Movement Disorders

Question 1

Why are basal ganglia disorders called “extra” pyramidal?

Answer 1

They do not affect the pyramidal system (AKA the corticospinal tract).

Question 2

What are the main components of the basal ganglia?

Answer 2

The caudate, putamen, globus palidus, subthalamic nucleus, substantia nigra.

Question 3

The striatum is composed of which two structures?

Answer 3

Caudate and putamen

Question 4

The lenticular nucleus is composed of which two structures?

Answer 4

Putamen and Globus palidus

Question 5

The most common movement disorder:

Answer 5

Tremor - rhythmic movement due to alternating contractions between agonist and antagonist muscles.

Question 6

What is chorea/athetosis?

Answer 6

Dance-like: refers to irregular, asymmetric movements that are random and continuous.

Snake-like: slow twisting and writhing movements that typically affect more distal muscles of the fingers, arms, legs, and neck.

Question 7

What is ballism?

Answer 7

Refers to violent, involuntary flailing of the extremities and can be thought of as an extreme form of chorea.

Question 8

Dystonia:

Answer 8

sustained, abnormal posture caused by the simultaneous activation of both agonist and antagonist muscles.

Question 9

Akathisia:

Answer 9

subjective sense of inner restlessness in which patients feel compelled to move continuously.

Question 10

What is festinating gait?

Answer 10

Trouble initiating gait, but steps become faster and faster until there is trouble stopping.

Question 11

What happens to resting tremors during sleep?

Answer 11

They vanish.

Question 12

Parkinson’s Disease Symptoms (TRAP mneumonic):

Answer 12

Tremor, Rigidity (cogwheel), Akinesia/Bradykinesia, Postural instability.

Question 13

What is often the first symptom of PD?

Answer 13

micrographia

Question 14

What percent of neurons in the SN must be destroyed before symptoms start in PD?

Answer 14

80%

Question 15

What is the pathological hallmark of PD?

Answer 15

Lewy Bodies: intracytoplasmic inclusions composed of alpha-synuclein.

Question 16

What test was approved by the FDA in 2011 to aid with diagnosis of PD?

Answer 16

DaTscan: a contrast agent used with single-photon emission CT (SPECT) to detect DA transporters.

Question 17

What is progressive supranuclear palsy?

Answer 17

A tauopathy characterized by midbrain atrophy and “reptilian stare.” Vertical gaze palsies, primarily with downward gaze, leads to spectacular falls early in the disease.

Question 18

Common meds that induce parkinsonism:

Answer 18

high potency typical antipsychotics and antiemetics

Question 19

What are two Parkinson-plus syndromes?

Answer 19

Multisystem atrophy cerebellar type and multisystem atrophy parkinsonian type. Shows atrophy of the pons and cerebellum.

Question 20

Describe essential tremor:

Answer 20

Action tremor, often bilateral, often familial, improves with EtOH, more common than parkinson’s disease.

Question 21

DA agonists carry which possible behavioral side effect?

Answer 21

Impulse control disorders such as pathological gambling or hypersexuality.

Question 22

What is Sinemet?

Answer 22

“Sin emesis”- or without vomit. The role of the carbidopa is to prevent the conversion of levadopa to dopamine by dopa decarboxylase and therefore prevent emesis. However, carbodipa does not cross the blood-brain barrier, so the levadopa can be converted to dopamine in the CNS. Levadopa is used instead of pure dopamine, as dopamine also cannot cross the blood-brain barrier.

Question 23

Orthostatic hypotension in PD patients can be treated with what?

Answer 23

Fludrocortisone

Question 24

Which structure is a common target for DBS in PD?

Answer 24

Subthalamic nucleus, also thalamus and globus pallidus.

Question 25

Lesions of the STN produce what?

Answer 25

Hemiballism: wild, flinging movements of the contralateral extremities.

Question 26

Initial treatment for PD:

Answer 26

DA agonsists Requip/Ropinirole and Mirapex/Pramipexole

Question 27

What is Stalevo?

Answer 27

Sinemet combined with catechol )-methyltransferase (COMT) inhibitors (entacapone, tolcapone).

Question 28

Hot cross buns on MRI

Answer 28

Sign of Multiple system atrophy

Question 29

Atrophy of the head of the caudate creating enlarged (boxcar) lateral ventricles and normal MRI:

Answer 29

Huntington’s Disease

Question 30

Mechanism of inheritance for HD?

Answer 30

AD, CAG repeats

Question 31

Each generation is affected earlier than the previous:

Answer 31

Anticipation

Question 32

Which medication might prove useful in treating chorea in HD?

Answer 32

Haloperidol

Question 33

The appearance of the midbrain on MRI is sometimes called the “face of the giant panda sign.”

Answer 33

Wilson’s Disease: enlarged liver and coarse tremor of arms (wing flapping tremor), movements are slow and patient is dysarthric.

Question 34

Wilson’s Disease, metabolism of which metal is abnormal?

Answer 34

Copper: will see Kayser-Fleischer rings around periphery of cornea.

Question 35

What can CO poisoning lead to?

Answer 35

Parkinsonism

Question 36

Treatment for spastic torticollis:

Answer 36

Botulinim toxin

Question 37

First-line treatment for restless leg syndrome:

Answer 37

DA agonists, such as roperinole

Question 38

Every patient with restless leg syndrome should be tested for what?

Answer 38

Iron deficiency

Question 39

Treatment for essential tremor:

Answer 39

Propranolol/Inderal

Question 40

Treatment for akathisia:

Answer 40

Propranolol/Inderal

Question 41

Treatment for NMS:

Answer 41

Bromocriptine (DA agonist), Dantrolene (calcium channel blocker)

Question 42

What is copropraxia?

Answer 42

Copropraxia is a tic consisting of involuntarily performing obscene or forbidden gestures, or inappropriate touching

Question 43

Most favorable risk/benefit ratio for patients with Tourette’s disorder:

Answer 43

Alpha adrenergic agonists (guanfacine/Tenex, clonidine)