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55 Cards in this Set

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What is the basal ganglia?
A collection of gray matter nuclei located deep within the white matter of the cerebral hemispheres.
What are the 6 sections of the basal ganglia?
Caudate, Putamen, Subthalamic nucleus, globus pallidus, and substania nigra
What comprises the striatum?
Caudate and Putamen
What is the striatum's role?
The major input nuclei to the basal ganglia
What is the role of the globus pallidus and substantia nigra?
They are the major output nuclei of the basal ganglia.
What makes up the lentiform nucleus?
Putamen and globus pallidus
What does the basal ganglia do?
It controls and regulates activities of the motor and premotor cortex via various circuits so that voluntary movements may be performed smoothly.
What are the two predominant pathways that form input to output nuclei through the basal ganglia?
The direct and indirect pathways.
What two structures have similar embryologic origin?
Globus pallidus interna and substantia nigra reticulata
What is the pathway of the direct pathway?
cortex -> striatum -> GPi/SNr -> thalamus -> motor cortex
What is the pathway of the indirect pathway?
cortex -> striatum -> GPe -> STN -> GPi/SNr -> thalamus -> motor cortex
When does movement result?
Movement results when thalamic cells are released from tonic inhibition.
What is the role of dopamine in the direct and indirect pathways?
Dopamine inhibits in the indirect pathway and facilitates the direct pathway.
What is thought to be responsible for the disorder in movement?
The shift in degree of inhibitory influence flowing from the basal ganglia to the thalamus.
What are the 4 major features of basal ganglia disease?
Hyperkinesia, hypokinesia, rigidity, and loss of automatic associated movements.
In a unilateral movement disorder caused by a focal basal ganglia lesion, the movement disorder is ______________ to the basal ganglia lesion.
Contralateral
What are the subtypes of hypokinesis?
bradykinesia and akinesia
What causes bradykinesia and akinesia?
Increased inhibitory basal ganglia outflow to the thalamus.
What are the 8 types of hyperkinesis?
tremor, dystonia, tics, chorea, athetosis, myoclonus, hemiballismus and tardive dyskinesia.
What is hemiballismus?
Large amplitude choreiform movements of PROXIMAL limbs resulting in flailing of the limb.
What is the typical cause of tardive dyskinesia?
Dopamine receptor blocking drugs.
What is rigidity?
Resistance to passive movement
Is rigidity velocity independent or dependent?
INdependent
What are the 2 types of rigidity?
Cogwheel rigidity and Lead pipe rigidity.
When is cogwheel rigidity seen?
In Parkinsonian disorders.
What is the cause of Parkinson's disease?
caused by a loss of dopaminergic neurons in the SUBSTANTIA NIGRA PARS COMPACTA.
What are some causes of Parkinsonism?
drug induced (neuroleptics, antiemetics), toxins (MPTP, manganese), tumor, vascular, infection
What is the pathophysiology of Parkinson's?
The balance between dopamine and acetylcholine is disrupted secondary to the depletion of dopamine within the nigrostriatal system. The net effect is to increase inhibition of the thalamus, through both the direct and indirect pathways, resulting in the paucity of movement seen in PD.
Parkinson's disease occurs when what percentage of the neurons have lost synthesizing capacity?
70%
What is the average age of onset of PD?
60
What percentage of the population of people over 50% have PD?
1%
What are the 4 clinical features of PD?
bradykinesia, resting tremor, rigidity, and postural instability
Other than the main 4 features, what else is common in PD?
Dementia in 30%, depression and dysautonomia
What are the treatments for PD?
L-dopa/carbidopa, dopamine agonists, anticholinergics, amantadine, COMT inhibitors and MAOIs.
What surgery is available for PD and describe a candidate?
Deep Brain stimulation; relatively young, clear diagnosis of PD, w/o dementia/depression, few medical comorbidities.
What are the types of tremors?
Resting, postural tremor and intention (kinetic).
Describe a resting tremor.
3-5 Hz, occurs at rest - treated with standard PD therapies
What are the subtypes of Postural tremors?
Physiologic, enhanced physiologic and essential
What is a postrual tremor most prominent?
When body part is voluntarily maintained against gravity, as when holding arms out in front of body.
Describe an essential tremor.
Frequency 5 - 8 Hz, autosomal dominant, slowly progressive, kinetic component, worsens with stress, stimulants, improves with alcohol
What are the treatments for an essential tremor?
Propanolol and Primidone
What is an intention tremor?
It is worst during guided voluntary movement (as with finger to nose testing)
What are some causes of intention tremors?
Cerebellar, brainstem, wilson's disease, drug toxicity, it is very resistant to medical therapy
What is dystonia?
Prlonged contraction of axial and/or limb musculature resulting in distortion of posture.
What is torticollis?
Dystonia in the cervical region with contraction and hypertrophy of contralateral sternocleidomastoid muscle.
What are some etiologies of dystonia?
Genetic, perinatal/cerebral palsy, Wilson's disease, drug induced (antipsychotics), focal intracranial disease)
What are the treatment options of dystonia?
Baclofen, Benzodiazepines, Botulinum toxin injections and L-dopa.
What is the treatment of tic disorders or Tourette's during severe periods?
Dopamine antagonists like Haldol
What is the triad of Huntington's disease?
chorea, cognitive decline and positive family history
What is Syndenham's chorea?
Autoimmune chorea preceded by a streptococcal infection.
What are causes of myclonus?
anoxic brain injury, encephalitis, toxic metabolic encephalopathies. Can also be a result of an epileptic d/o, paraneoplastic d/o, or neurodegenerative d/o.
What are the treatment options for myoclonus?
Valproic acid, Klonopin (clonazepam), and Keppra (levitiracetam).
What is hemiballism?
Flailing proximal limb movement.
What causes hemiballism?
It is most commonly secondary to ischemia, hemorrhage, or trauma of subthalamic nucleus.
What is tardive dyskinesia?
Hyperkinetic movment disorder that may be an early or tardive (delayed) side effect of dopamine receptor blocking drugs.