An intense, generalized, throbbing headache may occur in conjunction with flushing of the face and hands and numbness of the fingers (erythromelalgia). Episodes tend to be present on awakening from sound sleep. This condition, called erythrocyanotic, has been reported in a number of unusual settings: (1) in mastocytosis (infiltration of tissues by mast cells, which elaborate histamine, heparin, and serotonin); (2) with carcinoid tumors; (3) with serotonin-secreting tumors; (4) with some tumors of the pancreatic islets; and (5) with pheochromocytoma. Seventy-five percent of patients with pheochromocytoma reportedly have vascular-type headaches coincident with paroxysms of hypertension and release of catecholamines (Lance and Hinterberger) but the flushing phenomenon has been rare in our experience.
Headache Related to Various Medical Diseases
A cardinal feature of meningitis of varied causes is headache. When accompanied by fever and stiff neck, the diagnosis is almost assured. However, severe headache may occur with a number of infectious illnesses caused by banal viral infections, by organisms such as Mycoplasma, and particularly by influenza. There is often accompanying neck pain and slight stiffness. The suspicion of meningitis is raised, even subarachnoid hemorrhage, but there is no reaction in the CSF (“meningism”). The mild aseptic meningitis that accompanies HIV seroconversion may also be accompanied by headache. When persistent and moderately severe, the headache may be classified with “new daily persistent headache” described above.
Approximately 50 percent of patients with chronic and essential hypertension complain of headache, but the relationship of one to the other is probably coincidental. Minor elevations of blood pressure may be a result rather than the cause of headaches. Severe (accelerated) hypertension, with diastolic pressures of more than 120 mm Hg is, however, associated with headache, and measures that reduce the blood pressure relieve the cranial pain. In preeclampsia, the headaches occur at minor degrees of hypertension or normal levels in a woman who has otherwise low blood pressure. Abrupt elevations of blood pressure, as occur in patients who take monoamine oxidase inhibitors and then ingest tyramine-containing food, can cause headaches that are severe enough to simulate subarachnoid hemorrhage. However, it is the individual with moderately severe hypertension and frequent severe headaches that typically confronts the practitioner. In some of these patients, the headaches are of the common migrainous or tension type, but in others, they defy classification. The acute headache of pheochromocytoma correlates with the rate of increase of blood pressure rather than its absolute value. Curiously, headaches that occur toward the end of renal dialysis or soon after its completion are associated with a fall in blood pressure (as well as a decrease in blood sodium levels and osmolality).
Headaches frequently follow a seizure, having been recorded in half of one large series of epileptic patients analyzed by Schon and Blau but the pain was infrequently severe. In migraineurs, the postseizure headache may reproduce a typical migraine attack.
Experienced physicians are aware of many other conditions in which headache may be a principal symptom. These include fevers of any cause, carbon monoxide exposure, chronic lung disease with hypercapnia (headaches often nocturnal or early morning), sleep apnea, hypothyroidism, thrombocythemia, Cushing disease, withdrawal from corticosteroid medication or alcohol, mountain (altitude) sickness, exposure to nitrates, cyanotic heart disease, occasionally in adrenal insufficiency, and acute anemia with hemoglobin well below 10 g.
No attempt is made here to discuss the symptomatic treatment of headache that may accompany these many medical conditions. Obviously, the guiding principle is to address the underlying disease.
Headache Related to Diseases of the Cervical Spine