A Man with Sickle Cell Disease and Headache

Approximately 17% of patients with sickle cell disease have cerebrovascular symptoms; 75% of those patients have cerebral infarction, 20% have either cerebral or subarachnoid hemorrhage, and a small percentage have cerebral sinovenous thrombosis.

A 22-year-old man with sickle cell disease presented with headache and difficulty speaking after smoking marijuana. He had anomia, apraxia, and alexia and was unable to perform arithmetic. Diagnostic tests were performed, and management decisions were made. A new Case Record summarizes.

• What are the most common causes of stroke in young patients?

The most common causes of stroke in young patients are cardioembolism, dissection, migraine, angiopathies of various types, and other causes, including coagulopathies associated with the use of oral contraceptives or with the presence of antiphospholipid antibodies.

Clinical Pearl

• What are the characteristics of strokes associated with cannabis use?

Cannabis use is often considered in cases of cerebral vasoconstriction, although the pathogenetic mechanism is unknown. In a review article, 59 reported cases of cannabis-related stroke were summarized. They tend to occur in men (at a 5:1 ratio), usually occur while the drug is being smoked or within a half hour after the last exposure, and are almost always ischemic, with only a single report of hemorrhagic stroke.

Morning Report Questions

Q:Describe some of the cardiovascular abnormalities associated with sickle cell disease.

A: Among patients with sickle cell disease, cerebral infarction tends to occur in patients who have frequent crises and to occur at the time of crisis. Numerous studies have showed that intravascular coagulation occurs in patients with sickle cell disease. In addition to the coagulopathy, a generalized vasculopathy affects all vessels in sickle cell disease. The vasculopathy may lead to carotid occlusion, dissection, or intracranial aneurysm formation. There have been numerous case reports of cerebral aneurysms in patients with sickle cell disease, including many cases involving multiple aneurysms (an average of three per patient), small aneurysms (3 to 7 mm in diameter), and those that preferentially affect the posterior circulation, especially in unusual locations.

Figure 1. Imaging Studies.

Figure 2. Peripheral-Blood Smear.

Q:Do the strokes associated with sickle cell disease tend to be ischemic or hemorrhagic?

A: Stroke in patients with sickle cell disease may be both ischemic and hemorrhagic, depending on the patient’s age. Ischemic stroke has a bimodal distribution, peaking in the first decade of life and again after 25 years of age. Hemorrhagic stroke peaks in the second and third decades of life. It is important to remember that patients with sickle cell disease and stroke have an estimated lifetime risk of a second stroke of 50%, and most second strokes occur within 3 years after the first.