Download differential diagnosis of corpus callosum lesions

DIFFERENTIAL DIAGNOSIS OF
CORPUS CALLOSUM LESIONS USING
THE MIDLINE SAGITTAL PLANE
Chapel Hill, NC
Ames, Romy
Amunugama, Monika
Sepulveda, Francisco
Causil, Lazaro
Zamora, Carlos
Castillo, Mauricio
DISCLOSURES
The authors have no pertinent disclosures.
INTRODUCTION
The corpus callosum (CC) is the largest white matter
commissure between the cerebral hemispheres. It is
traditionally divided into 4 segments (rostrum, genu,
body and splenium) and 2 borders (upper and lower).
Some authors consider the “isthmus” (between the
posterior body and splenium) as a 5th segment. It is
susceptible to involvement by a variety of conditions
and diseases. Our aim is to demonstrate findings on
sagittal MRI that may lead to a correct diagnosis.
Lymphoma
Diffuse
DWM/DAI/Agenesis
Oligodendroglioma/GB
Mucopolysaccharidoses
Aicardi Sd./Post shunt
Ischemia
Callosotomy
Rostrum/
Genu
Partial agenesis
Krabbe Disease
CORPUS
CALLOSUM
LESIONS,
Predominant
locations
Venus thrombosis
Body
SEGMENTAL
Marchiafava-Bignami
Susac’s syndrome
Periventricular leukomalacia
ADEM
Hypoglycemia
Splenium
Focal
Carbamazepine
MERS
Adrenoleukodystrophy
Lower
Multiple Sclerosis
BORDERS
AVM/ Cavernoma
Upper
Lipomas
Acute Hematoma
LESIONS THAT AFFECT
THE CORPUS CALLOSUM
DIFFUSELY
GLIOBLASTOMA
A
B
C
(A) Axial T2WI, (B) sagittal post contrast T1WI and (C) coronal post contrast T1WI show an irregular T2hyperintense ring enhancing lesion (arrows) with transcallosal spread resulting in a “butterfly” configuration.
• Most common primary intracranial neoplasm of adults (12-15%).
• Frequently found at 45–70 years of age, poor prognosis.
• Imaging: ring enhancing lesions with central necrosis, preferential spread along
condensed white matter tracts such as the CC.
OLIGODENDROGLIOMA
A
B
C
(A) Axial unenhanced CT shows a calcified mass in the right frontal region (green arrows), (B) sagittal and (C)
axial post contrast T1WI show minimal enhancement, infiltration of the anterior CC (red arrows) and mass effect.
• 3rd most common glioma, 5-18% of all glial tumors.
• Imaging: heterogeneous, ill-defined tumor with variable enhancement,
tendency to involve the cortex and calcifications (50- 90%). It commonly arises
in the frontal lobes and can cross to the contralateral hemisphere via the CC.
PRIMARY LYMPHOMA
A
B
C
(A) Sagittal post contrast T1WI shows a nearly homogeneous enhancing lesion in the CC. (B) Axial T1WI
shows that the lesion crosses the midline through the genu (red arrow). The lesion is hypointense on axial
T2WI (green arrow, C) .
• 1-2% of intracranial tumors, particularly seen in immunocompromised patients
(where they are usually necrotic). Most are of the non Hodgkin's type.
• Imaging: restricted diffusion on DWI, predilection for the meninges and ependyma,
typically found in deep basal ganglia, periventricular region and CC.
MUCOPOLYSACCHARIDOSES
A
B
C
(A) Sagittal T2WI and T1WI (B) show dilated perivascular spaces in the CC. (C) Axial T1WI shows a cribriform
pattern (red arrow) of enlarged perivascular spaces in the subcortical and deep white matter and posterior
centrum semiovale (white arrow).
• Mucopolysaccharidoses lead to accumulation of glycosaminoglycans in many
organs including the brain.
• Imaging: enlarged perivascular spaces, usually followed by white matter changes
and atrophy.
POST SHUNT DECOMPRESSION GLIOSIS
A
B
(A) Sagittal FLAIR and (B) axial unenhanced CT show gliosis in the CC following shunting of prolonged
hydrocephalus. A “wavy” superior callosal border is typical of this complication.
• Prolonged ventriculomegaly causes stretching and impingement of the CC against
the rigid falx cerebri resulting in chronic ischemia. Rapid decompression of long
standing hydrocephalus results in transcallosal demyelination.
• Gliosis may affect the entire callosal thickness but an upper wavy border is typical.
POST SHUNTING CYSTIC
DEGENERATION
Sagittal T1 and axial FLAIR show cystic degeneration of the CC after chronic shunting probably
due to relief of compression against the falx.
