Brain Magnetic Resonance Imaging in Multiple …...tial diagnosis of multiple system atrophy with predomi-nantparkinsonism(MSA-P)andParkinsondisease(PD), since it frequently shows

Brain Magnetic Resonance Imagingin Multiple-System Atrophy and Parkinson Disease

A Diagnostic Algorithm

Kirsty Bhattacharya, MD; Daniela Saadia, MD; Barbara Eisenkraft, MD; Melvin Yahr, MD;Warren Olanow, MD; Burton Drayer, MD; Horacio Kaufmann, MD

Background: Brain magnetic resonance (MR) imagingoffers the potential for objective criteria in the differen-tial diagnosis of multiple system atrophy with predomi-nant parkinsonism (MSA-P) and Parkinson disease (PD),since it frequently shows characteristic abnormalities inpatients with MSA-P and is believed to be normal in pa-tients with PD.

Objective: To determine concordance between clini-cal and MR imaging–based diagnoses of MSA-P andPD.

Design: Two neuroradiologists identified and ratedstriatal and infratentorial abnormalities in 39 brainMR images and assigned a diagnosis of PD, MSA-P,or MSA with additional marked cerebellar ataxia(MSA-C).

Setting: Academic medical center.

Patients: Thirty-nine patients with parkinsonism, in-

cluding 21 with a clinical diagnosis of PD, 14 with MSA-P,and 4 with MSA-C.

Results: All patients with MSA and 14 (67%) of 21 pa-tients with PD had some abnormality on brain MR im-aging. Brainstem atrophy was seen in patients with MSA-Pand MSA-C. Putaminal atrophy was seen only in MSA-P.Putaminal hypointensity and lateral slitlike hyperinten-sity were seen in both PD and MSA-P but were alwaysmild in PD. Cerebellar abnormalities, seen in all pa-tients with MSA-C and 11 patients with MSA-P, were alsoidentified in 6 patients with PD, albeit always rated asmild. Nonconcordance between clinical and radiologi-cal diagnosis occurred in 2 patients with PD, 5 withMSA-P, and 1 with MSA-C.

Conclusion: Since several features on brain MR imagingare seen only in MSA-P, a simple diagnostic algorithm mayimprove the MR imaging diagnosis of MSA-P and PD.

Arch Neurol. 2002;59:835-842

M ULTIPLE-SYSTEM atro-phy (MSA) and Par-kinson disease (PD)are the 2 most preva-lent neurodegenera-

tive disorders that exhibit features of par-kinsonism and autonomic dysfunction.Many patients with MSA have, in addi-tion to parkinsonism, cerebellar ataxia, andthis combination is referred to as MSA-C.In the most frequent type of MSA, re-ferred to as MSA-P, patients have mostlyparkinsonian and few, if any, cerebellarsigns.1 Accordingly, the clinical distinc-tion between MSA-P and PD is often dif-ficult. Clinical history may be helpful be-cause, in patients with MSA-P, symptomsof autonomic failure frequently precedeparkinsonism but, not infrequently, par-kinsonism is the presenting feature. Com-plicating matters further, although pa-tients with PD usually present withparkinsonism, they may also have severe

autonomic failure in a later phase of thedisease, making the distinction fromMSA-P extremely difficult. The availabil-ity of new surgical and pharmacologictherapies that may be effective only in pa-tients with PD has heightened the need forprecise diagnosis.

In addition to clinical criteria, sev-eral tests are proposed to distinguish be-tween these disorders. The most com-monly used is the therapeutic response todopaminergic agents, the lack of whichsuggests a diagnosis of MSA-P. However,pharmacologic challenge is not specific, asmany patients with MSA-P may initiallyrespond to dopaminergic drugs. Other testsinclude neuroendocrine responses to hy-potension or centrally acting adrenergicagonists, which are blunted in patientswith MSA-P but preserved in patients withPD because brainstem-hypothalamic-pituitary pathways are affected only inMSA-P.2,3 Similarly, sphincter electromy-

ORIGINAL CONTRIBUTION

From the Departments ofNeurology (Drs Bhattacharya,Saadia, Yahr, Olanow, andKaufmann) and Radiology(Drs Eisenkraft and Drayer),Mount Sinai School ofMedicine, New York, NY.

