PD, PSP, MSAの進行
n 3 min
e test, if
pathological grading system was proposed in 2005 to
quantify GCI density and neuronal loss associated with
striatonigral degeneration and olivopontocerebellar
Although the origin of α-synuclein deposition in
GCIs is not yet understood, the crucial role of
Figure 1: Milestones of disease advancement and total disease course
The green rectangles indicate disease duration, commencing with the timepoint of ﬁrst symptoms.The vertical
lines denote time of clinical diagnosis of a parkinsonian or a cerebellar syndrome (Dx) and time of documentation
of milestones of disease advancement. Reproduced from O’Sullivan and colleagues,21
with permission from Oxford
University 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: 1172–78
C; cognitive disability, D; dysarthria, Dysphagia F; Frequent falls, R; Residential care,
U; urinary catheter,W; wheelchair dependent
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 three
patients, all with a clinical diagnosis of progressive supranuclear
palsy that was conﬁrmed on pathological examination.
The majority (76.7%) of the progressive supranuclear palsy
group coming to post-mortem were cognitively impaired at the
time of the initial assessment, and diagnosis was conﬁrmed in
89.1%. In four of the impaired patients, signiﬁcant coincident
Alzheimer pathology (Braak stage 4–5) was reported, although
no 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 lateral
sclerosis, plus one impaired patient with an initial clinical diagnosis
of progressive supranuclear palsy received a ﬁnal diagnosis of
multiple system atrophy. Diagnostic accuracy was 85.7% in the
Table 4 Percentage of cognitively impaired (DRS 125) patients in multiple system atrophy and progressive supranuclear
palsy groups according to Clinician Global Impression disease severity and disease duration at assessment
Multiple system atrophy Progressive supranuclear palsy
Disease duration Disease duration
CGI of disease severity 54 years 4 years Total 54 years 4 years Total
3–6 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) and
progressive supranuclear palsy (PSP) patients with cognitive
impairment (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)
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):333-338
Arq Neuropsiquiatr 2010;68(3)
omes: MRI morphometry
Table 1. Comparisons among measurements obtained**.
Parkinson PSP MSA-c MSA-p
used clinically to distinguish among PD, PSP, MSA-c and
MSA-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 and
propose MRI-based criteria to help the clinician to rec-
ognize these parkinsonian syndromes.
This is a cross-sectional study of sequential patients
was used for measurements of the midbrain a
and pons area (Apn)12-14
(Fig 1). These areas wer
according to the following parameters: two str
were drawn. The ﬁrst line was drawn so as to pa
the superior pontine notch and the inferior e
quadrigeminal plate. The second line was dra
lel to the ﬁrst line so as to pass through the in
tine notch. The area of the midbrain was trace
the delta-shaped part above the ﬁrst line. The
pons was traced as the area between the vent
Fig 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):333-338
from 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 diﬀ
MSA-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 regarding
Parkinson s disease, PSP and MSA-c.
Cut oﬀ Sensitivity Speciﬁcity Accuracy PPV PNV
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 to
arachnoiditis. 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 sagittal
MR 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 products
and 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 (Copyright
2007, American Medical Association).
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