Pure neural leprosy—mind the diagnosis

Brandsma, Wim; Post, Erik; Wagenaar, Inge; Alam, Khorshed; Shetty, Vanaja; Husain, S; Prakoeswa, Cita Rosita Sigit; Shah, Mahesh; Tamang, Krishna Bahadur

doi:10.47276/lr.92.1.38

Cited by 5 publications

(5 citation statements)

References 20 publications

(37 reference statements)

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“…This paper reports on four young adults diagnosed with Hirayama Disease, a relatively rare neurological condition in endemic leprosy countries that could be confused with neural leprosy. [2][3][4]25,26 The clinical features of this disease are very typical. When there is adequate knowledge of the nerve involvement pattern in leprosy, including possible findings on nerve palpation, the condition should not be confused with leprosy.…”

Section: Discussionmentioning

confidence: 99%

“…In a recent article Brandsma et al reported 76 (8.8%) patients with Pure Neural Leprosy (PNL) in a cohort of 868 leprosy patients, while in a second cohort, the figure was 7.5% (N 28/372). 2 Correspondence to: Wim Brandsma (e-mail: jwbrandsma@gmail.com) Kumar reported 4.2% in 1542 patients, and in a recent review article Kumar reported a range of 4-8% in India. 3,4 Van Brakel reported 8.7% of patients with PNL in a cohort of 703 newly diagnosed patients in Nepal.…”

Section: Introductionmentioning

confidence: 99%

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Hirayama Disease: the importance of the differential diagnosis in suspected neural leprosy

Brandsma

Maharjan

Sharma

et al. 2023

LEPROSY

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Pure neural leprosy, leprosy without skin manifestations, is common in leprosy. Patients with muscle weakness or paralysis without the classical leprosy cardinal signs, such as the typical skin lesions and skin smear positivity, are sometimes erroneously diagnosed as having neural leprosy. Sound knowledge of the innervation pattern of nerves that can become involved in leprosy can help differentiate pure neural leprosy from other neuromuscular conditions. Simple clinical testing procedures can aid in the differential diagnosis. When in doubt of a diagnosis, additional testing procedures can confirm or reject a diagnosis of neural leprosy. Hirayama Disease is a relatively rare disease of the lower cervical spine which presents mainly as paralysis of the forearm and hand muscles. We report four patients with Hirayama Disease referred to and diagnosed at one leprosy referral center. One patient was treated for leprosy before the diagnosis of Hirayama Disease. Clinical differentiation between the two neural diseases is relatively easy.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Hirayama Disease: the importance of the differential diagnosis in suspected neural leprosy

Brandsma

Maharjan

Sharma

et al. 2023

LEPROSY

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“…It is usually distal and symmetrical with proprioception, light touch, temperature, and pain sensory abnormalities without muscle weakness [ 17 , 19 , 21 ]. Tendon reflexes can be lost, reduced, or preserved, and electroneurography may show both demyelinating and axonal involvement or may be normal, thus suggesting the involvement of small fibers [ 17 , 19 , 20 , 21 ].…”

Section: Discussionmentioning

confidence: 99%

“…The most common presentations are mononeuropathy (79%) and multiple mononeuropathy (10.5%) [ 17 , 18 , 19 ]. A polyneuropathic form (P-PNL) is rarely reported in the literature, and diagnosis is often missed, especially in Western countries, where leprosy is not an endemic disease [ 20 , 21 , 22 , 23 , 24 ].…”

Section: Introductionmentioning

confidence: 99%

Leprosy Neuropathy in a Non-Endemic Area: A Clinical and Pathological Study

Cotti Piccinelli,

Tagliapietra,

Cavallaro

et al. 2023

Biomedicines

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The extent of nerve involvement in leprosy is highly variable in distribution and clinical presentation. Mononeuropathies, multiple mononeuropathies, and polyneuropathies can present both in the context of a cutaneous and/or systemic picture and in the form of pure neuritic leprosy (PNL). The differential diagnosis of leprosy neuropathy remains challenging because it is a very rare condition and, especially in Western countries, is often overlooked. We report one case of the polyneuropathic form of PNL (P-PNL) and one case of multiple mononeuropathy in paucibacillary leprosy. In both cases, the diagnosis was achieved by performing a sural nerve biopsy, which showed subverted structure, severe infiltration of inflammatory cells in nerve fascicles, granulomatous abnormalities, and the presence of alcohol-acid-resistant, Ziehl–Neelsen-positive bacilli inside the nerve bundles. Leprosy remains an endemic disease in many areas of the world, and globalization has led to the spread of cases in previously disease-free countries. In this perspective, our report emphasizes that the diagnostic possibility of leprosy neuropathy should always be taken into account, even in Western countries, in the differential diagnostic process of an acquired sensory polyneuropathy or multineuropathy and confirms that nerve biopsy remains a useful procedure in working up neuropathies with unknown etiology.

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“…

Sir,We read with interest the article by Brandsma et al entitled 'Pure neural leprosy-mind the diagnosis'. 1 The authors describe the demographics, prevalence, and nerve involvement in pure neural leprosy (PNL) from a large cohort of patients recruited originally in the TENLEP study. 2 The study has confirmed once again the existence of this subset of leprosy (PNL).

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confidence: 99%

Pure neural leprosy: a diagnostic conundrum

Narang

Mehta

Dogra

et al. 2021

LEPROSY

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Sir,We read with interest the article by Brandsma et al. entitled 'Pure neural leprosy-mind the diagnosis'. 1 The authors describe the demographics, prevalence, and nerve involvement in pure neural leprosy (PNL) from a large cohort of patients recruited originally in the TENLEP study. 2 The study has confirmed once again the existence of this subset of leprosy (PNL). Beyond that the situation is not as foggy as it has been made out to be and presently a lot more is known about the diagnosis and management of PNL.The authors have proposed a new definition for clinical diagnosis of PNL 'enlarged and/or tender peripheral (incl. cutaneous) nerves with or without sensory and/or motor impairment in the distribution area of the nerves commonly involved in leprosy in the absence of typical skin lesions for leprosy and a negative skin smear'. As per this definition, enlarged nerves alone are sufficient to establish the diagnosis of PNL. This is in contrast to the second cardinal sign of leprosy as per the WHO expert committee, which requires 'thickened or enlarged peripheral nerve with loss of sensation and/or weakness of the muscles supplied by that nerve'. 3 Nerve thickening is merely a soft-pointer towards the diagnosis of PNL, the inter-observer reliability and specificity of nerve palpation has been shown to be poor in several studies. [4][5][6] A study from India found only moderate reproducibility among eight experienced paramedical workers. 5 The specificity of ulnar and common peroneal nerve palpation in detecting neural involvement has been reported to be only 34% and 40%, respectively. 6 A palpable nerve is very likely to be recorded as a thickened nerve, especially by less experienced observers. False positive findings may occur because of poor examination technique or because of nonspecific enlargement of a nerve seen in some heavy manual workers. Additionally, several other diseases may present with nerve thickening, such as diabetes mellitus, Refsum's disease,

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Pure neural leprosy—mind the diagnosis

Cited by 5 publications

References 20 publications

Hirayama Disease: the importance of the differential diagnosis in suspected neural leprosy

Hirayama Disease: the importance of the differential diagnosis in suspected neural leprosy

Leprosy Neuropathy in a Non-Endemic Area: A Clinical and Pathological Study

Pure neural leprosy: a diagnostic conundrum

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