2. PSP; Steele-Richardson-Olszewski disease
成人発症の神経変性疾患;
早期からの姿勢不安定性, 垂直方向の核上性注視麻痺を特徴とする
臨床症状は特徴的であり, 眼は “Mona Lisa” stareと呼ばれ, 瞬きは少ない(0-4回/min).
頸部は後屈し, 声量は低下して不明瞭なうなり声に聞こえる.
動作は緩慢で, 後方に転倒しやすい.
下方視が困難であり, 嚥下機能も低下するため, 食事をよくこぼすようになる.
認知能も緩慢になるため, 返答や会話が遅くなる
複視や視野のぼやけ, 羞明,
不随意の眼瞼閉鎖は初期から出現する症状であり,
しばしば眼科を受診する.
多系統萎縮症と異なり, 著名な起立性低血圧は稀
This review provides an update on progressive supranuclear
palsy (PSP, or Steele-Richardson-Olszewski disease), an
adult-onset neurodegenerative disorder characterised by
early postural instability, which leads to falls, and a vertical
supranuclear-gaze palsy. Recent epidemiological studies
have shown that the disorder is more common than previously
recognised, that it is commonly misdiagnosed, and that it may
present to a wide range of hospital specialists. The diagnosis
of PSP hinges on clinical acumen. Attempts to identify a
suitable biomarker in the CSF or a specific and sensitive
imaging or neurophysiological technique have so far failed to
have a significant effect on the diagnostic process. Better
understanding of the molecular pathology of PSP has
highlighted the importance of tau-protein accumulation and
tau-genotype susceptibility in its pathogenesis. No drug
treatment significantly and consistently benefits patients, and
ReviewProgressive supranuclear palsy
Progressive supranuclear palsy:
where are we now?
David J Burn and Andrew J Lees
Lancet Neurology 2002;1:359-69
8. PSP 33名, PD 108名, MSA-P 19名, 健常人 50名のProspective study
T1-WIにて, 橋断面積/中脳断面積 (P/M), MCP-width/SCP-width ratioを評価し,
MR Parkinsonism Index = P/M x MCP/SCP を評価.
(MCP; Middle cerebellar peduncles, 中小脳脚, SCP; Superior cerebellar peduncles, 上小脳脚)
Radiology 2008;246:214-21
Figure 2
Figure 2: Sagittal and coronal T1-weighted volumetric spoiled gradient-echo MR images (15.2/6.8; section thickness, 0.6 mm; frequency- and phase-encoding ma-
trix, 256ϫ 256; flip angle, 15°) show midbrain area(1), pons area(2), MCP width(3), and SCP width(4) in (a) a control participant and(b) a patient with PSP. Images
show marked atrophy of both midbrain and SCP in the PSP patient in comparison with the healthy control participant. In the patient with PSP, values wereas follows:
midbrain area, 60 mm2
; pons area, 502 mm2
; MCP width, 8.15 mm; and SCP width, 1.70 mm. In the control participant, values were as follows: midbrain area, 108 mm2
;
pons area, 478 mm2
; MCP width, 10.05 mm; and SCP width, 4.10 mm.
NEURORADIOLOGY:MR Parkinsonism Index for Differentiation of Diseases Quattrone et al
1) 中脳断面積, 2) 橋断面積, 3) 中小脳脚幅, 4) 上小脳脚幅
a)は健常人, b)はPSP患者
9. 各パラメータ
MR Parkinsonism Index
Cutoffと感度, 特異度.
Radiology 2008;246:214-21was smaller than that in patients with
PD (P Ͻ .001) and in control partici-
pants (P Ͻ .001) (Fig 3). No difference,
however, was observed between pa-
tients with PD and control participants.
There was some overlap of individual
values among all groups (Fig 3). There
was no significant difference between
patients with possible PSP and patients
with probable PSP.
Sensitivity, specificity, and PPV of
MCP/SCP for differentiating patients
with PSP from those with PD, patients
with PSP from those with MSA-P, and
patients with PSP from control partici-
pants varied at different cutoff levels
(Table 4), and the optimal levels were
2.69 or greater, 2.43 or greater, and
2.69 or greater, respectively.
