Juvenile asymmetric segmental spinal muscular atrophy (Hirayama's disease): three cases without evidence of "flexion myelopathy".
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.
Willeit
J
J
Departments of Neurology, Paediatrics, and Magnetic Resonance and Spectroscopy, University of Innsbruck, Austria. Johann.Willeit@UIBK.AC.AT
Kiechl
S
S
Kiechl-Kohlendorfer
U
U
Golaszewski
S
S
Peer
S
S
Poewe
W
W
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*