Intracerebral hemorrhage (ICH) (2024)

CONTENTS

• Rapid Reference 🚀
• Pathophysiology
• Causes of ICH
• Imaging
  • Getting started – Which scans to order initially
  • Noncontrast CT scan
  • CT angiography +/- venography
  • MRI
  • Invasive angiography
• Treatment
  • Airway management
  • Anticoagulation reversal
  • Blood pressure management
  • Neurosurgical interventions
    • Supratentorial hematoma evacuation
    • Cerebellar hematoma
  • Other supportive measures
• Prognostication
• Related topics:
  • Primary intraventricular hemorrhage
  • Cerebral amyloid angiopathy
• Podcast
• Pitfalls

rapid reference: intracerebral hemorrhage

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history – key information

• Time of symptom initiation.
• Medications (especially antihypertensives & anticoagulants – including timing of the last dose).

initial lab panel

• Electrolytes, complete blood count.
• INR, PTT, fibrinogen.
• Anti-Xa activity for patients on riveroXaBAN/apiXaBAN.
• Toxicology screen if clinically warranted.
• Pregnancy evaluation as appropriate.
• ABG/VBG if patient is intubated.

anticoagulation management

• Reversal of medications:
  • Warfarin reversal: 📖
  • Dabigatran reversal: 📖
  • Xaban reversal: 📖
  • Thrombolysis reversal: 📖
  • Heparin reversal: 📖
  • Antiplatelet reversal: 📖 (DDAVP may be considered: 📖)
  • ⚠️ Don't delay reversal of warfarin/DOACs while awaiting labs – empiric reversal should be given immediately.(2022 AHA/ASA; 35579034)
• Treat endogenous coagulopathies:
  • Target platelet count >💯 📖

blood pressure control 📖

• Initial systolic Bp of 150-220 ➡️ target reduction to a systolic Bp of 130-150 mm.
  • ⚠️ Avoid dropping the systolic Bp <130 mm, as this may cause harm.
• Initial systolic Bp >220 ➡️ target reduction to a systolic Bp of ~140-180 mm.
• Preferred agents: Nicardipine or clevidipine infusion.

neurosurgical consultation – especially if: 📖

• Consider hematoma evacuation in cerebellar hemorrhage.
• Consider ventricular drain in hydrocephalus.

additional supportive care

• Fever control.
• EEG if altered mental status is disproportionately severe compared to abnormality on CT scan, or if there is other evidence of seizure. 📖
• Repeat CT scan with CT angiography (if CT angiography wasn't initially obtained). Also consider CT venography if there is suspicion for cerebral venous thrombosis. 📖
• DVT prophylaxis initially with intermittent pneumatic compression.

pathophysiology

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hematoma expansion

• Up to a third of patients whose CT is obtained within 3 hours of onset will experience an increase in hematoma size.
• Hematoma expansion is most common within the first 6-24 hours (yet it can occur within ~48 hours in coagulopathic patients).
• Risk factors for hematoma expansion include:
  • Anticoagulation.
  • Shorter duration since initiation of symptoms.
  • Various signs on CT scanning.

cytotoxic edema

• Cytotoxic edema occurs surrounding the hematoma (perihematomal edema). This worsens over a period of days, before improving. Edema volume may peak after ~5-6 days.(Shutter 2019)
• Remember that cytotoxic edema does not respond to steroids – only vasogenic edema does.

intracranial pressure (ICP)

• Intracranial pressure may increase due to the mass effect of the hemorrhage and the perihematomal edema, as well as either blood accumulation in the ventricles (if the hematoma extends into the ventricles) or mass effect on the ventricles leading to obstructive hydrocephalus.

terminology

• Intracerebral hemorrhage (ICH) refers to a primary or secondary non-traumatic hemorrhage.
• The term “hemorrhagic stroke” encompasses ICH, but also hemorrhagic conversion of an ischemic stroke and subarachnoid hemorrhage, and should be avoided in order to avoid confusion.

