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Neurology > Hypokinetic Movement Disorders (18, part 1) > Flashcards

Hypokinetic Movement Disorders (18, part 1) Flashcards

0
Q

What deficits characterize hypokinetic disorders (hypokinesias)?

A

Slowed movements with increased tone

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1
Q

Movement disorders are the result of damage to which structure?

A

Basal ganglia–> includes caudate, putamen, globus pallidus, subthalamic nucleus, substantia nigra

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2
Q

What are three frequently encountered hypokinesias?

A
  1. Idiopathic Parkinson’s disease
  2. Progressive supranuclear palsy
  3. Multiple system atrophy
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3
Q

What is the typical age range of onset in Parkinson’s? What does age have to do with prevalence?

A

55-65; prevalence increases sharply with age, especially over 70
(*by 80 years old, prevalence may be as high as 1:200)

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4
Q

What is the age cut-off for early-onset Parkinson’s?

A

Symptoms appear before age 50–> genetic factors more likely operative in these cases

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5
Q

Life expectancy of Parkinson’s ?

A

Close to normal with treatment, but not quite

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6
Q

What are the four primary features of Parkinson’s ? Which is most frequently 1st and which is not seen till late?

A
  1. Rest Tremor (most commonly the first symptom)
  2. Rigidity
  3. Bradykinesia (akinesia)
  4. Postural instability - NEVER seen in the first year of appearance of motor dysfunction in true idiopathic Parkinson’s –> balance impairment/falling
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7
Q

How do the motor features usually present initially? (unilateral or symmetric)

A

Typically are unilateral at first, but eventually become bilateral; initial asymmetry of dysfunction remains (side that started first will always be worse than the one that started later)

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8
Q

What is rigidity?

A

Increased muscle tone that is remains even with passive movement - (will stay stiff even when you manipulate the limb passively on physical exam)
*cogwheel rigidity = ratchet-like jerkiness with passive movement due to rigidity with tremor

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10
Q

What is rest tremor?

A

Tremor that appears with limb at rest and usually disappears with movement of the limb

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11
Q

If an individual with Parkinson’s type symptoms presents with postural instability (poor balance, falling down) w/in the first year of onset of motor symptoms what does this mean?

A

They almost certainly don’t have typical Parkinson’s, but rather another hypokinetic disorder ( progressive supranuclear palsy or multiple system atrophy)

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12
Q

What are secondary motor features that are characteristic of idiopathic Parkinsonisan’s?

A
  1. Masked face - reduced facial expressions
  2. Hypokinetic dysarthria - soft, slurred speech (characteristic!)
  3. Micrographia - handwriting gets smaller the longer they write
  4. Diminished blink frequency
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13
Q

Other than depression and anxiety (which may precede motor symptoms but are non-specific), what is a cognitive impairment that is seen in Parkinson’s ?

A

Executive dysfunction - difficulty making decisions or carrying out plans
* not a memory impairment; also should not appear early in course of disease, if does, then likely not idiopathic type Parkinson’s, but one of the other “Parkinsonism-plus”disorders

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14
Q

What is a common feature of Parkinson’s due to gastrointestinal autonomic dysfunction?

A

Constipation (prob most widely recognized)

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15
Q

What urinary symptoms due to autonomic dysfunction may occur?

A

Usually increased frequency/ urgency; some may have under active bladder function though

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16
Q

What cardiovascular autonomic signs may be evident in Parkinson’s ?

A

Orthographic hypotension

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17
Q

What symptoms may be seen due to autonomic thermoregulatory dysfunction in Parkinson’s?

A

Episodic profuse sweating (not to a dangerous degree, but may be aggravating/embarrassing)

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18
Q

What are 3 other symptoms of Parkinson’s (not related to sleep)?

A
  1. Anosmia (loss of smell, 90%)
  2. Blurred vision (particularly for near objects)
  3. Shoulder pain(may be presenting symptom, often misdiagnosed as bursitis)
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19
Q

What is the most characteristic sleep-related disorder for Parkinson’s? What is another common sleep complaint?

A
  1. REM sleep behavior disorder - retain ability to move while sleeping and will act out dreams
  2. Frequent awakenings (very common)
    * #1 may be less common than 2’ but is highly CHARACTERISTIC of Parkinson’s, don’t confuse the two terms
20
Q

What is the classic pathological abnormality in Parkinson’s?

A

Degeneration of pigmented nigrostratial neurons in the pars compacta of the substantia nigra
*as much as 70% may be lost by the time motor symptoms develop

21
Q

What is the characteristic microscopic pathological lesion in Parkinson’s? Where found?

A

Lewy bodies - cytoplasmic inclusion bodies seen in the surviving neurons in the substantia nigra and elsewhere (like in the enteric nervous system)

22
Q

What protein is responsible for Lewy bodies?

A

Alpha-synuclein

23
Q

What is the neurochemical hallmark of Parkinson’s?

