Cerebral Herniation Syndromes

Imaging findings and clinical correlation

Definition of Cerebral Herniation:

Herniation of brain tissue from one compartment (separated by calvarial and /or dural boudaries) to another

Compartments
Supratentorial
Right Left

Infratentorial

Spinal

Intracranial compartments
Brain tissue in each compartment is contained by (relative) intracranial boundaries:
Falx cerebri Tentorium cerebelli Skull base (foramen magnum)

Intracranial compartments
Neurocranium Falx cerebri

Tentorium Cerebelli

Skull Base (Foramen Magnum)

Intracranial Compartments
For each compartment there are laws of Intracranial volume en pressure Each compartment abides these laws as good as possible

Intracranial volume
Intracranial volume is constant and described by Monro-Kelly Doctrine:
Vol. Intracranial = V. Brain + V. CSF + V. Blood

Intracranial components are (almost) noncompressible Increase in one volume leads to decrease in another

Intracranial Pressure
Normal intracranial pressure: 5-15 mmHg

http://www.trauma.org/archive/neuro/icp.html

Intracranial Pressure
Pressure components:
Cerebral Perfusion Pressure (CPP) Mean Arterial Pressure (MAP) Intracranial Pressure (ICP)

CPP = MAP - ICP To maintain CPP: If ICP increases, MAP must increase (autoregulation)

Intracranial Pressure
When MAP cannot increase:
Increased ICP decreases CPP Decreased CPP leads to tissue ischemia Tissue ischemia leads to edema Edema leads to increased ICP Further increased ICP leads to:

Tissue death

When compartmental volume increases and pressure increases, brain tissue moves from one compartment to another

Causes of volume increase

Tumour
Blood
Subdural Epidural Parenchymous

Ischemia Infection

Types of herniation :
Subfalcine
Transtentorial
Descending Ascending

Tonsillar / Foramen Magnum Transcranial / Fungus Cerebri Miscellaneous

Transalar/ Transsphenoidal Middle ear encephalocele

Subfalcine Herniation

Mass effect in cerebral hemisphere forces brain tissue under the falx to opposite side

Subfalcine Herniation:
Structures involved
Falx Cingulate Gyrus Pericallosal Artery Anterior Cerebral Artery Corpus Callosum

Subfalcine Herniation:
Imaging findings
Ipsilateral cingulate gyrus is pushed down and under midline falx Contralateral cingulate gyrus is compressed Depression of ipsilateral corpus callosum and elevation / compression of contralateral corpus callosum

Acquired intracranail Herniation: MR Findings, Laine et al. AJR 1995;165: 967-973

Subfalcine Herniation

Falx meningioma with extensive surrounding edema

Subfalcine Herniation
Displaced pericallosal artery Ipsilateral cingulate gyrus herniates under falx

Contralateral cingulate gyrus compressed Compression of frontal horns of lateral ventricles

Depression / displacement of corpus callosum

Subfalcine Herniation:
Complications and Clinical Signs
Compression of the parafalcine cortex may lead to contralateral leg paresis Anterior cerebral artery infarction may lead to ipsilateral frontal infarcts Increased ICP leads to somnolence

http://missinglink.ucsf.edu/lm/ids_104_cerebrovasc_neuropath/Case4/CerebralArteryDistribution.htm

Subfalcine Herniation:
Complications
CT Angiogram of patient with subdural hematoma on the right shows displacement of anterior cerebral artery without evidence of infarction

Subfalcine Herniation:
Complications
Right sided mass effect was treated by craniotomy. Hypodensity in the right frontal lobe exemplifies anterior cerebral artery infarct after prolonged subfalcine herniation

Transtentorial Herniation

Descending Ascending

Descending Transtentorial Herniation

Supratentorial mass effect forces cerebral structures downward through the opening (incisura) of the tentorium

Descending Transtentorial Herniation:

Structures Involved
Tentorium Uncus Parahippocampal gyrus Perimesencephalic cistern Mesencephalon Posterior cerebral artery (-branches) Anterior Choroidal Artery Oculomotor Nerve (NIII)

Descending Transtentorial Herniation:

