Fragment 5.1

Parkinsonism (Segments 1-10) The clinical hallmarks of (motor) parkinsonism are shown in this video fragment. Segments 1-3 display bradykinesia, segment 4 hypokinesia, segment 5 the typical parkinsonian rest tremor, and segment 6 rigidity with the cogwheel phenomenon in various patients suffering from motor parkinsonism. Segment 7 shows the loss of postural reflexes with instability, the phenomena of propulsion (segment 8) and retropulsion (segment 9), and stooped posture (segment 10) in motor parkinsonism (courtesy Erik Wolters).

Fragment 5.2

Parkinson’s disease (Segments 1-5) This fragment shows a 6o-year-old woman suffering from right > left-sided Parkinson’s disease, with hypokinesia (masked face: segment 1), reduced arm swing (segment 2), tremor (segment 3), and bradykinesia in the right body-half (segments 4 and 5) (courtesy Teus van Laar).

Fragment 9.1

REM sleep behaviour disorder (1) Nocturnal video-polysomnography of a 78-yearold male patient with idiopathic Parkinson’s disease and REM sleep behaviour disorder (RBD). In this patient with severe comorbid depression, RBD manifests with expression of joy and laughter (courtesy Christian Baumann).

Fragment 9.2

REM sleep behaviour disorder (2) Nocturnal video-polysomnography of a 71-yearold female patient with idiopathic Parkinson’s disease. On the left side, sleep electro-encephalogram (bottom), electro-oculogram (top) and electro- myogram are visible. The patient is in rapid eye movement (REM) sleep. REM sleep behaviour in this patient manifests primarily with expression of fear and screaming, but not with motor activity (courtesy Christian Baumann).

Fragment 11.1

Allied health strategies in PD (Segments 1-2) Patients suffering Parkinson’s disease with progressive instability and/or freezings, limiting their activities of daily living, might be helped by external cues. Two examples are given: the first segment shows an elderly man, passing sliding doors without and later with acoustic cues (rhythmic ticks with his cane). The second segment shows the impact of visual cues on a late-stage PD patient with severe walking problems in the home situation (with lines on the floor) (courtesy Erik Wolters).

Fragment 12.1

Motor fluctuations in PD (Segments 1-4) Earlier or later, PD patients will develop levodopa- induced and to a lesser extent dopamine agonists-induced complications, such as hyperkinesias (segment 1), off-related dystonia (segment 2), unpredictable ‘on-off’ fluctuations (segment The patients displayed in this video-supplement have given their permission for use for personal medical educational purposes only, not for showing these videos in public. Therefore, the authors and editors of this book do not take any responsibility for legal claims resulting from actions not in accordance with this restricted permission. 3) and/or diphasic dyskinesias (segment 4), which were induced in this patient by threshold concentrations of L-dopa, related to a discontinuous medication schedule (courtesy Erik Wolters).

Fragment 12.2

Peak-dose chorea Videofragment showing typical levodopa-induced, peak-dose, choreatic hyperkinesia (courtesy Erik Wolters).

Fragment 12.3

Dopamine dysregulation syndrome In this video, note the disabling clinical expression of the consequences of a disorder associated with the abuse (overuse) of levodopa in a young PD patient (dopamine dysregulation syndrome) (courtesy Erik Wolters).

Fragment 14.1

CDS-LCIG (1) (segments 1-4). This video shows a 34-year-old man, suffering since the age of 24 from right-sided, subtle motor parkinsonism, diagnosed three years later as idiopathic PD, and treated with levodopa (segment 1: off-medication ). After 1.5 years of treatment, he developed motor fluctuations and dyskinesias, necessitating continuous dopaminergic stimulation; two years later, the effects of the medication became unpredictable, with alternating, short-lived ‘on’ with dyskinesias (UPDRS III: 15) and akinetic ‘off’ (UPDRS III: 40) periods (segment 2: ‘on’ with dyskinesias). In segments 3 and 4 the effects of LCIG (levodopa-carbidopa intestinal gel) 700 mg/ day (without bothersome dyskinesia) are evident 6 months and 3 years, respectively, after application (courtesy Angelo Antonini).

Fragment 14.2

CDS-CLIG (2) (segments 1-2) Here you see a young man suffering from idiopathic PD since the age of 11, treated with PEG-J-applied continuous (levodopa-carbidopa intestinal gel) CLIG before (segment 1) and after (segment 2) discontinuation of this application (courtesy Erik Wolters).

Fragment 17.1

Vascular parkinsonism Typical walking pattern of a hypertensive man, with small steps, difficulty with turning, freezing episodes, and a well preserved arm swing (lower body-half parkinsonism) (courtesy Erik Wolters).

Fragment 18.1

Ephedrine-induced parkinsonism (Segments 1-3) This videotape displays the clinical presentation of an ephedrine-induced encephalopathy manifesting with bradykinesia and hypokinesia (segment 1), the typical cock gait (segment 2) and the specific dysarthria (segment 3) (courtesy Andrzej Friedmann).

