Cough Syncope, a rare presenting symptom of Chiari I Malform
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Chiari I malformation (CMI) and atlanto-occipital assimilation are both fairly rare conditions. The prevalence of CMI is estimated to range between 1/1,000 to 1/5,000 individuals and the incidence of atlanto-occipital assimilation varies from 0.08 to 3.6%.

It is important to bear CMI in mind in the differential diagnosis of cough syncope, since there is an effective treatment that improves the patient’s symptoms and quality of life.

A 62-year-old male reported to pulmonology department due to a 1-year history of recurrent episodes of syncope associated with cough during viral respiratory infections. He also complained of episodic occipital headaches and neck pain that responded to standard anti-inflammatory agents.

He had never smoked and his past medical history included hypertension and hyperlipidaemia, treated with valsartan 80 mg OD and simvastatin 20 mg OD. Further investigation, including a paranasal and pharyngeal CT scan, was carried out. These scans incidentally revealed, besides signs of chronic rhinosinusitis, low positioning of the cerebellar tonsils through the foramen magnum, suggesting Chiari malformation.

Cerebral MRI was performed to confirm the diagnosis, which revealed a partial occipitalization of the C1 vertebra with inferior cerebellar tonsillar herniation measuring 8 mm below the foramen magnum. The patient was diagnosed with CMI and atlanto-occipital assimilation.

He was subsequently referred to the neurosurgery outpatient clinic, where he was submitted to decompression surgery consisting of a suboccipital craniotomy, removal of the C1 posterior arch and duraplasty, with clinical improvement. After surgery, the patient denied further syncopal episodes and reported improvement of his occipital and neck pain.

Source: https://www.ejcrim.com/index.php/EJCRIM/article/view/1466/1970
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