causes of ICH

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(#1/3) small vessel disease (“primary ICH”)

hypertensive intraparenchymal hemorrhage

• Location is most commonly the same locations where patients develop lacunar strokes (both processes relate to arteriolar lipohyalinization):(26046515)
  • Basal ganglia/external capsule (60-65%).
  • Thalamus (15-20%).
  • Pons or deep nuclei of the cerebellum (10%).
• Epidemiology:
  • Causes ~80% of all intracranial hemorrhage.(Torbey, 2019)
  • Risk factors include age, hypertension, and diabetes.
• Diagnosis:
  • Imaging often reveals additional chronic white matter changes and microhemorrhages (the latter may be seen on GRE/SWI MRI sequences).
  • Supported by other evidence of hypertension (e.g., left ventricular hypertension, chronic kidney disease).

cerebral amyloid angiopathy (CAA) – more on this below 📖

(#2/3) macrovascular etiologies

Macrovascular etiologies often require surgical resection and/or embolization by interventional radiology to avoid re-bleeding. Vascular malformations are the most common cause of ICH in young adults.(33288539)

• Aneurysmal rupture
  • Potential clues:
  • (1) Aneurysms usually have at least a small subarachnoid component, along with the intraparenchymal hematoma.(29262441)
  • (2) Often frontal location (due to aneurysm of the ACA or AComm) or temporal location (due to aneurysm of the MCA). 📖 The hematoma may extend down to a vessel within the circle of Willis.
  • (3) Relatively young patients.
  • (4) Personal/family history of aneurysmal disease.
  • Management: Consult neurosurgery and/or interventional radiology for aneurysmal protection. Manage blood pressure management mirrors the management of subarachnoid hemorrhage. 📖 (Torbey, 2019)
• Arteriovenous malformation (AVM).
• Cerebral venous thrombosis (CVT) with secondary hemorrhage
  • Risk factors and clues are discussed in the section below. 📖
• Cavernous malformation (aka., cavernomas or cavernous hemangiomas). These are notable for their unique popcorn-like appearance on T2 MRI images.
• Dural arteriovenous fistula (dAVF).
• Mycotic aneurysm may result from endocarditis or bacteremia.
• Moyamoya disease. 📖

(#3/3) other etiologies

• Malignancy: 📖
• Ischemic stroke with hemorrhagic conversion: 📖
• RCVS (reversible cerebral vasospasm syndrome): 📖
• Coagulopathy.
• Sympathomimetic drug use.
• Vasculitis: Usually caused by polyarteritis nodosa or lupus, but may also occur with ANCA vasculitis, rheumatoid arthritis, sarcoidosis, drug-induced vasculitis, or Henoch-Schonlein purpura.

which scans to order initially

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most patients receive CT angiography +/- CT venography (CTA +/- CTV)

• CT angiography (+/- CT venography) can be helpful to exclude vascular causes of hemorrhage. Obtaining CTA +/- CTV is a Class 2A recommendation for any patient with ICH.(2022 AHA/ASA; 35579034)
• Nearly everyone should receive a CTA (unless imaging and epidemiology is strongly consistent with ICH due to chronic hypertension).(33288539) Scenarios where CT angiography would be mandatory include: (2022 AHA/ASA; 35579034)
  • Lobar intracranial hemorrhage in a patient <70 years old.
  • Deep/posterior fossa ICH in a patient <45 years old.
  • Deep/posterior fossa ICH in a patient 45-70 years old without a history of hypertension.
• Patients will be returning to the CT scanner for serial scans (more on this below). If CT angiography wasn't performed during the first scan, it may be included in one of the followup CT scans.
• Whether to obtain a CT venogram (CTV) depends on the index of suspicion for cerebral vein thrombosis (CVT). Risk stratification for this is described next.

