chronic peripheral neuropathy and a history of leprosy. The patient was correctly treated in her youth, which is the best way to avoid renal pathology, but she developed a nephrotic syndrome years later.

The role of primary infection of Schwann cells in the aetiology of infective inflammatory neuropathies.

Numerous different pathogens are responsible for infective peripheral neuropathies and this is generally the result of the indirect effects of pathogen infection, namely anti pathogen antibodies cross reacting with epitopes on peripheral nerve, auto reactive T cells attacking myelin, circulating immune complexes and complement fixation. Primary infection of Schwann cells (SC) associated with peripheral nerve inflammation is rare requiring pathogens to cross the Blood Peripheral Nerve Barrier (BPNB) evade anti-pathogen innate immune pathways and invade the SC. Spirochetes Borrelia bourgdorferi and Trepomema pallidum are highly invasive, express surface lipo proteins, but despite this SC are rarely infected. However, Trypanosoma cruzi (Chaga's disease) and Mycobacterium leprae. Leprosy are two important causes of peripheral nerve infection and both demonstrate primary infection of SC. This is due to two novel strategies; T. cruzi express a trans-silalidase that mimics host neurotrophic factors and infects SC via tyrosine kinase receptors. M. leprae demonstrates multi receptor SC tropism and subsequent infection promotes nuclear reprogramming and dedifferentiation of host SC into progenitor stem like cells (pSLC) that are vulnerable to M. leprae infection. These two novel pathogen evasion strategies, involving stem cells and receptor mimicry, provide potential therapeutic targets relevant to the prevention of peripheral nerve inflammation by inhibiting primary SC infection.

A Randomized Controlled Double Blind Trial of Ciclosporin versus Prednisolone in the Management of Leprosy Patients with New Type 1 Reaction, in Ethiopia.

BACKGROUND: Leprosy Type 1 (T1R) reactions are immune-mediated events leading to nerve damage and preventable disability affecting hands, feet and eyes. Type 1 Reactions are treated with oral corticosteroids. There is little evidence on alternative treatments for patients who do not respond to steroids or experience steroid adverse effects. We report the results of a randomized controlled trial testing the efficacy and adverse effect profile of ciclosporin and prednisolone (CnP) in comparison to prednisolone only (P) in patients with new T1R in Ethiopia. Ciclosporin is a potent immunosuppressant. Outcomes were measured using a clinical severity score, recurrence rate, adverse events and quality of life. RESULTS: Seventy three patients with new T1R were randomized to receive CnP or P for 20 weeks. Recovery rates in skin signs was similar in both groups (91% vs 88%). Improvements in nerve function both, new and old, sensory (66% vs 49%) and motor (75% vs 74%) loss were higher (but not significantly so) in the patients on CnP. Recurrences rates of T1R (85%) were high in both groups, and recurrences occurred significantly earlier (8 weeks) in patients CnP, who needed 10% more additional prednisolone. Serious major and minor adverse events rates were similar in patients in the two treatment arms of the study. Both groups had a significant improvement in their quality of life after the study, measured by the SF-36. CONCLUSIONS: This is the first double-blind RCT assessing ciclosporin, in the management of T1R in Africa. Ciclosporin could be a safe alternative second-line drug for patients with T1R who are not improving with prednisolone or are experiencing adverse events related to prednisolone. This study illustrates the difficulty in switching off leprosy inflammation. Better treatment agents for leprosy patients with reactions and nerve damage are needed.

Occupational Exposure to Swine, Poultry, and Cattle and Antibody Biomarkers of Campylobacter jejuni Exposure and Autoimmune Peripheral Neuropathy.

INTRODUCTION: Foodborne Campylobacter jejuni infection has been associated with an increased risk of autoimmune peripheral neuropathy, but risks of occupational exposure to C. jejuni have received less attention. This study compared anti-C. jejuni IgA, IgG, and IgM antibody levels, as well as the likelihood of testing positive for any of five anti-ganglioside autoantibodies, between animal farmers and non-farmers. Anti-C. jejuni antibody levels were also compared between farmers with different animal herd or flock sizes. The relationship between anti-C. jejuni antibody levels and detection of anti-ganglioside autoantibodies was also assessed. METHODS: Serum samples from 129 Agricultural Health Study swine farmers (some of whom also worked with other animals) and 46 non-farmers, all from Iowa, were analyzed for anti-C. jejuni antibodies and anti-ganglioside autoantibodies using ELISA. Information on animal exposures was assessed using questionnaire data. Anti-C. jejuni antibody levels were compared using Mann-Whitney tests and linear regression on log-transformed outcomes. Fisher's Exact Tests and logistic regression were used to compare likelihood of positivity for anti-ganglioside autoantibodies. RESULTS: Farmers had significantly higher levels of anti-C. jejuni IgA (p < 0.0001) and IgG (p = 0.02) antibodies compared to non-farmers. There was no consistent pattern of anti-C. jejuni antibody levels based on animal herd or flock size. A higher percentage of farmers (21%) tested positive for anti-ganglioside autoantibodies compared to non-farmers (9%), but this difference was not statistically significant (p = 0.11). There was no significant association between anti-C. jejuni antibody levels and anti-ganglioside autoantibodies. CONCLUSIONS: The findings provide evidence that farmers who work with animals may be at increased risk of exposure to C. jejuni. Future research should include longitudinal studies of exposures and outcomes, as well as studies of interventions to reduce exposure. Policies to reduce occupational exposure to C. jejuni should be considered.

