The demographic and clinical characteristics of leprosy in Saudi Arabia.

Leprosy is a chronic disease caused by Mycobacterium leprae. Although the occurrence of leprosy has declined in Saudi Arabia, it has not yet been eradicated. To our knowledge, this descriptive retrospective study is the first to assess the clinical presentation of leprosy at the time of diagnosis in Saudi Arabia. All study subjects were leprosy patients admitted to Ibn Sina hospital, the only referral hospital for leprosy in Saudi Arabia, between January 2000 and May 2012. A total of 164 subjects, the majority of whom (65%) were between 21 and 50 years of age, were included, and the male-to-female ratio was 2.8:1. Of these 164 patients, 63% were Saudis, and 77% of all admitted patients were from the western region. Lepromatous leprosy was observed most frequently (33%), and 31% of cases had a positive history of close contact with leprosy. At the time of diagnosis, 84% of all subjects presented with skin manifestation. The prevalence of neurological deficit at the time of diagnosis was 87%. Erythema nodosum leprosum (E.N.L.) developed in only 10% of all subjects. Further studies are needed to determine the clinical characteristics pertaining to each type of leprosy in the region, and training courses in caring for and diagnosing patients with leprosy should be organized for health workers.

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.

Evaluation of simplified tests for the diagnosis of nerve function impairment in leprosy: the Sensory Motor Screening (SMS) study.

OBJECTIVE: Rapid and simple tests for diagnosing nerve function impairment (NFI) in leprosy are required in integrated settings. We examined whether simplified tests performed by newly trained general health workers (GHWs) have comparable diagnostic accuracy to the reference test conducted by experienced physiotherapists. DESIGN: This multi-centre study from India and Bangladesh evaluated three simplified tests named: ILEP Learning Guide Two (M2), Indian dance (M3), and a questionnaire (M4) in 408 people affected by leprosy. Sensitivity (Se) and specificity (Sp) of the three tests were calculated using the full assessment (M1) as reference. Se and Sp were calculated at both whole body and individual nerve levels: whether any NFI and if single NFI (voluntary muscle testing of lid gap, eye closure, little finger out, thumb up and foot up, sensory testing of hands and of feet) was present. RESULTS: M2 had 83% Se and 69% Sp, M3 had 76% Se and 84% Sp and M4 had 85% Se and 46% Sp in diagnosing any NFI. At the level of single NFI, M2 was most or similarly accurate in diagnosing single NFIs with highest prevalence (ST feet, ST hands, little finger out, thumb up), compared to M3 and M4. CONCLUSIONS: ILEP Learning Guide Two (M2) and Indian dance (M3) were found to be the most accurate simplified tests for diagnosing the presence of NFI compared to the reference. M2 was the most useful test, because of greatest accuracy for most of the common types of NFI and inclusion of sensory testing of the hands. M2 is considered to be a useful tool in the hands of GHWs with time constraints in integrated settings.

The flushing patient: differential diagnosis, workup, and treatment / The flushing patient: differential diagnosis, workup, and treatment

Cutaneous flushing-a common presenting complaint to dermatologists, allergists, internists, and family practitioners-results from changes in cutaneous blood flow triggered by multiple conditions. Most cases are caused by very common, benign diseases, such as rosacea or climacterum, that are readily apparent after a thorough taking of history and physical examination. However, in some cases, accurate diagnosis requires further laboratory, radiologic, or histopathologic studies to differentiate several important clinicopathologic entities. In particular, the serious diagnoses of carcinoid syndrome, pheochromocytoma, mastocytosis, and anaphylaxis need to be excluded by laboratory studies. If this work-up is unrevealing, rare causes, such as medullary carcinoma of the thyroid, pancreatic cell tumor, renal carcinoma, and others, should be considered. LEARNING OBJECTIVE: At the completion of this learning activity, participants should be familiar with the mechanisms of flushing, its clinical differential diagnosis, the approach to establish a definitive diagnosis, and management of various conditions that produce flushing.

