Chapter 20 Movement Disorders

Question 1

Movement Disorders Definition – A group of CNS degenerative diseases associated with involuntary movements or abnormalities of skeletal muscle tone and posture. They can be broadly classified as ___________ (too little) or ___________ (too much) movement disorders.

Answer 1

Movement Disorders Definition – A group of CNS degenerative diseases associated with involuntary movements or abnormalities of skeletal muscle tone and posture. They can be broadly classified as hypokinetic (too little) or hyperkinetic (too much) movement disorders.

Question 2

Pathophysiology of Parkinson’s Disease (PD; Hypokinetic Movement Disorder) – The predominant area of involvement is ___________ ___________, which are primarily ___________ in function. Affects the ___________-producing cells (substantia nigra and ___________ ___________) of basal ganglia, resulting in degeneration of nigrostriatal pathway and thereby causing decreased dopamine in the corpus striatum. This results in loss of inhibitory input to the cholinergic system, allowing excessive excitatory output.

Answer 2

Pathophysiology of Parkinson’s Disease (PD; Hypokinetic Movement Disorder) – The predominant area of involvement is basal ganglia, which are primarily inhibitory in function. Affects the dopamine-producing cells (substantia nigra and locus ceruleus) of basal ganglia, resulting in degeneration of nigrostriatal pathway and thereby causing decreased dopamine in the corpus striatum. This results in loss of inhibitory input to the cholinergic system, allowing excessive excitatory output.

Question 3

Parkinson’s Disease Etiology – Unknown. The most common movement disorder is Parkinson’s disease, affecting 1% of the population over 50 years of age. Incidence is 20/100,000 per year. Male:female ratio is 3:2. Associated with ___________ and ___________ use; 5% to 10% is hereditary (five genes identified so far).

Answer 3

Parkinson’s Disease Etiology – Unknown. The most common movement disorder is Parkinson’s disease, affecting 1% of the population over 50 years of age. Incidence is 20/100,000 per year. Male:female ratio is 3:2. Associated with pesticide and herbicide use; 5% to 10% is hereditary (five genes identified so far).

Question 4

Parkinson’s Disease Clinical Presentation
– The most common initial symptom is ___________ tremor (___________-rolling tremor) in the hands.
– Characterized by a triad of ___________ tremor, ___________, and muscle ___________.
– Features of advanced disease include ___________ facies, ___________ gait (shuffling), and postural instability due to loss of postural reflexes, resulting in fall to side or backward.
– ___________ phenomenon (transient inability to perform or restart certain tasks).
– ___________.
– ___________ (40%).

Answer 4

Parkinson’s Disease Clinical Presentation
– The most common initial symptom is resting tremor (pill-rolling tremor) in the hands.
– Characterized by a triad of resting tremor, bradykinesia, and muscle rigidity.
– Features of advanced disease include masked facies, festinating gait (shuffling), and postural instability due to loss of postural reflexes, resulting in fall to side or backward.
– Freezing phenomenon (transient inability to perform or restart certain tasks).
– Depression.
– Dementia (40%).

Question 5

Parkinson’s Disease Treatment – ___________ or ___________.

Answer 5

Parkinson’s Disease Treatment – Medical or surgical.

Question 6

Parkinson’s Disease
The goal of medical treatment is to increase ___________ action and decrease ___________ effect. A guiding principle is to start treatment when symptoms interfere with performing ADLs.

Answer 6

Parkinson’s Disease
The goal of medical treatment is to increase dopamine action and decrease cholinergic effect. A guiding principle is to start treatment when symptoms interfere with performing ADLs.

Question 7

Parkinson’s Disease
L-Dopa: Precursor of ___________. Given with ___________ (a dopa decarboxylase inhibitor), which prevents systemic metabolism of L-dopa (example: Sinemet).

Answer 7

Parkinson’s Disease
L-Dopa: Precursor of dopamine. Given with carbidopa (a dopa decarboxylase inhibitor), which prevents systemic metabolism of L-dopa (example: Sinemet).

