Neuromuscular diseases

Question 1

Which symptom is not characteristic for Myasthenia gravis?
A) diplopia
B) ptosis
C) dysarthria
D) aphasia
E) muscle weakness worsening with physical exercise

Answer 1

D) aphasia

Question 2

First-choice treatment in Myasthenia gravis:
A) atropin
B) acetylcholinesterase inhibitors
C) steroid
D) plasma exchange therapy
E) vitamins

Answer 2

B) acetylcholinesterase inhibitors

Question 3

Motor neurone disease:
A) Eaton–Lambert syndrome
B) Amyotrophic lateral sclerosis
C) Duchenne muscular dystrophy
D) Guillain–Barré syndrome

Answer 3

B) Amyotrophic lateral sclerosis

Question 4

Which symptom is not characteristic for Amyotrophic lateral sclerosis?
A) Babinski-reflex (extensor plantar reflex)
B) atrophy of the small hand muscles
C) fasciculation
D) dysphagia
E) paraesthesia

Answer 4

E) paraesthesia

Question 5

Which is not characteristic for Guillain–Barré syndrome?
A) albuminocytologic dissociation in the CSF
B) ascending paresis
C) cranial nerves are never affected
D) segmental demyelinisation
E) mortality is under 3%

Answer 5

C) cranial nerves are never affected

Question 6

When to suspect polymyositis?
A) numbness with paresis
B) paresis starts in the proximal limb muscles
C) normal erythrocyte sedimentation rate
D) paresis in the proximal limb muscles along with fever and pain

Answer 6

D) paresis in the proximal limb muscles along with fever and pain

Question 7

Which is not characteristic for polymyositis?
A) weakness of the proximal muscles
B) weakness of the external ocular muscles
C) weakness of the muscles of the neck
D) muscle pain

Answer 7

B) weakness of the external ocular muscles

Question 8

Usual onset of facio-scapulo-humeral muscular dystrophy:
A) first years of life
B) 2nd–3rd decade
C) 4th–5th decade
D) 6th decade

Answer 8

B) 2nd–3rd decade

Question 9

Which is not characteristic for myasthenia gravis?
A) damage of the synaptic folds
B) damage of the ACh-receptors
C) damage of the ACh-containing vesicles
D) disturbance of the neuromuscular transmission

Answer 9

C) damage of the ACh-containing vesicles

Question 10

Which symptom is not characteristic for myasthenia gravis?
A) pathologic fatigue of muscles
B) ocular muscles are often affected
C) bulbar muscles are often affected
D) pathologic reflexes

Answer 10

D) pathologic reflexes

Question 11

Which symptom is not provoked by muscarin?
A) nausea
B) salivation
C) fasciculation
D) bronchial secretion
E) vertigo

Answer 11

C) fasciculation

Question 12

Which is not characteristic for Duchenne muscular dystrophy?
A) X-linked recessive inheritance
B) loss of ambulation at 25–30 years of age
C) selective damage of the muscles
D) markedly high CK levels at early phases
E) absence of dystrophin in skeletal muscles

Answer 12

B) loss of ambulation at 25–30 years of age

Question 13

Frequent symptom in ALS:
A) dementia
B) agnosia
C) problems with urination
D) all of the above
E) none of the above

Answer 13

E) none of the above

Question 14

Which disease is associated more frequently with polymyositis?
A) adrenoleukodystrophy
B) Lyme disease
C) SLE
D) viral hepatitis

Answer 14

C) SLE

Question 15

Which is not true for lower motor neuron disease?
A) individual atrophy of the muscles
B) missing or decreased deep tendon reflexes
C) fasciculation
D) problems with urination

Answer 15

D) problems with urination

Question 16

A 25-year old female complains about gait disturbance for two months. Walking on stairs is most problematic. Laboratory tests reveal increased ESR, CK and gamma-globulin levels. What is the most likely diagnosis?
A) poliomyelitis
B) polymyositis
C) Coxsackie-infection
D) autosomal recessive muscular dystrophy
E) myasthenia gravis

Answer 16

B) polymyositis

Question 17

Which autoimmune disease may be associated with polyneuropathy?
1) Systemic Lupus Erythematosus
2) rheumatoid arthritis
3) Wegener’s-granulomatosis
4) Sjögren-syndrome

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 17

E) all of the answers are correct

Question 18

Specific to the Becker’s muscular dystrophy:
1) X-linked recessive inheritance
2) the onset of symptoms is about between 5-15 years of age
3) high serum CK values
4) the dystrophin protein in the muscle is abnormal