A

Dopamine deficiency- particularly in the substantia nigra in the midbrain and the striatum in the cerebrum

24
Q

What are 3 “red flag” symptoms that would suggest that a person doesn’t have idiopathic Parkinson’s, but instead one of the other “Parkinsonism-plus” syndromes?

A
  1. Symmetrical presentation w/ absence of tremor
  2. Early falls (postural instability w/in 1st year)
  3. Unresponsiveness to Levodopa
25
Q

What is bradykinesia?

A

Difficulty in initiating and terminating movement, slowness in carrying out movement, and difficulty with repetitive and/or simultaneous movements

26
Q

What is different about the rigidity and different facial expressions seen with Progressive supranuclear palsy compared to Parkinson’s?

A

Rigidity - usually effects the axial musculature, may also cause neck extension (rather than the flexion seen in PD)
Facial expression - appears “astonished” rather than lack of expression seen in PD

27
Q

What two symptoms may be more severe in PSP compared to PD?

A
  1. Postural instability - may even be the presenting symptom

2. Dysarthria - often early and severe

28
Q

What symptom is characteristic of PD, but is rarely seen in PSP?

A

Tremor

29
Q

What behavioral symptom is frequently seen in PSP, but not seen in either PD or multiple system atrophy?

A

Dementia - memory affected, unlike in PD

30
Q

What opthalmologic feature is a very definining feature and may even permit diagnosis of PSP?

A

Supranuclear gaze palsy - impairment of volitional downgaze (pt. cannot look down)

  • leads to “dirty tie” sign (messy eating) or difficulty going down stairs
  • in PD, the pts sometimes have trouble looking up, not down
31
Q

What is apraxia of eye opening, another opthalmologic feature of PSP?

A

Difficulty opening closed eyes when asked to do so–> but can still do it spontaneously

32
Q

What signs may be present in PSP that is not present in PD?

A

Pyramidal tract signs - like positive Babinski; indicates upper motor neuron damage
(Sleep disturbances may be present as well)

33
Q

Grossly, what 2 regions of the brain show atrophy in progressive supranuclear palsy?

A

Midbrain and cerebral cortex

34
Q

What protein is abnormally deposited in progressive supranuclear palsy? What structures are seen?

A

Tau protein–> seen in neurofibrillary tangles (unpIred straight filaments contained phospharylated tau protein)

35
Q

What three structures in brain show neuronal loss and gliosis in PSP?

A
  1. Globus pallidus
  2. Pedunculopontine nucleus
  3. Substantia nigra
36
Q

What is the most prominent neural helical abnormality of PSP?

A

Nigrostratial dopaminergic deficiency

37
Q

What is the age range of onset for Multiple System Atrophy?

A

50-55 (also younger than PD); similar to PSP however

*life expectancy ~ 5-10 years (maybe shorter than PSP)

38
Q

What three different clinical patterns may be observed in Multiple System Atrophy?

A
  1. Parkinsonism (eventually develops in ~90%)
  2. Progressive autonomic failure
  3. Cerebellar syndrome
    * used to be considered 3 distinct diseases
39
Q

What two neurological symptoms does multiple system atrophy share with PD? What features are similar to PSP?

A

Rigidity and Bradykinesia are typically seen

-like PSP, postural instability occurs early and also tremor is unusual

40
Q

Although it doesn’t affect all patients, what general features are considered a clinical hallmark of Multiple system atrophy?

A

Progressive autonomic failure - orthostatic hytension is perhaps the most widely recognized
*appears earlier and is more sever than that seen in PD; seen in 78% of pts with MSA

41
Q

What are 4 symptoms that may be due to cerebellar dysfunction in MSA?

A

Ataxia, dysarthria, coulombs or abnormalities, exaggerated rebound
*may only be evident in 55% of patients

42
Q

What pyramidal feature may be seen in MSA that is not seen in the others? What is one shared by PSP?

A
  1. Hyperreflexia

2. Positive Babinski

43
Q

What behavioral condition EXCLUDES a diagnosis of MSA? Which may be more characteristic for MSA than the others?

A

Dementia- not seen in MSA and may exclude the dx (seen only in PSP)
Personality changes - more characteristic of MSA
-also shares executive dysfunction as a feature, along with PD

44
Q

What are two “other” features that are characteristic of MSA? Which one may increase the risk of sudden death?

A
  1. Involuntary sighing
  2. Respiratory stridor
    * Raynaud’s and postural myoclonus are additional characteristic features
45
Q

What two areas of brain outside of brainstem, cerebellum, and spinal cord are characterized by cell loss and gliosis in Multiple System Atrophy?

A

Substantia nigra and posterior putamen

47
Q

What is the typical age of onset for Progressive supranuclear palsy?Life expectancy?

A

50-60 ( so somewhat younger than Parkinson’s; is also more rare)
Life expectancy is only about 10 years after dx- there is currently no effective treatment

48
Q

What is the microscopic pathological hallmark of MSA? What protein causes?

A

Glial cell cytoplasmic inclusion bodies –> stain for alpha-synuclein
* is in glial cells rather than neurons themselves as it is in PD, so don’t fuck that up

Neurology (23 decks)

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