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  • Title: Chiari I malformation associated with atlanto-occipital assimilation presenting as orthopnea and cough syncope.
    Author: Mangubat EZ, Wilson T, Mitchell BA, Byrne RW.
    Journal: J Clin Neurosci; 2014 Feb; 21(2):320-3. PubMed ID: 24080068.
    Abstract:
    Although it is not uncommon for patients with Chiari I malformations to present with respiratory complaints, cough syncope is a rare presenting symptom. We report an adult patient who had both a Chiari I malformation and atlanto-occipital assimilation, and complained of cough syncope, orthopnea, and central sleep apnea. The patient underwent decompressive craniectomy of the posterior fossa and a cervical level 2 laminectomy. However, due to an initial under-appreciation of the profound narrowing of the foramen magnum as a result of these concomitant pathologies, the patient had continued impaired cerebrospinal fluid flow, leading to a symptomatic pseudomeningocele and required a more extensive decompression that included a cervical level 3 laminectomy as well as a temporary lumbar drain. On 2 year follow-up, he remained asymptomatic.
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