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
    • ↑ Pressure on brainstem → dysfunction of respiratory center → irregular breathing

Clinical features


  • Global
    • Cushing triad: irregular breathing, widening pulse pressure, and bradycardia
      • Increase in systolic, decrease in diastolic blood pressure
    • Reduced levels of consciousness
    • Headache
    • Vomiting
    • Papilledema
    • Psychiatric changes
    • 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)DecorticateDecerebrate 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