Pure neural leprosy or amyloid neuropathy? Systematic review and clinical case report

Abstract

OBJECTIVE:To review the literature and to report a clinical case with initial suspicion of pure neural leprosy and final diagnosis of amyloid neuropathy. METHODS:The study was conducted in two stages. In stage one, a systematic literature review was carried out, with searches performed in the PubMed, Medline, and Lilacs databases, as well as in the leprosy sectoral library of the Virtual Health Library, using the following descriptors: neuritic leprosy, pure neural leprosy, primary neural leprosy, pure neuritic leprosy, amyloid polyneuropathy, amyloid neuropathies, and amyloid polyneuropathy. The search was carried out on May 28, 2020. Clinical trials, cohort studies, crosssectional studies, clinical cases, and case studies published in Portuguese, English or Spanish between 2010 and 2020 were included.Stage two reports a case with initial suspicion of pure neural leprosy. Laboratory tests, electroneuromyography, ultrasound, and biopsy of the sural nerve were requested.RESULTS: Twenty-three scientific texts were included. No publications were found that contained both topics together. The challenging diagnosis of pure neural leprosy and the possibility of using auxiliary resources in diagnosis were the most emphasized themes in the studies. In the clinical case, the patient's electroneuromyography showed sensitive and motor polyneuropathy of the lower limbs, which was predominantly sensory and axonal, symmetrical, of moderate intensity, and the mixed type (axonal-demyelinating). Ultrasonography of the sural nerve revealed changes in the contour of the deep fibular nerves; biopsy of the sural nerve showed an accumulation of amorphous eosinophilic material in the nerve path, and Congo red stain showed apple-green birefringence of the deposit under polarized light. The final diagnosis was amyloid neuropathy. CONCLUSIONS:The final clinical diagnosis was amyloid neuropathy. The diagnosis of pure neural leprosy in endemic areas in Brasil is still a challenge for the health system.

Caracterização de casos e indicadores epidemiológicos e operacionais da hanseníase: análise de séries temporais e distribuição espacial, Piauí, 2007-2021

“…17 O diagnóstico atrasado e o tratamento incorreto possibilitam o estabelecimento da polineuropatia hansênica, que compromete os nervos periféricos, com perda da capacidade de contração muscular e lesões de pele. 18 Como a doença afeta as pessoas em idade economicamente ativa, a incapacidade resulta em perdas financeiras, especialmente quando há impossibilidade de trabalho, impedindo a permanência nos empregos ou ocupações e causando problemas de reintegração no mercado de trabalho, marginalização na cadeia produtiva, problemas psicológicos com o isolamento social e, portanto, perda considerável da qualidade de vida. Consequentemente, há aumento dos gastos públicos com a assistência médica e os serviços sociais para esses indivíduos.…”

Characterization of cases and epidemiological and operational indicators of leprosy: analysis of time series and spatial distribution, Piauí state, Brazil, 2007-2021

“…17 Late diagnosis and incorrect treatment can lead to polyneuropathy caused by leprosy, which compromises peripheral nerves with loss of muscle contraction capacity and skin lesions. 18 As the disease affects people of economically active age, disability results in financial losses, especially when it is impossible to work, preventing them from remaining in jobs or occupations and causing problems with reintegration into the job market, marginalization in the production chain, psychological problems caused by social isolation and, therefore, considerable loss of quality of life. Consequently, there is an increase in public spending on medical care and social services for these people.…”

Lepromatous leprosy with atypical clinical presentation in an adolescent from an endemic area of Brazil - a clinical challenge

“…Electroneuromyography, ultrasound, or magnetic resonance imaging of neural trunks can be useful in diagnosing neural forms. 2,4,9,10 Multibacillary leprosy occurs in the lepromatous and borderline forms. 1,10 The latter mainly presents erythematous-violaceous plaques.…”

“…It can mimic several pathologies, including pityriasis versicolor, vitiligo, syphilis, peripheral neuropathies, and rheumatoid arthritis. 1,2 Brazil is considered endemic for leprosy according to data from the Brazilian Ministry of Health. 3 In 2019, 23,612 new cases were diagnosed, 5.6% in children under 15 years of age.…”