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Bilateral Thalamic Lesions

Bilateral Thalamic Lesions

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AJR:192, February 2009 W53
presentation is variable. Wernicke encephal-opathy is a medical emergency managedwith IV thiamine. T2-weighted MR imagesmay show symmetric high signal intensity inthe mamillary bodies, medial aspects o thethalami, tectal plate, periaqueductal graymatter, and dorsal medulla [4]. Contrast en-hancement is variable. Thiamine is an os-motic gradient regulator, and deciency candisrupt the blood–brain barrier, resulting incontrast enhancement [5]. Wernicke enceph-alopathy can have reduced diusion (Fig. 2)owing to ischemia-like changes in the thala-mi that should be dierentiated rom truevenous and arterial inarction [6].
Osmotic Myelinolysis
Osmotic myelinolysis accompanies rapidshits in serum osmolality; the classic settingis the rapid correction o hyponatremia [7].The classic lesion involves the central pons(central pontine myelinolysis). Other lesionsaect the basal ganglia, thalami, and whitematter (extrapontine myelinolysis). Acute T2hyperintensity and T1 hypointensity occur inthe aected regions. Contrast enhancementis uncommon, and reduced diusion may beseen [8] (Fig. 3).
Fabry Disease
Fabry disease is an X-linked disorder o glycosphingolipid metabolism leading toaccumulation o glycosphingolipids in thevascular endothelium, perithelium, smooth-muscle cells, heart, and brain that results inmyocardial ischemia and stroke [9]. On T2-weighted images, lesions o high signal in-tensity due to the vasculopathy may be seen
Bilateral Thalamic Lesions
Alice B. Smith
1,2
 James G. Smirniotopoulos
1,2
 Elisabeth J. Rushing
1,3
Steven J. Goldstein
4
Smith AB, Smirniotopoulos JG, Rushing EJ,Goldstein SJ
1
Department o Radiology and Radiological Sciences,Uniormed Services University, 4301 Jones Bridge Rd.,Bethesda MD 20814. Address correspondence to A. B.Smith (alsmith@usuhs.mil).
2
Department o Radiologic Pathology, Armed ForcesInstitute o Pathology, Washington, DC.
3
Department o Neuropathology and OphthalmicPathology, Armed Forces Institute o Pathology,Washington, DC.
4
Department o Radiology, University o KentuckyCollege o Medicine, Lexington, KY.
Neuroradiology/Head and Neck Imaging • Pictorial Essay
CME
This article is available or CME credit. See www.arrs.org or more inormation.
 WEB
This is a Web exclusive article.
AJR 
2009; 192:W53–W620361–803X/09/1922–W53© American Roentgen Ray Society
B
ilateral thalamic lesions are un-common. These paired lesionshave a limited dierential diag-nosis that includes metabolic andtoxic processes, inection, vascular lesions,and neoplasia. The dierential diagnosis canbe urther narrowed with the patient history,imaging characteristics, and presence or ab-sence o lesions outside the thalami.
Primary Neoplasm
Bilateral thalamic glioma is a rare neo-plasm, usually a diuse low-grade astrocy-toma (World Health Organization grade II),that occurs in both children and adults [1].Bilateral thalamic glioma has a poor progno-sis due to the location o the lesions [2]. Chil-dren typically have signs o increased intra-cranial pressure and movement disorders.Adults experience mental deterioration [1].Typically, expansion o both thalami is ac-companied by abnormal hyperintensity onT2-weighted images and hypointensity onT1-weighted images that is not associatedwith contrast enhancement. Hydrocephalusdepends on the degree o mass eect. Diu-sion is normal (Fig. 1).
Metabolic and Toxic Disorders
Many metabolic and toxic processes a-ect both thalami simultaneously.
Wernicke Encephalopathy 
Wernicke encephalopathy results rom adeciency o vitamin B
1
and is requently as-sociated with alcohol abuse [3]. The classicclinical triad is ataxia, altered consciousness,and abnormal eye movements; however, the
Keywords:
bilateral thalamic, metabolic brain disorders,prion disease, viral encephalitisDOI:10.2214/AJR.08.1585Received July 24, 2008; accepted ater revisionAugust 29, 2008.
OBJECTIVE.
The purpose o this study was to present the neuroimaging ndings anddierential diagnosis o bilateral thalamic lesions.
CONCLUSION.
The limited dierential diagnosis o bilateral thalamic lesions can beurther narrowed with knowledge o the specic imaging characteristics o the lesions incombination with the patient history.
Smith et al.Bilateral Thalamic LesionsNeuroradiology/Head and Neck ImagingPictorial Essay
 
