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  Table of Contents  
Year : 2016  |  Volume : 34  |  Issue : 1  |  Page : 95-97

Leprous neuromyositis: A rare clinical entity and review of the literature

1 Department of Medicine, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Neuropathology, Christian Medical College, Vellore, Tamil Nadu, India

Date of Submission23-Feb-2015
Date of Acceptance04-Jun-2015
Date of Web Publication15-Jan-2016

Correspondence Address:
Shubhanker Mitra
Department of Medicine, Christian Medical College, Vellore, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0255-0857.174120

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 ~ Abstract 

Mycobacterium leprae, the causative agent of leprosy (Hansen's disease), is a slow growing intracellular acid-fast bacillus that affects the skin, peripheral nerves and respiratory tract. In patients with suppressed cell-mediated immunity, the infiltration of the Bacilli can produce disseminated illness such as leprous neuromyositis. We reported a case of 56-year-old gentleman presenting with pyrexia of unknown origin, asymmetric sensory motor axonal polyneuropathy and was on chronic exogenous steroid therapy. On evaluation, his skin, muscle, nerve and bone marrow biopsy showed numerous globi of acid-fast Bacilli suggestive of leprous neuromyositis, a rare form of disseminated Hansen's disease. We reported this case in view of its rarity, atypical manifestation of a relatively rare disease and literature review on poor electrophysiological correlation in the diagnosis of leprous neuromyositis as compared to the histopathological examination.

Keywords: Chronic inflammatory demyelinating polyneuropathy, chronic sensory motor polyradiculoneuropathy, leprous neuromyositis

How to cite this article:
Mitra S, Gunasekaran K, Chacko G, Hansdak SG. Leprous neuromyositis: A rare clinical entity and review of the literature. Indian J Med Microbiol 2016;34:95-7

How to cite this URL:
Mitra S, Gunasekaran K, Chacko G, Hansdak SG. Leprous neuromyositis: A rare clinical entity and review of the literature. Indian J Med Microbiol [serial online] 2016 [cited 2020 Jun 5];34:95-7. Available from:

 ~ Introduction Top

Leprosy (Hansen's disease) is caused by Mycobacterium leprae. Skeletal muscle involvement is rare and first showed by Ishihara (“Myositis interstitialis leprosa”).[1] The first Indian work by Job et al. in 1969 showed that the involvement of striated muscle in leprosy is secondary to peripheral nerve involvement, but some studies point to a primary muscle lesion and others to systemic spread of Bacilli. The diagnostic specificity of electromyographic recording in leprous myositis is poor.[2]

With the decline in the incidence of leprosy, the disseminated M. leprae infection presenting as leprous neuromyositis is a rare clinical entity. We reported a patient who was treated with systemic glucocorticoid for chronic inflammatory demyelinating polyneuropathy (CIDP), made elsewhere based on electrophysiology studies alone. However, the histopathological examination of his skin, muscle, nerve and bone marrow showed M. leprae. Hence, he was diagnosed to have leprous neuromyositis.

 ~ Case Report Top

A 56-year-old gentleman from the state of Jharkhand, North-India presented with painful paraesthesia of bilateral lower limb which was slowly progressive over 7 years. He was evaluated by a local physician for the same; based on electrophysiology reports, he was diagnosed to have a CIDP and treated with long-term steroids. Six months prior to his presentation to our hospital, he developed rapid progression of motor weakness in bilateral feet and over 3 months progressed to proximal thigh muscle weakness. At the same time, he developed difficulty in lifting in the arms above the level of shoulders and tingling, numbness of his palms extending up to the elbow.

He was febrile, and general physical examination showed cushingoid body habitus, oral candidiasis, onycholysis and pallor. There were no palpable peripheral nerves or hypo-anaesthetic patches. Neurological examination revealed grade 2 power in the shoulder, elbow and forearm with a weak hand grip and grade 3 power in the hip, knee and plantar dorsiflexion. All deep tendon reflexes were absent in the lower limbs and upper limb. There was asymmetric loss of touch, pain and temperature sensations below the knees. There was no significant fall in the blood pressure with the postural change.

The differential diagnosis of infective (tuberculosis, Hansen's disease, melioidosis, histoplasmosis), inflammatory (sarcoidosis, amyloidosis, systemic vasculitis, cryoglobulinemia) and chronic lead poisoning causes were considered in the order of decreasing probability.

His hematologic investigations and chest radiograph were normal.

Nerve conduction and electromyography studies

It showed bilateral, asymmetric reduction of amplitudes, conduction velocity of compound muscle action potentials and sensory nerve action potentials in both upper and lower limbs suggestive of asymmetric demyelinating sensory motor demyelinating axonal polyneuropathy with lower limbs more involved than the upper limbs. Sympathetic skin response was absent in both upper and lower limbs suggestive of small fibre involvement.


