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.

Exploration of Neurofilament Light Chain and Nerve Ultrasound in Leprotic Neuropathy.

OBJECTIVES: To explore neurofilament light chain (NfL) levels in leprotic neuropathy compared to controls, and to determine if the changes correlate with ultrasonographic nerve findings. METHODS: Individuals with leprosy with signs or symptoms suggestive of peripheral nerve involvement were recruited. They were evaluated by clinical examination, functional scores, laboratory assessments (including NfL), nerve conduction studies (NCS), and ultrasound. Ultrasound was conducted in bilateral median, ulnar, tibial, fibular, sural, and vagus nerves as well as cervical roots 5 and 6. Results were compared to age, sex, and body mass index matched healthy controls. RESULTS: A total of 320 nerves from 20 patients and 480 nerves from 30 controls were evaluated. NfL was significantly elevated in those with leprosy with a mean and standard deviation of 7.50 + 2.83 compared with 3.42 + 1.18 in controls (P < .001). Ultrasound showed focal enlargement of the nerves, particularly at entrapment sites. Additionally, there were noticeable changes in neural Doppler signal, echogenicity, and epineural thickness among the measured nerve sites. NfL levels in those with leprosy correlated closely with nerve cross-sectional area at all sites (P < .05). Functional and clinical assessment scores correlated with NfL and sonographic cross-sectional area as well (P ≤ .05). CONCLUSIONS: NfL is elevated in leprotic neuropathy. Ultrasound showed specific morphological changes in individuals with leprosy, and nerve enlargement correlated with NfL levels. Thus, both modalities may be useful for the diagnosis, prognosis, and disease monitoring in those with leprotic neuropathy, and further investigations are warranted.

Role of Nerve Conduction Studies in Hansen's Disease.

Background and Objective: To report the role of nerve conduction study (NCS) in diagnosis, monitoring, and prognosis of Hansen's disease (HD). Materials and Methods: In a hospital-based prospecive observational study, the patients with HD as per World Health Organization (WHO) criteria were included; muscle wasting power, reflexes, and sensations were recorded. Motor NCS of median, ulnar, and peroneal nerves and sensory NCS of ulnar, median, and sural nerves were recorded. Disability was graded using WHO grading scale. The outcome was assessed after 6 months using modified Rankin scale. Results: In the present study, 38 patients with a median age of 40 (15-80) years and five females were included. The diagnosis was tuberculoid in seven, borderline tuberculoid in 23, borderline lepromatous in two, and borderline in six patients. The disability was grade 1 and 2 in 19 patients each. Out of 480 nerves studied, NCS was normal in 139 sensory (57.4%) and 160 (67.2%) motor nerves. NCSs were axonal in seven sensory and eight motor nerves, demyelinating in three nerves, and mixed in one in seven patients who had lepra reaction. NCS findings did not correlate with disability (p = 1.0) or outcome (0.304) and provided additional information in 11 nerves (seven patients). Peripheral nerves were enlarged in 79. NCSs were normal in 32 (29.90%) in thickened nerves. Conclusion: In HD, NCS abnormalities correlated with respective sensory or motor abnormality but related with neither disability nor the outcome.

A-waves are associated with neuropathic pain in leprosy.

INTRODUCTION/AIMS: The A-wave is a late response related either to demyelination or early axonal regeneration. It may be helpful in the evaluation of some peripheral neuropathies. In leprosy, previous studies suggested that A-waves could be a neurophysiological marker of pain in patients during reactions. Herein we have attempted to further assess the profile and clinical correlates of A-waves by exploring a large leprosy cohort. METHODS: Between 2015 and 2018, 63 patients with leprosy (47 men and 16 women) had A-waves in nerve conduction studies and were included in this study. We included patients regardless of whether they were experiencing leprosy reactions or not. We then compared clinical features in nerves with and without A-waves. RESULTS: The mean age of study participants was 46.5 ± 12.3 years and most had borderline leprosy. From this cohort, we assessed separately 83 motor nerves that demonstrated A-waves (group A+ ) and 29 motor nerves that did not demonstrate A-waves (group A- ). Neuropathic pain (NP) was found in 66 of 83 nerves in group A+ , but only 5 of 29 in group A- (79.5 vs 17.2%, P < .001). In contrast, no significant between-group difference emerged regarding presence of reactions, sensory function (based on Semmes-Weinstein evaluations), or muscle strength. A-waves were found in nerves with neuropathic pain experiencing (39 of 66 = 59%) or not experiencing (27 of 66 = 41%) leprosy reactions. DISCUSSION: These results show that A-waves are associated with neuropathic pain in leprosy patients, regardless of the nerves affected and the immune status (in reaction or not).

