Congenital Basilar Impression: Correlated Neurological Syndromes

Bassi, Pietro; C, Corona; Contri, P.; Paiocchi, Andrea; Loiero, Mario; Mangoni, A

doi:10.1159/000116832

Cited by 18 publications

(6 citation statements)

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“…The most frequent symptom of basilar impression is headache and it is characteristically located in the neck and occipital region (table1). 1 11 12 Although headache can occur spontaneously, it is typically triggered by movement and exercise as described in all the cases. Sometimes (case 4) the head is held rigid to minimise the discomfort.…”

Section: Discussionmentioning

confidence: 99%

Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases

Hayes

Parker

Ell

et al. 1999

Journal of Neurology, Neurosurgery & Psychiatry

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Objectives-To describe the clinical and neuroradiological features of basilar impression in patients with osteogenesis imperfecta type IV. Methods-Four patients with basilar impression were ascertained in a population study of osteogenesis imperfecta. All four had detailed clinical and neuroradiological examination with both CT and MRI of the craniocervical junction andposterior fossa structures. Results-All four showed significant compression of the posterior fossa structures and surgical decompression was performed with relief of symptoms. Conclusion-Symptoms of cough headache and trigeminal neuralgia occurring in patients with osteogenesis imperfecta are indications for detailed clinical and neuroradiological investigation to document basilar impression. (J Neurol Neurosurg Psychiatry 1999;66:357-364) Keywords: basilar impression; osteogenesis imperfecta Basilar impression, the most often occurring malformation of the craniocervical region, is the upward displacement of basilar and condylar portions of the occipital bone causing an infolding of the foramen magnum and leading to a translocation of the upper cervical spine into the brainstem.1 2 This has the potential for severe neurological consequences. Most commonly this condition is due to a congenital developmental anomaly, but it may also occur secondarily to a generalised bone disease such as osteomalacia, Paget's disease, hyperparathyroidism, rheumatoid arthritis, and osteogenesis imperfecta. 3Osteogenesis imperfecta is a clinically and genetically heterogeneous group of heritable disorders of connective tissue characterised by reduced bone mass (osteopenia) with associated bone fragility.4 Skeletal manifestations are due to a generalised deficiency of development of both membranous and endochondral bone and include markedly thinned calvarium with delayed closure of fontanelles and sutures and excessive Wormian bone formation. Until recently, the reported association of osteogenesis imperfecta and basilar impression was considered rare. Furthermore, it was presumed that basilar impression occurs equally in all types of osteogenesis imperfecta. However, the combination of increased awareness coupled with the advent of improved neuroimaging has led to wider screening and detection of the condition. 5 This might have remained an academic exercise were it not for anaesthetic and neurosurgical advances in foramen magnum and posterior fossa decompressive surgery which reduce the morbidity and mortality from intervention. Screening of at risk people is likely to disclose a proportion with subclinical basilar impression. Closer surveillance can then be instituted. However, a craniocervical malformation is likely to be clinically suspected before neuroimaging is performed. The range of clinical symptoms is determined by the particular combination of direct compression and vascular disruption resulting from upward displacement of the floor of the posterior fossa (table 1). Some of these symptoms may be transient, progressive, or catastrophic. The spec...

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Section: Discussionmentioning

confidence: 99%

Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases

Hayes

Parker

Ell

et al. 1999

Journal of Neurology, Neurosurgery & Psychiatry

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“…Repetitive microtrauma, a minor injury, or even a trivial event such as sneezing may initiate symptoms. [8][9][10] When the odontoid process encroaches into the foramen magnum, anterior neuroaxial compression and associated neurologic problems are common. The following most common signs and symptoms occur, in decreasing order of frequency: pain in the occiput and neck, vertigo, unsteady gait, paresis of the limbs, paresthesias, speech disturbances, hoarseness, double vision, syncope, auditory disturbances, interference with swallowing, occipital headaches, blurring of vision, neurological deficit, and, rarely, sudden death.…”

Section: Assimilation Of Atlas To Occiput (Occipitalization Of the Atmentioning

confidence: 99%

Cervical Vertebrae Anomalies—Incidental Findings on Lateral Cephalograms

Soni

Sharma

Sengupta

2008

The Angle Orthodontist

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Three cases of abnormal incidental findings on lateral cephalogram are presented. These patients reported for orthodontic consultation in their adolescence. While studying the patients' cephalograms, abnormal radiographic findings were discovered in their cervical vertebrae. Because the patients were asymptomatic, early diagnosis based on these radiographic findings made the patients aware of the situations. Lifestyle changes were instituted with specialist consultation in two patients to prevent or delay the onset of symptoms of an underlying pathology. Patients were educated about the likely future course of these findings. Specialist follow-up was advised to all the patients.

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“…Primary basilar invagination results from a congenital anomaly of the chondrocranium. 20,21 The malformed clivus and/or translocated odontoid peg impinges on the anterior craniospinal neuroaxis and causes neurologic symptoms, including upper motor neuron deficits, cranial nerve abnormalities, hydrocephalus, cerebellar dysfunction, syringomyelia, and even sudden death. [22][23][24] One of the patients with basilar invagination had Chiari type I malformation with syringomyelia and developed neurologic symptoms.…”

Section: (ϫ35)mentioning

confidence: 99%

Abnormal Basiocciput Development in CHARGE Syndrome

Fujita¹,

Aida²,

Asakura³

et al. 2008

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE:The causative gene of the common congenital malformation referred to as CHARGE syndrome is CHD7. Affected individuals often undergo head and neck imaging to assess abnormalities of the olfactory structures, hypothalamus-pituitary axis, and inner ear. We encountered a few children with severe hypoplasia of the basiocciput during a radiologic assessment of patients with CHARGE syndrome. To our knowledge, this anomaly has not been reported. Our purpose was to evaluate the incidence and severity of this anomaly in this syndrome.

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Congenital Basilar Impression: Correlated Neurological Syndromes

Cited by 18 publications

References 0 publications

Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases

Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases

Cervical Vertebrae Anomalies—Incidental Findings on Lateral Cephalograms

Abnormal Basiocciput Development in CHARGE Syndrome

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