2. Movement Disorders Flashcards

1
Q

“Extrapyramidal” means what?

A

SUBCORTICAL control of movement (outside of the Corticospinal/Corticobulbar tracts)

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2
Q

Examples of structures in the Extrapyramidal system

A

Basal Ganglia (composed of striatum and globus pallidus)
Substantia nigra
Subthalamic nucleus
Red nucleus
Brain Stem (Reticular formation/Cerebellum)

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3
Q

Blood supply to the Basal Ganglia is MOSTLY through what artery

A

Lenticulostriate arteries (tiny branches of MCA)

  • BG is supplied by many branches of “Internal Carotid Artery”.
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4
Q

In the Basal Ganglia, the (direct/indirect) pathway stimulates the Motor Cortex via the thalamus

A

Direct

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5
Q

In the Basal Ganglia, the (direct/indirect) pathway inhibits the Motor Cortex

A

Indirect

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6
Q

_________ is the excitatory NT, and ______ is the inhibitory NT for the basal ganglia circuitry.

A

Glutamate

GABA

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7
Q

The Direct Pathway results in overall (increased/decreased) motor activity.

A

Increased

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8
Q

Pattern of info for the DIRECT Pathway

A

Cortex–> Striatum–> GPi–> Thalamus–> Cortex

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9
Q

The Indirect Pathway results in overall (increased/decreased) motor activity

A

Decreased

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10
Q

Pathway of info for the Indirect Pathway

A

Cortex–> Striatum–> GPe–> Subthalamic Nucleus–> GPi–> Thalamus –> Cortex

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11
Q

Output from the Striatum (Caudate and Putamen) is always inhibitory/excitatory for both direct & indirect pathways

A

inhibitory (GABA)

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12
Q

The Substantia Nigra (pars COMPACTA) produces _______ to excite direct pathway and inhibit indirect pathway.

A

Dopamine

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13
Q

(D1/D2) receptor is an INHIBITORY receptor. Binding of dopamine inhibits (direct/indirect) pathway

A

D2; indirect

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14
Q

(D1/D2) receptor is an EXCITATORY receptor. Binding of dopamine excites (direct/indirect) pathway

A

D1; direct

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15
Q

Dopamine binding of D1 and D2 receptors all result in (facilitating/inhibiting) movement

A

facilitating

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16
Q

There are ____________ neurons in the Striatum whose axons DON’T LEAVE the striatum and synapse in both the GPe and GPi; they work to INHIBIT the direct pathway and EXCITE the Indirect pathway

  • Opposite effect to DOPAMINE
A

Cholinergic

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17
Q

Movement Disorder; Depigmentation of neurons in Substantia Nigra Pars Compacta; histologically can see a-synuclein deposits in cells causing LEWY BODIES

  • No dopamine –> decreased direct pathway and decreased inhibition of indirect pathway –> limited motion
A

Parkinson’s Disease

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18
Q

Slowness of movement; most characteristic feature of Parkinson’s

A

Bradykinesia

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19
Q

Stiffness and resistance to movement of a body part; primary symptoms of Parkinson’s

A

Rigidity

  • Cogwheel rigidity most common
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20
Q

Cardinal Motor & Non-motor features of Parkinson’s Disease

A
Motor:
Bradykinesia
Rigidity
Tremor
Postural Instability/Abnormal Gait

Non-Motor:
Expressionless Face
Dementia
Anosmia

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21
Q

Know these HYPERKINETIC movements

A
  1. Chorea (irregular, random movement: Dr. Felix doing hula hoops)
  2. Athetosis (Continuous writhing movement: like typing)
  3. Ballismus (sudden, vigorous, forceful movements: bullet-like)
  4. Dystonia (TWISTING movements and abnormal postures)
  5. Myoclonus (Sudden, brief, shock-like movements: jerk)
  6. Tremor (rhythmical and regular movements)
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22
Q

______ tremor occurs with voluntary contraction of the muscle (2 types)

  1. ______ tremor occurs during purposeful movements and disappears during rest.
  2. ______ tremor occurs with upright posture and resolves with rest/lying down.
A

Action

  1. Intention
  2. Postural
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23
Q

________ is coarse tremor that is worse at the END of purposeful movements; caused by cerebellar lesion/disease

_______ occurs at REST and is improved with purposeful movement. Tremor associated with PD!

_______ is tremor that worsens during times of emotional stress, metabolic alterations and intoxications

A

Cerebellar Tremor

Resting Tremor

Physiologic Tremor

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24
Q

Disease characterized by both types of ACTION tremors (intention & postural) of arms and head.

  • Nodding, voice changes, dysphagia and difficulty with fine motor tasks; WORSE with emotional stress and BETTER with ALCOHOL (wow!)
A

