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Clinical Neuroradiology

Review Article

Differential Diagnosis of Bilateral Thalamic Lesions

Jennifer Linn1, Adrian Danek2, Lisa Ann Hoffmann3, Klaus C. Seelos1, Hartmut Brckmann1

Abstract The thalamus is a conglomerate consisting of more than 100 nuclei. It functions as the last relay site of all the afferent pathways, with the exception of the olfactory pathway. Thus, it is the gateway to consciousness. The aim of this study is to provide an overview of the functional anatomy of the thalamus and its blood supply. Furthermore, the most frequent pathologies leading to bilateral lesions of the thalamus will be described. These disorders include, above all, vascular disorders, which must be differentiated into arterial ischemia and venous infarctions, but also different tumors, infectious and demyelinating diseases, as well as metabolic-toxic alterations. Key Words: Thalamus Bilateral lesions MRI
Clin Neuroradiol 2007;17:322 DOI: 10.1007/s00062-007-7000-x

Differentialdiagnose bithalamischer Pathologien

Zusammenfassung Die Thalami stellen ein Konglomerat aus ber 100 Kernen dar und fungieren als letzte Umschaltstation aller afferenten Bahnen auer der olfaktorischen und damit als Tor zum Bewusstsein. Diese Arbeit soll einen berblick ber die funktionelle Anatomie und die Gefversorgung der Thalami liefern. Darber hinaus werden die hufigsten Krankheitsbilder, die sich mit bilateralen Thalamuslsionen manifestieren knnen, vorgestellt. Dazu gehren vor allem vaskulre Erkrankungen, wobei zwischen arteriellen Ischmien und vensen Infarkten unterschieden werden muss, aber auch verschiedene Tumoren, infektise oder demyelinisierende Erkrankungen sowie metabolisch toxische Vernderungen. Schlsselwrter: Thalamus Bilaterale Lsionen MRT

Introduction As the main part of the diencephalon, the thalamus consists of more than 100 different nuclei. It plays a central role in sensory perception as the gateway to consciousness. Since the various nuclear regions of the thalamus have multiple functions, the clinical manifestation of thalamic pathologies varies greatly, depending on the site and the extent of the damage.

Bilateral lesions of homologous brain areas, in general, represent exceptional cases in topical diagnostics. While at least a part of the function of a brain area that has been affected by a unilateral lesion can often be compensated for by the opposite healthy side, bilateral lesions lead to a loss of function in homologous areas in both hemispheres. The absence of a healthy hemisphere to compensate for this loss causes specific symp-

Department of Neuroradiology, University Hospital Munich Grohadern, Munich, Germany, Department of Neurology, University Hospital Munich Grohadern, Munich, Germany, 3 Institute of Clinical Neuroimmunology, University Hospital Munich Grohadern, Munich, Germany.

Received: December 11, 2006; accepted: January 16, 2007

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Table 1. Clinical findings in bilateral lesions of the thalamus. Clinical aspects Incentive disorder (asthenia) Disorders of alertness and sleep Amnestic syndrome Thalamic dementia Partial Klver-Bucy syndrome (hyperorality, hypersexuality, increased distractibility) Disorder of executive functions Akinetic mutism (= absence of spontaneous vocal utterances with generally impoverished motor activity but intact eye movements) Complex movement disorders (e.g., tremor, dystonia, choreoathetosis, myoclonus) Personality changes Lesion site (Paramedian) thalamus Nonspecific thalamic nuclei, relay stations in ascending reticular activating system Anterior or inferomedial thalamus Bilateral paramedian thalamic infarction Paramedian thalamus Disconnection of the temporal poles (?) Mediodorsal nucleus as a relay to the frontal lobes Paramedian thalamus (cortex-basal ganglia-thalamus connection) Posterior and posterolateral thalamic nuclei Dorsomedial nuclei that have connections to the amygdala, the temporal neocortex, and the prefrontal region

toms, which, as a rule, are not seen when there is only unilateral damage of the affected region [2]. The aim of this study is to provide an overview of the neuroradiologic differential diagnoses of bithalamic lesions. The typical clinical symptoms of these lesions are compiled in Table 1.

borders the thalamus laterally. The thalamus is not a uniform cellular complex, but rather a conglomerate of numerous nuclei with various afferences and efferences. In most humans (ca. 7080%) the two thalamic bodies are connected by the adhesio interthalamica (massa intermedia), which runs through the third ventricle (Figure 1). The Anatomy of the Thalamus The thalamus is divided into three larger nuclear The thalamus measures ca. 3 1.5 cm, constitutes the groups by Y-shaped bands of white matter (internal main bulk of the diencephalon, and forms the lateral medullary lamina; Figure 2): the anterior nuclei in the boundary to the third ventricle. The internal capsule forking part of the Y, the ventrolateral nuclei which lie more laterally, and the medial nuclei, more medially. These three a b large nuclear groups are subdivided further according to cytological and functional aspects, with the result that today 120 subgroups can be distinguished [3]. Another large nuclear complex joins on to these nuclei caudally: the pulvinar (Figure 2). The medial and lateral geniculate bodies are located caudally and laterally from the pulvinar. Several small nuclear groups (intralaminar nuclei) can be detected within the internal medullary lamina, as well as a larger nuclear complex (centromedian nucleus). Each thalamic body is bordered by a thin layer of Figures 1a and 1b. Adhesio interthalamica and mamillothalamic tract. Axial T2-weighted imwhite matter, the external medulages. a) The adhesio interthalamica (arrow) connects the two thalamic bodies and runs lary lamina. Its thin layer of cells through the third ventricle. Dashed arrows mark the fornices. b) The mamillothalamic tract overlies the thalamic reticular nu(arrows) and the fornices (dashed arrows) together form a characteristic square. Anterior and cleus externally [1]. posterior commissures are also shown (arrowheads and thick arrow).

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Functional Anatomy of the Thalamus All afferent pathways, except the olfactory pathway, are relayed to the last subcortical neuron in the thalamus; they then reach the cortex via the thalamocortical projection pathways. Thus, the thalamus fulfills a central function in sensory perception (gateway to consciousness). Thalamic nuclei are differentiated by their functional aspects into specific and unspecific nuclei. The nuclei that project to primary, secondary, or tertiary cortical fields are termed specific relaying nuclei. The term nonspecific nuclei means those nuclei that lack direct fiber connections to the cortex, but, for example, are connected with the brain stem, the basal ganglia, or the reticular formation.

