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The three main signs of hyperekplexia are generalized stiffness, excessive startle response beginning at birth, and nocturnal myoclonus. [5] Affected individuals are fully conscious during episodes of stiffness, which consist of forced closure of the eyes and an extension of the extremities followed by a period of generalised stiffness and uncontrolled falling at times. [6]
The startle reflex is a brainstem reflectory reaction (reflex) that serves to protect vulnerable parts, such as the back of the neck (whole-body startle) and the eyes (eyeblink) and facilitates escape from sudden stimuli. It is found across many different species, throughout all stages of life.
Startle reflex; Sucking reflex — a primitive reflex, sucking at anything that touches the roof of an infant's mouth. Stretch reflex; Triceps reflex — jerking of the forearm when the triceps tendon is hit with a tendon hammer, stimulating the C7 and C6 reflex arcs.
People suffering from hypervigilance may become preoccupied with scanning their environment for possible threats. They might overreact to loud and unexpected noises, exhibit an overactive startle response or become agitated in highly crowded or noisy environments. They will often have a difficult time getting to sleep or staying asleep. [7]
A hypnic jerk, hypnagogic jerk, sleep start, sleep twitch, myoclonic jerk, or night start is a brief and sudden involuntary contraction of the muscles of the body which occurs when a person is beginning to fall asleep, often causing the person to jump and awaken suddenly for a moment.
Prepulse inhibition: preceding stimulus attenuates the startle response.. Prepulse inhibition (PPI) is a neurological phenomenon in which a weaker prestimulus (prepulse) inhibits the reaction of an organism to a subsequent strong reflex-eliciting stimulus (pulse), often using the startle reflex.
Reticular reflex myoclonus is thought to be a type of generalized epilepsy that originates in the brainstem, the part of the brain that connects to the spinal cord and controls vital functions such as breathing and heartbeat. Myoclonic jerks usually affect the whole body, with muscles on both sides of the body affected simultaneously.
Jumping Frenchmen of Maine syndrome must be distinguished from other conditions involving the startle reflex or tics. Tourette syndrome is characterized by multiple physical (motor) tics and at least one vocal (phonic) tic. There are many overlaps when compared clinically, but the abnormal "jumping" response is always provoked, unlike the ...