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 18

E) all of the answers are correct

Question 19

In myasthenia gravis:
1) in the neuromuscular junction, the postsynaptic acetylcholine receptors are impaired
2) autoimmune disease
3) thymectomy can be an effective therapy
4) often a malignant tumor lies in the background

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 19

A) 1st, 2nd and 3rd answers are correct

Question 20

It contains acetylcholinesterase inhibitor:
1) pyridostigmin/Mestinon®
2) nimesulid/Mesulid®
3) neostigmin/Stigmosan®
4) urapidil/Ebrantil®

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 20

B) 1st and 3rd answers are correct

Question 21

Common symptoms of ALS and acute Guillain-Barré syndrome:
1) paresthesia
2) pathological reflexes (pyramidal signs or primitive reflexes)
3) hereditary disorder
4) muscle weakness

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 21

D) only 4th answer is correct

Question 22

The typical liquor of acute Guillain-Barré syndrome is characterised by:
1) increased protein value
2) decreased protein value
3) normal cell count
4) increased cell count

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 22

B) 1st and 3rd answers are correct

Question 23

Therapy for acute Guillain-Barré syndrome:
1) steroid
2) vitamins (B1 vitamin)
3) azathioprine
4) plasma exchange therapy

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 23

C) 2nd and 4th answers are correct

Question 24

The neurological status of the Guillain-Barré syndrome may include:
1) paraparesis
2) hypo- or areflexia
3) hypaesthesia
4) diplegia facialis

A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct

Answer 24

E) all of the answers are correct

Question 25

Which of the following diseases/states may cause myoglobinuria: 1) ischemic muscle destruction 2) status epilepticus 3) excessive muscle work 4) burning illness A) 1st, 2nd and 3rd answers are correct B) 1st and 3rd answers are correct C) 2nd and 4th answers are correct D) only 4th answer is correct E) all of the answers are correct

Answer 25

E) all of the answers are correct

Question 26

Factors in the etiology of lateral amyotrophic sclerosis (ALS): 1) glutamate-excitotoxicity 2) anomalies of the cerebral vascular system 3) free radical-toxicity 4) immunological factors 5) disturbance of the protein homeostasis A) 1st, 2nd and 3rd answers are correct B) 1st, 3rd and 4th answers are correct C) 2nd, 3rd and 4th answers are correct D) 2nd, 3rd and 5th answers are correct E) 2nd, and 4th answers are correct F) 3rd, 4th and 5th answers are correct

Answer 26

B) 1st, 3rd and 4th answers are correct

Question 27

The differential diagnosis of amyotrophic lateralsclerosis (ALS) includes the following disease(s): 1) multiple sclerosis 2) multifocal motoric neuropathy 3) compression of the cervical spinal cord 4) cervical spondylosis 5) funicular myelosis A) 1st, 2nd and 3rd answers are correct B) 1st, 3rd and 4th answers are correct C) 2nd, 3rd and 4th answers are correct D) 2nd, 3rd and 5th answers are correct E) 2nd, and 4th answers are correct F) 3rd, 4th and 5th answers are correct

Answer 27

C) 2nd, 3rd and 4th answers are correct

Question 28

Plasma exchange can be used with good effect in: 1) inclusion body myositis 2) myasthenia gravis 3) spinal muscle atrophy 4) Guillain–Barré-syndrome 5) amyotrophic lateralsclerosis (ALS) A) 1st, 2nd and 3rd answers are correct B) 1st, 3rd and 4th answers are correct C) 2nd, 3rd and 4th answers are correct D) 2nd, 3rd and 5th answers are correct E) 2nd and 4th answers are correct F) 3rd, 4th and 5th answers are correct

Answer 28

E) 2nd and 4th answers are correct

Question 29

It can be helpful in the diagnosis of myasthenia gravis: 1) clinical symptoms 2) somatosensory evoked potential 3) repetitive stimulation 4) chest CT 5) muscle biopsy A) 1st, 2nd and 3rd answers are correct B) 1st, 3rd and 4th answers are correct C) 2nd, 3rd and 4th answers are correct D) 2nd, 3rd and 5th answers are correct E) 2nd and 4th answers are correct F) 3rd, 4th and 5th answers are correct

