17 - Cerebellum and Basal Ganglia Disroders

Question 1

the movements have an irregular, wavering course that seems to consist of continuous overshooting, overcorrecting, and then overshooting again around the intended trajectory

Answer 1

ataxia

Question 2

Coordinated actions of agonist and antagonist muscles acting on multiple joints, including the shoulder, elbow, and wrist, are required to smoothly perform this movement in a normal fashion

Answer 2

appendicular ataxia

Question 3

Lesions confined to the cerebellar vermis affect primarily the medial motor systems. Patients with such lesions therefore often have a wide-based, unsteady “drunklike” gait

Answer 3

truncal ataxia

Question 4

Lesions of the intermediate and lateral portions of the cerebellar hemisphere affect the lateral motor systems. Therefore, these patients have ataxia on movement of the extremities

Answer 4

appendicular ataxia

Question 5

In Parkinsons Disease, loss of dopaminergic neurons occurs in this structure?

Answer 5

substantia nigra pars compacta

Question 6

how is Parkinsons characterized? treated?

Answer 6

It is characterized by asymmetrical ‘pill-rolling’ resting tremor, bradykinesia, rigidity, and postural instability, which usually respond to therapy with levodopa.

Question 7

In Parkinson’s Disease, problems result from a decreased input this nucleus to the motor cortex of the brain?

Answer 7

These problems result from a decreased input from the ventrolateral (VL) nucleus of the thalamus to the motor cortex of the brain.

Question 8

an autosomal dominant neurodegenerative condition characterized by a progressive, usually choreiform movement disorder, dementia, and psychiatric disturbances, ultimately leading to death.

Answer 8

Huntingtons Disease

Question 9

Pathologic hallmark of Huntingtons Disease?

Answer 9

progressive atrophy of the striatum, especially involving the caudate nucleus.

Question 10

age of onset for Huntigntons Disease?

Answer 10

30 and 50 years, with prevalence of 4-5 cases per million.

Question 11

what gene is affected by Huntingtons Disease?

Answer 11

The abnormal gene is mapped on chromosome 4, and it includes a region containing multiple repeats of the trinucleotide sequence CAG in tandem.

Question 12

Rank the Common Movement Disorders by Movement Speed?

Answer 12

Bradykinesia
Rigidity
Dystonia
Athetosis
Chorea
Ballismus
Tic
Myoclonus
Tremor

Question 13

what cases is spasticity common in?

Answer 13

results from upper motor neuron lesions

Question 14

what cases would we see rigidity?

Answer 14

rigidity caused by basal ganglia disorders also called plastic, or lead pipe rigidity

Question 15

The patient assumes abnormal, often distorted positions of the limbs, trunk, or face that are more sustained or slower than in athetosis

Answer 15

Dystonia

Question 16

what is focal dystonia, like torticollis?

Answer 16

Dystonia which involves the neck muscles

Question 17

what is blepharospasm?

Answer 17

which involves the facial muscles around the eyes

Question 18

what is spasmodic dysphonia?

Answer 18

which involves laryngeal muscles

Question 19

what type of treatment does dystonia respond too?

Answer 19

botulinum toxin

Question 20

characterized by twisting movements of the limbs, face and trunk that sometimes merge with faster choreic movements, giving rise to the term choreoathetosis

Answer 20

athetosis

Question 21

Movements of the proximal limb muscles with a larger-amplitude, more rotatory or flinging quality than chorea

Answer 21

Ballismus

Question 22

what is a major cause of chorea?

Answer 22

Huntington’s disease ( autosomal dominant neurodegenerative disorder).