Motoneuron disease and Spinal Muscle Atrophy Flashcards
What percentage of ALS is familial?
a. 1 %
b. 5 %
c. 30 %
d. 50 %
e. it is always sporadic
b. 5 %
An elderly gentleman has recently noticed problems with walking, things falling
out of his hands… On examination, there are signs of upper and lower motor
neuron damage. The diagnosis is?
a. ALS
b. myasthenia gravis
a. ALS
IMAGE: clawed hand (interphalangeal flexion and metacarpophalangeal extension
in the 4th and 5th fingers) and atrophied 1st dorsal interosseus:
a. ALS
b. malformation of the n. ulnaris
b. malformation of the n. ulnaris
The patient is found to have muscle atrophy, fasciculations and a plantar
response in extension Which disease is most likely?
a. ALS.
a. ALS.
Spinal muscular atrophy type I (SMA I) is suspected in a newborn due to
hypotonia and fasciculations. Which is the best selection method for diagnosis?
a. electromyography
b. muscle biopsy and histological examination
c. magnetic resonance imaging
d. biochemical tests
e. genetic testing
e. genetic testing
Motor neurone disease is not characterised by:
a. in most cases, the disease starts in the extremities
b. patients show signs of both upper and lower motor neuron
impairment
c. a small percentage is accompanied by frontotemporal dementia
d. marked cognitive decline is common in the late stages of the disease
e. most patients die from respiratory failure or respiratory infection
d. marked cognitive decline is common in the late stages of the disease
Riluzole works by:
a. motorised tiles
b. glycine synapses
c. glutamate receptors
d. corticospinal route
e. centre for breathing in the pons
c. glutamate receptors
The preferred test for motor neurone disease:
a. evoked motor potentials
b. EMG
c. lumbar puncture
d. MRI)
b. EMG
Not found in ALS:
a. vertical nystagmus
b. the rest were lower and upper motor neuron signs
a. vertical nystagmus
Patients with ALS often have:
a. frontotemporal dementia
b. other dementias
c. other systems are not affected in ALS
a. frontotemporal dementia
. What is primary lateral sclerosis?
a. A form of ALS in which only the corticospinal tract is affected
a. A form of ALS in which only the corticospinal tract is affected
What does a person with a SOD1 mutation have?
a. ALS
a. ALS
Which is the most common disease affecting motoneurons in Europe?
a. Spinal muscular atrophy
b. ALS
c. Polymyositis
d. Hereditary spastic paraparesis
b. ALS
Which sign of pseudobulbar impairment is found in a patient with ALS?
a. clonic masseter reflex
b. the irresistible masseter reflex
c. gag reflex
d. palmomental reflex
a. clonic masseter reflex
What is the risk of a person with spinal muscular atrophy type II having a child with
the same condition if the partner is healthy?
a. Less than 1%
a. Less than 1%
The incidence of ALS is:
a. 2/1,000,000 per year
b. 2/100,000 per year
c. 20/100,000 per year
d. 200/100,000 per year
b. 2/100,000 per year
How many ALS patients do we have in Slovenia?
a. 10
b. 100
c. 1000
d. 10 000
e. cannot be assessed because the disease progresses too quickly
b. 100
In which form of motor neuron damage is only the upper motor neuron
damaged?
a. primary lateral sclerosis
b. progressive bulbar palsy
c. amyotrophic lateral sclerosis
d. progressive muscular atrophy
a. primary lateral sclerosis
What’s missing in motor neurone disease:
a. excessive salivation
b. excessive sweating
c. cramps
d. spasticity
e. pseudobulbar affect
b. excessive sweating
ALS is rarely affected:
a. the muscles that move the eyeballs
b. bulbar muscles
c. respiratory muscles
d. proximal arm muscles
e. distal arm muscles
a. the muscles that move the eyeballs
A disease that always affects the spinal cord:
a. multiple sclerosis
b. Binswanger’s disease
c. amyotrophic lateral sclerosis
d. MSA-P (multiple system atrophy with predominant parkinsonism)
e. Pick’s disease
c. amyotrophic lateral sclerosis
Not found in ALS:
a. fasciculations
b. atrophy of the tongue
c. horizontal nystagmus
d. scattering (irradiances) of reflexes
e. plantar response in extensia
c. horizontal nystagmus