POST SHUNTING FOCAL CALLOSAL
DEGENERATION
In this patient, sagittal T1, axial FLAIR, and coronal T2 show post shunting CC involvement only in the
body (arrow), unlike other cases in which the process is diffuse.
ISCHEMIC STROKE OF THE CORPUS
CALLOSUM
A
B
C
(A) Axial DWI and (B) axial ADC show restricted diffusion in the CC (red arrow). (C) Sagittal FLAIR shows
hyperintensity involving the entire thickness of the CC in this patient with acute stroke (green arrow).
• The corpus callosum has a rich blood supply from the anterior communicating artery
which supplies the genu, the pericallosal artery which supplies the body, and the
posterior pericallosal artery, a branch of the posterior cerebral artery, which feeds the
splenium, thus infarcts are rare and usually of the watershed type.
• Imaging: restricted diffusion (earliest sign) and increased T2/FLAIR signal.
AICARDI SYNDROME
A
B
C
(A) Sagittal T1WI shows dysgenesis of the CC (red arrow) and pontocerebellar hypoplasia (orange arrow).
(B) Axial T2WI, shows colpocephaly (green arrow). (C) Corornal T2WI shows polymicrogyria (pink arrow).
• Rare neurologic disorder resulting from an X-linked genetic defect, occurs almost
exclusively in females. Presents with infantile spasms and chorioretinal lacunae
(pathognomonic).
• Imaging: CC dysgenesis, colpocephaly, heterotopia, posterior fossa abnormalities
and intracranial cysts.
DANDY WALKER MALFORMATION
Sagittal T1WI shows CC (red arrow) and vermian hypoplasia, cephalad rotation of vermian remnant (pink
arrows), cystic dilatation of the 4th ventricle (green arrow) and an enlarged posterior fossa (orange arrow)
• DWM is the most common posterior fossa malformation. It can be associated with
supratentorial anomalies (CC dysgenesis and neuronal migration disorders).
• Imaging: vermian hypoplasia and cephalad rotation of vermian remnant, cystic
dilatation of 4th ventricle, enlarged posterior fossa with torcular-lambdoid inversion.
AGENESIS
TOTAL
A
HYPOPLASIA
B
(A) Sagittal T1WI shows CC agenesis (red arrow). (B) Sagittal T1WI shows CC hypoplasia (pink arrow).
• Dysgenesis can be complete or partial. In partial agenesis, a part of the corpus
callosum is absent.
• Imaging features: high-riding 3rd ventricle, parallel and non-converging lateral
ventricles, colpocephaly, and inverted Probst bundles indenting the superomedial
surface of the lateral ventricles.
LESIONS THAT FOCALLY
AFFECT THE CORPUS
CALLOSUM
ANTERIOR INVOLVEMENT,
PARTIAL AGENESIS
Sagittal T1WI shows partial agenesis of the CC (arrow). The genu and anterior body are absent and the
remaining CC is dysplastic and fused with the fornices.
ANTERIOR INVOLVEMENT
POST SURGICAL
A
B
(A) Sagittal post contrast T1WI and (B) axial T2WI shows resection of the anterior CC and part of the right
frontal lobe secondary to brain tumor.
• Post surgical changes can be seen in the CC either following callosotomy for
intractable seizures or following resection of a tumor that involved the CC.
SEGMENTAL BODY INVOLVEMENT
SUSAC’S SYNDROME
A
B
C
(A) Sagittal T2WI, (B) sagittal post contrast T1WI, and coronal T2WI (C) show CC ‘snowball’ lesions (arrows)
whose central location makes them highly suggestive of Susac’s syndrome.
• AKA: Retinocochleocerebral arteriopathy
• Clinical triad – encephalopathy, bilateral hearing loss, and branch retinal artery
occlusion. Usually self-limited.
• Imaging: Similar to MS/ transcallosal lesions/ gray matter microinfarctions but no
demyelination. Unlike MS, lesions are not isolated to the callososeptal interface.
SEGMENTAL BODY INVOLVEMENT
MARCHIAFAVA BIGNAMI
Sagittal T2WI shows diffuse swelling of the posterior body and splenium of the CC (arrow) with predilection
for its central portion.
• Primary degeneration of the corpus callosum secondary to alcoholism or malnutrition.
(vitamin B deficiency). Usually men aged 40-60 years.
• Imaging: callosal demyelination and necrosis with subsequent atrophy, with a
predilection for the central fibers.
SEGMENTAL BODY INVOLVEMENT
KRABBE DISEASE
A
B
(A) Mid sagittal T1WI shows diffuse CC thinning more pronounced in the body (arrow). Axial T2WI (B)
shows significant and diffuse CC atrophy in a patient with end stage disease.
• Autosomal recessive disorder with abnormal accumulation of galactocerebroside
in macrophages, leading to formation of large multinucleated globoid cells.