CT venography (CTV) if there is suspicion for cerebral vein thrombosis (CVT)

• Cerebral vein thrombosis with hemorrhagic transformation is an important cause of ICH, because this requires specific management. Clues suggesting the possibility of cerebral vein thrombosis include the following:
• (1) Epidemiological clues: 📖
  • Younger age (e.g., ~20-50 years old).
  • Hypercoagulability (including oral contraceptive use, pregnancy, antiphospholipid syndrome, sickle cell disease, dehydration).
  • Cranial infection (e.g., meningitis, mastoiditis, cerebral empyema).
• (2) Imaging clues: 📖
  • Lobar hemorrhage, gyriform hemorrhage, or bilateral hemorrhages.
  • Cerebral edema in excess of what might otherwise be expected.
  • (Hyperdensity in a venous sinus may occasionally be seen.)
• (3) Clinical clues:
  • Gradual onset of headache and neurologic deficits.

serial CT scan

• 🛣 Serial head CT can be useful within the first 24 hours after symptom onset to evaluate for hemorrhage expansion. In patients with stable examination and preserved level of consciousness, follow-up CT scans at ~6 hours and at 24 hours appear adequate to evaluate for hemorrhage expansion and final hematoma volume.(2022 AHA/ASA; 35579034)
  • Hematoma expansion will often occur within 6-24 hours (or later in anticoagulated patients).
• In patients with new abnormalities on neurological examination or inability to perform an examination (e.g., due to sedation), more frequent scanning may be useful.

noncontrast CT scan

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diagnosis

• Noncontrast CT scan is the standard diagnostic test to rapidly evaluate for acute intracranial hemorrhage.
• Clotting occurs within minutes to hours, causing the hematoma to be hyperdense. However, among patients on oral anticoagulants, hematomas may remain isodense or hypodense for longer periods of time.(33272397)
  • 💡 Isodense or hypodense blood on CT scan may be an indicator of coagulopathy.

hematoma volume

• Hematoma volume may be estimated as the multiple of the diameters divided by two (ABC/2). 🧮
  • A is the largest hematoma diameter on any axial CT image.
  • B is the largest diameter perpendicular to A, within that axial CT slice.
  • C is the number of axial CT slices showing hemorrhage, multiplied by the slice thickness (which may vary from 3-10 mm depending on the scanner).(34618759)
  • (Precise techniques vary. Some sources advise that to calculate “C” one should only include slices with at least 75% of the hematoma volume. Slices that include 25-75% of the hematoma volume may be counted as a half-slice.)(33272397)
• All measurements should be made in centimeters. This will yield a volume measured in milliliters (mL).
• Interpretation of hematoma volume:
  • <30 ml may suggest a relatively favorable prognosis.(Louis 2021)
  • >30 ml may correlate roughly with a moderate-severe ICH (this is the cutoff used in the ICH score).
  • >70 ml was previously considered to be uniformly lethal, but good outcomes may be achieved in some patients with surgery.(Louis 2021)
  • However, the location of the ICH is often more important than absolute blood volume (e.g., 20 ml of blood in the pons is more dangerous than 40 ml in the frontal lobe).

markers of hemorrhage expansion on noncontrast CT

• Swirl sign may indicate active hemorrhage: entry of unclotted fresh blood has lower attenuation than blood that has already clotted. There isn't necessarily a distinct “swirl,” rather the key finding is blood of varying density. 🌊
• Black hole sign: Defined as a relatively dark area within a hematoma that is not connected with adjacent brain tissue. This predicts hematoma growth with 32% sensitivity and 94% specificity.
• Blend sign: Hemorrhage contains hyperdense and hypodense regions.
• Fluid-fluid level: Fluid level in a hematoma suggests the presence of coagulopathy (albeit with only moderate sensitivity).(32299594)
  • 🔑 A blood-fluid level is highly specific for coagulopathy or use of anticoagulants and should prompt a search for these factors, if none have been identified.(33288539)
• Island sign: At least three small hemorrhages which are separated from the main hemorrhage.(32224753)

edema

• Edema takes several hours to develop, so it is usually not seen initially.
• An unusually extensive or irregular amount of edema raises the possibility of: (Louis 2021)
  • CVT (cerebral venous thrombosis) with subsequent hemorrhage. 📖
  • Ischemic stroke followed by hemorrhagic transformation. 📖
  • Tumor followed by intratumoral hemorrhage. 📖

CT angiography +/- venography (CTA +/- CTV)

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overview of CT angiography +/- venography

