(Cr), and theclinical usefulness ofrCBF studies byPETor SPECT in patients with moyamoya disease has also been reported (8,9). Acetazolamide is a vasodilative agent which causes in creases of regional blood flow in normal tissue and in ischemic regions supplied by a stenotic artery which reacts to acetazolamide. However, this agent cannot produce ef fects in an ischemic region where an artery does not react with acetazolamide. Therefore, these regions appear as relatively low perfusion areas compared to areas of normal tissue and ischemic regions which react to acetazolamide (10—17). The acetazolamide test is a useful method for detecting affected regions because of better discriminative images between normal and ischemic lesions. Its useful ness has been reported in patients with cerebroocclusive disease (12—15); however, clinical application of IMP SPECF with the acetazolamide test in moyamoya disease has not yet been reported. We describe the usefulness of brain SPECT in patients with moyamoya disease and dis cuss the clinical usefulness of the acetazolamide test for detecting the reactivity of cerebral blood vessels. MATERIALS AND MEIHODS @n S&@ Data were acquired for 9 normal controls and 16 patients (7 malesand9 females)who hadmoyamoyadiseaseandwhopre sented with ischemic symptoms. The patients' mean ages were 16.7 yr ±11.2yr (mean ±s.d.). Moyamoya disease was diag nosed by cerebral angiography in all patients as proposed by Suzuki et al. (7). IMP SPECF was also performed on normal controls in three different age populations. They had both CF examinations andIMPSPECFbecauseof abnormal EEGSafter head traumaor clinical symptoms such as epilepsy, where IMP SPECF and CF showed normalfindings. IMP SPECTProcedures A ring-typeemissioncomputedtomograph(SET-031,Shi madzuCo.,Kyoto,Japan)andaminicomputer system(ECUPSE S-120, Nipon Datageneral Co., Tokyo, Japan) were used. The cameraconsistedofa gantryassemblywith64scanningdetectors. This system had three ringsand simultaneouslyacquired 12par allelsliceswitha center-to-center interslicedistanceof L7cm.A high-resolutioncollimatorwas used.Iodine-123-IMP(111MBq) wasinjectedintravenouslyandSPECFimageswereobtained20 mm postinjection. In each slice, 600 kcts were acquired. Acqui sition times were 15—20 mis. The raw datawere reconstructedby WestudiedtheusefulnessofIMPSPECTw@iacetazolamide in 16patientswithmoyamoyadisease.Cerebral angiographywas performed for all patents who were classified in three grades accordingto theirangiOgraphIC stages.Methods:Techniques usedindudedring-typeemissioncomputed tomographywitha minicomputer system.Patientsrecehted111 MBqof 1@l-lMP andSPECTimageswereobtained20 mmposbnjectkn.Nine pa@ents were studiedu@ngiodoamphetan*ie(IMP)SPECT wffli and wfthoutacetazc@amide. IMP SPECTw@i acetazo lamidewas performed20 mmaftereach injecdonof 1 g of acetazolamide. Results:Lowperfusionareasintheupperand lowerfrontal,parietalandtemporalregionsingrades2 and3 ualngIMPSPECTwereobserved. The meancerebral-to-care belieractivityratios(C/Crabo)of sixregions(upperandlower frontal,temporal,parietaloccipitalandbasalganglia)ingrades 1,2 and3 were0.96to 1.06,0.91to 0.96and0.76to 0.88, respecthtaly. Conclusion: Our resuftsind@ate that measure marl of reg@nal cerebralt@ood flow(rCBF)eluddatescerebral hemodynamicfactors, indudingthereactivityofcerebral vessels wh@hcannotbedetectedangiographically inpatientswithmoy amoya disease, and that the acetazolamide test is useful for detecbngcerebralbkod flowreserve.Thetestcanbeusedto detect disease progress prospectively. Key Words: bc bralbloodflow J Nuci Med1994;35:44-50 moyamoyadIsease;cere PECT imaging of the brain with N-isopropyl-p-['@IJ iodoamphetamine (IMP) reflects regional cerebral blood flow (rCBF) and has become a routine diagnostic proce dure for cerebrovascular disease, brain tumors, dementia and other diseases of the brain. Its clinical usefulness in these etiologies has been reported elsewhere (1—6). Moyamoya disease is a cerebrovasculardisorder involv ing the distal portion of the internal carotid artery with basal telangiectasias comprising dilated collateral lenticu lostriate and thalamoperforating arteries (7). The abnormal findings of rCBF images are usually larger than regions of low density lesions observed in computed tomography Rece@Mar. 29,1993; re@sion acx@eptsd Se@27,1993. For correspondence and reprints contsot H@o@dHoshi, MD,Dept of R@1bl ogy, Mlyazald Medical College, Mtyazakl, 88916, Japan. 44 TheJournalof NuclearMedicine• Vol.35 • No. 1 • January1994 Cerebral Blood Flow Study in Patients with Moyamoya Disease Evaluated by IMP SPECT Hiroaki Hoshi, Takashi OhniShi, Seishi Jinnouchi, Shigemi Futami, Shigeki Nagamachi, Takao Kodama, Katsushi Watanabe, TakaShi Ueda and Shinichiro Wakisaka Department ofRadiolo@ and Dej@imiwiu ofNeuroswge@y, M@yaZakiMedical College, Miyazaki, Japan by on March 29, 2018. For personal use only. jnm.snmjournals.org Downloaded from
Transcript
(Cr),andtheclinicalusefulnessofrCBFstudiesbyPETorSPECT in patients with moyamoya disease has also beenreported (8,9).