Question 8

Parkinson’s Disease Dopaminergic agonists:
Ergot derivatives – ___________ (stimulates D2 receptors) and pergolide (stimulates D1 and D2 receptors).
Nonergot derivatives – ropinirole (Requip) and ___________ (Mirapex).

Answer 8

Parkinson’s Disease Dopaminergic agonists:
Ergot derivatives – bromocriptine (stimulates D2 receptors) and pergolide (stimulates D1 and D2 receptors).
Nonergot derivatives – ropinirole (Requip) and pramipexole (Mirapex).

Question 9

Parkinson’s Disease
___________ – an antiviral that potentiates release of endogenous dopamine and has mild anticholinergic activity.

Answer 9

Parkinson’s Disease
Amantadine – an antiviral that potentiates release of endogenous dopamine and has mild anticholinergic activity.

Question 10

Parkinson’s Disease
Anticholinergics – effective in ___________ ___________. Includes trihexiphenidyl (Artane), benztropine (Cogentin), procyclidine, and orphenadrine.

Answer 10

Parkinson’s Disease
Anticholinergics – effective in relieving tremor. Includes trihexiphenidyl (Artane), benztropine (Cogentin), procyclidine, and orphenadrine.

Question 11

Parkinson’s Disease
___________ of dopamine metabolism – inhibits ___________ ___________ (MAO)-B that is predominant in the striatum.
Selegiline – decreases oxidative damage in substantia nigra and slows disease progression.
Tolcapone – catechol-O-methyltransferase inhibitor, inhibits metabolism of dopamine in the liver, GI tract, and other organs.

Answer 11

Parkinson’s Disease
Inhibitors of dopamine metabolism – inhibits monoamine oxidase (MAO)-B that is predominant in the striatum.
Selegiline – decreases oxidative damage in substantia nigra and slows disease progression.
Tolcapone – catechol-O-methyltransferase inhibitor, inhibits metabolism of dopamine in the liver, GI tract, and other organs.

Question 12

Parkinson’s Disease
Surgical treatment is indicated in patients with advanced disease in whom medical treatment is ineffective or poorly tolerated. Mostly effective in relief of tremor. Complications include brain ___________, infection, and device failure.
Destructive surgery – ___________ or ___________.

Answer 12

Parkinson’s Disease
Surgical treatment is indicated in patients with advanced disease in whom medical treatment is ineffective or poorly tolerated. Mostly effective in relief of tremor. Complications include brain hemorrhage, infection, and device failure.
Destructive surgery – thalamotomy or pallidotomy.

Question 13

Parkinson’s Disease
Deep brain stimulator – electrode placed into ventral intermediate nucleus of the ___________.

Answer 13

Parkinson’s Disease
Deep brain stimulator – electrode placed into ventral intermediate nucleus of the thalamus.

Question 14

Parkinson’s Disease Causes for Disability
Social ___________.
Manual ___________ (inability to perform ADLs such as dressing, cutting food, writing, and fine motor skills).
___________ posture, resulting in loss of balance and increased risk of falls.
___________ of gait, resulting in retropulsion (staggering backward) or propulsion (stumble forward).
Speech impairment.
___________, resulting in silent aspiration.
___________ due to decreased frequency of spontaneous swallowing.

Answer 14

Parkinson’s Disease Causes for Disability
Social isolation.
Manual dexterity (inability to perform ADLs such as dressing, cutting food, writing, and fine motor skills).
Stooped posture, resulting in loss of balance and increased risk of falls.
Slowness of gait, resulting in retropulsion (staggering backward) or propulsion (stumble forward).
Speech impairment.
Dysphagia, resulting in silent aspiration.
Drooling due to decreased frequency of spontaneous swallowing.

