F-waves persistence in peripheral sensory syndromes.

BACKGROUND: The distinction between sensory neuronopathies (SN), which is by definition purely sensory, and sensory polyneuropathies (SP) and sensory multineuropathies (SM) is important for etiologic investigation and prognosis estimation. However, this task is often challenging in clinical practice. We hypothesize that F-wave assessment might be helpful, since it is able to detect subtle signs of motor involvement, which are found in SP and SM, but not in SN. OBJECTIVE: The aim of the present study was to determine whether F-waves are useful to distinguish SN from SP and SM. METHODS: We selected 21 patients with SP (12 diabetes mellitus, 4 transthyretin familial amyloid polyneuropathy, 4 others), 22 with SM (22 leprosy), and 26 with SN (13 immune-mediated, 10 idiopathic, 3 others) according to clinical-electrophysiological-etiological criteria. For every subject, we collected data on height and performed 20 supramaximal distal stimuli in median, ulnar, peroneal, and tibial nerves, bilaterally, to record F-waves. Latencies (minimum and mean) and persistences were compared across groups using the Kruskal-Wallis and Bonferroni tests. P-values < 0.05 were considered significant. RESULTS: All groups were age, gender, and height-matched. Overall, there were no significant between-group differences regarding F-wave latencies. In contrast, F-wave persistence was able to stratify the groups. Peroneal F-wave persistence was higher, bilaterally, in the SN group compared to SM and SP (p < 0.05). In addition, F-waves persistence of the ulnar and tibial nerves was also helpful to separate SN from SP (p < 0.05). CONCLUSION: F-wave persistence of the peroneal nerves might be an additional and useful diagnostic tool to differentiate peripheral sensory syndromes.

ANTECEDENTES: A distinção entre neuronopatias sensitivas (SN) e polineuropatias sensitivas (SP) e multineuropatias sensitivas (SM) é importante para a investigação etiológica e para o prognóstico. Contudo, esta tarefa é desafiadora na prática clínica. Hipotetizou-se que a avaliação das ondas-F pode ser útil, por ser capaz de detectar envolvimento motor nas SP e SM, mas não nas SN. OBJETIVO: Determinar se as ondas-F podem ajudar a distinguir entre SN, SP e SM. MéTODOS: Selecionou-se 21 pacientes com SP (12 diabetes mellitus, 4 ATTR-FAP e 4 com outras neuropatias), 22 com SM (22 hanseníases) e 26 com SN (13 imunomediadas, 10 idiopáticas e 3 com outras neuronopatias), de acordo com critérios clínicos, etiológicos e eletrofisiológicos. Para cada indivíduo, foi aferida a altura e foram aplicados 20 estímulos distais supramáximos nos nervos mediano, ulnar, fibular e tibial, bilateralmente, para registrar as ondas-F. Uma comparação foi feita, por grupo, das latências (mínimas e médias) e persistências pelos testes Kruskal-Wallis e Bonferroni. Valores de p < 0.05 foram considerados estatisticamente significativos. RESULTADOS: Todos os grupos foram pareados por idade, sexo e altura. Não houve diferença estatística significativa entre os grupos quanto às latências das ondas-F. A persistência da onda-F foi capaz de estratificar os grupos, sendo as dos nervos fibulares bilateralmente maiores no grupo SN que nos grupos SM e SP (p < 0.05). Adicionalmente, a persistência das ondas-F dos nervos ulnares e tibiais também foi útil para distinguir SN de SP (p < 0.05). CONCLUSãO: A persistência das ondas-F dos nervos fibulares pode ser uma ferramenta adicional e útil para diferenciar síndromes sensitivas periféricas.

High-resolution ultrasonography for early diagnosis of neural impairment in seropositive leprosy household contacts.