Acetazolamide is a vasodilative agent which causes increases of regional blood flow in normal tissue and inischemic regions supplied by a stenotic artery which reactsto acetazolamide. However, this agent cannot produce effects in an ischemic region where an artery does not reactwith acetazolamide. Therefore, these regions appear asrelatively low perfusionareas comparedto areas of normaltissue and ischemic regions which react to acetazolamide(10—17).The acetazolamide test is a useful method fordetecting affected regions because of better discriminativeimages between normal and ischemic lesions. Its usefulness has been reported in patients with cerebroocclusivedisease (12—15);however, clinical application of IMPSPECF with the acetazolamide test in moyamoya diseasehas not yet been reported. We describe the usefulness ofbrain SPECT in patients with moyamoya disease and discuss the clinical usefulness of the acetazolamide test fordetecting the reactivity of cerebral blood vessels.
MATERIALSAND MEIHODS
@nS&@Data were acquired for 9 normal controls and 16 patients (7
malesand9 females)who hadmoyamoyadiseaseandwho presented with ischemic symptoms. The patients' mean ages were16.7 yr ±11.2 yr (mean ±s.d.). Moyamoya disease was diagnosed by cerebral angiography in all patients as proposed bySuzuki et al. (7). IMP SPECF was also performed on normalcontrols in three different age populations. They had both CF
examinationsandIMPSPECFbecauseof abnormalEEGSafterhead traumaor clinical symptoms such as epilepsy, where IMPSPECF and CF showednormalfindings.
madzuCo.,Kyoto,Japan)andaminicomputersystem(ECUPSES-120, Nipon Datageneral Co., Tokyo, Japan) were used. Thecameraconsistedofa gantryassemblywith64scanningdetectors.This system had three ringsand simultaneouslyacquired 12parallelsliceswitha center-to-centerinterslicedistanceof L7 cm. Ahigh-resolutioncollimatorwas used. Iodine-123-IMP(111MBq)was injectedintravenouslyandSPECFimageswereobtained20mm postinjection. In each slice, 600 kcts were acquired. Acquisition times were 15—20mis. The raw datawere reconstructedby
Westudiedtheusefulnessof IMPSPECTw@iacetazolamidein16patientswithmoyamoyadisease.Cerebralangiographywasperformedfor all patents who were classified in three gradesaccordingto theirangiOgraphICstages.Methods:Techniquesusedindudedring-typeemissioncomputedtomographywithaminicomputersystem.Patientsrecehted111 MBqof 1@l-lMPand SPECTimageswereobtained20 mmposbnjectkn.Ninepa@entswere studiedu@ngiodoamphetan*ie(IMP)SPECTwffli and [email protected] SPECTw@iacetazolamidewas performed20 mm after each injecdonof 1 g ofacetazolamide.Results:Lowperfusionareasin the upperandlowerfrontal,parietalandtemporalregionsin grades2 and3ualngIMPSPECTwereobserved.Themeancerebral-to-carebelieractivityratios(C/Crabo)of six regions(upperand lowerfrontal,temporal,parietaloccipitalandbasalganglia)in grades1,2 and3 were0.96to 1.06,0.91to 0.96and0.76to 0.88,respecthtaly.Conclusion: Our resuftsind@atethat measuremarl of reg@nalcerebralt@oodflow (rCBF)eluddatescerebralhemodynamicfactors,indudingthereactivityofcerebralvesselswh@hcannotbedetectedangiographicallyinpatientswithmoyamoya disease, and that the acetazolamide test is useful fordetecbngcerebralbkod flow reserve.Thetestcanbe usedtodetect disease progress prospectively.
