Aqueductal stenosis and mental retardation associated with O<sub> S</sub>  odontoideum, "hypermobile" atlantoaxial dislocation and congenital stenosis of atlas leading to spastic quadriparesis

Das, Rupant K; Behari, Sanjay; Singhal, Namit; Jaiswal, Awadesh K.; Mahapatra, Ashok Kumar

doi:10.4103/1817-1745.31999

J Pediatr Neurosci

2007

DOI: 10.4103/1817-1745.31999

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Aqueductal stenosis and mental retardation associated with O_S odontoideum, "hypermobile" atlantoaxial dislocation and congenital stenosis of atlas leading to spastic quadriparesis

Rupant K Das

Sanjay Behari

Namit Singhal

et al.

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The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions

Flanagan

2015

Neurology Research International

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The craniocervical junction (CCJ) is a potential choke point for craniospinal hydrodynamics and may play a causative or contributory role in the pathogenesis and progression of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, MS, and ALS, as well as many other neurological conditions including hydrocephalus, idiopathic intracranial hypertension, migraines, seizures, silent-strokes, affective disorders, schizophrenia, and psychosis. The purpose of this paper is to provide an overview of the critical role of the CCJ in craniospinal hydrodynamics and to stimulate further research that may lead to new approaches for the prevention and treatment of the above neurodegenerative and neurological conditions.

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The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions

Flanagan

2015

Neurology Research International

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Os odontoideum with “free-floating” atlantal arch causing Cl-2 anterolisthesis and retrolisthesis with cervicomedullary compression

Behari

Jaiswal

Srivastava

et al. 2010

IJOO

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Background:Os odontoideum (OO) with C1-2 anterolisthesis and retrolisthesis may cause cervicomedullary injury both from anterior and posterior aspects. We analyzed fourteen such patients for biomechanical issues, radiological features and management of OO with free-floating atlantal arch and review pertinent literature.Materials and Methods:Fourteen patients having nonsyndromic, reducible atlantoaxial dislocation (AAD) with orthotopic OO were analyzed. During neck flexion, their C1 anterior arch-os complex displaced anteriorly relative to remnant odontoid-C2 body. The posteriorly directed hypoplastic remnant odontoid sliding below the atlas and forward translation of the C1 posterior arch caused concomitant cervicomedullary compression. During neck extension, there was retrolisthesis of the “free-floating” C1 arch-os complex into spinal canal. Spinal stenosis and lateral C1-2 facet dislocation; Klippel-Feil anomaly; and posterior circulation infarcts were also present in one patient each, respectively. Posterior C1-2 (n=10) or occipitocervical fusion (n=3) was performed in neutral position to stabilize atlantoaxial movements.Results:Follow-up (mean, 3.9 years) assessment revealed improvement in spasticity and weakness in 13 patients. One patient had neurological deterioration following C1-2 posterior sublaminar fusion, requiring its conversion to occipitocervical contoured rod fusion. One patient with posterior circulation stroke died prior to any operative intervention. Follow-up lateral view radiographs showed a bony union or a stable construct in these 13 patients.Conclusions:OO with free-floating atlantal arch may precipitate cord injury both during neck flexion and extension. This condition may be overlooked unless lateral radiographs of craniovertebral junction are undertaken in neck extension, along with the usual ones in neutral and flexed positions. Etiological factors include C1 ring-OO unrestrained movements above the hypoplastic odontoid; upward pull on OO by alar and apical ligaments; lax C1-2 facet joint ligaments; and congenital presence of horizontal facet joint surfaces that facilitates C1-2 translation.

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Aqueductal stenosis and mental retardation associated with O_S odontoideum, "hypermobile" atlantoaxial dislocation and congenital stenosis of atlas leading to spastic quadriparesis

Cited by 2 publications

References 0 publications

The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions

The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions

Os odontoideum with “free-floating” atlantal arch causing Cl-2 anterolisthesis and retrolisthesis with cervicomedullary compression

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Aqueductal stenosis and mental retardation associated with O S odontoideum, "hypermobile" atlantoaxial dislocation and congenital stenosis of atlas leading to spastic quadriparesis

Cited by 2 publications

References 0 publications

The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions

The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions

Os odontoideum with “free-floating” atlantal arch causing Cl-2 anterolisthesis and retrolisthesis with cervicomedullary compression

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Aqueductal stenosis and mental retardation associated with O_S odontoideum, "hypermobile" atlantoaxial dislocation and congenital stenosis of atlas leading to spastic quadriparesis