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ABPP > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

What are movement disorders?

A

Group of diseases that primarily involve subcortical brain structures that are part of the extrapyramidal motor system including the basal ganglia (caudate, putamen, and globus pallidus), subthalamic nucleus, and substantia nigra, and thalmaic nuclei, and the cortex. The EPS system regulates movement and maintains muscle tone and posture.

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2
Q

What is Huntington’s disease?

A

An autosomal dominant neurodegenerative disorder, defined genetically as trinucleotide repeat of CAG (36 or more) on chromosome 4. Earlier onset is inversely associated with number of CAG repeats.

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3
Q

What are core motor features of HD?

A

1) Chorea
2) Bradykinesia
3) Dystonia
4) Incoordination
Other features include unintended weight loss, sleep and circadian rhythm disturbances, and autonomic signs.
Motor changes are involuntary, unwanted movements,. initially occur in distal extremities such as fingers and toes or small facial movements. Gait is unstable. Unwanted movements may become more axial/truncal over time.

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4
Q

What is the neuropathology of HD?

A

Loss of spiny cells in caudate nucleus.
Indirect basal ganglia thalamocortical circuitry is most affected
As the disease progresses, further degeneration is observed in areas connected to striatal circuits, including frontal and temporal areas
At death, brain volume can be decreased as much as 25%.

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5
Q

What are common MRI findings in HD?

A

Caudate atrophy - resulting in “box car” ventricles.

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6
Q

What is mean age of onset for HD?

A

Between 30-50 with range of 2 to 85 years.

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7
Q

Which country has highest prevalence of HD?

A

Venezuela

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8
Q

Are psychiatric problems a concern in HD?

A

Yes, and there can be a high suicide rate. Psychiatric symptoms include irritability (may be first sign), depression, anxiety, OCD-like symptoms, and psychosis.

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9
Q

What is the disease duration of HD?

A

Usually 17-20 years. Causes of death include pneumonia (related to dysphagia), heart disease, and suicide.

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10
Q

Which is first, cognitive problems or motor problems, in HD course?

A

Cognitive decline can be seen before first motor symptoms. Executive functioning is particularly affected.
Cognitive changes can be detected up to 15 years prior to clinical onset. Measures that appear most sensitive in the prodromal period are psychomotor performance and attention/working memory measures

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11
Q

What is the treatment for HD?

A

Chorea is treated with dopamine receptor blocking or depleting agents (e.g., rerserpine or tetrabenazine)

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12
Q

What is Lewy body dementia?

A

Progressive neurodegenerative disorder characterized by parkinsonism and cognitive/decline/dementia. LBD is the 2nd most common form of dementia after AD. 20-30% of patients with a neurodegenerative dementia have comorbid AD and LBD pathology.

It is thought to include 3 types of dementia:
Parkinson’s disease with dementia (PDD)
Diffuse Lewy body disease (DLBD)
Lewy body variant of Alzheimer’s disease (LBV)

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13
Q

What are Lewy bodies?

A

Abnormal aggregates of protein that form within nerve cell and other areas. Alpha synuclein is the main component of these Lewy bodies. They are usually dispersed throughout cortex and brainstem with or without Alzheimer-type neuropathological change.

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14
Q

What are other neuropathological changes associated with Lewy body disease?

A

Beyond placement of Lewy bodies throughout, the substantia nigra loses black appearance with cell loss and dopamine depletion, hippocampus may atrophy, and there is a depletion of cholinergic neurons in the basal forebrain.

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15
Q

What is a risk factor for DLB?

A

ApoE4 allele status

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16
Q

What would MRI show for DLB?

A

Nondiagnostic.

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17
Q

What would PET show for DLB?

A

Diffuse glucose hypometabolism, including disproportionate involvement of the occipital lobes.

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18
Q

What would SPECT/DAT show for DLB?

A

Low dopamine transporter (DaT) uptake in the basal ganglia.

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19
Q

What is the incidence of DLB?

A

DLB is responsible for 20% of all dementias, after AD.

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20
Q

What is the mean age of onset for DLB?

A

Late 50s for DLBD and 50s to 80s for LBV.

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21
Q

Who is more likely to be affected, males or females, by DLB?

A

Males.

22
Q

What can aggravate symptoms in DLB?

A

Neuroleptics can worsen or cause development of EPS. Levodopa can aggravate hallucinations.

23
Q

What is the survival time for DLB?

A

Average of 7 years and is typically shorter than AD.

24
Q

What is the symptom timeline course for DLB?

A

Onset of motor and cognitive symptoms within 1 year of each other, although parkinsonism may occur before or after cognitive deficits are present.
The overall course is slow progression, marked with frequent fluctuations (marked confusion with complete lucidity).

25
Q

What are the McKeith criteria?

A

They are for DLB diagnosis. Include:
1) Onset of motor and cognitive symptoms within 1 year of each other, although parkinsonism may occur before or after cognitive deficits are present.
2) Fluctuating cognition with marked variations in attention and alertness (almost like a delirium in some cases)
3) Recurrent visual hallucinations that are usually well formed
4) Parkinsonism
The presence of REM behavior disorder in addition to 2 core clinical features increases sensitivity of diagnostic accuracy. Supportive features include neuroleptic sensitivity and low dopamine transporter uptake in the basal ganglia on SPECT/PET. Other psychiatric symptoms (depression and delusions) and autonomic dysfunction may be present.

26
Q

How can DLB be treated?

A

Can be treated with donepezil, rivastigmine. LBD patients actually respond better than those with AD. Hallucinations can be treated with low dose antipsychotics but due to neuroleptic sensitivity this could results in worsening of motor symptoms.

