During his motor examination, he evidenced symmetric bradykinesia and distal myoclonus. Video S1: The video shows the patient’s examination where he exhibits hypophonia and hypomimia. Patient 3 ( c and f) showed mild diffuse atrophy with minimal periventricular and subcortical white matter disease Patient 2 ( b and e) showed similar findings with somewhat lower burden of associated periventricular and spotty subcortical white matter increased signal. Patient 1 ( a and d) showed mild to moderate atrophy in the frontotemporal regions, minimally asymmetric, with right frontal encephalomalacia. Sagittal T2-weighted ( upper row) and axial FLAIR (lower row) brain MRIs. His condition deteriorated further and he died 7 years after the onset of his symptoms.īrain MRI of Case #1–3. Unfortunately, he later suffered multiple strokes, severely affecting his cognition and ambulation, rendering him wheelchair bound.
His cognition continued to deteriorate despite a trial of rivastigmine. Clonazepam 0.5 mg daily and venlafaxine 75 mg daily were sequentially added with mild benefit on sleep and depressive symptoms, respectively.
Levodopa was titrated to a dose of 1600 mg/day with mild improvement in gait speed but also with development of mild truncal dyskinesia. A diagnosis of possible bvFTD with parkinsonism was made based on apathy, poor personal hygiene, and predominantly executive dysfunction on cognitive testing. Patient and family declined amyloid-imaging and cerebral spine fluid (CSF) biomarkers. His brain MRI revealed atrophy in the frontal, temporal, and parietal regions, somewhat worse in the right hemisphere, with increased signal in the periventricular white matter and right frontal lobe (Fig. Frontal assessment battery (FAB) was 15/18. Montreal cognitive assessment (MoCA) score was 21/30 with deficits in executive function, phonemic fluency, and attention. In addition, he demonstrated bilateral ideomotor apraxia to transitive gestures. Unified Parkinson’s Disease Rating Scale (UPDRS) motor score was 23.5 on initial examination. His gait was slow and short-stepped, with stooped posture, reduced arm swing, gait freezing on turns, and inability to tandem walk (Additional file 1: Video S1). He exhibited symmetric rigidity and bradykinesia and hand myoclonus on outstretched arms. On exam, he was hypophonic and hypomimic. His family history was significant for AD in his mother and Parkinson disease (PD) in two paternal cousins. In the following months, his family noted a change in personality with apathy and lack of concern for his appearance.
He was forgetful and his gait shuffling and unstable. A 72-year-old man had progressive decline in gait and cognition following a fall 10 months earlier.
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