(REPRINTED) ARCH NEUROL / VOL 59, MAY 2002 WWW.ARCHNEUROL.COM835

©2002 American Medical Association. All rights reserved.Downloaded From: https://jamanetwork.com/ by a Non-Human Traffic (NHT) User on 12/05/2020

ography may show denervation in patients with MSA-P,because the Onuf nucleus, which houses preganglionicparasympathetic neurons in segments S2 to S4 of the spi-nal cord, is affected in patients with MSA-P but is nor-mal in patients with PD.4 However, these diagnostic tests

are frequently ambiguous, their sensitivity and specific-ity are controversial, and, most important, they are notwidely available.

Magnetic resonance (MR) imaging of the brain iswidely available and frequently shows abnormalities in

Table 1. Brain MR Imaging Findings in Patients Clinically Diagnosed With MSA-P, PD, and MSA-C*

MSA-P, NO. (%)(n = 14)

PD, No. (%)(n = 21) P Value†

MSA-C, No. (%)(n = 4) P Value‡

PutamenAtrophy 6 (43) 0 .002 0 .25Low signal (body) 11 (79) 6 (29) .006 3 (75) �.99Low signal (body) �1 6 (43) 0 .002 0 .25Lateral slitlike hyperintensity 11 (79) 11 (52) .16 2 (50) .53Lateral slitlike hyperintensity �1 5 (36) 1 (5) .006 0 .28

BrainstemAtrophy midbrain 5 (36) 0 .006 3 (75) .28Atrophy pons 6 (43) 0 .002 2 (50) �.99Atrophy medulla 6 (43) 0 .002 2 (50) �.99“Hot-cross bun” sign 1 (7) 0 .002 2 (50) .11

CerebellumAtrophy vermis 7 (50) 6 (29) .29 4 (100) .12Atrophy hemispheres 6 (43) 3 (14) .11 3 (75) .58Signal change, middle cerebellar peduncle 3 (21) 0 .05 2 (50) .53

*MR indicates magnetic resonance; MSA-P, multiple-system atrophy with predominant parkinsonism; PD, Parkinson disease; and MSA-C, MSA-P with markedcerebellar ataxia. Rating scale for MR imaging abnormalities is as follows: 0, normal; 1, mild abnormality; 2, moderate abnormality; and 3, severe abnormality.

†MSA-P vs PD.‡MSA-P vs MSA-C.

PATIENTS AND METHODS

Thirty-nine patients with parkinsonism, who were fol-lowed up at the Mount Sinai Medical Center, New York,NY, participated in this study. Eighteen patients had a di-agnosis of probable MSA according to published criteria1

(13 men and 5 women, aged 59±11 years [range, 39-74years]; disease duration, 5±2 years [range, 1-10 years]; allages and disease durations are expressed as mean±SD).Fourteen had predominantly parkinsonian signs and wereclassified as having MSA-P (9 men and 5 women; age, 59±11years [range, 39-71 years]; disease duration, 5±3 years[range, 1-10 years]). Four had predominantly cerebellar fea-tures and were classified as having MSA-C (all men; age,57±12 years [range, 45-74 years]; disease duration, 4±2years [range, 3-6 years]). Twenty-one patients had a diag-nosis of PD (15 men and 6 women; age, 64±11 years [range,40-81 years]; disease duration, 7±6 years [range, 1-20years]) according to United Kingdom PD Brain Bank cri-teria.23 No patient with PD had clinical evidence of auto-nomic dysfunction. Patients with PD, MSA-P, and MSA-Chad a similar age. The male-female ratio was 15:6 in PD,9:5 in MSA-P, and 4:0 in MSA-C.

All patients underwent 1.5-T MR imaging, with a pro-tocol that included sagittal T1-weighted images (repeti-tion time/echo time, 600/14 seconds; slice thickness, 5 mm),axial intermediate and T2-weighted sequences (repetitiontime/echo time, 2500/30-90 seconds; slice thickness, 5 mm),and inversion recovery axial T1 images (repetition time/echo time/inversion time, 2500/20/800 seconds; slice thick-ness, 4 mm).