Imaging Findings
Asymmetry of ambient cistern (ipsilateral widening / contralateral effacement) Widening of contralateral temporal horn of lateral ventricle Herniated brain is forced medially and inferiorly beneath tentorium, into perimesencephalic cistern Compression of ipsilateral cerebral peduncle by uncus Compression of contralateral cerebral peduncle against tentorial edge (Kernohans Notch)
Acquired intracranail Herniation: MR Findings, Laine et al. AJR 1995;165: 967-973

Descending Transtentorial Herniation

Chronic (hypodens) rightsided subdural hematoma with unilateral descending transtentorial herniation

Descending transtentorial herniation

Shift of midline structures
Blood Compression of lateral ventricles Widened temporal horn

Medial, downward displacement of uncus over right tentorial edge

Descending transtentorial herniation

Herniated uncus in perimesencephalic cistern Ipsilateral midbrain compression Asymmetric ambient cistern

Descending transtentorial herniation

Subdural collection on the left with massive descending transtentorial herniation

Descending transtentorial herniation

Clinical Findings
Oculomotor (NIII) nerve palsy by compression of third cranial nerve against tentorial edge:
Exotropic, hypotropic eye position (down and outward) Ipsilateral ptosis Ipsilateral fixed, dilated pupil

http://scalpelorsword.blogspot.com/2007/01/droopy-eye.html

http://www.bartleby.com

Descending transtentorial herniation

Clinical Findings
Classic localizing sign: Damage to the pyramidal tracts causes contralateral hemiparesis Kernohans (Notch) phenomenon: compression of contralateral cerebral peduncle against tentorial edge may result in ipsilateral hemiparesis: False localizing sign

Kernohan, J.W., & Woltman, H.W. (1929). Incisura of the crus due to contralateral brain tumor. Archives of Neurology and Psychiatry, 21, 274-287

Descending Transtentorial Herniation

Complications
Focal infarction of uncus / parahippocampal gyrus Infarction by compression of posterior cerebral artery (-branches) against the tentorial edge Infarction by direct compression of the anterior choroidal artery by the uncus Brainstem (Duret) hemorrhage

Complications:
Vascular structures at risk

MRI images of the vascular structures around the uncus (posterior cerebral artery and anterior choroidal artery). The latter originates from the anterior cerebral artery and traverses along the uncus dorsally (arrows)
Wiesmann et al. Identification and Anatomic Description of the Anterior Choroidal Artery by Use of 3D-TOF Source and 3DCISS MR Imaging AJNR Am J Neuroradiol 2001; 22: 305-310

Complications:
Vascular structures at risk
Anterior choroidal artery compression may lead to infarcts in the posterior limb of the internal capsule and the lateral aspect of the thalamus Posterior cerebral artery compression leads to cerebellar and occipital lobe infarction but may also cause thalamic infarcts

Complications:
Duret Hemorrhage
Prolonged and progressive transtentorial herniation leads to stretching and eventually rupture of perforating arteries and venules in the brainstem causing punctate hemorrhage
http://rad.usuhs.mil/rad/herniation/herniation.html#intro

Complications:
Duret Hemorrhage

Extensive descending transtentorial herniation resulting from gunshot wound to the head; Initial CT scan shows Duret hemorrhage

Ascending Transtentorial Herniation

Posterior fossa mass forces cerebellar structures upwards through tentorial incisura

Ascending Transtentorial Herniation

Structures involved
As in descending transtentorial herniation Quadrigeminal plate cistern can be involved

Ascending Transtentorial Herniation

Imaging findings
Upward displacement of upper cerebellar surface Asymmetry/ effacement of ambient cisterns Compression of mesencephalon Asymmetry/ effacement of quadrigeminal plate cistern Often bilateral changes

Ascending Transtentorial Herniation

Hemorrhagic arteriovenous malformation in the left cerebellar hemisphere with bilateral ascending transtentorial herniation

Ascending Transtentorial Herniation

Effacement of perimesencephalic cistern Upper surface of cerebellar hemisphere ascends through tentorial incisura Hemorrhagic lesion