Essential Tremor

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25
Essential tremors are... 1. tremors of (action/rest) 2. (symmetric/asymmetric) at onset 3. (high/low) frequency 4. (commonly/rarely) involve the head and voice
Action Symmetric High Commonly
26
Parkinson's tremors are... 1. tremors of (action/rest) 2. (symmetric/asymmetric) at onset 3. (high/slow) frequency 4. (commonly/rarely) involve the head and voice
Rest Asymmetric Slow (3Hx) --> "pill rolling" Rarely
27
Lentiform/Lenticular nucleus is composed of
Putamen | Globus Pallidus
28
_____ is the major input site of BG. _____ is the major output nucleus of BG. _____ is the second major output nucleus of BG.
Striatum GPi Substantia Nigra (pars reticulata)
29
How is Lewy Body Dementia different from Parkinson's Disease?
In Lewy body dementia, dementia appears FIRST and then is followed by PD-like motor features. In PD, core motor features appear first and BEFORE dementia.
30
Tx for essential tremor
1. Beta-blockers (PROPRANOLOL) | 2. Mysoline (barbitruate)
31
Hyperkinetic movements characterized by TWISTING movements and abnormal postures
Dystonia (2 types) - Generalized - Focal
32
Focal dystonia affect a single group of muscles and has 4 types. 1. ______ dystonia involves the neck. 2. ______ repetitive, involuntary squeezing of the eye muscles 3. ______ dystonia cramping of finger/hand 4. ______ dysphonia involves larynx; presents with hypophonia (soft speech)
1. Cervical 2. Blepharospasm 3. Focal Hand 4. Spasmodic
33
Tx for focal dystonia
Botox
34
Irregular, random movement: Dr. Felix doing hula hoops
Chorea
35
What conditions commonly produce Chorea
Huntington's Anti-Parkinson's meds Antipsychotics Post-Streptococcal Lupus Gravidarum (aka. hyperemesis/severe vomiting)
36
Choreiform movements caused by anti-parkinson's med are called
L-dopa induced dyskinesia
37
Choreiform movements caused by antipsychotics are called
Tardive Dyskinesia
38
Movement Disorder; FATAL AD condition; CAG trinucleotide expansion that accumulate over generations resulting in earlier and earlier appearances ("anticipation"); neuronal intranuclear inclusions of mutant huntingtin protein; causes behavioral changes, chorea and dementia;
Huntington's Chorea * aka. Huntington's Disease
39
Huntington's Chorea results in atrophy of _____ and dilation of _______.
Caudate Nucleus; Lateral Ventricles
40
Rapid Involuntary movements; usually patients can temporarily suppress, but eventually overwhelming anxiety and inner tension can only be relieved with allowing; can include obscene GESTURES or WORDS
Tics
41
Tic disorders are often seen as part of a triad of
ADHD OCD Tics
42
A tic disorder defined by multiple MOTOR tics + at least ONE VOCAL tic many times a day for more than a year; onset is BEFORE 18; tx only if the child is bothered by them
Tourette's Syndrome
43
A movement disorder that affects ONE SIDE (CONTRALATERAL lesion) of the body; involves large-amplitude involuntary jerking, irregular movements
Hemiballismus
44
Hemiballismus is due to a lesion in which part of the brain?
Subthalamic Nucleus
45
A lesion in the LEFT Subthalamic Nucleus causes _______ hemiballismus
RIGHT * CONTRALATERAL lesion
46
Movement disorders caused by exposure to medications that block dopamine are known collectively as
Extrapyramidal symptoms
47
Movement disorder caused by CHRONIC exposure to medications that block DOPAMINE within the CNS
Tardive Dyskinesia
48
Describe the movements of TD
Choreiform affecting the lips, mouth, and tongue (continuous sucking/chewing movements) * facial involvement
49
What are the common medications that cause TD?
1. Antipsychotics (haloperidol, fluphenazine, pimozide) | 2. Antiemetics (Metoclopramide, prochlorperazine)
50
Parkinson's is due to a problem in which part of the brain?
Substantia Nigra Pars Compacta (depigmentation of neurons producing DOPAMINE)
51
Huntington's is due to a problem in which part of the brain?
Caudate Nuclei (leads to dilatation of lateral ventricles)
52
_____ cerebellar peduncle connects to midbrain _____ cerebellar peduncle connects to pons _____ cerebellar peduncle connects to medulla
Superior Middle Inferior
53
_____ cerebellar peduncle connects to midbrain _____ cerebellar peduncle connects to pons _____ cerebellar peduncle connects to medulla
Superior Middle Inferior
54
Unlike the cerebrum, which works on a CONTRALATERAL basis, the cerebellum works _____ so cerebellar lesions cause _____ symptoms
ipsilaterally; ipsilateral
55
What section of cerebellum is responsible for proximal limb and truncal/core homonculus?
Vermis
56
What section of cerebellum is responsible for distal limb homonculus?
Intermediate zone/cerebellar hemisphere
57
1. Superior Cerebellar Peduncle is supplied by _______ 2. Middle Cerebellar Peduncle is supplied by ________ 3. Inferior Cerebellar Peduncle is supplied by ________
1. Superior cerebellar artery (branch of basilar artery) 2. AICA (branch of basilar artery) 3. PICA (branch of vertebral artery)
58
Unsteady gait that is irregular and uncoordinated
Ataxia
59
________ ataxia are caused by lesions that interrupt the sensory input; compensated with visual input. ________ ataxia are caused by lesions in cerebellum; doesn't compensate with visual input. ________ ataxia is a combination of both above.
Sensory Motor Spinocerebellar
60
Inherited atxia; AR condition caused by GAA trinucleotide repeats on the Frataxin (FXN) gene on chromosome 9; leads to frataxin (iron-binding protein) deficiency and iron accumulation in mitochondria
Friedreich's Ataxia
61
Inherited ataxia associated with progressive ataxia, cardiomyopathy, pes cavus (foot deformity), scoliosis, blindness/deafness
Friedreich's Ataxia
62
A disorder of mitochondrial myopathy that causes cerebellar ataxia and TONIC-CLONIC episodes; ragged red fibers on histological findings
MELAS
63
A disorder of mitochondrial myopathy that causes cerebellar ataxia and MYOCLONUS epilepsy; ragged red fibers on histological findings
MERFF
64
What are some common etiologies of ACQUIRED ataxias?
Prion disease (CJD) Chronic alcohol overuse Autoimmune Disease
65
What physical exams test for Ataxia?
1. Finger to nose (cerebellar hemisphere) 2. Heel to toe (cerebellar vermis) 3. Romberg Sign (+ means sensory ataxia)