Khan Abdul Aziz, Bhatti Sajid Nazir, Khan Ghayyur, Ahmed Ehtisham, Aurangzeb Ahsan, Ali Asghar, Khan Amjad, Afzal Saleem
Department of Neurosurgery, Ayub Medical College, Abbottabad, Pakistan.
J Ayub Med Coll Abbottabad. 2010 Apr-Jun;22(2):75-8.
The Chiari Malformation I (CMI) is a disorder of uncertain origin that has been traditionally defined as downward herniation of the cerebellar tonsils through the foramen magnum. The anomaly is a leading cause of syringomyelia and occurs inassociation with osseus abnormalities at the craniovertebral junction. In contrast to other Chiari malformations, CMI tends to present in the second or third decade of life and is sometimes referred to as the 'adult-type' Chiari malformation. The objective was to document clinical and radiological findings in Arnold Chiari Malformation-I.
This was a descriptive study carried out in Ayub Teaching Hospital Abbottabad at Neurosurgery Department during July 2008-July 2010. We examined a prospective cohort of 60 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine.
There were 40 female and 20 male patients. The age of onset was 24.9 +/- 15.8 years. Common associated radiological problems included syringomyelia (60%), scoliosis (25%), and basilarinvagination (12%), increased cervical lordosis 5 (8.5%), and Klippel Feil syndrome 2 (3.3%). The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (70% patients), tonsillar herniation of at least 5 mm (100% patients), and varying degrees of post fossa anomalies. Linical manifestations were headaches, pseudotumor-like episodes, a Meniere's disease-like syndrome, lower cranial nerve signs, and spinal cord disturbances in the absence of syringomyelia.
These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterised by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible for headaches, pseudotumor-like episodes, endolymphatic hydrops, syringomyelia, and hydrocephalus) and direct compression of nervous tissue.
Chiari I型畸形（CMI）是一种病因不明的疾病，传统上被定义为小脑扁桃体经枕骨大孔向下疝出。该异常是脊髓空洞症的主要病因，且常与颅颈交界区的骨质异常相关。与其他Chiari畸形不同，CMI往往在生命的第二个或第三个十年出现，有时被称为“成人型”Chiari畸形。目的是记录Arnold Chiari I型畸形的临床和影像学表现。
这是一项于2008年7月至2010年7月在阿伯塔巴德阿尤布教学医院神经外科进行的描述性研究。我们检查了60例有症状患者的前瞻性队列。所有患者均接受了头部和脊柱的磁共振成像检查。
有40例女性和20例男性患者。发病年龄为24.9±15.8岁。常见的相关影像学问题包括脊髓空洞症（60%）、脊柱侧弯（25%）和基底凹陷（12%）、颈椎前凸增加5例（8.5%）以及Klippel Feil综合征2例（3.3%）。最一致的磁共振成像表现是小脑后脑脊液间隙闭塞（70%的患者）、扁桃体疝出至少5毫米（100%的患者）以及不同程度的后颅窝异常。临床表现为头痛、类似假瘤的发作、梅尼埃病样综合征、下颅神经体征以及在无脊髓空洞症情况下的脊髓功能障碍。
这些数据支持越来越多的证据表明，CMI是一种轴旁中胚层疾病，其特征是后颅窝发育不全和正常发育的后脑过度拥挤。扁桃体疝出小于5毫米并不能排除诊断。CMI的临床表现似乎与脑脊液紊乱（导致头痛、类似假瘤的发作、内淋巴积水、脊髓空洞症和脑积水）以及神经组织的直接压迫有关。
