Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity

Abstract

Radiologic abnormalities of the lower cervical dural sac and spinal cord support the hypothesis that this disorder is a type of cervical myelopathy.

Cervical Spine MR Imaging Findings of Patients with Hirayama Disease in North America: A Multisite Study

“…The pathomechanism is debated, but the prevailing theory postulates that insufficient growth of the dura relative to the spinal column during puberty allows forward displacement of the dura in flexion with compression of the spinal cord, possibly leading to ischemia of the anterior horn cells at C8 and T1. 2 Most reported cases have been from Asia, particularly Japan, 1 though some have been reported from India, the Middle East, and Europe. [3][4][5][6] To date, there have only been a handful of cases reported from North America, and to our knowledge, there has been no published study characterizing the MR imaging features of this disease in North American patients.…”

“…Possible explanations include the older age of these patients at the time of MR imaging (37.8 years), because the findings of HD reportedly decrease with age. 2 Although the prevailing theory indicates that HD is a flexion-induced myelopathy resulting from a dura that becomes taut and forwardly displaced in flexion, the underlying pathomechanism is still debated. 6 We found a wide range of dural shift in patients with HD (0 -7 mm).…”

Association of the X-linked Androgen Receptor Leu57Gln Polymorphism with Monomelic Amyotrophy

“…To date, most studies have involved a very limited numbers of patients and have focused on case reports, mainly from Asian countries such as Japan and India. These studies have included 38 cases and 73 cases with MA in a Japanese population (Hirayama, 1972;Hirayama and Tokumaru, 2000), a sibling case in Turkey (Gucuyener et al, 1991), 44 patients and a case from India (Gourie-Devi and Nalini, 2003;Nalini et al, 2004), a sporadic case in an Italian man (Rigamonti et al, 2004), a young Swiss female patient with MA (Jeannet et al, 2005), and 15 male cases from 14 Indian families (Misra et al, 2005). Currently, the pathophysiology of MA is not well understood but various possibilities have been considered, including autoimmune and genetic factors, and ischemic changes of the spinal cord induced by neck flexion (Nalini et al, 2004).…”

Hirayama Disease: The Role of Mri

“…Гипоксия приводит к ишемии и некрозу перед-них рогов спинного мозга нижнешейного отдела позвоночника. Данные изменения являются от-личительной чертой данной патологии [1,2].…”