The ventroposterior and posteromedial nuclear complexes (nucleus ventralis posterolateralis [VPL] et posteromedialis [VPM]) belong to the most important specific nuclear regions having connections to the primary cortical fields. From these nuclei the somatosensory neurons of the medial lemniscus, the spinothalamic tract, and the trigeminothalamic tract are relayed further. Other important specific thalamic nuclei are the medial (corpus geniculatum mediale, CGM) and lateral geniculate bodies (corpus geniculatum laterale, CGL), which are relay sites for the auditory and visual pathways. They project to the primary visual cortex (Brodmanns area 17, CGL), or to Heschls gyri of the temporal lobe, i.e., to the auditory cortex (Brodmanns area 41, CGM).

e b

Figures 2a to 2f. Imaging anatomy of the thalamus. Each thalamic body is divided by Y-shaped bands of white matter (internal medullary lamina, arrowheads) into three larger nuclear groups: the anterior nuclei, which lie in the fork of the Y (asterisk), the ventrolateral nuclei, situated laterally (thick arrow), and the medial nuclei, lying medially (curved arrow). The pulvinar joins the thalamic bodies on the dorsocaudal side (thin arrow). a, b) Transverse (a) and coronal (b) T1-FLAIR-weighted 3T-MRIs of an adult test person. cf) Transverse T2- (c) and T1-weighted (d) and coronal (e, f) T2-weighted images of an infant with cystic degeneration of the gray matter of the thalami. Only the medullary lamina are preserved and well circumscribed (arrowheads).

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The anterior nucleus is a component of the limbic system. It projects via the mamillothalamic tract (Vicq dAzyrs bundle of fibers, Figure 1b), among others, to the mamillary bodies, from which it also receives afferences. The pulvinar is reciprocally connected with the association areas in the parietal and occipital lobes. Vascular Supply of the Thalamus The most important blood vessels that contribute to the arterial blood supply of the thalamus are the perforating arteries originating from the posterior communicating artery (PcoA) and the posterior cerebral artery (PCA). Furthermore, the anterior choroidal artery (AchA) can also be involved in supplying the lateral thalamus; however, the question of its contribution to vascular supply is still controversial [46]. Four main vascular territories are differentiated on the basis of the most important infarct patterns: the anterolateral, the inferomedial, the inferolateral, and the posterolateral regions of supply (Figure 3a). The anterolateral territory, i.e., the anterior thalamus, is generally supplied by the anterior thalamic perforating artery (synonyms include among others tuberothalamic artery and polar artery) originating from the middle third of the branch of the posterior communicating artery (RcomP). If these perforating arteries are

absent (in ca. 30% of the normal population), the area is supplied by the posterior thalamic perforating arteries from the P1 segment of the PCA (synonym, for example, paramedian artery) [7]. These posterior thalamic perforating arteries usually supply the inferomedial thalamus. According to Percheron there are four variants of outlets of the posterior perforating arteries [8, 9]. In the type IIb variant they have a common main trunk originating from a P1 segment of one of the PCAs. This means that an embolic occlusion of the main trunk results in a bilateral paramedian thalamic infarct (see below). The inferolateral territory, i.e., the lateral or caudal part of the thalamus, is supplied by five to ten inferolateral arteries (synonym, for example, thalamogeniculate arteries) from the P2 segment of the PCA [4]. The medial and the lateral posterior choroidal arteries supply the posterolateral territory of the thalamus, i.e., the pulvinar and the geniculate bodies. The medial posterior choroidal arteries consist of one to two blood vessels originating from the distal P1 or the proximal P2 segment. The posterior lateral choroidal arteries can number up to six and originate from the distal P2 or the proximal P3 segments of the PCA [46]. The venous drainage of the thalamus takes place via the deep cerebral veins, i.e., via the thalamostriatal veins and the lateral thalamic vein, which drain via the internal cerebral veins as well as over the basal veins of Rosenthal. The internal cerebral veins and generally also the basal veins of Rosenthal drain over the vein of Galen into the straight sinus (Figure 3b). Imaging Methods Magnetic resonance imaging (MRI) is the method of choice for differential diagnostic evaluation of bithalamic lesions. The routine diagnosis relies on, above all, diffusionweighted images, proton-density(PD), and T2-weighted sequences as well native and contrast-enhanced T1-weighted images, FLAIR- (fluidattenuated inversion recovery) and T2-weighted images, and arterial and/or venous time-of-flight magnetic resonance angiography (TOFMRA). Naturally, the sequence protocol must be adapted to the pre-

Figures 3a and 3b. Arterial blood supply and venous drainage of the thalamus. a) The main arterial vascular territories of the thalamus are indicated in color: the anterolateral (green), the inferomedial (blue), the inferolateral (yellow), and the posterolateral territory (violet and red). b) The thalami drain via the thalamostriate veins (thin arrows) and the lateral thalamic veins (thick arrows) into the internal cerebral veins (arrowheads). The caudal part of the thalamus is drained by the basal veins of Rosenthal (not shown).

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Table 2. Differential diagnosis of bithalamic vascular diseases. ADC: apparent diffusion coefficient; CTA: computed tomography angiography; DSA: digital subtraction angiography; DWI: diffusion-weighted imaging; FLAIR: fluid-attenuated inversion recovery; PD/T2: proton-density-/T2-weighted; T2*GRE: T2* gradient-echo sequence; TOF-MRA: time-of-flight magnetic resonance angiography. Disease Embolic territorial infarction top of the basilar artery syndrome Imaging methods FLAIR, PD/T2 T2*-GRE DWI Arterial TOF-MRA/CTA (DSA) FLAIR, PD/T2 T2*-GRE DWI Arterial TOF-MRA FLAIR, PD/T2 T2*-GRE DWI Venous TOF-MRA Unenhanced CT CTA (DSA) Typical findings Ischemic area appears hyperintense To detect hemorrhages Marked restriction of diffusion with reduction of ADC value Might reveal thrombus at the top of the basilar artery, but often normal in cases of small embolisms Only if intraarterial recanalization therapy is planned Multiple lacunar (< 10 mm) infarctions in thalami, basal ganglia, white matter, and brain stem To detect microbleeds Acute lesions show restriction of diffusion Typically normal (microangiopathic disease) (Bi)thalamic edema common Thrombosed veins show marked hypointense signal; might reveal hemorrhages Variable findings Lack of flow signal in the thrombosed veins Thrombus might show hyperdense signal (= cord sign) Shows filling defects in the affected veins Only if invasive recanalization therapy is planned

