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At least 145 records · Page 8Linked to original sources
PubMed1978

Basilar impression in an achondroplastic dwarf: causative role in tetraparesis.

The neurological and radiological findings in a case of chondrodystrophic dwarfism are described. The progressive tetraparesis proved to be based on a high-level medullary compression resulting from basilar impression and upper cervical stenosis. Surgical decompression led to the disappearance of the tetraparetic phenomena. Various aspects of chondrodystrophic dwarfism and basilar impression are discussed in relation to the neurological symptomatology, and the question of whether, in general, more attention should be given to the cranio-cervical region, is raised.

Achondroplasia↗
PubMed1994

Craniocervical abnormalities in osteogenesis imperfecta: genetic and molecular correlation.

Basilar impression (BI) assessed by either plain lateral skull radiograph or computerized tomography (CT) sagittal reconstruction of the craniocervical junction is a common finding occurring in 25% of subjects with osteogenesis imperfecta (OI). It appears to occur with highest frequency in a group of subjects with OI type IV B, i.e. patients with mild/moderate liability to fractures, normal sclerae but dentinogenesis imperfecta. Neurologic signs indicating compression of posterior fossa structures occur predominantly in subjects with BI and OI type IV. Screening is recommended for all patients with OI but particularly OI type IV B.

Cervical Vertebrae↗
PubMed1995

Craniovertebral junction anomalies in inherited disorders: part of the syndrome or caused by the disorder?

Patterns of skeletal abnormality at the craniovertebral junction in the normal population and in syndromes such as Down, Morquio etc, are compared and the recent embryological data and comparative anatomy reviewed. The authors' view based on their own clinical and radiological experience is that the os odontoideum is the product of excessive movement at the time of ossification of the cartilaginous dens and is exactly analogous to the unfused Type II odontoid fracture. True hypoplasia of the odontoid peg is part of a wider segmentation defect associated with Klippel Feil, occipitalised atlas and/or basilar invagination; it is hardly ever associated with instability.

Animals↗
PubMed1987

Skull radiograph measurements of normals and patients with basilar impression; use of Landzert's angle.

One hundred normal lateral skull radiographs were studied and those of ten patients with basilar impression attending Kenyatta Hospital, Nairobi. The mean shortest distance of the odontoid tip to McGregor's basal line was 1.2 +/- 2.28 mm below the basal line (range 6 mm below to 3 mm above basal line), in normals and 9 +/- 2.7 mm (6-14 mm) above basal line in patients. The mean basal angle was 113 degrees +/- 7 degrees (102 degrees-133 degrees) in normals and 122 degrees +/- 6 degrees (113 degrees-125 degrees) in patients. The mean nasion-basion-opisthion angle was 162 degrees +/- 4 degrees (154 degrees-169 degrees) in normals and 178 degrees +/- 5 degrees (173 degrees-185 degrees) in patients. The mean total length of clivus was 48 +/- 3.7 mm (43-56 mm) in normals and 44 +/- 6.6 (36-48 mm) in patients group. The mean median diameter of the foramen magnum was 39 +/- 5 mm (30-48 mm), atlas 21 +/- 3 mm (18-25 mm) axis 18 +/- 3 mm (14-23 mm), third cervical vertebra 16 +/- 2 mm (13-22 mm) in normals and in patients: 39 +/- 4 mm (36-45 mm), atlas 23 +/- 6 (15-30 mm) axis 19 +/- 4 mm (16-25 mm), third cervical vertebra 16 +/- 3 (14-20). There was a significant difference in the position of the odontoid tip and the nasion-basion-opisthion angle between the normal and patient groups. All the other parameters measured in this work did not differ significantly between the two groups.

Black People↗
PubMed1985

Brainstem auditory evoked potential (BAEP) abnormalities in subjects with craniovertebral malformations.

We report the data on 42 monaural BAEP recordings from 10 patients with craniovertebral malformations. Of 8 preoperative recordings from 3 patients 6 showed an increased I-III interpeak latency (IPL). Of 28 recordings taken shortly (1-12 months) after surgery 21 showed an abnormal I-III IPL. On the other hand, of 6 recordings from 3 patients 20 years after surgery 5 were in the normal range and 1 showed an abnormal I-III IPL. Although the most important site of craniovertebral malformations is the cervicomedullary junction, the results reported suggest unilateral or asymmetrical bilateral involvement of the medullary-pontine junction, on the evidence of BAEP wave III, in a large proportion of patients with these malformations.

Adult↗
PubMed2000

Polysomnographic respiratory findings in patients with Arnold-Chiari type I malformation and basilar invagination, with or without syringomyelia: preliminary report of a series of cases.

