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Abstract Details

Pick’s Disease Is a Rare but Recognizable Etiology Underlying Progressive Apraxia of Speech
Aging, Dementia, and Behavioral Neurology
P3 - Poster Session 3 (5:30 PM-6:30 PM)
9-009
To describe patients with progressive apraxia of speech (PAOS) caused by underlying Pick’s Disease.
With progression, most PAOS patients develop atypical parkinsonian features suggestive of corticobasal syndrome (CBS) or progressive supranuclear palsy (PSP), and most autopsy confirmed cases have had an underlying 4-repeat tauopathy.
We reviewed patients enrolled in the Neurodegenerative Research Group’s ongoing studies on speech and language disorders and identified those with PAOS who had autopsy confirmed Pick’s disease.
Three patients were identified, all women. P1 reported onset of speech problems at age 54, with study enrollment at age 60. She had phonetic AOS and mild cognitive impairment (MCI), but no aphasia, parkinsonism, or behavioral changes. Over the next 2 years she developed personality and cognitive changes consistent with behavioral variant frontotemporal dementia (bvFTD), passing away at age 64. P2 had speech changes starting at age 47, but by enrollment at 49 met criteria for bvFTD with phonetic AOS. She developed agrammatism but no parkinsonism or limb apraxia and passed away at 53. P3 had speech changes at 58 and enrolled at age 60, at which time she had phonetic AOS and MCI. She had no follow up visits and died at age 63. Neuroimaging in P1 and P2 showed inferior frontal, insular, and anterior-medial temporal involvement, primarily on the right, as well as superior prefrontal involvement. In contrast, P3 had bilateral premotor but no temporal involvement.
Our findings highlight that Pick’s can present with PAOS. In contrast to PAOS caused by 4-repeat tau, our cases did not develop motoric impairment suggestive of PSP or CBS. All had phonetic AOS, which is more common among younger PAOS patients. Two of the three had prominent temporal hypometabolism, which is unusual for PAOS. These findings may prove useful in identifying Pick’s as a cause of PAOS.
Authors/Disclosures
Katherine Jones
PRESENTER 		No disclosure on file
Jonathan Graff-Radford, MD, FAAN Dr. Graff-Radford has received personal compensation for serving as an employee of Mayo Clinic. Dr. Graff-Radford has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NINDS/NIH. Dr. Graff-Radford has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology. Dr. Graff-Radford has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Open evidence . The institution of Dr. Graff-Radford has received research support from NIH. The institution of Dr. Graff-Radford has received research support from Eisai. The institution of Dr. Graff-Radford has received research support from Cognition therapeutics. Dr. Graff-Radford has received personal compensation in the range of $5,000-$9,999 for serving as a Faculty Member with IMPACT AD .
Rene Utianski Rene Utianski has nothing to disclose.
Joseph Duffy The institution of Joseph Duffy has received research support from NIH. Joseph Duffy has received publishing royalties from a publication relating to health care.
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.
Mary M. Machulda, PhD (Mayo Clinic) The institution of Dr. Machulda has received research support from NIH.
Dennis W. Dickson, MD (Mayo Clinic) Dr. Dickson has nothing to disclose.
Jennifer Whitwell, PhD (Mayo Clinic) The institution of Dr. Whitwell has received research support from NIH.
Keith A. Josephs, Jr., MD, FAAN (Mayo Clinic) Dr. Josephs has nothing to disclose.
Hugo Botha, MD (Mayo School of Graduate Medical 好色先生, Rochester) Dr. Botha has received research support from NIH. An immediate family member of Dr. Botha has received personal compensation in the range of $500-$4,999 for serving as a Study Section Member with NIH.