Paradoxical Myotonia in Paramyotonia Congenita

Video Author: Richard J. Barohn, MD, FAAN
Published on: 12.31.2013
Associated with: CONTINUUM: Lifelong Learning in Neurology. 19(6, Muscle Disease):1598-1614, December 2013

Video demonstrates the characteristic paradoxical increase in myotonia with repetitive hand grips, or paramyotonia, seen in patients with paramyotonia congenita. The patient is instructed to squeeze her hand closed as tightly as she can and then open her hand quickly. This maneuver is repeated to evaluate forwarm-up of myotonia or paradoxical worsening.

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Creator: Marcel Hungs, MD, PhD
Duration: 0:24
Video shows a 56-year-old woman with psychogenic movement of both hands at bedtime. She is alert and has no urge to move her hands. The movements interfere with her sleep onset, disappear in sleep, and reoccur upon awakening. The movements are at times also seen during the day in wakefulness.
Creator: Soňa Nevšímalová, MD, DSc
Duration: 0:44
Video demonstrates confusional arousal in an adult man. The patient has an arousal, appears confused, and gets out of bed, demonstrating automatic behavior. This is an example of a hybrid attack in which the patient begins the episode with a confusional arousal and proceeds for exhibit somnambulistic behavior.
Creator: Rama Maganti, MD
Duration: 1:54
Video demonstrates confusional arousal in an adult man, demarcated by sudden arousal, confusion, searching behavior, and rapid return to baseline with amnesia for the event when conversing with the technologist.
Creator: Rama Maganti, MD
Duration: 0:56
Video demonstrates sleep terror in an adult woman. She screams suddenly, beginning from slow-wave non-REM sleep. The video segment after the event illustrates conversation with the technologist in which the patient recalls being awakened, but has little recollection for the event, and returns to baseline fairly quickly.
Creator: Soňa Nevšímalová, MD, DSc
Duration: 1:30
Video demonstrates an episode of sleep terror in a child that consists of sudden arousal, increase in sympathetic tone, confusion, aggressive behavior, inconsolability, and increased aggression.
Creator: Geert Mayer, MD, PhD
Duration: 0:37
Video demonstrates REM sleep behavior disorder in an adult man. Note the purposeful body movements correlating with dream enactment against electrographic augmentation of EMG tone.
Creator: Geert Mayer, MD, PhD
Duration: 1:54
Video demonstrates aggressive behavior in patients with REM sleep behavior disorder necessitating prompt safety modifications and pharmacologic interventions
Creator: Soňa Nevšímalová, MD, DSc
Duration: 0:34
Video demonstrates the often-stereotyped rhythmic movement of body rocking in a child. Body rocking tends to have a frequency of 1 Hz to 3 Hz and creates noise that sometimes awakens family members or bed partners.
Creator: Rama Maganti, MD
Duration: 0:24
Video demonstrates head banging in a child. Head banging consists of stereotyped and repetitive rhythmic movements of the head that occur during wake-to-sleep transitions and wakefulness.
Creator: Geert Mayer, MD, PhD
Duration: 0:24
Video demonstrates body rocking in an adult leading to 1-Hz to 2-Hz movement artifact in the polysomnogram. Body rocking is a sleep-related rhythmic movement disorder that interferes with normal sleep and can result in self-inflicted bodily injury.
Creator: Soňa Nevšímalová, MD, DSc
Duration: 0:24
Video demonstrates rhythmic stereotyped body rocking in a child. Rhythmic movement disorders usually begin in the first year of life and spontaneously remit by 4 years of age.
Creator: Reproduced with permission from Fife TD, Iverson DJ, Lempert T, et al. Practice parameter: therapies for benign paroxysmal positional vertigo (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology.
Duration: 0:46
Video demonstrates canalith repositioning treatment for right posterior canal benign paroxysmal positional vertigo (BPPV). The mirror image maneuver can be done for left posterior canal BPPV.
Creator: Reproduced with permission from Fife TD, Iverson DJ, Lempert T, et al. Practice parameter: therapies for benign paroxysmal positional vertigo (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology.
Duration: 0:17
Video demonstrates the Dix-Hallpike maneuver to the right. This maneuver should evoke paroxysmal positional nystagmus for right-sided benign paroxysmal positional vertigo related to the posterior semicircular canal. The mirror image of this maneuver constitutes a left-sided Dix-Hallpike maneuver.
Creator: Kevin A. Kerber, MD, MS
Duration: 0:10
The cross-cover test is performed with the patient focusing on an object in the distance. No vertical misalignment is observed, so the test is negative for skew.
Creator: Kevin A. Kerber, MD, MS
Duration: 0:34
Spontaneous nystagmus and gaze testing in a patient who presented with acute vestibular syndrome and findings that localize to the right vestibular nerve. The patient has spontaneous left-beat nystagmus. The velocity of the nystagmus increases when he looks to the left and decreases when he looks to the right. When he looks to the right, the nystagmus does not change direction. When he looks up, the nystagmus remains left-beat. Thus, the patient has a unidirectional horizontal nystagmus.
Creator: Reproduced with permission from Weber KP, Aw ST, Todd MJ, et al. Head impulse tests in unilateral vestibular loss: vestibulo-ocular reflex and catch-saccades. Neurology 2008;70(6): 454Y463.
Duration: 1:24
Video demonstrates overt versus covert corrective saccades. In some patients, corrective saccades are easily visualized by the examiner (ie, overt saccades) whereas in other patients they may be difficult to observe or may even be imperceptible to the examiner (ie, covert saccades). This video shows examples of overt and covert corrective saccades after the head-impulse test in two patients with unilateral vestibular loss.
Creator: Reproduced with permission from Weber KP, Aw ST, Todd MJ, et al. Head impulse tests in unilateral vestibular loss: vestibulo-ocular reflex and catch-saccades. Neurology 2008;70(6):454Y463.
Duration: 1:08
Video demonstrates a head-impulse test with scaled peak head velocities of 100-/s (A), 200-/s (B), and 300-/s (C) in a patient with left vestibular loss. Note the increasing salience of the catch-up saccades with stimulus size. The picture-in picture shows the online feedback trace (500 milliseconds) of angular head velocity (-/s) for the examiner. Head velocity was measured with a search coil mounted on a dental impression plate.