Motor System & Movement Disorders Flashcards

(59 cards)

1
Q

Hyperkinesias

A

Tremor
Chorea
Tics

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2
Q

Essential Tremor - Clinical Characteristics

A

Tremor with posture and action, usually symmetrical, affecting the upper extremities (hands) and head more than the lower extremities

Usually presents with insiduous onset, worsening with age; affects up to 14% of people over 65

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3
Q

Chorea - Clinical characteristics

A

Irregular, brief, dancing-like, jerky movements that travel from one body part to another

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4
Q

Essential Tremor - Treatment

A
Beta-blockers (Propanolol) 
Primidone
Topramate
Gabapentin
Clonazepam 

Botulinum Toxin
Assistive devices

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5
Q

Motor signs of Parkinson’s Disease

A
Resting tremor, unilateral 
Bradykinesia 
Rigidity 
Postural instability / gait problems 
Dysarthria
Dysphagia
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6
Q

Tic - Definition

A

Brief, intermittent movements or sounds that are sudden, abrupt, and transient; vary in intensity but are repetitive and recur at regular intervals

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7
Q

Tourette Syndrome - Diagnostic Criteria & Clinical Characteristics

A

Sensorimotor disorder - abnormal motions combined with abnormal urges to move

Tics may be motor or vocal

Age of onset < 16 years; more common in males

Symptoms last > 1 year; 25% persist into adulthood

Associated with ADHD, OCD, poor impulse control

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8
Q

Differential Diagnosis of Parkinsonism Hypokinesia

A
Parkinson's Disease
Lewy Body Dementia 
Frontotemporal Dementia w/ Parkinsonism 
Progressive Supranuclear Palsy
Multiple Systems Atrophy
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9
Q

Treatment of Tourette Syndrome

A

Education and support groups
Treatment of co-morbid OCD, ADHD
Cognitive Behavioral Therapy

Treatment of tics only if interfering with life

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10
Q

Medical treatment of tics

A

Clonidine
SSRIs
Neuroleptics

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11
Q

Chorea-athetosis

A

Repetitive involuntary, slow, sinuous, writing movements

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12
Q

Dystonia - Definition

A

Co-contraction of muscle agonists and antagonists resulting in sustained muscle contractions causing twisting, abnormal postures

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13
Q

Etiology of Dystonia

A

Primary: DYT (1-12); protein Torsin is mutated in DYT-1

Secondary:

Cerebral palsy, neurodegenerative diseases, ischemic brain injury (stroke), traumatic brain injury, toxins

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14
Q

What is the most common classification of dystonia in adults?

A

Cervical dystonia

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15
Q

Classification of dystonia

A

Focal
Segmental
Multifocal
Generalized

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16
Q

Examples of focal dystonias

A
Blepharospasm
Hemifacial spasm
Oromandibular dystonia
Laryngeal dystonia 
Cervical dystonia
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17
Q

Task-specific dystonia

A

Writer’s cramp
Musician’s dystonia
Golfer’s dystonia

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18
Q

Atypical features of Parkinsonism

A
Rapid progression of the disease
Early onset of dementia, autonomic instability
Confusion or hallucinations 
Eye movement abnormalities (downgaze)
Poor response to dopaminergic treatment 

*Red flags for non-PD causes of parkinsonism; generally have worse prognosis than PD

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19
Q

Motor neuron pool

A

The population of alpha motor neurons that innervates the muscle fibers within a single muscle

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20
Q

What is the size principle of motor unit recruitment?

A

A given synaptic current induces a relatively larger voltage change in small motor neurons compared to large motor neurons; thus, small motor neurons that generate small forces via small motor units are recruited for voluntary muscular action (i.e. gripping an egg) first

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21
Q

Tonic muscle fibers

A

Non-spiking muscle fibers; shorten slowly and efficiently generate isometric tension with low fatigability

I.e. extraocular muscles

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22
Q

Slow twitch muscle fibers

A

Generate action potentials to twitch but fatigue slowly owing to high concentrations of myoglobin and many mitochondria

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23
Q

Fast twitch oxidative muscle fibers

A

Activate quickly; have many mitochondria and fatigue moderately slowly

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24
Q

Fast twitch glycolytic fibers

A

Activate quickly; fatigue rapidly owing to fewer mitochondria and dependence on anaerobic glycolysis for ATP generation

