Abstract Details

Title A Case of Rapidly Progressive Dementia: Differentiating Dementia with Lewy Body from Creutzfeldt-Jacob Disease

Topic Aging and Dementia

Presentation(s) P04 - (-)

Poster/Presentation
Number 214

Objective

Background BACKGROUND: Once diagnosed, DLB has a three to seven year survival. Classically, patients have parkinsonism and visual hallucinations. In contrast, CJD is a rapidly progressive dementia with succession to death in less than one year, caused by prion disease. Diagnostic differences include clinical observations, EEG, CSF, and MRI results.

Design/Methods DESIGN/METHODS: We present a literature review and case report of a 69 year old male patient with rapidly progressive dementia leading to institutionalization within two months and death within five months. He initially presented with two weeks of acute onset memory loss, generalized weakness, falls, and intermittent action tremor. Examination revealed memory difficulties, paratonia, rare myoclonic jerks, and intermittent high frequency postural tremor. Three weeks later, the patient had hallucinations, dysphonia, and dysphagia. In the following months, he responded to well to risperidone. His cognitive deficits fluctuated and myoclonus increased the week prior to death.

Results RESULTS: In this patient with sudden onset dementia, serum studies were unremarkable. CSF studies, including cultures and tau protein, were normal. MRI revealed increased FLAIR signal within the supratentorial gray matter and cerebellum, hippocampi and basal ganglia, though repeat MRI one month prior to death was normal. PET scan showed scattered decreased perfusion. Four EEGs were obtained over the course of his illness, only demonstrating variable degrees of slowing with occasional triphasic waves correlating to the degree of encephalopathy. No PLEDs were identified. A presumptive diagnosis of DLB was made. The patient died of cardiopulmonary complications. Brain pathology confirmed Lewy body disease.

Conclusions CONCLUSIONS: This case demonstrates the variable course of DLB. Differentiating between rapidly progressive dementias requires analysis of the sensitivities and specificities of a variety of neurologic tests.

Authors/Disclosures
Kate Daniello, MD, FAAN (University of Massachusetts) PRESENTER	Dr. Daniello has nothing to disclose.
Jay Pathmanathan, MD, PhD (Penn Neuroscience Center)	No disclosure on file
Noah Rosen, MD, FAAN	Dr. Rosen has received personal compensation for serving as an employee of Northwell Health. An immediate family member of Dr. Rosen has received personal compensation for serving as an employee of New York University. Dr. Rosen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Allergan/ Abbvie. Dr. Rosen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lundbeck. Dr. Rosen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Pfizer . Dr. Rosen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amneal. Dr. Rosen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Allergan/ Abbvie. Dr. Rosen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Pfizer. Dr. Rosen has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer.

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