Chronic hypertensive encephalopathy

Deep venous thrombosis

sumptive clinical diagnosis in each individual case. For special questions it may be useful to perform an auxiliary magnetic resonance spectroscopy (MRS) or a perfusion MRI. The MRI examinations shown in this overview were in part performed on a 1.5-Tesla scanner (Vision, Siemens) or on a 3-Tesla scanner (Signa, General Electrics). In some instances native computed tomography (CT) provides important supplementary information, for example, about the presence of calcification or of fatty portions and in some cases to ensure an acute hemorrhage. As an alternative or supplement to an MRA, a venous or arterial CT angiography (CTA) can be useful, especially with patients who are less cooperative. Digital subtraction angiography (DSA) should only be performed in exceptional single cases if interventional treatment is being considered. Pathologies Leading to Bithalamic Lesions Vascular Diseases Table 2 summarizes the typical imaging findings of vascular diseases leading to bithalamic lesions. Embolic Territorial Infarction As described above, the thalamus is divided into four main vascular territories. Consequently, territorial in-

farctions in these different regions lead to typical infarct patterns (Figure 3a). Bithalamic territorial infarctions are a special case: they occur in connection with an embolism at the top of the basilar artery and typically extend mesencephally (top of the basilar artery syndrome, Figure 4). The most important clinical symptoms of this syndrome are summarized in Table 3. The cause of this typical, bilateral infarct pattern is commonly a thrombosis at the top of the basilar artery or an embolic occlusion of the common main trunk of the posterior thalamic perforating arteries from the proximal P1 segment of a PCA. This means that the presence of a type IIb arterial outlet variant, according to Percheron (see above, [8, 10]), predisposes to this type of infarction. Diffusion-weighted MRI shows a marked restriction of diffusion typical of ischemia and a clear reduction of the apparent diffusion coefficient (ADC). The MRA often appears normal in cases of small embolisms that only occlude perforating arteries, but might reveal a thrombus at the top of the basilar artery. Chronic Hypertensive Encephalopathy Systemic hypertension of long duration, either untreated or treated inadequately, leads to alterations of the brain parenchyma, which are subsumed under the main

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Figures 4a to 4d. Bilateral paramedian thalamic infarct in a case of basilar thrombosis. a) Diffusion-weighted MRI shows a diffusion restriction in paramedian parts of the thalamic bodies. b) In the transverse T2-weighted sequence the acute bilateral, paramedian thalamic infarctions already appear hyperintense in the territory of the posterior thalamic perforating arteries, which originate in the P1 segment (arrows). c, d) In the coronal FLAIR-weighted images the extension of the infarcts into the midbrain is visible (arrowheads). Moreover, there is also a partial infarct of the PCA on the left with infarct of the mesial temporal lobe (asterisk).

category of chronic hypertensive encephalopathy (synonyms: subcortical arteriosclerotic encephalopathy [SAE] or Binswangers disease). Chronic hypertension causes damage to the small blood vessels supplying the brain and results in white matter lesions, lacunar infarctions, and parenchymal bleeding, and these lesions can manifest clinically as vascular dementia. Lacunar infarctions measure up to 10 mm and are caused by the occlusion of a perforating artery. Lacunar ischemias comprise 1520% of all infarctions. These lesions are etiologically due to arteriosclerotic, or thrombotic alterations and hyalinosis in the long penetrating end arteries that supply the gray matter. Lacunas are most frequently localized in the thalamus, putamen, globus pallidus, and the pons (Figure 5); they occur more seldom in the internal capsule and the caudate nucleus. On MRI they are visualized as small, well-demarcated focal lesions that are hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. Diffusion-weighted imaging (DWI) is important in order to identify acute lacunar infarction: within the first hours of a stroke restriction

Table 3. Clinical aspects of the top of the basilar artery syndrome. Coma vigile, reduced vigilance Reduced ability to learn and memory disorders, including reduced autobiographic memory Confabulation Disturbances of temporal orientation Changes in character with apathy, aggression, agitation Disorders of sleep regulation Akinetic mutism Asthenia Amnesia Disorder of executive functions Klver Bucy syndrome Vertical gaze paralysis (midbrain)

Hypersomnia Absence of spontaneous verbal expressions, with general immobility but intact eye movements Loss of psychic self activation with loss of interests, wanting initiative, spiritual emptiness

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Figures 5a to 5c. Bilateral lacunar thalamic infarct in a patient with chronic hypertensive encephalopathy. The 67-year-old patient had arterial hypertension for several years. The transverse T2-weighted images show predominantly periventricular microangiopathic lesions of the white matter, part of which are confluent (a, b, arrows); bilateral lacunar thalamic infarcts in the dorsal, inferomedial thalamus on the right and in the dorsolateral thalamus on the left (c, thick arrows); as well as a residual older hemorrhage, in the area of the left external capsule (c, thin arrow).

of diffusion is visible on diffusionweighted MRI. The lesions can show petechial bleeding (microhemorrhages), which can appear in T1-weighted MRI as extremely small hyperintense lesions (hemorrhagic microangiopathy). Often, however, their presence can be detected only on T2*-weighted gradient echo sequences as small hypointensities [11, 12]. Susceptibility-weighted imaging, a modern imaging technique that uses both magnitude and phase images from a high-resolution gradient echo sequence, might be even more sensi-

Figures 6a to 6d. Thrombosis of the internal cerebral veins. 40-year-old patient with headache of several days duration and progressive reduction of vigilance. a) The diffusionweighted MRI shows, on both sides of the thalami, right more extensively than left, a clear diffusion restriction (arrows). b, c) Bithalamic edema, hypointense in the transverse T1-weighted sequence (b) and hyperintense in the coronal FLAIR sequence (c, arrows). d) The source image of the venous two-dimensional TOF-MRA does not show a flow signal in the deep cerebral veins but normal flow in the right transverse sinus (arrow).

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tive in detecting those microbleeds than conventional T2*-weighted images [13]. The microbleeds are likewise preferentially localized in the basal ganglia and the thalamus. MRA typically yields a normal finding for hypertensive encephalopathy, as this is a microangiopathic disorder. Deep Cerebral Venous Thrombosis Since the venous drainage of the thalamus occurs via the deep cerebral veins (see above), a thrombosis of the internal cerebral veins generally leads to bithalamic edemas (Figure 6). Unenhanced CT examination depicts the edema as hypodense lesions, while it often demonstrates the thrombotic material in the deep cerebral veins as a hyperdense structure (the so-called cord sign). CTA shows filling defects in the affected veins. Venous TOF-MRA shows no flow signal in the thrombosed veins and T2*-weighted gradient echo sequence allows visualization of the thrombosed veins as

marked hypointense structures. In diffusion-weighted MRI (Figure 6a) variable and often reversible signal alterations are found in deep cerebral venous thrombosis. At first, the vasogenic edema dominates; this leads to an elevation of the ADC value [14, 15]. However, areas of reduced ADC are also observed, and they can be reversible as well [14, 15]. Susceptibility artifacts, which can be caused by a hemorrhagic transformation, can also additionally complicate the interpretation of diffusion-weighted MRI [1417]. The value of diffusionweighted MRI for imaging venous infarctions is controversial. While a study of Manzione et al. suggested that patients who would benefit from endovascular therapy [17] might be identified by the ADC (ADC value), other authors were not able to confirm this hypothesis [14, 15]. Tumors Table 4 summarizes the typical imaging findings of bithalamic tumors.

