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Flashcards in BASAL GANGLIA Deck (22)
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1. Progresssive supranuclear palys is characterized by all of the following EXCEPT
A. frequent association with Wilson disease
B. inability to look downward
C. prominent sings of pseudobulbar palsy
D. loss of cells in the globus pallidus and pariaqueductal gray
E. neurofibrillary tangles in surviving cells

1-A Progressive supranuelear palsy is assoiated with Parkinson disease. It is characterizes by paresis of downgaze later parasis of upgaze occous. Pseudobulbar palsasy, which is always present. Results from multiple hemospherie lesions that destroy upper motor neurons (UMNs) bilaterally, it leads to a loss of cals in the globus pallidus, pariaqueductal gray, red nueleus, sub stantia nigra, and dentate nucleus. Usually the cerebral and cerebellar cortices are not affected Neurofibrillary tangles are found in surviving neurons.

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2. Whiech thalamic neclues projects to the striatum ?
A. Centromedian nucleus
B. Mediodoral nucleus
C. Ventral lateral nucleus
D. Ventral anterior nucleus
E. Ventral posterolateral nucleus

2-A The striatum (caudate nucleus and putamen) receives thalamic input from the centromedian nucleus, the largest of the intralaminaar nuclei

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3. The globus pallidus projects to the thalamus via the
A. fasciculus D. ansa penducularis
B. stria medullaris E. stria terminalis
C. ansa lentacularis

3-C The globus pallidus projects to the thalamus via the lenticular and thalamic penduncle) interconneets the amydolaid nueleus and the hypothalamus. It also interconneets the orbitofrontal cortex and the thalamus (mediodorsal nucleus). The fasciculus retroflexus (habenulonterpeducular tract) interconnects the habenular nucleus and the interpeducular nuclaus. The stria medullaris (thalami) interconnects the septal area (nuclei) and the habenular nuclei. The stria terminalis projects from the amygdolaid complex to the septual are and the hypothalamus.

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4. All of the following stantements concerning Huntington disease are corret EXCEPT
A. it is mherited as an autosomal dominant trait
B. it is associate with severe degeneration of the subthalamic nucleus
C. it is associated with hydrocephalus
D. patients have dementia
E. patients have choreiform movements

4-B Huntington chorea. Inerited as an autosomal dominant trait, is associated with severe dogeneration of the striatum and cell loss in the cerebral cortex. Striatal cell loss results in widening of the frontal horn of the lateral ventricle : this is called hydrocephalus ex vacuo. A DNA marker linked to Huntington chorea is located on chromosome 4.

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5. Wilson disease is characterized by all of the following EXEPT
A. it is a disorder due to a defect in the metabolism of copper
B. it is inherited as an autosomal dominant trait
C. tremor, rigidity, athetoric, or choreiform movements are present
D. a corneal Keyser-Fleischer ring is pathongnomonic
E. lesions are found in the liver and lentifornucleus

5-B Wilson disease (hepatolenticular degeneration) is a familian metabolic disease transmittedas an autosomal recessive trait. Low serum coruloplasmin, or low serum copper, and increased liver biopsy shows a high copper content. The corneal Kayser. Fleisher ring is pathognomonic of this disease. A gene loecus has been found on chromosome 4

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6. The berasal ganglia inelude all of the following structures EXCEPTM the
A. glabus pallidus D. lentiform nucleus
B. caundate nucleus E. subthalamic nucleus
C. putamen

6-E The basal ganglia include the caudate nucleus, putamen, and pallidus. The putamen and globus pallidus together are the lentiform (lanticular) nucleus. The subthalamic nucleus, a diencephalic nucleus, is not a basal ganglion but is a component of the striatal system. From an embryologic standpoint, the amygdaloid nucleus (archistriatum) and the elaustrum are basal ganglia, because they are derived from the telencephalic corpus striatum

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7. Which of the following constellations is NOT correct ?
A. Lantiform nucleus-putamen and globus pallidus
B. Noestriatum = stiatum = caudate nucleus and putamen
C. Paleostriatum = pallidum = globus palidus
D. Arehistriatum = amygdaloid nucleus.
E. Corpus straitum = caudate nucleus putamen, and claustrum

7-E The corpus stratium includes the caudate nucleus, the putamen, and the globus pallidus. The enigmatic elaustrum is found between the extreme capsule.

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8. All of the following statements concerning neurotransmitters are correct EXCEPT
A. the neocortex projects glutamantergic fibers to the striatum
B. the stratum projects gamma-aminobutyrieacids (GABA)-ergic fibers to the globus palidus and substantia nigra.
C. dopaminergic neurons ore found in the putamen
D. the substantia nigra projects dopaminergie fibers to the striatum.
E. the globus pallidus projects GABA ergicfibers to the thalamus

8-C Dopimane-containing neurons (cell bodies) are found in the pars compacta of the substantia nigra in the ventra tegmental area of the midbrain. The predominant cell type in the putamen is the medium sized gamma-aminobutyric acid (GABA)-ergic spiny neutron.

