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

17
Q

What is the median survival rate of ALS

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

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
What does an MRI of the brain typically show in ALS (while usually normal)
increased signal in the corticospinal tracts and hypointensity of the primary motor cortex
26
When should a muscle biopsy be performed in ALS
never
27
Upper motor neuron lesions are associated with what symptoms
- slowness - stiffness - incoordination - hyperreflexia
28
Lower motor neuron lesions are associated with what symptoms
- weakness - atrophy - amyotrophy - fasciculations
29
Describe what happens to muscle mass in UMN vs LMN ALS
UMN: slight decrease LMN: major decrease
30
describe what happens to muscle strength in UMN vs LMN ALS
UMN: spastic LMN: flaccid
31
describe what happens to muscle tone in UMN vs LMN ALS
UMN: increased LMN: decreased
32
describe what happens to deep tendon reflexes in UMN vs LMN ALS
UMN: hyperreflexic LMN: hyporeflexic
33
are fasciculations present in UMN or LMN ALS
LMN ALS
34
are fibrillations present in UMN or LMN ALS
LMN ALS
35
Which form of ALS (UMN or LMN) are the following tests positive in: Babinski, Hoffman, pronator drift
UMN ALS