Session Information
Date: Sunday, October 7, 2018
Session Title: Parkinsonism, MSA, PSP (Secondary and Parkinsonism-Plus)
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: We here reported argyrophilic grain disases (AGD) with clinical diagnosis of Parkinson dementia.
Background: AGD is a four-repeat tauopathy and its accumulation (argyrophilic grain (AG)) contributes to cognitive decline in the elderly. Saito et al (2002) proposed propagation paradigm of AG, starting from ambient gyrus (Stage I), spreading to temporal lobe (Stage II) and reaching frontal cortex (Stage III) to cause dementia. AGD also involved brain stem, including substantia nigra but its clinical significance is yet to be determined.We here reported AGD with clinical diagnosis of Parkinson dementia.
Methods: Case Report: A eighty year- old man first visited our hospital with gait disturbance. He first experienced the symptom at the age of 74 and the first fall just before the visit. At the age of 85, he had frequent falls backwards with gait freezing and admitted to our hospital. Neurological examination revealed gait freezing, postural instability, slight impaired upward gaze and bradykinesia. There was no tremor or rigidity. Brain MRI showed subtle volume loss of the midbrain and severe atrophy of amygdala predominant on the right side. An FDG PET scan showed decreased uptake in the bilateral cerebrum predominantly in the medial temporal region. CSF levels of amyloid beta, tau and phosphorylated tau were within normal range. DaT scan showed a decrease predominantly in dopamine uptake in the left putamen like dot shaped. He subsequently complained of dysphagia and was wheelchair- bound at the age of 85. He died of respiratory failure at the age of 85. The total clinical course was 11 years.
Results: Neuropathology: Macroscopially, there was marked atrophy of the amygdala preferentially on the right side. Histologically, abundant argyrophilic grains were present in the limbic system especially the amygdala, with ballooned neurons and bush-like astrocytes. Saito AGD stage was stage III. In addition, grains and 4-repeat tau positive astrocytes involved subthalamic nucleus, substantia nigra and tegmentum of the midbrain.
Conclusions: AGD occasionally presented parkinsonism and received clinical diagnosis of Lewy body dementia or progressive supranuclear palsy. AGD should be included in differential diagnosis of age- related dementia with parkinsonism.
To cite this abstract in AMA style:
R. Sengoku, D. Kaneda, M. Kameyama, A. Tokumaru, K. Ishii, K. Kanemaru, Y. Saito, S. Murayama. An atutopsy case of argyrophilic grain disease, clinically presenting with Parkinson dementia [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/an-atutopsy-case-of-argyrophilic-grain-disease-clinically-presenting-with-parkinson-dementia/. Accessed June 14, 2025.« Back to 2018 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/an-atutopsy-case-of-argyrophilic-grain-disease-clinically-presenting-with-parkinson-dementia/