Amyotrophic lateral sclerosis and neurosarcoidosis: a case report.
Amyotrophic lateral sclerosis (ALS) remains a clinical diagnosis without definable biomarkers. The pathomechanism of motor neuron degeneration in ALS has yet to be elucidated. Here we present a case of limb-onset ALS, with autopsy findings of Bunina bodies and skein-like inclusions, as well as sarcoid granulomas predominating among motor neurons. The targeting of the motor neurons by the sarcoid inflammation raises questions regarding the role of cellular immunity in the pathomechanisms for ALS.
Chen
I-Hweii Amy
IH
Department of Neurology and the Eleanor and Lou Gehrig MDA/ALS Research Center, Columbia University, New York, New York, USA.
Mitsumoto
Hiroshi
H
Vonsattel
Jean P
JP
Hays
Arthur P
AP
D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D001344:Autopsy; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008875:Middle Aged; D009046:Motor Neurons / Q000378:metabolism / Q000473:pathology; D012507:Sarcoidosis / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology; D025801:Ubiquitin / Q000378:metabolism
D025801:Ubiquitin