PD, PSP, MSAの進行PD発症期間は重なるが, 比較的若い年齢で発症.急速進行し, 診断から10年も満たずに死亡.hostaticat leastn 3 minme thatevodopanosis ispportingns such, whichbladdermalitiesincludedetrusorunctionncreasedasound.MSA ise test, ifng.eagues25ng theves inuptakesociatedpathological grading system was proposed in 2005 toquantify GCI density and neuronal loss associated withstriatonigral degeneration and olivopontocerebellarataxia.31Although the origin of α-synuclein deposition inGCIs is not yet understood, the crucial role of50 60Age (years)PDPSPMSA70DxDxF CCCRFDxFRDWUDUWR80Figure 1: Milestones of disease advancement and total disease courseThe green rectangles indicate disease duration, commencing with the timepoint of ﬁrst symptoms.The verticallines denote time of clinical diagnosis of a parkinsonian or a cerebellar syndrome (Dx) and time of documentationof milestones of disease advancement. Reproduced from O’Sullivan and colleagues,21with permission from OxfordUniversity Press. C=cognitive disability. D=dysarthria or dysphagia. Dx=clinical diagnosis. F=frequent falls.MSA=multiple system atrophy. PD=Parkinson’s disease. PSP=progressive supranuclear palsy. R=residential care.U=urinary catheter.W=wheelchair dependent.Lancet Neurol 2009; 8: 1172–78C; cognitive disability, D; dysarthria, Dysphagia F; Frequent falls, R; Residential care,U; urinary catheter,W; wheelchair dependent
認知症合併率MSAでは認知機能低下は20%程度のみ.PSPでは59%であり, 認知症合併例では他のパーキンソニズムをきたす疾患を考慮すべきといえる.125 or MMSE score 24. The MMSE score was used for tenta-tive classiﬁcation only when a DRS score was not available.Cognitive status was not assessed or was not assessable in threepatients, all with a clinical diagnosis of progressive supranuclearpalsy that was conﬁrmed on pathological examination.The majority (76.7%) of the progressive supranuclear palsygroup coming to post-mortem were cognitively impaired at thetime of the initial assessment, and diagnosis was conﬁrmed in89.1%. In four of the impaired patients, signiﬁcant coincidentAlzheimer pathology (Braak stage 4–5) was reported, althoughno patient received a primary diagnosis of Alzheimer’s disease.Cases where an alternative diagnosis was made included cortico-basal degeneration, Lewy body disease and amyotrophic lateralsclerosis, plus one impaired patient with an initial clinical diagnosisof progressive supranuclear palsy received a ﬁnal diagnosis ofmultiple system atrophy. Diagnostic accuracy was 85.7% in theTable 4 Percentage of cognitively impaired (DRS 125) patients in multiple system atrophy and progressive supranuclearpalsy groups according to Clinician Global Impression disease severity and disease duration at assessmentMultiple system atrophy Progressive supranuclear palsyDisease duration Disease durationCGI of disease severity 54 years 4 years Total 54 years 4 years Total3–6 15% (137) 24% (197) 20% (334) 61% (138) 63% (123) 62% (261)1–2 22% (23) 0 (15) 13% (38) 50% (40) 30% (10) 46% (50)Total 16% (160) 23% (212) 20% (372) 58% (178) 61% (133) 59% (311)Numbers in parentheses show the total number of patients in the cell regardless of impairment and the percentages are depicted outside the parentheses.CGI = Clinician Global Impression.Figure 4 Percentage of multiple system atrophy (MSA) andprogressive supranuclear palsy (PSP) patients with cognitiveimpairment (DRS total score 125) at different disease durations.Data table shows total n per duration year for each group.Brain 2010: 133; 2382–2393