Patients with craniocervical disorders (CCD) show a wide variety of symptoms and signs suggesting cerebellar and/or high cervical lesion. The anatomic localization of respiratory centers and their possible injury may explain the presence of respiratory disturbances in these diseases. The aim of this preliminary study was to evaluate the polysomnographic findings in a group of patients with CCD, most of them with Arnold-Chiari malformation type I, since sleep apnea has been referred to in isolated cases in the literature. Eleven patients (seven females and four males) with CCD diagnosed by magnetic resonance imaging referred from the neurosurgery unit were submitted to clinical history, physical examination with sleep questionnaires, and scored on the Epworth Sleepiness Scale. Full night polysomnography was performed in an Oxford SAC system where EEG, electro-oculography, electrocardiography, chin and leg electromyography, chest and abdominal efforts, airflow, and oximetry were recorded continuously. Nine patients presented with Arnold-Chiari type I malformation, of whom six showed associated syringomyelia. The other two had basilar invagination. Ninety percent of these patients complained of sleep problems (snoring, choking, and witnessed apneas) and 72% presented hypersomnolence (ESS >9). The polysomnographic findings showed sleep fragmentation in 81% of the patients and a reduction of rapid eye movement sleep in 63%. The apnea/hypopnea index was above 5 in 72%, with a predominance of central apnea. Patients with craniocervical disorders present a higher probability of displaying sleep respiratory disturbances. Their sleep complaints should be assessed and patients should be submitted to an overnight sleep recording in order to identify sleep apnea.

Adult↗
PubMed2001

[Occipitocervical fusion in rheumatoid arthritis].

A case of spontaneous occipitocervical fusion is discussed. The authors believe that fusion at the occipitocervical junction will occasionally occur in rheumatoid arthritis in the adult population. As a result of fusion, the dens axis is unable to exert compression on the spinal cord with increased neck flexion.

Aged↗
PubMed2002

[Radiological changes in the cervical spine in rheumatoid arthritis -- prognostic factors obtained by a cross-sectional study].

OBJECTIVE: The involvement of the cervical spine is the most serious skeletal manifestation of rheumatoid arthritis (RA). Instabilities of the upper cervical spine can lead to neurological complications and signs of vertebrobasilar insufficiency. The study investigates the relationship between the course of the RA and the degree of radiological changes in the cervical spine and describes prognostic factors of cervical spine involvement. MATERIAL AND METHODS: Clinical data were gathered from 205 patients with RA. Standardized X-ray examination of the cervical spine was performed and arthritic changes were assessed by a semiquantitative score. The association of clinical symptoms with defined radiological changes was evaluated. RESULTS: Radiological changes in the cervical spine related to RA were found in 67.8% of the patients. Anterior atlantoaxial dislocations were detected in 25.4%, subaxial dislocations in 55.6% and a vertical displacement of the dens in 21.5% of the cases. The degree of radiological changes in the cervical spine was not directly correlated with neurological symptoms. Age above 55 years, disease duration of more than 10 years, an onset of cervical pain later than 11 years after the diagnosis of RA, steroid therapy for more than 5 years, bilateral shoulder involvement and the presence of severe erosive changes in at least one peripheral joint were positively associated with the development of severe changes in the cervical spine. In patients with basilar impression, paraesthesia in the upper extremities was significantly more common. CONCLUSION: Prognostic factors are helpful for the assessment of risk of cervical spine involvement in RA. Since definite clinical hints for cervical involvement are absent, the cervical spine has to be included in the routine examination of patients with RA.

Adult↗
PubMed1998

[Occipitocervical fusion in chronic polyarthritis].

This report will relate our experience with the y-plate, which we used in the treatment of 39 patients (32 women and 7 men) with a mean age of 62.6 years (range 47 to 79 years) between 1987 and 1994. All patients had rheumatoid arthritis. Occipitocervical fusion was indicated by instabilities within the occipitocervical region and in cases with additional basilar invagination and/or after transoral dens resection for decompression of the spinal cord. The length of the fusion depended on the pathological changes of the subaxial cervical spine. Before surgery, 35 patients suffered from strong and 4 patients from moderate pain in the neck and/or the back of the head. On a linear scale from 0 to 10, the pain was rated as 8:1 on average (range 4 to 10). 31 patients had an instability of the atlantoaxial region and 19 patients a basilar invagination of the odontoid. A cervical myelopathy was found in 20 cases. One surgeon fused the occiput to C2 in 22 cases, to C3-C5 in 8 cases and to C7-T2 in 9 cases. An important factor in this operation is the integration of atlantoaxial screws in order to resist the translational dislocation of C1/C2. In 13 patients a resection of the odontoid had to be performed to adequately decompress the spinal cord. A reduction of C2 without dens resection was performed in cases with reducible instability C1/2. 32 of the patients could be controlled with a minimum follow-up of 12 months (average 32.2 months, range 12 to 66 months). Out of the other 7, 6 patients had died. At the time of follow-up, the pain was rated as 2.3 on average (range 0 to 10). A myelopathy was present in 2 cases. Six patients required further operations on the cervical spine; 4 patients developed an instability at the level(s) below the fusion and an enlargement of the fusion to these levels has been performed. The fusion rate was 96.9%, despite breakage of the implant in 3 and a screw loosening in 2 patients. According to the criteria of Conaty, the result was satisfying in 25 (75%) and not satisfying in 8 patients (25%). These results show the effectiveness of the occipitocervical fusion with the y-plate in rheumatoid arthritis. A transoral dens resection is only indicated in cases with basilar invagination causing a compression of the spinal cord or in such cases where a compression caused by the dens or the retrodental pannus formation cannot be treated by a reduction of the second cervical vertebra alone.

