Presentation
PLATYBASIA AND BASILAR INVAGINATION PRESENTING AS CEREBROVASCULAR ACCIDENT Abstract The congenital and acquired deformities of thecraniovertebral junction (basilar invagination,basilar impression, or platybasia) can present in the form ofslowly progressive [wwjournals.com]
The aim of the present study was to investigate one such skull base deformity, platybasia, in ITN patients in comparison with a randomized control population. [ncbi.nlm.nih.gov]
Not only are the ocular signs and symptoms of these entities often the presenting complaints but, equally important, they simulate those of multiple sclerosis, tumor of the brain stem or cerebellum, and other lesions of the posterior fossa, which have [jamanetwork.com]
— 67-year-old woman with polyostotic Paget's disease who presented complaining of headache and unsteadiness. [ajronline.org]
Entire Body System
- Asymptomatic
In most cases, subluxation less than this is asymptomatic. Lateral x-rays taken in extension and flexion are used to demonstrate AAS. [dizziness-and-balance.com]
Although asymptomatic patients are often followed conservatively, many authors favor surgery even if no symptoms of cord compression are evident in rheumatoid patients. [neuroradiologyonthenet.blogspot.com]
Patients may be asymptomatic or complain of headaches, neck pain, cough, trigeminal neuralgia, limb weakness, altered muscle reflexes, quadriparesis, nystagmus, and ataxia. [rrnursingschool.biz]
While it can be closely associated with basilar invagination and basilar impression, in isolation it is asymptomatic and essentially is a misused term when used to describe congenital defects. [statpearls.com]
As an isolated finding, platybasia is not clinically important, and most patients are asymptomatic ( 13 ). When the basal angle is measured on lateral skull images, accurate X-ray techniques are essential for correct angles. [ajnr.org]
- Disability
When symptoms progress and threaten disability, treatment is by surgical decompression or by transoral odontoidectomy with reduction of the basilar impression and craniovertebral junction bony realignment, and atlanto-axial fixation [ 14, 15 ]. [doi.org]
As mentioned before, this can be a disabling condition, and may cause full disability in some patients, often requiring legal support to claim economic aid, special occupational conditions, occupational disability, and disability degree certification. [bmcmusculoskeletdisord.biomedcentral.com]
This causes brainstem compression leading to severe neurological disability, respiratory compromise and even sudden death; anaesthetizing such a patient thus is quite a challenge. [ispub.com]
Recently, an increasing number of medical reports have implicated osteoporotic fractures as a cause of serious neurological deficit and painful disabling spinal deformities. This has been corroborated by the present authors as well. [science.gov]
- Prolonged Immobilization
Therapeutic strategies vary from prolonged immobilization to posterior fossa or ventral brain stem decompression with occipitocervical fusion. However, the results are not uniformly satisfactory (Sawin and Menezes 1997). [rrnursingschool.biz]
Respiratoric
- Painful Cough
Patients may be asymptomatic or complain of headaches, neck pain, cough, trigeminal neuralgia, limb weakness, altered muscle reflexes, quadriparesis, nystagmus, and ataxia. [rrnursingschool.biz]
Table 1 Basilar invagination: some radiological signs Basilar Impression or Invagination of the Skull Basilar invagination, the commonest malformation of the craniocervical junction [ 9 ], is a well recognised cause of neck pain, cough headache, lower [doi.org]
Jaw & Teeth
- Prognathism
He described 4 types: orthognathous dolichocephalic, orthognathous brachycephalic, prognathous dolichocephalic and prognathous brachycephalic. [doi.org]
Simple craniosynostosis Skull anomaly Treacher collins syndrome Clinical Information Autosomal dominant disorder characterized by acrocephaly, exophthalmos, hypertelorism, strabismus, parrot-beaked nose, and hypoplastic maxilla with relative mandibular prognathism [icd9data.com]
Musculoskeletal
- Neck Pain
These abnormalities can result in neck pain; syringomyelia; cerebellar, lower cranial nerve, and spinal cord deficits; and vertebrobasilar ischemia. Diagnosis is by MRI or CT. [merckmanuals.com]
Patients may be asymptomatic or complain of headaches, neck pain, cough, trigeminal neuralgia, limb weakness, altered muscle reflexes, quadriparesis, nystagmus, and ataxia. [rrnursingschool.biz]