Two experienced neuroradiologists (B.D. and B.E.),blind to the clinical diagnosis, visually rated the MRimages for changes previously described in brain MRimages of patients with PD, MSA-P, and MSA-C.5-22 Theseincluded (1) putaminal signal hypointensity relative tothe globus pallidus on T2-weighted images, (2) linear slit-like hyperintensity in the posterolateral margin of theputamen on T2-weighted images, (3) putaminal atrophy,(4) cerebellar atrophy (vermis and cerebellar hemi-spheres), (5) brainstem atrophy (midbrain, pons, andmedulla), (6) decreased width of the substantia nigra parscompacta, and (7) abnormal signal intensity in the middlecerebellar peduncles. Images were rated on a scale from 0to 3, where 0 was normal; 1, mild; 2, moderate; and 3,severe abnormalities.

The raters gave a radiological diagnosis of PD,MSA-P, or MSA-C to each MR image. A radiological diag-nosis of MSA-P was based on findings of moderate to se-vere putaminal abnormalities or mild putaminal changetogether with brainstem or cerebellar abnormality. A ra-diological diagnosis of PD was based on near-normal MRimages, with few or no abnormalities, or the presence ofonly mild abnormality in the putamen, brainstem, or cer-ebellum. A radiological diagnosis of MSA-C was given ifcerebellar changes in the brainstem or cerebellum weremoderate or severe regardless of putaminal change. In ad-dition, the neuroradiologists used their overall impres-sion to assign a diagnosis.

Statistical analysis was performed with Fisher exact ttest for qualitative MR imaging variables and t test for analy-sis of unpaired quantitative independent variables. Signifi-cance was set at P=.05.



the striatum, brainstem, and cerebellum in patientswith MSA-P.5-22 Therefore, MR imaging may be a use-ful aid to clinical diagnosis. In this study, neuroradi-ologists who were blind to the clinical diagnosis ratedbrain MR images of patients with clinically diagnosedPD, MSA-P, and MSA-C. On the basis of previouslypublished characteristic MR imaging abnormalities,5-22

neuroradiologists assigned a diagnosis of PD, MSA-P,or MSA-C to each MR image. The concordance be-tween the clinical and MR imaging diagnosis was thenassessed and an algorithm was devised to improveradiological diagnosis.

RESULTS

MR IMAGING FINDINGS

Putamen

No patient with PD had atrophy of the putamen, but 11had slitlike hyperintensity of its posterolateral margin and6 had hypointensity of its body relative to the globuspallidum, always graded as mild (grade 1) (Table 1;Figure 1) with the exception of 1 patient, in whom slit-like hyperintensity was graded as moderate. Patients with

A

C

B

D

Figure 1. A, Normal iron distribution, ie, globus pallidum hypointensity relative to the putamen in a patient with a clinical diagnosis of Parkinson disease(axial section, 1.5 T, T2 weighting). B, Reversal of normal iron distribution with severe putaminal hypointensity relative to the globus pallidum (inset)in a patient with a clinical diagnosis of multiple-system atrophy with predominant parkinsonism (axial section, 1.5 T, T2 weighting). C, Normal iron distributionin the globus pallidum relative to the putamen in a patient with a clinical diagnosis of Parkinson disease (coronal section, 1.5 T, T2 weighting).D, Reversal of normal iron distribution with mild putaminal hypointensity relative to the globus pallidum (arrow) in a patient with a clinical diagnosisof multiple-system atrophy with predominant parkinsonism (coronal section, 1.5 T, T2 weighting).