Follow-up assessment of patients with Pure Neural Leprosy in a reference center in Rio de Janeiro-Brazil.

INTRODUCTION: Pure Neural Leprosy (PNL) is a rare clinical form of leprosy in which patients do not present with the classical skin lesions but have a high burden of the disability associated with the disease. Clinical characteristics and follow up of patients in PNL are still poorly described in the literature. OBJECTIVE: This paper aims to describe the clinical, electrophysiological and histopathological characteristics of PNL patients, as well as their evolution after multidrug therapy (MDT). METHODS: Fifty-two PNL patients were selected. Clinical, nerve conduction studies (NCS), histopathological and anti-PGL-1serology were evaluated. Patients were also assessed monthly during the MDT. At the end of the MDT, all of the patients had a new neurological examination and 44 were submitted to another NCS. RESULTS: Paresthesia was the complaint most frequently reported by patients, and in the neurological examination the most common pattern observed was impairment in sensory and motor examination and a mononeuropathy multiplex. Painful nerve enlargement, a classical symptom of leprosy neuropathy, was observed in a minority of patients and in the motor NCS axonal injuries, alone or in combination with demyelinating features, were the most commonly observed. 88% of the patients did not present any leprosy reaction during MDT. There was no statistically significant difference between the neurological examinations, nor the NCS pattern, performed before and after the MDT. DISCUSSION: The classical hallmarks of leprosy neuropathy are not always present in PNL making the diagnosis even more challenging. Nerve biopsy is an important tool for PNL diagnosis as it may guide therapeutic decisions. This paper highlights unique characteristics of PNL in the spectrum of leprosy in an attempt to facilitate the diagnosis and management of these patients.

Clinical and electrophysiological characteristics of neuropathic pain in leprosy patients: A prospective cross-sectional study.

Introduction Neuropathic pain is a common and disabling late complication of leprosy. We investigated the clinical and electrophysiological characteristics of neuropathic pain in leprosy patients by evaluating nerve conduction, sympathetic skin response (SSR) and A-waves. Methods Twenty one leprosy patients with neuropathic pain validated by the Douleur Neuropathique en 4 (DN4)Questionnaire were selected for study. Pain intensity was measured by the visual analog scale. Demographic and clinical data were collected for all patients. Clinical data included appraisal of the median, ulnar, radial, tibial and common peroneal nerves, assessment of the sympathetic skin response and conventional electrophysiological recordings. Results Among all electroneuromyographic presentations, multifocal mononeuropathy was still the most prevalent. Sensory loss was observed more frequently than motor deficits. As most patients presented advanced clinical forms of leprosy and were under treatment, this high mean was found and the ulnar nerve was most frequently affected. The sympathetic skin response was absent in 16 patients. Higher DN4 Questionnaire scores were observed in women and in those receiving corticosteroid therapy. These inferences are possible to be made, but our study's limitations don't allow us to be certain about it. The statistical significance found only permits us to evidence what we related on the textual part of the study. Limitations The small number of patients studied, the lack of sophisticated diagnostic methods for leprosy, as well as the difficulties in assessing nerve conduction were the main limitations of this study. Conclusion The neurophysiological and clinical findings in leprous neuropathy were modest despite the conspicuous neuropathic pain. Although electrophysiological studies are a vital tool to verify nerve damage, variations in the clinical presentation of leprosy neuropathic pain render the diagnosis challenging. Further studies are needed to describe the neurophysiological evolution of this disease.