Central and peripheral nervous system involvement in neuromelioidosis.

We report a case of a 33-year-old Sri Lankan man who presented with flaccid quadriparesis with brainstem signs and acute motor axonal polyneuropathy. MRI of the brain showed multiple abscesses with ring enhancement seen predominantly in the brainstem and upper cervical cord. The patient was initially treated with intravenous immunoglobulin, considering this to be a form of Guillain-Barré syndrome. Cerebrospinal fluid, however, showed lymphocytic pleocytosis with raised protein. Tests for Brucella, tuberculosis, toxoplasmosis, syphilis and HIV were negative. Chest X-ray revealed a cavity in the left lung, which, on bronchoscopy, showed a collection of purulent secretions. Culture of these secretions grew Burkholderia pseudomallei. The patient was treated with two courses of intravenous antibiotics, with resultant radiological improvement; however, with significant morbidity.

Hansen Neuropathy: Still a Possible Diagnosis in the Investigation of a Peripheral Neuropathy.

INTRODUCTION: Leprosy is still one of the most frequent causes of peripheral neuropathy. Although regarded as eradicated in Portugal, is still documented in neuropathological study of patients with clinical peripheral neuropathy without proper diagnosis. MATERIAL AND METHODS: Review of the cases of Hansen disease neuropathy diagnosed in Neuropathology Unit of Centro Hospitalar do Porto between 1978 and 2013, atending to gender, age, clinical manifestations and neuropathological findings. RESULTS: Twenty one patients were identified with neuropathological diagnosis of Hansenâs disease neuropathy, predominantly male. The mean age at diagnosis was 52 years, and sensory symptoms predominate as neurological manifestation of disease. Interval between symptoms and diagnosis was 1-38 years. In most nerve samples tuberculoid type of disease was identified. Bacilli were detected in skin and nerve in 44% of cases. DISCUSSION: Mononeuritis is the most common presentation of leprosy but other clinical manifestations are possible, including skin lesions. Infection with M. leprae injures myelinated and unmyelinated fibres, with replacement of nerve tissue by collagen fibrosis. The diagnosis of leprosy is only achieved by neuropathological study of skin lesions and / or peripheral nerve, supported by the identification of the bacillus. CONCLUSION: Hansen disease remains a public health problem in tropical areas and, although rare, still described in Western countries reason why should still be considered as a diagnostic possibility in the investigation of peripheral neuropathy.

Introdução: A lepra continua a ser uma das causas mais frequentes de neuropatia periférica. Apesar de tida como erradicada em Portugal, ainda se documenta no estudo neuropatológico de doentes com clínica de neuropatia periférica sem diagnóstico etiológico definido.Material e Métodos: Revisão dos casos neuropatia por doença de Hansen diagnosticados na Unidade de Neuropatologia do Centro Hospitalar do Porto no período de 1978 e 2013 atendendo ao género, idade, manifestações clínicas e achados neuropatológicos.Resultados: Foram identificados 21 doentes com diagnóstico neuropatológico de neuropatia por doença de Hansen, com predomínio do sexo masculino. A idade média ao diagnóstico foi de 52 anos, sendo a sintomatologia sensitiva predominante. O intervalo entre sintomatologia e diagnóstico oscilou entre 1 a 38 anos. Na maioria foi identificada forma tuberculoide em biópsia de nervo e detetados bacilos em pele e nervo em 44% dos casos.Discussão: A mononeurite é a forma mais comum de apresentação de hanseníase, podendo cursar com outras manifestações clínicas incluindo lesões cutâneas. A infeção pelo M. leprae lesiona fibras mielinizadas e não mielinizadas, com substituição do tecido nervoso por colagénio resultando em fibrose. O diagnóstico da lepra é apenas conseguido por estudo neuropatológico das lesões cutâneas e/ou nervo periférico, adjuvado pela identificação do bacilo.Conclusão: A doença de Hansen continua a ser um problema de saúde pública em áreas tropicais e, apesar de rara, ainda descrita em países ocidentais, devendo ser considerada como uma hipótese de diagnóstico na investigação de neuropatia periférica.

Mechanisms of distal axonal degeneration in peripheral neuropathies.

Peripheral neuropathy is a common complication of a variety of diseases and treatments, including diabetes, cancer chemotherapy, and infectious causes (HIV, hepatitis C, and Campylobacter jejuni). Despite the fundamental difference between these insults, peripheral neuropathy develops as a combination of just six primary mechanisms: altered metabolism, covalent modification, altered organelle function and reactive oxygen species formation, altered intracellular and inflammatory signaling, slowed axonal transport, and altered ion channel dynamics and expression. All of these pathways converge to lead to axon dysfunction and symptoms of neuropathy. The detailed mechanisms of axon degeneration itself have begun to be elucidated with studies of animal models with altered degeneration kinetics, including the slowed Wallerian degeneration (Wld(S)) and Sarm knockout animal models. These studies have shown axonal degeneration to occur through a programmed pathway of injury signaling and cytoskeletal degradation. Insights into the common disease insults that converge on the axonal degeneration pathway promise to facilitate the development of therapeutics that may be effective against other mechanisms of neurodegeneration.