PD with putaminal rim hyperintensity were signifi-cantly older than those without this finding (68±10 vs59±10 years; P=.02). Six patients with MSA-P had at-rophy of the putamen (P=.002 vs PD); 11 had slitlike hy-perintensity of its posterolateral margin (P=.16 vs PD),graded as mild in 6 and moderate or severe in 5 (P=.006vs PD); and 11 had hypointensity of its body (P=.006 vsPD), graded as mild in 5 and moderate or severe in 6(P=.002 vs PD) (Figure 2). Contrary to patients withPD, patients with MSA-P with and without putaminal rimhyperintensity were of a similar age. No patient withMSA-C had atrophy of the putamen, but 3 had mild hy-pointensity of its body and 2 had mild slitlike hyperin-tensity of its posterolateral margin.

Substantia Nigra

Decreased width of the substantia nigra pars compactawas identified in 1 of 21 patients with PD and “smudg-ing” of its posterior border in 2. No patient with eitherMSA-P or MSA-C had radiological changes in the sub-stantia nigra.

Brainstem Findings

No patient with PD had brainstem atrophy. Seven pa-tients with MSA-P had brainstem atrophy (Figure 3).Five patients had atrophy affecting the midbrain (ratedmild in 4 and moderate in 1), 6 patients had atrophy af-fecting the pons (rated mild in 5 and moderate in 1), and6 patients had atrophy affecting the medulla (rated mildin 4 and moderate in 2). Cruciform hyperintensity of thepons, the “hot-cross bun” sign (Figure 4), was presentin 1 patient.

Brainstem atrophy was seen in 3 patients withMSA-C. Two patients had moderate atrophy in all brain-stem areas. The hot-cross bun sign was present in 2.

Cerebellar Findings

Six patients with PD had some cerebellar abnormality,all rated mild. All 6 of these patients had atrophy of thevermis and 3 had atrophy of the cerebellar hemispheres.One patient had mild signal abnormality in the middlecerebellar peduncle (Figure 5). Eleven patients with

A B C

Figure 2. A, Normal posterolateral putaminal margin in a patient with a clinical diagnosis of Parkinson disease (axial section, 1.5 T, T2 weighting). B, Mild slitlikehyperintensity of the posterolateral putaminal rim (inset, arrow) in a patient with a clinical diagnosis of multiple-system atrophy with predominant parkinsonism(axial section, 1.5 T, T2 weighting). C, Moderate slitlike hyperintensity of the posterolateral putaminal rim (inset, arrow) in a patient with a clinical diagnosisof multiple-system atrophy with predominant parkinsonism (axial section, 1.5 T, T2 weighting).

A B C

Figure 3. A, Normal brainstem and cerebellum in a patient with a clinical diagnosis of Parkinson disease (sagittal section, 1.5 T, T1 weighting). B, Mild brainstem andcerebellar atrophy in a patient with a clinical diagnosis of multiple-system atrophy with predominant parkinsonism (sagittal section, 1.5 T, T1 weighting). C, Severebrainstem and cerebellar atrophy in a patient with a clinical diagnosis of multiple-system atrophy with predominant parkinsonism (sagittal section, 1.5 T, T1 weighting).



MSA-P had cerebellar abnormalities (P=.006 vs PD), 7had atrophy of the cerebellar vermis, 5 mild and 2 mod-erate; 6 had atrophy of the cerebellar hemispheres, 5 mildand 1 moderate; and 3 had abnormal signal in the middlecerebellar peduncle, 1 mild, 1 moderate, and 1 severe.

All patients with MSA-C had cerebellar abnormali-ties; all had atrophy of the vermis, 1 mild, 2 moderate, and2 severe; 3 had atrophy of the cerebellar hemispheres, 1mild, 1 moderate, and 1 severe; and 2 had abnormal sig-nal in the middle cerebellar peduncles, 1 mild and 1 mod-erate. The combination of infratentorial and putaminal

abnormalities was present in 6 patients with PD, 10 withMSA-P (P=.02 vs PD), and all patients with MSA-C.

