Lepra neural pura. Aspectos diagnósticos en un caso clínico / Pure neural leprosy. Diagnostic aspects of a clinical case

Introducción. 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 exposición con enfermos y en ocasiones por reactivación. Una posibilidad inhabitual es la lepra neural pura, caracterizada por neuropatía, pero sin lesiones en la piel. Se describe un paciente con lepra neural pura y se revisan los aspectos diagnósticos. Caso clínico. Varón de 40 años, inmigrante, diagnosticado y tratado de lepra 20 años antes. Acudió por parestesias y disestesias dolorosas en las manos y las piernas sin lesiones en la piel. Se demostró mononeuritis múltiple aguda con principal afectación de cubitales. La enfermedad, tipificada como tuberculoide paucibacilar, se trató y en pocas semanas la mejoría fue evidente. Conclusiones. En este caso de lepra neural pura por reactivación, el diagnóstico temprano permitió un rápido tratamiento. Es recomendable la evaluación de la neuropatía integrada con criterios clínicos, electrofisiológicos y ecográficos. De este modo se consigue una alta sensibilidad y especialmente una precocidad en el diagnóstico y la instauración del tratamiento, y por consecuencia una mejor recuperación funcional (AU)

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 (AU)

Ulnar nerve sonography in leprosy neuropathy.

A 23-year-old woman presented with a half-year history of right forearm sensory and motor dysfunction. Ultrasound imaging revealed definite thickening of the right ulnar nerve trunk and inner epineurium, along with heterogeneous hypoechogenicity and unclear nerve fiber bundle. Color Doppler exhibited a rich blood supply, which was clearly different from the normal ulnar nerve presentation with a scarce blood supply. The patient subsequently underwent needle aspiration of the right ulnar nerve, and histopathological examination confirmed that granulomatous nodules had formed with a large number of infiltrating lymphocytes and a plurality of epithelioid cells in the fibrous connective tissues, with visible atypical foam cells and proliferous vascularization, consistent with leprosy. Our report will familiarize readers with the characteristic sonographic features of the ulnar nerve in leprosy, particularly because of the decreasing incidence of leprosy in recent years.

Incomplete peripheral facial nerve palsy and ulnar neuropathy due to leprosy mistaken as faciobrachial stroke.

A middle-aged woman was referred to our hospital emergency ward in view of acute onset left faciobrachial weakness. An urgent MRI of the brain was performed, which did not reveal any abnormality and hence a neurology consultation was arranged in order to rule out acute stroke. However, examination and retrospective history taking proved to be a valuable aid in this patient's diagnosis. The incomplete lower motor neuron facial nerve palsy and hand weakness due to leprosy in reaction was confused by the general practitioner as a faciobrachial stroke.

Zancolli lasso procedure for correction of paralytic claw hands.

PURPOSE: To evaluate the outcome of 20 claw hands corrected with the Zancolli lasso procedure. METHODS: 20 patients aged 7 to 48 (mean, 28) years with claw hand deformity for a mean of 33.3 months secondary to leprosy (n=17), traumatic ulnar nerve injury (n=2), or tardy ulnar nerve palsy (n=1) underwent the Zancolli lasso procedure, in which the flexor digitorum superficialis (FDS) of the middle finger was divided in to 4 slips (one for each finger) and reattached to itself after passing through the proximal pulley. Deformity, power, movement, and function of the hands were evaluated before and after surgery. RESULTS: The mean duration of surgery was 58.4 (range, 50-107) minutes. The mean duration of follow-up was 18 (range, 12-24) months. Deformity correction was good in 15 patients, fair in 3, and poor in 2. The mean angle improvement was maximum in the little finger, followed by the ring, index, and middle fingers. The transverse metacarpal arch (compared with the normal hand) was increased/reversed in 7 patients and decreased in 13 patients. The grip and pinch strength increased in 13 patients, remained unchanged in 6, and decreased in one. Motion and function of the hand was good in 12 patients, fair in 6, and poor in 2. One patient developed swan neck deformity in the little finger owing to suture tension of the transferred slip, and another had paraesthesia over the index finger. They underwent reoperation using the FDS of the ring finger; no sensory or motor recovery was achieved. CONCLUSION: The Zancolli lasso procedure is easy to perform and does not require extensive postoperative training. It restores synchronous finger flexion and spares other superficialis tendons, thus avoiding swan neck deformity of the fingers.

Extensive ulnar nerve necrosis: a complication of tuberculoid leprosy.

