1. ID 322 – Familial Creutzfeldt-Jakob disease. All night v-polysomnography study.
- Author
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Maeztu, C., López-Bernabé, R., Candel, J., Salmerón, P., and Gómez, C.
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CREUTZFELDT-Jakob disease , *POLYSOMNOGRAPHY , *PRION diseases , *GENETIC mutation , *ELECTROENCEPHALOGRAPHY - Abstract
Introduction The familial form of Creutzfeldt-Jakob disease (fCJD) is a dominantly inherited prion disease caused by mutations in the prion protein gene. We describe the sleep structure assessed by night v-polysomnography in a patient with Glu200Lys mutation. Patient A 53 y/o bus driver man came to hospital because of loss of memory. Seven months later disartria appeared, followed by ataxia and generalized myoclonus; the illness progressed to stupor and unresponsiveness and finally death after ten months. Results The first EEG showed a continuous focal right parietal epileptiform activity, with normal background activity. Two months later, a pattern of generalized synchronous periodic sharp waves appeared in the awake state. An all night video-polysomnography showed an abnormal sleep structure with no recognition of asleep stages; there were no vertex sharp waves nor spindles or K complex; with no REM sleep during awakenings the background activity was a diffuse low amplitude theta-delta activity, with generalized periodic bi-triphasic sharp waves. Abnormal respiratory events, were present with an apnea-hypopnea index of 79. Conclusion Night polysomnography in fCJD shows a very abnormal sleep structure, with a significant decrease in total sleep time, sleep efficiency, fragmentation, loss of normal sleep graphoelements, loss of REM sleep and periodic breathing with abnormal arousals. [ABSTRACT FROM AUTHOR]
- Published
- 2016
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