Shinsuke Fujioka1, Avi A Algom2, Melissa E Murray3, Monica Y Sanchez-Contreras4, Pawel Tacik5, Yoshio Tsuboi6, Jay A Van Gerpen7, Ryan J Uitti8, Rosa Rademakers9, Owen A Ross10, Zbigniew K Wszolek11, Dennis W Dickson12. 1. Department of Neurology, 4500 San Pablo Road, Jacksonville, FL 32224, USA; Department of Neuropathology, 4500 San Pablo Road, Jacksonville, FL 32224, USA; Department of Neuroscience Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA; Department of Neurology, Fukuoka University, 7-45-1 Nanakuma, Jonan-ku, Fukuoka, Fukuoka 814-0180, Japan. Electronic address: shinsuke@cis.fukuoka-u.ac.jp. 2. Department of Neurology, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: aaalgom@yahoo.com. 3. Department of Neuropathology, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: murray.melissa@mayo.edu. 4. Department of Neuroscience Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: sanchezcontreras.monica@mayo.edu. 5. Department of Neurology, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: Tacik.Pawel@mayo.edu. 6. Department of Neurology, Fukuoka University, 7-45-1 Nanakuma, Jonan-ku, Fukuoka, Fukuoka 814-0180, Japan. Electronic address: tsuboi@cis.fukuoka-u.ac.jp. 7. Department of Neurology, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: vangerpen.jay@mayo.edu. 8. Department of Neurology, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: uitti@mayo.edu. 9. Department of Neuroscience Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: Rademakers.rosa@mayo.edu. 10. Department of Neuroscience Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: ross.owen@mayo.edu. 11. Department of Neurology, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: wszolek.zbigniew@mayo.edu. 12. Department of Neuropathology, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address: dickson.dennis@mayo.edu.
Abstract
INTRODUCTION: Tremor is thought to be a rare feature of progressive supranuclear palsy (PSP). METHODS: We retrospectively reviewed the database of the CurePSP brain bank at Mayo Clinic Florida to retrieve all available clinical information for PSP patients. All patients underwent a standard neuropathological assessment and an immunohistochemical evaluation for tau and α-synuclein. DNA was genotyped for the MAPT H1/H2 haplotype. RESULTS: Of the 375 PSP patients identified, 344 had a documented presence or absence of tremor, which included 146 (42%) with tremor, including 29 (20%) with postural/action tremors, 16 (11%) with resting tremor, 7 (5%) with intention tremor, 20 (14%) with a combination of different types of tremor, and 74 (51%) patients who had tremor at some point during their illness, but details were unavailable. The tremor severity of 96% of the patients (54/55) who had this data was minimal to mild. The probability of observing a tremor during a neurological examination during the patient's illness was estimated to be ∼22%. PSP patients with postural/action tremors and PSP patients with resting tremor responded to carbidopa-levodopa therapy more frequently than PSP patients without tremor, although the therapy response was always transient. There were no significant differences in pathological findings between the tremor groups. CONCLUSIONS: Tremor is an inconspicuous feature of PSP; however, 42% (146/344) of the PSP patients in our study presented some form of tremor. Because there is no curative therapy for PSP, carbidopa/levodopa therapy should be tried for patients with postural, action, and resting tremor.
INTRODUCTION:Tremor is thought to be a rare feature of progressive supranuclear palsy (PSP). METHODS: We retrospectively reviewed the database of the CurePSP brain bank at Mayo Clinic Florida to retrieve all available clinical information for PSPpatients. All patients underwent a standard neuropathological assessment and an immunohistochemical evaluation for tau and α-synuclein. DNA was genotyped for the MAPT H1/H2 haplotype. RESULTS: Of the 375 PSPpatients identified, 344 had a documented presence or absence of tremor, which included 146 (42%) with tremor, including 29 (20%) with postural/action tremors, 16 (11%) with resting tremor, 7 (5%) with intention tremor, 20 (14%) with a combination of different types of tremor, and 74 (51%) patients who had tremor at some point during their illness, but details were unavailable. The tremor severity of 96% of the patients (54/55) who had this data was minimal to mild. The probability of observing a tremor during a neurological examination during the patient's illness was estimated to be ∼22%. PSPpatients with postural/action tremors and PSPpatients with resting tremor responded to carbidopa-levodopa therapy more frequently than PSPpatients without tremor, although the therapy response was always transient. There were no significant differences in pathological findings between the tremor groups. CONCLUSIONS:Tremor is an inconspicuous feature of PSP; however, 42% (146/344) of the PSPpatients in our study presented some form of tremor. Because there is no curative therapy for PSP, carbidopa/levodopa therapy should be tried for patients with postural, action, and resting tremor.
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