A-waves associated are with neuropathic pain in leprosy

Introduction/Aims:The A-wave is a late response related either to demyelination or early axonal regeneration. It may be helpful in the evaluation of some peripheral neuropathies. In leprosy, previous studies suggested that A-waves could be a neurophysiological marker of pain in patients during reactions. Herein we have attempted to further assess the profile and clinical correlates of A-waves by exploring a large leprosy cohort. Methods: Between 2015 and 2018, 63 patients with leprosy (47 men and 16 women) had A-waves in nerve conduction studies and were included in this study. We included patients regardless of whether they were experiencing leprosy reactions ornot. We then compared clinical features in nerves with and without A-waves. Results:The mean age of study participants was 46.5 ± 12.3 years and most had borderline leprosy. From this cohort, we assessed separately 83 motor nerves that demonstrated A-waves (group A+) and 29 motor nerves that did not demonstrate A-waves (group A-). Neuropathic pain (NP) was found in 66 of 83 nerves in group A+,but only 5 of 29 in group A-(79.5 vs 17.2%,P< .001). In contrast, no significant between-group difference emerged regarding presence of reactions, sensory function (based on Semmes-Weinstein evaluations), or muscle strength. A-waves were found in nerves with neuropathic pain experiencing (39 of 66=59%) or not experiencing (27 of 66=41%) leprosy reactions. Discussion: These results show that A-waves are associated with neuropathic pain in leprosy patients, regardless of the nerves affected and the immune status (in reaction or not).

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.

Clinical and Neurophysiological Features of Leprosy Patients with Neuropathic Pain.

Neural pain is a frequent symptom in leprosy disease. There is a paucity of data regarding neural pain diagnostics resulting in common prescriptive errors when neuritis is confused with neuropathic or mixed nociceptive-neuropathic pain. The present study identified important demographic, clinical, and neurophysiological features of 42 leprosy neuropathy patients presenting neuropathic pain (NP). During routine evaluations, patients were selected asking if they had ever experienced neural pain. Data analyses of their pain characteristics, clinical examination results, and both the Douleur Neuropathique 4 Questionnaire and Hamilton Depression Scale scores were used to classify these patients. The most common word they used to describe the sensation of pain for 25 (60%) of these patients was "burning." In the early stages of the disease and before leprosy diagnosis, 19 (45%) had already complained about NP and leprosy treatment was unable to prevent its occurrence in 15 (36%). Leprosy reactions, considered NP risk factors, occurred in 32 (76%) cases. Knowledge of typical NP characteristics could be used to develop more effective therapeutic approaches for a notoriously difficult-to-treat pain condition.

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: Current status and relevance.

Pure neuritic leprosy has always been an enigma due to its clinical and management ambiguities. Although only the Indian Association of Leprologist's classification recognizes 'pure neuritic leprosy' as a distinct sub group of leprosy, cases nonetheless are reported from various countries of Asia, Africa, South America and Europe, indicating its global relevance. It is important to maintain pure neuritic leprosy as a subgroup as it constitutes a good percentage of leprosy cases reported from India, which contributes to more than half of global leprosy numbers. Unfortunately, a high proportion of these patients present with Grade 2 disability at the time of initial reporting itself due to the early nerve involvement. Although skin lesions are absent by definition, when skin biopsies were performed from the skin along the distribution of the affected nerve, a proportion of patients demonstrated leprosy pathology, revealing sub-clinical skin involvement. In addition on follow-up, skin lesions are noted to develop in up to 20% of pure neuritic leprosy cases, indicating its progression to manifest cutaneous disease. Over the decades, the confirmation of diagnosis of pure neuritic leprosy has been subjective, however, with the arrival and use of high-resolution ultrasonography (HRUS) for nerve imaging, we have a tool not only to objectively measure and record the nerve thickening but also to assess the morphological alterations in the nerve including echo texture, fascicular pattern and vascularity. Management of pure neuritic leprosy requires multidrug therapy along with appropriate dose of systemic corticosteroids, for both acute and silent neuritis. Measures for pain relief, self-care of limbs and physiotherapy are important to prevent as well as manage disabilities in this group of patients.

