VCOM Carolinas Research Day 2023
Clinical Case-Based Reports
Type I Chiari Malformation Presenting with Syncope, Bradycardia and Complex Migraines Brooke Reese OMS-III, Jennifer Horawski, MD, Lisa Wright, MD Edward Via College of Osteopathic Medicine – Carolinas Campus, Spartanburg, SC
Abstract # CBR-15
Introduction Title: Type I Chiari malformation presenting in a 48-year-old female with syncope, bradycardia, and complex migraines. Context/Impact: Chiari malformations are an uncommon disease with a prevalence of less than 1% in the United States. We present a 48-year-old female who presented to the emergency department following a syncopal episode. On cardiac workup, the patient was found to have Mobitz Type I AV block. The patient was discharged with a pacemaker but continued to suffer from complex migraines. One year later, the patient was found to have a Chiari malformation on brain MRI. Report of Case: A 48 year-old female with a past medical history of hypertension, hyperlipidemia, non-insulin dependent type II diabetes mellitus and complex migraines presented to the emergency department following a syncopal episode. The patient had been seen in the emergency department three months prior for migraines which she described as a burning sensation that involved the left side of her face and head. She reported the same burning sensation immediately prior to her current syncopal episode. Her husband reported she was eating when she no longer could speak, starred at her hands, loss consciousness and fell. Emergency medical services arrived at the house, found long pauses in her heart rhythm, and administered of 0.5 mg of atropine which improved her heart rate into the sixties. Upon arrival the to the emergency department the patient was stable but still unresponsive. Her mental status did improve, and she was able to answer questions and follow commands. At that time, she was found to have left sided upper and lower extremity weakness, left upper extremity drift and a National Institute of Health Stroke Score of 2. She was admitted with a working diagnosis of bradycardia with concern for transient ischemic attack. The patient underwent a CT head, CTA head and CTA neck which were unremarkable. Cardiology evaluated the patient for bradycardia and diagnosed Mobitz Type I. The patient was given a pacemaker and was discharged. She continued to suffer from migraines that originated in the roof of her mouth, travel up the left side of her face and were associated with chest pressure and palpitations. These migraines occurred consistently for a year following her hospital admission. This prompted a brain MRI from the patient’s primary care provider which disclosed cerebellar herniation 8 mm below the foramen magnum. Comments/Conclusion: Although relatively uncommon, Chiari Malformation should be considered in a patient presenting with atypical chest pain, palpitations, hypertension, headache, and bradycardia for an extended period. Considering a brain MRI in these patients is essential to rule out this diagnosis. Currently, there has been little reported on the connection between sinus bradycardia and Chiari malformation. • A Chiari malformation is a condition in which brain tissue extends downward into the spinal canal. This malformation can be further classified into four types, with type I being the most common. • Prevalence estimated to be slightly less that 1 in 1,000. • Type I is often asymptomatic. Anomalies of the skull base and spine are seen in 30%-50% of these patients. • Cervical cord syrinx is seen in about 35% of Type I cases; more common in symptomatic patients. • Symptoms of Type I Chiari Malformation can include: • bradycardia • drop attacks • severe head and neck pain • loss of pain and temperature in upper torso and arms • weakness in arms and hands • spasticity • dizziness and balance problems
• While uncommon, current literature has determined a causal relationship between Chiari Type I and sinus bradycardia. However, current research has not reported on a connection between Chiari Type I and Mobitz Type I. • Because this patient experienced simultaneous complex migraine symptoms, chest pressure and palpitations on multiple occasions leads us to believe there is possible correlation between this patient’s brain anomaly and cardiac arrythmia. • Studies have shown that clinical presentations of Chiari Type I like sinus bradycardia and hypertension can be resolved by decompressive surgery. Our patient has chosen to forgo surgery at this time.
Figure 1: Patient Timeline
ʹͲʹͳ • ED VIST • ǡ ʹͲʹͳ • ED VISIT • Ǣ ǡ ʹͲʹʹ • PRIMARY CARE FOLLOW UP • ǡ ʹͲʹʹ • MRI BRAIN W*WO CONTRAST • ͺ Ǣ ʹͲʹʹ • CERVICAL SPINE MRI • ͶǦͷǡ ͷǦǡ Ǧǡ Ǧ ͳ Figure depicts the presentation timeline of a 48-year-old female patient with a past medical history of hypertension, hyperlipidemia, and Type II Diabetes Mellitus.
Figure 3: Mobitz Type I
MRI Arnold Chiari Malformation Types I-IV
Figure shows progressive prolongation of the PR interval before a dropped beat. Mobitz I is usually a benign arrythmia with low risk of progression to 3 rd degree block.
• Localization of the autonomous cardiovascular centers in the brainstem raises the question whether the Type I Chiari Malformation had any connection with the Mobitz Type I AV block. • The occurrence of these two pathologies concurrently may suggest a link between brain stem compression and arrythmias and warrants further investigation.
• blurred vision • photophobia • sleep apnea
[A] CM I, downward displacement of the cerebella tonsils by > 4 mm beneath the foramen magnum into the cervical spinal canal. [B] CM II, downward displacement of the medulla, fourth ventricle and cerebellum into the cervical spinal canal, as well as elongation of the pons and fourth ventricle. [C] CM III, includes a form of dysraphism with a portion of the cerebellum and/or brainstem pushing out through a defect in the back of the head or neck. [D] CM IV, cerebellum fails to develop normally.
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