Tourette Syndrome/Amyotrophic Lateral Sclerosis/Guillain-Barre

Question 1

echopraxia

Answer 1

mimicking gestures

Question 2

copraxia

Answer 2

obscene gestures

Question 3

copralalia

Answer 3

obscene words

Question 4

echolalia

Answer 4

repetition of words

Question 5

palilalia

Answer 5

repetition of word/phrase with increasing rapidity

Question 6

What might you expect to find on Tourette syndrome PE?

Answer 6

normal

Question 7

Tourette Syndrome: diagnostic criteria

Answer 7

1. Motor tics and phonic tics both need to be present (not necessarily together)
2. Tics throught the day (or intermittent and last 1 year)
3. Characteristics or severity must change over time
4. Start before age 18*

Question 8

What is 1st line treatment of mild, non-disabling tics?

Answer 8

Comprehensive behavioral therapy

Question 9

What is treatment for moderate to severe tics?

Answer 9

Clonidine or guanfacine

alpha adrenergic agonists

Question 10

Amyotrophic Lateral Sclerosis: Pathophysiology

Answer 10

Necrosis of the UMN and LMN in the motor cortex of brainstem and spinal cord

Question 11

What are signs (3) of UMN damage in ALS?

Answer 11

1. Spasicity
2. Stiffness
3. Hyperreflexia

Question 12

What are the signs (4) of LMN damage in ALS?

Answer 12

1. Progressive bilateral fasiculations
2. Muscle atrophy
3. Hyporeflexia
4. Muscle weakness

Question 13

dysarthria

Answer 13

difficulty speaking (bulbar so LMN)

Question 14

dysphagia

Answer 14

difficulty swalllowing (bulbar, so LMN)

Question 15

Which two voluntary muscles are spared in ALS?

Answer 15

voluntary eye muscles

urinary sphincter

Question 16

What is the first line treatment for ALS?

Answer 16

Riluzole (inhibits glutamate)

-modest benefit, only extends life by up to 6 months

Question 17

Acute inflammatory demylinating polyradiculopathy of peripheral (LMN)?

Answer 17

Guillian-Barre

Question 18

Describe the clincal presentation of Guillian-Barre Syndrome

Answer 18

1. Symmetric, progressive ascending weakness and parethesias
2. DTRs absent/depressed***
3. Autonomic dysfunction (ex. arrhthymias, BP instability)
4. No atrophy
5. Radicular pain
6. Ataxia affecting gait out of proportion with muscle weakness

Question 19

How is Guillian- Barre diagnosed?

Answer 19

Lumbar puncture: HIGH protein in CSF with normal WBC count

“albuminocytologic dissociation”

Question 20

What needs to be done to classify Guillian-Barre?

Answer 20

Nerve conduction studies

Question 21

Are corticosteroids helpful for Guillian barre?

Answer 21

NO

Question 22

Tx for guillian barre?

Answer 22

1. Immunotherapy (IVIG)

2. Plasmaphoresis

Question 23

What is the recovery sequence for Guillian-Barre?

Answer 23

Bulbar (speech, swallowing)—>LE–>DTR

Question 24

What are 3 indicators of poor prognosis in Guillian Barre?

Answer 24

1. Cranial nerve involvement
2. Intubation
3. Maximum disability at time of initial presentation