Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

DM Neuro 1 > Other BG disorders > Flashcards

Other BG disorders Flashcards

1
Q

List disorders included in atypical parkinsonisms

A
  1. Progressive Supranuclear Palsy
  2. Multiple systems atrophy
  3. Lewy Body Dementia
  4. Corticobasal Degeneration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

List types/categories of secondary parkinsonisms

A
  1. Vascular
  2. Metabolic
  3. Toxic/Drug-Induced
  4. Infectious
  5. Tumor/mass
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Describe the clinical features of vascular parkinsonism

A
  1. Clinical features are caused by small CVAs impacting many areas including BG and motor system regions
    • acute or delayed progressive onset of parkinsonims = 1 year after stroke with evidence of infarcts in or near BG
    • insidious onset, extensive subcortical white matter lesions, + parkinsonism features
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

describe the S/S and treatment for vascular parkinsonism

A
  1. Common symptoms: symmetrical lower-body parkinsonism
    • Gait unsteadiness
        • freezing, festinating
    • Bradykinesia, Akinesia, Hypokinesia
    • Absence of tremors
    • Rigidity
    • Pyramidal signs
  2. Treatment: CVA management
    • minimal responses to levadopa
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

relevant background info for progressive supranuclear palsy (PSP)

A
  1. most common atypical parkinsonism
  2. Incidence → 1.29 per 100,000
    • increases to 14/7 per 100,000 ages 80-89
  3. Prevalence = 6 out of 100,000
  4. average age of onset = 60s
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

describe the pathophysiology of PSP

A

Unknown

Atrophy of frontal convexity, subthalamic nucleus, thalamus, and midbrain structures as well as depigmentation of substantia nigra and other brainstem nuclei

accompanied by rapid astrogliosis and neuronal loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

how is PSP diagnosed?

A
  1. typcially diagnosed in mid 60s
  2. early on, clinical exam serves a primary mode of dx
  3. MRI:
    1. frontal lobe, subthalamic, putamen atrophy
    2. Morning Glory Sign
    3. Hummingbird Sign
  4. Misdiagnosis early → depression, dementia, PD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

List S/S of PSP

A
  1. Gait disturbances
  2. Falling backwards suddenly
  3. Motor Impulsivity → Rocket Sign
  4. Severe axial rigidity
  5. Supranuclear opthalmoplegia
  6. Dysphagia and dysarthria
  7. Frontal cognitive dysfunction
  8. sleep disturbances
  9. Surprised expression → Fixed Mona Lisa stare
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what is supranuclear opthalmoplegia?

A
  1. limited veritcal eye movement
  2. loss of convergence
  3. impaired vertical saccades
  4. loss of visual acuity
  5. loss of eyelid control

*often first sign that helps distinguish PSP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

how is PSP treated?

A

No effective trx for PSP

  1. Anti-PD medications may be slightly effective, but tends to be minimal and short-lasting
    • 40% response rate
  2. Amantadine, Botulinum injections, Anti-depressants sometimes used
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

describe the prognosis for PSP

A
  1. Rapidly progressive disease
    • severe disability in 3-5 years of onset; mortality commonly seen 5-8 years
    • if responsive to meds, can live up to decade after onset
    • serious complications common:
      • pneumonia
      • falls w/injury
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Describe Multiple Systems Atrophy

A
  1. Rare neurodegenerative disorder with autonomic dysfunction
  2. two subtypes:
    • MSA - P → primary and earliest symptoms resemble parkinsonism
    • MSA - C → cerebellar subtypes, primary symptoms feature ataxia, dysarthria, abnormal eye movements
  3. Onset = early 50s
    • rarely diagnosed past 70 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Describe the pathophysiology for Multiple Systems Atrophy

A

Cause = unknown

  1. Distinguishing factor found to be accumulation of proteins in glial cells
    • most involvement seen in oligodendrocytes
  2. Regions most involved: BG, cerebellum, pons, inferior olivary nucleus, intermediolateral column of thoracic and sacral spinal cord
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

MSA diagnosis

A
  1. Clinical examination
  2. PET scan, DaTscan
  3. MRI
    • Will see more widespread damage
    • Hot cross bun sign
  4. Often misdiagnosed with PD
    • often corrected once pts do not respond to dopamine therapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is the premotor phase of MSA?