Table 4. Differential diagnosis of bithalamic tumors. DWI: diffusion-weighted imaging; FLAIR: fluid-attenuated inversion recovery; Gd: gadolinium; MRS: magnetic resonance spectroscopy; NAA: N-acetyl-aspartate; PD/T2: proton-density-/T2-weighted; PWI: perfusion-weighted imaging; T1: T1-weighted images; T1 IR: T1 inversion recovery-weighted images. Tumor Gliomas Diffuse astrocytoma (WHO II grade) Imaging methods Typical findings

Anaplastic astrocytoma (WHO III grade)

Glioblastoma multiforme (WHO IV grade)

FLAIR, PD/T2 T1 Gd MRS PD/T2 T1 Gd MRS PD/T2 T1 Gd MRS T1 IR PWI DWI T2 T1 T1 + Gd DWI CT PD/T2 T1 T1 + Gd (with fat saturation) CT

All gliomas

Volume increase of the affected thalami, homogeneous hyperintense signal No contrast enhancement Elevated creatinine peak compared to choline; elevation of NAA, elevation of choline Mixed signal intensities Contrast enhancement Elevation of choline correlates with proliferation activity Mixed signal intensities (solid areas show low signal, cystic or necrotic areas show high signal) Irregular/inhomogeneous contrast enhancement Elevation of choline Detailed visualization of the relevant anatomic midline structures Blood volume measured in the tumor correlates with malignancy To differentiate the tumor from an abscess Isointense to hyperintense compared to gray matter; cystic and/or necrotic areas are hyperintense; intratumoral hemorrhages can be present (iso- or hypointense) Isointense to hyperintense compared to gray matter Strong, homogeneous contrast enhancement Restriction of diffusion Can show calcifications Cystic areas appear hyperintense; solid areas appear isointense to gray matter Fatty areas show hyperintense signal Solid areas show contrast enhancement, fat saturation after administration of contrast agent can help to confirm the presence of fatty areas Can show calcifications and fatty areas

Dysontogenetic tumors Germinomas



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Gliomas a b Primary thalamic tumors account for only ca. 1% of all tumors of the central nervous system (CNS) [18]. The so-called intrinsic thalamic gliomas originate in the subependymal glial cells of the third ventricle and grow laterally out from the medial nuclei [19]. Notably, these tumors have the tendency to remain within the thalami, although astrocytic tumors in the hemispheres of the cerebral cortex commonly spread out along the nerve fibers in the white matter [20]. The spread of tumors to Figures 7a and 7b. Bithalamic astrocytoma, grade III, in a 14-year-old male adolescent with Recklinghausens disease. The tumor is hyperintense in the FLAIR sequence (a) and shows the opposite side commonly occurs small, focal uptake of contrast medium in the contrast-enhanced T1-weighted image (b, arvia the adhesio interthalamica (see row). Besides, a hydrocephalus is present. Figure 1a; [20, 21]). The bilateral origin of highly symmetrical spread Dysontogenetic Tumors is currently under discussion [22]. Germinomas of the CNS affect above all children and The typical clinical symptoms of bithalamic astrocytoadolescents. The age peak lies around 1012 years of mas are personality changes and mental decline, but age; 90% of patients are < 20 years of age. In up to 90% without any essential motor or sensory impairment of the cases, the tumors are localized near the midline [23]. It has been hypothesized that these symptoms of the brain around the third ventricle; in 510% of the arise due to the impairment of the dorsomedial nuclei, cases, in the thalamus or the basal ganglia [28] (Figwhich have connections to the amygdala, the temporal ure 8). As a rule, germinomas are sharply demarcated neocortex, and the prefrontal region [24]. Most cases of from the brain tissue; however, they can also grow invabilateral thalamic astrocytomas occur in children [25]. sively. Tumors in the thalamus and in the basal ganglia These patients nearly always need a shunt because of are often already very large at the time of diagnosis, compression of the third ventricle caused by the tumor since they give cause for examination only relatively (Figure 7). late. They appear isointense to hyperintense compared Both low-grade astrocytomas (WHO I and II) to gray matter in T1- and T2-weighted sequences, and and higher-grade gliomas (WHO III and glioblastocan show hyperintense cystic or necrotic areas in the mas; [23]; Figure 7) have been observed. The diffuse T2 weighted sequences. More seldom are hypointense astrocytomas of WHO II grade generally appear in regions, which are caused by hemorrhage. These tuFLAIR and PD- or T2-weighted imaging as homogemors can be calcified and show a strong, homogeneous neously hyperintense, cause a volume increase of the contrast enhancement as well as a diffusion restriction. affected thalamus, and typically show no contrast enIt is necessary to examine the entire neural axis preophancement. eratively, since germinomas tend to metastasize via the MRS can help to differentiate gliomas from other cerebrospinal fluid. tumors, such as primary CNS lymphomas [26], or from Teratomas, as tumors that contain parts of all three pyogenic brain abscesses [27] by providing metabolic primary germ layers, typically show a very inhomogeinformation. Bilateral diffuse thalamic gliomas on MRS neous signal with calcifications, fatty and cystic areas, show an elevated creatinine peak compared to choline as well as isointense soft-tissue regions showing a con[20]; however, whether this finding is characteristic of trast enhancement. The MRI examination is above all the bilateral manifestation is still controversial [21]. An helpful for the precise determination of the site of the increase in choline can itself serve as a measure of the tumor in relation to the midline structures. Fatty areproliferation activity of the tumor, since it correlates as show a hyperintense signal in T1-weighted images. with the membrane metabolism [25].

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Figures 8a to 8c. Germinoma in a 9-year-old boy. Contrast-enhanced T1-weighted images in transverse (a, b) and sagittal (c) slices. The tumor in the area of the adhesio interthalamica and the paramedian thalami on both sides shows a strong, homogeneous contrast enhancement (ac, thin arrows). Other tumor manifestations are present in the subependymal layer on the anterolateral margin of the anterior horn of the right lateral ventricle (a, thick arrow), as well as in the suprasellar and hypothalamic regions (c, thick arrow).

