|Year : 2006 | Volume
| Issue : 2 | Page : 119-121
Neuromyositis in Hansen's disease
JB Agadi1, Anita Mahadevan2, N Gayathri2, SK Shankar2
1 Department of Neurology, Bowring and Lady Curzon Hospitals, Bangalore Medical College, Bangalore - 560 029, India
2 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore - 560 029, India
J B Agadi
Department of Neurology, Bowring and Lady Curzon Hospitals, Bangalore Medical College, Fort, Near City Market, Bangalore - 560 029
Source of Support: None, Conflict of Interest: None
Muscle involvement in leprosy is rare, but not unknown. We report an interesting case of a 59 year old gentleman who presented with icthyosis, arthralgias, fever and peripheral neuropathy, mimicking a collagen vascular disease. Histological evaluation of superficial peroneal nerve and peroneus brevis revealed unusual dual involvement by Hansen's disease, with numerous lepra bacilli in both. Involvement of skeletal muscle by Mycobacterium is very rare. The clinical presentation and treatment details of the case with a brief review of literature, is presented.
Keywords: Collagen vascular disease, denervation, leprous neuritis, leprous myositis
|How to cite this article:|
Agadi J B, Mahadevan A, Gayathri N, Shankar S K. Neuromyositis in Hansen's disease. Ann Indian Acad Neurol 2006;9:119-21
| Introduction|| |
Leprosy (Hansen's disease) is a chronic infectious disease caused by Mycobacterium leprae , a microorganism to which only a small portion of any given population is susceptible. Although M. leprae can be found nearly anywhere in the body outside of the central nervous system, even in the more disseminated forms of Hansen's, there is significant damage only in superficial nerves, the skin, the anterior third of the eye, the upper respiratory tract and testes. We report a rare instance of skeletal muscle involvement along with peripheral neuritis in a 59 year old gentleman, clinically diagnosed to have collagen vascular disease.
| Case Report|| |
This fifty nine year old male came to neurological services with a history of burning sensation in both lower limbs of eight months duration, weakness of proximal and distal muscles of both lower limbs and increased pigmentation of hands and feet, of six months duration. He had been evaluated for complaints of fever with arthralgias, three months prior to his presentation to us, for which no cause was found. He had undergone coronary artery bypass graft in March 2003 for ischemic heart disease and was on clopidogrel and aspirin. His daughter was suffering from rheumatoid arthritis. He had consulted dermatologists for his skin problem without much benefit.
On examination, he had generalized icthyosis. Skin over both hands and feet was hyper pigmented and scaly. Higher mental functions and cranial nerves were normal. He had mild bilateral wasting of hypothenar muscles. There were no fasciculations and the muscle tone was normal in all four limbs. Power in the upper limbs was normal (Gr IV), while in the lower limbs, mild weakness was noted in the proximal muscles and dorsiflexors of the feet. Deep tendon reflexes were elicitable and the plantar response was flexor bilaterally. All modalities of sensation were impaired below the mid calf bilaterally. Vibration and the joint position sense was impaired distally below ankle joint bilaterally. Peripheral nerves were not thickened. Other system examination was normal.
Hemogram revealed mild anemia and raised ESR (90 mm/hr, Westergren). Blood biochemistry including sugars, blood urea, creatinine LFT, electrophoresis, B12 and folic acid levels and serum CPK were within normal limits. He had high titres of RA factor and increased C reactive protein, but ANA was negative. Serum was non reactive for HBsAg and HIV 1 and 2. CSF examination was normal.
Nerve conduction studies showed features of a combination of demyelinating and axonal sensorimotor peripheral neuropathy. There was severe axonopathy of both sural nerves and moderate demyelination with axonopathy involving left posterior tibial and bilateral common peroneal nerves. Demyelination and axonopathy of a milder degree was seen in the upper limbs, involving left ulnar motor and sensory, right ulnar and right median sensory conductions, with mild left median sensory axonopathy. EMG studies were not carried out.
With a clinical diagnosis of collagen vascular disease, a left superficial peroneal nerve and peroneus brevis muscle biopsy was performed to establish diagnosis.
The nerve biopsy had 6-8 expanded fascicle enclosed within a markedly thickened, vascularised and inflamed perineurium infiltrated by lymphocytes, plasma cells and numerous foam cells. The endoneurial contents were replaced by sheets of foamy histiocytes that surrounded endoneurial vessels, while epitheloid granulomas with Langhan's giant cells were seen subperineurially [Figure - 1]B. The inflammatory infiltrate extended into the epineurium, to surround arterioles and venules. Extensive loss of myelinated fibers was noted in some fascicles, while others showed pockets of surviving large diameter fibers and selective loss of small fibers. Lepra stains revealed numerous globi of acid fast bacilli within the foam cells [Figure - 1]B, inset, endothelial cells in endoneurial and epineurial vessels and occasional perineurial cells. Simultaneous presence of both lepromatous and tuberculoid features, suggests a transitional stage with upgrading/downgrading lepra reaction of Hansen's disease.