CONCORDANCE BETWEEN RADIOLOGICALAND CLINICAL DIAGNOSIS

The neuroradiologists “erroneously” diagnosed 5 pa-tients with a clinical diagnosis of MSA-P as having PD(Table 2). Of these, mild atrophy of the putamen waspresent in 1, 3 had mild hypointensity of its body, and 4had mild hyperintensity of its posterolateral margin. Two

A B

Figure 4. A, Normal pons in a patient with a clinical diagnosis of Parkinson disease (axial section, 1.5 T, T2 weighting). B, “Hot-cross bun” sign: cruciformdegeneration of pontine fibers secondary to brainstem atrophy (inset) in a patient with a clinical diagnosis of multiple-system atrophy with predominantparkinsonism (axial section, 1.5 T, T2 weighting).

A B

Figure 5. A, Normal cerebellum in a patient with a clinical diagnosis of Parkinson disease (axial section, 1.5 T, T2 weighting). B, Abnormal signal in the middlecerebellar peduncles (arrow) in a patient with a clinical diagnosis of multiple-system atrophy with predominant parkinsonism (axial section, 1.5 T, T2 weighting).



of these patients had brainstem atrophy and 2 had mildcerebellar atrophy. Patients with MSA-P “wrongly” di-agnosed radiologically as having PD had significantlyshorter disease duration (4±1 vs 6±3 years; P=.05), al-though they were of similar age in comparison with pa-tients with MSA-P “correctly” diagnosed by the neurora-diologists.Theneuroradiologists“erroneously”diagnosed2 patients with a clinical diagnosis of PD as having MSA-P. One patient had mild signal abnormality in the middlecerebellar peduncle and the other had mild putaminal andcerebellar abnormalities.

The 1 patient with MSA-C “erroneously” diagnosedby the neuroradiologists as having PD had only mildchanges in the putamen, brainstem, and cerebellum.

COMMENT

We found that several features on brain MR images ofpatients with parkinsonism are diagnostic of MSA-P, asthey are never seen in patients with PD. These includedputaminal and brainstem atrophy and abnormal signalin the middle cerebellar peduncles. Other abnormalitieswere found in both patients with MSA-P and thosewith PD, but they varied in severity. These included hypo-intensity of the putaminal body, slitlike hyperintensityof the lateral putaminal border, and atrophy of the cer-ebellar vermis or hemispheres. When the severity of theseabnormalities was graded, it was always mild in patientswith PD. All patients with MSA-P and two thirds ofpatients with PD had some abnormality on brain MRimaging.

Two patients with PD were assigned a diagnosis ofMSA-P by the neuroradiologists. One was a 38-year-oldwoman with onset of PD at age 18 years. On MR imagingshe had mild signal change in the middle cerebellar pe-duncle. We speculate that patients with juvenile-onset PDmay have different MR imaging characteristics than thosein patients with adult-onset PD. The other patient was a

68-year-old man with disease duration of 4 years, asym-metric onset of tremor, excellent levodopa responsive-ness, and dyskinesias, making a diagnosis of MSA-P un-likely. On brain MR imaging, he had mild hypointensityof the body of the putamen, mild hyperintensity of its lat-eral rim, and mild atrophy of the cerebellar vermis andhemispheres.

The 5 patients with MSA-P, assigned an MR imag-ing diagnosis of PD by the neuroradiologists, had, in gen-eral, mild putaminal and cerebellar abnormalities on brainMR images. Their mean disease duration was less than 5years (range, 1-5 years), significantly shorter than thatof patients with MSA-P assigned the “correct” MR im-aging diagnosis. Brain MR imaging may be of limited valuein patients with MSA-P early in their disease, as it mayshow only mild abnormalities. However, in these 5 pa-tients there were abnormalities present, which were spe-cific to MSA-P; 1 patient had mild atrophy of the puta-men and 2 had brainstem atrophy. The patient withMSA-C neuroradiologically diagnosed as having PD hadonly mild putaminal and cerebellar abnormalities but alsomild brainstem atrophy, an abnormality not found in pa-tients with PD. In sum, nonconcordance between clini-cal and radiological diagnoses occurred in cases with mildputaminal hypointensity, mild hyperintensity of the lat-eral putaminal border, and mild cerebellar atrophy, whichwere abnormalities found in all 3 patient groups. Iden-tifying abnormalities found only in MSA-P, such as at-rophy of the putamen and brainstem or abnormal signalchange in the cerebellar peduncle, improves the accu-racy of radiological diagnosis in MSA-P and PD.