Nerve thickening in leprosy patients and risk of paralytic deformities: a field based study in Agra, India.

This paper examines the extent of nerve thickening among leprosy patients detected in the field in Agra district. All the clinically diagnosed leprosy patients were examined in detailed for thickening of local cutaneous nerves and peripheral nerve trunks. In each case all the major nerve trunks in both upper and lower extremities, forehead and neck were examined for thickening. Nerve thickening was found in 94% of multibacillary (MB) patients and among 52% paucibacillary (PB) patients. Nerve thickening was found to be more in males, in prevalent cases than in new (untreated) cases and increased significantly with age and delay in diagnosis (P<0.001). Visible deformities of grade > or =2 were found in 10% (58/573) of the leprosy patients; paralytic deformity accounted for 78% (45/58). Claw hand alone or in combination was seen in 82% (37/45) of patients with paralytic deformities. Risk (odd ratio) for deformities was observed to be high (15-18 times) with increasing number of nerves among patients with neuritic leprosy but correlated with delay in diagnosis of over 5 years. Likewise, deformities were more often seen in those with skin lesions, provided they had > or =3 thickened nerves. Findings suggest that early detection and treatment is useful in preventing deformities.

Nerve function impairment in leprosy: design, methodology, and intake status of a prospective cohort study of 2664 new leprosy cases in Bangladesh (The Bangladesh Acute Nerve Damage Study).

The Bangladesh Acute Nerve Damage Study (BANDS) is a prospective cohort study designed to investigate epidemiological, diagnostic, therapeutic and operational aspects of acute nerve function impairment in leprosy. The study is based at a single centre in Bangladesh, in an area with a high prevalence of leprosy. The centre, Danish Bangladesh Leprosy Mission, has a well-established vertical leprosy control programme. In this paper, the study design and methodology are described, together with definitions of nerve function impairment (NFI) used in this and subsequent papers. The study recruited 2664 new leprosy cases in a 12-month period. The male:female ratio is 1.25:1, and 17.61% of the cohort are under 15 years of age. In all, 83.33% of the cohort are paucibacillary (PB), and 16.67% multibacillary (MB). However, the MB rate amongst males is 19.72%, and amongst females is 12.85%, despite an equal period of delay to diagnosis. 55% of patients presented for treatment within 12 months of developing symptoms 6.12% of the total number of cases were smear positive, and 36.71% of the MB cases were smear positive. 9.61% of the total number of cases were graded as having World Health Organisation (WHO) disability grade 1, and 5.97% had grade 2. Amongst MB cases, 27.48% had WHO grade 1 disability present, and 18.24% had grade 2 present, compared with 6.04% and 3.51%, respectively, amongst PB cases. A total of 11.90% of the cohort had sensory NFI of any kind, and 7.39% had motor NFI. Ninety patients presented with NFI needing treatment (3.38%), and of these, 61 (67.78%) had silent NFI. MB patients had a prevalence of reaction/NFI needing treatment nearly 7 times higher than PB cases (15.32% amongst MB; 2.30% amongst PB), and males nearly double that of females (5.67% amongst males, 2.96% amongst females). The most commonly affected nerve by function impairment was the posterior tibial (sensory) with 6.46% of nerves affected (9.38% of patients), followed by the ulnar nerve with 3.23% of nerves impaired (5.56% of patients). Future research and publications, building on this foundation, will focus on the following areas: the incidence of NFI and reactive events, the risk factors for developing NFI, and the response to treatment of patients developing acute NFI.

Nerve function impairment in leprosy at diagnosis and at completion of MDT: a retrospective cohort study of 786 patients in Bangladesh.