• This is the front-line test to evaluate for an underlying cause of ICH due to speed and safety (especially for causes which require more immediate management).
• CT angiography may be useful to detect an underlying aneurysm or vascular malformation. CT venography is utilized to evaluate for cerebral venous thrombosis.
• ⚠️ Please note that contrast dye is not nephrotoxic.📖 Appropriate imaging should not be deferred due to renal dysfunction.
• Both CT angiography and venography may be performed together (CTAV) following a single administration of contrast dye.

spot sign on CT angiography

• The spot sign reveals leakage of contrast into the hematoma (predicting expansion with 51% sensitivity and 85% specificity).
• The spot sign must not be in anatomic continuity with any adjacent blood vessels.(32224753)

MRI

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MRI generally isn't performed immediately, due to logistic constraints, but should be performed within 2 days of ictus if you wish to administer gadolinium. The reason is that after 2 days, the blood becomes hyperintense on T1 and you may not be able to see beneath the hemorrhage when the gadolinium is administered. If you are searching for amyloid, a non-gadolinium enhanced MRI will suffice.

MRI may be superior for detecting certain underlying pathologies:

• Tumor with intra-tumoral hemorrhage.
• Ischemic stroke with hemorrhagic transformation.
• Cerebral amyloid angiopathy (CAA) with multiple occult cerebral micro-bleeds.
• Underlying hypertensive microangiopathy (may be suggested by white matter lesions and small lacunar strokes).
• MRI is the most sensitive modality for cavernous malformations, which may be occult on CT angiography and invasive angiography.(33272397)

MR angiography +/- MR venography

• Usually not the front-line mode of vascular imaging.
• May be useful to provide vascular imaging (e.g., if there is a concern for venous sinus thrombosis, but CT venography hasn't been performed).

invasive angiography

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basics

• This is the definitive study to evaluate for aneurysm, dural arteriovenous malformation (dAVF), or vasculitis.
• Angiography can be both diagnostic and therapeutic (if an aneurysm or arteriovenous malformation is discovered, some may be embolized immediately).

indications for invasive angiography

• Invasive angiography is typically obtained if other imaging studies raise concern for a possible vascular lesion, yet are unable to resolve this issue definitively.
• 🛣 Examples of indications for invasive angiography include the following clinical scenarios, if CT angiography cannot reveal a cause:(2022 AHA/ASA; 35579034)
  • (1) Lobar ICH in patient <70 years old.
  • (2) Deep/posterior fossa ICH in a patient <45 years old.
  • (3) Deep/posterior fossa ICH in a patient 45-70 years old without a history of hypertension or signs of small vessel disease on imaging.
  • (4) Patients with primary intraventricular hemorrhage. 📖

airway management

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• Intubation may be required for airway protection. However two thirds of patients won't require intubation.(31092052)
• There are no validated or well-defined criteria for intubation – this is a clinical judgement based on examination and trajectory.
• Care is required to avoid stimulation and hypertension, which could worsen intracranial pressure elevation.🌊

blood pressure management

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blood pressure targets

• 🛣 In patients with an initial systolic Bp of 150-220, acute lowering to target a systolic Bp of 130-150 mm may be reasonable. However, lowering the blood pressure below <130 mm is potentially harmful and should be avoided.(2022 AHA/ASA; 35579034)
• Benefit from antihypertensives is maximized if they are started immediately (when the risk of hematoma expansion is greatest).
• Personalization of blood pressure targets may be reasonable in patients with unusually high or low baseline blood pressure. For example, in a patient presenting with a systolic blood pressure >220 mm, it might be reasonable to target a systolic Bp 140-180.
• The optimal duration of blood pressure control is not known. Theoretically, benefit would be expected to extend through the period when there is a high risk of hemorrhage expansion (e.g., perhaps the first ~24 hours in patients without coagulopathy). Gradual transition to oral antihypertensives may be reasonable after >24 hours, among patients who have stabilized.(Louis 2021)

preferred agents

• ⚠️ Before initiating antihypertensives, always make sure that pain and anxiety have been adequately treated. Especially among intubated patients, hypertension can be a manifestation of pain. Proper analgesia and sedation may go a long way towards blood pressure control.
• Clevidipine 💉 may provide the smoothest control of blood pressure, since it has a short half-life (allowing it to be a truly titratable agent).
• Nicardipine is more widely available and less expensive. Nicardipine is effective, but close attention is required to avoid causing overshoot hypotension (discussed further here 💉).