Aged↗
PubMed2006

Basilar impression in osteogenesis imperfecta: can it be treated with halo traction and posterior fusion?

Basilar impression (BI) and hydrocephalus complicating osteogenesis imperfecta (OI) is usually treated by anterior transoral decompression and posterior fixation. Nevertheless, it may be questioned if posterior fusion following axial halo traction is adequate in patients with symptomatic BI complicating OI. We report on a case with progressive symptomatic hydrocephalus and BI complicating OI that was successfully treated by halo traction followed by posterior occipitocervical fusion. However, after a symptom free interval of 2 years the patient suffered from recurrence of symptomatic hydrocephalus needing additional ventriculoperitoneal (VP) shunt placement. In conclusion, posterior fusion without additional VP shunt placement may not be effective in the long term for ameliorating symptoms and signs and halting progressive hydrocephalus in BI complicating OI.

Adult↗
PubMed2000

Surgery for syringomyelia: an analysis based on 163 surgical cases.

OBJECT: The authors analyzed the cases of 163 patients with syringomyelia to assess the appropriate surgical procedure. METHODS: Depending on the aetiological factors and treatment considerations the series was classified into three groups. Group I were cases where there was no definite demonstrable aetiological factor; Group II cases had basilar invagination and/or Chiari malformation; and Group III consisted of cases where the syrinx was secondary to an obvious aetiology, such as a mass lesion either in the posterior cranial fossa or in the spine or a severe kyphotic spinal deformity. Post-traumatic syringomyelia and syrinx in association with spina bifida were not studied. CONCLUSIONS: We concluded that for Group I cases syringosubarachnoid shunting is the ideal form of treatment. In Group II cases foramen magnum bony decompression is satisfactory and physiological. Good results were obtained even in cases where either a foramen magnum decompression alone or in combination with a syringo-subarachnoid shunt was done. Only syringosubarachnoid shunt (without a foramen magnum decompression) in Group II cases was found to produce poor outcome. Group III cases should be treated for the primary aetiological problem. Only syrinx drainage procedure without treatment of aetiology in these cases produced poor results. It was observed that clinical outcome rather than radiological improvement is the reliable indicator of the surgical result.

Adolescent↗
PubMed2001

Trigeminal neuralgia associated with achondroplasia. Case report with literature review.

A 59-year-old male with a history of 2 years of typical trigeminal neuralgia manifested the characteristics of achondroplasia. X-ray and magnetic resonance imaging demonstrated basilar impression, deformity of the posterior fossa with marked asymmetry of petrous bone and compression of pons and trigeminal nerve by the left vertebral artery and anterior inferior cerebellar artery. Microvascular decompression was performed through a suboccipital craniectomy. The neuralgia disappeared soon after surgery and remains completely resolved until today. This is the first reported case of trigeminal neuralgia in a patient with achondroplasia. The deformity of the skull base was considered to influence the development of the trigeminal neuralgia.

Achondroplasia↗
PubMed2004

Circumferential decompression of the foramen magnum for the treatment of syringomyelia associated with basilar invagination.

Posterior fossa decompression utilizing suboccipital craniectomy and duraplasty remains the standard surgical treatment for Chiari-associated syringomyelia. In the presence of basilar invagination, anterior decompression, typically transoral odontoidectomy, or posterior decompression may be performed. We report two cases in which anterior and posterior (circumferential) decompression of the foramen magnum was used to treat cervical syringomyelia successfully. These cases demonstrate that circumferential decompression of the foramen magnum may be necessary in some cases of cervical syringomyelia associated with basilar invagination and Chiari malformation.

Adult↗
PubMed1994

Chronic ventilator use in osteogenesis imperfecta congenita with basilar impression: a case report.

Osteogenesis imperfecta, a rare connective tissue disorder, is known to be associated sometimes with the invagination of the basilar skull. This deformity may disturb respiratory function secondary to brain stem compression and hydrocephalus. In addition, the deformed thoracic cage and fragile ribs make pulmonary care more complicated. A case of 24-year-old man is presented with brain stem compression syndrome secondary to osteogenesis imperfecta congenita with basilar impression. He developed respiratory failure and became tracheostomy positive-pressure ventilator dependent at the age of 21 years. He also suffered multiple skeletal abnormalities and mental retardation, and following the brain stem compression, severe quadriparesis. The patient's condition is stable since he has been using the ventilator and he is currently living in the community.

Adult↗