Table 1 Basilar invagination: some radiological signs Basilar Impression or Invagination of the Skull Basilar invagination, the commonest malformation of the craniocervical junction [ 9 ], is a well recognised cause of neck pain, cough headache, lower [doi.org]
- Severe Osteoporosis
Indeed, basilar impression can complicate the course of any disease associated with severe osteoporosis, os- Fig. 1.37. Basilar invagination in a 32-year-old woman. [rrnursingschool.biz]
Eyes
- Diplopia
The symptoms may also include clumsiness, numbness along one side of the body, unsteadiness of the gait, vertigo, weakness of one side of the body, loss of control of bowel and bladder, diplopia (double vision), periodic or constant cramping pain of one-sided [xpertdox.com]
The most frequent ocular symptoms are: retro-orbital headache, diplopia, photopsia, blurred vision, and photophobia. [bmcmusculoskeletdisord.biomedcentral.com]
Psychiatrical
- Distractibility
The distraction and reduction was maintained with the help of bone graft and/or spacers. [jcvjs.com]
Another approach is atlantoaxial joint distraction and direct lateral mass fixation using screws (Goel, 2004). [dizziness-and-balance.com]
Goel A: Progressive basilar invagination after transoral odontoidectomy: treatment by atlantoaxial facet distraction and craniovertebral realignment. Spine 2005;30:E551–E555. [doi.org]
Face, Head & Neck
- Short Neck
She had blue sclera and short neck ( figure 1 ), right hypoglossal palsy, bilateral pyramidal tract signs, and gaze-evoked nystagmus. [n.neurology.org]
Klippel-Feil syndrome type II is massive fusion of two of seven cervical vertebrae associated with short neck and low hair line. [ncbi.nlm.nih.gov]
A short neck, craniofacial asymmetry, and kyphoscoliosis are common associated features. The foramen magnum is often deformed to varying extents. [rrnursingschool.biz]
Neurologic
- Lhermitte Sign
Lhermitte's sign (a tingling on neck movement, flexion in this case) can be demonstrated at any stage. A plain lateral x-ray, with odontoid views, is a good place to start, but is not 100% sensitive (Riew, Hilibrand et al. 2001). [dizziness-and-balance.com]
Tingling down the back, often into the legs, with neck flexion (Lhermitte sign) may occur. Uncommonly, pain and temperature senses (spinothalamic tract function) are impaired in a stocking-glove pattern. [merckmanuals.com]
- Spastic Quadriplegia
Spastic quadriplegia can result from platybasia. Basilar invagination or compression of posterior fossa structures may lead to cerebellar or brainstem compressive syndromes. [emedicine.medscape.com]
- Hyperreflexia
According to Sawin and Menezes series in persons with osteogenesis imperfecta (1997), symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis [dizziness-and-balance.com]
Workup
X-Ray
- Cervical Spine X-Ray
Relevant diagnostic work-up included craniogram, cervical spine x-ray, computed tomography (CT) of the brain and craniocervical junction, magnetic resonance imaging of the brain, electroencephalography, ophthalmologic examination, urinary tract ultrasonography [ncbi.nlm.nih.gov]
Treatment
Treatment: Unless there is impingement or compression of nerve roots or other neural tissue, no treatment is specifically indicated for platybasia; however, neural compression requires optimal treatment. [neurosurgery.ucla.edu]
[…] duration of treatment (OR 0.28). [science.gov]
Subsequent refinement of radiology facilitated diagnosis and the later advent of surgical treatment. © 2007 S. [doi.org]
It is now believed that atlantoaxial fixation forms the basis of treatment in all types of basilar invagination. Decompression of bone either by transoral route or by posterior foramen magnum decompression are suboptimal forms of treatment. [ncbi.nlm.nih.gov]
Prognosis
In patients who are considered poor surgical risks, neurologic progression is likely and the one-year prognosis is poor. Neurosurgical treatment is quite difficult. [dizziness-and-balance.com]
Constantin Ionescu-Tirgoviste and Cristian Guja Resting metabolic rate in obese type 2 diabetic patients candidates for metabolic surgery 214 Anna Mirela Stroie and Mircea Penescu Adipose tissue mass and lean tissue mass predict medium-term outcome and prognosis [acad.ro]
The prognosis for patients with Paget disease who have giant cell tumors usually is good. High doses of steroids have been shown to reduce tumor mass. Radiation and surgery also have been used to treat symptomatic giant cell tumors. [emedicine.medscape.com]
