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Flashcards in CM- Movement Disorders Deck (44)
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1
Q

What is a tremor?

What are the 4 types?

A

A tremor is a rhythmic oscillation of any body part.
Amplitude = variable
Frequency = predictable

  1. essential tremor - arms, hands, fingers during eating and writing
  2. rest tremor- occurs when the muscles are relaxed [lying at sides, on lap]
  3. dystonic “tremor” -twisting and repetitive motions and/or painful and abnormal postures or positions, such as twisting of the neck (torticollis) or writer’s cramp
  4. task-specific tremor -occurs with a highly specific task like writing or speaking
2
Q

A patient presents with a paucity of spontaneous movement and decreased amplitude of movement. What is this called?
What is it called if the person has absent spontaneous movement?

A

Bradykinesia = slowness, paucity of spontaneous movement

Akinesia = absence of spontaneous movement

3
Q

A patient presents with laterocollis, torticollis, anterocollis, or retrocollis. These are examples of what?
What is the reason why these occur?

A

These are examples of focal dystonia and are due to abnormal muscle contractions leading to sustained posture [co-contraction of agonist-antagonist]

Patterned/predictable muscle postures
interrupts and induced by limb movement

4
Q

What is blepharospasm?

A

dystonia where there is contraction of the b/l obicular ocularis [uncontrolled contraction of the eyelids]

5
Q

A patient presents with non-rhythmic, abrubt, unsustained writhing. The movements occur at random, unpredictable times and appear as dance-like, flowing movements. What is this an example of?

A

Chorea

6
Q

A patient has proximal, large-amplitude hemibody movements. What is this called?
What is the pathophysiology behind the problem?

A

Hemiballism- caused by a lesion in the contralateral STN of the basal ganglia disrupting inhibition of the “indirect circuit” and increasing thalamic firing

7
Q

A patient presents with a non-rhythmic, abrupt, unsustained “jerk-like” movement.
It is random, and unpredictable in timing. What does this describe?
What disorder are you likely to see it with?
What is the pathophysiological cause?

A

Myoclonus which is often seen with HD.

Pathophysiology: dysregulation of the pyramidal pathways from the cortex to the spinal cord

8
Q

What can induce myoclonus?

A
  1. spontaneous
  2. reflexive
  3. action-induced
  4. wake-sleep transition
9
Q

What is a tic?
What are examples of motor tics?
What are examples of phonic/verbal tics?

A

Tic is a brief, repetitive, stereotypic movement or sound.
URGE–> tic–>transient relief–> URGE

Motor: dsytonic, myoclonic, stereotypic, complex

Verbal: guttural sounds, throat-clearing, coprolalia[swearing]

10
Q

A patient was started on anti-psychotics a few weeks ago.
Now they are presenting with abnormal movements with complex, repetitive chewing movements, tongue protrusion, and choreiform limb movements, pelvic thrusting and dystonia.
What is the likely problem?

A

Tardive dyskinesia/dystonia

-often iatrogenic

11
Q

What is dyskinesia?

A

Abnormal, involuntary, hyperkinetic movements

12
Q

What are the 6 components of parkinsonism?

A
  1. bradykinesia
  2. stooped posture
  3. shuffling gait [“freezing gait’]
  4. resting tremors
  5. postural instability, falls
  6. cogwheel rigidity [resistance to movement around a joint]
13
Q

What falls under the broad category of parkinsonism?

A
  1. parkinson’s disease
  2. stroke
  3. iatrogenic/medications
  4. neurodegenerative disorders [Multiple System Atrophy affecting autonomics, nervous system, movement
14
Q

What are 5 neurodegenerative diseases that can closely mimic PD?

A
  1. MSA -multiple system atrophy
  2. DLBD- diffuse lewy body disease
  3. CBD- cortico-basal degeneration
  4. PSP- progressive supranuclear palsy
  5. Alzheimer’s
15
Q

What is the pathophysiology behind Parkinson’s disease?

A
  1. Loss of dopaminergic neurons in the substantia nigra, zona compacta–> progressive degeneration of basal ganglia connections, accumulation of Lewy bodies/a-synuclein
  2. widespread neurotransmitter dysregulation
    - Locus ceruleus [NE]
    - dorsal raphe [serotonin]
    - nucleus basalis of Meynert [Ach]
    - GABA, glutamate
16
Q

At what age does PD usually present?

Are most cases sporadic or genetic?

A

It is adult onset [age 21 and up, with the avg age being 60]

Most cases are sporadic and idiopathic.
Fewer cases are genetic:
AR - Park 2, 6, 7, 3 10 14 15 16
AD- Park 1, 8, 5, 11, 13, 17,18

17
Q

What motor symptoms are associated with Parkinson disease?

A
  1. asymmetric rest tremor
  2. cogwheel rigidity
  3. bradykinesia
  4. postural instability
  5. flexed posture [stooped]
  6. freezing [shuffling gait]
18
Q

What is the core diagnostic criteria for PD?

[if no labs or imaging is available]

A
  1. Bradykinesia and at least one of the following
  • rigidity
  • rest tremor
  • postural instability
19
Q

What criteria is supportive for PD?

A

3 of the following:

  1. response to levodopa
  2. responds to med for >5 years
  3. clinical course of >10 years
  4. unilateral onset
    5, persistent asymmetry
  5. rest tremor
  6. progressive
20
Q

When attempting to diagnose PD, what criteria would be:

  1. definitive
  2. probable
  3. possible
A

Definitive:

  1. clinical
  2. histological

Probable:

  1. 3/4 [rigidity, asymmetric, bradykinesia, postural instability]
  2. exclude other etiology
  3. responds to L-dopa

Possible:

  1. tremor OR bradykinesia + rigidity OR asymmetry
  2. exlude other etiology
  3. response to L-dopa
21
Q

Describe why parkinson’s disease is hypokinetic.

A

Lack of dopamine –> more inhibition of lateral globus pallidus–> less inhibition of STN –> more activation of SNr and MGP–> more inhibition of the thalamus –> less activation of the cortex

22
Q

Describe hyperkinetic disorders via pathway in the basal ganglion,

A

More inhibition of STN–> less activation of SNr, GPi–> less inhibition of thalamus–> more activation of the cortex

23
Q

What are the 4 main types of drugs used to treat Parkinson’s?

A
  1. Levodopa, carbidopa
  2. COMT inhibitors [entacapone, tolcapone]
  3. MAO-B inhibitors [selegeline, rasagiline]
  4. Dop agonists [ropinirole, pramipexole]
24
Q

What are signs that a neurodegenerative case might not be PD, but rather MSA, DLBD, PSP, CBD?

A
  1. no response/inadequate response to levodopa
    2 cognitive decline on neuropsych testing
  2. significant comorbidities
  3. psych disorder inadequately treated
25
Q

What does the pneumonic DBS in PD mean?

A

Deep Brain Stimulation:

  1. Does not cure PD
  2. Bilateral to improve gait
  3. Smooths out “on-off” fluctuation
  4. Improves tremor, rigidity, dyskinesia, akinesia,[postural instability responds least]
  5. Never improves L-dopa unresponsive symptoms
  6. programming required freq followup
  7. decreases meds, does NOT eliminate them
26
Q

What is the most prevalent movement disorder?
Who is most frequently affected by it?
How is it inherited?

A

Essential Tremor- action tremor that affects bilateral limbs +/- head and voice tremor with the absence of other progressive neuro signs

It has a bimodal distribution with peaks in the second and sixth decade [1-10% of the population over 65]
It is autosomal dominant

27
Q

What is the pathophysiology of essential tremor?

A
  • Cerebellum is thought to be a generator
  • “torpedos” - swelling of proximal purkinje cells in the cerebellum

Loss of purkinje cell inhibition to thalamo-cortical motor neuron pathways –>spontaneous depolarization or oscillation resulting in tremor

28
Q

What drugs are used to treat essential tremors?

What are the surgical treatments for essential tremor?

A

Meds:
Beta blockers - propanolol, primidone

Surgery:

  1. Lesioning Vm thalamic nuclei can disrupt cerebello-thalamic-cortical motor neuron loop
  2. thalamic DBS
  3. Thalamatomy [surgical ablation of Vm]
29
Q

Writers and musician’s cramp are example of what movement disorder?

A

Focal “task-specific” dystonia

30
Q

What is the mechanism behind tardive dystonia?

How is it treated?

