Unusual Movement Disorders (Part 4): NBIA, Wilson Disease, Fahr Disease, ALS and Other Neurodegenerative Disorders
Video 14.86 (C14c86): PKAN with Parkinsonism and Dystonia
Risor grin, dystonia, and mild parkinsonism with preserved gait.
Video 14.87 (C14c87): Classic PKAN (Eye-of-the-Tiger Sign)
Generalized dystonia, retrocollis, mild parkinsonism, and characteristic MRI findings.
Video 14.88 (C14c88): Advanced PKAN
Severe dystonia, retrocollis, opisthotonus, and extreme writing impairment.
Video 14.89 (C14c89): Childhood PKAN
Risor grin, upper-limb dystonia, and preserved ambulation.
Video 14.90 (C14c90): Progressive PKAN
Evolution from mild parkinsonism to severe dystonia and dysphagia.
Video 14.91 (C14c91): Dystonic Storm in PKAN
Severe retrocollis, opisthotonus, jaw dystonia, and limb posturing.
Video 14.92 (C14c92): Mild Adult PKAN
Subtle parkinsonism with writing-induced tremor and eye-of-the-tiger MRI sign.
Video 14.93 (C14c93): Advanced PKAN with Postural Instability
Parkinsonism, dystonia, retropulsion, and prominent risor grin.
Video 14.94 (C14c94): PLA2G6 Neurodegeneration Imaging
Cerebellar atrophy in PLA2G6-associated neurodegeneration.
Video 14.95 (C14c95): BPAN with Parkinsonism
Atremulous parkinsonism and slow spastic gait.
Video 14.96 (C14c96): FAHN Syndrome
Parkinsonism, spasticity, dysarthria, and cerebellar atrophy with iron deposition.
Video 14.97 (C14c97): Wilson Disease with Intention Tremor
Low-frequency kinetic tremor and visible Kayser-Fleischer rings.
Video 14.98 (C14c98): Wilson Disease with Parkinsonism
Postural tremor, mild parkinsonism, and giant panda sign on MRI.
Video 14.99 (C14c99): Wilson Disease with Dystonia and Parkinsonism
Asymmetric dystonia, risor grin, KF rings, and improvement after copper chelation.
Video 14.100 (C14c100): Wilson Disease with Wing-Beating Tremor
Mixed parkinsonian and cerebellar tremor with classic wing-beating tremor.
Video 14.101 (C14c101): Wilson Disease with Generalized Dystonia
Cervical and limb dystonia, hypophonia, and residual KF ring changes.
Video 14.102 (C14c102): Progressive Wilson Disease
Worsening dystonia and parkinsonism related to poor treatment compliance.
Video 14.103 (C14c103): Severe Wilson Disease
Anarthria, dystonia, parkinsonism, spasticity, and marked postural instability.
Video 14.104 (C14c104): Wilson Disease with Leg Tremor
Mild dystonia, risor grin, and irregular resting leg tremor.
Video 14.105 (C14c105): Fahr Disease with Minimal Symptoms
Mild reduction in arm swing despite extensive basal ganglia calcification.
Video 14.106 (C14c106): Fahr Disease with Mild Chorea
Subtle chorea in the setting of widespread basal ganglia calcifications.
Video 14.107 (C14c107): Fahr Disease with Generalized Chorea
Mild chorea accentuated by cognitive activation.
Video 14.108 (C14c108): Fahr Disease with Oculomotor Apraxia
Oculomotor apraxia, mild chorea, and extensive cerebral calcifications.
Video 14.109 (C14c109): Fahr Disease with Orobuccal Dyskinesia
Mild oral dyskinesias and intermittent upper-limb chorea.
Video 14.110 (C14c110): Fahr Disease with Generalized Dystonia
Blepharospasm, torticollis, jaw dystonia, and writer's cramp.
Video 14.111 (C14c111): Fahr Disease with Freezing of Gait
Parkinsonism, ideomotor apraxia, and severe cue-responsive freezing.
Video 14.112 (C14c112): Bulbar Myasthenia Gravis
Fatigable dysarthria and dysphagia with worsening speech over time.
Video 14.113 (C14c113): Bulbar ALS
Dysarthria, breathy voice, tongue fasciculations, and preserved gait.
Video 14.114 (C14c114): Progressive Bulbar ALS
Hypophonic dysarthria with increasing respiratory effort.
Video 14.115 (C14c115): ALS with Tongue Fasciculations
Hypophonia, diplophonia, and fasciculating tongue without significant atrophy.
Video 14.116 (C14c116): ALS with Upper and Lower Motor Neuron Signs
Hand muscle wasting, hyperreflexia, clonus, and gait impairment.
Video 14.117 (C14c117): Primary Lateral Sclerosis (PLS)
Spastic dysarthria, pseudoparkinsonian slowness, hyperreflexia, and spastic gait.
Video 14.118 (C14c118): Advanced PLS
Spastic speech, emotional incontinence, hyperreflexia, and severe gait impairment.
Video 14.119 (C14c119): Creutzfeldt-Jakob Disease with Myoclonus
Positive and negative myoclonus causing reverse asterixis.
Video 14.120 (C14c120): Creutzfeldt-Jakob Disease with Parkinsonism
Rapidly progressive parkinsonism with freezing, festination, and stereotyped hand movements.
Video 14.121 (C14c121): Hereditary Spastic Paraplegia (HSP)
Spastic gait, ankle clonus, and diffuse hyperreflexia.
Video 14.122 (C14c122): Complicated HSP
Bradykinesia, cerebellar signs, and marked pyramidal findings.
Video 14.123 (C14c123): HSP with Sensory Ataxia
Spastic gait, impaired vibration sense, and cerebellar abnormalities.
Video 14.124 (C14c124): SPG7-Associated Hereditary Spastic Paraplegia
Spastic ataxic gait, cerebellar signs, and confirmed SPG7 mutation.