Question 15

Parkinson’s Disease Rationale for Rehabilitation – Functionally based, i.e., objectively assessed using unified PD rating scale (UPDRS), which includes assessment of walking ___________, ___________, backward and forward stepping, ability to navigate obstacles, fine motor tasks, equilibrium, and simultaneous and sequential tasks.

Answer 15

Parkinson’s Disease Rationale for Rehabilitation – Functionally based, i.e., objectively assessed using unified PD rating scale (UPDRS), which includes assessment of walking speed, distance, backward and forward stepping, ability to navigate obstacles, fine motor tasks, equilibrium, and simultaneous and sequential tasks.

Question 16

Parkinson’s Disease Rehabilitation Strategies
Physical therapy
– ___________ training (hip extension, pelvic tilt, and standing).
– Postural ___________.
– ROM (passive/active and relaxation techniques).
– Ambulation – use of walker with wheels. Sometimes may use weighted walker to prevent retropulsion.
– Conditioning (quadriceps and hip extensor strengthening).
– ___________ exercises for coordination of foot placement.
– ___________ board or balance feedback trainers to improve body alignment and postural reflexes.

Answer 16

Parkinson’s Disease Rehabilitation Strategies
Physical therapy
– Posture training (hip extension, pelvic tilt, and standing).
– Postural reflexes.
– ROM (passive/active and relaxation techniques).
– Ambulation – use of walker with wheels. Sometimes may use weighted walker to prevent retropulsion.
– Conditioning (quadriceps and hip extensor strengthening).
– Frenkel’s exercises for coordination of foot placement.
– Wobble board or balance feedback trainers to improve body alignment and postural reflexes.

Question 17

Parkinson’s Disease Rehabilitation Strategies
Occupational therapy
– Adaptive equipments such as plate guards, cups/utensils with large handles, and swivel forks and spoons.
– Replace buttons on clothing with ___________/zipper closures.

Answer 17

Parkinson’s Disease Rehabilitation Strategies
Occupational therapy
– Adaptive equipments such as plate guards, cups/utensils with large handles, and swivel forks and spoons.
– Replace buttons on clothing with Velcro/zipper closures.

Question 18

Parkinson’s Disease Rehabilitation Strategies
Other strategies
– ___________ evaluation.
– ___________ breathing exercises to improve dysarthria.

Answer 18

Parkinson’s Disease Rehabilitation Strategies
Other strategies
– Swallow evaluation.
– Diaphragmatic breathing exercises to improve dysarthria.

Question 19

HYPERKINETIC MOVEMENT DISORDERS
Include tremors, tics, ___________ syndrome, dystonia (generalized and focal), dyskinesia, chorea, hemiballismus, myoclonus, and asterixis.

Answer 19

HYPERKINETIC MOVEMENT DISORDERS
Include tremors, tics, Tourette’s syndrome, dystonia (generalized and focal), dyskinesia, chorea, hemiballismus, myoclonus, and asterixis.

Question 20

Tremor – Rhythmic oscillation of a body part. Occurs in 6% of the population. Treatment includes ___________, primidone, benzodiazepines (BZDs; alprazolam), anticonvulsants (gabapentin and topiramate), and Botox.

Answer 20

Tremor – Rhythmic oscillation of a body part. Occurs in 6% of the population. Treatment includes propranolol, primidone, benzodiazepines (BZDs; alprazolam), anticonvulsants (gabapentin and topiramate), and Botox.

Question 21

Tics – Sustained ___________ muscle contractions that are rapid and stereotyped and often occurring in the same extremity or body part during stress.

Answer 21

Tics – Sustained nonrhythmic muscle contractions that are rapid and stereotyped and often occurring in the same extremity or body part during stress.

Question 22

Tourette’s Syndrome – Involuntary use of obscenities (___________) and obscene gestures (___________). Treated using neuroleptics (pimozide and haloperidol).

Answer 22

Tourette’s Syndrome – Involuntary use of obscenities (coprolalia) and obscene gestures (copropraxia). Treated using neuroleptics (pimozide and haloperidol).