PD 21名, MSA-C 11名, MSA-P 8名, PSP 20名で評価T1 矢状断で中脳(Ams), 橋面積(Apn)を評価(A)(橋のNotchと四丘の下部を結ぶ線と平行の下端の線)T2 水平断で上小脳脚(SCP), 中小脳脚直径(MCP)を評価(B, C)Arq Neuropsiquiatr 2010;68(3):333-338Arq Neuropsiquiatr 2010;68(3)omes: MRI morphometryTable 1. Comparisons among measurements obtained**.Parkinson PSP MSA-c MSA-pApn(mm2)556.6(472.9-622.2)443.4(321.1-508.4)303.4(235.4-408.6)331.6(195.1-468.2)Ams(mm2)154.8(129.3-190.9)82.1(52.5-113.4)151.4(126.7-166.2)117.8(88.3-154.8)MCP(mm)17.1(14.8-19.0)14.5(10.4-16.9)9.7(7.2-12.5)11.7(6.5-19.3)SCP(mm)3.7(3.1-4.2)2.0(1.5-2.9)3.3(2.5-3.7)3.3(1.5-4.4)334used clinically to distinguish among PD, PSP, MSA-c andMSA-p with good sensitivity, speciﬁcity and accuracy.The aim of this study was to evaluate the diagnostic val-ue of structural anatomic changes identiﬁed by MRI andpropose MRI-based criteria to help the clinician to rec-ognize these parkinsonian syndromes.METHODPatientsThis is a cross-sectional study of sequential patientswas used for measurements of the midbrain aand pons area (Apn)12-14(Fig 1). These areas weraccording to the following parameters: two strwere drawn. The ﬁrst line was drawn so as to pathe superior pontine notch and the inferior equadrigeminal plate. The second line was dralel to the ﬁrst line so as to pass through the intine notch. The area of the midbrain was tracethe delta-shaped part above the ﬁrst line. Thepons was traced as the area between the ventFig 1. Measurements of midbrain and pons areas (Fig 1A, sagittal T1-weighted); measurement of SCP (Fig 1B,axial T2-weighted); measure of MCP (Fig 1C, axial T2-weighted).
PD, MSA, PSPの鑑別において,Arq Neuropsiquiatr 2010;68(3):333-338from cases with MSA-c and MSA-p did not diﬀer. How-ever, values of Ams was signiﬁcantly diﬀerent (p0.05).the diagnosis (p0.01). In MSA-c cases,and MCP measures were signiﬁcantly diﬀMSA-c: multiple system atrophy with cerebellar signs; MSA-p: multiple system atrophy with parkinsonian features;PSP: progressive supranuclear palsy; Ams: midbrain area Apn: pons area; MCP: middle cerebellar peduncle; SCP:superior cerebellar peduncle; NS: non-signiﬁcant.Table 3. Cut oﬀ, sensitivity, speciﬁcity, accuracy, positive and negative predictive values regardingParkinson s disease, PSP and MSA-c.Cut oﬀ Sensitivity Speciﬁcity Accuracy PPV PNVParkinsonApnAmsMCPSCP477133153.380.0%65.5%71.4%65.5%97.1%93.5%96.9%93.5%90.0%80.0%85.0%80.0%95.2%90.0%95.2%90.5%87.2%74.4%79.5%74.4%PSPApn*AmsMCP*SCP‒105‒3‒95.0%‒80.0%‒97.5%‒100%‒96.7%‒91.7%‒95.0%‒100%‒97.5%‒87.5%MSA-cApnAmsMCPSCP*10531512‒77.5%66.7%66.7%‒100%93.8%97.8%‒88.3%51.7%90.0%‒100%72.7%90.9%‒80.8%91.8%89.8%‒MSA-c: multiple system atrophy with cerebellar signs; PSP: progressive supranuclear palsy; Ams: midbrain area;Apn: pons area; MCP: middle cerebellar peduncle; SCP: superior cerebellar peduncle; *non-signiﬁcant (p0.05);PPV: predictive positive values; PNV: predictive negative values.
Fig 3. A and B, Sagittal (A) and axial (B) T2-weighted spinal cord MR images show hemosiderin deposition along (A) and around (B) the cord surface. Note associated severe cord atrophy(A) (dotted arrow). C and D, Axial T2-weighted MR images at the level of the cauda equina from patients with SS show peripheralization (C) and clumping (D) of the nerve roots due toarachnoiditis. E, An axial cut at the lumbar levels on a CT myelogram from a patient with SS shows clumping of nerve roots of the cauda equina due to arachnoiditis. F, T2-weighted sagittalMR image of the lumbosacral area from a patient with SS shows a lesion that was suspected of being a possible source of the chronic bleeding. A biopsy was performed, and blood productsand fibrous tissue were detected. C and E reprinted with permission from Kumar N. Superficial siderosis: associations and therapeutic implications. Arch Neurol 2007;64:491–96 (Copyright2007, American Medical Association).脊髄へのヘモジデリン沈着AJNR 2012;31:5-14脳表面へのヘモジデリン沈着所見は≥60yの一般人口の0.7%で認める所見.原因は脳出血やSAH, アミロイドアンギオパチー, アルツハイマーなど言われているが, ハッキリとは判明していないのが現状.Neurology® 2009;73:202–205