Leprosy household contacts (HC) represent a high-risk group for the development of the disease. Anti-PGL-I IgM seropositivity also increases the risk of illness. Despite significant advances in leprosy control, it remains a public health problem; and early diagnosis of this peripheral neuropathy represents one of the main goals of leprosy programs. The present study was performed to identify neural impairment in leprosy HC by analyzing differences in high-resolution ultrasonographic (US) measurements of peripheral nerves between leprosy HC and healthy volunteers (HV). Seventy-nine seropositive household contacts (SPHC) and 30 seronegative household contacts (SNHC) underwent dermato-neurological examination and molecular analysis, followed by high-resolution US evaluation of cross-sectional areas (CSAs) of the median, ulnar, common fibular and tibial nerves. In addition, 53 HV underwent similar US measurements. The US evaluation detected neural thickening in 26.5% (13/49) of the SPHC and only in 3.3% (1/30) among the SNHC (p = 0.0038). The CSA values of the common fibular and tibial nerves were significantly higher in SPHC. This group also had significantly greater asymmetry in the common fibular and tibial nerves (proximal to the tunnel). SPHC presented a 10.5-fold higher chance of neural impairment (p = 0.0311). On the contrary, the presence of at least one scar from the BCG vaccine conferred 5.2-fold greater protection against neural involvement detected by US (p = 0.0184). Our findings demonstrated a higher prevalence of neural thickening in SPHC and support the role of high-resolution US in the early diagnosis of leprosy neuropathy. The combination of positive anti-PGL-I serology and absence of a BCG scar can identify individuals with greater chances of developing leprosy neuropathy, who should be referred for US examination, reinforcing the importance of including serological and imaging methods in the epidemiological surveillance of leprosy HC.

Saphenous nerve transfer: A new approach to restore sensation of the sole.

BACKGROUND: Loss of protective sensation of the sole may lead to repeated trauma, chronic nonhealing ulcers, and even amputation. Saphenous nerve (SN) to posterior tibial nerve (PTN) transfer can restore sensation of the sole. METHOD: This study was conducted in a tertiary referral center in Central India. Twenty-one patients (32 feet) diagnosed with loss of sensation of the sole were included in this study. Causes of loss of sensation were Hansen's disease (n = 18), complex sciatic nerve injury (n = 1), lumbosacral spinal tumor (n = 1), and lumbosacral meningomyelocele (n = 1). Seventeen feet (14 patients) had ulcers on the sole. Preoperative and postoperative sensory tests performed on the sole included tests for touch, pain, temperature, pressure, vibration, and two-point discrimination. Results were classified as per the British Medical Research Council (MRC) scoring system. RESULTS: Seventeen patients (26 feet) were available for follow-up at 6 months after surgery. All patients had improvement in sensory parameters. Ulcers completely healed in 13 feet and reduced in size in four feet. MRC score improved from S0 in 22 feet and S1 in 10 feet to S3 + in 20 feet, S3 in four feet, and S2 in two feet. CONCLUSIONS: Sensory neurotization with SN transfer to PTN can restore protective sensation to the sole and help in the healing of ulcers.

Comprehensive electrophysiology in leprous neuropathy - Is there a clinico-electrophysiological dissociation?

OBJECTIVE: The diagnosis of leprous neuropathy is mostly empirical and electrophysiological studies may not truly represent the clinical findings. This study comprehensively evaluates the neuroelectrophysiology and looks at clinico-electrophysiological dissociation. METHODS: Conventional electrophysiological recording included evaluation of median, ulnar, radial, tibial, and common peroneal nerve; an extended protocol included great auricular, phrenic, and facial nerves, along with sympathetic skin response and blink reflex. Nerve biopsy and slit skin smear were done to aid categorization. RESULTS: Forty-six patients of leprosy were enrolled. Mononeuritis multiplex was the commonest presentation. Sensory loss was commoner than motor deficits. Approximately 60% of all cases were nerve-biopsy proven. Nerve thickening was present in 38.7% (214/552) of nerves examined. Clinico-electrophysiological dissociation between nerve thickening and nerve conduction findings was present in median, ulnar, great auricular, and common peroneal nerves. CONCLUSION: Electrophysiological findings outnumber occurrence of nerve thickening and clinical deficits in leprous neuropathy. From a clinical perspective, enlargement of great auricular, ulnar, and common peroneal nerves may be more sensitive in predicting electrophysiological abnormalities. SIGNIFICANCE: A comprehensive nerve conduction study including great auricular and phrenic nerves, coupled with a sympathetic skin response, may aid in detecting cases with paucity of findings since such a combination is seldom seen in other disorders.

Decompression of peripheral nerve trunks in leprosy prevents the development and progression of deformities?

