Inclusion body myositis (IBM)
Gayathri, N.; Anisya-Vasanth; Veerendra Kumar, M.; Das, S.; Santosh, V.; Yasha, T.C.; Ramamohan, Y.; Taly, A.B.; Gourie-Devi, M.; Shankar, S.K.
Clinical Neuropathology 19(1): 13-20
2000
ISSN/ISBN: 0722-5091 PMID: 10774946 Document Number: 517077
Clinical, histological, immunohistochemical and ultrastructural features of 5 cases of inclusion body myositis-4 sporadic (s-IBM) and one hereditary (h-IBM) form are described. These patients (3 men, 2 women) had chronic progressive weakness of varying severity in all 4 extremeties with sparing of cranial muscles. Elevation of CPK was noted in 2 patients. Electromyography revealed features of myopathy in 4 and additional neurogenic changes in 2 subjects. Clinical diagnosis was often other than inclusion body myositis. Presence of characteristic eosinophilic inclusions within the vacuoles established the diagnosis. The inclusions were congophilic and showed positivity to ubiquitin, beta-amyloid and SMI-31 in the sporadic cases while congophila was absent in the hereditary form. Immunostaining to hyperphosphorylated-tau was negative in both s-IBM and h-IBM. Membraneous whorls were observed at ultrastructural level. None of the patients improved with steroids and trial with other immunosuppressants was unsuccessful.