The use of fat saturation after administration of contrast agent can help to prove the presence of fatty areas. In an auxiliary CT the calcified and fatty parts of the tumor are especially clearly visible. Other Bithalamic Tumors Primary CNS lymphomas are above all localized in the frontal and parietal lobes; however, the thalamus and the basal ganglia are also affected in up to 17% of the cases [29]. Metastases can affect both sides of the thalamus (Figure 9); such involvement can manifest clini-

cally, for example, as severe amnesia [30]. Furthermore, still other rare bithalamic tumors have been described, e.g., the hypothalamic spongioblastoma [31] and the fibrous histiocytoma [32]. Infectious Diseases Viral Diseases The neurotrophic viruses show a particular predilection for certain brain areas: for example, herpes simplex virus for the frontotemporal cortex. Similarly, the family of flaviviruses typically affects the subcortical gray mat-

Figures 9a to 9c. Bithalamic metastasis in a 55-year-old female patient known to have carcinoma of the breast. Solitary, large metastasis, which extends over both thalami, shows central necrotic portions (ac, arrows) and a strong ring enhancement after administration of contrast agent (c). a) Transverse T2-weighted image, b) transverse unenhanced, and c) sagittal contrast-enhanced T1-weighted images.


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ter, i.e., the thalamus, basal ganglia, substantia nigra, and the cerebellum [33]. Japanese encephalitis, for example, is a severe flavivirus infection, which not only occurs in Japan, but also in the moderate and tropical zones of Asia and Northern Australia. It is transmitted by mosquito bites; a direct person-to-person transmission is not possible. In 95% of young, healthy infected adults the disease takes a completely symptom-free course or manifests with only slight headaches and low fever. These patients are cured without any sequelae. However, children < 15 years of age and older persons are overproportionally affected, and the course of their illness is severe. About 80% of these affected individuals have residual neurologic disorders, about 10% die. At first, the symptoms are those of a flue, nausea, vomiting, and diarrhea. After 24 days the patient develops high fever, neck stiffness, light sensitivity, cramps, and disturbances of consciousness that can progress to coma, and paralytic symptoms. Typically, the thalamus is bilaterally involved in Japanese encephalitis (in > 90% of the cases; [33]). Imaging of the thalamus reveals either a mixed signal or a hypointense signal in T1-weighted and a hyperintense signal in T2-weighted sequences. In addition, there is evidence of alterations in the basal ganglia, the midbrain, the pons, the cerebellum, and the cerebral cortex [33]. The West Nile virus encephalitis is also caused by a flavivirus. MRI shows typical alterations in the basal ganglia and the thalamus, more seldom in the brain stem [34]. Other flavivirus infections such as Murray Valley encephalitis and the St. Louis encephalitis result in nonspecific MRI alterations. A bilateral involvement of the thalamus has also been reported for rabies encephalitis [35]. This is also true for a number of other viral diseases, such as Epstein-Barr virus encephalitis [36] or HIV-1 encephalitis [37]. However, involvement of the thalamus is not typical for these types of encephalitis. Bacterial Diseases In principle, many bacterial infections of the CNS can lead to bithalamic lesions, but the manifestation is not considered pathognomonic for a particular disease. Bilateral thalamic lesions have, for example, been reported in rare cases of tuberculous meningoencephalitis [38]. This disease typically manifests with basal meningitis and/or focal intracerebral lesions, the tuberculomas [39], which can also be localized in the thala-

mus. Infarctions (also thalamic) can occur as a possible complication of chronic cerebral meningitis, but they are rarely focused in the bithalamic area [40, 41]. Fungi Cerebral fungal infections (e.g., histoplasmosis [42] and cryptococcosis [39]) can occur in all brain regions, including the thalamus, but they have no predilection for the diencephalon. Malaria Neurologic manifestations appear in ca. 2% of all cases of malaria [43]. Bilateral involvement of the thalamus in malaria (Plasmodium falciparum) has been described; it manifested as bilateral edema or infarctions [44]. In addition, only the cerebellum can be affected. The most frequent finding in cerebral malaria is, however, diffuse brain edema [44]. Toxoplasmosis Toxoplasmosis is a typical opportunistic infection of immunoincompetent persons, typically AIDS patients. There is evidence of multiple, roundish intracerebral lesions, which appear hypointense on T1-weighted sequences and hyperintense on T2-weighted sequences. Typically, the lesions show a ring enhancement after admission of contrast agent and a perifocal edema (Figure 10). Only single case reports are available on toxoplasmosis with lesions that did not enhance [45]. Older lesions show calcifications on CT. Although the most common localization is the basal ganglia, the thalamic bodies are also frequently affected [46]. Gonzales et al. reported, for example, on a patient with a central pain syndrome due to a thalamic abscess caused by toxoplasmosis [47]. Demyelinating Disorders Multiple Sclerosis Although multiple sclerosis (MS) is basically considered a disease of the white matter, several neuropathologic studies have demonstrated that the gray matter can also be affected in MS (Figure 11). The frequency of gray matter involvement is often underestimated. The use of magnetization transfer (MT) imaging, diffusion tensor imaging (DTI), and MRS has made more frequent findings of diffuse alterations in the gray matter of MS patients possible, also in newer MR studies. These alterations can in part be detected already at an early stage of the disease [48]. The cortex, thalamus, basal ganglia,

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and the hippocampus can be affected (Figure 11). MRS investigations showed that the neuroaxonal marker Na b

acetyl-aspartate (NAA, [49]) is reduced in the affected thalamus. Adalsteinsson et al. suggest that only the secondary-progressive form of MS shows such a lowered NAA peak in otherwise normal-appearing gray matter. This might allow an early differential diagnosis of the relapsing-progressive form, distinguishing it from the secondary progressive form [50]. Acute Demyelinating Encephalomyelitis Acute demyelinating encephalomyelitis (ADEM) is an immunologically induced, severe demyelinating disease of the brain and/or the spinal cord. It has an acute course, but, as a rule, is reversible. It shows a

Figures 10a to 10d. Toxoplasmosis with bithalamic foci. 40-year-old HIV-positive male patient with multiple intracerebral foci of toxoplasmosis. On both sides in the thalami roundish, in T2-weighted sequences (a) hyperintense lesions are visible, which clearly show contrast enhancement in the contrast-enhanced T1-weighted images (bd). In addition, there are several further lesions, in part with clear surrounding edema, for example, right occipital (a, c, arrowheads) and in the basal ganglia, left (a, c, thick arrows).