Peripheral nerve involvement results in deformities formation in leprosy. High doses of (40-60 mg) steroids along with the anti-leprosy drugs is preferred even though the 70-75% cases develop deformity with the above treatment. 772 ulnar nerves, 120 median nerves and 108 posterior tibial nerves not responding to above medical treatment in 12 weeks, were undertaken for external and internal nerve trunk decompression. These cases were followed-up for 5-20 years at various intervals. The pain in nerves (neuritis) recovered in all cases of ulnar, median and posterior tibial nerves. Full sensory recovery with pin prick/feather or cotton wool touch was seen in 50% cases of all the three nerves. 20% cases maintain the pre-operative levels of sensory status. Plantar ulcers healed within 6 months after decompression of posterior tibial nerve. Only 6 cases showed reoccurrence. Overall motor recovery in ulnar nerve was seen 89% and 70% in median nerve. The sensory recovery restores protective sensation which prevents secondary injuries. The improvement of motor power gave better functional hands and improved the appearance which in absence of surgical intervention was not possible.

A comparison of the change in clinical severity scale score and a retrospective physician assessment of neurological outcome in individuals with leprosy associated nerve function impairment after treatment with corticosteroids.

OBJECTIVES: To determine whether the measured change in score of a validated clinical severity scale reflected physician assessed improvement in individuals who had received corticosteroid therapy for leprosy associated nerve damage. DESIGN: Patients with nerve function impairment who participated in a randomised controlled trial of corticosteroids were classified into two groups using a retrospectively determined physician assessment of improvement. One group consisted of patients who had recovered or improved the other of patients who were unchanged or had deteriorated. The change in the clinical severity scale scores of these two groups was compared. RESULTS: The change in the clinical severity scale scores of the 34 eligible individuals in the two groups were significantly different (P = 0.003). Individuals in the group who recovered or improved had a greater change in severity score than those whose nerve function was unchanged or deteriorated. CONCLUSION: The scale for measuring the severity of leprosy Type 1 reactions (T1Rs) and/or nerve function impairment reflects the clinical improvement of individuals with leprosy associated nerve damage.

Avaliação anatômica do túnel do tarso - estudo das estruturas nervosas e suas correlações em dissecção de cadaveres / Anatomical evaluation of the tarsal tunnel - study of nerve structures and their correlations in cadaver dissection

Apresenta-se estudo anatômico do túnel do tarso em 38 pés de cadáveres com avaliação do retináculo dos flexores, das estruturas do túnel do tarso e das relações entre suas estruturas e o nervo tibial. Estabeleceu-se a linha “AB”, definida do centro do maléolo medial a um ponto localizado a 1cm distalmente à tuberosidade póstero-superior do calcâneo, utilizada como ponto de referência para mensurações do nervo tibial e seus ramos. Encontrou-se o retináculo com aspecto delgado em 73,68% e adiposo em 26,31%. A divisão do nervo tibial em nervo plantar medial e lateral ocorreu em 31,57% dos pés proximalmente ao túnel do tarso, em 2,63% à entrada desse e 65,78% no interior do túnel. O nervo calcâneo medial apresentou origem em 50% dos pés proximalmente à linha “AB”, em 36,84% distalmente a ela e, em 13,15%, tanto proximal como distalmente..

An attempt towards prevention and management of disabilities and deformities in leprosy.

History of prevention of deformities is practically as old as the appearance of the deformities themselves, unfortunately without much understanding to start with. In medieval era and even earlier, leprosy and deformities were treated synonymously and the disease's infectivity too was closely associated with appearance of deformities. Hence, to reduce chances of deformities caused by leprosy in healthy population, the patients having deformities were driven away from the society presuming that only deformed patients spread the disease. Unfortunately, it never worked. However, in later period, factors behind the deformities and disabilities were recognized and understood. These are basically limited to involvement of peripheral nerves and their proper management (medical treatment, surgical interventions, physiotherapy, ergonomics and counseling) by one rule of thumb i.e. early, timely and adequately.

Avaliação anatômica do túnel do tarso – estudo das estruturas nervosas e suas correlações em dissecção de cadáveres / Anatomical assessment of the tarsal tunnel - study of the nervous structures and their correlation in dissecting corpses

Apresenta-se estudo anatômico do túnel do tarso em 38 pés de cadáveres com avaliação do retináculo dos flexores, das estruturas do túnel do tarso e das relações entre suas estruturas e o nervo tibial. Estabeleceu-se a linha “AB”, definida do centro do maléolo medial a um ponto localizado a 1cm distalmente à tuberosidade póstero-superior do calcâneo, utilizada como ponto de referência para mensurações do nervo tibial e seus ramos. Encontrou-se o retináculo com aspecto delga-do em 73,68% e adiposo em 26,31%. A divisão do nervo tibial em nervo plantar medial e lateral ocorreu em 31,57% dos pés proximalmente ao túnel do tarso, em 2,63% à entrada desse e 65,78% no interior do túnel. O nervo calcâneo medial apresentou origem em 50% dos pés proximalmente à linha “AB”, em 36,84% distalmente a ela e, em 13,15%, tanto proximal como distalmente.