• Imaging: involvement of pyramidal tracts, CC and parieto-occipital white matter,
which appears hyperintense on T2. Subcortical U fibers are generally spared.
SEGMENTAL BODY INVOLVEMENT
VENOUS THROMBOSIS
A
B
C
(A) Mid sagittal, (B) coronal and CC) axial T2 images show involvement of the CC body (arrow) and
adjacent white matter in a patient with thrombosis of the inferior sagittal sinus.
• Risk factors: Dehydration, oral contraceptives, pregnancy, cirrhosis, extrinsic
compression, invasion by tumors.
• Imaging: variable diffusion abnormalities in deep gray nuclei and CC. May involve
part or all of the CC. Hemorrhage is common.
SEGMENTAL BODY INVOLVEMENT
PERIVENTRICULAR LEUKOMALACIA
A
B
(A) Sagittal T1WI and (b) axial T2WI shows thinning of the CC body (arrow) in periventricular leukomalacia
• 2nd most common CNS complication in premature infants after hemorrhage.
• Imaging: early stage periventricular white matter necrosis, subacute stage cyst
formation and late stage parenchymal loss, ventricular enlargement, thinning of the
CC due to degeneration of transcallosal fibers mostly in splenium and posterior body.
SEGMENTAL POSTERIOR INVOLVEMENT
ADRENOLEUKODYSTROPHY
A
B
C
(A) Sagittal and (B) axial post contrast T1WI show the characteristic pattern of symmetric contrast
enhancement at the zone of active demyelination (red arrows). (C) Axial T2WI shows confluent and symmetric
hyperintense signal abnormalities in the CC splenium , occipital and parietal lobes bilaterally (pink arrow).
• Autosomal recessive disorder caused by acyl-CoA synthetase deficiency, leading
to accumulation of very long chain fatty acids in tissues/plasma.
• Imaging: 80% show demyelination in the splenium spreading symmetrically to
the occipital/parietal lobes. Contrast enhancement of zones of active
demyelination is usually observed.
SEGMENTAL POSTERIOR INVOLVEMENT
CARBON MONOXIDE INTOXICATION
A
B
C
(A) Axial T2 and (B) DWI show high signal in the splenium (arrow). (C) Axial DWI shows restricted
diffusion in the globus pallidi bilaterally.
• In acute intoxication, patients may develop progressive white matter demyelination
which is variable, ranging from discrete perivascular CC foci to axonal destruction.
• Imaging: MRI is not specific, including high signal in T2 and FLAIR sequences as
well as a diffusion restriction in the CC.
SEGMENTAL POSTERIOR INVOLVEMENT
DIFFUSE AXONAL INJURY
A
B
C
(A) Axial DWI shows restricted diffusion in the splenium. In a different patient T2WI (B) shows a high signal
splenial lesion with loss of anisotropy (arrow) on the (C) DTI directionality map.
•
•
•
•
Consequence of trauma, involves parasagittal white matter, CC and brainstem.
Results from acceleration/deceleration that shear axons.
Splenium and posterior CC are more commonly affected.
Imaging: hemorrhage, hypointensity on T2/SWI images. Non hemorrhagic lesions
show restricted diffusion.
SEGMENTAL POSTERIOR INVOLVEMENT
INFLUENZA RM 14/01/2008
RM 19/12/2007
A
B
C
(A) Patient presenting with encephalopathy and a splenial lesion on DWI (arrow). One month later after
resolution of symptoms (B-C) the lesion has essentially resolved.
SEGMENTAL POSTERIOR INVOLVEMENT
ADEM
A
B
(A & B) Axial T2 images show typical patchy white and gray matter ADEM lesions with little mass effect.
Note bilateral splenial involvement in A (arrows).
• Rare, immune mediated inflammatory disease characterized by wide spread
demyelination triggered by viral infection or vaccination.
• Children and adolescents are commonly affected.
• Imaging: Bilateral and asymmetrical white matter lesions similar to MS although
callososeptal interface involvement is unusual and lesions are typically larger.
TRANSIENT LESIONS IN THE SPLENIUM OF
THE CORPUS CALLOSUM
• Various causes: patients receiving antiepileptic
drugs, hypoglycemia, post seizures, high-altitude
and infective agents (influenza, rotavirus, mumps,
Escherichia coli, adenovirus).
• Possibly due to intramyelinic edema.
• Needs to be considered to avoid unnecessary
invasive diagnostic and therapeutic measures.
PHARMACOLOGICAL AGENTS
(Carbamazepine)
1 MONTH LATER
A
B
22-year-old female with acute withdrawal of carbamazepine. (A) sagittal post contrast T1WI shows
hypointensity in the splenium of the CC (red arrow). (B) One month later, follow-up sagittal T1WI shows no
abnormal signal changes.
