Case 989--A 77-Year-Old Man with Parkinsonism and Rapidly Progressive Dementia

Contributed by Henrique Soares Dutra Oliveira1, Pedro Sudbrack de Oliveira1, Victor Calil1, Philippe Joaquim Oliveira Menezes MacÍdo1, Nathalie Canedo2, Luiz Felipe Rocha Vasconcellos1.
1Institute of Neurology, Federal University of Rio de Janeiro. Brazil, 2Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro. Brazil.


A 77-year-old man presented with a three-year history of asymmetric rest tremor in the upper limbs associated with bradykinesia, early gait instability with frequent falls, dysphagia and cognitive impairment. He had both short and long-term memory loss associated with marked personality changes, with fluctuating cognition. There were no seizures or other parkinsonism-related symptoms, such as hyposmia, constipation and sleep disturbances. Past medical history was significant for depression, anxiety and hypertension, for which he took imipramine, clonazepam and hydrochlorothiazide. On examination he presented facial hypomimia, limitation on upward gaze, hypophonia and asymmetrical rigid-akinetic parkinsonism. He scored 26 out of 30 points in the Mini-Mental State Examination. There was no evidence of orthostatic hypotension or pyramidal tract dysfunction. Motor abnormalities were worse on left side and did not respond to levodopa therapy. Because of medical history and physical examination, we suspected atypical parkinsonism and requested a brain MRI, which showed a diffuse lesion with focal ring-enhancing nodule (Figs. 1a, 1b, 1c and 1d). An open brain biopsy was performed due to clinical and radiological findings.


H&E stained sections of the biopsy showed white matter with increased numbers of atypical cells (Fig. 1e). Immunostains for both p53 and IDH1-R132H are negative. A Ki67 immunostain was positive (Fig. 1f). What is your diagnosis?


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