Avaliação sensitiva de hansenianos pelos monofilamentos Semmes-Weinstein em serviço terciário de fisioterapia / Effectiveness of Semmes-Weinstein monofilaments in leprosy neuropathy in physiotherapy tertiary sevice

OBJETIVOS: Identificar a frequência das alterações da sensibilidade de mãos e pés de hansenianos através dos monofilamentos Semmes-Weinstein no hospital terciário.MÉTODO: Trinta pacientes do setor de fisioterapia do Hospital da Clínicas da FMRP-USP foram avaliados clinico-epidemiologicamente e submetidos ao teste por monofilamentos de agosto a dezembro de 2004.RESULTADOS: A média de idade dos pacientes foi de 48,4 anos, sendo 80% do sexo masculino e 70% oriundos da região de Ribeirão Preto. Classificavam-se como multibacilares 70% dos pacientes e 80% apresentavam-se com Grau I de incapacidade. Quanto ao teste nos membros superiores, o nervo ulnar foi o mais acometido nos paucibacilares (78%) e nos multibacilares (83%). Nos membros inferiores, o ramo plantar medial do nervo tibial posterior encontrou-se acometido em todos os pacientes do grupo multibacilar. A sensação protetora estava ausente nas mãos em 26% no grupo pauci e 46% no multibacilar, e nos pés 44% no paucibacilar e 56% no multibacilar. O diagnóstico foi tardio em 37% dos pacientes, apresentando no mínimo dois nervos com perda da sensação protetora.CONCLUSÃO: Os resultados evidenciaram que o acompanhamento da neuropatia da hanseníase pelos monofilamentos S-W, mostrou-se capaz de identificar alterações da sensibilidade em múltiplos nervos das extremidades, tanto nos pacientes paucibacilares quanto multibacilares, tornando-se evidente a gravidade dos pacientes atendidos nos serviço de atenção terciária à saúde.

OBJECTIVES: To identify the frequency of changes in the sensibility of hands and feet from leprosy patients through the Semmes-Weinstein monofilament in tertiary hospital.METHOD: Thirty patients of the Physiotherapy Service of Hospital das Clínicas FMRP-USP were evaluated from August to December 2004. The patients were clinically and epidemiologically evaluated. Afterwards, monofilaments were tested.RESULTS: The mean age was 48.4 years, 80% male and 70% came from the Ribeirão Preto region. Seventy percent of the patients were classified as multibacilary and 80% presented grade 1 of incapacity. Concerning to Semmes-Weinstein test on the upper limbs, the ulnar was the most impaired nerve on the paucibacillary patients (78%) and on the multibacillary (83%). On the lower limbs, the plantar medial branch from the tibial posterior nerve had been impaired in all patients. The protective sensation was absent on the hands in 26% on the paucibacillary group and 46% on the multibacillary, and on the feet in 44% on the paucibacillary and 56% on the multibacillary. Thirty seven percent of the patients have received late diagnosis, showing at least two nerves without protective sensation.CONCLUSIONS: The results showed the reliable use of Semmes-Weinstein monofilaments during the follow up of leprosy patients. This tool was able to identify sensitive changes in multiple nerves of the extremities, for paucibacilary and multibacilary patients, what clearly shows the severity of incoming patients in health tertiary care service.

[Leprosy: a rare imported disease]. / Une maladie d'importation rare: la lèpre.

Although rare in occidental countries, leprosy is an endemic disease throughout the world. Physicians may encounter imported cases and thus need to be aware of this diagnosis. We here report a 41-year-old male patient from French West Indies who presented with nonspecific extensive skin lesions and a peripheral neuropathy. Skin biopsy examination led to the diagnosis of borderline lepromatous leprosy.

Blink reflex, H-reflex and nerve-conduction alterations in leprosy patients.