Key Words: bcbralbloodflow
J Nuci Med1994;35:44-50
moyamoyadIsease;cere
PECT imaging of the brain with N-isopropyl-p-['@IJiodoamphetamine (IMP) reflects regional cerebral bloodflow (rCBF) and has become a routine diagnostic procedure for cerebrovascular disease, brain tumors, dementiaand other diseases of the brain. Its clinical usefulness inthese etiologies has been reported elsewhere (1—6).
Moyamoya disease is a cerebrovasculardisorderinvolving the distal portion of the internal carotid artery withbasal telangiectasias comprising dilated collateral lenticulostriate and thalamoperforating arteries (7). The abnormalfindings of rCBF images are usually larger than regions oflow density lesions observed in computed tomography
[email protected],1993;re@sionacx@eptsdSe@27,1993.For correspondence and reprints contsot H@o@dHoshi, MD, Dept of R@1bl
ogy, Mlyazald Medical College, Mtyazakl, 88916, Japan.
filteredbackprojectionusing a 64 x 64 matrixwith a Ramachandran-Butterworthfilterwith an orderof 8 and a cutoff frequencyof 28mm.Thefullwidthat halfmaximwas 15mmin thecenterof the gantry by phantom study.
DataAnalysisDistiibufion ofLow rCBFAreas. Thirty-two hemispheres of 16
patientswere classified in three grades accordingto angiographicstagesas reportedby Suzukiet al. (7). Accordingto theirreports,theangiographicfindingsof eachstageareas follows:
Stage 1. Narrowingof the carotid fork.Stage 2. Dilatationof the mainintracerebralarteries.May
amoyavessels are observedover the carotid fork.Stage 3. The defectionof the middleand anteriorcerebral
arteries. The moyamoyavessels are clearlydemonstrated.
Stage 4. Occlusionof the internalcarotidarteryextendsas faras thejunction ofthe posteriorcommunicatingartery,and finally, the posterior cerebral artery. The moyamoyavessels becomerough.
Stage 5. All of the mainarteriesarisingfromthe internalcarotid artery completely disappear. The moyamoyavessels are limited to the syphon and are minimizedand have a tendency to be reduced.
Stage 6. The syphon of the internalcarotid arterydisappearscompletely and the original moyamoya vessels arealsocompletelymissing.Inadditionto thesestages,patients in the present study included stage 0 on rightside in a case with unilateral moyamoya diseasewhose angiogramwas normalon the rightside.
Three groups are shown in Table 1. Mean ages of thesethree groups did not significantly differ (p > 0.05). IMPSPECF images were analyzed by two experienced observers without previous knowledge of angiographicstages.
Cerebral-w-CerebellarRatios. The circular ROIs were set onSPECT images in the upper and lower frontal, temporal, parietalandoccipitalregions,andthebasalganghiaandcerebellarhemispheres (Fig. 1). The size of the ROIwas 20 mm in diameter,andthemeanvaluesof twoorthreeROIsinsix regionsincludingtheupperandlower frontal,temporal,parietal,occipitalandthebasalganglia were calculated. Cerebral-to-cerebellaractivity ratios(C/C ratio) were estimated by dividing counts in the regions bythose in the cerebellum. Values were estimated as mean ±s.d.accordingto grade.
C/C Ratios in IMP SPECT with the Acetazolamide Test. IMPSPEC!'withacetazolamidewasperformedin ninepatients.ToobtainIMPSPECFwithacetazolamide,imageswereobtainedasecondtimewithin1wk afterthefirstscan.Thesamedoseof IMPwas injected 20 mm after intravenousinjection of acetazolamide
TABLE IClassificationof Grade
(C) (D)FIGURE 1. ROtsin eachbrainslice.(A)Orbitomeatalplane.(8)34 mm aboveorbitomeatalplsne. (C) 51 mm aboveorbitomeatalplane.(D)68 mm aboveorbitomeatalplane.1 = cerebellum,2 =lowfrontalcortex,3 = highfrontalcortex,4 = temporalcortex,5 =parietalcortexand 6 = occipitalcortex@
(1 g). Data acquisitionswere done using the same instrumentsandprocedures as those for IMP SPECF without acetazolamide.