Question 23

Dystonia – Slow sustained contractions of muscles that frequently cause twisting movements or abnormal postures.
– Idiopathic.
– Focal (___________, blepharospasm, oromandibular dystonia, and writer’s cramp).
– Generalized (___________ disease and lipid storage disorders).
– Neurodegenerative diseases such as PD and Huntington’s disease.
– Acquired with perinatal brain injury, CO poisoning, and encephalitis.
Treatment includes anticholinergics, baclofen, carbamazepine, clonazepam, and Botox for focal dystonias.

Answer 23

Dystonia – Slow sustained contractions of muscles that frequently cause twisting movements or abnormal postures.
– Idiopathic.
– Focal (torticollis, blepharospasm, oromandibular dystonia, and writer’s cramp).
– Generalized (Wilson’s disease and lipid storage disorders).
– Neurodegenerative diseases such as PD and Huntington’s disease.
– Acquired with perinatal brain injury, CO poisoning, and encephalitis.
Treatment includes anticholinergics, baclofen, carbamazepine, clonazepam, and Botox for focal dystonias.

Question 24

Tardive Dyskinesia – Involuntary choreiform movements of face and tongue such as chewing, sucking, licking, puckering, and smacking due to hypersensitivity of dopamine receptors due to long-term blockade.
– Associated with long-term ___________ medication use (20%).
– Decreased since the advent of atypical neuroleptics such as clozapine, risperidone, and olanzapine.
– Treatment includes ___________.

Answer 24

Tardive Dyskinesia – Involuntary choreiform movements of face and tongue such as chewing, sucking, licking, puckering, and smacking due to hypersensitivity of dopamine receptors due to long-term blockade.
– Associated with long-term neuroleptic medication use (20%).
– Decreased since the advent of atypical neuroleptics such as clozapine, risperidone, and olanzapine.
– Treatment includes BZDs.

Question 25

Ataxia – Usually associated with cerebellar disease
– Causes include stroke, MS, acute/chronic alcohol toxicity, and hereditary (slowly progressive) ___________ ataxia.
– Treatment includes compensatory techniques, gait training, and assistive devices.

Answer 25

Ataxia – Usually associated with cerebellar disease
– Causes include stroke, MS, acute/chronic alcohol toxicity, and hereditary (slowly progressive) Friedreich’s ataxia.
– Treatment includes compensatory techniques, gait training, and assistive devices.

Question 26

Athetosis – Slow, ___________, and ___________ movements affecting face and upper extremities.

Answer 26

Athetosis – Slow, writhing, and repetitive movements affecting face and upper extremities.

Question 27

Chorea – Nonstereotyped, ___________, ___________ movements involving oral structures.

Answer 27

Chorea – Nonstereotyped, unpredictable, jerky movements involving oral structures.

Question 28

Hemiballismus – Extremely violent ___________ of unilateral arm and leg secondary to infarct or bleeding in ___________ nuclei.

Answer 28

Hemiballismus – Extremely violent flinging of unilateral arm and leg secondary to infarct or bleeding in subthalamic nuclei.

Question 29

Myoclonus – Sudden jerky irregular contraction of muscle.
– Can be physiological (sleep jerks and hiccups).
– Essential (increasing with activity).
– ___________.
– Symptomatic (part of underlying encephalopathy or stroke).
– Spinal myoclonus (group of muscles innervated by spinal segments). Occurs in spinal cord disorders such as tumor, trauma, or MS.
– Treatment includes ___________, valproate, and Keppra.

Answer 29

Myoclonus – Sudden jerky irregular contraction of muscle.
– Can be physiological (sleep jerks and hiccups).
– Essential (increasing with activity).
– Epileptic.
– Symptomatic (part of underlying encephalopathy or stroke).
– Spinal myoclonus (group of muscles innervated by spinal segments). Occurs in spinal cord disorders such as tumor, trauma, or MS.
– Treatment includes clonazepam, valproate, and Keppra.