We therefore propose a simple diagnostic algo-rithm for the diagnosis of PD and MSA-P using brain MRimages (Figure 6). Atrophy of the putamen or brain-stem and abnormal signal in the middle cerebellar pe-duncle are diagnostic of MSA-P. Hypointensity of the bodyof the putamen, hyperintensity of its lateral rim, and at-rophy of the cerebellar vermis and hemispheres are foundin both MSA-P and PD and are diagnostic of MSA-P onlywhen severity is rated as more than mild, ie, moderateor severe. Mild putaminal hypointensity, mild putam-inal lateral slitlike hyperintensity, and mild cerebellar at-rophy could represent PD or early MSA-P of less than 5years’ duration, and thus follow-up MR imaging is needed.If brain MR imaging is normal, the patient could havePD or MSA-P of less than 1 year’s duration, as, althoughMR imaging was abnormal in all patients with MSA-P,no patient had disease duration of less than 1 year.

With this algorithm, the 3 patients with MSA-P andthe 1 patient with MSA-C “wrongly” diagnosed radio-logically as having PD would not have been assigned abrain MR imaging diagnosis of MSA-P or MSA-C, as theyhad abnormalities never found in patients with PD. There-fore, the proposed algorithm increases the concordancebetween radiological and clinical diagnoses.

The major limitation of this algorithm is that it hasnot been validated by neuropathologic data and clinicaldiagnosis could be wrong. It is possible that patients withPD who had MR imaging abnormalities could have re-ceived an incorrect clinical diagnosis and actually hadMSA-P, and that patients with MSA-P who had mild MRimaging abnormalities could have indeed had PD. How-

Table 2. Brain MR Imaging Findings in PatientsWith MSA-P With Concordant Radiological Diagnosisof MSA-P and Nonconcordant Radiological Diagnosis of PD*

Diagnosisof MSA-P(n = 9)

Diagnosisof PD

(n = 5)P

Value

Age, y (mean ± SD) 61 ± 12 57 ± 10 .25Disease duration, y (mean ± SD) 6 ± 3 4 ± 1 .05Putamen, No. (%)

Lateral slitlike hyperintensity 7 (78) 4 (80) �.99Lateral slitlike hyperintensity �1 5 (71) 0 .09

Low signal (body) 8 (89) 3 (60) .51Low signal �1 (body) 6 (75) 0 .03

Atrophy 5 (56) 1 (20) .30Atrophy �1 2 (40) 0 .51

Brainstem, No. (%)Brainstem atrophy 8 (89) 2 (40) �.99

Cerebellum, No. (%)Cerebellar atrophy 5 (56) 2 (40) �.99

*MR indicates magnetic resonance; MSA-P, multiple-system atrophywith predominant parkinsonism; and PD, Parkinson disease. Rating scalefor MR imaging abnormalities is as follows: 0, normal; 1, mild abnormality;2, moderate abnormality; and 3, severe abnormality.



ever, several factors suggest that the clinical diagnosis wasthe right one. First, during an average of 7 years of follow-up, no patient with clinically diagnosed PD developedsigns or symptoms suggestive of MSA-P,24 but this is notconclusive. Second, all patients with clinically diag-nosed PD had asymmetric onset of symptoms and signsand were levodopa responsive, and almost all devel-oped dyskinesias over time, features unusual in MSA-Pbut again nonconclusive. Similarly, several factors sug-gest that the clinical diagnosis was the correct one inMSA-P. All patients with MSA-P had early autonomic fail-ure, had symmetric onset of parkinsonism, were poorlylevodopa responsive or were levodopa unresponsive, and,within an average of 5 years of follow-up, did not de-velop dyskinesias.

