Sarcoid neuromyopathy with selective involvement of the intramuscular nerves.
A 35-year-old man affected with pulmonary sarcoidosis had a 12-year history of fatigue and pain in the limbs, with normal neurological examination, except for diffusely absent deep tendon reflexes. Muscle biopsy samples showed multiple noncaseating granulomas, most prominent around the intramuscular nerves, with predominance of CD4+ cells. Intramuscular nerve bundles surrounded by granulomas were immunolabelled with laminin alpha1, alpha2, beta1 and gamma1 chain, and collagen IV. Sural nerve biopsy samples were normal. This patient showed a unique histopathological pattern of sarcoid neuromyopathy characterized by distribution of granulomas or infiltrating cells around intramuscular nerve fibers. The clinical picture, restricted to nonspecific symptoms of fatigue and myalgia, and loss of deep tendon reflexes, correlated well with the selective localization of sarcoid lesions in contiguity with the intramuscular nerves. To our knowledge, this peculiar clinico-pathological correlation has not been reported previously.
Gemignani
F
F
Institute of Neurology, University of Parma, Italy.
Bellanova
M F
MF
Salih
S
S
Margarito
F P
FP
Marbini
A
A
D000328:Adult; D006801:Humans; D007150:Immunohistochemistry; D007866:Leg / Q000294:innervation / Q000473:pathology; D008297:Male; D018908:Muscle Weakness / Q000209:etiology / Q000473:pathology; D018482:Muscle, Skeletal / Q000294:innervation* / Q000473:pathology*; D009135:Muscular Diseases / Q000473:pathology*; D012507:Sarcoidosis / Q000473:pathology*; D013497:Sural Nerve / Q000473:pathology