A

disorder moths to years before first motor symptoms appear

Symptoms:

  1. sexual dysfunction
  2. urinary urge incontinence
  3. OH
  4. inspiratory stridor
  5. REM sleep behavior
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

List S/S of MSA

A
  1. Autonomic symtpoms
  2. Bradykinesia, rigidity, tremors
  3. Gait and limb ataxia
  4. Head/oral dyskinesias and dystonias
    • “coat-hanger pain”
  5. Antecollis
  6. Pisa Syndrome
  7. Oculuomotor disturbances
    • MCA-C > MSA-P
  8. Speech deficits
  9. Sleep disorders
  10. Cognitive deficits RARE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what types of autonomic symptoms may be present with MSA?

A
  1. OH
  2. supine HTN
  3. Urinary and sexual dysfunction
  4. respiratory and breathing problems
  5. sleeping issues
  6. impotence is often first symptom in males
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

describe how the symptoms bradykinesia, rigidity, and tremors in MSA differ PD

A
  1. More symmetric appearance early on than PD
  2. Tremors:
    • higher frequency
    • lower amplitude
    • can have jerky, stimulus sensitive, myoclonic component
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what is coat-hanger pain?

A

pain in neck and shoulder when standing up

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what is antecollis?

A

largely due to neck dystonia and results in neck flexed and head down/forward

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what is Pisa syndrome?

A

abnormal posture in which the body is leaning to one side, form of axial dystonia

sig postural instability

frequent falls

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what types of speech deficits may be seen in MSA?

A

often mild, quivering voice early on

becomes unintelligible later stages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

how is MSA treated?

A

no effective treatment

  1. anit-PD meds only effective in about 1/3 of pts, but used with caution
    • can lead to worsening OH
    • response usually lasts <2-3 years
  2. meds are mainly supportive therapy for symptom management
  3. surgical intervention to prolong life in the form of tracheostomy, often included in later stages of disease gastrostomy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Describe MSA prognosis

A
  1. 50% of pts require walking aids within 3 yrs after onset of motor symptoms
  2. 60% become wheelchair-dependent after 5 years
  3. average time to becoming dependent/bedridden is 6-8 yrs
  4. Mortality ranges from 5-10 years after onset of symptoms
    • common causes → cardiorespiratory failure, urosepsis, sudden death
  5. cerebellar phenotype and later onset of automonic symptoms predict slower disease progression
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is Lewy Body Dementia?

A

presence of dementia, prominent hallucinations and delusions, fluctations in alterness, and gait and balance disorder

third most common type of progressive dementia

men > women (4:1)

26
Q

describe the pathophysiology of LBD

A

cause = unknown

abnormal proteins deposits (Lewy Bodies) develop in nerve cells

deposits will form throughout cerebral cortex (temporal > frontal = parietal) and brainstem, but see sig deposits in olfactory cortex, limbic cortex, hipppocampus, BG and midbrain

27
Q

LBD risk factors and diagnosis

A
  1. Risk factors
    • >60 years old
    • Male > female
    • certain comorbidities (PD, REM sleep behavior disorder)
    • Family history of LBD or PD
  2. Diagnosis
    • clinical diagnosis
    • post-mortem autopsy only definitive way to confirm presence of LB
28
Q

LBD S/S

A
  1. Cognitive decline early
  2. Behavioral and Personality changes
    • depression, apathy, anxiety, agitation
  3. PD like movement disorders
  4. Autonomic dysfunction
    • most commonly see hypotension, bradycardia, and digestive dysregulation
  5. loss of smell
  6. Sleep difficulties
29
Q

describe cogntive symptoms in LBD

A
  1. Dementia
    • attention, visuospatial, executive fxn
    • memory okay early stages
  2. Fluctuates between confusion and coherence
  3. Hallucinations, paranoid ideation
30
Q

what does the symptom “sleep difficulties” in LBD include?

A
  1. REM sleep behavior disorder
  2. Insomnia
  3. Excessive daytime sleepiness
  4. Restless leg syndrome
31
Q

how is LBD treated?

