Inclusion body myositis (IBM).
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 extremities 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.
Gayathri
N
N
Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India.
Anisya-Vasanth
Veerendra Kumar
M
M
Das
S
S
Santosh
V
V
Yasha
T C
TC
Ramamohan
Y
Y
Taly
A B
AB
Gourie-Devi
M
M
Shankar
S K
SK
D000328:Adult; D003402:Creatine Kinase / Q000097:blood; D003937:Diagnosis, Differential; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D018908:Muscle Weakness / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D018482:Muscle, Skeletal / Q000473:pathology; D018979:Myositis, Inclusion Body / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D010375:Pedigree; D014617:Vacuoles / Q000473:pathology
D003402:Creatine Kinase