27
Q

What are considerations to be made when treating DLB patients?

A

1) Level of supervision. May ultimately require 24/7 care.
2) Driving. May need to stop as visuospatial deficits and slowed processing speed progress.
3) Work. Disability may be needed in near future.
4) Capacity. Affairs should be put in order including living will and power of attorney.
5) Psychological and psychiatric issues. Hallucinations/psychosis, depression
6) Rehab. PT can help gait. voice training for hypophonia. cognitive compensatory strategies.

28
Q

What is Progressive Supranuclear Palsy?

A

Also known as Steel-Richardson-Olszeski syndrome. It is the most common PD+ syndrome and is a progressive neurodegenerative disorder. Involves erosion of subcortical structures as well as subcortical-cortical connections (particularly to prefrontal cortex) leading to triad of motor, cognitive, and emotional/personality symptoms.

29
Q

What are core diagnostic symptoms of PSP?

A

1) Vertical gaze palsy (difficulties looking down)
2) Axial rigidity
3) Postural instability with falls (often backward)
Other common symptoms are:
1) Dysarthria
2) Dysphagia
3) Gait disorder
4) Early cognitive impairment
5) Behavioral changes (apathy, depression, anxiety, pseudobulbar affect, neurobehavioral symptoms)

30
Q

What is the neuropathology of PSP?

A

Mostly a sporadic disease with no genetic forms
Dopamine depletion in substantia nigra, caudate, and putamen
Neuronal loss and gliosis in the globus pallidus, subthalamic nucleus, etc.
Neurofibrillary tangles and neuropil threads in basal ganglia, brainstem, forebrain
Dopaminergic, cholinergic, adrenergic neurotransmitter systems are affected
Disconnection of pathways from subcortical structures to the prefrontal cortex

31
Q

What are MRI findings for PSP?

A

Atrophy of the midbrain tegmentum (i.e., hummingbird sign)

32
Q

What are PET findings for PSP?

A

Hypometabolism in midline frontal regions, brainstem, and midbrain.

33
Q

What are SPECT findings for PSP?

A

Reduced dopamine transporter uptake bilaterally on DAT scan.

34
Q

Are there risk factors for PSP?

A

None really.

35
Q

What is the onset of PSP?

A

Typically in 60s, but can occur earlier.

36
Q

Can dementia occur in PSP?

A

Yes, 50-80% of cases have dementia

37
Q

What are other causes of morbidity in PSP beyond dementia?

A

Head injury and fractures related to falls.

Choking or aspiration related to swallowing problems.

38
Q

What is the survival rate for PSP?

A

Survival time is 6 to 9 years from symptom onset. Most common cause of death is respiratory arrest due to pneumonia or degeneration of brain stem respiratory centers.

39
Q

What determines severity of PSP?

A

Older age of diagnosis is associated with shorter survival time. Early presence of falls, bradykinesia, and/or impaired downward gaze predict shorter survival.

40
Q

Discuss more the presentation of PSP.

A

Usually presents as postural instability and falls (typically backwards). Tremor is usually absent. Oculomotor dysfunction is hallmark feature but may not be observed until a few years into the disease (around 3). This involves inability to look down on command, whereas spontaneous gaze may be intact since the associated lesion is at the level above the eye gaze nuclei in the brainstem. Cognitive dysfunction occurs early in the course. Late in stage of disease patients are dependent on wheelchair and may become mute. There are many subtypes of PSP, with some appearing more like PD, PPA, cerebellar, or corticobasal syndrome.

41
Q

What considerations do you have for patients with PSP?

A

1) Close supervision due to falls and poor judgment
2) Restrict driving due to vision and cog changes
3) Transition to disability
4) Consider capacity issues
5) Emotional incontinence issues. Antidepressants may be helpful.
6) May need to make modifications in home to reduce falls, change diet due to swallowing problems
7) PT and ST might be helpful.

42
Q

Can you treat PSP with donepezil, etc?

A

These medications do not work, or there is not enough evidence to suggest that they work.

43
Q

Can you treat PSP with Sinemet?

A

Provides some benefit, but not as much as it does for Parkinson’s disease, with respect to motor symptoms.

44
Q

What is cortical basal degeneration?

A 
It is a tauopathy with features that can overlap with PSP, FTD, PD, and AD. CBD is often reserved to characterize the presentation, including:
Asymmetric motor symptoms
Parkinsonism
Tremor
Limb dystonia
Gait abnormalities
Myoclonus
Alien hand phenomenon
Cortical sensory loss 
Dyspraxia
45
Q

What are early signs of cortical basal degeneration?

A

Early motor symptoms include “clumsiness” of affected limb. Apraxia typically begins in one limb and spreads to other limbs over time.
Early cognitive symptoms include impairments in language production, executive functioning, and attention/concentration

46
Q

What is the onset of CBD?

A

Typically in 60s.

47
Q

What is the course of CBD?

A

Usually insidious and progressive with death occurring 6-8 years following onset.

48
Q

What does neuroimaging show for CBD?

A

Focal, asymmetric cortical atrophy (particularly in frontal/parietal areas contralateral to the most affected side). Similar asymmetry would be observed with hypometabolism on PET.

49
Q

What is Capgras syndrome?

A

Well formed delusional disorder in which a person believes that a spouse, parent, friend, or other has been replaced by an identical looking imposter.

50
Q

What is reduplicative paramnesia?

A

Well formed delusional disorder in which person believes their home has been replaced by an identical looking home.

ABPP (20 decks)

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