A

no effective trx for LBD

  1. largely symptom management
    • cholinesterase inhibitors
      • cognitive deficits, used commonly w/Alz
    • Antipsychotics
      • need to monitor closely for serious AE
    • Antidepressants
    • Clonazepam (used for anxiety)
32
Q

list serious AE associated with antipyschotics that you need to monitor for

A
  1. sudden changes in consciousness
  2. impaired swallowing
  3. acute confusion
  4. episodes of delusions or hallucinations
  5. appearance or worsening of PD symptoms
33
Q

what is the prognosis for LBD?

A
  1. Mortality 5-8 years after onset of symptoms
    • some variability in this (can survive up to 20 years)
34
Q

Describe Corticobasal degeneration

A
  • rarest of atypical parkinsonisms
  • asymmetric parkinsonism symptoms + early cog deficits
  • (eventually progresses to bilateral symptoms)
  • onset 60-80 years
  • Women > Men
35
Q

Describe the pathophysiology for corticobasal degeneration

A
  1. Cause = unknown
  2. Focal cortical and substantia nigra neuronal loss
    • glial lesions, most commonly involving astrocytes
    • neurons give ballooned appearance
    • gives appearance of over-pronounced sulci
      • “brain shrinkage”
36
Q

List CBD S/S

A
  1. Parkinsonism symptoms
    • Bradykinesia, akinesia, dyskinesia, disequilibrium, rigidity, dystonia
  2. Cog impairments - tend to present earlier than PSP
  3. Visuospatial impairments
  4. Apraxia
  5. Myoclonus
  6. Dysphagia
  7. Progressive aphasia
  8. Apraxic speech
37
Q

what types of cog impairments may be present in CBD?

A
  1. dementia
  2. Alzheimer’s disease
  3. Frontal behavior-spatial syndrome
    • clinical triad
      • executive dysfunction
      • behavioral or personality changes
      • visuospatial deficits
38
Q

How is CBD treated?

A

no effective trx or cure for CBD

treatment is largely supportive and symptom-dependent

39
Q

what is the prognosis for CBD?

A

prognosis is variable

mean survival of 6-8 years from symptom onset

however, multiple cases w/survival >10 yrs have been reported

40
Q

What is dystonia?

A

hyperkinetic movement disorder characterized by involutnary muscle contractions that cause slow, repetitive movements or abnormal postures

41
Q

what is the typical onset for dystonia?

A

childhood and early adulthood

early-onset → typically begins w/limb involvement

adult-onset → typically begins w/neck and/or facial involvement

42
Q

what are the 5 classifications for dystonia?

A
  1. focal → 1 body region
  2. segmental → 2 continous body regions
  3. multifocal → 2 non-continous body regions
  4. generalized → invovle axial involvement and 2 other areas in extremity
  5. hemi-dystonia → one half of body involved
43
Q

describe the pathophysiology of dystonia

A
  1. cause = typically idiopathic, some cases genetic or acquired
  2. pathophysiology is not widely understood in idiopathic dystonia. No easily ID damage can be found on imaging.
    • thought to be due to disruption within the basal ganglia, but for reasons unknown
44
Q

List S/S of dystonia

A

typically patterned, twisting, may be tremulous

  1. Common initial symptoms
    • sporadic foot cramping and/or toe dragging or excessive inversion during swing phase
    • severe hand cramping w/writing
  2. General symptoms
    • can see eyelid dystonias causing excessive and rapid blinking or inability to open eyes
    • neck and trunk can be impaired, leading to sig postural abnormalities
    • limb involvement frequently leads to severe contractures
45
Q

what are the characteristics of dystonic movements?

A
  1. often will be intermittent in early stages, only appearance during voluntary movements or stress
    • later stages tends to be constant whether at rest or in activity
  2. throughout all strages is shown to be worsened w/activity and improves w/rest
  3. Can be circumstantial
    • may only affect one specification of movement, while allowing others to occur unimpeded
      • ex: muscian w/dystonia unable to play instrument but can write easily
46
Q

how is dystonia treated?

A
  1. Pharmacologically
    • focal → botox
    • anticholinergic agents, GABAergic agents, and dopaminergic agents have shown to have some effect on managing symptoms for more invovled classifications of dystonia, though mixed results
  2. Surgical
    • deep brain stimulation (DBS)
      • when meds fail or AE too severe
    • Thalamotomy, pallidotomy, anterior cervical rhizotomy may be performed
47
Q

what is the prognosis for dystonia?

A
  1. considered to be progressive disease, though HIGH variability in what that looks like person to person
  2. childhood onset more likely to spread to other body regions
48
Q

what is tardive dyskinesia?

A

drug-induced disorder characterized by involuntary and uncontrolled stiff, jerky movements of face and body

  • amphetamine, meth, haloperidol
  • phenothiazines (neuroleptic for schizophrenia)
49
Q

describe the etiology of tardive dyskinesia

A
  1. ~500k cases in US
  2. risk factors:
    • age (>55 years)
    • older women
    • amount of drug ingested
    • long-term use of drug
    • alcohol or drug abuse
    • black and Asain populations
50
Q

how is tardive dyskinesia diagnosed?

A
  1. review of medication history
  2. clinical exam
51
Q

list the S/S of tardive dyskinesia

A
  1. slow, gradual onset of symptoms after ingestion of med
  2. dyskinesia of face most common
    • grimacing, sticking out tongue, sucking or fish-like movements common
  3. choreoathetoid or dystonic movements in limbs
  4. abnormal postural tone and adjustments
    • increased trunk extension
    • increased lordosis
    • neck flexion and rotation
52
Q

how is tardive dyskinesia treated?

A
  1. cessation or dosage adjustment of caustive med
  2. currently 2 FDA-approved meds
53
Q

what is the prognosis for tardive dyskinesa?

A

highly variable

  1. typically see an irreversible course, with long-term presence of symptoms
  2. some pts can see symptoms reversed w/cessation of meds
54
Q

what is Huntington’s Disease?

A

genetic, progressive, neurodegenerative disease characterized by uncontrolled movements, cog dysfunction and behavioral changes due to hyperactivity of basal nuclei circuitry

55
Q

T/F: Huntington’s Disease is considered to be common

A

FALSE
rare disease

56
Q

describe the onset and prevelance of Huntington’s disease

A

onset → 30s-50s

(possible juveline onset less than 20 years)

late onset less common

more common in Caucasian populations of Western European descent than those of Asian or African American ancestry

57
Q

describe the pathophysiology of Huntington’s Disease

A
  1. cause = chromosomal abnormality
    • DOMINANT autosomal trait
  2. Normal genes produce protein called huntingtin however in HD that protein is cut into smaller, toxic fragments which accumualte in neurons
    • leads to disruption of normal neuronal function, atrophy, neuronal death
    • severe loss of neurons seen in caudate and putamen
  3. early stages of HD characterized by loss of prejection neurons of the striatum in the indirect (NO-GO) pathways, causing disinhibition of thalamus
58
Q

how is HD diagnosed?

A
  1. Review of family history
  2. genetic testing:
    • predictive testing
    • confirmatory testing
    • prenatal testing
  3. Clinical exam
  4. Radiographic evidence
    • PET, MRI, fMRI, CT scan
59
Q

List HD S/S

A
  1. movement, cognitive and pyschological disorder
  2. choreform movements
    • involuntary, rapid and jerky movements
    • extremities, trunk, face
  3. Motor impersitance
    • common ex: “Fly-catcher’s tongue”
  4. Dystonia
  5. Ataxia
  6. EOM and VS impairments
  7. Speech and swallowing deficits
  8. Impaired memory, attention, executive function
  9. Mood changes, depression, anxiety, uncharacteristic anger and irritability
  10. As disease progresses, athetosis, akinesia, and bradykinesia develop
60
Q

HD progresses in what general stages?

A
  1. Pre-manifest
  2. early stage
  3. middle stage
  4. late stage
61
Q

how is HD treated?

A
  1. no effective trx for HD
    • largely symptom management
    • generally pharmaceutical interventions are not started until chorea interferes w/function
62
Q

what is the prognosis for HD?

A

mortality → 15-25 years after onset

cause often respiratory failure

suicide common

DM Neuro 1 (16 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions