Pathological, ultrasonographic, and electrophysiological characterization of clinically diagnosed cases of pure neuritic leprosy.

A subset of neuritic form of leprosy, called pure neuritic leprosy (PNL), seen in a minority of leprosy patients, is characterized by peripheral neuropathy without skin lesions and an absence of acid-fast bacilli on skin smears. Patients with PNL are often started on drug therapy without confirmation of diagnosis. We, therefore, did a prospective study of clinically diagnosed PNL patients with correlation of ultrasonographic and biopsy findings. A total of 100 consecutive patients with PNL, diagnosed according to the consensus case definition, were included in the study. All patients underwent nerve conduction study, peripheral nerve ultrasonography, and sural nerve biopsy. Multiple mononeuropathies were present in 75% of cases, mononeuropathy in 18%, and polyneuropathy in the remaining 7%. Compared to clinical examination, ultrasonographic assessment of the peripheral nerves was not only better at the detection of thickening but also helped in characterization of their fascicular architecture, echogenicity, and vascularity. A total of 32 cases were confirmed on nerve biopsy, out of which 75% had demonstrable lepra bacilli. Cranial nerve involvement, presence of trophic ulcers, and bilateral thickening of the great auricular nerve were significantly associated with the positivity of lepra bacilli. A significant improvement in the disability score happened after multidrug therapy. A comprehensive electrophysiologic, ultrasonographic, and histological evaluation may be helpful in establishing a diagnosis of PNL with greater confidence, while ruling out other non-leprosy diagnoses.

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)

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.

Pure neuritic leprosy presenting as ulnar nerve neuropathy: a case report of electrodiagnostic, radiographic, and histopathological findings.

Hansen's disease, or leprosy, is a chronic infectious disease with many manifestations. Though still a major health concern and leading cause of peripheral neuropathy in the developing world, it is rare in the United States, with only about 150 cases reported each year. Nevertheless, it is imperative that neurosurgeons consider it in the differential diagnosis of neuropathy. The causative organism is Mycobacterium leprae, which infects and damages Schwann cells in the peripheral nervous system, leading first to sensory and then to motor deficits. A rare presentation of Hansen's disease is pure neuritic leprosy. It is characterized by nerve involvement without the characteristic cutaneous stigmata. The authors of this report describe a case of pure neuritic leprosy presenting as ulnar nerve neuropathy with corresponding radiographic, electrodiagnostic, and histopathological data. This 11-year-old, otherwise healthy male presented with progressive right-hand weakness and numbness with no cutaneous abnormalities. Physical examination and electrodiagnostic testing revealed findings consistent with a severe ulnar neuropathy at the elbow. Magnetic resonance imaging revealed diffuse thickening and enhancement of the ulnar nerve and narrowing at the cubital tunnel. The patient underwent ulnar nerve decompression with biopsy. Pathology revealed acid-fast organisms within the nerve, which was pathognomonic for Hansen's disease. He was started on antibiotic therapy, and on follow-up he had improved strength and sensation in the ulnar nerve distribution. Pure neuritic leprosy, though rare in the United States, should be considered in the differential diagnosis of those presenting with peripheral neuropathy and a history of travel to leprosy-endemic areas. The long incubation period of M. leprae, the ability of leprosy to mimic other conditions, and the low sensitivity of serological tests make clinical, electrodiagnostic, and radiographic evaluation necessary for diagnosis. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

Clinical, electrodiagnostic and pedobarographic assessments of leprotic patients with trans-tibial amputation.

The aim of present study was to investigate clinical, electrodiagnostic and pedobarographic findings of non-amputee limb in chronic leprotic patients with unilateral trans-tibial amputation to determine neuropathy and plantar foot pressure in non-amputee limb. During the present prospective cross-sectional study, 10 chronic leprotic patients with unilateral trans-tibial amputation were evaluated. The study was conducted in Tabriz Bababaghi and Imam Reza Hospitals at summer of 2008. Sensory nerve conduction (SNAP) and Compound Motor Action Potentials (CMAP) studies were performed in association with pedobarographic assessment. No reliable response was detected from tested sensory and motor nerves, except a very low amplitude finding in deep preoneal nerve of one patient. In comparing with healthy group, static total plantar area, dynamic total plantar area, static rarefoot peak pressure and dynamic rarefoot peak pressure were lower in leprotic patients (p = 0.047, p = 0.004, p = 0.029 and p < 0.001), while static forefoot peak pressure and dynamic forefoot peak pressure were higher in these patients (p = 0.011 and p = 0.031). All of leprotic patients with unilateral trans-tibial amputation suffered from severe neuropathy. Also, these patients have high plantar pressure under the forefoot. Collectively, severe neuropathy and abnormal plantar foot pressure expose in non-amputee foot expose leprotic patients to the higher risk of secondary amputation.

Role of ulnar nerve sonography in leprosy neuropathy with electrophysiologic correlation.