Figures 11a to 11c. Bilateral thalamic lesions in multiple sclerosis. 32-year-old male patient with history of multiple sclerosis for 8 years. In the transverse T2-weighted sequence (a) lesions are visible in the paramedian right thalamus and in the anterolateral left thalamus (arrows). The transverse (b) and sagittal (c) FLAIR images show typical demyelinating plaques in the periventricular area (arrows).


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predilection for children (age peak of 35 years). Etiologically, the illness is often preceded by a nonspecific, mostly viral infection of the upper respiratory tract or by a vaccination. To suggest a diagnosis of ADEM, the intracerebral lesions should all be in the same stage of development and no new foci should appear after the onset of the initial clinical symptoms. Although it is first of all a disease of the white matter, the gray matter can also be affected, including the thalamus [51]. Whereas the thalamus is very rarely affected in MS, it is involved in 40% of ADEM cases. Some authors therefore suggest that involvement of the thalamus can be used as a possible differential diagnostic criterion to distinguish ADEM from MS [51]. Congenital Metabolic Diseases There is a plethora of congenital metabolic diseases. To deal with them fully would go beyond the scope of this review. Therefore, we will only briefly explain a selection of these generally very rare pathologies, for which adult forms have also been described and which typically involve the thalamus. The most important findings are compiled in Table 5.

Leighs syndrome Leighs syndrome belongs to the group of mitochondriopathies. It is genetically a heterogeneous illness that is characterized by progressive neurodegeneration. In most cases the disease first becomes manifest at the age of 2 years. Although rare, occurrences later in childhood or in adulthood have been reported. MRIs show bilateral, symmetrical hyperintensities in the T2-weighted sequences, which are frequently localized in the basal ganglia (predilection for the posterior putamen), the brain stem, and the thalamus [52]. A focal involvement of the dorsomedial nuclei is typical for the thalamus. The brain stem commonly exhibits an involvement of the central pathways and of the periaqueductal gray. In T1-weighted sequences the lesions are hypointense with occasional evidence of focal hyperintense areas, which are caused by blood or myelin degradation products. In the acute phase the affected areas show a diffusion restriction. The lesions typically do not show any contrast enhancement. MRS reveals evidence of an elevated choline peak, a reduced NAA, and in many cases a lactate peak. The MELAS syndrome (myopathy, encephalopathy, lactic

Table 5. Selected congenital metabolic diseases presenting with bilateral thalamic lesions. CT: computed tomography; DWI: diffusion-weighted imaging; MRI: magnetic resonance imaging; MRS: magnetic resonance spectroscopy; NAA: N-acetyl-aspartate; T1: T1-weighted images; T2: T2weighted images. Disease Leighs syndrome Characteristics Mitochondriopathy Typically manifests at age of 2 years; seldom in later childhood or adulthood T2: bilateral symmetrical hyperintensities in the basal ganglia, brain stem, thalamus (dorsomedial nuclei) T1: hypointense lesions with focal hyperintensive areas DWI: diffusion restriction in the acute phase MRS: choline elevated, reduced NAA, and possibly a lactate peak Lysosomal disorder Infantile forms: typically involved are the thalamus, supratentoral striatum, and cerebellar white matter Native CT: bilateral symmetrical hyperdense image of the thalamus DWI: diffusion restriction in the ventral part of the thalamus Juvenile and adult forms: atrophy of the cerebellum; thalamus, striatum, and white matter typically normal Infantile, juvenile, adult forms Infantile form: T2: ventral thalamus, focally hypointense; dorsal thalamus, hyperintense Infantile, juvenile, adult forms T2: diffusely hypointense signal in the thalamus Only the infantile form


Type B (Tay-Sachs disease) Type O (Sandhoffs disease) Type AB

Krabbes disease Cerebrosidosis (globoid cell leukodystrophy) Progressive autosomal recessive leukodystrophy Infantile form: Early: in the native CT hyperdensities are seen in the thalamus and the basal ganglia; periventricular white matter appears hypodense MRI in course of disease: thalamus and basal ganglia hyperintense in T1 Late infantile-juvenile and adult forms: Leukoencephalopathy

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acidosis, stroke-like episodes), glutaraciduria type I, Wilsons disease, as well as perinatal hypoxia (see below) are the most important differential diagnoses for this syndrome. Gangliosidosis GM2 Gangliosidosis GM2 is a congenital lysosomal disorder, which is characterized by the accumulation of GM2 gangliosides in the brain. Three main types can be distinguished biochemically: type B (Tay-Sachs disease), type O (Sandhoffs disease), and type AB (GM2 variant AB). Types B and O have known infantile, juvenile, and adult forms; the GM2 variant AB exists only in an infantile form. In the infantile forms the thalamus, the striatum (the putamen more pronounced than the caput nuclei caudati), and the supratentorial and the cerebellar white matter are typically affected. The usual finding on imaging of infantile gangliosidosis is a bilateral, symmetrical hyperdense thalamus on native CT examination [53]; on MRI the signal is hypointense in the T2-weighted sequence or hyperintense in the T1-weighted sequence. In Sandhoffs disease a diffusely hypointense signal in the thalamus can be observed in the T2-weighted sequence; in Tay-Sachs disease, on the contrary, there is a focal hypointense signal in the ventral thalamus, while the dorsal part appears hyperintense. The white matter is hypointense in the T1-weighted sequence, and hyperintense in the T2-weighted sequence, as signs of hypoor demyelination. The striatum also typically appears as a hyperintense signal in the T2-weighted sequence. Diffusion MRI shows a diffusion restriction in the ventral thalamus. The lesions do not show any contrast enhancement. In contrast to the infantile types, the juvenile and adult forms are characterized by atrophy of the cerebellum. The thalamus, striatum, and white matter are here typically normal. The brain stem is seldom involved in these forms; when it is, it shows a hyperintense signal in T2-weighted sequences. Krabbes Disease Krabbes disease is a progressive, autosomal recessive hereditary, degenerative leukodystrophy, which affects the central as well as the peripheral nervous system [54]. The classic findings on imaging diagnostics at a very early stage of the disease are hyperdensities in the thalamus and basal ganglia on native cranial CT, whereas the

