title: Juvenile asymmetric segmental spinal muscular atrophy (Hirayama's disease): three cases without evidence of "flexion myelopathy".
abstract: Compression of the cervical spinal cord during neck movements ("flexion myelopathy") was proposed to be the main pathomechanism of juvenile asymmetric spinal muscular atrophy (JASSMA). We present 3 patients with the clinical appearance of JASSMA and typical high-intensity signals in the anterior horn cell region of the lower cervical spinal cord (T2-weighted magnetic resonance images) but without evidence of dynamic spinal cord compression. In all these patients pathomechanism distinct from mechanical damage must be assumed.
authors.affiliation: Departments of Neurology, Paediatrics, and Magnetic Resonance and Spectroscopy, University of Innsbruck, Austria. Johann.Willeit@UIBK.AC.AT
authors.forename: J
authors.initials: J
authors.lastname: Willeit
authors.forename: S
authors.initials: S
authors.lastname: Kiechl
authors.forename: U
authors.initials: U
authors.lastname: Kiechl-Kohlendorfer
authors.forename: S
authors.initials: S
authors.lastname: Golaszewski
authors.forename: S
authors.initials: S
authors.lastname: Peer
authors.forename: W
authors.initials: W
authors.lastname: Poewe
mesh_terms: D000293:Adolescent; D002574:Cervical Vertebrae; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009135:Muscular Diseases / Q000209:etiology*; D013117:Spinal Cord Compression / Q000209:etiology*; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications*