THE RHYTHMS OF AMBES (AROUSAL RELATED MOTOR BEHAVIORAL EPISODES) IN AGRYPNIA EXCITATA: A VIDEO MOTOR ANALYSIS

ARTURO GARAY; DANIEA GIARDINO; CRISTIÁN HUCK IRIART; SUSANA BLANCO; ANTHONY T. REDER

Vancouver

Congreso; World Sleep 2019; 2019

World Sleep Socienty

Introduction: ?Agrypnia Excitata? (AE) is a term coined originally by Lugaresi and Provini to describe a syndrome caused by a dysfunction in thalamo-limbic circuits producing severe insomnia, mental confusion, dream enactment, motor and autonomic activation. This syndrome is observed in fatal familial insomnia (FFI), l autoinmune encephalopathy and delirium tremens (Lugaresi E. and Provini F., Sleep Med. Rev. 2001). In patients with AE, oscillatory EEG rhythms have been observed during "pseudosleep", but also in REM sleep in patients with Agrypnia Excitata-Fatal Familial Insomnia (AE-FFI) (Garay A., Neurology 1994). Episodes of AMBEs with and without behavioral correlates constitute an intriguing finding in patients with AE (Antelmi E. et al., Sleep Med. Rev. 2016). Now, we attempt to characterize this oscillatory behavior observed in a proven case of FFI (Garay A., Neurology 1994; Reder A. et al, Neurology 1995) using motor video analysis techniques (VMA).Materials and methods: We analized raw data of polysomnograms (n=5) and in particular, a nocturnal video polysomnogram of a case of FFI (Garay A. et al. Neurology 1994, Reder A. et al. Neurology 1995). The video analysis of 8 hours of S-VHS tape recorded was done by computer programs using Python 2.7 scripting OpenCV 3.2.0, Numpy 1.13.1, Scipy 1.1.0 and Matplotlib 2.2.2 libraries. These programs were employed to register and analyze the movements of the head and legs as a function of time during four hours considered for analysis. Frame analysis (30fps) detect changes of integrated monochromatic intensity of a selected region of interest (ROI) normalized with the integrated intensity of a quiescent ROI as background. The fundamental frequencies were obtained by Fast Fourier Transform (FFT). Statistical analysis used the covariance matrix of data and a probability less than 0.05 was considered significant. Results: AE-FFI was characterized, according to standar polysomnograms, during AMBEs, with an intra atypical REM sleep fragmentation/?pseudosleep? cycling behavior with an increase of near one-minute centered bursts (p< 0.05). Segmental analysis using VMA obtained during AMBES showed significant peaks for head (H) and right or left legs ( Lr /Ll) movements, cycling in a range of 1.5 to 20 minutes with a low correlation between H and Lr /Ll (r covariance H/ Ll : 0.04; H/ Lr /Ll : 0.08). Conclusions: This case of AE-FFI demonstrates, a) a disconnection between cortical and sleep postural behavior, b) a disconnection of a central motor pattern generator (CMPG) that mediates the dream enacting behavior and c) a loss of rhythmicity of a central pattern motor generator. These functional correlates suggest a state of thalamo-limbic-brainstem disconnection which may have implication in this and other clinical settings.