Inflammatory Cytokines Are Involved in Focal Demyelination in Leprosy Neuritis.

Mycobacterium leprae (ML) infection causes nerve damage that often leads to permanent loss of cutaneous sensitivity and limb deformities, but understanding of the pathogenesis of leprous neuropathy that would lead to more effective treatments is incomplete. We studied reactional leprosy patients with (n = 9) and without (n = 8) acute neuritis. Nerve conduction studies over the course of the reactional episode showed the findings of demyelination in all patients with neuritis. Evaluation of patient sera revealed no correlation of the presence of antibodies against gangliosides and the clinical demyelination. In nerve biopsies of 3 patients with neuritis, we identified tumor necrosis factor (TNF), TNF receptors, and TNF-converting enzyme in Schwann cells (SCs) using immunofluorescence. To elucidate immunopathogenetic mechanisms, we performed experiments using a human SC line. ML induced transmembrane TNF and TNF receptor 1 expression in the SCs; TNF also induced interleukin (IL)- 6 and IL-8 production by the SCs; and ML induced IL-23 secretion, indicating involvement of this previously unrecognized factor in leprosy nerve damage. These data suggest that ML may contribute to TNF-mediated inflammation and focal demyelination by rendering SCs more sensitive to TNF within the nerves of patients with leprous neuropathy.

Primary Motor Cortex Representation of Handgrip Muscles in Patients with Leprosy.

BACKGROUND: Leprosy is an endemic infectious disease caused by Mycobacterium leprae that predominantly attacks the skin and peripheral nerves, leading to progressive impairment of motor, sensory and autonomic function. Little is known about how this peripheral neuropathy affects corticospinal excitability of handgrip muscles. Our purpose was to explore the motor cortex organization after progressive peripheral nerve injury and upper-limb dysfunction induced by leprosy using noninvasive transcranial magnetic stimulation (TMS). METHODS: In a cross-sectional study design, we mapped bilaterally in the primary motor cortex (M1) the representations of the hand flexor digitorum superficialis (FDS), as well as of the intrinsic hand muscles abductor pollicis brevis (APB), first dorsal interosseous (FDI) and abductor digiti minimi (ADM). All participants underwent clinical assessment, handgrip dynamometry and motor and sensory nerve conduction exams 30 days before mapping. Wilcoxon signed rank and Mann-Whitney tests were performed with an alpha-value of p<0.05. FINDINGS: Dynamometry performance of the patients' most affected hand (MAH), was worse than that of the less affected hand (LAH) and of healthy controls participants (p = 0.031), confirming handgrip impairment. Motor threshold (MT) of the FDS muscle was higher in both hemispheres in patients as compared to controls, and lower in the hemisphere contralateral to the MAH when compared to that of the LAH. Moreover, motor evoked potential (MEP) amplitudes collected in the FDS of the MAH were higher in comparison to those of controls. Strikingly, MEPs in the intrinsic hand muscle FDI had lower amplitudes in the hemisphere contralateral to MAH as compared to those of the LAH and the control group. Taken together, these results are suggestive of a more robust representation of an extrinsic hand flexor and impaired intrinsic hand muscle function in the hemisphere contralateral to the MAH due to leprosy. CONCLUSION: Decreased sensory-motor function induced by leprosy affects handgrip muscle representation in M1.

Leprosy neuropathy evaluated by NCS is independent of the patient's infectious state.

