hyperkinetic movement disorders: symposium co-sponsored by the parkinson study group and movement...

Hyperkinetic Movement Disorders: Symposium Co-Sponsored by the Parkinson Study Group and Movement Disorder Society Sunday, October 14, 1990 Grand Ballroom A, Hilton Hotel, Atlanta, GA Program 1:3O 1:3O -1:45 1 :45 -2:00 2:OO -2:lS 2:15 -2:3O 2:3O -2:45 2:45 -3:OO 3:OO -3:lS 3:15 -3 :.? 0 3:3O -3:45 3:45 -4:00 4:OO -4:IS 4:15 -4:3O Introduction Koller Evaluation of Early Brain Changes in Corticobasal Ganglionic Degeneration Wilson SPECT in Corticobasal Degeneration Golbe Jaw Tremor with Severe Dysarthria: A Variant of Essential Tremor Ranaway a Quantitative Analysis of Tremor and Chorea Using the VPL Data Glove Will Adult Manifestations of Gilles de la Tourette’s Syndrome Goetz A Controlled Trial of Propoxyphene and Naltrexone in Tourette’s Syndrome: Preliminary Results Kurlan Treatment of Paroxysmal Dystonia in Multiple Sclerosis with Acetazolamide Sethi Familial Torsion Dystonia and Cerebellar Ataxia: Variable Phenotypes in a Family Adlw Break G5HTP Is Not Useful in Cerebellar Syndromes Singer Extraneural Metabolism in Early Huntington’s Disease O’Brien AIDS Dementia Complex Presenting As an Unusual Unilateral Hand Tremor, Clumsiness, and Posturing Singer 4:30 Postencephalitic Movement Disorder With -4:45 Spontaneous Remission Perlnzutter 4:45 Dilantin-induced Dyskinesia -5:00 Hawison Abstracts Evaluation of Early Brain Changes in Corticobasal Ganglionic Degeneration R. E. Wilson, S. Bums, R. M. Kdem, K. Shannon, and W. 0. Whetsell, Jr, Nashville, TN Corticobasal ganglionic degeneration (CBGD) is a neurode- generative disease that presents with asymmetrical akinesia, rigidity, and apraxia. Postmortem examination (8 reported cases) typically reveals widespread cortical neuronal dropout as well as involvement of the basal ganglion. We present the case of a 64-year-old woman with a I-year history of im- paired right-hand dexterity following 8 years of left-hand dystonia. Her memory and verbal skills were normal. She had ideational apraxia and left cortical sensory loss. Magnetic resonance imaging (MRI) showed atrophy of the right sen- sorimotor cortex and symmetrical enlargement of the third and lateral ventricles. Positron emission tomography (PET) scan showed glucose hypometabolism in the atrophic cortex, but the reduction was more extensive than indicated by MRI. The right basal ganghon was hypometabolic but the basal ganglia were anatomically symmetrical. A right frontal brain biopsy was normal. Baclofen mildly reduced left arm tone. This is the first report of brain biopsy in a patient with clinically diagnosed CBGD. In this case cerebral histological findings were normal even though clinical symptoms and glucose uptake indicate functional abnormality. The functional cortical alteration that precedes detectable morpholog- ical change may give rise to subcortical changes as a conse- quence of altered corticobasal ganglionic projections. The etiology of the functional change is unknown. Single-photon Emission Computed Tomography Imaging in Corticobasal Degeneration L. I. Golbe, New Brunswick, NJ Corticobasal degeneration (CBD) is generally an autopsy diagnosis. Only 7 cases have been reported in detail and 298 Annals of Neurology Vol 28 No 2 August 1990

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Hyperkinetic Movement Disorders: Symposium Co-Sponsored by the Parkinson Study Group

and Movement Disorder Society Sunday, October 14, 1990

Grand Ballroom A, Hilton Hotel, Atlanta, GA

Program

1:3O

1:3O -1:45

1 :45 -2:00

2:OO -2:lS

2:15 -2:3O

2:3O -2:45

2:45 -3:OO

3:OO -3:lS

3:15 -3 :.? 0

3:3O -3:45

3:45 -4:00

4:OO -4:IS

4:15 -4:3O

Introduction Koller

Evaluation of Early Brain Changes in Corticobasal Ganglionic Degeneration Wilson

SPECT in Corticobasal Degeneration Golbe

Jaw Tremor with Severe Dysarthria: A Variant of Essential Tremor Ranaway a

Quantitative Analysis of Tremor and Chorea Using the VPL Data Glove Will

Adult Manifestations of Gilles de la Tourette’s Syndrome Goetz

A Controlled Trial of Propoxyphene and Naltrexone in Tourette’s Syndrome: Preliminary Results Kurlan

Treatment of Paroxysmal Dystonia in Multiple Sclerosis with Acetazolamide Sethi

Familial Torsion Dystonia and Cerebellar Ataxia: Variable Phenotypes in a Family Adlw

Break

G5HTP Is Not Useful in Cerebellar Syndromes Singer

Extraneural Metabolism in Early Huntington’s Disease O’Brien

AIDS Dementia Complex Presenting As an Unusual Unilateral Hand Tremor, Clumsiness, and Posturing Singer

4:30 Postencephalitic Movement Disorder With -4:45 Spontaneous Remission

Perlnzutter

4:45 Dilantin-induced Dyskinesia -5:00 Hawison

Abstracts

Evaluation of Early Brain Changes in Corticobasal Ganglionic Degeneration R. E. Wilson, S. Bums, R. M. Kdem, K. Shannon, and W. 0. Whetsell, Jr, Nashville, T N Corticobasal ganglionic degeneration (CBGD) is a neurode- generative disease that presents with asymmetrical akinesia, rigidity, and apraxia. Postmortem examination (8 reported cases) typically reveals widespread cortical neuronal dropout as well as involvement of the basal ganglion. We present the case of a 64-year-old woman with a I-year history of im- paired right-hand dexterity following 8 years of left-hand dystonia. Her memory and verbal skills were normal. She had ideational apraxia and left cortical sensory loss. Magnetic resonance imaging (MRI) showed atrophy of the right sen- sorimotor cortex and symmetrical enlargement of the third and lateral ventricles. Positron emission tomography (PET) scan showed glucose hypometabolism in the atrophic cortex, but the reduction was more extensive than indicated by MRI. The right basal ganghon was hypometabolic but the basal ganglia were anatomically symmetrical. A right frontal brain biopsy was normal. Baclofen mildly reduced left arm tone. This is the first report of brain biopsy in a patient with clinically diagnosed CBGD. In this case cerebral histological findings were normal even though clinical symptoms and glucose uptake indicate functional abnormality. The functional cortical alteration that precedes detectable morpholog- ical change may give rise to subcortical changes as a conse- quence of altered corticobasal ganglionic projections. The etiology of the functional change is unknown.

Single-photon Emission Computed Tomography Imaging in Corticobasal Degeneration L. I . Golbe, New Brunswick, NJ Corticobasal degeneration (CBD) is generally an autopsy diagnosis. Only 7 cases have been reported in detail and

298 Annals of Neurology Vol 28 No 2 August 1990

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