Abstract Details

Title Disproportionately Enlarged Subarachnoid-space Hydrocephalus and Progressive Supranuclear Palsy

Topic Movement Disorders

Presentation(s) P1 - Poster Session 1 (11:45 AM-12:45 PM)

Poster/Presentation
Number 5-023

Objective We aimed to evaluate the prevalence of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) in parkinsonian disorders, including progressive supranuclear palsy (PSP), and evaluate associations with clinical features and imaging biomarkers.

Background Gait disturbance is the leading symptom of the Hakim’s triad of normal pressure hydrocephalus (NPH) and is also a cardinal feature of PSP. DESH is a distinct imaging pattern of NPH with undetermined frequency, clinical significance, and underlying mechanisms in parkinsonian disorders.

Design/Methods We conducted a cross-sectional study in 290 patients, including 181 PSP (62%), 36 corticobasal syndrome, 21 Parkinson’s disease, and 52 controls. An automated machine-based method for DESH (D) detection was applied to volumetric head MRI scans and DESH was determined as present (D+) or absent (D-). Using DESH classification plus a manual measurement for Evans’ index (E); cutoff > 0.3 = E+, patients were classified into four groups: Imaging-suggestive of hydrocephalus (D+E+), DESH positive only (D+E-), Evans’ index positive only (D-E+), and no imaging evidence of hydrocephalus (D-E-). Demographic, clinical and imaging metrics were ascertained and compared across groups.

Results Of the 290 patients, 214 were classified as: D+E+ (n=20); D+E- (n=8); D-E+ (n=71) and D-E- (n=115). 18/20 (90%) D+E+ cases were PSP. The D+E+ patients were older, had greater midbrain and cortical atrophy, more periventricular and deep white matter hyperintensities and larger cistern areas on MRI, lower striatal metabolism on [¹⁸f]fluorodeoxyglucose-PET and greater degeneration of long-range white matter tracts on diffusion tensor imaging, compared to the D-E- patients. D+E+ patients had a tendency to have hypermetabolism in the paracentral lobule on [¹⁸f]fluorodeoxyglucose-PET.

Conclusions DESH can be present in parkinsonian disorders, especially PSP, where it is associated with worse clinical and imaging outcomes. Its presence may be a mechanistic byproduct of degeneration that develop during the process of CSF flow re-distribution.

Authors/Disclosures
Muhui Fu, MD, PhD (Kaohsiung Chang Gung Memorial Hospital) PRESENTER	Dr. Fu has nothing to disclose.
Jeffery Gunter (Mayo Clinic and Foundation)	The institution of Jeffery Gunter has received research support from NIH. Jeffery Gunter has received intellectual property interests from a discovery or technology relating to health care.
Ryota Satoh, PhD	Dr. Satoh has nothing to disclose.
Rodolfo G. Gatto, MD, PhD (Mayo Clinic Neurology and Neurosurgery)	Dr. Gatto has nothing to disclose.
Farwa Ali, MD (Mayo Clinic)	Dr. Ali has nothing to disclose.
Heather Clark	The institution of Heather Clark has received research support from NIH. Heather Clark has received publishing royalties from a publication relating to health care.
Julie Stierwalt (Mayo Clinic)	The institution of Julie Stierwalt has received research support from NIH. Julie Stierwalt has received publishing royalties from a publication relating to health care.
Mary M. Machulda, PhD (Mayo Clinic)	The institution of Dr. Machulda has received research support from NIH.
Yehkyoung C. Stephens, PA	Mrs. Stephens has nothing to disclose.
Hossam Youssef, MD	Mr. Youssef has nothing to disclose.
Nha Trang Thu Pham (Mayo Clinic)	Nha Trang Thu Pham has received personal compensation for serving as an employee of Mayo Clinic.
Clifford R. Jack, Jr., MD (Mayo Clinic)	The institution of Dr. Jack has received research support from NIH. The institution of Dr. Jack has received research support from Alexander Family Alzheimer's Disease Research Professorship of the Mayo Clinic.
Val J. Lowe, MD (Mayo Clinic)	Dr. Lowe has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AVID Radiopharmaceutical. Dr. Lowe has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai Inc. The institution of Dr. Lowe has received research support from AVID Radiopharmaceuticals.
Keith A. Josephs, Jr., MD, FAAN (Mayo Clinic)	Dr. Josephs has nothing to disclose.
Jennifer Whitwell, PhD (Mayo Clinic)	The institution of Dr. Whitwell has received research support from NIH.

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