INTRODUCTION: Leprosy causes nerve injury, which mimics clinical and neurophysiological conditions, rendering it an excellent model of peripheral neuropathy. METHODS: A retrospective study including 822 nerve conduction studies (NCS) of 509 patients was developed to appraise the electrophysiological pattern of leprosy neuropathy. NCS of motor and sensory nerves performed before, during, and after multidrug therapy (MDT) were analyzed. RESULTS: During the three periods of MDT, while NCS alterations were similar regarding extension, topography, damage severity, and type of lesion, NCS showed that sensory was more frequent (sural nerve) (92-96%) than motor impairment (70-77%) (ulnar nerve). CONCLUSION: Once axonal loss has been installed, nerve function is little affected by inflammatory, immune and/or bacterial events since chronic neuropathy has been established, inevitably leading to the well-known leprosy sequelae occurring at any time before and/or after leprosy diagnosis.

Primary neural leprosy: systematic review.

The authors proposed a systematic review on the current concepts of primary neural leprosy by consulting the following online databases: MEDLINE, Lilacs/SciELO, and Embase. Selected studies were classified based on the degree of recommendation and levels of scientific evidence according to the "Oxford Centre for Evidence-based Medicine". The following aspects were reviewed: cutaneous clinical and laboratorial investigations, i.e. skin clinical exam, smears, and biopsy, and Mitsuda's reaction; neurological investigation (anamnesis, electromyography and nerve biopsy); serological investigation and molecular testing, i.e. serological testing for the detection of the phenolic glycolipid 1 (PGL-I) and the polymerase chain reaction (PCR); and treatment (classification criteria for the definition of specific treatment, steroid treatment, and cure criteria).

Primary neural leprosy: systematic review / Hanseníase neural primária: revisão sistemática

The authors proposed a systematic review on the current concepts of primary neural leprosy by consulting the following online databases: MEDLINE, Lilacs/SciELO, and Embase. Selected studies were classified based on the degree of recommendation and levels of scientific evidence according to the “Oxford Centre for Evidence-based Medicine”. The following aspects were reviewed: cutaneous clinical and laboratorial investigations, i.e. skin clinical exam, smears, and biopsy, and Mitsuda's reaction; neurological investigation (anamnesis, electromyography and nerve biopsy); serological investigation and molecular testing, i.e. serological testing for the detection of the phenolic glycolipid 1 (PGL-I) and the polymerase chain reaction (PCR); and treatment (classification criteria for the definition of specific treatment, steroid treatment, and cure criteria).

Os autores propuseram-se a realizar uma revisão sistemática em conceitos atuais sobre a hanseníase neural primária, consultando as seguintes bases bibliográficas on-line: MEDLINE, Lilacs/SciELO e Embase. Os estudos selecionados foram classificados conforme o grau de recomendação e o nível de evidência científica de acordo com o “Oxford Centre for Evidence-based Medicine”. Os seguintes temas foram revisados: investigações clínica e laboratorial cutâneas, ou seja, exame, esfregaço e biópsia de pele e reação de Mitsuda; investigação neurológica (anamnese, eletroneuromiografia e biópsia de nervo); investigação sorológica e testes moleculares, ou seja, testes sorológicos para detecção de um glicolipídio fenólico e reação de cadeia de polimerase (PCR) e tratamento (critérios de classificação para definição de tratamento específico, tratamento com esteroides e critérios de cura).

An unusual cause of mononeuritis multiplex.

A middle-aged man of South Asian decent presented with a 4-month history of bilateral sensory disturbance affecting the median nerve distribution and dorsum of both feet. Neurological examination was otherwise normal. A patchy absence of sensory responses was noted on nerve conduction studies and electromyogram (NCS/EMG). Over the next 3 months sensory symptoms progressed to involve median, radial, ulnar, sural and peroneal nerves bilaterally. Repeat NCS/EMG confirmed a mononeuritis multiplex predominantly involving the sensory fascicles. Areas of hypopigmentation, a right-lower motor facial weakness and ophthalmic branch trigeminal nerve involvement were noted on examination. Punch skin biopsy as well as sural nerve biopsy demonstrated chronic granulomatous inflammation without evidence of Mycobacterium. A slit skin smear test demonstrated Mycobacterium leprae consistent with a diagnosis of primary neuritic leprosy. In the appropriate clinical context, leprosy should be included in the differential diagnosis of mononeuritis multiplex.