Sta@sdceDatawere reportedas means ±s.d. Meansfor agegroupswere
comparedusingthenonpairedStudent'st-test.TheC/Cratiosinsix regionsbased on IMP SPECF imageswithoutacetazolamidewere also comparedusingthe nonpairedStudent'st-test. Thepaired Student's t-test was used for comparingC/C ratios in sixregions based on IMP SPEC!' images with and without acetazolamide.
RESULTS
Dlstilbutbons of Low rCBF Areas In IMP SPECTThe numbersof low rCBF regions in the 16 patients are
shown in Table 2. The frontal and parietal lobes were themost affectedareas, but low perfusionareaswere observedin all of the anteriorand posterior circulation regions, thenumbers of which ranged from 4 to 9 out of 16 hemispheres. There was no lateralityin the numbersof affectedareas. Table 3 shows the distributionof low rCBF areasaccording to grade. In grade 1, low rCBF areas were notobserved except for one parietalregion on one side in onepatient. In grade 2, from 3 (15.8%) to 8 (42.1%) of 19hemispheres showed low rCBF, which was observed mostfrequentlyin the frontalregion (42.1%)and less frequentlyin the basal ganghia(15.8%). In grade 3, low rCBF areaswere observed in more than5 (62.5%)of 8. The percentageof low rCBF areas increased in proportionto the severityof the respective grade.
Brain SPECT in Moyamoya Disease•Hoshi at al. 45
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GradeNo. of hemispheresFrontalTemporalParletalOccipitalBasalgan@iaUpperLower1
235
1980
8 (42.1%)6 (75%)0
6 (31.6%)5 (62.5%)0
4 (21.1%)7 (87.5%)1(20%)
5 (26.3%)7 (87.5%)0
5 (26.3%)5 (62.5%)0
3(15.8%)6(75%)n
= 16; number ofhemispheres= 32.
TABLE 2Distributionof LowrCBFAreas
Case ReportsFigures2A and B show rightand left cerebralangiogram
from a 6-yr-old boy. He was admitted to our hospital because of transient ischemic attacks (hAs) of aphasia andhemiparesis of the right upper extremity. Angiographicstageswere grade1 (Stage1)on the right sideandgrade2(Stage 3) on the left side. Figure 2C shows IMP SPECTimages without acetazolamide. Activities were slightly decreased throughout the entire left cerebral hemisphere.The C/C ratios rangedbetween 0.89 and 1.03.
Figure 2D shows results of IMP SPECF with acetazolamide in the same patient as was shown in Figure 2C.There was markedlydecreased activity throughoutthe entire left cerebral hemisphere in comparison with IMPSPECF images without acetazolamide. The C/C ratios alsodecreased between 0.73 and 0.88, except for those in theoccipital region.
Figures3A and B show rightand left cerebralangiogramfrom a 10-yr-oldgirl. She had been complaining of headaches and TIM consisting of unconsciousness for 2 mo.The angiographic stages were grade 2 (Stage 3) on bothsides. Figure 3C shows IMP SPECF without acetazolamide. Activity decreased slightly throughout the entire left cerebral hemisphere. The C/C ratios were between0.73 and 0.80. Figure 3D shows IMP SPECT images withacetazolamide. There is decreased activity in the left andright frontal regions and in the left temporal region whencompared with IMP SPECF images without acetazolamide. The C/C ratios also decreased between 0.58 and0.64. Activity increased slightly in the left occipital regionand in the left basal ganglia. The C/C ratios changed from0.78 to 0.80 and from 0.73 to 0.83, respectively.
DISCUSSION
Moyamoya disease is a rare cerebrovascular disorderinvolving the distal portion of the internal carotid arteryand/or the circle of Willis with basal telangiectasias cornprisingdilated collateral lenticulostriateand thalamoperforating arteries (7A 18—20).The name mcyamcya (“puffofsmoke―)was derived from the angiographicappearancesof the cerebrovascular abnormalitiesof collateral vessels.The initial symptoms at onset are a neurological deficitwhich is caused by ischemic changes in the brain or bysubarachnoid hemorrhage (21,22). The disease progresses
C/CRatiosInIMPSPECTTables 4 and 5 show estimates of mean C/C ratios in
each region according to age and grades, respectively.There were occasional decreases according to age in normal controls, but these were not significantlydifferent. Inmoyamoya disease, the mean C/C ratios rangedfrom 0.96to 1.06 and from 0.91 to 0.96 in grades 1 and 2, respectively. The mean C/C ratios in grade 3 were relatively low(0.76-0.88) compared to those in grades 1 and 2. The C/Cratios in stage 2 were significantlylower thanthose in stage1 in one (temporal)ofsix regions, andthose in grade3 weresignificantly lower than those in stage 2 in three (upperfrontal, temporal and parietal)of six regions, and those instage 3 were significantly lower than those in stage 1 in allregions. The mean values had a tendency to decrease asthe stage of the groups increased.