W54 AJR:192, February 2009
Smith et al.
in the deep white and gray matter. T1 hyperin-tensity in the pulvinar is a common and sensi-tive nding [10]. Pulvinar hypointensity maybe seen on T2-weighted images but not con-sistently (Fig. 4). The cause o these changesin signal intensity is undetermined [9].
Fahr Disease
Fahr disease is a rare disease o unknowncausation. It is characterized clinically byneuropsychiatric abnormalities and parkin-sonian or choreoathetotic movement disor-der. Extensive bilateral calcication o thedeep gray matter is present and most re-quently involves the globus pallidus (Fig. 5).Other areas o involvement include the puta-men, caudate nuclei, thalami, and dentatenuclei [11]. Calcium–phosphorus metabo-lism is normal in these patients [11]. The T1and T2 signal intensity in the calcied re-gions varies with disease stage and calcica-tion [11]. The dierential diagnosis o theseparenchymal calcications includes endo-crinologic disorders such as hyperparathy-roidism, hypoparathyroidism, and pseudo-hypoparathyroidism.
Wilson Disease
Wilson disease is an autosomal recessiveinborn error o copper metabolism. Patientshave cirrhosis, corneal Kayser-Fleischer rings,and degeneration o the basal ganglia. I thepatient is not treated, the disease is progres-sive and atal. MR images show symmetricT2 hyperintensity o the deep gray matter:putamina, globus pallidi, caudate nuclei, andthe thalami. T1 signal intensity in the basalganglia and thalami is usually reduced, butT1 signal intensity may increase owing to theparamagnetic eects o copper [12]. Contrastenhancement does not occur (Fig. 6). Evi-dence o restricted diusion may be seen onearly images and is ollowed by return to nor-mal diusivity ater necrosis and spongiormdegeneration have occurred [13].
Leigh Disease
Leigh disease is a genetically heterogeneousmitochondrial disorder in which progressiveneurodegeneration leads to respiratory ailureand death in childhood. Patients have elevatedlevels o lactate in the CSF, serum, and urine.On T2-weighted images hyperintensity maybe seen in the involved regions, most requent-ly the basal ganglia, diencephalon, brainstem,thalami, and dentate nuclei [14]. MR spectros-copy reveals a decreased level o 
 N 
-acetylaspartate with elevated choline and lactate lev-els [15]. Contrast enhancement is uncommon(Fig. 7). In the acute phase, reduced diusionmay be seen.
Infection
Many viral orms o encephalitis involvethe thalami, including West Nile encephali-tis, Japanese encephalitis, Murray Valley en-cephalitis, Eastern equine encephalitis, andrabies. West Nile encephalitis is a single-strand RNA virus o the favivirus amilytransmitted to humans rom birds by culicinemosquitoes. It has been a summer seasonalepidemic in the United States since 1999.West Nile encephalitis causes bilateral T2hyperintensity in both thalami, the basalganglia, and the midbrain. Sulcal T2 hyper-intensity has also been reported, suggestingleptomeningeal infammation [16] (Fig. 8).Contrast enhancement is variable. Reduceddiusion has been reported, most oten inthe posterior limb o the internal capsule,corona radiata, and subcortical white matter[16].Creutzeldt-Jakob disease (CJD) is a rareneurodegenerative disease caused by the ac-cumulation o prion proteins in neurons. Per-sons with CJD experience rapidly progressivedementia. The disease is classied into threetypes. Most common (
