The armadillo: a model for neuropathy of leprosy and potentially other neurodegenerative diseases

Sharma, Rahul; Lahiri, Ramanuj; Scollard, David M.; Peña, María; Williams, Diana L.; Adams, Linda B.; Figarola, John H.; Truman, Richard W.

doi:10.1242/dmm.010215

Cited by 71 publications

(52 citation statements)

References 39 publications

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“…Apesar de o agente etiológico, Mycobacterium leprae, ter sido identificado há mais de um século, pouco se sabe sobre o mecanismo patofisiológico subjacente à lesão do nervo. 5 A principal limitação na investigação nesta área surge na obtenção de amostras de tecido para estudo, fato agravado por se tratar de uma doença multifocal e com largas áreas poupadas de lesão. 5 A infeção pelo Mycobacterium leprae lesiona fibras mielinizadas e não mielinizadas.…”

Section: Discussionunclassified

“…5 A principal limitação na investigação nesta área surge na obtenção de amostras de tecido para estudo, fato agravado por se tratar de uma doença multifocal e com largas áreas poupadas de lesão. 5 A infeção pelo Mycobacterium leprae lesiona fibras mielinizadas e não mielinizadas. De forma sumária pode dizer-se que, no processo de infeção e subsequente destruição nervosa, o bacilo invade o nervo, resultando numa resposta imunológica promovida pelas células de Schwann com desmielinização, degenerescência axonal e posterior regeneração com fibrose tornando os nervos espessados e por isso frequentemente palpáveis.…”

Section: Discussionunclassified

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Neuropatia da Doença de Hansen: Um Diagnóstico a Considerar na Investigação da Neuropatia Periférica

et al. 2015

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Introduction: Leprosy is still one of the most frequent causes of peripheral neuropathy. Although regarded as eradicated in Portugal, is still documented in neuropathological study of patients with clinical peripheral neuropathy without proper diagnosis. Material and Methods: Review of the cases of Hansen disease neuropathy diagnosed in Neuropathology Unit of Centro Hospitalar do Porto between 1978 and 2013, atending to gender, age, clinical manifestations and neuropathological findings. Results: Twenty one patients were identified with neuropathological diagnosis of Hansen’s disease neuropathy, predominantly male. The mean age at diagnosis was 52 years, and sensory symptoms predominate as neurological manifestation of disease. Interval between symptoms and diagnosis was 1-38 years. In most nerve samples tuberculoid type of disease was identified. Bacilli were detected in skin and nerve in 44% of cases. Discussion: Mononeuritis is the most common presentation of leprosy but other clinical manifestations are possible, including skin lesions. Infection with M. leprae injures myelinated and unmyelinated fibres, with replacement of nerve tissue by collagen fibrosis. The diagnosis of leprosy is only achieved by neuropathological study of skin lesions and / or peripheral nerve, supported by the identification of the bacillus. Conclusion: Hansen disease remains a public health problem in tropical areas and, although rare, still described in Western countries reason why should still be considered as a diagnostic possibility in the investigation of peripheral neuropathy.

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Section: Discussionunclassified

Neuropatia da Doença de Hansen: Um Diagnóstico a Considerar na Investigação da Neuropatia Periférica

et al. 2015

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“…Fascicular biopsy is thought to be contraindicated in the diagnosis of leprosy given the risk of damaging the parent nerve. 18,28 In our experience, fascicular biopsy of some proximal nerves such as the sciatic nerve is well tolerated, and based on the established practice of ulnar nerve fascicular transfer for brachial plexus reconstruction, we believed that the need for tissue diagnosis in this case outweighed the risk especially since the delayed treatment of ulnar nerve lesions is known to lead to permanent atrophy and contractures. 15 …”

Section: Fascicular Biopsymentioning

confidence: 90%

Pure neuritic leprosy presenting as ulnar nerve neuropathy: a case report of electrodiagnostic, radiographic, and histopathological findings

