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Atlantoaxial Instability in Individuals with Down Syndrome: Multimedia

Author: Norberto Alvarez, MD, Assistant Professor, Department of Neurology, Harvard Medical School; Consulting Staff, Department of Neurology, Boston Children's Hospital
Contributor Information and Disclosures

Updated: Dec 8, 2008

Multimedia

This radiograph shows the normal relationships be...
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Media file 1: This radiograph shows the normal relationships between the anterior arch of C1 and the odontoid bone, and the odontoid bone and the posterior arch of C1.
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This radiograph shows the normal relationships be...

This radiograph shows the normal relationships between the anterior arch of C1 and the odontoid bone, and the odontoid bone and the posterior arch of C1.

In this 28-year-old woman with Down syndrome conf...
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Media file 2: In this 28-year-old woman with Down syndrome confirmed by chromosomal analysis, routine lateral cervical radiograph showed a mild degree of subluxation. The patient had no clinical signs of cord compression.
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In this 28-year-old woman with Down syndrome conf...

In this 28-year-old woman with Down syndrome confirmed by chromosomal analysis, routine lateral cervical radiograph showed a mild degree of subluxation. The patient had no clinical signs of cord compression.

This radiograph is of the same patient shown in I...
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Media file 3: This radiograph is of the same patient shown in Image 2, taken 8 years later when the patient, a woman with Down syndrome confirmed by chromosomal analysis, was aged 36 years. Routine lateral cervical radiograph taken at age 28 years showed a mild degree of subluxation, but the patient had no clinical signs of cord compression. Further clinical and radiologic follow-up showed no change either in the clinical picture or in the radiographs. This radiograph, taken in a fairly neutral position since the patient did not allow for good flexion and extension positions, shows that the anterior subluxation, approximately 7-8 mm between the anterior arch of C1 and the odontoid bone, is still present. The distance between the posterior arch of C1 and the odontoid bone is approximately 12 mm, allowing enough space for the cervical spine.
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This radiograph is of the same patient shown in I...

This radiograph is of the same patient shown in Image 2, taken 8 years later when the patient, a woman with Down syndrome confirmed by chromosomal analysis, was aged 36 years. Routine lateral cervical radiograph taken at age 28 years showed a mild degree of subluxation, but the patient had no clinical signs of cord compression. Further clinical and radiologic follow-up showed no change either in the clinical picture or in the radiographs. This radiograph, taken in a fairly neutral position since the patient did not allow for good flexion and extension positions, shows that the anterior subluxation, approximately 7-8 mm between the anterior arch of C1 and the odontoid bone, is still present. The distance between the posterior arch of C1 and the odontoid bone is approximately 12 mm, allowing enough space for the cervical spine.

This female with Down syndrome due to chromosomal...
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Media file 4: This female with Down syndrome due to chromosomal translocation developed progressive gait deterioration, weakness, loss of muscle tone in the legs, and increased deep tendon reflexes in both arms and legs. Radiographs of the cervical spine documented a marked subluxation. She underwent fusion of the cervical spine.
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This female with Down syndrome due to chromosomal...

This female with Down syndrome due to chromosomal translocation developed progressive gait deterioration, weakness, loss of muscle tone in the legs, and increased deep tendon reflexes in both arms and legs. Radiographs of the cervical spine documented a marked subluxation. She underwent fusion of the cervical spine.

This female with Down syndrome due to chromosomal...
(Enlarge Image)
Media file 5: This female with Down syndrome due to chromosomal translocation, also shown in Image 4, developed progressive gait deterioration, weakness, loss of muscle tone in the legs, and increased deep tendon reflexes in both arms and legs. After undergoing fusion of the cervical spine, follow-up radiograph revealed a distance between the anterior arch of C1 and the odontoid bone of almost 10 mm. The posterior distance was difficult to evaluate because of the wires. This radiograph was taken after the patient underwent a second operation because of residual instability.
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This female with Down syndrome due to chromosomal...

This female with Down syndrome due to chromosomal translocation, also shown in Image 4, developed progressive gait deterioration, weakness, loss of muscle tone in the legs, and increased deep tendon reflexes in both arms and legs. After undergoing fusion of the cervical spine, follow-up radiograph revealed a distance between the anterior arch of C1 and the odontoid bone of almost 10 mm. The posterior distance was difficult to evaluate because of the wires. This radiograph was taken after the patient underwent a second operation because of residual instability.

More on Atlantoaxial Instability in Individuals with Down Syndrome

Overview: Atlantoaxial Instability in Individuals with Down Syndrome
Differential Diagnoses & Workup: Atlantoaxial Instability in Individuals with Down Syndrome
Treatment & Medication: Atlantoaxial Instability in Individuals with Down Syndrome
Follow-up: Atlantoaxial Instability in Individuals with Down Syndrome
Multimedia: Atlantoaxial Instability in Individuals with Down Syndrome
References
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Further Reading

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Keywords

atlantoaxial instability, Down syndrome, atlantooccipital joints, atlanto-occipital joints, occipitocervical articulation, AAI, DS, brainstem syndrome

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Contributor Information and Disclosures

Author

Norberto Alvarez, MD, Assistant Professor, Department of Neurology, Harvard Medical School; Consulting Staff, Department of Neurology, Boston Children's Hospital
Norberto Alvarez, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Medical Editor

Michael J Schneck, MD, Associate Professor, Departments of Neurology and Neurosurgery, Stritch School of Medicine, Loyola University; Associate Director, Stroke Program, Director, Neurology Intensive Care Program, Medical Director, Neurosciences ICU, Loyola University Medical Center
Michael J Schneck, MD is a member of the following medical societies: American Academy of Neurology, American Society of Neuroimaging, Neurocritical Care Society, and Stroke Council of the American Heart Association
Disclosure: boehringer-ingelheim Honoraria Speaking and teaching; sanofi/bms Honoraria Speaking and teaching; pfizer Honoraria Speaking and teaching; genentech Honoraria Speaking and teaching; ucb pharma Honoraria Speaking and teaching; talecris Consulting fee Other; nmt medical  Independent contractor; NIH Grant/research funds Independent contractor; vernalis Grant/research funds Independent contractor; sanofi Grant/research funds Independent contractor

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Neurology, Division of Pediatrics, Department of Pediatrics, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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