Cough Syncope, a Rare Presenting Symptom of Chiari I Malformation and Atlanto-Occipital Assimilation

Chiari I malformation and atlanto-occipital assimilation are both fairly uncommon conditions. Symptoms usually present during adolescence or adulthood, typically consisting of headache or neck pain. Cough-associated syncope is an unusual presenting symptom. The diagnosis of this condition in a pulmonology department is even rarer. We report the case of a 62-year-old male referred to our pulmonology department due to complaints of cough-associated syncope. After several examinations, a pharyngeal CT scan incidentally showed low positioning of the cerebellar tonsils. Cerebral MRI confirmed the diagnosis of Chiari I malformation and atlanto-occipital assimilation and the patient was effectively treated with surgical decompression.

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%.The most frequent clinical presentation is a paroxysmal headache or neck pain. Cough syncope is an unusual form of presentation, described in few cases.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.