Peripheral nerve lesions are the most important cause of disability in leprosy patients. Electrophysiological studies are used in the diagnosis and prognosis of neuropathy. Nerve conduction is the most frequently used electrophysiological test method to detect neuropathy, although it evaluates only a part of the peripheral nervous system. Blink reflex and H-reflex are electrophysiological tests which evaluate facial and trigeminal nerve function. This study determined the frequencies of blink reflex, H-reflex and motor and sensory nerve conduction alterations in twenty five heterogeneous, clinic patients with lepromatous leprosy and a control group of 20 healthy subjects. Study results showed a decrease in motor and sensory nerve conduction in 40% and 30%, respectively. In blink reflex (BR), right R1 was altered in latency in 20% of patients, left R1 in 20%, right ipsilateral R2 in 16%, left ipsilateral R2 in 20%, and right and left contralateral R2 were altered in 32% of patients. There was an absence of H-reflex in 16% (n = 4) and prolonged latency in 4% (n = 1).

Avaliação neurologica completa na neuropatia hanseniana / Complete neurologica assessment in Hansen´s disease

Dada a importância da afectação neurológica da Doença de Hansen, os autores tentam descrever neste artigo a forma de realizar uma exploração neurológica completa. Para efeito, são focados os pontos fundamentais desta prática, como a palpação e identificação dos nervos periféricos engrossados, o estudo da sensibilidade superficial e profunda, a exploração da mobilidade voluntária, o exame dos reflexos e a avaliação dos processos neutróficos secundários ao trauma. Devemos destacar que, na maioria das situações, para o diagnóstico da doença e fundamentalmente no trabalho de campo, realizaremos uma exploração baseada em todos estes pontos, embora muito mais simplificada. Como anexo do artigo serão apresentados os resultados obtidos com a exploração neurológica de 71 doentes controlados no Sanatório Fontilles.

Leprosy affects the tibial nerves diffusely from the middle of the thigh to the sole of the foot, including skip lesions.

This study investigated where leprosy affects the posterior tibial nerve and whether neurolysis is beneficial. Nine patients with bilateral posterior tibial leprous neuropathy with no sensorimotor recovery were studied. Preoperative sensory-muscle and nerve conduction velocity testing revealed the tarsal tunnel to be the site of a severe lesion in all cases. During surgery, the most proximal site of the nerve lesion was detected by electrically stimulating the spinal roots from the second lumbar nerve to the fourth sacral nerve, evoking efferent mixed nerve compound action potentials that were recorded from the exposed tibial nerve. In all patients, the nerve compound action potentials became normal only proximal to the sciatic nerve bifurcation. Epineuriotomy within these seemingly unaffected segments revealed fibrosis of the interfascicular epineurium. Interfascicular neurolysis was performed on all affected segments. A 2-year follow-up showed an increase in girth of the proximal calf musculature in six of eight patients (the ninth patient had no recordable nerve conduction velocity). It was concluded that (1) leprosy affects the tibial nerves in a scattered way from the sciatic nerve main trunk distally to the exit of the tarsal tunnel; and (2) interfascicular, microsurgical neurolysis is beneficial provided that it is performed on all affected nerve segments.

Mycobacterium leprae binds to a 25-kDa phosphorylated glycoprotein of human peripheral nerve.

Mycobacterium leprae, the causative agent of leprosy, specifically invades and destroys the peripheral nerve, which results in the main clinical manifestation of the disease. Little is known about the bacteria-nerve protein interaction. We show in the present work that M leprae binds to a 25 kDa glycoprotein from human peripheral nerve. This protein is phosphorylatable and it binds to lectins which have alpha-mannose specificity. This M leprae-protein interaction could be of importance in the pathogenesis of leprosy.

Pathologic changes in a tibial nerve with surviving M. leprae in a healed tuberculoid leprosy patient.

A tibial nerve from a disease-arrested borderline tuberculoid (BT) leprosy patient was dissected out and examined almost in its entirety using hematoxylin and eosin staining, a modified Fite's stain for acid-fast bacilli (AFB), solochrome cyanin stain for myelin, and van Gieson's stain for fibrous tissue. Fibrosis of the perineurium and epineurium and fibrous replacement of the nerve parenchyma, which was maximum at the ankle joint area, were seen. In focal areas inflammation was present, especially in the epineurium around blood and lymph vessels. Even 21 years after adequate antileprosy therapy, AFB were present in the endoneurium in all except 2 of the 10 segments of the nerve, evoking hardly an inflammatory reaction or other ill effects. It is pointed out that BT leprosy should also be considered a generalized disease, especially when there is peripheral nerve trunk involvement and, in such cases, a longer duration of currently available antileprosy therapy is advisable. Trauma to nerve trunk plays a major role in producing nerve destruction and paralysis.