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Developmental and Acquired Disorders of The Spine
Published in Milosh Perovitch, Radiological Evaluation of the Spinal Cord, 2019
Developmental abnormalities of the spinal canal represent various disorders of the bony structures of the spine which may cause damage to the spinal cord by exerting a pressure on its substance. The atlas fusion, for example, since it was extensively studied by McRae in 1953, has been known to produce neurologic signs when the anteroposterior diameter of the bony canal is narrowed to 19 mm or less. The atlas fusion can be further complicated by the merging of other cervical vertebrae.4 Standard radiographs and tomograms, usually preceding other radiologic examinations, will indicate the presence of this abnormality. Myelographic studies in such cases (Myodil,® Oxygen®) showed a flattening of the upper cervical cord at the level of narrowing. Platybasia and basilar invagination (basilar impression) affect the spinal cord by compression. Basilar invagination can cause a narrowing of the spinal cord by an angulation of the spine, mostly behind the odontoid process. In this way, the spinal cord is compressed and flattened to a considerable degree.5 Thus occurring compression is often associated with a vascular impairment and obstruction to the flow of the CSF. Basilar invagination is usually documented on radiographs and tomograms of the base of the skull and the upper cervical spine, combined with various linear measurements. These radiologic examinations most of the time precede consideration for a myelography or vertebral angiography.
Otological Effects of Paget's Disease
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
Skull expansion may lead to disfigurement, typically frontal bossing. Extensive involvement of the skull base, producing softening of the bone, may lead to platybasia – the descent of the cranium on the cervical spine – which may lead to vertebrobasilar compromise. Skull involvement may also lead to headaches and other cranial nerve involvement.
Metabolic and endocrine bone disorders
Published in Ashley W. Blom, David Warwick, Michael R. Whitehouse, Apley and Solomon’s System of Orthopaedics and Trauma, 2017
Deformities are seen mainly in the lower limbs. Long bones bend across the trajectories of mechanical stress; thus the tibia bows anteriorly and the femur anterolaterally (see Figure 7.23a). The limb looks bent and feels thick, and the skin is unduly warm - hence the term ‘osteitis deformans’ If the skull is affected, it enlarges; the patient may complain that old hats no longer fit. The skull base may become flattened (platybasia), giving the appearance of a short neck.
Long-term surgical outcome of Chiari type-I malformation-related syringomyelia: an experience of tertiary referral hospital
Published in Neurological Research, 2022
Anas Abdallah, İrfan Çınar, Betül Güler Abdallah
During the study period, 54 patients with CRS were surgically treated with the PFDD and tonsillar coagulation. Among them, 46 (24 males and 22 females) patients met the study criteria. The mean age was 35.4 ± 9.8 (range; 16–53). Four patients were ≤18 years old (16–18) and were included in the study due to their comparable body mass indices with adults. Headaches were the most commonly seen presenting symptoms that were observed in 24 (52.2%) patients. Table 1 shows the baseline demographic and clinical characteristics of the patients. Impaired cerebellar function tests, such as gait ataxia, nystagmus, dysmetria, and dysdiadochokinesia, were the most common neurological findings in the 32 (69.6%) patients examined. Table 2 summarizes the clinical findings from the first presentation. Tonsillar herniation in 27 (58.7%) patients was <10 mm. The syrinx cavities were in the cervical and cervicothoracic regions in 24 (52.2%) and 18 (39.1%) patients, respectively. The mean preoperative axial S/C was 0.59 ± 0.12 (0.31–0.92). S/C < 0.59 was observed in 20 (43.5%) patients. On cranial CT, 10 (21.7%) patients presented with radiological hydrocephalus (Evans index > 0.30). Septation in syrinx was seen in 17 (37.0%) patients. Syringobulbia was seen in 4 (8.7%) patients. In our patients, craniocervical anomalies, such as basilar impression (n = 5), platybasia (n = 3), atlantoaxial subluxation (n = 3), and Klippel Feil (n = 2), were observed in seven (15.2%) patients. Scoliosis and kyphosis were recorded in 6 (13.0%) and 4 (8.7%), respectively.
Craniometrical imaging and clinical findings of adult Chiari malformation type 1 before and after posterior fossa decompression surgery with duraplasty
Published in British Journal of Neurosurgery, 2019
Mehdi Nikoobakht, Hamidreza Shojaei, Peter C. Gerszten, Seyedeh Fahimeh Shojaei, Reza Mollahoseini, Maziar Azar
Related to the posterior fossa morphology as an etiologic factor in the development of CM-1, there is also considerable interest in the structural parameters of the cranio-cervical region that may help differentiate CM-1 symptomatology from healthy people.9 The craniometric parameters of this region are of paramount importance to understand and purpose adequate treatments for the symptomatic CM-1 patients.10 For example, the odontoid retroflexion is thought to compromise the posterior cranial fossa. Other research efforts have explored the relationship of platybasia, clivus underdevelopment, tentorial angulation and other posterior fossa and cerebral anomalies in patients with a diagnosis of CM-1.8
Position-dependent arm dyskinesia due to severe craniocervical malformation
Published in The Journal of Spinal Cord Medicine, 2022
Francisco de Assis Aquino Gondim, José Arnaldo Mota Arruda, David Nunes de Lima Junior, Florian P. Thomas
Karl von Rokitansky was the first to report platybasia and Ernst Alexander Homén the first to relate severe neurological manifestations to an odontoid process compressing the medulla oblongata.5 To our knowledge, our patient’s movement disorder associated with craniocervical malformation has not been previously reported. She experienced left arm dyskinesia when she bent her neck and flexed the forearm while touching the elbow on a flat surface.