Pure neural leprosy without cutaneous manifestations is a relatively rare manifestation of leprosy. It can present as a mono- or poly-neuritis with sensory and/or motor impairment. Neural leprosy may or may not be associated with thickening of the involved nerve. We report the case of a 14 year old boy with extensive ulnar nerve necrosis who was diagnosed to have tuberculoid leprosy. What makes this case unique is that we have here a case of pure neural leprosy with a single nerve turned 'necrotic'.

Factors affecting functional outcome of surgical correction of claw hand in leprosy.

The objective of this paper is to study the results and the factors that impact the results of claw hand surgery in leprosy. 110 patients who underwent claw hand correction between 2002 and 2006 were followed up and studied. Brand's criteria for objective assessment and a visual analog scale for subjective assessment were used. The factors studied were age, sex, clinical, duration of paralysis, long flexor tightness, degree of contracture and type of surgery. Objective assessment showed Excellent or Good results in 76.6%, Fair in 20% and Poor in 3.4%. Subjective assessment showed that 80.8% were fully satisfied or satisfied. Regression analysis showed that age, degree of contracture,duration of paralysis and long flexor contracture were seen as significant factors impacting results of claw hand correction.

Neurophysiological patterns of ulnar nerve neuropathy in leprosy reactions.

BACKGROUND: Leprosy neuropathy, despite being primarily demyelinating, frequently leads to axonal loss. Neurophysiological examination of the nerves during Type 1 (T1R) and Type 2 reactions (T2R) may give some insight into the pathophysiological mechanisms. METHODS: Neurophysiological examinations were performed in 28 ulnar nerves during a clinical trial of steroid treatment effectiveness, 19 patients with T1R and nine with T2R. The nerves were monitored during a period of 6 months; there were eight assessments per nerve, for a total of 224 assessments. Nine neurophysiological parameters were assessed at three sites of the ulnar nerve. The compound motor action potential amplitudes elicited at wrist, elbow and above, as well as the conduction velocity and temporal dispersion across the elbow, were chosen to focus on the changes occurring in the parameters at the elbow tunnel. RESULTS AND CONCLUSION: Neurophysiological changes indicating axonal and demyelinating processes during both T1R and T2R were detected across the elbow. Changes in demyelination, i.e. a Conduction Block, as a primary event present during T2R, occurring as an acute phenomenon, were observed regularly; in T1R Temporal Dispersion, a subacute phenomenon, was seen. During treatment remyelination occurred after both types of reactions.

Digital neuropathy of the median and ulnar nerves caused by Dupuytren's contracture: Case report.

INTRODUCTION: Digital neuropathy is a pure sensory neuropathy of a digital nerve. It may be caused by acute or chronic local trauma or pressure, or accompany systemic illnesses such as rheumatoid disease, leprosy, Raynaud disease, dysproteinemia, or diabetes mellitus. We describe an extraordinary case of digital neuropathy of the median and ulnar nerves caused by Dupuytren contracture. CASE REPORT: A 56-year-old right-handed man was presented with numbness and tingling of the little finger of the right and ring finger of the left hand. The clinical and EMG findings in this patient were consistent with a lesion of the median and ulnar palmar digital nerves of the right and left ring and little fingers. CONCLUSION: Dupuytren tissue usually affects the palmar fascia, superficial to the digital nerves, and it may rarely affect the spiral cord in the digits. A spiral cord may cause sensory loss due to impingement of digital nerves or Dupuytren tissue may have been compressing the palmar digital nerves against the relatively inelastic deep transverse metacarpal ligament. As a result, digital neuropathy can develop in those with Dupuytren's contracture, and nerve conduction studies should also be performed to determine the condition. New studies are needed to provide better diagnostic criteria for the condition.

A randomized clinical trial of oral steroids for ulnar neuropathy in type 1 and type 2 leprosy reactions.

BACKGROUND: Steroids regimens in leprosy neuropathies are still controversial in botth types of reactions. METHOD: For this trial, 21 patients with ulnar neuropathy were selected from 163 leprosy patients, 12 with type 1 reaction (T1R) and nine with type 2 (T2R). One experimental group started with prednisone 2 mg/kg/day and the control group with 1 mg/kg/day. A clinical score based on tests for spontaneous pain, nerve palpation, sensory and muscle function was used. Neurophysiological evaluation consisted on the motor nerve conduction of the ulnar nerve in three segments. Student "t" test for statistical analysis was applied on the results: before treatment, first week, first month and sixth month, between each regimen and types of reaction. CONCLUSION: In both reactions during the first month higher doses of steroids produced better results but, earlier treatment with lower dose was as effective. Short periods of steroid, 1 mg/Kg/day at the beginning and,tapering to 0,5 mg/Kg/day or less in one month turned out to be efficient in T2R.