periventricular white matter appears hypodense. At this early stage the MRI can still show normal signal in the affected regions. In the course of the disease the thalamus and basal ganglia also show a hyperintense signal in T1-weighted MRIs, and a hypointense signal in T2weighted sequences. This lesion pattern is, however, only typical of the infantile form of the disease. The late infantile-juvenile and the adult forms are above all characterized by leukoencephalopathy. Wilsons Disease (Hepatolenticular Degeneration) Wilsons disease is a congenital, autosomal recessive hereditary disease of the copper metabolism. Accumulation of copper in the hepatocytes and extrahepatic tissue leads to formation of the so-called Kayser-Fleischer rings and degeneration of the basal ganglia. Patients can present with hepatic as well as neurologic symptoms. MR examination shows symmetrical alterations in the putamen, thalamus, caput nuclei caudate, midbrain, pons, and cerebellum [55]. These changes are mostly visible in the T2-weighted sequence as hyperintense signals or there is a mixed signal behavior. The ventral nuclear group of the thalamus and the outer part of the putamen appear to be preferentially involved [56]. This leads to a characteristic concentric-laminar hyperintensity in the putamen in the T2-weighted sequence. In the acute stage of the disease after clinical symptoms have appeared, diffusion-weighted MRI reveals a reduced diffusion with lowered ADC values in the affected basal ganglia [57]. In the midbrain the tegmentum is above all affected, but also the substantia nigra, the nucleus ruber, and the inferior tectum. Axial MRI slices through the mesencephalon typically show the face of the giant panda sign, an effect resulting from the hyperintense signal of the tegmentum (with the exception of the nucleus ruber) and the hypointense signal of the superior colliculi [55]. A shortened T1 relaxation time can often be detected in the basal ganglia and thalamus of untreated patients. This is caused by the paramagnetic effects of copper. Generally, however, basal ganglia and thalamus appear hypointense in the T1-weighted sequence. Furthermore, global brain atrophy can be observed with consecutive ventricular dilation [55]. Three subgroups have been distinguished clinically; they also differ from each other on imaging diagnostics. Especially in the subgroup that manifests clinically with ataxia and tremor, focal thalamic lesions were detected [58]. It has been shown that MR signal changes in Wilsons disease may be reversible after


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penicillamine therapy [59]. All in all, however, the MR alterations do not correlate well with the magnitude of the clinical symptoms [55, 56]. Acquired Metabolic and Degenerative Diseases Table 6 summarizes the typical imaging findings of selected acquired metabolic and degenerative diseases leading to bithalamic lesions. Creutzfeldt-Jakob Disease The Creutzfeldt-Jakob disease (CJD) belongs to the group of transmissible spongiform encephalopathies. These diseases are characterized by a rapidly progressive, fatal clinical course. Spongiform alterations, neuronal loss, astrocytosis, and the deposit of prion proteins can be detected histologically. Today it is believed that this protein (the prion) is the infectious agent [60]. Of the various subtypes of CJD, the sporadic CJD (sCJD) is the most widespread, having an incidence of ca. 1 per 1,000,000 per year. The more seldom forms include the

iatrogenic CJD (transmitted by CJD-infected surgical instruments, or tissue or hormone preparations) and the familial CJD (associated with mutations of the prion protein gene). A new form of CJD was first described in 1996, the so-called variant CJD (vCJD). It differs clinically and pathologically from forms known until then. It spreads to humans via bovine spongiform encephalopathy (BSE), a disease occurring epidemically in cows. Definitive diagnosis of a case of vCJD can only be confirmed neuropathologically by an in vivo biopsy or an autopsy. Typical MRI alterations have, however, been detected: the pulvinar sign and the hockey stick sign [60, 61]. These signs are due to bilateral increases of signal intensity in the posterior thalamus (pulvinar sign) or in the dorsomedial thalamic nuclei (hockeystick sign). Moreover, signal increases have also been described for CJD in the caput nuclei caudati, in the periaqueductal gray matter, and in the neocortex [62]. The FLAIR sequence is the most sensitive for detect-

Table 6. Selected acquired metabolic and degenerative diseases. DWI: diffusion-weighted imaging; FLAIR: fluid-attenuated inversion recovery; Gd: gadolinium; MRS: magnetic resonance spectroscopy; PD/T2: proton-density-/T2-weighted; T1: T1-weighted images; T2*-GRE: T2* gradient echo sequence. Disease Creutzfeldt-Jakob disease Imaging methods FLAIR Typical findings Pulvinar sign = corresponds to bilateral increases of signal intensity in the posterior thalamus; hockey-stick sign = corresponds to bilateral increases of signal intensity in the dorsomedial thalamic nuclei; hyperintense signal in caudate nucleus, periaqueductal gray matter, and in the neocortex Diffusion restriction in those regions Hyperintense signal in the medial part of the thalami, in the mamillary bodies, in the hypothalamus, and in the periaqueductal gray matter Hypointense signal in the affected areas Mamillary bodies show contrast enhancement Diffusion restriction Excessive bilateral calcifications of the basal ganglia, the thalami, the cerebellum, and the supratentorial white matter Pontine myelinolysis: confluent hyperintense lesion in the central pons, with spar ing of the periphery and the corticospinal pathways Extrapontine manifestations: typically affect basal ganglia and white matter; involvement of thalami (lateral geniculate bodies) less common Hyperintense signal of the affected areas Acute state: mild to moderate hypointense signal in the affected areas, isointense signal intensity less common Subacute state: lesions might be hyperintense Lesions only rarely show contrast enhancement Might show small hemorrhages Slight restriction of diffusion Restriction of diffusion within the first 24 h of life T1 shortening within 23 days T2 prolongation within the first 24 h, T2 shortening within 67 days Elevated lactate (within few hours after birth)

DWI Thiamine deficiency encephalopathy FLAIR, PD/T2 (Wernickes encephalopathy) T1 T1 + Gd DWI Fahrs disease CT Pontine und extrapontine myelinolysis (osmotic demyelinization syndrome)


