7. 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.
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pathological grading system was proposed in 2005 to
quantify GCI density and neuronal loss associated with
striatonigral degeneration and olivopontocerebellar
ataxia.31
Although the origin of α-synuclein deposition in
GCIs is not yet understood, the crucial role of
50 60
Age (years)
PD
PSP
MSA
70
Dx
Dx
F C
C
C
RFDx
F
RD
WUD
UW
R
80
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
8. 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 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
11. 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):333-338
Arq Neuropsiquiatr 2010;68(3)
omes: MRI morphometry
Table 1. Comparisons among measurements obtained**.
Parkinson PSP MSA-c MSA-p
Apn
(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)
334
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.
METHOD
Patients
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).
12. 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
Parkinson
Apn
Ams
MCP
SCP
477
133
15
3.3
80.0%
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%
PSP
Apn*
Ams
MCP*
SCP
‒
105
‒
3
‒
95.0%
‒
80.0%
‒
97.5%
‒
100%
‒
96.7%
‒
91.7%
‒
95.0%
‒
100%
‒
97.5%
‒
87.5%
MSA-c
Apn
Ams
MCP
SCP*
105
315
12
‒
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.
19. 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).
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
![MSA
孤発性, 急速進行性の神経変性疾患
自律神経障害, パーキンソン症候群, 小脳失調を3徴とし,
どれが前面にでるかでMSA-P, Cと表現される.
錐体路障害も認められる.
組織学的には, 基底核, 小脳, 橋, オリーブ下核, 脊髄の
グリオーシスを伴う神経脱落を認める.
発症率は0.6/100000-yr, >50yrでは3/100000-yr.
有病率は1.9-4.9/100000.
平均発症年齢は60y[34-83], 男女差は無し.
平均予命は発症から7-9yr程度と予後は悪い.
Lancet Neurol 2009; 8: 1172–78](data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7)