POST SEIZURES SPLENIAL LESION
1 MONTH
LATER
A
B
C
30 -year-old male with seizures. (A) Axial DWI shows diffusion restriction in the splenium of the CC (red
arrow)(B) Sagittal FLAIR shows high-intensity in the splenium (green arrow). (C) Axial DWI one month later
shows resolution of the signal abnormalities.
HYPOGLYCEMIA
A
B
C
Patient with severe hypoglycemia. (A) Axial DWI and (B) ADC map show diffusion restriction in the splenium
of the CC (red arrow). (C) Axial FLAIR shows mild high-intensity in the splenium (green arrow).
•
•
•
Severe hypoglycemia usually affects the occipital lobes and splenium.
Other affected sites: hippocampi, basal ganglia, internal capsules, cerebellum.
Imaging: restricted diffusion (cytotoxic edema). Completely reverses within 1-2 days
following appropriate correction of hypoglycemia.
LESIONS THAT
PREFERENTIALLY AFFECT
THE BORDERS OF THE
CORPUS CALLOSUM
UPPER BORDER
ARTERIOVENOUS MALFORMATION (AVM)
A
B
C
(A) Axial T2WI, (B) coronal T1WI and (C) sagittal T1WI post contrast shows an enhancing lesion with multiple
flow voids in the upper and mid CC regions.
• In this location: 10% of all cerebral AVMs.
• Commonly present with intraventricular hemorrhage.
• Imaging: shows typical signal voids and tortuous vasculature.
UPPER BORDER CAVERNOMA
A
B
(A) Mid sagittal T1 and (B) Axial FLAIR images show a cavernoma in the genu and anterior body of the CC.
• Cavernoma of the CC is very rare.
• Imaging appearance is similar to cavernomas in other regions of the brain.
• They may be located anywhere in the corpus callosum.
UPPER BORDER
CORPUS CALLOSUM LIPOMA
Sagittal T1WI shows a curvilinear lipoma along the upper margin of the CC (arrow).
• Lipomas are considered congenital malformations of the primitive meninx.
• They are classified as curvilinear or tubulonodular (most common).
• Imaging: fat-containing lesions in the interhemispheric fissure closely related to
the corpus callosum. In curvilinear types the CC is normal or mildly dysplastic.
LOWER BORDER, ACUTE HEMATOMA
A
B
(A) Axial unenhanced CT and (B) sagittal post contrast CT show a hematoma (arrow) in the upper CC in a
patient who was found to have an aneurysm of the pericallosal artery.
• Ruptured aneurysms of the anterior cerebral artery or the pericallosal artery may
cause CC hemorrhage. Hemorrhage in this location can also be associated with
ventriculostomies. Other less frequent etiologies: isolated cavernoma of the CC or
microhemorrhages occurring in severe, non-lethal high-altitude cerebral edema.
LOWER BORDER
MULTIPLE SCLEROSIS
A
B
(A) & (B) Sagittal FLAIR images (2 different patients) show bright lesions typically affecting the callososeptal
margin (arrows).
• Most common inflammatory demyelinating disease of the CNS.
• Young and middle aged females are commonly affected.
• Typical location: periventricular region (“Dawson fingers”), callososeptal surface
(93%), centrum semiovale, deep white matter and basal ganglia.
• In chronic disease the CC undergoes atrophy and becomes thin.
CONCLUSION
MRI in the sagittal plane is useful in the
evaluation of the CC and may provide
clues with respect to the pattern and
extent of different diseases narrowing
the differential diagnosis.
REFERENCES
1.
2.
3.
4.
5.
B Battal, M Kocaoglu, V Akgun et al. Corpus callosum: Normal
imaging appearance, variants and pathologic conditions. Journal of
Medical Imaging and Radiation Oncology 54 (2010) 541–549.
S. A. Friese, M. Bitzer, D. Freudenstein et al. Classification of
acquired lesions of the corpus callosum with MRI. Neuroradiology
2000) 42: 795±802.
A Fitsiori, D Nguyen, A Karentzos et al. The corpus callosum: white
matter or terra incognita. The British Journal of Radiology, 84 (2011),
5–18.
Aamish Z Kazi, Priscilla C Joshi, Abhimanyu B Kelkar et al. MRI
evaluation of pathologies affecting the corpus callosum: A pictorial
essay. Indian J Radiol Imaging. 2013 Oct-Dec; 23(4): 321–332.
David L. Kasow, Sylvie Destian, Carl Braun, John C et al. Corpus
Callosum Infarcts with Atypical Clinical and Radiologic
Presentations. AJNR Am J Neuroradiol 21:1876–1880, Nov/Dec
2000.