Pure motor disorders

Question 1

**5 common Sx of MNJ disorder

Answer 1

1. pure motor Sx
2. fluctuating ptosis
3. diplopia
4. bulbar Sx
5. limb weakness

Question 2

what is myasthenia gravis

Answer 2

autoimmune dis. of NMJ

- bimodal age of onset 50

Question 3

clinical features of myasthenia

Answer 3

• ptosis
• opthalmoparesis
• facial muscle weakness
• bulbar weakness
• limb weakness
• dyspnea
  fluctuating and fatiguable weakness

Question 4

pathophys of myasthenia

Answer 4

ABs to Ach receptors

Question 5

3 testing steps to diagnose myasthenia

Answer 5

1. bedside test (tensilon test)
2. electrodiagnostic test
   - rep. nerve stim
   - single fiber EMG
3. serological test
   - Ach ABs

Question 6

what is seen in rep. nerve stim

Answer 6

fatigue in muscle with stimulations

Question 7

3 classes of myasthenia mgmt

Answer 7

1. symptomatic
   - mestinon
   - plasma exchange
   - IVIG
2. DMARD
   - corticosteroids
   - azathioprine
   - cyclosporine
3. disease modifying surg
   - thymectomy

Question 8

MG prognosis

Answer 8

mortality less than 5% now

Question 9

*** what is a pure motor syndrome with proximal upper and lower extremity weakness

Answer 9

myopathy

Question 10

4 screening bloods for myopathy

Answer 10

1. CK
2. TSH
3. ESR/CRP
4. ANA

Question 11

4 additional myopathy investigations

Answer 11

1. EMG and nerve conduction
2. MRI of proximal muscles
3. muscle biopsy
4. genetic testing

Question 12

7 broad cats. of muscle diseases

Answer 12

1. musc. dystophy
2. congential
3. mitochondrial
4. metabolic
5. endocrine
6. meds/toxins
7. inflammatory

Question 13

***what is a progressive pure motor syndrome involving mixed upper and lower motor neuron findings in bulbar and limb muscles

Answer 13

diffuse motor neuron disorder

Question 14

3 subtypes of motor neuron disease

Answer 14

1. upper and lower neuron
2. lower
3. upper

Question 15

what is epi of ALS

Answer 15

peak in 50-75

- 3 year survival

Question 16

what is ALS

Answer 16

progressive disorder of UMN and LMN

• pyramidal cells in motor cortex
• alphamotor neurons

Question 17

2 types of ALS onset

Answer 17

1. spinal
   - upper limb more common than lower
   - distal focal limb weakness
   - typically unilateral
2. bulbar
   - CN ,7,910

Question 18

*key feature of ALS

Answer 18

paradoxial mixture of UMN and LMN findings in involved region

Question 19

what is needed for ALS diagnosis

Answer 19

• progressive disorder of UMN and LMN
• supportive EMg
• nothing else to exlpain findings

Question 20

natural Hx of ALS

Answer 20

relentless progressive disease

• quadrapelgia
• resp muscle involvement and ultimitely failure