Answer 29

B) 1st, 3rd and 4th answers are correct

Question 30

In the Eaton-Lambert syndrome, plasmapheresis may be effective because the basis of the disease is the formation of antibodies against the acetylcholine receptor. A) both the statement and the explanation are true and a causal relationship exists between them; B) both the statement and the explanation are true but there is no causal relationship between them; C) the statement is true, but the explanation is false; D) the statement is false, but the explanation itself is true E) both the statement and the explanation are false

Answer 30

C) the statement is true, but the explanation is false;

Question 31

The Tensilon (edrophonium) test is used to differentiate myasthenic and cholinergic crises because administration of edrophonium improves the symptoms dramatically in myasthenic crisis A) both the statement and the explanation are true and a causal relationship exists between them; B) both the statement and the explanation are true but there is no causal relationship between them; C) the statement is true, but the explanation is false; D) the statement is false, but the explanation itself is true E) both the statement and the explanation are false

Answer 31

A) both the statement and the explanation are true and a causal relationship exists between them;

Question 32

In Guillain–Barré-syndrome preceding viral infection can always be proved, because immunological processes play a role in the pathogenesis of the disease. A) both the statement and the explanation are true and a causal relationship exists between them; B) both the statement and the explanation are true but there is no causal relationship between them; C) the statement is true, but the explanation is false; D) the statement is false, but the explanation itself is true E) both the statement and the explanation are false

Answer 32

D) the statement is false, but the explanation itself is true

Question 33

In the diagnosis of Guillain-Barré syndrome the primary diagnostic tool is sural nerve biopsy, because the pathological basis of the disease is the segmental demyelination of the peripheral nerves. A) both the statement and the explanation are true and a causal relationship exists between them; B) both the statement and the explanation are true but there is no causal relationship between them; C) the statement is true, but the explanation is false; D) the statement is false, but the explanation itself is true E) both the statement and the explanation are false

Answer 33

D) the statement is false, but the explanation itself is true

Question 34

Acute Guillain-Barré syndrome has fatal outcome in 50% of the cases, because respiratory paralysis, diaphragm paralysis may occur as a complication. A) both the statement and the explanation are true and a causal relationship exists between them; B) both the statement and the explanation are true but there is no causal relationship between them; C) the statement is true, but the explanation is false; D) the statement is false, but the explanation itself is true E) both the statement and the explanation are false

Answer 34

D) the statement is false, but the explanation itself is true

Question 35

The first diagnostic step is: A 28-year-old man presented with progressing lower extremity weakness and ascending numbness that started about a week ago. He reported that he had a strong diarrhea three weeks ago. A) cranial CT B) repetitive nerve stimulation C) thoracolumbar spine MRI D) detailed neurological physical examination

Answer 35

D) detailed neurological physical examination

Question 36

Which disease is the most likely?A 28-year-old man presented with progressing lower extremity weakness and ascending numbness that started about a week ago. He reported that he had a strong diarrhea three weeks ago. A) Guillain–Barré syndrome B) diabetic polyneuropathy C) amyotrophic lateral sclerosis D) myasthenia gravis

Answer 36

A) Guillain–Barré syndrome

Question 37

The most likely cerebrospinal fluid finding is:A 28-year-old man presented with progressing lower extremity weakness and ascending numbness that started about a week ago. He reported that he had a strong diarrhea three weeks ago. A) decreased protein level B) normal protein protein concentration C) cell-protein dissociation D) elevated cell count

Answer 37

C) cell-protein dissociation

Question 38

Possible complications: A 28-year-old man presented with progressing lower extremity weakness and ascending numbness that started about a week ago. He reported that he had a strong diarrhea three weeks ago. 1) paralysis of respiratory muscles 2) cranial nerve lesion 3) deep vein thrombosis 4) vegetative disturbances A) 1st, 2nd and 3rd answers are correct B) 1st and 3rd answers are correct C) 2nd and 4th answers are correct D) only 4th answer is correct E) all of the answers are correct

Answer 38

E) all of the answers are correct

Question 39

In sural nerve biopsy we would find the following: A 28-year-old man presented with progressing lower extremity weakness and ascending numbness that started about a week ago. He reported that he had a strong diarrhea three weeks ago. 1) neutrophil granulocyte infiltration 2) segmental demyelination 3) intact nerve 4) axonopathy may occur A) 1st, 2nd and 3rd answers are correct B) 1st and 3rd answers are correct C) 2nd and 4th answers are correct D) only 4th answer is correct E) all of the answers are correct

Answer 39

C) 2nd and 4th answers are correct