Movement Disorders Flashcards
4 types of Hyperkinetic Disorders
Tremor- fine stereotyped oscillation
Chorea-Medium speed complicated moves
Athetosis-slow complicated movements
Ballism-fast complicated movements, violent flinging of limbs
Tics jerk like very brief repetitive movements
2 Types of Hypokinetic Disorders
Parkinson’s and syndromes with rigidity
Dopamine Blockade medications (Haldol, Antipsychotics, Antiemetics, Reglan)
Basal Gangila is made up of 4 structures
Striatum: Caudate and Putamen
Globus Pallidus
Subthalamic Nucleus
Substantia Nigra
What is the basal ganglia involved in?
Above anatomy (motor system) is involved in negative and positive feedback loops between cortex and spinal anterior horn cells (grey matter)
3 Types of neurotransmitters
Glutamate –excitatory
Dopamine –either excitatory to D-1 receptors or inhibitory to inhibitor D2 receptors
GABA (gama amino buteric acid) -inhibitory
Principle Defect in Parkinson’s Disease
Loss of dopamine in the Substantia Nigra in the INDIRECT system
4 basic features of Parkinson’s Disease
1- Rigidity defined :Bidirectional increase in muscle tone (flexion and extension) like a lead pipe—(recall Spastic is unidirectional)
2- Tremor –at rest 3 to 5 hertz “pill rolling”or “Pronator”in forearm and hand
3- Bradykinesa -slowness to start moving
4- Loss of postural reflexes –part of dysautonomia – loss of BP upon standing
which 2 symptoms of Parkinson’s Disease is most diagnostic
Rigidity and Bradykinesia are most diagnostic
First line Medication for Parkinson’s
at first – with Carbidopa/levodopa=Sinemet. This drug set replaces PREsynaptic dopamine. It is the most effective medication option.
4 dopamine agonists used for Parkinsons (post synaptic)
Ropinirole = Requip name brand
Pramipaxole = Mirapex name brand
Pergolide, Ergot family
Bromocryptine , Ergot family
Ergots are not used because?
Ergots are associated with heart valve fibrosis and Pulmonary fibrosis therefore not used much
3 types of Enzyme Inhibitors –work at the synaptic cleft between neurons
Eldepryl MAO (monoamine oxidase inhibitor)
Azelect MAO inhibitor also
Comtan COMT
Parkinson’s Surgical Treatment–Stimulation with a CNS pacer
Modern approach 3 locations: Ventral intermediate thalamus—Globus Palidus Interna—Subthalamic Nucleus
All have best result on tremor, less on motor skills
1/200 mortality rate
Morbidity with altered mentation and dysarthria possible
what is the clinical feature On/ Off state seen in parkinson’s
Freezing sudden loss of motor skill suddenly regained. Freezing is a walking issue not a tremor issue. Can freeze for minutes.
Dysautonomia
low BP poor increase with standing=orthostatic drop –Managed with elastic stockings –meds to increase BP Midodrine and Florinef
If dopamine levels are too high what would you see?
Sudden drowsiness
Gambling
Hypersexuality
Hallucinations
Chorea
involuntary medium speed purposeless movements in different body parts
Parakinesia
the cover up movement to make a choreaform movement look purposeful ex: arm moves up-chorea pt will move to smooth hair-purposeful cover up
Huntington’s Disease and the 3 main traits?
Autosomal Dominant Neurodegenerative Disease with 3 main traits:
1-Chorea
2-Dementia
3-Personality disorder
Personality changes associated with Huntington’s Disease
irritable
apathetic
rigid thinker
depression
Dementia –
Short-term memory loss
Loss of executive functions (Planning)
Altered speech (dysphasia, lack of language function)
Treatments for Huntington’s Disease
Tetrabenazine generic name – depletes presynaptic dopamine decreasing motor drive
Dopamine Postsynaptic Blockers
4 ddx Examples for hypo kinetic disorders
1-PSP=Progressive Supranuclear Palsy-vertical gaze palsy-
2-CBD Cortical Basilar Degeneration-
3 MSA Multiple System Atrophy Two Types
Type P –MSA-P
4- Lewy body dementia –Rapidly progressive dementia with progressive rigidity and refractory to Dopamine meds
Major indicators of Parkinson’s
40-70 y.o. slowly progressive rigidity/bradykinesia with later dementia/dysautonomia and good response to dopamine =Parkinson's
Hypokinetic Very common Disabling Bradykinesia/Rigidity/Tremor Defect: Dopamine lacking Rx: Sinemet
Characteristics of Huntington’s Disease
Hyperkinetic Uncommon Fatal Chorea/Dementia/Personality Disorder Defect: Dopamine excessive (clinically) Rx: Tetrabenazine/Haldol