Ulnar neuropathy in hansen disease: clinical, high-resolution ultrasound and electrophysiologic correlations.

OBJECTIVE: To assess the relationship between the cross-sectional area (CSA) of the ulnar nerve by ultrasound (US) with clinical and electrophysiologic findings in Hansen ulnar neuropathy. METHODS: Twenty-one patients (42 arms) with Hansen disease (mean age 30.0 ± 12.97, range 13-61 years, borderline tuberculoid 29%, borderline lepromatous, 19% lepromatous leprosy 42%, and pure neuritic type 10%) were examined clinically for ulnar sensory and motor weakness. The ulnar nerve was ultrasonographically examined from the wrist to the axilla, and CSA was measured at the level of maximum enlargement. Ulnar sensory nerve conduction was recorded orthodromically with ring electrodes placed at the fifth digit and amplitude of sensory nerve action potential (SNAP) recorded 3 cm proximal to the distal wrist crease. Motor conduction velocity (MCV) was recorded at the wrist-below the elbow, below the elbow-above the elbow, and above the elbow-axilla segments. RESULTS: Out of the 42 arms with Hansen disease, 76% had clinically motor weakness, and 43% had sensory loss in the upper limbs innervated by the ulnar nerve. As compared with healthy subjects, the patients with Hansen ulnar neuropathy had a statistically significant reduction in SNAP (P ≤ 0.0001) and MCV (P ≤ 0.0001). It was observed that the maximum enlargement of the ulnar nerve in all the patients was a few centimeters above the elbow segment. The mean CSA of ulnar nerve above the medial epicondyle was 18 ± 15 mm as compared with controls 4.83 ± 1.12 mm (P < 0.0001). In addition to nerve thickening, US depicted abnormality in morphology. In 55%, the nerve was hypoechoic, and in 7.1%, the nerve pattern was oligofascicular. Color Doppler (CD) flow signals were observed in all the nerves with loss of fascicular pattern and in 40% of the nerves that were hypoechoic. A statistically significant correlation was found between CSA of ulnar nerve above the medial epicondyle vs. MCV at BE-AE and compound muscle action potentials (CMAP) above the elbow in the patients with clinical motor weakness (r = -0.55, P < 0.001) and (r = -0.57, P < 0.001), respectively. There was no statistical significant correlation between CSA and SNAP in the patients with (r = -0.52, P = 0.23) and without (r = -0.07, P = 0.83) sensory loss. CONCLUSIONS: In leprosy patients, a positive correlation exits between the presence of motor weaknesses of the ulnar nerve innervated muscles, sonographically thickening of the ulnar nerve, and motor conduction slowing of the ulnar nerve at the BE-AE segment. In addition, US provided information on nerve morphologic alterations regarding the echo texture and location of nerve enlargement.

Nerve conduction studies in paucibacillary and multibacillary leprosy: a comparative evaluation.

Involvement of peripheral nerves in patients with leprosy results in sensory, motor and autonomic dysfunctions along with deformities and disability. Pattern of nerve involvement is different for different forms of leprosy. In this study, we evaluated and compared the nerve conduction parameters of paucibacillary leprosy with that of multibacillary leprosy. In this study, 40 consecutive patients of leprosy (19 cases of paucibacillary and 21 cases of multibacillary leprosy) were included. Nerve conduction studies were performed according to the standard procedure described in the manual of the machine. We observed that patients with multibacillary leprosy had significantly more severe changes in nerve conduction parameters as compared to that of paucibacillary leprosy. In paucibacillary leprosy, the dominant pattern of nerve involvement was that of mononeuropathy, however, in 6 paucibacillary cases the nerve involvement was in form of mononeuritis multiplex. Electrophysiological assessment also revealed involvement of clinically uninvolved nerves. Nerve conduction parameters were suggestive of mixed axonal as well as demyelination of the peripheral nerves.

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.