discussion: theoretical rationale & risks

• Rationale: Blood pressure reduction should theoretically reduce hematoma expansion. This would be expected to be most effective within the immediate post-hemorrhage period when hematoma expansion risk is greatest.
• Risks:
  • Neurological: Reduced blood pressure could impair perfusion of penumbra surrounding the hematoma. This could cause necrosis, leading to worsening edema and intracranial pressure (leading to a cascade of secondary brain insults).
  • Renal failure may result from excessive blood pressure reduction.

supratentorial hematoma evacuation

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STITCH-I Trial (15680453)

• RCT involving 1,033 patients randomized to medical management vs. early surgical hematoma evacuation.
• Outcomes were nearly identical between two groups. However, there was a non-significant trend toward benefit from surgery in patients with hematoma extending to within 1 cm of the cortical surface. Likewise, there were trends toward benefit from early surgery among patients with lobar hemorrhage and Glasgow Coma Scale score of 9-12.
• Patients with ICH >1 cm from cortical surface or GCS<9 tended to do worse with surgery.

STITCH-II Trial (23726393)

• RCT involving 601 patients predicted to obtain maximal benefit from surgery based on the results of the STITCH-1 trial (patients were conscious, had superficial hemorrhage <1 cm from the cortical surface, had a hemorrhage volume of 10-100 ml, and had no intraventricular hemorrhage).
• Patients were randomized to early surgery vs. early conservative approach.
• Outcomes were nearly identical between the two groups.

evidence summary

• Available evidence suggests no outcome benefit for early hematoma evacuation. Some patients in the medical arm of these studies did cross over into the surgical arm due to progressive deterioration, so surgery might still be beneficial in selected cases with progressive deterioration over time.
• Emerging evidence suggests that minimally invasive surgery could be more promising.

possible indications for surgery?

• Large hematoma with significant midline shift.
• Elevated ICP refractory to medical management.
• Hemorrhage secondary to an underlying lesion that could benefit from resection (e.g., arteriovenous malformation or tumor).

cerebellar hematoma

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basics of cerebellar hemorrhage

• The cerebellum is unique due to its enclosed location.
• Potential consequences of a cerebellar hemorrhage include:
  • (1) Direct compression causing catastrophic damage to the adjacent brainstem.
  • (2) Compression of CSF flow due to obstructive compression of the fourth ventricle may cause obstructive hydrocephalus.
  • (3) Herniation (which may include upwards transtentorial herniation of the cerebellum).
• Patients may tend to deteriorate more rapidly than patients with other types of ICH (e.g., often within a 12-24 hour timeframe).
• Patients with cerebellar hematomas tend to have a better prognosis than other types of hemorrhage. Put together, this implies a greater benefit for hematoma evacuation in this location.

surgical evacuation for cerebellar hemorrhage

• No RCTs exist (these patients were excluded from the STITCH trials).
• Ventriculostomy placement without decompression and/or hematoma evacuation is not recommended, as this increases the chances of causing upward transtentorial brain herniation.
  • (Note also that if an external ventricular drain is placed, the pressure it measures may not accurately reflect the pressure in the posterior fossa.)
• 🛣 Indications for immediate surgical evacuation: (2022 AHA/ASA; 35579034)
  • Larger volume (>15 ml).
  • Neurological deterioration.
  • Brainstem compression.
  • Hydrocephalus.

intraventricular hemorrhage & EVD

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basics of intraventricular hemorrhage

