What do we know about syncope (fainting) in persons with the Chiari I malformation (CM-I)? In this post, I will discuss some of the medical reports published on this topic, and, in Part 2 will summarize the cases and discuss why these spells occurred.
The first report on fainting in CM-I was published by Corbett et al. in 1976. In his report, ‘Sneeze syncope’, basilar invagination, and Arnold-Chiari type 1 malformation, Corbett described an adult man with CM-I who would faint following a sneeze.
Two years later, Dobkin described an adult with CM-I with repeated fainting episodes that occurred when he moved his neck in extension (looking up and back). An EEG showed no evidence of seizures.
In 1982, Hampton, et al. presented three persons with CM-I, syringomyelia, and syncope. A 52 y/o woman would blackout without warning while standing. She only had one fainting spell after surgical treatment of the Chiari malformation. A 20 y/o man presented with a 3-month history of blackouts, always preceded by headache. A 21 y/o man developed a headache quickly followed by fainting when he strained to urinate. In both men, the fainting spells resolved following posterior fossa decompression.
In 1991, Weig et al. described two persons with frequent syncope and CM-I. A 23 y/o woman would develop vertigo, become dazed, and “within seconds”, would lose consciousness. She regained consciousness within 1-2 minutes. Most of her episodes occurred following movement of the head. Prior to the discovery of the Chiari malformation, she had been considered to have complex partial seizures or basilar migraine. Her EEG was normal. The fainting spells resolved following posterior fossa decompression. The second person, a 22 y/o man, developed weakness in his arms with coughing and then fainted for 30 to 60 seconds. His EEG was normal and the spells also resolved following surgical decompression.
In 1992, Alarcon et al. reported a 66 y/o woman with CM-I and syringomyelia whose symptoms included headache, syncope, blurred vision, and “an "electric-like paroxysmal tingling of the hands” that occurred with coughing.
In 1994, Alegre et al. described a 31 y/o man, who, following “brisk head movement,” lost consciousness, became apneic (stopped breathing), and suffered a cardiac arrest.
In 1995, Martinez Soto et al. reported spontaneous recurrent syncope as the sole symptom of CM-I. Symptoms disappeared following treatment of the Chiari I malformation by posterior fossa decompression.
In 1999, Ziegler & Mallonee report a case of sudden death in a person with “repeated attacks of headache and syncope.” Diagnosis had been basilar migraine prior to discovery of CM-I and syringomyelia. A Chiari I malformation and syringomyelia was discovered at autopsy.
In 2002, Aguiar et al. described a person with glossopharyngeal neuralgia and “episodes of syncope when eating or swallowing.”
In 2005, Prilipko et al. reported the first case of a person with CM-I with syncope and postural orthostatic tachycardia syndrome (POTS).
These reports provide a picture of clinical presentation of persons whose fainting spells are related to the Chair I malformation. In Part 2, I will summarize the cases and discuss why the fainting spells occurred.
Links
Corbett
http://www.ncbi.nlm.nih.gov/pubmed/932754?ordinalpos=31&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Dobkin
http://www.ncbi.nlm.nih.gov/pubmed?term=Chiari%20syncope%20AND%20dobkin%5BAuthor%5D
Hamptom
http://www.ncbi.nlm.nih.gov/pubmed/7143010?ordinalpos=29&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Weig
http://www.ncbi.nlm.nih.gov/pubmed/1922816?ordinalpos=20&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Alarcorn
http://www.ncbi.nlm.nih.gov/pubmed/1596400?ordinalpos=19&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Alegre
http://www.ncbi.nlm.nih.gov/pubmed/7814184?ordinalpos=16&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Martinez Soto
http://www.ncbi.nlm.nih.gov/pubmed/7786546?ordinalpos=15&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Zeigler
http://www.ncbi.nlm.nih.gov/pubmed/15613193?ordinalpos=5&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Prilipko
http://www.ncbi.nlm.nih.gov/pubmed/15965223?ordinalpos=3&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum