Intracranial hemorrhage can be classified by etiology and location. One case control study found that a history of hypertension increased the risk of ICH by 3.9-fold (7). Hypertension, the presumed cause of hemorrhage in 70% to 90% of patients, causes bleeding that arises from penetrating vessels of 80- to 300-µm diameter that are derived from the main vessels of the circle of Willis or the basilar artery (8). This pathoanatomic factor accounts for the characteristic locations of hypertensive hemorrhages: basal ganglia (35 to 45%), subcortical white matter (25%), thalamus (20%), cerebellum (15%), and pons (5%). Chronic hypertension leads to fibrinoid necrosis, which reduces the tensile strength of the vessel wall (9,10). Classic neuropathologic studies suggest that most hemorrhages arise from small “Charcot-Bouchard” aneurysms. Although the precise factor that finally precipitates the hemorrhage is unknown, it has been speculated that transient increases in blood pressure are the proximate cause of the hemorrhage in many patients (11).

In the 10% to 30% of patients with ICH in whom there is no evidence of hypertension, the bleeding is often centered in the subcortical white matter of one of the lobes, termed lobar, subcortical, white matter, or slit hemorrhage, the latter describing the pathologic appearance of the hemosiderin-stained cavity
years after the stroke (12,13). In patients older than 65 years, amyloid angiopathy may exceed hypertension as a cause for hemorrhages, particularly multiple and recurrent lobar or cortical-subcortical brain hemorrhages (14). Amyloid deposition is usually related to aging in the brain, because 60% of individuals older than 90 years have pathologic evidence of amyloid deposition (15). Amyloid angiopathy results from the deposition of amyloid in the media and adventitia of smalland medium-sized arteries located near the surface of the cerebral cortex and in the leptomeninges; among the several theories of causation it has been suggested that there is abnormal catabolism of γ-trace protein in the cerebrospinal fluid (16). The epsilon 2 and 4 alleles of apolipoprotein E are associated with increased β-amyloid protein deposition, fibrinoid necrosis, and increased risk of recurrent bleeding among patients with ICH secondary to amyloid angiopathy (17).

More bloody, nontraumatic brain hemorrhages are associated with the etiologic factors listed in Table 14.1. Of course, a thorough history excludes a traumatic etiology. Besides chronic hypertension, inquiry should be made about alcoholism, use of illicit drugs, and use of anticoagulants. Heavy alcohol use can impair coagulation and affect the integrity of the intracerebral vessels, thereby increasing the risk of hemorrhage (18). Intracerebral hemorrhage in patients taking sympathomimetic drugs may be related to acute hypertension, arteriovenous malformations, aneurysms, or drug-induced vasculitis. An increasingly common type arises as a result of anticoagulant drugs or an endogenous coagulopathy. Coagulopathy owing to hepatic failure, thrombocytopenia, and leukemia can all lead to brain hemorrhage. Aspirin and dipyridamole use also have been associated with ICH. Although low serum cholesterol has been reported to be a risk factor for ICH, the data need to be confirmed with a larger prospective study (19, 20 and 21). Clinical trials of lipid-lowering statin medications more recently have shown a reduction in risk of stroke without an increase in rate of ICH (22,23).

The clinical syndrome of brain hemorrhage is one of abrupt onset and rapid evolution of symptoms and signs over minutes or hours. Generally, no prodromal symptoms occur to herald the stroke. Headache, nausea, and vomiting are often the initial symptoms and are attributed to increased intracranial pressure (ICP). A few patients have a seizure at the onset of bleeding, but this is rarely a persistent management issue. Meningismus may result from intraventricular blood that travels to the subarachnoid space but it is surprising how often this sign is absent. Acute hypertension and a diminished level of consciousness are frequent. It has become apparent that expansion of the hematoma may occur, particularly in the first few hours after onset, and the progression of neurological signs during this period is often caused by this ongoing bleeding and enlargement of the hematoma (24). Surrounding edema begins to develop immediately, possibly from the release of osmotically active proteins from the hematoma. This brain swelling worsens over the next several days and can persist for 2 or more weeks (25). This is followed by secondary distortion of the diencephalon
and upper-midbrain, resulting in progressive loss of consciousness and pupillary enlargement. Nearly two thirds of patients with ICH have a smooth progression of neurological symptoms, unlike ischemic stroke and subarachnoid hemorrhage where over 80% of patients present with symptoms maximal at onset (26). A low Glasgow Coma Scale (GCS) score (less than 9), a large hematoma, and intraventricular blood each increase the risk of neurological deterioration and poor outcome (27,28).