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9. All of the following statements concerning glatumatergic neuronts are correct EXCEPT
A. they are found in the globus pallidus
B. they excite gamma-aminobutyric acid (GABA)- ergic neurons of the putamen
C. they project from the subthalamic nucleus to the globus pallidus
D. they project from the cortex to the subthalamic nueleus.
E. they project from the cortex to the thalamus

9-A Glutamatergic neurons have not been indentified in the globus pallidus, they have been located in the subthamalic nucleus and thalamus

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10. All of the following statements concerning dopaminergic acuronts neurons are correct EXCEPT
A. they are found in the pars compacta of the substantia nigre
B. they are found in the subthalamic nucleus
C. they project to the putamen
E. they are though to regulate the production of striatal peptides and peptide mRNA

10-B Dopaminergic neurons of the substantia nigra project via the nigrostriatal pathway to the striatum (caudate nucleus and putamen). Dopamine released in the striatum is thought to regulate the production of nouropeptides and peptide mRNA within ther resident striatal neurons. The subthalamic nucleus receives gamma-aminobutiryc acid (GABA)-ergic input from the lateral (external) segment of the globus pallidus. The subthalamic nucleus project excitatory glutamateric input to both segment of the globus pallidus.

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11. All of the following statements concerning the substantia nigra are correct EXCEPT
A. it receives input form the caudate nucleus
B. it receives input from the putamen
C. it receive dopamineregic input the from the striatum
D. it project to the thalamus
E. it is found in the midbrain

11-C The substantia nigra receives gamma-aminobutyri acid (GABA)-argic input from the caudate nucleus and putamen. It project dopaminergic fibers to the caudate nucleus and putamen. It project GABA-ergic fibers to the ventral lateral and ventral anterior thalamic nuclei.

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12. The striatum receives major input from all of the following nuclei EXCEPT the
A. substantia nigra D. sensory cortex
B. contromedian E. subthalamic nucleus

12-E The striatum (cuadete nueleus and putamen) receives major input from there sources : the neocortex, ineluding the motor and sensory cortices : the thalamus (centromedian nucleus) : and the substantia nigra (pars compacta). The substalamic nucleus has important reciprocal conctions with the globus pallidus : it does not project to the striatum.

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13. All of the following statements concerning the globus pallidus are correct EXEPT
A. it receives input from the motor cortex
B. it receive input from the putamen
C. it receive input from the subthalamic nueleus
D. it projects to the ventral anterior nueleus
E. it projects to the ventral lateral nucleus

13-A The globus pallidus, the major effector nucleus of the striatal system, project to the ventral anterior, ventral lateral, and centromedian thalamic. It receives from the striatum (the caudate and putamen) The globus pallidus also has reciprocal connections with the subthalamic nucleus. The motor cortex does project to the glabus pallidus.

14

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

14. Is the overall most common cause of chorea

14-F Syndenham cromea (St. Vitus Dance) is the most commen cause of chorea ovarell. It usually found in girls in the head of the caudate nucleus, putamen, and globus pallidus. In Huntington disease, there is massive loss of neurons in the caudatoputamen.

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A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

15. Results from a loss of dopaminergie neurons in the pars compacta of the substantianigra

15-E Parkison disease resules from a loss of dopaminergic neurons in the pars ompacta of the substantia nigra.

16

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

16. Acorneal kayser Fleischer ring is pathongnomonic for this dyskinesia

16-B Hepatolenticular degeneration. Wilson disease, in an autosomal disorder duo to a defect in the metabolisme of copper. The kayser-Fleiser ring is a green band of pigmentation found around the limbus in Descement membrane ; it is a pathognomonic of Wilson disease.

17

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

17. Results from a lesion of the subthalamic nucleus

17-C Hemiballism results from a contralateral lesion (usually vascular) of the subthalamic nucleus. It is eharacterized by violent flinging (ballistic) movements of one or both extremities

18

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

18. Is characterized by repetitive ehoreicmovements affecting the face, limbs, and trunk, which results from treatment with antipsychotic drugs.

18-C Tardive dyskinesia is a syndrome characterized by repetitive chorcic movement affecting the face and trunk, which results from treatment with antipsychotic drugs (e.g.. phenothiazines, butyrophenones, or metoclopramedi)

19

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

19. Can be traced to a dingle gene defect on chromosome 4

19-D Huntington disease has its gene has been assigned to the esterase D locus on chromosome 13.

20

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

20. Has its gene lueus on chromosome 13

20-B

21

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

21. Is characterized by cortical atropy and loss of neurons in the head of the caudate nuclous

D Huntington disease is characterized by cortical atropy and loss of naurons in the head of the caudate nucleus, which results in hydrocephalus ex vacou

22

A. Chorea gravidarum D. Huntington
B. Hepatolenticular degeneration E. Parkinson
C. Huntington disease

22. Central nervous system (CNS) lesions are characterized rized by nocresis and cavitation of the putamen

22-B Wilson disease is characterized by necrosis and cavitation of the putamen.