Movement Disorders Flashcards

1
Q

4 types of Hyperkinetic Disorders

A

Tremor- fine stereotyped oscillation
Chorea-Medium speed complicated moves
Athetosis-slow complicated movements
Ballism-fast complicated movements, violent flinging of limbs
Tics jerk like very brief repetitive movements

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2
Q

2 Types of Hypokinetic Disorders

A

Parkinson’s and syndromes with rigidity

Dopamine Blockade medications (Haldol, Antipsychotics, Antiemetics, Reglan)

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3
Q

Basal Gangila is made up of 4 structures

A

Striatum: Caudate and Putamen
Globus Pallidus
Subthalamic Nucleus
Substantia Nigra

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4
Q

What is the basal ganglia involved in?

A

Above anatomy (motor system) is involved in negative and positive feedback loops between cortex and spinal anterior horn cells (grey matter)

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5
Q

3 Types of neurotransmitters

A

Glutamate –excitatory
Dopamine –either excitatory to D-1 receptors or inhibitory to inhibitor D2 receptors
GABA (gama amino buteric acid) -inhibitory

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6
Q

Principle Defect in Parkinson’s Disease

A

Loss of dopamine in the Substantia Nigra in the INDIRECT system

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7
Q

4 basic features of Parkinson’s Disease

A

1- Rigidity defined :Bidirectional increase in muscle tone (flexion and extension) like a lead pipe—(recall Spastic is unidirectional)
2- Tremor –at rest 3 to 5 hertz “pill rolling”or “Pronator”in forearm and hand
3- Bradykinesa -slowness to start moving
4- Loss of postural reflexes –part of dysautonomia – loss of BP upon standing

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8
Q

which 2 symptoms of Parkinson’s Disease is most diagnostic

A

Rigidity and Bradykinesia are most diagnostic

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9
Q

First line Medication for Parkinson’s

A

at first – with Carbidopa/levodopa=Sinemet. This drug set replaces PREsynaptic dopamine. It is the most effective medication option.

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10
Q

4 dopamine agonists used for Parkinsons (post synaptic)

A

Ropinirole = Requip name brand
Pramipaxole = Mirapex name brand
Pergolide, Ergot family
Bromocryptine , Ergot family

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11
Q

Ergots are not used because?

A

Ergots are associated with heart valve fibrosis and Pulmonary fibrosis therefore not used much

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12
Q

3 types of Enzyme Inhibitors –work at the synaptic cleft between neurons

A

Eldepryl MAO (monoamine oxidase inhibitor)
Azelect MAO inhibitor also
Comtan COMT

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13
Q

Parkinson’s Surgical Treatment–Stimulation with a CNS pacer

A

Modern approach 3 locations: Ventral intermediate thalamus—Globus Palidus Interna—Subthalamic Nucleus
All have best result on tremor, less on motor skills
1/200 mortality rate
Morbidity with altered mentation and dysarthria possible

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14
Q

what is the clinical feature On/ Off state seen in parkinson’s

A

Freezing sudden loss of motor skill suddenly regained. Freezing is a walking issue not a tremor issue. Can freeze for minutes.

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15
Q

Dysautonomia

A

low BP poor increase with standing=orthostatic drop –Managed with elastic stockings –meds to increase BP Midodrine and Florinef

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16
Q

If dopamine levels are too high what would you see?

A

Sudden drowsiness
Gambling
Hypersexuality
Hallucinations

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17
Q

Chorea

A

involuntary medium speed purposeless movements in different body parts

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18
Q

Parakinesia

A

the cover up movement to make a choreaform movement look purposeful ex: arm moves up-chorea pt will move to smooth hair-purposeful cover up

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19
Q

Huntington’s Disease and the 3 main traits?

A

Autosomal Dominant Neurodegenerative Disease with 3 main traits:
1-Chorea
2-Dementia
3-Personality disorder

20
Q

Personality changes associated with Huntington’s Disease

A

irritable
apathetic
rigid thinker
depression

Dementia –
Short-term memory loss
Loss of executive functions (Planning)
Altered speech (dysphasia, lack of language function)

21
Q

Treatments for Huntington’s Disease

A

Tetrabenazine generic name – depletes presynaptic dopamine decreasing motor drive
Dopamine Postsynaptic Blockers

22
Q

4 ddx Examples for hypo kinetic disorders

A

1-PSP=Progressive Supranuclear Palsy-vertical gaze palsy-
2-CBD Cortical Basilar Degeneration-
3 MSA Multiple System Atrophy Two Types
Type P –MSA-P
4- Lewy body dementia –Rapidly progressive dementia with progressive rigidity and refractory to Dopamine meds

23
Q

Major indicators of Parkinson’s

A
40-70 y.o. 
slowly progressive rigidity/bradykinesia
 with later dementia/dysautonomia 
and good response to dopamine 
=Parkinson's
Hypokinetic
Very common
Disabling
Bradykinesia/Rigidity/Tremor
Defect: Dopamine lacking
Rx: Sinemet
24
Q