This retrospective cohort study includes all new leprosy patients registered for multidrug therapy (MDT) in 1990 at the Danish-Bangladesh Leprosy Mission project in Bangladesh. The main objective was to determine the extent of nerve-function impairment (NFI) at diagnosis and at completion of MDT, and to identify opportunities for intervention and their relative impact on the prevention of disabilities (POD). A total of 786 patients were included; 486 males and 300 females. There were 315 PB, and 471 MB patients. In terms of the WHO leprosy disability grading system, at the time of diagnosis 31/315 (9.8%) had grade 1 or grade 2 disability in the PB group, and 177/471 (37.6%) in the MB group. The incidence rate of NFI during MDT was 3.5 per 100 person years at risk (PYR) in the PB group, and 7.5 per 100 PYR in the MB group. In the MB group 37 (7.9%) previously normal patients sustained NFI during MDT, whilst 19 (4.0%) with NFI at diagnosis showed complete recovery at completion of MDT. The most commonly involved nerves were the ulnar (motor function) and the posterior tibial nerves (sensibility). Reversal reactions were observed in 0.6% of the PB patients during MDT, giving an incidence rate of 1 per 100 PYR. The percentage of MB patients diagnosed with reversal during MDT was 14.2%, giving an incidence rate of 6 per 100 PYR. The percentage of MB patients diagnosed with ENL during MDT was 2.1%, with an incidence rate of 1 per 100 PYR. It was concluded that early detection of new cases of leprosy would prevent disabilities in more than 30% of all patients, thus having the highest impact in the quest for the prevention of disabilities. POD activities during and after MDT will prevent disabilities in approximately 10% of all cases. This study also indicates that treatment with prednisolone is effective and should be available at field level for all patients with recent NFI.

Intra- and inter-tester reliability of sensibility testing in leprosy.

We conducted an intra- and inter-tester agreement study of three sensory screening tests used in nerve function assessment of leprosy patients: the Semmes-Weinstein monofilament (SWM) test, moving 2-point discrimination (M2PD), and the pin prick test. The weighted kappa (Kw) statistic was used as the reliability coefficient. The SWM had intra-observer Kws ranging from 0.83 to 0.92 and inter-observer Kws ranging from 0.76 to 0.89. The M2PD had intra- and inter-tester Kws ranging from 0.75 to 0.82 and 0.54 to 0.82, respectively. Inter-tester agreement for the pin prick test ranged from 0.45 to 0.85. There was evidence that the main source of variability between testers was testing skill and experience. Among the experienced physiotechnicians there was no significant difference between intra- and inter-tester reliability. We conclude that reliability of the SWM test was very good, closely followed by the M2PD test. Reliability of the pin prick test was less good than that of the SWM and M2PD, making it less suitable for serial testing.

Vibration sense and tarsal disintegration.

The extent of loss of vibration and pressure sensations was assessed in 21 leprosy patients with disintegration of the tarsus. Feet which had and did not have tarsal disintegration both showed severe impairment of pressure sensation, but the loss of vibration sense was more severe in feet which had undergone the destructive process. It appears that loss of deep sensation is an important factor in the process of tarsal disintegration in feet which are already anaesthetic. Measurement of vibration sense using a biosthesiometer may be a valuable clinical test in the investigation and follow-up of the patient with the insensitive foot to identify those at risk of developing tarsal disintegration.

[Visual evoked potentials in chronic Chagas-Mazza disease]. / Potenciales evocados visuales en la enfermedad de Chagas-Mazza crónica

Both modalities of visual evoked potentials (VEP), flash (F) and pattern (P), were carried out in 17 patients with chronic Chagas disease. The patients included, between 21 and 65 years old, presented an evolution period of more than 7 years and a minimum of two positive serologies. Those patients with diabetes, alcoholism, leprosy, syphilis and degenerative diseases of the central nervous system, so as intoxications of different etiologies, and visual disorders detected through ophthalmological examination were discarded. Everyone was studied by means of routine clinical and complementary analysis, electrocardiogram, ophthalmological examination and specific analyses for the disease, like Machado-Guerreiro reaction, immunofluorescence and hemoagglutination tests. The VEP results showed alterations in the morphology of the record and a decrease of the potential amplitude in 35% of these patients. The electroneurophysiological alterations would suggest a correlation with anatomopathologic findings, that show loss of neuronal groups in autopsies of chronic Chagasic patients.