85% o cases) is thesporadic orm, o which no cause has beenidentied. The amilial orm accounts or ap-proximately 15% o cases, and the inectious(variant CJD) or iatrogenic orm is least com-mon, making up less than 1% o cases. Imag-ing may reveal T2 prolongation and reduceddiusion in the basal ganglia, thalami, andcortex (cortical ribboning) [17] (Fig. 9).There is no contrast enhancement. Diusecortical atrophy occurs late in the course.A key imaging nding in variant CJD isthe pulvinar sign—high T2 signal intensityin the pulvinar (Fig. 10). This sign has a sen-sitivity o 68–90% or variant CJD and wasonce considered pathognomonic o variantCJD; however, it can also occur in sporadicCJD [18, 19]. The hockey stick sign (sym-metric pulvinar and dorsomedial hyperinten-sity) is characteristic o variant CJD [18].Cortical ribbon hyperintensity is rarely seenin variant CJD (Fig. 11).
Vascular Occlusion
Bilateral thalamic arterial inarcts are un-common. The thalami are supplied by bothanterior (anteroinerior thalami) and poste-rior (medial thalami) circulation, but severalvariations occur. Top o the basilar syndromeresults in inarcts o the superior cerebellarand posterior cerebral artery territories (Fig.12). The artery o Percheron, a variant, is asolitary arterial trunk arising rom the proxi-mal segment o the posterior cerebral arteryand supplying the paramedian thalami androstral midbrain bilaterally. Occlusion causesbilateral thalamic inarction.Deep venous thrombosis typically resultsin bilateral symmetric involvement o thethalami and occasionally the basal ganglia.The causes include pregnancy, oral contra-ceptives, inection, trauma, and dehydration,but the cause is undetermined in 20–25% o patients [20]. An abnormally hyperdensevein may be seen on CT scans, and corre-sponding T1 hyperintensity rom clot in thesinuses may be seen on MR images. CT andMR venography show no areas o contrastenhancement or signal intensity in the deepvenous sinuses. Diusion-weighted imagingmay show heterogeneous signal intensity[21]. Patchy contrast enhancement may beseen (Fig. 13).Mild to moderate cerebral hypotensioncauses refex shunting o blood rom the an-terior to the posterior circulation to preservethe brainstem, basal ganglia, and cerebel-lum. Severe reduction in blood fow exceedsthis mechanism, and protective shunting o blood no longer occurs. The result is damageto the deep cerebral nuclei, brainstem, andmost active regions o the cerebral cortex[22]. Diusion-weighted MRI is the earliestimaging technique to have abnormal nd-ings [22] (Fig. 14).Posterior reversible encephalopathy syn-drome (Fig. 15) is a disorder o cerebral vas-cular autoregulation. The multiple causes,which are oten but not always associatedwith hypertension [23], include glomerulone-phritis, preeclampsia and eclampsia, anddrug toxicity (cyclosporin). Symptoms in-clude headache, seizures, and visual distur-bance. CT and MRI typically show symmet-ric areas o vasogenic edema predominantlyinvolving the posterior circulation. Localizedmass eect, hemorrhage, and subtle enhance-ment are uncommon. Diusion-weightedMRI ndings usually are normal, but occa-sionally reduced diusion occurs, suggestingthe presence o cytotoxic edema [24].
Conclusion
Bilateral thalamic lesions have a variety o causes, and knowledge o the associated im-aging ndings can help narrow the dieren-tial diagnosis.
 
AJR:192, February 2009 W55
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A
Fig. 1—
52-year-old woman with bilateral thalamic glioma.
A,
Axial T2-weighted MR image shows hyperintensity and bilateral diuse enlargement o thalami resulting in hydrocephalus due to mass eect.
B,
Axial T1-weighted gadolinium-enhanced MR image shows bilateral low signal intensity within thalami and no associated contrast enhancement.
C,
Apparent diusion coecient map shows high signal intensity in both thalami.
C
B

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