Abstract
The objective of this study is to discuss the possible role of cervical posterior epidural plexus engorgement during cervical flexion in the pathogenesis of Hirayama myelopathy. In Hirayama disease, MRI during neck flexion often shows that the posterior dura detaches from the posterior arches compressing the spinal cord. Autopsies demonstrated asymmetric changes in the anterior horns consistent with chronic ischemic damage, attributed to arterial insufficiency during flexion or to microcirculatory changes due to compression by the tight dura. In a 15-year-old patient with 5-year history of distal upper limbs weakness, MRI demonstrated marked venous engorgement of the posterior epidural plexus in cervical flexion, confirmed by angiography. Laminectomy from C3 to C6 with duraplasty was performed. At one-year follow-up, the clinical condition of the patient remained stable. In Hirayama myelopathy, compression of the spinal cord by the tight dura is probably the most important pathogenetic factor. However, venous congestion in flexion might play an additional role in determining spinal cord ischemic changes.
Similar content being viewed by others
References
Polo A, Dossi MC, Fiaschi A et al (2003) Peripheral and segmental spinal abnormalities of median and ulnar somatosensory evoked potentials in Hirayama’s disease. J Neurol Neurosurg Psychiatry 74:627–632
Hirayama K (2000) Juvenile muscular atrophy of distal upper extremity (Hirayama disease): focal cervical ischemic poliomyelopathy. Neuropathology 20:S91–S94
Chi-jen C, Hui-Ling H, Ying-Chi T et al (2004) Hirayama flexion mielopathy: neutral position MR imaging findings—importance of loss of attachment. Radiology 231:39–44
Hemant AS, Rakesh SS, Firosh KK et al (2008) Imaging features in Hirayama disease. Neurol India 56:22–26
Kikuchi S, Shinpo K, Nino M et al (2002) Cervical myelopathy due to a “tight dural canal in flexion” with a posterior epidural cavity. Intern Med 41:746–748
Hirayama K, Toyokura Y, Tsubak T (1959) Juvenile muscular atrophy of unilateral extremity: a new clinical entity. Psychiatria et Neurologia Japonica 61:2190–2197
Toma S, Shiozawa Z (1995) Amyotrophic cervical myelopathy in adolescence. J Neurol Neurosurg Psychiatry 58:56–64
Kira J, Ochi H (2001) Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy. J Neurol Neurosurg Psychiatry 70:798–801
Chen CJ, Chen CM, Wu CL et al (1998) Hirayma disease: MR diagnosis. AJNR Am J Neuroradiol 19:365–368
Elsheickh B, Kissel JT, Christoforidis G et al (2009) Spinal angiography and epidural venography in juvenile muscular atrophy of the distal arm “Hirayama disease”. Muscle Nerve 40:206–212
Patel TR, Chiocca EA, Freimer ML, Christoforidis GA (2009) Lack of epidural pressure change with neck flexion in a patient with Hirayama disease: case report. Neurosurgery 64:E1196–E1197
Criscuolo GR, Oldfield EH, Doppman JL (1989) Reversible acute and subacute myelopathy in patients with dural arteriovenous fistulas. Foix-Alajouanine syndrome reconsidered. J Neurosurg 70:354–359
Fujimoto Y, Oka S, Tanaka S, Nishikawa K et al (2002) Pathophysiology and treatment for cervical flexion myelopathy. Eur Spine J 11:276–285
Konno S, Goto S, Muratami M et al (1997) Juvenile muscular atrophy of distal upper extremity: pathologic findings of the dura mater and surgical management. Spine 22:486–492
Chiba S, Yonekura K, Nonaka M et al (2004) Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction. Intern Med 43:79–81
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ciceri, E.F., Chiapparini, L., Erbetta, A. et al. Angiographically proven cervical venous engorgement: a possible concurrent cause in the pathophysiology of Hirayama’s myelopathy. Neurol Sci 31, 845–848 (2010). https://doi.org/10.1007/s10072-010-0405-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10072-010-0405-3