• Intraventricular hemorrhage occurs in >40% of patients with ICH. It is associated with worse prognosis.
• Complications of intraventricular hemorrhage may include:
  • Hydrocephalus and elevated intracranial pressure.
  • Central (neurogenic) fever. 📖

external ventricular drain (EVD) for patients with ICH

• Ventricular drainage may be considered for hydrocephalus (usually within the context of intraventricular hemorrhage). An ideal candidate would be a patient who was initially doing well, but then subsequently develops hydrocephalus with neurologic deterioration.
• 🛣 Guideline recommendation: in patients with hydrocephalus that is contributing to a reduced level of consciousness, ventricular drainage should be performed to reduce mortality.(2022 AHA/ASA; 35579034)
  • (Unfortunately, overall evidence suggests that ventricular drainage increases survival without increasing the likelihood of a good neurologic outcome. This implies that patients whose lives are saved by drain placement often have poor neurologic outcome.) (10751114, 22732889)

intraventricular tPA

• There has been some interest in the utility of intraventricular tPA, to promote dissolution of thrombus following intraventricular hemorrhage.
• The CLEAR III trial was a multi-center RCT evaluating intraventricular tPA for management of patients with an ICH complicated by intraventricular hemorrhage being managed by an EVD (external ventricular drain).(28081952)
  • 500 patients were randomized to receive intraventricular tPA vs. saline.
  • Intraventricular tPA improved survival, but didn't increase the number of survivors with good functional outcome.
• Intraventricular tPA is used in some centers, particularly when it is felt that the patient predominantly has intraventricular hemorrhage without significant parenchymal hematoma, and when the hydrocephalus might resolve if the intraventricular hematoma dissipates.

seizure diagnosis & management

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incidence of seizures

• Convulsive seizures occur in ~10% of patients, but the rate of subclinical seizures may be substantially higher.(34618759, LaRoche 2018)
• Risk factors for seizures:(35420918)
  • Cortical involvement (e.g., lobar hemorrhages).
  • Coexistence of subarachnoid and/or subdural hemorrhage.
  • Complications, including rebleeding.

monitoring & diagnosis

• Indications for continuous EEG:
  • Impaired consciousness that is out of proportion to what would be expected based on the CT scan.
  • Unexplained fluctuations in mental status.
  • Any history or clinical signs of seizure.
  • (More discuss about general indications for EEG monitoring: 📖)
• ICH may also be associated with LPDs (lateralized periodic discharges), GPDs (generalized periodic discharges), SIRPIDs (stimulus-induced rhythmic periodic or ictal discharges), and FIRDA (frontally predominant intermittent rhythmic delta activity).(LaRoche 2018)

management

• Seizure prophylaxis isn't recommended.
  • Prior evidence has not revealed benefit with prophylactic phenytoin (which may relate to phenytoin's numerous side-effects).
• Antiepileptic therapy is indicated for a patient with witnessed seizure, electroencephalographic seizure, or nonconvulsive status epilepticus. 📖

other supportive measures

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fever control

• Any fever should be treated aggressively (e.g., with scheduled acetaminophen).
• If fever is refractory to antipyretics, an external cooling device may be used to achieve aggressive normothermia (similar to treatment of a post-arrest patient).
• Neurogenic fever may occur, especially among patients with intraventricular extension of the hematoma.(33952393) However, this remains a diagnosis of exclusion.📖

DVT prophylaxis

• 🛣 Intermittent pneumatic compression should be used initially for DVT prophylaxis.(2022 AHA/ASA; 35579034)
• 🛣 Initiating low-dose unfractionated heparin or low molecular-weight heparin prophylaxis at 24-48 hours from ICH onset may be reasonable.(2022 AHA/ASA; 35579034) Generally this is started after serial CT scans show stability of the hemorrhage. Patients with underlying coagulopathy or imaging evidence of ongoing hemorrhage expansion may require a more personalized approach.

nutritional support

• 🛣 Formal dysphagia screening should be implemented before initiation of oral intake to reduce the risk of pneumonia.(2022 AHA/ASA; 35579034)

sodium management

• Avoid hyponatremia or rapid decreases in sodium.
• Routine use of hypertonic saline isn't supported by evidence.
• Boluses of hypertonic saline or hypertonic bicarbonate may be used to manage elevated intracranial pressure, ideally as a bridge to more definitive therapy (e.g., an external ventricular drain).

prognostication

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overall prognosis

• Mortality is high, with only half of patients alive after one year and only ~30% alive after five years.
• Only ~20% of patients achieve functional independence after one year.(33272397, Louis 2021)

predictors of poor outcome

• Hematoma volume >60 ml.
• Glascow Coma Scale <8.
• Deep or infratentorial location.
• Intraventricular extension.
• Increasing age.

