Video 1: Corticobasal degeneration with myoclonus. Right upper limb dystonia with shoulder abduction, and elbow + wrist + fingers flexion, associated with myoclonic jerks (at rest and triggered by posture and action).
PolygraphicEMG recordings show brief and synchronous bursts on the upper arm muscles. (l.mpg) Video 2: Corticobasal degeneration with apraxia. Tremor, akinesia, myoclonic jerks and left hand dystonia. Symbolic motor sequences are not properly performed: cross sign is performed from right to left shoulder, thumbing one's nose is replaced by a kiss, showing that someone is a fool is done uncompletely and cross sign is not possible with left hand. (2.mpg)
Video 3: Multiple system atrophy (MSA-P). At a early stage, DOPA-resistant akineto-rigid syndrome is associated with inability to walk without aid and postural instability. (3.mpg)
Video 4: Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Vertical voluntary saccades are slow and with limitation of the maplitude of the saccades. Horizontal voluntary saccades are reduced and several ocular movements (several small saccades) are needed to move the eyes from side to another. Oculocephalic reflexes (VOR) are normal, demonstrating the supranuclear location of the dysfunction. Postural instability is associated. (4.mpg)
Video 5: Abnormal eye movements in Progressive supranuclear palsy. Vertical and horizontal volontary saccades are reduced. Several saccades are needed to reach the target. Presevations are observed, the patient is looking at the previous target while asked to look in another direction. Frontalis muscle contractions are associated with upward saccades.
Oculocephalic reflexes are normal (but difficult to perform because of distractibility and cervical stiffness), demonstrating the supranuclear location of the dysfunction. (5.mpg)
Video 6: Frontal behavior in Progressive supranuclear palsy. As described by Lhermitte, frontal behavioral signs include grasping reflex (even when forbidden), utilization behavior and collection of the examinator's personal stuff without order, imitation behavior with imitation of all the mimics and gestural activities of the examinator, including nonsense as eating a metallic object. (6.mpg)
Video 7: Postural instability in Progressive supranuclear palsy. Spontaneous postural instability. A gentle pulling test marked the patent fall, without any attempt or ability to recover his balance (loss of postural reflexes). (7.mpg)
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