Epidemiology


Etiology


Pathophysiology


  • Cushing’s reflex
    • ↑ ICP → ↓ CPP → compensatory activation of the sympathetic nervous system → ↑ systolic blood pressure → stimulation of aortic arch baroreceptors → activation of the parasympathetic nervous system (vagus) → bradycardia c
    • ↑ Pressure on brainstem → dysfunction of respiratory center → irregular breathing

Clinical features


  • General ↑ ICP:
    • Headache (worse in AM, worse supine, worsened by Valsalva/coughing).
    • Nausea/Vomiting (often projectile, without nausea).
    • Papilledema (blurring of optic disc margins).
    • Altered Mental Status (AMS), depressed level of consciousness.
    • Cushing Triad (late, impending herniation): Hypertension (widened pulse pressure), Bradycardia, Irregular respirations. c2
    • In infants: macrocephaly, bulging fontanel, sunset sign

Subtypes

TypeStructure CompressedClassic Sign
SubfalcineACA (Anterior Cerebral Artery)Contralateral leg weakness
UncalCN III (Oculomotor Nerve)Ipsilateral fixed, dilated pupil
CentralBrainstem (Rostral-Caudal)Decorticate → Decerebrate posturing
TonsillarMedullaRespiratory / Cardiac arrest
  • Subfalcine (Cingulate)
    • What: Cingulate gyrus is displaced under the falx cerebri. This is the most common type of herniation.
    • Vessels: Compression of the anterior cerebral artery (ACA) can occur.
    • Clinical signs: May be asymptomatic or cause contralateral lower limb weakness due to motor cortex ischemia.
  • Transtentorial (Uncal)
    • What: Medial temporal lobe (specifically the uncus) is forced through the tentorial notch.
    • CN III Palsy: This is a classic and critical finding.
      • Early: Compression of parasympathetic fibers on the exterior of CN III causes an ipsilateral fixed and dilated pupil (“blown pupil”).
      • Late: Compression of motor fibers of CN III leads to a “down and out” gaze.
    • Vessels: Ipsilateral posterior cerebral artery (PCA) compression can cause contralateral homonymous hemianopsia.
    • Motor: Compression of the contralateral cerebral peduncle against the tentorium (Kernohan’s Notch) causes ipsilateral hemiparesis (a false-localizing sign).
    • Brainstem: Brainstem displacement can cause tearing of paramedian basilar artery branches, leading to fatal Duret hemorrhages.
  • Central Herniation
    • What: Downward displacement of the diencephalon and brainstem through the tentorial notch.
    • Clinical signs: Leads to a rapid decline in consciousness, decorticate posturing progressing to decerebrate posturing, and eventually death.
  • Tonsillar
    • What: Cerebellar tonsils are forced through the foramen magnum.
    • Clinical signs: Compresses the medulla, which contains the cardiorespiratory centers. This leads to respiratory arrest, circulatory collapse, and rapid death.
    • Clinical pearl: This is why a lumbar puncture (LP) is contraindicated in patients with suspected ↑ICP until an intracranial mass lesion has been ruled out by imaging (e.g., CT scan). An LP can create a pressure gradient that precipitates tonsillar herniation.

Diagnostics


Treatment

  • 1. Emergency Stabilization & First-Line Measures (Tier 1):
    • Airway & Breathing: Intubate if GCS ≤ 8; maintain normoxia (PaO2 > 60 mmHg) and normocapnia (PaCO2 35–40 mmHg).
    • Hemodynamic Control: Maintain CPP = 60–70 mmHg (CPP = MAP – ICP); avoid hypotension (SBP < 90 mmHg). Use IV vasopressors (e.g., norepinephrine) if needed to support MAP.
    • Positioning: Elevate head of bed (HOB) to 30–45° with neck midline to facilitate jugular venous outflow.
    • Analgesia/Sedation: IV propofol or fentanyl to prevent coughing, agitation, and reduce cerebral metabolic demand (CMRO2).
    • Temperature Control: Aggressive antipyretics and cooling (fever increases CMRO2 and ICP).
  • 2. Medical Interventions (Tier 2):
    • Osmotic Therapy:
      • Hypertonic Saline (3% or 23.4% NaCl): First-line in hypotensive/hypovolemic pts; draws water from parenchyma into vascular space. Target serum Na 145–155 mEq/L. c
      • Mannitol (20%): Bolus 0.5–1.0 g/kg IV. Contraindicated in hypotension, hypovolemia, and severe renal failure. Target serum Osm < 320 mOsm/kg.
    • Glucocorticoids (Dexamethasone):
      • Indicated only for vasogenic edema due to CNS tumors/metastases or abscesses.
      • Contraindicated in TBI, stroke, and ICH (worsens mortality/outcomes).
    • Hyperventilation (Target PaCO2 26–30 mmHg):
      • Causes cerebral vasoconstriction -> rapid reduction in cerebral blood volume.
      • Temporary/Bridge therapy only (lasts < 24–48 hrs; excessive vasoconstriction causes secondary ischemic stroke).
  • 3. Refractory Management & Surgical Interventions (Tier 3):
    • CSF Drainage: External Ventricular Drain (EVD) for therapeutic CSF removal and continuous monitoring.
    • Surgical Evacuation / Decompression: Urgent craniotomy/craniectomy for hematoma evacuation or decompressive hemicraniectomy for refractory malignant edema/stroke.
    • Metabolic Suppression: Barbiturate coma (Pentobarbital) or therapeutic hypothermia (32–35°C) to suppress CMRO2 in medically refractory cases without surgical targets.