Abstract
Eight patients with a juvenile type of distal and segmental muscular atrophy of the upper extremities (DSMA), a type of cervical flexion myelophathy, were evaluated using MR imaging. In the neutral position there was no spinal cord compression, but in flexion the spinal cord was displaced anteriorly and was compressed by the posterior surfaces or margins of the vertebrae and/or any herniated disks in all cases. In flexion, compression of the cord was exaggerated in seven patients by the anterior displacement of the posterior margin of the thecal sac, which was accompanied by dilated posterior internal vertebral veins. In patients suspected of having DSMA, MR images made in flexion are regarded essential for verifying the diagnosis.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Breig A, El-Nadi AF (1966) Biomechanics of the cervical spinal cord: relief of contact pressure on and overstretching of the spinal cord. Acta Radiol Diagn 4: 602
Breig A, Turnbull I, Hassler O (1966) Effects of mechanical stresses on the spinal cord in cervical spondylosis: a study on fresh cadaver material. J Neurosurg 24: 45
Penning L, van der Zwaag P (1966) Biochemical aspects of spondylotic myelopathy. Acta Radiol Diagn 5: 1090
Reid JD (1960) Effects of flexion-extension movements of the hand and spine upon the spinal cord and nerve roots. J Neurol Neurosurg Psychiat 23: 214
Levy LM (1982) An unusual case of flexion injury of the cervical spine. Surg Neurol 17: 255
Wilder BL (1982) The etiology of midcervical quadriplegia after operation with patient in the sitting position: hypothesis. Neurosurgery 11: 530
Iwasaki Y, Tashiro K, Kikuchi S, Kitagawa M, Isu T, Abe H (1987) Cervical flexion myelopathy: a “tight dural canal mechanism”: case report. J Neurosurg 66: 935
Kikuchi S, Tashiro K, Kitagawa M, Iwasaki Y, Abe H (1987) A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama's disease): flexion myelopathy with tight dural canal in flexion. Clin Neurol 27: 412
Sobue I, Saito N, Iida M, Ando K (1978) Juvenile type of distal and segmental muscular atrophy of upper extremities. Ann Neurol 3: 429
Hirayama K, Toyokura Y, Tsubaki T (1959) Juvenile muscular athropy of unilateral upper extremity: a new clinical entity. Psychiatr Neurol Jpn 61: 2190
Mukai E, Matsuo T, Muto T, Takahashi A, Sobue I (1987) Magnetic resonance imaging of juvenile-type distal and segmental muscular atrophy of upper extremities. Clin Neurol 27: 99
Matsumura K, Inoue K, Yagishita A (1984) Metrizamide CT myelography of Hirayama's disease. A localized atrophy of the lower cervical spinal cord. Clin Neurol 24: 848
Mukai E, Sobue I, Muto T, Takahashi A, Goto S (1985) Abnormal radiological findings on juvenile-type distal and segmental muscular atrophy of upper extremities. Clin Neurol 25: 620
Condon BR, Hadley DM (1988) Quantification of cord deformation and dynamics during flexion and extension of the cervical spine using MR imaging. J Comput Assist Tomogr 12: 947
Reynolds H, Carter SW, Murtagh FR, Rechtine GR (1987) Cervical rheumatoid arthritis: value of flexion and extension views in imaging. Radiology 164: 215
Pilgaard S (1968) Unilateral juvenile muscular atrophy of upper limbs. Acta Orthop Scand 39: 327
Leys D, Petit H (1987) Amyotrophie juvenile distale chronique unilaterale localisee a un membre superieur (type Hirayama). Un cas europeen. Rev Neurol (Paris) 143: 611
Delerue O, Hurtevent JF, Destee A (1990) Amyotrophie juvenile, monomelique, benigne d'une main (de type Hirayama): 1 nouvelle observation. Acta Neurol Belg 90: 82
Schnyder H, Meyer M (1991) Die benigne fokale Amyotrophie. Schweiz Med Wochenschr 121: 167
Gargano FP (1980) Extradural venography. In: Post MJD (ed), Radiological evaluation of the spine. New York, Masson, pp 579–592
Shapiro R (1984) Myelography. 4th ed. New York Medical, Chicago, pp 613–627
Takahashi M, Sakamoto Y, Miyawaki M, Bussaka H (1988) MR visualization and clinical significance of the anterior longitudinal epidural venous plexus in cervical extra-axial lesions. Comput Med Imag Graph 12: 169
Hirayama K, Tomonaga M, Kitano K, Yamada T, Kojima S, Arai K (1985) The first autopsy case of “juvenile muscular atrophy of unilateral upper extremity”. Neurology (Tokyo) 22: 85
Takahashi M, Yamashita Y, Sakamoto Y, Kojima R (1989) Chronic cervical cord compression: clinical significance of increased signal intensity on MR images. Radiology 173: 219
Masaki T, Hashida H, Sakuta M, Kunogi J (1990) A case of flexion myelopathy presenting juvenile segmental muscular atrophy of upper extremities: successful treatment by cervical spine immobilization. Clin Neurol 30: 625
Author information
Authors and Affiliations
Additional information
Correspondence to: K. Hasuo
Rights and permissions
About this article
Cite this article
Hasuo, K., Uchino, A., Matsumoto, S. et al. Magnetic resonance imaging in a juvenile type of distal and segmental muscular atrophy of the upper extremities. Eur. Radiol. 4, 119–124 (1994). https://doi.org/10.1007/BF00231197
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00231197