Agrypnia excitata

Clinic

• AE is a syndrome characterized by loss of sleep and permanent motor and autonomic hyperactivation.
• Disruption of the sleep-wake rhythm consists in the disappearance of spindle-delta activities, and the persistence of stage 1 NREM sleep.
• REM sleep persists but fails to stabilize, appearing in short recurrent episodes, isolated, or mixed with stage 1 NREM sleep.
• Diurnal and nocturnal motor, autonomic and hormonal overactivity is the second hallmark of AE.
• Of particular interest is the finding that norepinephrine secretion is extremely elevated at all hours of the day and night whereas the nocturnal melatonin peak is lacking.
• Oneiric stupor is probably an exclusive sign of AE and consists in the recurrence of stereotyped gestures mimicking simple daily life activities.

Related disease

• FFI
• Morvan syndrome
• Delirium tremens
• REM Related parasomnias

Pathophysiology

• Agrypnia excitata is due to an intra-limbic disconnection releasing the hypothalamus and brain-stem reticular formation from cortico-limbic inhibitory control.
• This pathogenetic mechanism is visceral thalamus degeneration in FI, whereas it may depend on autoantibodies blocking voltage-gated potassium (VGK) channels within the limbic system in Morvan syndrome, and in the sudden changes in gabaergic synapses down-regulated by chronic alcohol abuse within the limbic system in Delirium tremens.