Despite the ease with which hemorrhages are demonstrated on computed tomography (CT) scanning, it is necessary to identify the clinical syndrome in order to suspect the diagnosis and distinguish it from an ischemic stroke. Although headache (and vomiting) is a frequent but nonspecific accompaniment of ICH, it may help delineate the site of a lobar hemorrhage (13). Frontal lobe hemorrhage is associated with frontal headache and contralateral weakness. Temporal lobe hemorrhage causes pain anterior to the ipsilateral ear and, when in the dominant hemisphere, a fluent aphasia with poor auditory comprehension. Hemorrhage in the parietal lobe is also associated with ipsilateral anterior temporal headache but causes signs of a hemisensory deficit. Occipital lobe hemorrhage causes severe ipsilateral eye pain and dense hemianopia. The clinical syndromes of lobar hemorrhage sometimes are difficult to distinguish from emboli to the same regions. Computed tomography scanning allows rapid distinction between the two.

During the evolution of a putaminal hemorrhage, the signs of raised intracranial pressure rapidly blend with progressive contralateral hemiparesis and hemisensory loss. The eyes deviate away from the side of the paretic limbs. Visual field defects are common if the level of consciousness allows accurate testing. Hemorrhages in the dominant hemisphere may result in significant nonfluent aphasia (or fluent, if located more posteriorly), and those in the nondominant hemisphere may cause dysprosody of speech and anosognosia. Large hemorrhages produce almost immediate coma, and smaller hemorrhages can lead to more gradual impairment of consciousness and signs of upper brainstem compression.

Thalamic hemorrhage also produces a contralateral hemiplegia and hemisensory deficit. However, the hemisensory deficit is often more pronounced than with a putaminal hemorrhage. Visual field deficits, aphasia, and contralateral neglect also may be seen as with putaminal hemorrhages. Because of the near midline location of thalamic hemorrhages, they are more likely to lead to pupil abnormalities, ocular palsies, and alteration of consciousness than are putaminal hemorrhages of similar size. The classical presentation of obtundation associated with downward and inward deviation of the eyes is well known to clinicians.

Cerebellar hemorrhage is often associated with repeated vomiting, vertigo, and the inability to walk, but without limb ataxia in many patients. It is most often mistaken for “labyrinthitis” or confused with basilar artery occlusion. Therefore, it is imperative to test gait in patients with vomiting and dizziness. As the hemorrhage enlarges, conjugate gaze palsy develops such that the patient cannot look toward the side of the hemorrhage. Sometimes a sixth cranial nerve palsy precedes the conjugate gaze palsy. Dysarthria and a mild ipsilateral facial weakness may be present. As these signs of brainstem dysfunction occur, mentation is altered. Although this alteration may be subtle initially, it can progress rapidly to frank coma, pinpoint pupils, and decerebration.

Pontine hemorrhage is the most likely to lead to rapid onset of deep coma and bilateral signs of brainstem dysfunction (Fig. 14.1). The pupils are pinpoint, but light reaction is often preserved if the pupils are observed with a magnifying glass. Bilateral horizontal conjugate gaze paresis is usually present and a skew deviation also may be present. Ocular bobbing frequently is observed. Quadriplegia and bifacial weakness are the most common motor findings. Progression of the hemorrhage may lead to cessation of respiration.

After an initial clinical assessment, the patient is taken to the radiology suite for a plain CT scan to confirm the diagnosis of brain hemorrhage and to assess the size of the clot. The characteristic CT picture of ICH is an area of increased density in the parenchyma measuring 40 to 90 Hounsfield units, which is related to the hemoglobin content of the extravasated blood (29). Therefore, it must be remembered that blood may appear virtually isodense to brain on the CT scan if the patient has a significant anemia (30). The scan allows the clinician to assess the topography of the hemorrhage and the extent of concomitant ventricular, subarachnoid, or subdural blood. The pattern of these features may suggest an etiology for the hemorrhage. Additionally, the CT scan demonstrates secondary complications such as hydrocephalus and herniation. In the authors’ experience, the level of consciousness in ICH is closely associated with the degree of horizontal displacement of midline structures: a 4- to 6-mm shift of the pineal calcification corresponds to drowsiness, 6- to 8-mm with stupor, and greater than 8- to 9-mm with coma (31). Compression of the basal cisterns is also a dependable sign of serious mass effect and is likely to be associated with moderately or greatly raised ICP.

