Abstract Details

Title Early Clinical Features in Japanese Patients with Pathologically Proven Progressive Supranuclear Palsy with Cerebellar Ataxia

Topic Movement Disorders

Presentation(s) P04 - (-)

Poster/Presentation
Number 163

Objective

Background BACKGROUND: Several clinical variants of PSP have been identified. We have defined a variant of PSP, PSP-C. Because patients with PSP-C develop cerebellar ataxia as the initial and principal symptom, they might be clinically misdiagnosed as having MSA-C. Because a specific biomarker of PSP has not yet been found, signs and symptoms are useful for making a diagnosis. We compared the signs and symptoms in PSP-C with those in MSA-C in pathologically proven patients.

Design/Methods DESIGN/METHODS: We reviewed the medical records of four consecutive Japanese patients with pathologically proven PSP-C as well as 11 consecutive Japanese patients with pathologically proven MSA-C. We recorded the presence or absence of clinical features both early in (within two years of disease onset) and at any time during the disease course in these patients. We compared the signs and symptoms early in the disease course in PSP-C patients with those in MSA-C patients.

Results RESULTS: All of the PSP-C patients (4/4, 100%) developed cerebellar ataxia and falls early in their disease course. Additionally, three of them (3/4, 75%) developed supranuclear vertical gaze palsy and postural instability. All of the PSP-C patients developed cardinal signs of PSP during the disease course. In contrast, in MSA-C patients, three of the 11 patients (27.3%) developed falls and none (0/10,0%) developed supranuclear vertical gaze palsy early in the disease course. The frequencies of falls and development of supranuclear vertical gaze palsy early in the disease course were higher in PSP-C than in MSA-C (p=0.026 and p=0.011).

Conclusions CONCLUSIONS: Early falls and supranuclear vertical gaze palsy predict the diagnosis of PSP-C in patients with cerebellar ataxia. Such a symptom and sign may help distinguish between PSP-C and MSA-C early in the disease course.

Authors/Disclosures
Masato Kanazawa, MD, FAAN (Niigata University of Health and Welfare) PRESENTER	Dr. Kanazawa has nothing to disclose.
Takayoshi Shimohata, MD, FAAN (Department of Neurology, Gifu University)	Dr. Shimohata has nothing to disclose.
Mari Tada, MD, PhD (Niigata University, Brain Research Institute)	Dr. Tada has nothing to disclose.
Osamu Onodera, MD	Dr. Onodera has nothing to disclose.
Hitoshi Takahashi	No disclosure on file
Masatoyo Nishizawa, MD (Niigata University of Health and Welfare)	No disclosure on file
David H. Margolin, MD, PhD (Genzyme Corporation)	Dr. Margolin has received personal compensation for serving as an employee of uniQure, Inc.. Dr. Margolin has stock in Cerevance, Inc. Dr. Margolin has stock in Datacubed Health. Dr. Margolin has stock in uniQure, Inc.. Dr. Margolin has received intellectual property interests from a discovery or technology relating to health care.

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