CHIARI TYPE 1 MALFORMATION PRESENTING AS CHRONIC COUGH IN A MIDDLE-AGED FEMALE

Document Type

Conference Proceeding

Publication Date

6-17-2022

Publication Title

Journal of General Internal Medicine

Abstract

CASE: 39 year-old non-smoking female with history of iron deficiency anemia presented to clinic with chronic cough of 5 years duration, occurring daily with no identifiable exacerbating factors. She tried Flonase, Claritin, and omeprazole without improvement. Methacholine challenge testing, PFTs, and CT chest were unremarkable. A trial of inhalers did not bring about any symptomatic relief. Allergy testing, esophagram, pH monitoring, and laryngoscopy were all unremarkable. Patient began to experience syncopal episodes during her coughing spells. Echocardiogram and holter monitor showed no abnormalities. Patient was then evaluated by neurology, who found unremarkable EEG and CT head. MRI brain revealed a significant Chiari 1 malformation. She was referred to neurosurgery and underwent Chiari decompression with patch duraplasty with subsequent resolution of her cough and syncopal spells. IMPACT/DISCUSSION: Chronic cough (longer than eight weeks) is a common complaint in outpatient medicine. In adults with normal lung physiology, upper airway cough syndrome, asthma and gastroesophageal reflux are responsible for 90% of cases. A trial of empiric treatment should be attempted but if there is no resolution of symptoms, less common causes should be explored. This patient's concurrent syncope prompted assessment of cardiogenic and neurogenic etiologies. Chiari malformations are anatomic anomalies of the craniocervical junction with downward displacement of the cerebellar structures. They are largely classified into four groups. Chiari type 1 malformation (CM1) is the most common and is defined by abnormally shaped cerebellar tonsils displaced below the level of the foramen magnum. This patient's age at presentation is atypical, as most occur at approximately 18 years. The most common symptom is headache due to meningeal irritation exacerbated by cough and other valsalva maneuvers. Brainstem compression may also cause hoarseness, vocal cord paralysis, recurrent aspiration. Less common manifestations include syncope, weakness and hiccups. Diagnosis is best made by MRI. Treatment for symptomatic patients is ideally through decompression surgery. CONCLUSION: Chronic cough is most often related to pathologies related to GERD, asthma or upper airway cough syndrome. However, there are significant and correctable more rare pathologies that the clinician should consider. Chiari malformations should be considered in patients with unexplained chronic cough who may have concurrent headache, aspiration or hoarseness. Less common symptoms include syncope. After diagnosis via MRI, correction of Chiari malformation can be performed via decompression surgery.

Volume

37

Issue

Suppl 2

First Page

S358

Last Page

S359

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