If the hemorrhage is in an unusual location or has unusual features, a contrast-enhanced CT scan can be performed to assess for an underlying aneurysm, vascular malformation, or tumor. Initially, however, the hemorrhage itself may obscure the recognition of these pathologic entities. Often, a contrast-enhanced CT scan yields more information if performed at
least 1 week after the hemorrhage. An arteriovenous malformation or aneurysm should be suspected if subarachnoid blood is present.

Although angiography is not currently part of the routine evaluation of ICH, it continues to be the mainstay for identifying an underlying vascular abnormality. Of paramount importance initially is identifying hemorrhages that have occurred secondary to aneurysmal rupture. These clots usually appear on CT scan as lobar hemorrhages in the frontal or temporal lobes, or largely within the sylvian fissure. In most cases the parenchymal hemorrhage is accompanied by subdural or subarachnoid hemorrhage. Parenchymal hemorrhage secondary to aneurysmal rupture is associated with a high rate of rehemorrhage and mortality. Therefore, early identification of these patients with angiography allows the clinician to plan timely surgical intervention. Angiography is useful in delineating vascular malformations as well. However, an angiogram performed within several days of the hemorrhage may fail to identify the lesion because of compression by the hemorrhage and surrounding edema. In this situation, a repeat angiogram is indicated 2 to 3 weeks after the hemorrhage. Vascular malformations may not be visualized even by an angiogram performed after the hemorrhage has resolved and the edema has subsided (32). These angiographically occult lesions may be the sites of recurrent hemorrhage. A prospective study of cerebral angiography in ICH indicated that it had a low yield in identifying an underlying vascular abnormality in patients older than 45 years with a history of hypertension and a putaminal, thalamic, pontine, or cerebellar ICH (33). Patients with lobar ICH, isolated intraventricular hemorrhage, normotensive, or who are younger than 45, were more likely to have diagnostic angiographic abnormalities.

Although magnetic resonance imaging (MRI) is not routinely obtained during the initial evaluation, it may be particularly helpful in identifying small brainstem hemorrhages and revealing vascular malformations that cannot be seen with angiography. Acute blood on MRI generally appears isointense to hypointense on T1-weighted imaging and hypointense on T2-weighted imaging. Although it may miss small aneurysms and vascular malformations, MRI is superior to CT and angiography in detecting cavernous malformations and can give information regarding the time course of the hemorrhage (34).

The initial goals for management are to prevent subsequent neural damage from rebleeding, edema, or hypoxia; localize the location and extent of the hemorrhage; and determine its etiology. The initial assessment includes evaluation of airway, breathing, and circulation, neurological examination, and searching for signs of trauma or rhabdomyolysis if the patient is found some time after the ICH occurred.

Correct identification of the location and etiology of the hemorrhage begins by obtaining as many details as possible about the prior medical history and about the initial onset of neurological symptoms. Frequently, a characteristic pattern and progression of symptoms and signs identify the location of the hemorrhage. These patients then receive a thorough neurological examination. Although brief, requiring 5 to 10 minutes, the initial evaluation must be comprehensive because it is the basis for judging subsequent improvement or deterioration in neurological function. The most important aspect of the initial examination is an assessment of the level of consciousness. The level of consciousness at the onset and the rate of progression of obtundation are important for assessing prognosis as well as guiding subsequent management decisions. Patients presenting to the hospital in coma or becoming comatose before definitive intervention have a mortality rate exceeding 50% (35,36).

While the patient is assessed clinically, simultaneous management must be oriented toward preventing neurological deterioration. Abnormalities in electrolytes and coagulation parameters are identified and corrected. Elevated serum glucose should be treated aggressively. If a coagulopathy is present and hepatic disease is suspected, liver function studies
should be performed. Fresh-frozen plasma and vitamin K may need to be administered if the prothrombin time is elevated. If the patient had received heparin recently, protamine 1 mg per 100 units of heparin may be administered, watching for anaphylaxis and hypotension. Following the use of thrombolytic agents, treatment with platelets, fresh-frozen plasma, and cryoprecipitate should be initiated. Laboratory testing might include a toxicology screen in younger patients, or if there is any suspicion of illicit drug use. Phenytoin may be administered to prevent seizures, particularly for lobar hemorrhages, although there are little data to support doing this. The authors do not routinely start anticonvulsant medications unless the patient has had a seizure. Mannitol is generally given if the patient is stuporous or comatose (see the following and Chapter 3).