ICH score 🧮

• The ICH score is widely used to provide risk stratification. It is composed of the following elements associated with worse outcomes:
  • Lower GCS (Glasgow Coma Scale).
  • Age >80.
  • Hematoma volume >30 ml.
  • Intraventricular hemorrhage.
  • Infratentorial origin of hemorrhage.
• There are numerous limitations to the ICH score. For example: ()
  • Transient impairment of neurologic function may increase the ICH score (e.g., due to sedation or hypercapnia).
  • Hydrocephalus may cause impaired mental status. This may be quite treatable, with improvement following external ventricular drain placement. Attempts to prognosticate may be inaccurate in the context of untreated hydrocephalus.
  • There is no differentiation between tiny intraventricular hemorrhage vs. massive intraventricular hemorrhage.
  • There is no differentiation between a pontine bleed vs. a cerebellar bleed (although the latter is more treatable).
  • There is no differentiation between an 18-year-old versus a 79-year-old.
  • ICH score doesn't account for pre-existing functional capacity (e.g., frailty).
• ICH score was designed to stratify disease severity, not to be an acute prognostication tool. Thus, the ICH score shouldn't be used as the primary basis for limiting life-sustaining therapies.(35579034)
• For an in-depth discussion of the optimal use of the ICH score: 🌊

FUNC score 🧮

• The FUNC score provides an estimate of the likelihood of functional independence at 90 days.
• Tables below provide more granular detail about how the FUNC score is obtained and interpreted.(18556582)
• ⚠️ Note that many of the limitations of the ICH score discussed above also apply to the FUNC score.

primary intraventricular hemorrhage

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basics

• Primary intraventricular hemorrhage refers to hemorrhage into ventricles without associated parenchymal ICH.
• Primary intraventricular hemorrhage is rare, accounting for 3% of nontraumatic intracranial hemorrhage.(34618759)

causes of intraventricular hemorrhage

• ~90% are idiopathic.
• ~10% have an underlying etiology, including:
  • Hypertension.
  • Arteriovenous malformation (AVM), arteriovenous fistula, or aneurysm.
  • Moyamoya disease.
  • Coagulopathy.
  • Choroid plexus tumor or ependymal lesion.

investigation of the etiology of an intraventricular hemorrhage

• CT scan with CT angiography is often the initial investigation.
• 🛣 In patients with spontaneous intraventricular hemorrhage and no detectable parenchymal hemorrhage, catheter intra-arterial digital subtraction angiography is recommended to exclude a vascular cause.(2022 AHA/ASA; 35579034)

role for external ventricular drain (EVD)

• Drainage is indicated for acute obstructive hydrocephalus.
• Intraventricular fibrinolytic therapy may be beneficial for some patients, if available.(33272397)
• Management may resemble the management of secondary intraventricular hemorrhage. 📖

cerebral amyloid angiopathy

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basics

• Cerebral amyloid angiopathy is a cerebrovascular disorder caused by deposition of beta-amyloid within small-to-medium arteries within the cerebral cortex, white matter, and leptomeninges.
• Clinical manifestations include lobar intracerebral hemorrhage, ischemic stroke, dementia, and reversible leukoencephalopathy.

epidemiology

• Cerebral amyloid angiopathy has a prevalence of ~5% among people >65YO, accounting for ~15% of all spontaneous intracranial hemorrhages.(34618759) Amyloid angiopathy is associated with Alzheimer's disease.(33272397)
  
• This is the most common cause of lobar hemorrhage in normotensive elderly patients. However, half of lobar ICH in the elderly aren't due to amyloid angiopathy, so these two factors alone do not establish the underlying mechanism of the hemorrhage.(32299594)
• Intracranial hemorrhage is often provoked by anticoagulation or thrombolysis.