Atrophy of the putamen and moderate or severehypointensity of its body were found only in patientswith MSA-P and not in patients with PD or MSA-C. Thissuggests that putaminal atrophy and hypointensity ofthe putaminal body, thought to be due to increase iniron deposition in the putamen,5,6 are indicative of “end-organ” striatal damage, as they were not present or weremild in patients with PD or MSA-C with mild extrapyra-midal signs but were present with increased severity in

patients with MSA-P and severe extrapyramidal signs.This is in agreement with previous studies finding corre-lation between degree of putaminal atrophy and severityof extrapyramidal signs.17,21,22

Slitlike hyperintensity of the posterolateral putam-inal margin has not, to our knowledge, been reported inPD.18 We found definite but mild slitlike hyperintensityof the lateral putamen in 11 of 21 patients with a clini-cal diagnosis of PD. Mild putaminal rim hyperintensityis frequently seen on brain MR images in normal aging(B.D., unpublished data, 2000) and may represent age-related reactive gliosis. Interestingly, patients with PD whohave this finding were significantly older than those with-out it. We found that all patients with MSA-P with pro-nounced putaminal rim hyperintensity had concomi-tant putaminal atrophy, suggesting that marked putaminalrim hyperintensity may be due to extracellular fluid ac-cumulation in the putaminal capsule secondary to atro-phy of the nucleus.20

Although radiological abnormalities of the substan-tia nigra, including decreased width and smudging ofits posterior border, have been reported in MSA-P, MSA-C,and PD, we found a low frequency, in only 2 of 21patients with PD and no patients with MSA-P or MSA-C.

MR Image

Normal

Yes

≤1 y >1 y Brainstem

AbnormalSignal MCP

PutaminalAtrophy

Putaminal BodyHypointensity

PosterolateralPutaminal Slitlike

Hypointensity

What Is the Degreeof Abnormality?

Moderate/Severe Mild

Atrophy

PD MSA-P

CerebellarAtrophy

Cerebellum Putamen

No

What Is theDisease Duration?

Where Is theAbnormality?

What Is theAbnormality?

Early MSA-P/PD Early MSA-P/PD

Figure 6. Algorithm for the magnetic resonance (MR) imaging diagnosis of multiple-system atrophy with predominant parkinsonism (MSA-P) andParkinson disease (PD). Red indicates MR imaging diagnosis of MSA-P; blue, MR imaging diagnosis of PD; green, MR imaging diagnosis of PDor early MSA-P; and MCP, middle cerebellar peduncles.



Therefore, substantia nigra abnormality was not in-cluded in the algorithm.

In conclusion, it is often difficult to distinguish clini-cally between patients with MSA-P and PD, and high–field-strength brain MR images may be useful, as patients withMSA-P show specific abnormalities that can distinguishthem from patients with PD. Other abnormalities are foundin both disorders, but, when the severity of the abnormal-ity is graded, it is always mild in patients with PD. On thebasis of these findings, we have designed a simple algo-rithm to help distinguish patients with MSA-P frompatients with PD by means of brain MR imaging.

Accepted for publication January 22, 2002.Author contributions: Study concept and design

(Drs Bhattacharya, Saadia, Yahr, Drayer, and Kauf-mann); acquisition of data (Drs Bhattacharya, Saadia,Eisenkraft, Olanow, Drayer, and Kaufmann); analysisand interpretation of data (Drs Bhattacharya, Saadia,Eisenkraft, Drayer, and Kaufmann); drafting of the manu-script (Drs Bhattacharya, Saadia, Drayer, and Kauf-mann); critical revision of the manuscript for importantintellectual content (Drs Bhattacharya, Saadia, Eisenkraft,Yahr, Olanow, Drayer, and Kaufmann); statistical exper-tise (Drs Bhattacharya, Saadia, and Kaufmann); adminis-trative, technical, or material support (Drs Eisenkraft andDrayer); study supervision (Drs Yahr, Olanow, Drayer,and Kaufmann).

Corresponding author and reprints: Horacio Kauf-mann, MD, Mount Sinai School of Medicine, Campus Box1052, New York, NY 10029 (e-mail: [email protected]).

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Brain Magnetic Resonance Imaging in Multiple …...tial diagnosis of multiple system atrophy with predomi-nantparkinsonism(MSA-P)andParkinsondisease(PD), since it frequently shows