title: [Hirayama disease in Germany: case reports and review of the literature].
abstract: Hirayama disease is a juvenile benign distal upper limb muscular atrophy rarely observed in Europe, usually monomelic involving C7-Th1 innervated muscles. It is characterized by insidious onset and a self-limited course within a few years. The pathogenesis of this mostly sporadic disease is not fully clarified. Cervical flexion myelopathy with mechanical ischemic damage of spinal motoneurons is the best established pathogenetic hypothesis, but neurodegenerative and autoimmune causes are also debated. Typically, young men of Asian origin are affected. Here we describe three German Caucasian patients with Hirayama disease and provide an up-to-date review of the literature.
authors.affiliation: Klinik für Neurologie, Klinikum der Goethe-Universität Frankfurt, Schleusenweg 2-16, 60528 Frankfurt/Main, Deutschland. jun-suk.kang@em.uni-frankfurt.de
authors.forename: J-S
authors.initials: JS
authors.lastname: Kang
authors.forename: S
authors.initials: S
authors.lastname: Jochem-Gawehn
authors.forename: H
authors.initials: H
authors.lastname: Laufs
authors.forename: A
authors.initials: A
authors.lastname: Ferbert
authors.forename: P
authors.initials: P
authors.lastname: Vieregge
authors.forename: U
authors.initials: U
authors.lastname: Ziemann
mesh_terms: D000293:Adolescent; D000328:Adult; D001132:Arm / Q000294:innervation; D002574:Cervical Vertebrae / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D004576:Electromyography; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009046:Motor Neurons / Q000502:physiology; D018482:Muscle, Skeletal / Q000294:innervation; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000503:physiopathology; D020760:Spinal Cord Ischemia / Q000503:physiopathology; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis* / Q000503:physiopathology; D055815:Young Adult