Muscle biopsy included the epimysial connective tissue. Within the epimysium, a leash of hypertrophied nerve twigs expanded by large epitheloid granulomas was found. The inflammatory infiltrates extended into the muscle along the interstitial connective tissue, splaying apart the myofibres [Figure - 1]A. The muscle essentially appeared normal, but for a few hyalinised and hypertrophied fibres, foci of necrosis, with myophagocytosis and occasional regenerating fibres. Collection of foam cells and lymphocytes were seen around both necrotic and non necrotic fibres [Figure - 1]C and in the perivascular regions involving several vessels in epi and perimysium. Lepra bacilli were found in clumps within foamy macrophages [Figure - 1]D and perivascularly in epimysium. The lepra bacilli in addition, could be detected within relatively well preserved skeletal muscle fibres and in subsarcolemmal satellite cells.
The patient is currently on multidrug regime for Hansen's disease and has relief from symptoms of sensory dysesthesias.
| Discussion|| |
Inflammatory myopathies constitute a heterogenous group of subacute, chronic and rarely acute acquired diseases of skeletal muscle, which have in common, the presence of moderate to severe muscle weakness and presence of inflammation on muscle biopsy. Secondary inflammatory myopathy can occur in association with systemic or connective tissue disease or with bacterial, viral or parasitic infections.
Involvement of skeletal muscle in leprosy is extremely rare, though examples of leprous myositis have been recorded in the literature.,,,, Earlier workers have studied involvement of skeletal muscle in leprosy, both by electromyography, and muscle biopsy.,,,, Sibelle and co-workers investigating for the presence of early muscle changes in lepromatous leprosy by simultaneous electromyography and muscle biopsy, found evidence of denervation in 10 of 13 (76.9%) subjects tested, inflammation in the connective tissue of the muscle in 8 cases and presence of acid fast bacilli in five. Three cases showed fascicular atrophy. The authors concluded that while EMG was sensitive in detecting early changes of denervation, muscle biopsy was essential to demonstrate presence of leprous myositis.
Denervation on electrophysiology was also demonstrated by Werneck and colleagues. Of 40 cases of leprosy studied (23 lepromatous, 13 tuberculoid, borderline 2, indeterminate 2), electrophysiological evidence of denervation in anterior tibial muscles was recorded in 77.5% of cases. Biopsy from the same muscles showed denervation in 45%, inflammation in 12.5% and acid fast bacilli in 24% of cases.
Histologically, the skeletal muscle biopsies studied in Hansen's disease revealed presence of inflammation without distinct signs of myofibre degeneration. Inflammation is often localized within the interstitial connective tissue,,, as collections of foamy histiocytes (Virchow cells), at times, forming "lepromas". Infiltration of intramysial nerve twigs was also noted as observed in our case. Presence of granulomas in the muscle in cases of tuberculoid leprosy has rarely been found.
Detection of acid fast bacilli in muscle biopsies from cases of Hansen's, ranges from 25-54% in different series. Frequency of detection of acid fast bacilli was not dependant on the histological type of Hansen's being demonstrable both in the tuberculoid and lepromatous forms. Koranne and colleagues examining 24 cases of tuberculoid leprosy, noted that presence of lepra bacilli had no relation to proximity of the muscle biopsied to cutaneous or neural pathology, as muscle biopsies not underlying cutaneous lesions also had acid fast bacilli. The presence of acid fast bacilli was relatively constant within the interstitium, though occasional workers reported bacillary presence within undamaged muscle., The muscle fibres themselves rarely showed signs of sarcolemmal degeneration or necrosis, except on ultrastructure and more often, in cases of lepromatous leprosy.
Striated muscle involvement in cases of leprosy has been considered secondary to peripheral neuropathy. In our case, the granulomatous pathology involving the nerve twigs in the epimysium, appeared to extend into the underlying muscle along intramuscular nerve twigs. The common finding of denervation on electrophysiology and histology of muscle, also support this contention. However, Koranne and colleagues found simultaneous involvement of liver and lymph nodes in 21% of cases that showed muscle involvement, suggesting that muscle involvement in leprosy maybe consequent to systemic spread of bacilli.
In our case, the presence of fever, arthralgias and peripheral neuropathy mimicked a vasculitic neuropathy. It was only the nerve and muscle biopsy that helped establish the etiology of neuropathy. The presentation with fever and joint pains heralded a lepra reaction. The presence of icthyosis in this case was probably co-incidental and unrelated to the original disease process. The muscle involvement, though not symptomatic, would have been missed if nerve biopsy alone had been performed. Subclinical muscle involvement in Hansen's disease is therefore not rare and its presence does not appear to influence prognosis.
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