25
Slow motor units
Slow twitch muscle fibers innervated by small motor neurons; recruited during sustained activities such as maintaining upright posture
26
Fast fatigable motor units
Fast twitch glycolytic muscle fibers innervated by large motor neurons; recruited during activities such as running and jumping
27
Fast fatigue-resistant motor units
Fast twitch oxidative muscle fibers innervated by medium sized motor neurons
28
How are muscle spindles innervated?
1a sensory afferents detect muscle stretch y motor neuron efferents cause contraction of the muscle spindle
29
Golgi tendon organs
Proprioceptors that reside at the muscle/tendon junction and signal muscle tension information via type Ib sensory afferents
30
What is muscle tone? How is it maintained?
Muscle tone is the resistance of a muscle to stretch Muscle spindles maintain a baseline non-zero firing rate and so are able to signal subtle changes in muscle stretch or shortening by increasing or decreasing their firing rates; resultant decreased or increased activation of alpha motor neurons maintains tone
31
What is the mechanism of voluntary motor action error correction?
Lifting a box labeled 'books' that is actually empty causes greater contraction of muscle spindles relative to the muscle; muscle spindles signal via a drop in afferent Ia firing, rapidly reducing alpha motor neuron drive and reducing muscle contraction
32
Crossed extension reflex - mechanism?
Cutaneous nociceptors innervate spinal interneuronal motor networks that coordinate extensor relaxation and flexor contraction on the same side as the stimulus and a converse extensor contraction and flexor relaxation on the contralateral side
33
What is the role of the reticular formation in motor control?
The reticular formation is responsible for anticipatory responses to voluntary movement that help maintain postural control and balance i.e. Experimentally lesioning the RF in a cat causes the cat to fall over when it bats at an object because it is not able to anticipate and compensate for the shift in weight
34
What is the role of the superior colliculus in motor control?
The superior colliculus computes a map of auditory and visual space onto body coordinates; descending projections of the colliculospinal (tectospinal) tract target motor neurons that control axial musculature of the neck to generate coordinated orienting responses
35
Primary Motor Cortex = Broadman's Area X?
4
36
Which neuron type of M1 makes up the corticospinal tract?
Pyramidal neurons of layer 5 of the primary motor cortex project their axons through the descending corticospinal tract via the internal capsule, cerebral peduncle, and medullary pyramids
37
What is the role of the premotor cortex?
Premotor cortex makes up 25% of the corticospinal tract; premotor cortex neurons are involved when movement is initiated by an external cue Premotor cortex also houses 'mirror neurons' which are active while observing another person perform an action with an intended consequence
38
What is the role of the supplementary motor cortex?
Supplementary motor cortex is involved in self-cued movements, i.e. mental rehearsal of movement
39
What are two clues to the plasticity of the motor cortex?
Stroke - damage to part of the motor cortex results in acute impairment of the ability to control the affected area; over time, adjacent areas of cortex can sprout new connections and subserve motor control over the affected body part Practice - the repeated performance of a given action leads to expansion of that region of cortex; this is the basis behind 'constraint-induced movement therapy' in which the good limb is constrained so that the disabled limb will be preferentially used
40
Dysdiadochokinesia
Inability to perform rapid, alternating movements; characterstic of cerebellar lesion
41
Romberg Test
Patient stands with eyes closed and feet together; evaluated for unsteadiness with a positive test determined by degree of unsteady swaying ``` Suggestive of: Impaired proprioception (DC/Spinal Cord) Impaired vestibular function Impaired cerebellar function (vermis) ```
42
Hemiparetic vs. Paraparetic Gait
Hemiparetic gait - one-sided weakness; arm on affected side may remain flexed, affected leg adducts/swings out Paraparetic gait - weakness of both legs
43
Neuropathic Gait
i.e. "foot drop" gait; affected foot remains flat (not dorsiflexed) during swing phase, so patient has to lift leg higher to compensate
44
Myopathic Gait
Weakness of proximal muscles, i.e. Duchenne Muscular Dystrophy Patient walks with extension of spine and protruded belly May also see Trendelenberg gait - 'wobbling' of the pelvis due to weakness of gluteal muscles
45
Pathophysiology of Huntington Disease
Degeneration of D2 neurons in the caudate with loss of indirect pathway; leads to hyperkinesia including chorea
46
Histology of Parkinson Disease
Lewy body (synuclein) accumulation in the substantia nigra
47
Histology of Dementia with Lewy bodies
Lewy body (synuclein) accumulation in the frontotemporal lobes Seen later in the course of idiopathic Parkinson's Disease
48
Histology of Multiple System Atrophy
Synuclein accumulation in glia
49
What are environmental risk factors for Parkinson's Disease? What are protective factors?
Risk factors: Well water, pesticides, heavy metal toxins Protective factors: tea, coffee, cigarettes
50
What genetic mutation is associated with severe, early onset Parkinsons?
PARK7 mutation of DJ1
51
L-DOPA
First-line therapy in Parkinson's Disease; converted to dopamine by dopamine decarboxylase in surviving dopaminergic cells of the substantia nigra
52
Carbidopa
Administered as combo therapy with L-DOPA Blocks intestinal dopamine decarboxylase, preventing breakdown of L-DOPA in the intestine; reduces L-DOPA requirements by 90%
53
What is a typical dose of L-DOPA + Carbidopa?
100mg L-DOPA + 25mg Carbidopa
54
Dopamine Receptor (D2) agonists
Longer half lives to blunt the "on-off" L-DOPA response Adverse effects: Nausea, hypotension, narcolepsy
55
Sinetmet
L-DOPA / Carbidopa combo drug Adverse effects: Short term: Nausea, hypotension, depression, psychosis Long term: Diskinesia, psychosis
56
Use of Amantadine in Parkinson's
Facilitates release of endogenous dopamine
57
Use of anticholinergics in Parkinson's
Limited - may help treat tremor Benzatropine Diphenhydramine Side effects: dry mouth, constipation, urinary retention
58
Use of MAOIs in Parkinson's
Prevent breakdown of dopamine, prolonging the action of dopamine produced by L-DOPA i.e. Selegiline - often the first drug started after diagnosis of Parkinson's when brain cells are still making some dopamine
59
Use of COMT inhibitors in Parkinson's
Prevent breakdown of L-DOPA and dopamine by COMT Entacapone Tolcapone