Profound hypoxia of the newborn


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ing these changes. The lesions often show a restricted diffusion in DWIs. Thiamine Deficiency Encephalopathy (Wernickes Encephalopathy) Wernickes encephalopathy is a noninflammatory CNS disease caused by vitamin B1 deficiency. The thiamine deficiency can occur together with a general nutritional deficiency, but it is above all observed in cases of chronic alcoholism with resorption disorders. The classic triad
a b

of symptoms includes oculomotor disorders, gait ataxia, and a psychoorganic syndrome. In Wernickes encephalopathy histopathologic alterations occur in the medial part of the thalamus and in the mamillary bodies (capillary and glial proliferation as well as demyelination), periaqueductally in the pons, the medulla oblongata, the basal ganglia, and even in the cortical gray matter [63]. The resulting capillary proliferation in this form of encephalopathy frequently leads to hemorrhages. Signal increases are seen on MRI in the T2- and FLAIR-weighted sequences in the medial part of the thalamus (adjacent to the third ventricle, Figure 12), in the mamillary bodies, the hypothalamus, and in the midbrain (periaqueductal gray matter). The affected areas appear hypointense in T1-weighted images. When contrast medium is administered, the mamillary bodies typically show a contrast enhancement (Figure 12d). Contrast enhancement can also be seen in the medial portion of the thalamus and the periaqueductal gray matter. The diffusion MRI reveals a diffusion restriction in these areas [63]. On the 18F-FDG-PET (fluorodeoxyglucose positron emission tomography) hypometabolism is observed in the diencephalic gray matter, the medial temporal lobes, the limbic system and also in retrosplenial areas [64]. Fahrs Disease (Cerebrovascular Ferrocalcinosis) Fahrs disease is a rare, degenerative neurologic disease characterized by excessive bilateral calcifications of the basal ganglia. Clinically progressive dystonia, parkinsonism, and neuropsychological alterations are observed. The lateral portion of the globus pallidus is commonly affected. In addition, calcifications often involve the thalamus, putamen, nucleus caudatus, cerebellum (above all the dentate nucleus) and

Figures 12a to 12d. Thiamine deficiency encephalopathy. 24-year-old male patient with a central oculomotor disorder, severe psychoorganic syndrome with vigilance reduction, and mnestic disorders. After irradiation and chemotherapy for a Schminke tumor, the patient had started vomiting without interruption. In the transverse (a) and coronal (b) T2-weighted images there are bilateral symmetrical signal increases in the paramedian thalami (arrows). The diffusionweighted MRI shows a diffusion restriction in the affected areas (c, arrow). After administration of contrast medium the mamillary bodies show uptake of contrast medium (d, arrow, contrast-enhanced T1-weighted images in transverse slice).


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Figures 13a to 13c. Fahrs disease. The symmetrical calcifications in the caudate nucleus (a, arrows), globus pallidus (b, thin arrows), dorsolateral thalamus (b, thick arrows), and the cerebellar hemispheres (c, arrows) are readily identifiable on the native CT.

the supratentorial deep white matter, including the internal capsule (Figure 13). The diagnostic method of choice for Fahrs disease is CT. In T1- as well as T2-weighted MRI sequences the calcifications show various signal intensities (hyper- or hypointense), depending on the amount of calcifications [65]. Furthermore, also noncalcified areas of the white matter can show a hyperintense signal in T1 and T2 weighted sequences. These alterations are probably caused by underlying and slowly progressive metabolic or inflammatory processes, which later lead to calcifications. The most important differential diagnosis of Fahrs disease is the symmetrical calcification of the basal ganglia, which is a normal finding and is preferentially observed in the anterior part of the globus pallidus. Other rare differential diagnoses must also consider calcifications of endocrinological or congenital diseases. These more readily affect only the basal ganglia, and less often the thalamus. Pontine and Extrapontine Myelinolysis (Osmotic Demyelination Syndrome) Pontine and extrapontine myelinolysis is an acute-occurring demyelination that is caused by rapid shifts in the serum osmolality. The disease is typically triggered by a too rapid balancing of a hyponatremia. In about half of the cases, myelinolysis affects the pons; in the other half, there are extrapontine manifestations, which typically affect the basal ganglia and the white matter, less

frequently the cortex and the hippocampus. An involvement of the thalamus has been described in rare cases, in the course of which the lateral geniculate body is affected [66]. These affected areas usually appear in the acute stage to be slightly too moderately hypointense in T1-weighted sequences, more seldom as isointense, and in T2-weighted images as hyperintense. In subacute stages, i.e., after 14 months, they can be detected in the T1-weighted sequence as hyperintense alterations that correspond to coagulation necroses. In many cases T2-weighted sequences show small bleedings. Diffusion MRI reveals a slight diffusion restriction [67], contrast enhancement is possible but rare. The typical imaging findings for pontine myelinolysis are confluent hyperintense alterations in the central pons, while the periphery and corticospinal pathways are spared. Profound Hypoxia of the Newborn Hypoxic injury of the lateral thalami, the posterior putamina, hippocampi, and corticospinal tracts is typically seen in neonates who have suffered from profound hypotension or cardiac arrest. Standard MRI performed immediately at the day of birth can be normal, wheras MRS shows elevation of lactate within a few hours after birth [68], and DWI typically shows a restriction of diffusion within the first 24 h after birth, but may initially yield false-negative results [66]. T1- and T2-weighted images show subtle pathologic signal intensities with T2 prolongation within the first 24 h, and T1 as well as

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Figures 14a to 14d. Profound neonatal hypoxic injury. a, b) MRI of a term neonate, performed on day 1 after birth. At the day of birth, the mother did no longer feel any intrauterine movements, and the child had to be resuscitated immediately after birth. a) T2-weighted image, b) T1-weighted image. The basal ganglia, the posterior limb of the internal capsule, and the adjacent parts of the thalami show pathologic signal intensities (while the posterior limb of the internal capsule lacks its normal T2-weighted hypointensity and T1weighted hyperintensity, the basal ganglia and the posterolateral nucleus of the thalami show pathologic T2-weighted hypointense, and T1-weighted hyperintense signals; the changes in the thalamus are at that time quite subtle, but, proven by the follow-up examination, nevertheless pathologic). c, d) Follow-up MRI performed 2 weeks later demonstrates a beginning cystic degeneration of the basal ganglia, and a diffuse swelling of the white matter with development of a severe cystic encephalomalacia. (MRIs by courtesy of D. Prayer, MD, Vienna, Austria.)

T2 shortening within 23 and 67 days after birth, respectively (Figure 14; [69]). The prognosis of these infants with hypoxic-ischemic thalamic lesions is typically poor. Conclusion This study gives an overview of the topographic and functional anatomy of the thalamus as well as its blood supply. It also provides a survey of the imaging results for the most important bithalamic diseases. Knowledge of the typical differential diagnoses of these bilateral thalamic lesions is essential in order to recognize emergency situations like, for example, deep cerebral venous thrombosis. Since the deep gray matter is a great challenge to stereotactic neurosurgeons, imaging diagnostic tools must also contribute as much as possible to the noninvasive diagnosis of inflammatory and tumor diseases. To achieve this goal, a multimodal

procedure, in which MRI plays an essential role, is often indispensable.

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Address for Correspondence Jennifer Linn, MD Department of Neuroradiology University Hospital Munich Grohadern Marchioninistrae 15 81377 Mnchen Germany Phone (+49/89) 7095-2501, Fax -2509 e-mail:


Clin Neuroradiol 2007 No. 1 Urban & Vogel