Characteristics of Huntington’s Disease

A
Hyperkinetic
Uncommon
Fatal
Chorea/Dementia/Personality Disorder
Defect: Dopamine excessive (clinically)
Rx: Tetrabenazine/Haldol
25
Dystonia Definition
sustained muscle contraction (sometimes a local group of muscles ie: neck ) leading to abnormal posture
26
3 examples of dystonia
Cervical Dystonia –torticollis- neck group localized lateralized. Patient may use the sensory trick: the examiner finds the patient’s hand on their chin Writers Cramp –very specific to writing only-unilateral-action provoked Spastic Dysphonia –(Dianne Reem of NPR radio) voice action specific
27
Hyperkinetic Disorder Tremor
Most common movement disorder Def: Rhythmic involuntary movement Types Resting (as in Parkinson's) 3-5 hz Intention/Kinetic (as in finger to nose test ) Essential = (check thyroid) worse with caffeine better with alcohol (briefly) and bilateral
28
Treatment for Hyperkinetic Disorder Tremor
Beta blockers beware not to use in asthma or bradycardia Mysoline name brand=Primidone generic- low dose 50 mg hs and titrate up Maybe Neurontin= Gabapentin and Topamax name brand Botox –pretty aggressive option Steriotactic surgery with brainstimulator
29
Tics
Def stereotyped repetitive jerk like(=fast) simple or complex movements Usually preceded by sensory urge Can be suppressed briefly Non purposeful
30
Tourette's Syndrome
Onset before 21 Tics and vocalizations (sounds, rarely formed words) Behavioral problems OCD ( ex hand washing +rituals), ADD, unwanted intrusive thoughts, depression, mania, phobias
31
Treatment for motor tics
Treatment- motor tics Clonodine (Catapress) very mild drug both on effect and side effects Dopamine blockers have potent effects and side effects (dyskinesia)
32
Tardive Dyskinesia (T.D.)
Usually encountered as iatrogenic illness with prolonged (>3 month) use of Dopamine blockers (psychiatric patients, diabetics with gastroparesis) 5 year incidence of T.D. is as high as 25% Tics/Dystonia/Chorea perhaps all 3
33
Myoclonus
Involuntary sudden shocklike muscle jerks limited or generalized Etiology usually CNS insult –anoxic/ischemic /alcoholics/ Can be spontaneous or action related Can be rhythmic or irregular Origin –Take your pick Cortical/spinal cord or nerve
34
Restless Leg Syndrome
RLS is a neurologic movement disorder of the limbs often associated with sleep Affects 10% of the adult population Most people have mild symptoms and do not need treatment Signs and Symptoms: Compelling urge to move the limbs Motor restlessness: floor pacing, rubbing the legs Symptoms worse at rest and temporary relief on activity Usually at night
35
2 Types of Restless leg syndrome
Primary RLS is genetic, mean age of onset 27 | Secondary RLS: pregnancy, anemia, ferritin deficiency, renal failure, peripheral neuropathy
36
4 core symptoms for restless leg syndrome
Urge to move legs because of irritating sensation Worse at rest Relief with movement Worse at night
37
Treatment of Primary restless leg syndrome
Dopamine agonists: Pramipexole (Mirapex), Ropinirole (Requip) Levadopa helps but has rebound issues Benzodiazepines Anticonvulsants
38
Treatment of Secondary restless leg syndrome
Correcting underlying disorder | Anemia, TSH, B12, Diabetes
39
ataxia definition
An inability to coordinate muscle activity during voluntary movement. Often due to disorders of the cerebellum or the posterior columns of the spinal cord; may involve the limbs, head or trunk
40
Causes of ataxia
Head trauma, stroke, TIA, cerebral palsy, MS, Varicella, Paraneoplastic syndromes, Tumor, alcohol, heavy metals, solvents, Medications: barbiturates, phenobarbital, benzodiazepines., genetics,
41
S&S of ataxia
gait impairment, unclear speech-(do not confuse with spastic dysphonia-a dystonic disorder), hand incoordination and tremor with movement
42
Ddx of ataxia
``` vestibular disease- this disease has dizziness, vertigo sensory ataxia(syphillis example) – positive Romberg (usually provoked by motion) ```
43
A person requires at least 2 of the 3 following senses to maintain balance while standing:
Proprioception- where the body is in space Vestibular function- where the head is in space Vision- monitors for changes in body position
44
What is Rombergs test used for
The Romberg test is used to investigate ataxia A positive test: the ataxia is sensory in nature Think: B12 deficiency, sensory peripheral neuropathy- CIDP chronic inflammatory demyelinating polyradiculapahty
45
Progressive and symmetric Acute, Subacute, Chronic Ataxia
Acute and reversible: intoxications- ETOH, drugs, glue sniffing, varicella Subacute: ETOH/malnutrition, tumors, solvents, Lyme Chronic: Inherited (SCA), hypothyroid, chronic infection
46
Focal Acute, Subacute, Chronic Ataxia
Acute: CVA, abscess Subacute: glioma, metastatic, MS, AIDS Chronic: MS, congenital lesions
47
Tests done for Ataxia
Symmetric: toxicology, TSH, Lyme titer, B1,B12, B1- Wernickies- Thaimine Deficiency: ataxia, encephalopathy and nystagmus (only 16% have all three) Focal: CT/MRI, CSF--