A
  1. dopamine D2 receptor blockade [from antipsych drugs]
  2. supersensitivity and upregulation of SN postsynaptic receptors

Treat by:

  1. Eliminate the dopamine receptor blocking agent
  2. add atypical antipsychotic w/ low affinitD2 receptors, bind 5HT receptors and have low propensity for tardive dystonia
  3. dopamine depleters
  4. anticholinergics for dystonia
31
Q

What is treatment for dystonia?

A
  1. L-dopa
  2. anti-cholinergics
  3. antihistamine
  4. benzodiazapine
  5. GABAb agonists
32
Q

A patient presents with elevated LFTs. He has a “wing-beating” tremor, dysarthria and drooling, dystonia and Parkinsonism. He is cognitively impaired and has dementia and psychosis.
On physical exam, you note Kayser-Fleischer rings in his eyes seen with the slit lamp exam.

What is the likely diagnosis?
How do you make the diagnosis?
What is treatment?

A

Based on presentation, this seems to be Wilson’s Disease [inborn error in copper metabolism that causes hepatic cirrhosis and basal ganglia damage].

Diagnosis:

  1. High serum Cu, low ceruloplasmin, high urine Cu
  2. Liver biopsy
  3. Genetic test

Treatment: D-pencillamine, liver transplant

33
Q

What are the primary causes of hemiballism/hemichorea in the elderly?
The young?

A
Elderly = vascular, nonketotic hyperglycemia
Young = infectious, inflammatory
34
Q

A 9 year old girl had a recent GABHS streptococcal pharyngitis.
She is now presenting with chorea, motor impersistance, hypotonia and a tic. She also is demonstrating behavioral abnormalities.
What do you suspect that her labs will show?
What do you think the issue is?
What 4 types of drugs should you consider to treat?

A

Her presentation sound like Sydenham’s chorea [part of Jones criteria for rheumatic fever, a sequelae of GABHS infection]

Labs show:

  1. elevated antistreptolysin O
  2. elevated DNA-ase B
    • throat culture for s. pyogenes

Treatment:

  1. antiepileptic
  2. dopamine antagonist/depleter
  3. treat the rheumatic heart disease
  4. penicillin
35
Q

What drugs are used to treat symptomatic chorea?

A
  1. dopamine receptor blockade
  2. presynaptic dopamine depletion
  3. glutamate antagonist
  4. antiepileptic
  5. GABA agonist
36
Q

Describe the genetics of Huntington disease.

A
It is an autosomal dominant disease that is due to a mutation on chromosome 4 [Huntingtin gene].
It is a CAG [polyQ] repeat.
27-35 = premutation
36=39 = 40 = 100%
>60 = juvenile onset
37
Q

What is the Huntington clinical triad?

A
  1. chorea and other motor features
  2. psychiatric -[depression, personality change, OCD]
  3. cognitive [exec function, dementia]
38
Q

What age does Juvenile Onset Huntington disease usually begin?
What is the inheritence?
How many repeats are there likely to be on chromosome 4?

What is the clinical phenotype?

A

Before 21
autosomal dominant- paternal inheritence
>60 repeats [if over 80 they get it before 10]

Parkinsonism
Dystonia
Myoclonus
Seizures

39
Q

What are the 6 main clinical requirements for a tic to be considered Tourette’s syndrome?

A
  1. multiple motor and phonic tics
  2. tics many times a day for atleast a year
  3. location, freq, number, severity vary
  4. before age 21
  5. no other explanation
  6. witnessed by examiner
40
Q

A parkinson’s patient is over the age of 65 and is in late stage parkinson’s. They have no cognitivie/psychiatric symptoms or comorbidities. What medicine should they be on?

A

Levodopa

41
Q

A Parkinson’s patient is under 65 with mild/moderate stage disease. They do not have any cognitive/psych symptoms or comorbidities. What drug should they be on?

A

Dopamine agonist [ropinirole, prampexole]

42
Q

What 2 drugs should be avoided in patients with cognitive/psych symptoms and parkinsons?

A
  1. dopamine agonists

2. amantadine– glutamate antagonist

43
Q

What Parkinson drug should be avoided with meds that potentiate 5HT, NE or monoamines in the brain?

A

MAO-B blockers because you can get HTN

44
Q

What parkinson drugs should be avoided in CHF, renal failure and patients over 75?

A

Amantadine