Fragment 20.1

Progressive supranuclear palsy (1) (segments 1-5) Patient suffering PSP with typical, vertical, supranuclear, downward gaze paralysis (segment 1), bradykinesia (segment 2), pseudobulbar speech (segment 3), micrography (segment 4) and postural instability with falls (segment 5) (courtesy Andrew Lees).

Fragment 20.2

Progressive supranuclear palsy (2) (segments 1-5) 71-year-old patient with progressive supranuclear palsy. Segment 1 shows the patient’s hypophonia, segment 2 the bilateral bradykinesia. In segment 3 and 4, the patient’s postural instability and bradykinetic gait might be appreciated. Typical slow vertical saccades are shown in segment 5 (courtesy Christian Baumann).

Fragment 22.1

DYT1 dystonia Young man with a DYT1 mutation (early-onset torsion dystonia), showing a generalized torsion dystonia (courtesy Erik Wolters).

Fragment 22.2 / 22.2.2

Secondary dystonia (Segments 1-2) The first patient suffers from a pantothenate kinase-associated neurodegeneration (PKAN), formerly called Hallervorden-Spatz disease (segment 1). The second patient suffers from the X-linked dystonia-deafness syndrome (Mohr-Tranebjaerg syndrome) (segment 2) (courtesy Erik Wolters).

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Focal/segmental dystonia (Segments1-4) The first patient shows mainly focal blepharospasms with a slight dystonia of the perioral muscles (segment 1), the second patient suffers from a severe, segmental Meige syndrome with overflow to the trapezius muscles (segment 2), the third patient suffers from a focal torticollis spasmodica (segment 3), and the fourth patient from a focal spasmodic dysphonia. This last segment shows this patient’s initially normal breathing, followed by supraglottic hyper-adduction during phonation, resulting in a typical dysphonic speech. The final part of this segment evidences the significant improvement after injection with botulinum toxin in the thyro-arythenoid muscles, producing reduced supraglottic adduction (segment 4) (courtesy Martin Horstink).

Fragment 23.1

Wilson’s disease (Segments 1-6) Mild signs of parkinsonism can be appreciated in this video fragment of a young woman suffering Wilson’s disease: hypokinesia (segment 1), resting tremor (segment 2), intention tremor (segment 3), bradykinesia (segment 4), instability and loss of postural reflexes with retropulsion (segment 5) as well as the typical corneal Kayser-Fleischer rings (segment 6) (courtesy Erik Wolters).

Fragment 26.1

Essential tremor Here you might appreciate the clinical expression of a patient suffering a mild, (asymmetric postural and accompanying action) tremor of both hands (courtesy Erik Wolters).

Fragment 26.2

Parkinson tremor Unilateral resting tremor with a shortlasting suppression of the tremor amplitude after positioning of the hands (courtesy Erik Wolters).

Fragment 26.3

Enhanced physiological tremor This patient shows a distal, high-frequency tremor with small amplitude, especially in the left hand/ thumb (courtesy Erik Wolters).

Fragment 26.4

Tremor associated with dystonia (TAWD) Young woman suffering from cervical dystonia and torticollis, with an irregular, jerky, postural tremor of the head with a directional preponderance, using a sensory trick to reduce the tremor amplitude (courtesy Erik Wolters).

Fragment 26.5

Cerebellar tremor Woman diagnosed with MS, showing titubation of the head, cerebellar ataxia, and an intention tremor of both hands (courtesy Erik Wolters).

Fragment 26.6

Task-specific tremor Patient with a task-specific tremor of the right hand, only when writing (primary writing tremor) (courtesy Erik Wolters).

Fragment 26.7

Orthostatic tremor Patient without tremor in the sitting position, showing continuous movements of both legs, starting directly after rising. EMG showed a high-frequency tremor (17 Hz) in the standing position, which disappeared immediately upon sitting (courtesy Erik Wolters).

Fragment 26.8

Midbrain (‘Holmes’ or ‘rubral’ tremor) Young man with a posttraumatic midbrain tremor, consisting of a low-frequency position, rest and intention tremor of his left arm (courtesy Erik Wolters).

Fragment 26.9

Essential palatal tremor Young-onset, rhythmic, low-frequency contractions of the tensor veli palatine resulting in elevations of the roof of the soft palate, with opening/ closing of the Eustachian tube and the symptom of ear-clicking (which, unfortunately, cannot be appreciated in this video) (courtesy Erik Wolters).

Fragment 27.1

Psychogenic tremor Inconsistent distal tremor of the right hand, with variable frequency and amplitude. This tremor originally had the characteristics of a position tremor, but later on, there were also aspects of a resting tremor. The tremor is suppressed by entrainment (courtesy Erik Wolters).

Fragment 27.2

Bilateral Vim-DBS in essential tremor PD (2) (segments 1-2) In this video you might see the significant effects of bilateral thalamic Vim-DBS in a 70 yr old lady with a disabling essential tremor before (segment 1) and after (segment 2) Vim-DBS (courtesy Christian Baumann).

Fragment 27.3

STN-DBS in tremor-dominant PD (1) In this video you might appreciate the quick response of rest tremor to STN-DBS macrostimulation during operative implantation of bilateral subthalamic electrodes in a 68-year-old patient with tremor-dominant idiopathic Parkinson’s disease (courtesy Christian Baumann).