C/C Ratiosin IMP SPECTwithAcetazolamideTestTable 6 shows comparisons of the C/C ratios with and
without acetazolamide amongnine patients. In grade 1, themean C/C ratios of IMP SPECF without acetazolamide didnot differ significantly from those with the acetazolamidetest. There were significant differences between the twoC/C ratios in the upper and lower frontal, parietal andtemporal regions in grade 2. Although most of the meanC/C ratios of IMP SPECF with acetazolamide were lowerthan those without acetazolamide in grades 2 and 3, therewere no significantdifferences between the two C/C ratiosof the basal gangliaand the occipital region in grade 2, andbetween the two C/C ratios of all six regions in grade 3.
TABLE3Distilbutionof LowrCBFAreas
46 TheJournalof NudearMedicine•Vol.35•No. I •January1994
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slowly and there is no effective treatment. Moyamoya disease is diagnosed angiographicallyand follow-up examinations are necessary to evaluate disease progress. Althoughbilateral involvement by this disease is common, cases ofunilateral involvement by moyamoya disease have alsobeen reported(18). Ourpresent study includedone case ofunilateral moyamcya disease.
The CT findings in this disease consist of low densityregions which indicate brain infarction due to stenosis orobstruction of a cerebral artery (23). Frontal and parietalregions are frequently affected. Ischemic lesions on CF orMR images consist of cerebral infarction, brain atrophyand ventricular dilatation (24—28).In the present Study,low rCBF areas were observed most frequently in thefrontal and parietal lobes. However, the basal ganglia andoccipital regions were involved less frequently. The abnormal findingsof rCBF images are usually largerthanregionsof low density lesions observed in CT (27).
The C/C ratios of the regions decreased accordingto thegrades, especially in the upper and lower frontal, temporaland parietal regions. Only the temporal region showedsignificant differences between the C/C ratios in grade 1and those in grade 2. Three regions (upperfrontal, temporal and parietalregions) showed significantdifferences between the C/C ratios in grade 2 and those in grade 3. Allregions differedsignificantlyaccordingto the C/C ratios ingrade 1 and those in grade 3. These results showed that theC/C ratios and grades correlate well in the peripheralregions suppliedby the anteriorand middlecerebralarteries,
but that the posterior circulation regions, including theoccipital region and basal ganglia, are less affected becausethese regions are supplied by branches of the middle andposterior cerebral arteries. These findings are compatiblewith data reported previously (8).
Kuwabara et al. (9) reported that the right side wasinvolved more frequently than the left side in unilateralmcyamoya disease, and that areas of low rCBF lesions on
the rightside were largerthan those on the left side, evenin bilateral mcyamcya disease. However, there was nolateralpredominanceof affected areas in our study.
Denays Ct al. have reported that rCBF changes according to age (29). The C/C ratio is small in a 1-mo-old baby,however, this ratio increased from age 2 mo to 1yr; it thendecreased slightly until young adulthood and decreasedgradually thereafter. The change is small after 2 mo cornpared to before 2 mo of age. Our study included a 4-yr-oldboy, but the other patients ranged in age from 6 to 46 yr.We thinkthatthere is little effect on C/C ratiosby changingrCBF values due to aging when compared to individualdifferences as shown in Table 4.
Lassen et al. (11) reported the effect ofacetazolamide on
rCBF. Acetazolamide causes significant increase of rCBF,which reduces symptoms of acute mountainsickness. Theusefulness of the acetazolamide test in cerebro-occlusivedisease has also been reported (12-14). Vorstrup et al.reported that acetazolamide is valuable for detecting reduced rCBF reserve in patients with occlusive carotid disease (12). Acetazolamide is a vasodilator which offers the
TABLE 5C/CRatiosAccordingto Grade
47BrainSPECTin MoyamoyaDisease•Hoshiat al.
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convenience of intravenous administration with rapid onset of vasodilation. Maximum response occurs approximately 25 mm postinjection and has a half-time of 90 mm(15).
