BackgroundLeprosy neuropathy is considered the most common peripheral neuropathy of infectious etiology worldwide, representing a public health problem. Clinical diagnosis of primary neural leprosy (PNL) is challenging, since no skin lesions are found and the slit skin smear bacilloscopy is negative. However, there are still controversial concepts regarding the primary-neural versus pure-neural leprosy definition, which will be explored by using multiple clinical-laboratory analyses in this study.Methodology/Principal findingsSeventy patients diagnosed with primary neural leprosy from 2014 to 2016 underwent clinical, laboratorial and neurophysiological evaluation. All patients presented an asymmetric neural impairment, with nerve thickening in 58.6%. Electroneuromyography showed a pattern of mononeuropathy in 51.4%. Positivity for ELISA anti-PGL1 was 52.9%, while the qPCR of slit skin smear was 78.6%. The qPCR of nerve biopsies was positive in 60.8%. Patients with multiple mononeuropathy patterns showed lower levels of anti-PGL-1 (p = 0.0006), and higher frequency of neural thickening (p = 0.0008) and sensory symptoms (p = 0.01) than those with mononeuropathy.Conclusions/SignificancePNL is not a synonym of pure neural leprosy, as this condition may include a generalized immune response and also a skin involvement, documented by molecular findings. Immunological, molecular, and neurophysiological tools must be implemented for diagnosing primary neural leprosy to achieve effective treatment and reduction of its resultant disabilities that still represent a public health problem in several developing nations. Finally, we propose a algorithm and recommendations for the diagnosis of primary neural leprosy based on the combination of the three clinical-laboratorial tools.

The hereditary neuropathy with liability to pressure palsies (HNPP) is an autossomal dominant disorder manifesting recurrent mononeuropathies. Objective Evaluate its clinical and nerve conduction studies (NCS) characteristics, searching for diagnostic particularities. Method We reviewed the neurological manifestations of 39 and the NCS of 33 patients. Results Family history was absent in 16/39 (41%). The onset complaints were weakness in 24, pain in 6, sensory deficit in 5 and paresthesias in 4. Pain was seen in 3 other patients. The following neuropathy patterns were found: multiple mononeuropathy (26), mononeuropathy (7), chronic sensorimotor polyneuropathy (4), chronic sensory polyneuropathy (1) and unilateral brachial plexopathy (1). NCS showed a sensorimotor neuropathy with focal conduction slowing in 31, two had mononeuropathy and another brachial plexopathy. Conclusion HNPP presentation is variable and may include pain. The most frequent pattern is of an asymmetrical sensory and motor neuropathy with focal slowing at specific topographies on NCS.

BackgroundHousehold contacts constitute the highest risk group for leprosy development, and despite significant progress in the disease control, early diagnosis remains the primary goals for leprosy management programs.MethodsWe have recruited 175 seropositive and 35 seronegative household contacts from 2014 to 2016, who were subjected to an extensive protocol that included clinical, molecular (peripheral blood qPCR, slit-skin smear qPCR, skin biopsy qPCR) and electroneuromyographic evaluations.Results/Principal findingsThe positivity of peripheral blood qPCR of seropositive contacts was 40.6% (71/175) whereas only 8.6% (3/35) were qPCR positive in seronegative contacts (p = 0.0003). For the slit-skin smear, only 4% (7/175) of seropositive contacts presented positive bacilloscopy, whereas the qPCR detected 47.4% (83/175) positivity in this group compared with only 17.1% (6/35) in seronegative contacts (p = 0.0009). In the ENMG evaluation of contacts, 31.4% (55/175) of seropositives presented some neural impairment, and 13.3% (4/35) in seronegatives (p = 0.0163). The presence of neural thickening conferred a 2.94-fold higher chance of ENMG abnormality (p = 0.0031). Seropositive contacts presented a 4.04-fold higher chance of neural impairment (p = 0.0206). The peripheral blood qPCR positivity presented odds 2.08-fold higher towards neural impairment (OR, 2.08; p = 0.028). Contrarily, the presence of at least one BCG vaccine scar demonstrated 2.44-fold greater protection against neural impairment (OR = 0.41; p = 0.044).Conclusions/SignificanceELISA anti-PGL-I is the most important test in determining the increased chance of neural impairment in asymptomatic leprosy household contacts. The combination of the two assays (ELISA anti-PGL-I and peripheral blood qPCR) and the presence of BCG scar may identify individuals with higher chances of developing leprosy neuropathy, corroborating with the early diagnosis and treatment.

