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- W2488784805 abstract "Objective: To describe a case of Creutzfeldt-Jakob disease (CJD) presenting with bilateral alien hand syndrome.Background: CJD is a rapidly progressive neurologic disorder that commonly presents with dementia. While unilateral alien limb phenomenon (ALP) is known to be a rare manifestation of CJD, this the first case report of bilateral alien hand syndrome as the presenting symptom.Methods: Case reportResults: A 71-year-old right-handed female presented to the hospital with progressive neurologic decline and respiratory distress. Three months prior, she had developed involuntary movements of her left hand. She reported feeling as if it was not under her control, and would constantly grab it and hold it down with the right. At times she would not even recognize it as her own and eventually lost all control of the arm. Shortly afterwards, she developed similar symptoms in the right hand followed by vertigo, frequent falls and severe cognitive impairment. On examination, the patient was non-verbal and did not follow commands. A right-sided gaze preference was present along with diffuse hyperreflexia and hypertonia. Throughout the examination, the patient’s arms were constantly reaching out into the air with occasional semi-purposeful movements alternating with bizarre posturing. Evaluation included MRI brain, which revealed restricted diffusion changes in the basal ganglia, caudate and thalami bilaterally with asymmetric cortical ribboning in the right parietal region. A periodic pattern on EEG and increased concentration of CSF 14-3-3 protein further supported the diagnosis of CJD. The patient’s respiratory distress worsened and she expired one week after admission.Conclusion: ALP in the setting of CJD has been reported to most commonly involve the left hand, which may reflect a right hemispheric neglect-like syndrome. The reason for the bilateral hand involvement in our case is unclear but may represent a new variant of ALP in CJD. Disclosure: Dr. Michael has nothing to disclose. Dr. Tavee has nothing to disclose." @default.
- W2488784805 created "2016-08-23" @default.
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- W2488784805 date "2016-04-05" @default.
- W2488784805 modified "2023-09-24" @default.
- W2488784805 title "Creutzfeldt-Jakob Disease Presenting with Bilateral Alien Hand Syndrome (P4.055)" @default.
- W2488784805 hasPublicationYear "2016" @default.
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