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Neurology India, Vol. 58, No. 6, November-December, 2010, pp. 939-941 Case Report Skeletal muscle involvement in human immunodeficiency virus infection: A report of four cases Shashikala A Sangle, Abhijit Dasgupta, Shripad D Ratnalikar, Rahul V Kulkarni Department of Medicine, Byramjee Jeejeebhoy Medical College, Pune, India Date of Acceptance: 27-Aug-2010 Code Number: ni10261 PMID: 21150063 Abstract Human immunodeficiency virus (HIV) may affect any part of the neuraxis and may affect skeletal muscle in many ways, ranging from myofiber atrophy in the wasting syndrome to inflammatory muscle disease and a host of opportunistic infections involving muscle. We report here a case series of 4 zidovudine-naοve patients with proven HIV infection with myopathy. One was a case of HIV wasting syndrome, and the three others were diagnosed as HIV polymyositis. Muscle biopsy proved invaluable in the characterization of these casesKeywords: Atrophy, HIV, HIV wasting syndrome, inflammation, muscle biopsy, polymyositis Introduction HIV-related skeletal muscle diseases may present as a broad clinicopathological spectrum. [1] The repertoire comprises type II myofiber atrophy in the HIV wasting syndrome; inflammation in HIV polymyositis, inclusion body myositis and nemaline rod myopathy; various pyogenic, mycobacterial and parasitic opportunistic infections; and myoglobinuria. Besides, zidovudine-induced myopathy, diffuse infiltrative lymphocytosis syndrome and local neoplasms involving skeletal muscle are distinct clinicopathological entities. We report a series of four cases of retroviral myopathy, all zidovudine naοve, three of them being HIV polymyositis and 1 being a case of HIV wasting syndrome. Case Reports Case 1 A 40-year-old woman presented with complaints of difficulty getting up from squatting position, difficulty lifting arms overhead, pain in thighs and fever on and off since 3 months. She was emaciated and had oral candidiasis. There was weakness, grade 3 in the proximal muscles of lower limbs and grade 4 power in the shoulder girdle muscles bilaterally. Reflexes were normal. Muscles were tender. Creatine phosphokinase (CPK) was 1310 IU/L. CD4 count was 32/mm 3 . Electromyography (EMG) was myopathic, and muscle biopsy suggested myositis. She was started on highly active antiretroviral therapy (HAART) but died of a severe bout of acute diarrhea. Case 2 A 40-year-old man on anti-tuberculosis therapy presented with a complaint of difficulty getting up from squatting position since 3 weeks. He had proximal (grade 3) as well as a mild distal weakness (grade 4) limited to his lower limbs. There was mild decrease in sensation over his feet bilaterally. Thigh muscles were tender. CPK was 72 IU/L and CD4 count was 131/mm 3 . Nerve conduction studies revealed mild sensorimotor neuropathy. EMG was myopathic, and muscle biopsy showed features of inflammation. He was started on prednisolone at a dose of 0.5 mg/kg/d and referred for HAART but was lost to follow-up. Case 3 A 35-year-old man presented with a complaint of difficulty getting up from squatting position since 3 months. Proximal weakness in limbs, more in the lower limb than in the upper, was noted, as were multi-dermatomal herpes zoster scars and oral candidiasis. Tone and reflexes were normal. CPK was 671 IU/L and CD4 count was 35/mm 3 . EMG was myopathic, and muscle biopsy was suggestive of myositis. Along with d4T-based HAART, prednisolone was started at a dose of 0.5 mg/kg/d. He regained complete power within 3 months. Case 4 A 13-year-old boy, with both parents having succumbed to HIV, presented with history of chronic diarrhea, loss of weight and loss of appetite since 6 months. He was grossly emaciated, with body mass index of 14.5 kg/m 2 . Tone was reduced in all muscle groups. There was a predominant proximal weakness in all limbs. Reflexes were all depressed. CD4 count was 49/mm 3 and CPK was 75 IU/L. EMG was myopathic. Muscle biopsy showed atrophy of muscle fibers with fatty infiltration of muscle. The patient was started on nutritious diet, vitamin supplementation and antiretroviral therapy (ART). There was no clinical improvement, and he died 4 months later. Electromyography Electromyography in all cases showed small-amplitude polyphasic potentials with complete interference. No features of muscle hyper-irritability were seen. Muscle biopsy findings Cases 1, 2 and 3 showed features of muscle fiber necrosis with interspersed endomysial lymphocytic infiltrates, suggestive of polymyositis [[Figure - 1], representative photomicrograph of case 1]. Case 4 demonstrated severe myofiber atrophy with abundant fatty infiltration. Discussion The first report of an HIV-associated inflammatory myopathy was published by Dalakas et al. in 1986. [2] Their patients developed AIDS-related complex soon after. HIV myositis may occur early in the disease, in the stage of full-blown AIDS, [2] or be a manifestation of immune reconstitution after starting HAART. [3] Correlating viral load with various patterns of HIV myopathy would be interesting but was not logistically possible in our cases. The disease usually presents as a subacute proximal weakness with or without myalgia.CPK may be raised to 10 to 15 times the normal value. EMG shows a myopathic pattern, often with features of muscle hyper-irritability. Overall, there may be little to distinguish the process from acquired polymyositis. [4] Muscle biopsy reveals a perivascular, perimysial or endomysial mononuclear infiltrate, as well as necrotic and degenerating muscle fibers. [2] Nemaline rods have been documented in some cases. [5] HIV myopathy may present with rhabdomyolysis with myoglobinuria. [6] In HIV wasting syndrome, there is a preferential atrophy of type II myocytes. Mycobacterial and other opportunistic infections may occur. In zidovudine-induced myopathy, mitochondrial dysfunction is the postulated etiology, and the histological hallmark is the demonstration of "ragged red fibers" on muscle biopsy. [7] When HAART is begun, immune reconstitution inflammatory syndrome (IRIS)-related myositis remains another concern. Interesting insights have now been gained into the pathogenesis of HIV myositis. It was found that, HIV sequences or transcriptional products were not present within the muscle fibers or the cultured myotubes of patients with HIV myositis, thus indicating that viral replication does not take place within the muscle. HIV myositis is now considered to be caused by infection of the lymphoid cells, rather than direct infection of muscle fibers by the virus. [8] A previous study of biopsy-proven HIV myositis has demonstrated no correlation of the severity of weakness or the stage of HIV infection with the CPK values at diagnosis. HIV myositis responded well in most cases to treatment with corticosteroids and immunosuppressive agents. [9] In our series, all cases occurred in HAART-naοve patients with AIDS. Due to the poor correlation between clinical findings and CPK values, as exemplified chiefly by cases 2 and 4, HIV polymyositis and the myopathy of HIV wasting syndrome could be reliably distinguished by muscle biopsy alone. This distinction is clinically significant as some cases of HIV polymyositis respond reasonably well to treatment. It would be ideal, therefore, that muscle biopsy be performed in all cases of HIV myopathy, especially when features of hyper-irritability are not evident on EMG. We conclude that HIV myopathy may produce a wide variety of clinicopathological manifestations, necessitating proper characterization by all available means, including muscle biopsy. Moreover, HIV should be increasingly included in the differential diagnosis of patients presenting with features of inflammatory myositis. References
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