J Korean Soc Emerg Med.  2006 Jun;17(3):277-279.

Syncope Associated with Type 1 Arnold-Chiari Malformation

• Park SA
• Lim H
• Park JH
• Lee TK
• Sung KB

Abstract

A type I Arnold-Chiari malformation (ACM1) is characterized by herniation of cerebellar tonsils at least 3 mm below the plane of the foramen magnum and can present with various clinical symptoms, frequently occipital headache, lower cranial nerve palsy, or ataxia. However, syncope has rarely been described as an initial manifestation of ACM1. An 18-year-old girl experienced a falling to the ground with a loss of consciousness for a minute. The fall occurred with a sensation of vertigo when she was extending her neck to pick up a shuttlecock. Electroencephalography, autonomic function tests, and echocardiography did not demonstrate abnormalities. An magnetic resonance image showed a ACM1. A second-degree atrioventricular block was noted on the initial 24-hour EKG, but not on the second and the third ones taken after neck movement had been restricted by a brace. The initiation of syncope with a sensation of vertigo during excessive head movement and the absence of any other common causes of syncope except for the transient second-degree atrioventricular block disappearing after restriction of neck movement raise the possibility that the syncope was due to the ACM1. Therefore, we recommend that a differential diagnosis of syncope should include ACM1 on the initial evaluation.

Keyword

Arnold-Chiari Malformation; Magnetic Resonance Imaging; Syncope

MeSH Terms

Adolescent
Arnold-Chiari Malformation*
Ataxia
Atrioventricular Block
Braces
Cranial Nerve Diseases
Diagnosis, Differential
Echocardiography
Electrocardiography
Electroencephalography
Female
Foramen Magnum
Head Movements
Headache
Humans
Magnetic Resonance Imaging
Neck
Palatine Tonsil
Sensation
Syncope*
Unconsciousness
Vertigo