performed 20—25mm after intravenous injection of [‘23IJIMP.Radionuclide injection was performed in a quiet, dimly lit roomwith eyes open. A Siemens Orbiter SPECT gamma camera wasused with a general-purpose, parallel-hole collimator interfacedto a MDS A3 computer system. Sixty-four views were obtainedat 5.6°angular increments with an acquisition time of 30 sec perview, resulting in a total acquisition time of approximately 32mm. A Gaussian pre-filter was applied to the raw data prior toreconstruction. Transaxial images were reconstructed at one pixel

perslice usingan attenuation correctionand a Ramp filter(cutoff= 1.0). Coronal and sagittal slices were then reconstructed from

this transaxial data at two pixels per slice. Two pixel transaxialslices were photographed for hardcopy along with the sagittal andcoronal slices.

In view of the progressionand instability of neurologic symptoms and radiographic and scintigraphic findings, a right externalcarotid to right middle cerebral artery bypass operation wasperformed 1mo followingheradmission. Exceptfor mild residualhemiparesis, the patient's symptoms subsided. A follow-up ang

iogram and a brain flow study using @mTc@HMPAOwere ohtamed (Figs. 3, 4, 6). Technical details used for the [‘231]IMPimages were duplicated for the @mTc@HMPAOimages. Thepatient's clinical neurologic status has remained stable and nonew symptoms or neurologic deficits have developed in thesucceeding3 yr.

DISCUSSION

Moyamoya disease is a rare form of cerebrovasculardisease (CVD) involving the distal internal carotid arteryand/or the circle of Willis (1). Pathological findings inmoyamoya disease include fibrous or fibro-cellular intimalthickening at major cranial arteries (6). Progressivenarrowing and occlusion ofthe main cerebral arteries withdevelopment of parenchymal, leptomeningeal and transdural collaterals is demonstrated on angiography. Bilateralinvolvement has been suggested as a definite criterion fordiagnosis of the disease (2) but evolution from unilateralto bilateral disease has been postulated in some cases (3).Pseudoangiomatous collateral network at the base of thebrain, characterized as “moyamoya―(“puffof smoke―)vessels on angiography, is considered to result from avariety of vaso-occiusive conditions including diseasessuch as neurofibromatosis and sickle cell disease (4). How

BrainSPECT changesin a patientwith cerebralvaso-occlusive disease caused by moyamoya disease are presented.Brain SPECT after a revascularization procedure demonstratedsignificantimprovementof brainflow whichwas confirmed by angiography. Clinical and magnetic resonance featuresare reviewed.

J NucI Med 1992; 33:1692—1695

oyamoya disease is a vaso-occlusive disorder involving the large vessels of the brain for which no definitetherapy has been described. Diagnosis and follow-up ofmoyamoya patients is done with angiograms which demonstrate the characteristic appearance and dynamic natureof the disease. External carotid-to-internal carotid artery(EC-IC) anastomoses have been utilized in various vasoocclusive disorders of the brain with varying degrees ofsuccess. This paper describes the scintigraphic and radiographic patterns of a successful EC-IC anastomosis in apatient with moyamoya disease.

CASE REPORT

Moyamoya disease was first diagnosed following a right hemispheric cerebrovascular accident when our female patient was18 yr old. At the age of 21, she presented with progressivenumbness and weakness of the left arm and left lower extremity.The patient partially recovered and over the next 3 yr she didwell except for intermittent episodes ofmild weakness and numbness involving the left upper and lower extremities. At the age of24, the patient developed a more severe episode of weakness inthe left arm and left leg which prompted her admission forevaluation. A magnetic resonance (MR) image of the head, an[‘23111MPbrain study and cerebralangiogramwere all obtainedwithin two days (Figs. 1—5).

Brain single-photon computerized tomography (SPECT) was

ReCeivedMar.6, 1992;revisionacceptedApr.21, 1992.For rejxints contact: Mariano Femandez-lJloa, MD, lhiiversity of Cincinnati

MediCalCenter, Radioisotope L.aboratory, Mail Location #577, Cincinnati, OH45267.

