Headache attributed to arteritis, cerebral venous thrombosis, and other vascular intracranial disturbances

Headache with variable characteristics and associated signs and symptoms may occur in all forms of arteritis. Giant cell arteritis, one of the most common forms, involves branches of the external and, more rarely, of the internal carotid arteries. It occurs in patients over the age of 50 and is characterized by fever, new-onset headache, prominence and tenderness of the temporal artery, claudication of the masticatory muscles on chewing, amaurosis fugax, and visual loss. Headache is the initial symptom in 48% of patients and is present in 90%. Primary central nervous system angiitis is a rare and highly fatal disease in which headache is one of the most frequent symptoms despite the fact that, given its non-specific characteristics, it is of little diagnostic relevance. Headache may also be attributed to several secondary central nervous system arteritides such as Behçet's disease, Takayasu disease, polyarteritis nodosa, Kawasaki disease, Wegener's granulomatosis, systemic lupus erythematosus, and vasculitits caused by viruses, bacteria, fungi, rickettsiae, and protozoa. Thrombosis of the cerebral veins and sinuses is a distinct cerebrovascular disorder that, unlike arterial stroke, most often affects young adults and children. Headache is the most frequent but least specific symptom of venous sinus thrombosis, being present in more than 90% of patients. Headache can also be attributed to other vascular intracranial disorders such as cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), benign (or reversible) angiopathy of the central nervous system, and pituitary apoplexy.