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. 2015 May 21:1:15007.
doi: 10.1038/npjparkd.2015.7. eCollection 2015.

Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center

Tao Xie  1 , Un J Kang  2 , Sheng-Han Kuo  2 , Markos Poulopoulos  3 , Paul Greene  4 , Stanley Fahn  2
Affiliations

Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center

Tao Xie et al. NPJ Parkinsons Dis. .

Abstract

Background/objectives: The clinical diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remains challenging due to heterogeneity of the diseases.

Aims: Here we compared the clinical features of PSP and MSA to gain insight into their diagnosis and prognosis, particularly the prognostic value of down-gaze palsy latency in PSP progression.

Methods: We reviewed clinical features of pathologically confirmed 10 PSP and 13 MSA patients, incidentally matched in age-at-onset, gender, and disease duration, followed at Columbia University Medical Center during 1994-2009.

Results: The final antemortem diagnosis was incorrect in 30% of PSP (all lacking down-gaze palsy) and 23% of MSA patients. Falls in the first year of the disease, pyramidal involvement and freezing of gait during the course were similar between PSP and MSA. Ataxia and apraxia were in 50% of the PSP patients. Parkinsonism responsive to levodopa treatment was in 30% of the PSP (all with resting tremor) and 50% of the MSA patients. Dysautonomia in MSA could occur as early as 3 years preceding the motor symptoms, with 46% within the first year of the motor onset, but 15% did not have dysautonomia in life. The latency of down-gaze palsy and urogenital dysfunction and MMSE scores at first visit in PSP, and the latency of falls and wheelchair confinement in MSA were all associated with the disease progression.

Conclusions: We confirmed most of the previously published characterizations, and identified for the first time the prognostic value of down-gaze palsy latency in PSP progression.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Correlation between disease duration and important clinical features. The correlation between disease duration and the latency of down-gaze palsy (top left panel, in 7 patients with down-gaze palsy before death, with Pearson’s correlation coefficient r=0.902, P=0.005; and top right panel, in 10 patients including the 3 patients without down-gaze palsy in life, taking the duration of the disease as the latency in these 3 patients, with r=0.8893, P=0.001), MMSE score at first visit (on average the first 2.7 years after disease onset, mid left panel, r=0.920, P=0.009) and the latency of dysautonomia (mid right panel, r=0.752, P=0.031) in PSP; and the latency of falls (bottom left panel, r=0.675, P=0.016) and WCB (wheel chair bound, bottom right panel, r=0.990, P=0.010) in MSA.
See this image and copyright information in PMC

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