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Neuro > Tourette & ALS > Flashcards

Tourette & ALS Flashcards

1
Q

Describe Tourette Syndrome

A
  • motor and phonic tics
  • onset during childhood (2-15 with peak 11-12)
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2
Q

Describe the rule of 3rds with Tourette Syndrome

A

3rd will resolve
3rd will remain the same
3rd will progress

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3
Q

What is the functional and chemical pathophysiology of TS

A

functional: disturbances in the mesolimbic circuit leading to disinhibition

chemical: dopamine and serotonin excess/pathways

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4
Q

What are some of the comorbidities associated TS

A

ADHD, OCD, ODD, anxiety/mood, sleep disorders

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5
Q

Describe motor tics

A

eye blinking, face grimace, shoulder shrug, head jerk, kicking, jumping, inappropriate gestures, SIB

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6
Q

Describe phonic tics

A

grunt, bark, moan, throat clear, sniffle, bad words, echoing, repeating

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7
Q

Describe the arc of a tic

A
  • feeling it build, an urge to complete the tic
  • doing the tic
  • feeling of relief
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8
Q

Describe the DSM-V criteria for TS

A

Requires all 4 to be met
- multiple motor and 1+ vocal tics
- may wax and wane but >1yr
- onset before age 18
- not secondary to substances or other medical conditions

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9
Q

Tx of TS

A
  • education
  • based on QOL
  • combo tx of tics and comorbidities
  • decrease tics (HRT, CBIT, CBT)
  • possible meds (SSRI)
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10
Q

Describe why kids with TS are more likely to have academic underachievement

A
  • lack of education on the teachers/schools/classmates end
  • comorbidities limiting
  • distracted by tics
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11
Q

Describe ALS

A

progressive terminal neurodegenerative disorder that causes muscle weakness, disability, associated with the upper and lower motor neurons

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12
Q

What is the most common initial symptom of ALS

A

asymmetric hand weakness (80%)

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13
Q

What physical exam signs are positive in upper motor neuron ALS

A

Babinski, Hoffman, pronator drift

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14
Q

What are the characteristics of UMN ALS

A

Limb/Trunk: slowness of movement, incoordination, stiffness, hyperreflexia, spasticity

Bulbar: dysphagia, dysarthria, jaw stiffness, clonus, laughing/crying

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15
Q

What are characteristics of LMN ALS

A

Limb/Trunk: weakness, atrophy, fasciculation, fibrillation on EMG, foot drop, poor rise from chair

Bulbar: poor eye/lip closure, hoarseness, weak mouth muscles

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16
Q

What is the 1st line medication for ALS

A

Riluzole

17
Q

What is the median survival rate of ALS

A

3-5 years

18
Q

What is ALS called in the UK

A

Motor Neuron Disease

19
Q

What is ALS characterized by at the cellular level

A

motor neuron degeneration and death with gliosis replacing lost neurons/scarring

20
Q

Approximately what % of ALS cases are genetic

A

10%

21
Q

What are some potential pathogeneses of ALS

A
  • reduced uptake of glutamate leading to excitotoxicity
  • mutations in genetic components/dysregulated RNA metabolism
  • defective axonal transport
22
Q

What are the structural associations of ALS

A
  • primary motor cortex (B4)
  • corticubulbar tract or corticospinal (pyramidal) tract

Corticobulbar: CN 5, 7, 9-12 (mastication, facial, throat, tongue)
Corticospinal: skeletal muscles of trunk & limbs

23
Q

Describe the ALS pattern of spread for unilateral arm onset

A
  1. contralateral arm
  2. ipsilateral leg
  3. contralateral leg
  4. bulbar muscles
  5. respiratory muscles months-years later
24
Q

Describe the ALS pattern of spread for bulbar onset

A
  1. one arm
  2. contralateral arm
  3. respiratory muscles months-years later
25
Q

What does an MRI of the brain typically show in ALS (while usually normal)

A

increased signal in the corticospinal tracts and hypointensity of the primary motor cortex

26
Q

When should a muscle biopsy be performed in ALS

A

never

27
Q

Upper motor neuron lesions are associated with what symptoms

A
  • slowness
  • stiffness
  • incoordination
  • hyperreflexia
28
Q

Lower motor neuron lesions are associated with what symptoms

A
  • weakness
  • atrophy
  • amyotrophy
  • fasciculations
29
Q

Describe what happens to muscle mass in UMN vs LMN ALS

A

UMN: slight decrease
LMN: major decrease

30
Q

describe what happens to muscle strength in UMN vs LMN ALS

A

UMN: spastic
LMN: flaccid

31
Q

describe what happens to muscle tone in UMN vs LMN ALS

A

UMN: increased
LMN: decreased

32
Q

describe what happens to deep tendon reflexes in UMN vs LMN ALS

A

UMN: hyperreflexic
LMN: hyporeflexic

33
Q

are fasciculations present in UMN or LMN ALS

A

LMN ALS

34
Q

are fibrillations present in UMN or LMN ALS

A

LMN ALS

35
Q

Which form of ALS (UMN or LMN) are the following tests positive in: Babinski, Hoffman, pronator drift

A

UMN ALS

Neuro (22 decks)

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