An unusual case of rapidly progressive aphasia with mild dementia
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1
Tanaka Clinic, Japan
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2
VA Boston Healthcare System, United States
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3
School of Medicine, Boston University, United States
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4
Sakakibara Onsen Hospital, Japan
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5
Kato Clinic, Japan
Background: Primary progressive aphasia (PPA) is a clinical syndrome defined by progressive deterioration of language with preservation of activities of daily living and evidence of relatively normal non-verbal abilities on neuropsychological testing (Mesulam and Weintraub, 1992). Although additional cognitive changes may emerge later in the course of the illness, deficits must be grossly confined to the language domain for at least 2 years to fulfill the criteria for diagnosis. In this report we describe an unusual case of rapidly progressive aphasia with mild dementia.
Case Report: The patient is a 67-year-old, right-handed man who had minimal impairment of auditory comprehension and no other language or cognitive deficits in December 2017, as described by family and friends. By February 2018, he had markedly impaired language function. By July 2018, meaningful verbal communication with him was impossible. On admission to the hospital in December 2018, on the Japanese Standard Language Test for Aphasia (SLTA) he was found to have severe impairment of auditory comprehension, sentence repetition, and word-finding ability. All primary sensory and motor system functions were intact. His Raven’s Colored Progressive Matrices (RCPM) performance was 14/36; performance time on the Trail Making Test (TMT) Part A was 188sec, Part B was 561 sec. IQ measured by Kohs’ Block Design Test was 74. CSF was normal and his EEG showed no periodic synchronous discharges (PSD). MRI revealed bi-frontal and left temporal atrophy. IMP-SPECT demonstrated decreased CBF in the left fronto-temporal cortex.
Discussion: Cases of Rapidly Progressive Aphasia (RPA) have appeared only rarely in the literature. In five such cases, as in ours, mild language impairment progressed rapidly to severe aphasia within less than one year. Neuropathologically, a diagnosis of Creutzfeldt-Jacob disease (CJD) was made in four of these five cases; Alzheimer’s disease (AD) was the diagnosis in the fifth. In other reports, diagnoses of motor neuron disease (MND), pseudobulbar palsy, dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and Parkinson disease (PD) were made. Our case was not clinically compatible with CJD, MND, DLB, PSP or PD, although Alzheimer’s disease or an Alzheimer disease variant remains a possibility.
Conclusion: The speed and character of language deterioration in this patient, as well as the duration of his aphasia before the onset of his additional cognitive decline, offer a new clinical perspective on the relation of rapidly progressive brain deterioration to language function in the clinical syndrome of rapidly progressive aphasia.
References
Mesulam MM, Weintraub S, et al. Spectrum of primary progressive aphasia. Baillieres Clin Neurol, 1992;1:583-609.
Keywords:
Alzheime disease,
Pprogressive aphasia,
Dementia,
Language function,
Rapid progression
Conference:
Academy of Aphasia 57th Annual Meeting, Macau, Macao, SAR China, 27 Oct - 29 Oct, 2019.
Presentation Type:
Poster presentation
Topic:
Not eligible for student award
Citation:
TANAKA
Y,
Albert
M,
Cahana-Amitay
D,
Sawaki
M,
Nakamura
T,
Noda
E,
Kato
Y and
Tanaka
M
(2019). An unusual case of rapidly progressive aphasia with mild dementia.
Front. Hum. Neurosci.
Conference Abstract:
Academy of Aphasia 57th Annual Meeting.
doi: 10.3389/conf.fnhum.2019.01.00015
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Received:
24 Apr 2019;
Published Online:
09 Oct 2019.
*
Correspondence:
MD, PhD. YUTAKA TANAKA, Tanaka Clinic, Ikoma-Gun, Japan, EZZ05540@nifty.com