The aim of this study was to describe the results of a Brazilian Consensus on Small Fiber Neuropathy (SFN). Fifteen neurologists (members of the Brazilian Academy of Neurology) reviewed a preliminary draft. Eleven panelists got together in the city of Fortaleza to discuss and finish the text for the manuscript submission. Small fiber neuropathy can be defined as a subtype of neuropathy characterized by selective involvement of unmyelinated or thinly myelinated sensory fibers. Its clinical picture includes both negative and positive manifestations: sensory (pain/dysesthesias/pruritus) or combined sensory and autonomic complaints, associated with an almost entirely normal neurological examination. Standard electromyography is normal. A growing list of medical conditions is associated with SFN. The classification of SFN may also serve as a useful terminology to uncover minor discrepancies in the normal values from different neurophysiology laboratories. Several techniques may disclose sensory and/or autonomic impairment. Further studies are necessary to refine these techniques and develop specific therapies.

Background The early recognition of neural impairment in leprosy, especially in primary neural forms, represents a challenge in clinical practice and a peripheral nerve biopsy may be required for diagnostic confirmation. This study aims to characterize the epidemiological, clinical, electroneuromyographic, laboratory and histopathological aspects of patients undergoing peripheral nerve biopsy during investigation of primary neural cases in leprosy. Methods A total of 104 patients with peripheral neuropathy who were referred to a national reference centre for leprosy were biopsied from 2014 to 2018. All cases underwent clinical, laboratory, histopathological and electroneuromyographic evaluations. Results Of 104 biopsied patients, leprosy was confirmed in 89.4% (93/104). The biopsied nerves were the ulnar (67.8% [63/93]), superficial fibular (21.5% [20/93]), sural (8.6% [8/93]), radial (1.1% [1/93]) and deep fibular (1.1% [1/93]). Twenty-nine percent (27/93) presented histopathological abnormalities and 4.4% (4/93) presented acid-fast bacilli. Nerve and superjacent skin quantitative polymerase chain reaction were positive in 49.5% (46/93) and 24.8% (23/93) of cases, respectively. Patients with multiple mononeuropathy had a higher frequency of histopathological abnormalities (p=0.0077). Conclusions This study reinforces peripheral nerve biopsy's role as an important tool in the investigation of primary neural cases, contributing to the early diagnosis and also reducing diagnostic errors and the need for empirical treatment.

Anterior tarsal tunnel syndrome is a compressive neuropathy of the deep peroneal nerve in the anterior ankle at the extensor retinaculum. Several factors may be the cause of anterior tarsal tunnel syndrome: trauma, fractures, subluxation, abnormal posture, edema and others, although there is no association with infectious diseases 1,2. We report on a 37-year-old woman, with a family history of leprosy, with positive serological (ELISA anti-PGL 1) and molecular (real-time PCR/qPCR of slit-skin smear) tests, without skin lesions. Electroneuromyography showed a demyelinating sensory-motor deep peroneal nerve impairment in the ankle, with secondary axonal loss. Peripheral sensory nerve biopsy, confirmed by molecular methods, revealed an atypical primary neural leprosy 3,4 .

Objetivo: Descrever os riscos do uso de ansiolíticos e indutores do sono na população idosa. Revisão bibliográfica: A polifarmácia cresce cada vez mais dentro da população idosa, em vista disso, o próprio processo de envelhecimento natural gera alterações nas etapas de farmacocinética e farmacodinâmica dos fármacos trazendo, ao idoso, algumas reações adversas. Os medicamentos mais utilizados dentro dessa faixa etária são representados pela classe dos ansiolíticos e indutores do sono. O uso prolongado de benzodiazepínicos, cujo consumo alcança de 15% a 43% em adultos com idade de 65 anos ou mais, aumentam o risco de efeitos colaterais, tais como, dependência, sedação, comprometimento cognitivo e psicomotor e quedas. Já os indutores do sono representados, principalmente, pelos antidepressivos tricíclicos, podem gerar efeitos de sedação, hipotensão postural, constipação e demência nos idosos, devido a sua alta ação anticolinérgica. Considerações finais: Evidencia-se que medicamentos como, ansiolíticos e indutores do sono, devem ser prescritos com cautela pelos médicos, visando minimizar efeitos adversos que podem ser causados por esses fármacos quando utilizados a longo prazo.