1692 The Journalof NuclearMedicine•Vol. 33 •No. 9 •September1992

SPECT, Magnetic Resonance and AngiographicFeatures in a Moyamoya Patient Before and AfterExternal-to-Internal Carotid Artery BypassKenjirou Ohashi, Mariano Fernandez-Ulloa, and Larry C. Hall

Department ofRadiology, Radioisotope Laboratory, University ofCincinnati Medical Center, Cincinnati, Ohio

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FIGURE3. Transaxialpre-andpost-EC-lCbypassimagesshowinitialmarkeddecreaseduptakeintherightcerebralcortexwithsubsequentimprovement.

brain barrier and they concentrate rapidly in neuronsproportionally to regional blood flow. Generally, retentionin the brain parenchyma is stable for up to 24 hr with [1231]IMP (13) and for about 8 hr with 99mTc@HMPAO(14),which makes both suitable for SPECT. Brain SPECT with[‘231]!MPor [99mTc}HMPAOhas been suggested as a usefulstudy for evaluation of regional blood flow (12) and indiagnosing and managing patients with CVD, seizure disorder, dementia, brain tumor, trauma and psychiatricdisorders (15,16—18).Due to the wide availability of 99mTcand its superior counting statistics, 99mTc@HMPAO seemsto be more useful than [‘231]IMPfor clinical use.

Moyamoya disease is one ofthe entities in which a brainSPECT study plays an important role by showing abnorma! blood flow (19). Areas of hypoperfusion resulting

FIGURE 4. Lateralviewofa rightcommoncarotidangiogram.There is occlusion of the carotid terminus just distal to theposteriorcommunicatingartery (arrow).Extensiveperforatingcollateralsarenoted(arrowheads)demonstratingthe typical“puffof smoke―appearanceof moyamoyadisease.

FIGURE 1. CoronalT2-weightedimages.(A) Image at thefrontal horns level demonstratesabsenceof the normalcarotidterminus flow void (arrow)and sylvian branches of the right middlecerebralartery(arrow-head).Dilatedmoyamoyacollateralvesselsare representedby the heterogeneouslow signalwithin the rightcoronaradiata(curvedarrow). (B) Imageat the atna of the lateralventriclesdemonstratesa parasagittalright frontal infarct (shortarrow)and focalasymmetricatrophylaterallyrepresentingeffectsof chronic ischemia(openarrowheads).

ever, most cases are idiopathic, as initially reported inJapanese children and young adults (5).

A wide range of presenting symptoms have been described that correlate with age (7). In patients under 21 yrold, transit ischemic events are the most common manifestation. In patients over 2 1 yr, intracranial hemorrhage,mostly subarachnoid hemorrhage, is common. Computedtomography (CT) and/or MR imaging are the initial diagnostic studies in practice and can confidently establishthe diagnosis ofmoyamoya (8—10).However, angiographyis usually performed to confirm the diagnosis and to plansurgical treatment (1,11).

Lipophylic radiopharmaceuticals, such as [‘231]IMPand99mTc..HMPAO,are capable oftraversing the intact blood

FIGURE 2. Axial protondensityimages.(A) Imagesat thebasalganglialevelshowheterogeneoushighsignalin the rightexternalcapsulecausedbymoyamoyacollateralswithsurrounding gliosis (curved arrow). There is high signal in right sylvianbranthesduetoslowflow(arrowheads).(B)Verteximageshowsa right parasagittalhigh signal zone caused by infarct, bridgingthe pre and post centralgyri (arrow).

SPECT, MagneticResonanceand Angiographyin MoyamoyaDisease•Ohashiet al 1693

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in our patient resulted in improved flow through surgicalanastomoses, reduced flow through “moyamoya―collateral vessels and increased perfusion through the rightmiddle cerebral artery branches, as shown on angiography(Fig. 6). This corresponded to the SPECT images whichshowed markedly improved perfusion in the right cerebralhemisphere after surgical intervention.

The natural history of moyamoya disease is variable.Some patients remain in a stable condition on medicaltherapy for an extended period of time (23). Medicaltreatment includes vasodilators, anticonvulsants, anticoagulants and steroids. As for surgical treatments, in addition to superficial temporal-middle cerebral artery bypasssurgery, several indirect revascularization procedures havebeen developed (24). Encouraging results with neurological and angiographic improvements have been reported,particularly with encephalo-duro-arteriosynangiosis, inwhich development of spontaneous anastomoses is achieved by adhering the donor scalp arteries to the duralsurface (25).

Moyamoya disease is an entity affecting the youngpopulation, in whom early bypass interventions may prevent persistent brain damage secondary to infarctions. Abrain SPECT study with lipophylic agents is a promisingtool to establish noninvasively the status ofbrain flow andto monitor patients with moyamoya before and after intervention. Long-term follow-up with neurological monitoring is necessary for a better understanding of the roleof brain SPECT imaging in revascularization proceduresofthe brain.