Payne¹,

Baccon²,

Dossett³

et al. 2015

JNS

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Hansen’s disease, or leprosy, is a chronic infectious disease with many manifestations. Though still a major health concern and leading cause of peripheral neuropathy in the developing world, it is rare in the United States, with only about 150 cases reported each year. Nevertheless, it is imperative that neurosurgeons consider it in the differential diagnosis of neuropathy. The causative organism is Mycobacterium leprae, which infects and damages Schwann cells in the peripheral nervous system, leading first to sensory and then to motor deficits. A rare presentation of Hansen’s disease is pure neuritic leprosy. It is characterized by nerve involvement without the characteristic cutaneous stigmata. The authors of this report describe a case of pure neuritic leprosy presenting as ulnar nerve neuropathy with corresponding radiographic, electrodiagnostic, and histopathological data. This 11-year-old, otherwise healthy male presented with progressive right-hand weakness and numbness with no cutaneous abnormalities. Physical examination and electrodiagnostic testing revealed findings consistent with a severe ulnar neuropathy at the elbow. Magnetic resonance imaging revealed diffuse thickening and enhancement of the ulnar nerve and narrowing at the cubital tunnel. The patient underwent ulnar nerve decompression with biopsy. Pathology revealed acid-fast organisms within the nerve, which was pathognomonic for Hansen’s disease. He was started on antibiotic therapy, and on follow-up he had improved strength and sensation in the ulnar nerve distribution. Pure neuritic leprosy, though rare in the United States, should be considered in the differential diagnosis of those presenting with peripheral neuropathy and a history of travel to leprosy-endemic areas. The long incubation period of M. leprae, the ability of leprosy to mimic other conditions, and the low sensitivity of serological tests make clinical, electrodiagnostic, and radiographic evaluation necessary for diagnosis. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

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“…In 1971, Kirchheimer et al successfully cultured M. leprae in this exotic-looking house-cat-sized animal, which is present only on the American continents (209). Their low body temperature (32°C to 35°C) and long life span (12 to 15 years) make these animals preferred for propagating M. leprae (211). Dasypus novemcinctus is the only animal to develop regularly disseminated infections following intravenous inoculation; however, 30% of the infected animals do develop systematic infections.…”

Section: Animal Inoculationmentioning

confidence: 99%

“…Dasypus novemcinctus is the only animal to develop regularly disseminated infections following intravenous inoculation; however, 30% of the infected animals do develop systematic infections. The organs of the reticuloendothelial system are the most affected (208), with bacterial concentrations reaching 10 9 to 10 11 M. leprae bacteria/g (211). The growth of M. leprae is extremely slow, with a doubling time of 14 days.…”

Section: Animal Inoculationmentioning

confidence: 99%

Current and Past Strategies for Bacterial Culture in Clinical Microbiology

et al. 2015

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SUMMARY A pure bacterial culture remains essential for the study of its virulence, its antibiotic susceptibility, and its genome sequence in order to facilitate the understanding and treatment of caused diseases. The first culture conditions empirically varied incubation time, nutrients, atmosphere, and temperature; culture was then gradually abandoned in favor of molecular methods. The rebirth of culture in clinical microbiology was prompted by microbiologists specializing in intracellular bacteria. The shell vial procedure allowed the culture of new species of Rickettsia . The design of axenic media for growing fastidious bacteria such as Tropheryma whipplei and Coxiella burnetii and the ability of amoebal coculture to discover new bacteria constituted major advances. Strong efforts associating optimized culture media, detection methods, and a microaerophilic atmosphere allowed a dramatic decrease of the time of Mycobacterium tuberculosis culture. The use of a new versatile medium allowed an extension of the repertoire of archaea. Finally, to optimize the culture of anaerobes in routine bacteriology laboratories, the addition of antioxidants in culture media under an aerobic atmosphere allowed the growth of strictly anaerobic species. Nevertheless, among usual bacterial pathogens, the development of axenic media for the culture of Treponema pallidum or Mycobacterium leprae remains an important challenge that the patience and innovations of cultivators will enable them to overcome.

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The armadillo: a model for neuropathy of leprosy and potentially other neurodegenerative diseases

Cited by 71 publications

References 39 publications

Neuropatia da Doença de Hansen: Um Diagnóstico a Considerar na Investigação da Neuropatia Periférica

Neuropatia da Doença de Hansen: Um Diagnóstico a Considerar na Investigação da Neuropatia Periférica

Pure neuritic leprosy presenting as ulnar nerve neuropathy: a case report of electrodiagnostic, radiographic, and histopathological findings

Current and Past Strategies for Bacterial Culture in Clinical Microbiology

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