OBJECTIVE: The purpose of this study was to evaluate the diagnostic usefulness of ulnar nerve sonography in leprosy neuropathy with electrophysiologic correlation. METHODS: Twenty-one consecutive patients with leprosy (12 men and 9 women; mean age +/- SD, 47.7 +/- 17.2 years) and 20 control participants (14 men and 6 women; mean age, 46.5 +/- 16.2 years) were evaluated with sonography. Leprosy diagnosis was established on the basis of clinical, bacteriologic, and histopathologic criteria. The reference standard for ulnar neuropathy in this study was clinical symptoms in patients with proven leprosy. The sonographic cross-sectional areas (CSAs) of the ulnar nerve in 3 different regions were obtained. Statistical analyses included Student t tests and receiver operating characteristic curve analysis. RESULTS: The CSAs of the ulnar nerve were significantly larger in the leprosy group than the control group for all regions (P < .01). Sonographic abnormalities in leprosy nerves included focal thickening (90.5%), hypoechoic areas (81%), loss of the fascicular pattern (33.3%), and focal hyperechoic areas (4.7%). Receiver operating characteristic curve analysis showed that a maximum CSA cutoff value of 9.8 mm(2) was the best discriminator (sensitivity, 0.91; specificity, 0.90). Three patients with normal electrophysiologic findings had abnormal sonographic findings. Two patients had normal sonographic findings, of which 1 had abnormal electrophysiologic findings, and the other refused electrophysiologic testing. CONCLUSIONS: Sonography and electrophysiology were complementary for identifying ulnar nerve neuropathy in patients with leprosy, with clinical symptoms as the reference standard. This reinforces the role of sonography in the investigation of leprosy ulnar neuropathy.

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.

Tactile threshold detection in leprosy patients with an electronic algometer.

OBJECTIVE: To propose an electronic method for sensitivity evaluation in leprosy and to compare it to the Semmes-Weinstein monofilaments. METHODS: Thirty patients attending the Dermatology outpatient clinic of HCFMRP-USP were consecutively evaluated by both the electronic aesthesiometer and Semmes-Weinstein monofilaments on hand and foot test points. The intraclass correlation coefficient (ICC) was calculated to determine the variability of the electronic measures and the Kappa coefficient was calculated to determine the agreement between methods according to their categories (altered and non-altered tactile sensitivity). RESULTS: The ICC was approximately 1, demonstrating repeatability. The Kappa coefficient showed more than 75 and 63% agreement on the hand and foot points, respectively. The mean agreement between the 2 methods for the 7 points of the right and left hand was 77.14 and 75.71%, respectively. The mean agreement for all 10 points was 74.33 and 63.66% on the right and left foot, respectively. In cases of disagreement the detection of altered tactile sensitivity by the electronic esthesiometer on the right and left foot was 90.91 and 84.25%, respectively, with no detection by the monofilaments. CONCLUSION: The results suggest that the electronic esthesiometer is a reliable and easy application, capable of evaluating alterations of tactile sensitivity in leprosy patients.

Leprosy and the peripheral nervous system: basic and clinical aspects.

Leprosy is one of the most common causes of nontraumatic peripheral neuropathy in the developing world. The causative agent, Mycobacterium leprae, has a predilection for Schwann cells, where the organism multiplies unimpeded by organism-specific host immunity, resulting in destruction of myelin, secondary inflammatory changes, and destruction of the nerve architecture. The cardinal diagnostic features of leprosy are anesthetic skin lesions, neuropathy, and positive skin smears for the bacilli. However, patients may rarely present without skin lesions in pure neuritic leprosy. Electrodiagnostic findings early in the disease reveal demyelinating features, such as slowing of conduction velocity and prolongation of latencies, but as the disease progresses secondary axonal damage commonly ensues. Electrodiagnostic studies are also useful to monitor for toxicity secondary to therapy, particularly thalidomide-associated neuropathy. Nerve biopsy of a sensory cutaneous nerve is sometimes essential to confirm a diagnosis of leprosy. Significant advances in understanding of the pathogenesis, mapping of the genome, and other advances in molecular biology may result in better preventive and therapeutic modalities, and the goal of eradicating leprosy as a global problem may yet be realized.

Neurohanseníase: emprego de metilprednisolona em pulsoterapia intravenosa / ?

foram selecionados12 pacientes com diagnóstico de hanseníase, com neuropatia periférica, independentemente do tempo de instalação dos sintomas neurológicos e da forma clínica de hanseníase. Os pacientes estavam ainda em tratamento específico ou já haviam terminado o mesmo e usavam ou não corticóide oral. Todos os pacientes submeteram-se à avaliação clínica, ao estudo eletroneuromiográfico e, em alguns deles, foi realizada a biópsia de nervo. O esquema terapêutico preconizado foi a administração de um grama de metilprednisolona intravenosa durante 90 minutos, em três dias consecutivos, seguidos de um intervalo de quatro dias. Este mesmo procedimento foi repetido nas duas semanas subseqüentes. O corticóide oral foi gradualmente suspenso. Os pacientes foram posteriormente acompanhados ambulatorialmente para avaliação da evolução dos sintomas, utilizando-se a Escala Analógica Visual (VAS). Nova eletroneuromiografia foi realizada três meses após o término da pulsoterapia. Aqueles pacientes que não aprsentaram melhora clínica, com relação as queixas sensitivas, foram submetidos a novo ciclo de pulsoterapia e reavaliados clinicamente. Nos pacientes com maior comprometimento neurológico (sintomas sensitivos mais exuberantes, deficit motor, amiotrofia) foi realizada pulsoterapia mensal (um grama de metilprednisolon em dose única). Observamos, após a primeira pulsoterapia, uma queda de seis a sete pontas na escala analógica visual, em 58 porcento dos pacientes. Não houve,no entanto, melhora tão importante na evolução eletroneuromiográfica. A pulsoterapia com metilprednisolona intravenosa é uma opção de tratamento que deve ser considerada na neuropatia da hanseníase devido à boa resposta terapêutica, à menor incidência dos efeitos colaterais observados com o uso prolongado de corticóide e ao melhor controle sobre a sua administração

