Symptomatic atlantoaxial dislocation in Down's syndrome

Chaudhry, Vinay; Sturgeon, Carol; Gates, Abigail; Myers, Gary

doi:10.1002/ana.410210616

Cited by 28 publications

(13 citation statements)

References 10 publications

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“…Imaging of the cord by MRI can be helpful in diagnosis. The prognosis depends upon the severity of SCI, the presence of associated bony abnormalities [Chaudhry et al, 1987], and perhaps prompt treatment (see below). Ligamentous laxity might also predispose to the development of AOD, which can also result in SCI [Hungerford et al, 1981;Martich et al, 1992].…”

Section: Discussionmentioning

confidence: 99%

New onset focal weakness in children with Down syndrome

Worley

Shbarou

Heffner

et al. 2004

American J of Med Genetics Pt A

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New onset focal weakness is relatively common in patients with Down syndrome (DS), and has broad differential diagnosis. Ten cases of new onset focal weakness in patients with DS were encountered or are currently being followed in two DS clinics, with a combined population of patients of approximately 850, for a clinic population prevalence of 1.2%. The median age at presentation was 4 years old (range 1 month-44 years). The causes of new onset focal weakness were: stroke from Moyamoya disease (two patients); stroke from vaso occlusive disease (one patient); stroke from venus sinus thrombosis (one patient); traumatic subdural hematoma (one patient); brain abscess (one patient); spinal cord injury (SCI) from cervical spinal stenosis (two patients); SCI from atlantoaxial instability (AAI) (one patient); and brachial plexus injury (one patient). Of the 10 patients with focal weakness, 8 had potentially treatable conditions, and 5 had surgery. The differential diagnosis of new onset focal weakness in DS is broad, with diseases reported involving all levels of the nervous system from brain to muscle. For some diagnoses, expeditious diagnosis may improve outcome.

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Section: Discussionmentioning

confidence: 99%

New onset focal weakness in children with Down syndrome

Worley

Shbarou

Heffner

et al. 2004

American J of Med Genetics Pt A

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“…1 Surgical treatment for reduction of the subluxation, ®xation and fusion are mandatory for symptomatic cases.…”

Section: Discussionmentioning

confidence: 99%

Occiput-cervical fusion for symptomatic atlantoaxial subluxation in a 32-month-old child with Down syndrome: a case report

Tseng

Cheng

1998

Spinal Cord

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Atlantoaxial subluxation in Down syndrome rarely becomes neurologically symptomatic in very young children. The authors present a 32-month-old girl with Down syndrome, who had tetraporesis due to an atlantoaxial subluxation. She was treated with halo immobilization and partial reduction of the subluxation at ®rst, followed by removal of posterior arch of the atlas and posterior fusion with wire ®xation. Halo immobilization was continued for 3 months after operation. After 1-year follow-up, her motor functions were normal, and the dynamic roentgenogram of the cervical spines showed good stability. The authors recommend posterior fusion and postoperative halo immobilization for the treatment of the symptomatic atlantoaxial subluxation in young Down syndrome patients, even in a 32-month-old child.

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“…To that end, the Academy reviewed 41 separate cases regarding sudden, significant spinal cord injuries in children with Down's syndrome. 5,613,14,15,25,29 These cases provide ample documentation that those injuries can occur, but does not provide illumination as to how to prevent or treat them. In their summary they state: "Almost all symptomatic patients had their first radiograph at the time the symptoms were recognized, so it is generally unknown whether asymptomatic atlanto-axial instability (AAI) progresses to symptomatic AAI with or without trauma."…”

Section: Down's Syndrome and The Special Olympicsmentioning

confidence: 99%

Down’s Syndrome and Craniovertebral Instability: Topic Review and Treatment Recommendations

Brockmeyer

2005

Nep J Neurosci

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Down’s syndrome, is the most common inherited chromosomal disorder in humans occurring in 1.5 in every 1,000 live births and is characterized by multiple neurological as well as nonneurological abnormalities. The issue of craniovertebral instability in Down’s syndrome patients is a very controversial topic. Multiple studies and editorials have been written over the last two decades and much of the information is conflicting and confusing. The goal of this review is to provide a rational synthesis of this previous work and provide recommendations by which parents of Down’s syndrome patients, pediatricians, family practitioners and other health professionals may make intelligent choices regarding screening of Down’s syndrome patients. It is a second goal of this review to synthesize the surgical data on this topic and provide recommendations for anesthesiologists, otolaryngologists, orthopedic surgeons and neurosurgeons. Nepal Journal of Neuroscience, Volume 2, Number 1, 2005, Page: 52-58

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Symptomatic atlantoaxial dislocation in Down's syndrome

Cited by 28 publications

References 10 publications

New onset focal weakness in children with Down syndrome

New onset focal weakness in children with Down syndrome

Occiput-cervical fusion for symptomatic atlantoaxial subluxation in a 32-month-old child with Down syndrome: a case report

Down’s Syndrome and Craniovertebral Instability: Topic Review and Treatment Recommendations

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