REFERENCES

1. Hilal 5K. Arterial occlusive disease in infants and children. In: NewtonTH, Potts DO, ed. Radiologyofthe skulland brain. Angiography,volume2, book 4. St. Louis: C. V. Mosby Co.; 1974:2286—2309.

2. Matsushima T, Fukui M, Fujii K, et al. Two pediatric cases with occlusionsofthe ipsilateral internal carotid and posterior cerebral arteries associatedwith moyamoya vessels: “unilateral―moyamoya disease. Surg Neurol1990;33:276—280.

3. MatsushimaT, Take 5, Fujii K, et al. A case of moyamoyadiseasewithprogressive involvement from unilateral to bilateral. Surg Neuroll988;30:471—475.

4. Roach ES, Riela AR. Assorted systemic conditions. In: Pediatric cerebrovasculardisorders.Mount Kisco, NY: Futura PublishingCo.; 1988:115—134.

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6. Yamashita M, Oka K, Tanaka K. Cervico-cephalic arterial thrombi andthromboemboli in moyamoya disease—possible correlation with progressive intimal thickening in the intracranial major arteries. Stroke1984;15:264—270.

7. NishimotoA, TakeuchiS.Abnormalcerebrovascularnetworkrelatedtotheinternalcarotidarteries.JNeurosurgl968;29:255—260.

8. TakahashiM, MiyauchiT, KowadaM. Computedtomographyof moyamoya disease: demonstration of occluded arteries and collateral vesselsas important diagnostic signs. Radiology 1980;l34:67l—676.

9. Fujisawa I, Asato R, Nishimura K, et al. Moyamoya disease:MR imaging.Radiology1987;l64:103—105.

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FIGURE5. Coronalpre-andpost-EC-lCbypassimagesalsoshow improvementof right cortical uptake after surgery. Noteresidualfocal defect (arrow)causedby infarctedcortex andcorrespondingto thefindingson MR images.

from CVD tend to be larger on brain SPECT than lowdensity areas on CT and may correlate better with clinicalsymptoms (19—21).

Frontal lobe hypoperfusion and posterotemporal andoccipital lobe hyperemia have been described using ‘33Xeinhalation methods in patients with moyamoya disease(22,23). A similar pattern has been reported with @mTc@HMPAO (19). Accordingly, involvement ofmajor anteriorcirculations and relative preservation of posterior circulations are the contributing factors for this pattern. Presurgical brain SPECT images in this case showed extensiveareas of decreased perfusion in the right cerebral hemisphere disproportionate to the MR findings and clinicalpresentation. Disproportionate abnormal brain SPECTstudies are known to occur in CVD (15). Bypass surgery

FIGURE6. Lateralviewofa rightcommoncarotidangiogramfollowingEC-IC bypass.When comparedto the preoperativeangiogram,there is earlierand morecompletefillingof rightmiddle cerebral artery branches (arrows). There is also lessopacification of the moyamoya parenchymalcollaterals whencomparedto the preoperativeangiogram.

1694 The Journalof NuclearMedicine•Vol. 33 •No. 9 •September1992

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(continuedfrompage1684)I

SELF-STUDYTEST I@ Gastrointestinal NuclearMedicineANSWERSChoy,

etal. havereportedan increasein specificityfor acutecholecystitis 4. WeissmannHS,SugarmanLA.BadiaJD,FreemanLM.Improvingthespecifici(from83% to 100%),compared to the use of delayed imaging without ty andaccuracyofTc-99m-IDAcholecystigraphywithdelayedviews.J Nucia lossof sensitivity. Med1980;21:P17.Occasionally,

it isdifficultto distinguishpooled activitywithinthe proximal duodenum from the gallbladder itself, or one may mistake pooled ITEMS 21—24: Factors Affecting 99mTc IDA Uptakeactivitywithinthe duodenumforthegallbladder.Intheseinstances,it ANSWERS:21,1@22,@ 23, F;24,Fisusefultoadminister200—300mlofwaterbymouth.Thiswillfacilitate Sincethe developmentof the originalIDAderivatives,work on theflushingofactivityfromtheduodenum,buthasnoeffectonthegallblad- molecularstructureof thesecompoundshasproceededin thedirecder.Sincethe duodenum isclearly identifiedin this patient,and activity tion of producing an agent with ideal biokinetics.The @mTcIDAagentswithinit isseento changeovertime,it shouldnotbe mistakenforthe arecarriedinbloodnonspecificallyboundtoplasmaproteins,particularlyga1lt@adderHenceimagingafteradministeringwatertothispatientwould albumin.Thelipophilicityofthecompoundisdirectlyrelatedtothelevelnot provide further useful information. of proteinbinding. Substitutionsof nonpolar groups on the phenyl ringRf•r.nc•s ofthemoleculemakeitmorelipophilic.Proteinbindingpreventsrenal1. Al-Sheikh W, Serafini A, Barkin J, Spoliansky G, Hourani M The role of excretion and promotes hepatic uptake. This is an important considera

