Abstract Details

Title Bilateral Alien Limb and the Spectrum of Creutzfeldt-Jakob Disease Phenomenology: A Case Series and Review of the Literature.

Topic Movement Disorders

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 263

Objective

To report a rare presentation of a rare disease and illustrate the broad phenomenology seen in Creutzfeldt-Jakob Disease (CJD).

Background CJD is caused by prion protein pathology of the central nervous system resulting in rapidly progressive dementia, disability, and death with a mean survival time of 6 months. Most often associated with myoclonus, the spectrum of phenomenology on presentation encompasses the scope of hypokinetic and hyperkinetic movement disorders. CJD has been associated with ataxia, parkinsonism, choreoathetosis, dystonia, tremor, and other movement disorders. Corticobasal syndrome in CJD is well-described. Alien limb phenomenon (ALP), a purposeless and involuntary movement of a limb that seems to move of its own volition, is a rare feature of this presentation. All 32 cases of ALP in CJD reported in the medical literature to date are of unilateral involvement.

Design/Methods To describe 2 cases of CJD: one with video examination of bilateral alien limb phenomenon, the other with generalized ataxia.

Results Patient 1 presented with acute onset of right hemiparesis followed by development of bilateral alien limb phenomenon and rapid deterioration of cognition and gait. The patient was misdiagnosed with bilateral parietal strokes at 2 independent centers. Brain MRI showed diffusion restricting cortical ribboning in the cerebral hemispheres that did not follow a vascular territory. Patient 2 had subacute onset of parkinsonism and acute onset of psychosis with debilitating axial and appendicular ataxia. Brain MRI showed diffusion restriction and T2 FLAIR hyperintensity abnormalities in bilateral caudate, medial thalami, and cerebellar hemispheres. These findings are consistent with known presentations and imaging patterns seen in CJD.

Conclusions These two cases demonstrate the diversity of movement disorder phenomenology and imaging abnormalities associated with CJD. We present the first case of bilateral alien limb phenomenon reported in CJD and highlight the importance of a high clinical suspicion for CJD as a stroke mimicker.

Authors/Disclosures
Dana A. Sugar, MD (University of New Mexico) PRESENTER	Dr. Sugar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abbvie.
Neepa J. Patel, MD, FAAN (Rush University Medical Center)	Dr. Patel has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abbvie Pharmaceuticals. Dr. Patel has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Amneal Pharmaceuticals. Dr. Patel has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Supernius Pharmaceuticals. Dr. Patel has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Boston Scientific.

��ɫ��

��ɫ��