radiology

• Hemorrhage location:
  • Usually lobar hemorrhage, more often in the occipital and parietal lobes.(29262441)
  • Sometimes with finger-like projections of the hemorrhage (image below) or extension that involves subarachnoid, subdural, or intraventricular hemorrhage.
  • 💡 Hint: If the patient has underlying cognitive decline, and the hemorrhage is lobar/cortical and with a small degree of high cortical subarachnoid hemorrhage, chances are you are looking at an amyloid hemorrhage.
• GRE/SWI sequences on MRI may show additional microhemorrhages at the gray-white junction and/or convexity hemosiderosis.👇

differential diagnosis of microhemorrhages 🌊&&

• Cerebral amyloid angiopathy (hemorrhages usually occur at the grey-white junction, sparing the basal ganglia).
• Chronic hypertensive encephalopathy (lesions tend to be distributed in basal ganglia, thalami, external capsule, pons, and deep nuclei of the cerebellum).
• DAI (diffuse axonal injury; typically occur at the grey-white matter junction, dorsolateral brainstem, and splenium of the corpus callosum).
• Hemorrhagic metastasis (especially renal cell carcinoma or melanoma).
• Hypoxia/ARDS, including COVID. 📖
• PRES (posterior reversible encephalopathy syndrome)
• Intracranial embolism:
  • Fat embolism syndrome.
  • Gas embolism (e.g., due to ECMO, malpositioned vascular catheter, air embolism after removal of central venous catheter).
  • Septic embolism due to endocarditis.
• Iatrogenic:
  • Status post cardiac bypass.
  • Radiation-induced cerebral vasculopathy.
  • Cerebral reperfusion syndrome status post carotid endarterectomy.
• Vasculitis (primary or secondary).
• Intravascular lymphoma.
• Neurosarcoidosis.
• Thrombotic microangiopathies.
• AHLE (acute hemorrhagic leukoencephalitis).
• CADASIL, Moyamoya disease.
• Cavernous malformation, especially type 4.

clinical implications of cerebral amyloid angiopathy

• The short-term prognosis is often favorable, but there is a high risk of hemorrhage recurrence (~7.5%/year) and subsequent cognitive decline.(32299594)
• 💡 If you diagnose with someone with cerebral amyloid angiopathy, those patients should not be restarted on anticoagulation as they carry a very high risk of recurrent hemorrhage.

inflammatory cerebral amyloid angiopathy

• This is also known by various other names (cerebral amyloid angiopathy-related inflammation; amyloid beta-related angiitis). Clinically, these entities are largely synonymous.
• This is a rare form of CNS vasculitis that occurs among patients with cerebral amyloid angiopathy (CAA) wherein perivascular beta-amyloid triggers inflammation.
• Mean age of onset is in the 70s.(26425252)
• Clinical presentation may be acute or subacute, often involving:
  • Headache.
  • Reduced level of consciousness.
  • Behavioral change.
  • Focal neurological sign and seizures.
• Radiographic features: 🌊
  • Vasogenic edema is usually the main finding, involving the subcortical white matter, with associated mass effect. There may be a single lesion, or multifocal lesions. Lesions can extend to involve the cortical grey matter. Indeed, there may be leptomeningeal contrast enhancement in about half of patients.
  • If MRI is utilized, there should be features of underlying cerebral amyloid angiopathy (i.e., microhemorrhages).
  • MR angiography can show thickening/enhancement of medium-sized arteries, with multifocal stenoses.
• Differential diagnosis may include such entities as:
  • PML (progressive multifocal leukoencephalopathy).
  • PCNSL (primary CNS lymphoma) or glioma.
  • CVT (cerebral venous thrombosis).
  • Infection (e.g., cerebritis, toxoplasmosis).
  • Tumefactive multiple sclerosis.
  • AHLE (acute hemorrhagic leukoencephalopathy).
  • Asymmetric PRES (posterior reversible encephalopathy syndrome).
• Treatment:
  • Inflammation may respond to steroid followed by cyclophosphamide.(Louis 2021)

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To keep this page small and fast, questions & discussion about this post can be found on another page here.

• Cerebellar hematomas may threaten brainstem compression as well as hydrocephalus, so these patients potentially benefit the most from urgent surgical evacuation and/or ventricular drainage.
• Steroid doesn't help ICH. Don't use steroid in these patients unless there is some other indication.

Acknowledgement: Thanks to Dr. Richard Choi (@rkchoi) for thoughtful comments on this chapter.

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