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.

Kinship and Leprosy in the Contacts of Leprosy Patients: Cohort at the Souza Araújo Outpatient Clinic, Rio de Janeiro, RJ, 1987-2010.

A broad variety of factors have been associated with leprosy among contacts, including socioeconomic, epidemiological, and genetic characteristics. Data from 7,174 contacts of leprosy patients from a leprosy outpatient clinic in Rio de Janeiro, Brazil, 1987-2010, were analyzed to investigate the effects of kinship, individual, and contextual factors on leprosy. Multivariate analyses were performed using a robust estimation method. In the prevalence analysis, close kinship (sibling OR = 2.75, offspring OR = 2.00, and other relatives OR = 1.70), socioeconomic factors, and the duration of exposure to the bacillus were associated to leprosy. In the incidence analysis, significant risks were found for all categories of kinship (parents RR = 10.93, spouse, boyfriend/girlfriend, and bride/groom RR = 7.53, sibling RR = 7.03, offspring RR = 5.34, and other relatives RR = 3.71). Once the treatment of the index case was initiated, other factors lost their significance, and the index case bacteriological index and BCG (Bacillus Calmette-Guérin vaccine) protection had a greater impact. Our findings suggested that both genetic susceptibility and physical exposure play an important role in the epidemiology of leprosy, but it was not possible establishing the role of genetic factor. Analyses of other factors related to the genotype of individuals, such as genetic polymorphisms, are needed.