His peripheral nerve biopsy, skeletal muscle and skin showed:

The endoneurial, perineurial and epineurial compartments had diffuse infiltrates of foamy histiocytes, lymphocytes and plasma cells with perivascular lymphohistiocytic infiltrates extending into the vessel walls [Figure 1]a. Fite Faraco stain showed numerous acid-fast Bacilli in globi [Figure 1]b.
Figure 1: (a) Nerve biopsy (H and E): Dense infiltrates of foamy histiocytes, lymphocytes and plasma cells in endoneurial (blue arrow), perineurial (purple arrow) and epineurial (black arrow) compartments and perivascular lymphohistiocytic infiltrates extending into the vessel walls (green arrow). (b) Nerve biopsy (Fite Faraco stain) - numerous acid-fast Bacilli (individually scattered as well as arranged in globi)

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The skeletal muscle biopsy showed perivascular and focal intramural lymphocytes and foamy histiocytes (splaying apart myofibres) involving perimysial and epimysial blood vessels Bacilli [Figure 2]a with numerous acid-fast Bacilli within globi and scattered [Figure 2]b. A review of the bone marrow trephine biopsy with a Fite Faraco stain showed the presence of numerous acid-fast Bacilli. The skin biopsy showed 4+ bacillary index for M. lepra Bacilli with perineurial and perivascular infiltrates of lymphocytes and sheets of foamy histiocytes [Figure 3]a and numerous globi of acid-fast Bacilli [Figure 3]b.
Figure 2: (a) Skeletal muscle biopsy (H and E, ×400) - perivascular (black arrow) and focal intramural infiltrates of lymphocytes and foamy histiocytes. Focal inflammatory cells within the endomysium splaying apart myofibres. (b) Skeletal muscle biopsy (×1000) numerous acid-fast Bacilli arranged in globi

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Figure 3: (a) Skin biopsy (H and E) - perivascular infiltrates of lymphocytes and sheets of foamy histiocytes (arrow). (b) Skin biopsy (Fite Faraco stain)- numerous acid-fast Bacilli (arrow)

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He was initiated on rifampicin 600 mg once a month for 12 months, clofazimine 300 mg once a month for 12 months, clofazimine 50 mg once daily for 12 months and dapsone 100 mg once a day for 12 months. At 2nd month follow-up, his fever subsided. There was increase in appetite and weight gain with improvement in his motor power in all limbs.

 ~ Discussion Top

Since the introduction of multi-bacillary treatment regimen in 1982, the national case detection rate has shown a declining trend. In 2012, World Health Organisation ranked the highest number of new case detection in India (more than 1.2 lakh cases) and contributed 58% of the global leprosy burden.[3] The involvement of skeletal muscle, facilitated by depressed cell-mediated immunity in leprosy, presenting as leprous neuromyositis is rare. This is caused by infiltration of the interstitium and muscle cells by the chronic inflammatory cells (foamy macrophages, plasma cells and histiocytes) containing the Bacilli.[2],[4],[5],[6]

Gupta et al. showed 34% of all cases of leprosy showed histological changes in muscle biopsies constituted the most common pathological lesion.[4] Striated muscle involvement in cases of leprosy has been considered as secondary to peripheral neuropathy.

Striated muscle involvement in cases of leprosy has been considered by some to be secondary to peripheral neuropathy.[2] However, Job et al. observed the presence of inflammation around blood vessels in the muscle and felt that this supported the view that bacteria reached the muscle via the blood stream. In their study on 24 untreated cases of proven tuberculoid leprosy, Koranne et al. found that 19 cases had lepra Bacilli in skeletal muscle.[5] They found simultaneous involvement of liver and lymph nodes in 21% of cases that showed muscle involvement, suggesting that muscle involvement in leprosy may be consequent to systemic spread of Bacilli.[7] In the present case, the inflammatory infiltrates and Bacilli were found primarily in relation to blood vessels as well as in the bone marrow, thus supporting the view that Bacilli reached these tissues through a haematogenous route.

Thus, from the discussion, it is clear that electrophysiological changes of chronic denervation pattern is more prevalent in early leprosy, and the leprous myositis is relatively less frequent than leprous interstitial myositis.

There are two main learning points from this case. Firstly, the diagnostic performance of electromyographic abnormality in lepromatous neuromyositis is of questionable correlation. This patient is a resident of high endemic area of leprosy; hence, the index of suspicion of Hansen's disease is high. The use of systemic glucocorticoid masked his clinical progression and led to widespread dissemination of the M. leprae to muscle and bone marrow.

Secondly, this patient presented with a rare clinical syndrome of leprous neuromyositis. This patient did not present with the classical neurocutaneous manifestations of leprosy but rare clinical syndrome of leprous neuromyositis. Therefore, the clinicians should have high index of suspicion and bear the epidemiological background of the patient while interpreting the laboratory diagnosis.

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Conflicts of interest

There are no conflicts of interest.

 ~ References Top

Ishihara S. A study of myositis interstitialis leprosa. Int J Lepr 1959;27:341-6.  Back to cited text no. 1
Werneck LC, Teive HA, Scola RH. Muscle involvement in leprosy. Study of the anterior tibial muscle in 40 patients. Arq Neuropsiquiatr 1999;57:723-34.  Back to cited text no. 2
WHO|24 August. Vol. 87, 34, p. 317-28. WHO; 2012. Available from: [Last cited on 2015 Jun 06].  Back to cited text no. 3
Gupta JC, Jesupadam T, Gupta MC, Gupta DK. A histopathologic study of striated muscle biopsies in leprosy. Int J Lepr Other Mycobact Dis 1975;43:348-55.  Back to cited text no. 4
Koranne RV, Singh R, Iyengar B. Mycobacterium leprae in the striated muscle of tuberculoid leprosy patients. Lepr India 1978;50:375-80.  Back to cited text no. 5
Agadi J, Mahadevan A, Gayathri N, Shankar S. Neuromyositis in Hansen′s disease. Ann Indian Acad Neurol 2006;9:119.  Back to cited text no. 6
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Raval SN, Sengupta U, Ramu G, Prabhune PV, Desikan KV. A study of continuous bacillaemia in borderline and lepromatous type of leprosy. Lepr India 1982;54:623-33.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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