Acetazolamide studies in cases of normal vasculaturedemonstrate a range of increase of rCBF of 57%—70%(16).The possible mechanisms involved include inhibitionof theenzyme carbonic anhydrase in the brain parenchyma,which causes acute decrease in pH. The effect of acetazolamide on rCBF is probably explained by a decrease inbrainpH. Thus, acetazolamide causes an increase in rCBF
FiGURE 2. (A)Rightand (B)leftcerebralangiogramfroma 6-yr-oldboy.Narrowingofthe rightcarotidfork isobservedwithoutatenosisor obstructionof the branchesin therightantenorandmiddlecerebralarte.y.Thedefectsof the middleand antedorcerebralaiteries are observedin the left side. Themoyamoya vessels are clearly demonstrated. The anglographic stages wereGroup I (Stage 1) on the right side andGroup2 (Stage3) on the leftside. (C) IMPSPECTimagebeforeacetazolamide.Actlvitydecreased slightlyin the entireleftcarebral hemisphere.C/C ratios are between0.89and1.03.(D)IMPSPECTimageafteracetazolamide.ActMtydecreasedmarkedlyIn theentireleftcerebralhemisphere.C/Cratiosalsodecreasedbetween0.73and0.88, exceptforthose in the occipital region.
without change of the flow distributionin normal subjects(16). Although acetazolamide can also cause increases ofregional blood flow in the brain, it cannot cause such increases in blood supply in ischemic regions or even innormal regions where there are no cerebral blood flowreserves. Decreased reactivity to acetazolamide is thoughtto be due in large part to a decreased cerebral vascularreserve secondary to vasodilation in the resting state as acompensatory mechanism for proximal stenosis and/or occlusion (13). This results in relatively low activity regionscompared to blood flow in normal tissue.
FIGURE 3. (A)Rightand (B)leftcerebral— anglogram In a 10-yr-old girl. The defects of
them@e andanteriorcerebralaitedesareobservedon both sides. The moyamoyavesselsare alsoclearlydemonstrated.TheangiographicstageswereGroup2 (Stage3)onbothsides.(C)IMPSPECTimagebeforeacetazolamide.ActMtlesdecreasedslightlyin the entire left cerebral hemisphere.C/Cratiosare between073 and 0.80. (D) IMPSPECTimageafteracetazolamide.rCBFdecreasedmarkedlyinthe leftandrlghtfrontel regionsandthe lefttemporalregion.C/Cratios also decreasedbetween 0.58 and0.64. Activitiesincreased Slightlyin the left
U= ocxI@ regionand the left basal gangliaL C/Cratloschangedto0.8OfromO.78and
U 0.83 from 0.73, respectively.
D
L=U
The acetazolamide test can detect low rCBF reserveregions because of relatively highcontrast images betweennormalor ischemic lesions with reactive vessels and thosewith no reactive vessels. Hyperventilation is also used toevaluate the cerebral blood flow reserve, because decreased PaCO2in the arterialblood after hyperventilationcauses constriction of the cerebral artery (30). However,this procedure can be dangerous in some cases and itrequires the patient's effort. Quantitativeanalysis of IMPSPECF is also difficult because the degree of decrease ofrCBF depends on the degree of such effort by the patient.
In the present study, mean C/C ratios without acetazolamide in grade 1 were not changed after acetazolamide.The mean C/C ratios in grade 2 were lower than those inthe upper and lower frontal, temporal and parietalregionsafter acetazolamide and they were statistically significant.This shows that cerebral vascular reserve in these fourregions decreased in grade 2. Therewere cerebralvascularreserves in all six regions of grade 1 and in the occipitalregion and the basal ganglia in grade 2. These results indicate that perfusion in the posterior region and central region around the basal ganglia which are supplied by themoyamoya vessels or the posterior cerebral artery, consists of a perfusion reserve exceeding that of the other fourregions in grade 2. In other words, relatively low rCBFreserves were observed in the anterior circulation areas.
The C/C ratios in all six regions did not change much ingrade 3. C/C ratios indicate relative activity which de
creases in regions with low rCBF reserves in comparisonto normalor ischemic regions without vascular reactivity.We speculate that this is a result of the reactivity of cerebralvessels to decreases in acetazolamide throughouttheentire brain in grade 3 is useful.
In conclusion, IMP SPECF imaging is useful for evaluating rCBF in patients with moyamoya disease. The acetazolamide test is useful for detecting cerebralblood flowreserve, including that in normal rCBF regions. The testcan also prospectively detect disease progress.
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