A randomized clinical trial of oral steroids for ulnar neuropathy in type 1 and type 2 leprosy reactions / Ensaio clínico sobre o tratamento com esteróides via oral da neuropatia ulnar em reação tipo 1 e tipo 2 da hanseníase

BACKGROUND: Steroids regimens in leprosy neuropathies are still controversial in botth types of reactions. METHOD: For this trial, 21 patients with ulnar neuropathy were selected from 163 leprosy patients, 12 with type 1 reaction (T1R) and nine with type 2 (T2R). One experimental group started with prednisone 2 mg/kg/day and the control group with 1 mg/kg/day. A clinical score based on tests for spontaneous pain, nerve palpation, sensory and muscle function was used. Neurophysiological evaluation consisted on the motor nerve conduction of the ulnar nerve in three segments. Student "t" test for statistical analysis was applied on the results: before treatment, first week, first month and sixth month, between each regimen and types of reaction. CONCLUSION: In both reactions during the first month higher doses of steroids produced better results but, earlier treatment with lower dose was as effective. Short periods of steroid, 1 mg/Kg/day at the beginning and,tapering to 0,5 mg/Kg/day or less in one month turned out to be efficient in T2R.

INTRODUÇÃO: O tratamento da neuropatia da hanseníase com esteróides é ainda controverso nos dois tipos de reações. MÉTODO: Neste ensaio, de 163 pacientes foram selecionados 21 com neuropatia ulnar, 12 com reação tipo 1 e 9 com tipo 2. Um grupo experimental iniciou com 2 mg/kg/dia e o grupo controle com 1 mg/kg/dia. Foi composto um escore clínico pela avaliação da sensação dolorosa espontânea, palpação de nervos e funções sensitiva e motora. Realizou-se a condução nervosa motora do nervo ulnar em três segmentos. Aplicaram-se os estudos estatísticos com o teste t de Student nos resultados: antes do tratamento, primeira semana, primeiro mês e sexto mês. CONCLUSÃO: Em ambas as reações dosagens mais elevadas iniciais produziram melhores resultados, mas a dose menor quando administrada precocemente foi igualmente efetiva. Períodos curtos com doses efetivas, 1 mg/Kg/dia no início e reduzindo-se para 0,5 mg/Kg/dia ou menos em um mês foram eficientes na reação tipo 2.

Voluntary muscle testing and dynamometry in diagnosis of motor impairment in leprosy: a comparative study within the INFIR Cohort Study.

AIM: To evaluate hand muscle weakness detected through dynamometry as an indicator for change in motor nerve function detected by Voluntary Muscle Testing (VMT) of ulnar and median nerves. DESIGN: The research was carried out as part of the INFIR Cohort Study among 303 subjects newly diagnosed with MB leprosy in two centres in UP state, northern India. METHODS: To assess grip strength, key pinch and pulp-to-pulp pinch we adapted the cuffs of adult and neonatal sphygmomanometers. The testing was carried out at diagnosis and at each visit during a 2-year follow-up. RESULTS: 303 subjects with newly diagnosed MB leprosy were included in the study. We found statistically significant differences in grip strength, key pinch and pulp-to-pulp pinch between groups defined by ulnar VMT grades at time of diagnosis. There was also a statistically significant difference in hand grip between groups defined by median VMT at diagnosis. In each case, strength tended to reduce with increasing motor involvement. We explored reduction in grip strength, key pinch or pulp-to-pulp pinch as indicators of change in ulnar VMT during follow-up. A 25% reduction over two visits was the most effective indicator. Changes were also associated with marginal changes in motor and sensory nerve function, most commonly associated with Type I reactions. CONCLUSION: Dynamometry is recommended as an additional method that may be used to monitor changes in nerve function in leprosy, particularly in subjects with early motor impairment of the ulnar nerve.

Reconstructive surgery in upper limbs in leprosy.

Of all the deformities in leprosy, 80% require minor surgery and the rest major surgery. Upper and lower limbs and face are mainly affected by deformities. Either the median or ulnar nerve and rarely the radial nerve are involved in isolation or in combination. Tendon transfer is the only option available in cases of deformities like simian hand, ulnar claw hand, wrist drop and failure to the metacarpals. The only motor function to be restored in median nerve palsy is opponensplasty. For radial nerve palsy standard FCU transfer, FDS transfer and FCR transfer are the reconstructive methods. For ulnar nerve palsy, an intrinsic minus hand function is restored by the motors PL, ECRL, FDS, EIP or EDM extended by four tail fascia lata graft onto lateral or ulnar bands of fingers. When ulnar nerve is part of much more extensive paralysis, reducing the availability of suitable motors, static mathods of conection of claw hand are done.