Statistical analysis indicated that the cerebellar tonsillar descent (CTD) grade, basilar invagination and platybasia influenced the clinical outcome (P platybasia were indicated to be predictors of poor clinical prognosis. [science.gov]
Etiology
Pathology Etiology congenital achondroplasia Down syndrome Chiari malformations craniocleidodysostosis craniofacial anomalies osteogenesis imperfecta acquired Paget disease osteomalacia rickets trauma fibrous dysplasia hyperparathyroidism 5 hypoparathyroidism [radiopaedia.org]
Many factors may contribute to the etiology of the VPD in 22q11DS [ 2 ]. An abnormally obtuse cranial base angle, also known as platybasia, is a common finding in 22q11DS [ 3, 4, 5 ]. [e-aps.org]
Overview Etiology Congenital anomalies of the CVJ Acquired anomalies of the CVJ Trauma : to the bones or the supporting ligaments of the CVJ Inflammatory: rheumatoid arthritis, ankylosing spondylitis Genetic syndromes: Down syndrome, achondroplasia, osteogenesis [amboss.com]
The etiology of VPD in 22q11DS is multifaceted [ 2 ] and is likely to be a sum of many factors. Our findings indicate that platybasia does not play a prominent role in VPD. [e-sciencecentral.org]
What is the etiology of Os Odontoid(eum)? Etiology questionable, possibly congenital failure of fusion or a previously unrecognized fracture. What must be assessed with Os Odontoid(eum)? Integrity of the transverse ligament must be assessed. [quizlet.com]
Epidemiology
[…] imperfecta Malignancies: chondrosarcoma, multiple myeloma, metastatic deposits References: [1] Chiari malformations Definition : caudal displacement of the cerebellum with/without the medulla oblongata, through the foramen magnum, due to CVJ anomalies Epidemiology [amboss.com]
35 Elisa Mădălina Chirca, Rodica Luca and Georgescu Dan-Emil The prevalence of caries in first permanent molar in a group of school children aged 6 to 7 years in Piteşti 41 Flaviu Otniel Ciobotă, Gheorghe Cristea, Mariana Ioniţă and Ioan Liviu Mitrea Epidemiological [acad.ro]
Epidemiology Interestingly, the most common developmental abnormality at this site is atlantooccipital assimilation or occipitalization of the atlas, which frequently is noted in many other congenital anomalies. [statpearls.com]
Pathophysiology
In the current report, the primary focus will be on clinical aspects of these CVJ abnormalities; the pathologic and radiologic aspects, such as developmental and pathophysiologic background or radiographic analysis, will be discussed briefly, confined [ncbi.nlm.nih.gov]
[…] anomalies Epidemiology Incidence : Type I Chiari malformation is the most common anomaly; 1 in 1,000 live births Sex: ♀ > ♂ Associations Neural tube defects (esp. meningomyelocele ) Syringomyelia Hydrocephalus Klippel-Feil syndrome Basilar invagination Pathophysiology [amboss.com]
The pathophysiology of arthritic changes associated with Paget disease is unknown. Enlargement of joints and altered biomechanics may cause abnormal stress across joints, giving rise to degenerative changes. [emedicine.medscape.com]
Pathophysiology The basilar impression can be divided into two types: primary, which is the congenital defect that is frequently associated with syndromes and other vertebral abnormalities, and secondary which is attributed to development issues resulting [statpearls.com]
Prevention
[…] which the odontoid process abnormally prolapses upward through the foramen magnum) 2 in OI results from abnormally soft bone at the cranio-cervical junction and the odontoid process protruding through the foramen magnum. 3 Appropriate screening may prevent [n.neurology.org]
Surgery is often indicated to prevent or treat neurological symptoms. [amboss.com]
[…] bone metabolic and functional impairments in children with cerebral palsy - preliminary results 137 Raluca Oana Munteanu, Emilia Rusu, Diana Simona Stefan and Gabriela Radulian Nutrition, physical activity and lifestyle factors in childhood obesity prevention [acad.ro]
To prevent the syrinx from expanding and harming your spinal cord, your doctor may perform surgery to remove the syrinx, though observation is a common treatment as well. [spineuniverse.com]
SECURITY The law establishes an obligation to adopt security measures to prevent the unauthorized access to the computer database containing personal data or damage to the integrity or quality of the data. [institutchiaribcn.com]