Potenciales evocados visuales en la enfermedad de Chagas-Mazza crónica / Visual evoked potentials in chronic Chagas-Mazza disease

Se efectuaron potenciales evocados visuales (PEV) en sus variantes flash (F) y pattern (P), en 17 pacientes afectados de enfermedad de Chagas crónica. La edad del grupo se halló comprendida entre los 21 y 65 años. Se incluyeron pacientes con período de evolución mayor de 7 años y dos serologías positivas como mínimo. La selección fue descartando a los portadores de diabetes, alcoholismo, lepra, sífilis y enfermedades degenerativas del sistema nervioso central, como así intoxicaciones de diversas etiologías y afecciones visuales detectadas en el exámen oftalmológico y específicos como la reacción de Machado-Guerreiro, test de inmunofluorescência y test de hemoaglutinación. Los resultados de los PEV mostraron fundamentalmente alteraciones en la morfología del trazado y disminución de la amplitud del potencial, en el 35% de los pacientes estudiados. Estas alteraciones aelectroneurofisiológicas permitirían inferir correlación a los hallazgos anatomopatológicos, que muestran pérdida de grupos neuronales en autopsias de pacientes chagásicos crónicos

An attempt to reduce the loss of pain and touch sensations in leprosy patients.

An attempt was made to improve the perception of pain and touch sensations at the leprosy lesions. The loss of pain and touch sensations in a lesion was graded using Pain/Touch-Sensation-Testing-and-Grading devices. Application of a solution containing 1 mg of histamine per ml of DMSO, at the affected area decreased the grades of the loss of pain sensation in 11 (31.4%) patients and of touch sensation in 8 (22.8%) patients, out of the 35 patients tested, indicating an improvement in the perception at the lesion. This effect, however, did not persist even for 5 minutes. A higher concentration (2 mg/ml) of histamine produced reduction in the sensory loss in a larger percentage (47% for pain and 35.3% for touch) of patients, though the duration of this effect was still not prolonged.

Weight appreciation in the hand in normal subjects and in patients with leprous neuropathy.

The Weber fraction was used as an index of the sensitivity with which subjects appreciated weights ranging from 20 to 500 g suspended from the middle finger. Normal subjects were able to appreciate weight when it caused cutaneous compression alone. The sensitivity increased when subjects lifted weights by flexing the metacarpophalangeal joint. This increase was more marked for weights ranging from 20 to 100 g than for weights ranging from 200 to 500 g. When subjects lifted weights by flexing the elbow joint, the sensitivity with which they appreciated weight was comparable to that from cutaneous compression alone. Leprous subjects having glove anaesthesia were unable to appreciate any weight up to 500 g when it caused cutaneous compression alone. However, they were able to appreciate weights above 200 g when they lifted weights by flexing the metacarpophalangeal or elbow joints.

Cutaneous anaesthesia in necrobiosis lipoidica.

Cutaneous sensation in plaques of necrobiosis lipoidica was assessed in five non-diabetic and seven diabetic patients. Eleven of the twelve showed partial or complete anaesthesia of the affected skin. These findings are important in the differential diagnosis of tuberculoid leprosy. Further studies of nerve function in cutaneous granulomas need to be carried out.

Transtornos neurológicos associados ao mal perfurante plantar leprótico

Os autores analisam os distúrbios neurológicos sensitivos, motores e tróficos em 100 pacientes de lepra, 32 dos quais portadores de mal perfurante plantar. Concluem ser o m.p.p. complicação tardia do processo patológico hanseniano que se manifesta tôda vez que profundas modificações sensitivas, motoras e tróficas, devidas principalmente a transtornos da inervação profunda dos pés, alteram a arquitetura dos segmentos distais dos membros inferiores.