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

Tao Xie, Un Jung Kang, Sheng Han Kuo, Markos Poulopoulos, Paul Greene, Stanley Fahn

Research output: Contribution to journalReview article

10 Citations (Scopus)

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.

Original languageEnglish
Article number15007
JournalParkinson's Disease
Volume1
DOIs
StatePublished - 1 Jan 2015

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Progressive Supranuclear Palsy
Multiple System Atrophy
Paralysis
Primary Dysautonomias
Apraxias
Wheelchairs
Parkinsonian Disorders
Levodopa
Tremor
Ataxia
Gait
Age of Onset
Freezing
Disease Progression

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Xie, Tao ; Kang, Un Jung ; Kuo, Sheng Han ; Poulopoulos, Markos ; Greene, Paul ; Fahn, Stanley. / Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center. In: Parkinson's Disease. 2015 ; Vol. 1.
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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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Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center. / Xie, Tao; Kang, Un Jung; Kuo, Sheng Han; Poulopoulos, Markos; Greene, Paul; Fahn, Stanley.

In: Parkinson's Disease, Vol. 1, 15007, 01.01.2015.

Research output: Contribution to journalReview article

TY - JOUR

T1 - Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center

AU - Xie, Tao

AU - Kang, Un Jung

AU - Kuo, Sheng Han

AU - Poulopoulos, Markos

AU - Greene, Paul

AU - Fahn, Stanley

PY - 2015/1/1

Y1 - 2015/1/1

N2 - 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.

AB - 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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U2 - 10.1038/npjparkd.2015.7

DO - 10.1038/npjparkd.2015.7

M3 - Review article

AN - SCOPUS:84960852597

VL - 1

JO - Parkinson's Disease

JF - Parkinson's Disease

SN - 2042-0080

M1 - 15007

ER -