hepatobiliaryscintigraphyin differentiatingacutecholecystitisfrom acute non- tion in the jaundiced patient where these agents compete for proteinbiliary pancreatitis.Am J Gastroenterol 1983;78:502-506. binding sites with bilirubin.

2. Bakar RJ, Marion MA. Biliaryscanning with Tc-99m-pyridoxylidene glutamate—Rf@ncestheeffect of food in normal subjects. [Concise Communication].J Nuci Med 1. LobergMD,NunnAD,PorterDW.Developmentofhepatobiliaryimagingagents.1977;18:793-795.

In:FreemanLM,WeissmannHS,eds.NuclearMedicineAnnual1981.New3.ChoyD,ShiEC,McLeanRG,HoschA,MurrayPC,HamJM.Cholescintigraphy York:Raven Press;1981:1-33.in

acute cholecystitis: use of intravenous morphine. Radiology 2. NicholsonRW,Herman KJ.ShieldsRA,TestaHJ.The plasma proteinbinding1984;151:204-207.of HIDA.EurJ NuciMed 1980;5:311-312.

12. Ell PH, Jarritt PH, Costa DC, et al. Functional imaging ofthe brain. SeminNuclMed 1987;17:214—229.

13. KuhI DE, Barrio JR. Huang SC, et al. Quantifying local cerebral bloodflowby N-isopropyl-p-I-l23-iodoamphetainine(IMP)tomography.JNuclMed 198223:196—203.

14. Sharp PF, Smith FW, Gemmell HG, et al. Technetium-99mHM-PAOstereoisomers as potential agents of imaging regional cerebral blood flow:human volunteer studies. JNuclMed l98627:l71—177.

15. LeeRGL,Hill IC, HolmanBL,etal. Predictivevalueofperfusiondefectsize using N-isopropyl-(I-l23)-p-iodoamphetamine emission tomographyin acute stroke. JNeurosurg 1984;61:449—452.

16. von Schulthess OK, Ketz E, Schubiger PA, et al. Regional quantitativenoninvasive assessment of cerebral perfusion and function with N-isopropyl-(I-123)..p-iodoamphetamine.JNuclMed 1985,26:9—16.

17.GemmellHG, SharpPF, SeasonJAO, et al. DifferentialdiagnosisindementiausingthecerebralbloodflowagentTc-99mHM-PAO:a SPECFstudy.J CompusAssist Tomogr1987;l1:398—402.

18.vanHeertumRL, O'ConnellRA. Functionalbrainimagingin theevaluation of psychiatric illness. Semin NuclMed 199l21:24—39.

19. Mountz JM, Foster NL, Ackermann RI, et al. SPECF imagingof moyamoya disease using Tc-99m-HM-PAO: comparison with computed tomographyfindings.JComputAssist Tomogr1988;12:247-250.

20. UedaT, KinoshitaK, WatanabeK, etal.Earlyanddelayedsinglephotonemission CT in various cerebral diseases using N-isopropyl-p-(I-123)-iodoamphetamine.Neuroradiology1988;30:123—131.

21. UedaT, KinoshitaK, WatanabeK, of al. Local cerebralblood flowmeasurement using 1-123 IMP SPECf in patients with cerebrovasculardiseases.NeurolMedChir(Tokyo)1987;27:4l5—421.

22. Takeuchi S, Tanaka R, Ishii R, et al. Cerebral hemodynamics in patientswith moyamoya disease. A study of regional cerebral blood flow by the‘“Xeinhalationmethod.SurgNeuroll98523:468-474.

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Note:Forfurtherin-depthinformation,pleaserefertothe syllabuspagesincludedatthe beginningofNuclearMedicineSelf-StudyProgram I: Part I.

SPECT, Magnetic Resonance and Anglography in Moyamoya Disease •Ohashi et al 1695

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1992;33:1692-1695.J Nucl Med.   Kenjirou Ohashi, Mariano Fernandez-Ulloa and Larry C. Hall  and After External-to-Internal Carotid Artery BypassSPECT, Magnetic Resonance and Angiographic Features in a Moyamoya Patient Before

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