The Alien Limb Phenomenon & Creutzfeldt-Jakob Disease (P03.257)

Abstract

Objective: To report clinical features of patients with alien limb phenomenon (ALP) associated with Creutzfeldt-Jakob disease (CJD). Background CJD is a rare prion disease that destroys cortical and subcortical neurons. The classic clinical signs of CJD are rapidly progressing dementia, ataxia, and myoclonus. Although ALP has been described in a few patients with CJD, there are no large case series assessing ALP caused by CJD. The neuroanatomic correlate of ALP in CJD is unknown. Design/Methods: Using the Mayo Clinic Medical Records Linkage system and the authors9 personal case series, we identified all patients evaluated at our institution between 1/1/1996-7/28/2011 with concomitant ALP and CJD. Only patients meeting strict clinical criteria for ALP and CJD were included. Results: We identified 13 patients, including six women. The median age at onset of CJD symptoms was 69 years (range 39-73). Two patients were classified as possible CJD, five as probable CJD, and six as definite CJD (with pathologic confirmation). The median time to onset of ALP symptoms was 0.5 weeks (range 0-16 weeks). The ALP afflicted the left side in nine patients and only one patient in that group was left hand dominant. The median time to death from CJD symptom onset was 10.5 weeks (range 8-64 weeks). In four patients ALP was the sole presenting neurologic sign. In four others it was the initial manifestation coexisting with other deficits. ALP prompted three other patients to seek initial evaluation. ALP was either initially or eventually accompanied by ipsilateral hemineglect, ideomotor apraxia and/or cortical sensory loss in nine patients. The contralateral parietal lobe demonstrated T2 hyperintensity and/or restricted diffusion in all eight patients who had abnormal MRI neuroimaging. Conclusions: ALP can be a feature of CJD. Isolated or early ALP should trigger suspicion of CJD. The neuroanatomic correlate of ALP in CJD appears to be the contralateral parietal lobe. Disclosure: Dr. Rubin has nothing to disclose. Dr. Graff-Radford has nothing to disclose. Dr. Boeve has nothing to disclose. Dr. Josephs has nothing to disclose. Dr. Aksamit has nothing to disclose.