MR parkinsonism index values.—
Patients with PSP showed the highest
MR parkinsonism index values with re-
spect to all other groups (patients with
PSP vs patients with MSA-P, P Ͻ .001;
patients with PSP vs patients with PD,
P Ͻ .001; patients with PSP vs control
participants, P Ͻ .001) (Fig 3). There
was no overlap of individual values for
the MR parkinsonism index between
the patients with PSP (median, 19.42;
Table 2
Measurements for Brain Structures in Patients with PSP, MSA-P, and PD and Control
Participants
Group
Midbrain Area
(mm2
)
Pons Area
(mm2
)
SCP Width
(mm)
MCP Width
(mm)
PSP patients (n ϭ 33) 63 (38–94) 406 (230–516) 2.4 (1.6–3.3) 7.0 (5.1–9.4)
MSA-P patients (n ϭ 19) 110 (63–132) 343 (244–492) 2.9 (1.9–3.9) 5.9 (3.4–7.7)
PD patients (n ϭ 108) 119 (70–163) 471 (372–615) 3.7 (3.0–4.8) 8.7 (8.1–10.2)
Control participants (n ϭ 50) 122 (94–168) 469 (386–622) 3.8 (3.1–4.5) 8.8 (8.1–10.5)
Note.—Values are medians, and numbers in parentheses are ranges. For all measurements, P Ͻ .001 (Kruskal-Wallis test).
Table 3
Pairwise Comparisons between Groups
Group Comparison
Midbrain Area
P Value
Pons Area
P Value
SCP Width
P Value
MCP Width
P Value
PSP patients vs MSA-P patients Ͻ.001 .013 .001 .022
PSP patients vs PD patients Ͻ.001 Ͻ.001 Ͻ.001 Ͻ.001
PSP patients vs control participants Ͻ.001 Ͻ.001 Ͻ.001 Ͻ.001
MSA-P patients vs PD patients NS Ͻ.001 Ͻ.001 Ͻ.001
MSA-P patients vs control participants .019 Ͻ.001 Ͻ.001 Ͻ.001
PD patients vs control participants NS NS NS NS
Possible PSP patients vs probable PSP patients .003 NS Ͻ.001 .018
Note.—P values were determined with Mann-Whitney U test, with Bonferroni correction. NS ϭ not significant.
218 Radiology: Volume 246: Number 1—January 2008
strated that 6% of patients with PSP had a
P/M that overlapped with the P/M of con-
trol participants, a finding that was consis-
tent with our findings.
Pathologic evidence has also dem-
onstrated the atrophy of SCPs in pa-
tients with PSP (1,17), whereas atrophy
of MCPs has been found in patients with
MSA (19). Results in recent MR imaging
studies have confirmed these pathologic
observations in both diseases. Atrophy
of the SCPs has been shown on volumet-
ric MR images in patients with PSP
(15), and decreased width of MCPs re-
cently has been demonstrated on mid-
sagittal MR images in patients with MSA
(20). On this basis, in our study we
measured the width of both SCPs and
MCPs on MR images and also investi-
gated the MCP/SCP. Our data confirm
pathologic findings showing that the
SCP width was significantly smaller in
Table 4
MR Parkinsonism Index for Differentiation of Patients with PSP from Patients with PD
and MSA-P and Control Participants
Cutoff and Statistical Values
MR Parkinsonism
Index Value
MCP/SCP
Value
P/M
Value
PSP patients vs PD patients
Cutoff value Ն13.55 Ն2.69 Ն4.88
Sensitivity (%) 100 78.8 90.9
Specificity (%) 100 88.9 93.5
PPV (%) 100 68.4 81.1
PSP patients vs MSA-P patients
Cutoff value Ն12.85 Ն2.43 Ն4.62
Sensitivity (%) 100 93.9 97.0
Specificity (%) 100 89.5 94.7
PPV (%) 100 93.9 97.0
PSP patients vs control participants
Cutoff value Ն13.58 Ն2.69 Ն4.65
Sensitivity (%) 100 78.8 97.0
Specificity (%) 100 88.0 94.0
PPV (%) 100 81.2 91.4
Note.—Optimal cutoff values were determined by using receiver operating characteristic curve analysis.
NEURORADIOLOGY:MRParkinsonismIndexforDifferentiationofDiseases Quattroneetal