Intraoperative spinal root stimulation to detect most proximal site of leprous ulnar neuritis.

In 10 patients with leprous ulnar neuritis, we investigated the most proximal site of lesion in the affected nerves. Spinal roots C8 and T1 were stimulated intraoperatively to evoke efferent mixed compound nerve action potentials which were recorded from the exposed ulnar nerves. The site at which amplitudes reached a maximum was considered the most proximal site of lesion. Nerve damage was found far proximally from the thickened segments in otherwise inconspicuous sections. Epineuriotomy within these apparently unaffected segments revealed fibrosis of the interfascicular epineurium in 9 patients, which is an indication for microsurgical interfascicular neurolysis.

Clinical profile and electrodiagnostic study of peripheral neuropathy.

A clinical analysis and electrophysiological study was performed in 40 cases of peripheral neuropathy. Motor nerve conduction velocity (MNCV) and electromyography (EMG) were also recorded in 20 healthy volunteers. Twelve cases were of diabetic neuropathy (DN), nine of Guillain-Barre syndrome (GBS), eight of idiopathic, six of leprous, three of toxic neuropathy and one each of acute intermittent porphyria (AIP) and carcinomatous neuropathy. The onset was insidious in a majority of the cases (70%), the presenting symptom being paraesthesia (27.5%). A variable sensory loss was the commonest finding (77.5%). Two patients each had evidence of cranial nerve involvement and affection of urinary bladder and bowel. In both DN and GBS there was a significant reduction in the MNCV especially in the peroneal nerves. In DN 41.6% had evidence of decreased motor unit potentials and 16.6% showed spontaneous fibrillatory activity. For GBS these figures were 77.7% and 8.3% respectively. The peripheral nerve involvement was variable in leprosy and the EMG was normal in all, but one case.

Guillain-Barré syndrome. A clinical electrophysiological and biochemical study.

Fifty-six consecutive patients with Guillain-Barré syndrome representing 49% of all cases of peripheral neuropathy (except those due to diabetes mellitus and leprosy) admitted to the Postgraduate Institute of Medical Education and Research, Chandigarh over a period of three years were studied. All patients developed weakness of limbs within one day to three weeks. Attenuation of deep tendon jerks (98%) paresthesia (66%), cranial nerve involvement (41%) and antecedent infection (32%) were the common clinical features. The common patterns of motor weakness were predominantly proximal in all the four limbs (45%) or predominantly proximal in lower limbs along with distal muscles in upper limbs (29%). Electrodiagnostic studies revealed prolonged distal (motor) latency (82%), reduced motor nerve conduction velocity (74%), sensory nerve conduction abnormality (85%) and evidence of denervation (41%). Evoked motor response of median, ulnar, common peroneal and tibial nerves exhibited significant increase in duration and reduction in amplitude. The maximum incidence of electrophysiological abnormality occurred between four to 12 weeks after the onset of neurological symptoms. Four patients died and 11 showed poor recovery. Long intervals (greater than 3 weeks) between peak deficit and onset of recovery and coexistence of reduced motor nerve conduction velocity with evidence of denervation on EMG were found to be associated with poor recovery.

Eletrodiagnóstico na lepra: exame elétrico clássico

O eletrodiagnóstico nos casos de lepra pode fornecer os seguintes resultados: 1. Eletrodiagnóstico normal ou com reações elétricas anormais quantitativas e qualitativas isoladas, nos casos de lepra sem espessamentos nem amiotrofias ou com espessamentos sem amiotrofias e nas mutilações neurotróficas garves. 2. R.D. tipo ramuscular, nas neurites ramusculares, particularmente na paralisia facial periférica parcelada hanseniana. 3. R.D. tipo troncular, nas neurites tronculares, inclusive na paralisia facial periférica total hanseniana e, nas neurites múltiplas hansenianas. O eletrodiagnóstico na lepra, particularmente nos casos de R.D. tipo ramuscular, é útil para elucidação diagnóstica; também, auxilia o diagnóstico diferencial entre as neurites lepróticas e processos amiotróficos-paral¡ticos resultantes de lesões radiculares, plexuais e medulares. Ainda é valioso para orientação fisioterapêutica e na avaliação prognóstica da evolução da neurites hansenianas e epineurectomias.