18 January 2023 : Case report
[In Press] Reversible Cerebral Vasoconstriction Syndrome Associated with Vasospasm of the Colic Arteries and Possibly Coronary Vasospasm
Challenging differential diagnosis, Rare diseaseHikaru Kitamura1ABCDEF, Toshinori Nishizawa 12ABCDEF, Gautam Anil Deshpande13ABCDEF, Hiroko Arioka1ABCDEF
Am J Case Rep In Press; DOI: 10.12659/AJCR.938322
Available online: 2023-01-18, In Press, Corrected Proof
Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule
Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder characterized by recurrent thunderclap headaches and reversible cerebral vasoconstriction.
Herein, we present the case of a man in his forties with a disease entity related to RCVS accompanied by vasospasm of the extracerebral blood vessels throughout the body. The patient presented to the Emergency Department with a severe headache and epigastric pain. Initially receiving a misdiagnosis of functional pain, he continued to experience severe recurrent headaches, most often after urinating or defecating and was referred to our department. Suspecting RCVS, we performed magnetic resonance angiography, which revealed beaded irregularity in the right anterior cerebral and V4 vertebral arteries. The patient also had epigastric pain that coincided with each headache. Electrocardiography revealed pronounced ST-segment elevation in leads I and aVL and inverted T wave in lead III, while abdominal computed tomography angiography showed narrowing of the colic arteries. We named this disease “reversible systemic vasoconstriction syndrome” (RSVS) as a potential suggested terminology for the future.
RSVS is a clinical syndrome characterized by thunderclap headaches and simultaneous unbearable pain in extracerebral organs. To the best of our knowledge, this is the first case report of RCVS with coronary and colic artery vasospasm. We need to take great care of patients with chest or abdominal pain accompanied by recurrent thunderclap headaches, since they can be misdiagnosed with functional or psychogenic disorders.
Keywords: Headache Disorders, Primary; Vasospasm, Intracranial; Cerebrovascular Disorders
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