Ascending Transtentorial Herniation

Effacement of quadrigeminal plate cisterns

Herniation of cerebellum through tentorial incisura

Ascending Transtentorial Herniation

Right sided hemorrhagic contusions with mass effect in the right cerebellum causing unilateral ascending transtentorial herniation

Ascending Transtentorial Herniation

Unilateral ascending transtentorial herniation on the right side with compression of the right cerebral peduncle Dilated temporal horn of left ventricle indicating onset of hydrocephalus

Ascending Transtentorial Herniation

special notes
Subtle imaging changes Less frequent (-ly noted?) than descending herniation

Often bilateral (no asymmetry)

Differing appearance of perimesencephalic cisterns with different gantry angles in CT may complicate imaging findings

Ascending Transtentorial Herniation

Clinical findings and Complications
Slowly evolving posterior fossa mass effect will often present with signs of hydrocephalus: Headache, nausea, vomiting etc. Compression of arteries (posterior cerebral, superior cerebellar) may lead to cerebral / occipital or cerebellar infarction Rapidly expanding lesions present with emergency clinical findings due to compression of brainstem nuclei: respiratory failure, coma and death (often coexistent with foramen magnum herniation)

Tonsillar / Foramen Magnum Herniation

Posterior fossa mass effect forces cerebellar tonsils downward through the foramen magnum

Tonsillar Herniation
Structures involved

Skull base / foramen magnum

Cerebellar tonsils Posterior inferior cerebellar artery

Tonsillar Herniation

Right sided tonsillar herniation

Tonsillar Herniation
Anteriorly displaced cervical myelum

Inferiorly displaced right cerebellar tonsil

Tonsillar Herniation
Special notes
(Subtle) extension of cerebellar tonsils in the spinal canal may be seen in normal individuals or in Chiari I malformation Extension of 5 mm or more below the foramen magnum is considered abnormal
Often coexisting with ascending transtentorial herniation
Aboullez etal. Position of cerebellar tonsils in the normal population and in patients with Chiari I malformation: A quantative approach with MR Imaging. J Comp Assist Tomogr 1985;9: 1033-1036

Tonsillar Herniation

Chiari I malformation with extension of the cerebellum in the upper cervical spinal canal in a patient with a frontal mass

Tonsillar Herniation
Clinical findings / Complications
Compression of brain stem nuclei causes respiratory / cardiac failure, coma, death Compression of posterior inferior cerebellar artery (PICA) may cause cerebellar infarcts

Patients with Chiari I malformation can be symptom free but may experience dysesthesia with cervical flexion: Lhermitte phenomenon

Transcranial Herniation
Fungus Cerebri

Intracranial mass effect forces cerebral structures outward through (iatrogenic) calvarial defect

Transcranial Herniation

Decompressive right frontal craniotomy in two patients with severe right sided mass effect. Cerebral structures herniate through calvarial defect. Note that there is no midline shift. Mass effect is orientated away from contralateral hemisphere.

Transcranial Herniation
Complications
Decompression usually improves patient survival by lowering intracranial pressure and preventing / undoing intracranial herniations

Herniation may lead to infarction of herniated tissue Exposed brain / dura is prone to infection

Miscellaneous

Transalar / Sphenoidal Herniation Middle Ear Encephaloceles

Transalar / Sphenoidal Herniation

Anterior cranial fossa mass effect forces cerebral structures over the edge of the sphenoid bone into the middle cranial fossa

Transalar / Sphenoidal Herniation

Coexisting with other forms of herniation Rarely recognized; Imaging may show displacement of middle cerebral artery No specific clinical signs. Complications include (rarely) middle cerebral artery infarct

Middle ear (Skull base) encephalocele

Cerebral structures bulge through (acquired) skull base defect NO PRESSURE COMPONENTS

Middle Ear Encephalocele

Left temporal bone defect with protruding cerebral tissue

Papanikolaou et al. Skull Base. 2007 September; 17(5): 311-316

Middle Ear Encephalocele

Cerebral tissue protruding in left temporal bone

Middle Ear Encephalocele

Focal osseous defect in left tegmen
Protrusion of cerebral structures in epitympanic space surrounding the ossicles

Skull base encephalocele

May occur anywhere along the skull base Osseous defects may be iatrogenic or congenital

No specific clinical findings