| Reversible cerebral vasoconstriction syndrome | |
|---|---|
| Classification and external resources | |
| ICD-10 | I67.8 |
Reversible cerebral vasoconstriction syndrome (RCVS, sometimes called Call-Fleming syndrome or by a number of other names) is a poorly understood disease in which the arteries of the brain develop vasospasm without a clear cause (such as hemorrhage or trauma).[1] It may be present in varying amounts for months at a time.[1] Vasospasm narrows arteries and can trigger severe headaches that wax and wane. When the vasospasms subside the headaches are relieved. Although the pathology is not known definitively, it is thought SSRIs, uncontrolled hypertension, endocrine abnormality, and neurosurgical trauma are indicated to potentially cause vasospasm.[2]
Symptoms result from vasospasms that narrow arteries, especially those around the circle of Willis, which can lead to a dramatic headaches that are often of the thunderclap headache (sudden-onset) character. Ischemia is thought to cause various lesions and upper motor neuron damage in these patients which presents 3–4 days after migraine onset as focal neurological symptoms such as dysarthria, unilateral weakness, unsteady gait, and/or hyperreflexia.[1]
This condition features the unique property that the patient's cerebral arteries can spontaneously constrict and relax back and forth over a period of time without intervention and without clinical findings. Vasospasm is common post subarachnoid hemorrhage and cerebral aneurysm but in RCVS only 25% of patients have symptoms post subarachnoid hemorrhage.[3]
Calcium channel blockers (nimodipine,[4] verapamil which acts as a phenylalkylamine) reduce the calcium influx through L-type calcium channels in smooth muscle cells. This leads to the vasodilation of arteries, which relieves symptoms and can act to prevent vasospasm.
The disease carries the name of Gregory Call and Marie Fleming, the first authors of the 1988 report in which doctors from Massachusetts General Hospital in Boston, Massachusetts (including Miller Fisher) described four patients with the characteristic symptoms and abnormal cerebral angiogram findings.[1]