Motor neuron disease

Question 1

what is motor neuron disease?

Answer 1

a group of relentlessly progressive neurodegenarative conditions

Question 2

what is characteristic of MND?

Answer 2

only upper and or lower motor neurons are affected and there is sparing of the nerves relaying the sensory and autonomic signaling

Question 3

what is the sign of paralysis in UMN?

Answer 3

spastic

Question 4

what is the sign of muscle tone in UMN?

Answer 4

hypertonic

Question 5

what are the signs of the reflexes in UMN?

Answer 5

hyperreflexia (brisk reflex)

Question 6

do you see a babinski/hoffman signs?

Answer 6

yes

Question 7

do you see fasiculations in UMn?

Answer 7

no

Question 8

what is the sign of paralysis in LMN?

Answer 8

flaccid

Question 9

what is the sign of muscle tone in LMN?

Answer 9

hypotonic

Question 10

what is the sign of reflexes in LMN?

Answer 10

hyporeflexia or absent reflex

Question 11

do you see the babinski/hoffman’s sign. in LMN?

Answer 11

no

Question 12

do you see fasiculation in LMN?

Answer 12

yes

Question 13

what familial gene mutations have been identified?

Answer 13

protein superoxide dismutase on chromosome 21 but are few and far between

Question 14

what is associated with the overlap of ALS and frontotemporal dementia??

Answer 14

C9oRF hexanucleotide repeat expansion

Question 15

what is the peak incidence of MND?

Answer 15

50-75

Question 16

which gender is more affect by MND?

Answer 16

males

Question 17

which race of people are more affected?

Answer 17

caucasians

Question 18

what is the most common form of MND?

Answer 18

ALS

Question 19

what is the presentation of ALS?

Answer 19

simultaneous upper and lower motor neuron symptoms often asymmetrical involvement of limbs

Question 20

what are teh late stae symptoms of ALS?

Answer 20

dysarthria, dysphagia, dyspneoa, recurrent aspiration/pneumonia

Question 21

what is progressive musclar atrophy?

Answer 21

this presents as purely LMN pathology

Question 22

what is the least common phenotype of MND?

Answer 22

Primary lateral sclerosis

Question 23

what is primary lateral sclerosis?

Answer 23

presents purely with UMN signs

Question 24

what is progressive bulbar palsy?

Answer 24

LMN disease affecting CN 9,10 and 12

Question 25

what Cranial nerves are affected in Progressive bulbar palsy?

Answer 25

9,10,12

Question 26

what are the symptoms of Progresesive bulbar palsy?

Answer 26

dysphagia, dysarthria, tongue fasciculation and reduced jaw and gag reflex

Question 27

what are the causes of of Progressive bulbar palsy?

Answer 27

ALS, Guillian-Barre, Myasthenia Gravis

Question 28

what is pseudobulbar palsy?

Answer 28

UMN disease affect cranial nerves 9,10,12

Question 29

what cranial nerves are affected by Pseudobulbar palsy?

Answer 29

9,10,12

Question 30

what are the symptoms of pseudobulbar palsy?

Answer 30

dysphagia, slow sspeech and brisk hae jerk reflex

Question 31

what are the causes of Pseudobulbar palsy?

Answer 31

ALS, MS, Stroke

Question 32

what is the mainstay management of MND?

Answer 32

specialist MND servies and supportive care

Question 33

what is the management for communication needs/

Answer 33

speech and language therapy

Question 34

what is the management for nutritional needs?

Answer 34

dieticians, parentral feeding

Question 35

what is the management of respiratory needs?

Answer 35

BiPAP ventilation at night

Question 36

what is the pharmacological management of MND?

Answer 36

Riluzole

Question 37

what is the mechanism of action of Riluzole?

Answer 37

blocks glutaminergic neurotransmissionin the CNS

Question 38

on average how long does Riluzole extend life by?

Answer 38

3 months

Question 39

what are the complications of MND? (6)

Answer 39

Facial weakness (haggard appearance)
Ptosis, opthalmoplegia and bilateral “Christmas tree-like” cataracts
HOllowing of the temples due to temporalis muscle wasting and atrophy of the jaw muscles
early frontal balding