Neurology - Neuromuscular diseases(38) Flashcards
NEU - 6.1
Which symptom is not characteristic for Myasthenia gravis?
A) diplopia
B) ptosis
C) dysarthria
D) aphasia
E) muscle weakness worsening with physical exercise
ANSWER
D) aphasia
EXPLANATION
Aphasia: disturbance of higher integral process of the brain due to the damage of certain cortical areas and connecting pathways. It is not the symptom of myasthenia gravis (MG).
NEU - 6.2
First-choice treatment in Myasthenia gravis:
A) atropin
B) acetylcholinesterase inhibitors
C) steroid
D) plasma exchange therapy
E) vitamins
ANSWER
B) acetylcholinesterase inhibitors
EXPLANATION
Acetylcholinesterase inhibitors: Myasthenia gravis is caused by the disturbance of neuromuscular transmission due to the damage of the postsynaptic membrane function triggered by the anti-ACh-receptor antibody.
NEU - 6.3
Motor neurone disease:
A) Eaton–Lambert syndrome
B) Amyotrophic lateral sclerosis
C) Duchenne muscular dystrophy
D) Guillain–Barré syndrome
ANSWER
B) Amyotrophic lateral sclerosis
EXPLANATION
Eaton-Lambert-syndrome is the disease of the presynaptic neuromuscular transmission (usually caused by anti-calcium channel antibodies of paraneoplastic origin) Amyotrophic lateral sclerosis: signs of both upper and lower motor neuron lesion can be observed Duchenne disease: X-linked muscular dystrophy of recessive inheritance Guillain-Barré-syndrome: inflammatory, immune-mediated polyganglioradiculitis with segmental demyelinisation
NEU - 6.4
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
E) paraesthesia
EXPLANATION
Paraesthesia: not a sign of ALS. Sensory system is not affected in this disease. ALS is a disease of both the lower and upper motor neurons (with the degeneration of the corticospinal tract).
NEU - 6.5
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
C) cranial nerves are never affected
EXPLANATION
Involvement of cranial nerves can be present in 50% of cases. Signs of lower cranial nerve dysfunction: dysarthria, dysphagia.
NEU - 6.6
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
D) paresis in the proximal limb muscles along with fever and pain
EXPLANATION
Polymyositis: generalized, subacutely progressing, mostly symmetric, chronic disease of the skeletal muscles with histologic signs of inflammation. Weakness of truncal and proximal muscles of the extremities associated with pain and fewer. (Pathological immunological processes in the background.)
NEU - 6.7
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
B) weakness of the external ocular muscles
EXPLANATION
Polymyositis: generalized, subacutely progressing, mostly symmetric, chronic disease of the skeletal muscles with histologic signs of inflammation. Weakness of truncal and proximal muscles of the extremities associated with pain and fewer. (Pathological immunological processes in the background.) Weakness of the ocular muscles is not typical of the disease. Myositis of the periocular muscles is part of the steril inflammation of the structures of the orbita.
NEU - 6.8
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
B) 2nd–3rd decade
EXPLANATION
The onset of FSHD is most frequent at the 2nd and 3rd decade.
NOT IN 2024
NEU - 6.9
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
C) damage of the ACh-containing vesicles
EXPLANATION
Myasthenia gravis is caused by the disturbance of neuromuscular transmission due to the damage of the postsynaptic membrane function triggered by the anti-ACh-receptor antibody. Damage of the ACh-containing vesicles is not a pathogenetic factor of this disease.
NEU - 6.10
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
D) pathologic reflexes
EXPLANATION
Pathologic reflexes are signs of the pyramidal tract (central paresis), and are not signs of myasthenia gravis.
NEU - 6.11
Which symptom is not provoked by muscarin?
A) nausea
B) salivation
C) fasciculation
D) bronchial secretion
E) vertigo
ANSWER
C) fasciculation
EXPLANATION
Fasciculation: it’s a nicotinergic symptom.
NEU - 6.12
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
B) loss of ambulation at 25–30 years of age
EXPLANATION
Duchenne disease: X-linked muscular dystrophy of recessive inheritance (severe form of dytrophia musculorum progressiva). Progressive weakness of the truncal, limb girdle, upper arm, gluteal, hip flexor and knee extensor muscles. The onset is in the infancy, patients usually die before 20-year of age.
NEU - 6.13
Frequent symptom in ALS:
A) dementia
B) agnosia
C) problems with urination
D) all of the above
E) none of the above
ANSWER
E) none of the above
EXPLANATION
ALS is a disease of both the lower and upper motor neurons (with the degeneration of the corticospinal tract). Sometimes dementia is associated with ALS (not frequently). In these cases significant neuronal damage and gliosis can be observed in the premotor gyrus and the temporal lobe.
NEU - 6.14
Which disease is associated more frequently with polymyositis?
A) adrenoleukodystrophy
B) Lyme disease
C) SLE
D) viral hepatitis
ANSWER
C) SLE
EXPLANATION
Polymyositis: generalized, subacutely progressing, mostly symmetric, chronic disease of the skeletal muscles with histologic signs of inflammation. Weakness of truncal and proximal muscles of the extremities associated with pain and fewer. (Pathological immunological processes in the background.) Classification: a) Myositis without other basic disease, b) Dermatomyositis: myositis associated with dermatitis, c) Paraneoplastic: myositis associated with malignances, d) Myositis associated with collagen diseases, e) Childhood form.
NEU - 6.15
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
D) problems with urination
EXPLANATION
ALS is a disease of both the lower and upper motor neurons (with the degeneration of the corticospinal tract). Problems with urination doesn’t occure in this disease. Function of the sphincter muscles and sensory system is preserved.
NEU - 6.16
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
B) polymyositis
EXPLANATION
Polymyositis: generalized, subacutely progressing, mostly symmetric, chronic disease of the skeletal muscles with histologic signs of inflammation. Weakness of truncal and proximal muscles of the extremities associated with pain and fewer. (Pathological immunological processes in the background.) Laboratory tests reveal increased ESR, gamma-globulin and markedly elevated CK levels.
NEU - 6.17
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
E) all of the answers are correct
EXPLANATION
Polyneuropathy may be associated with all autoimmune diseases listed here.
NEU - 6.18
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
E) all of the answers are correct
EXPLANATION
Becker’s-syndrome: X-linked, recessive inherited muscular dystrophy (more benign than the Duchenne dystrophy). Clinically, Becker’s syndrome is characterised by slowly progressive muscle weakness of certain muscle groups (muscles of the trunk, shoulders, upper arms, as well as gluteal muscles, hip flexors, knee extensors). Becker’s dystrophy begins in the first and second decades, and despite the weakness the patients may remain ambulatory until the 3rd decade and may live till 40 to 50 years of age. Serum CK is higher than normal, but far below than in the Duchenne syndrome.
NEU - 6.19
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
A) 1st, 2nd and 3rd answers are correct
EXPLANATION
In myasthenia gravis (MG), there is an impaired transmission in the neuromuscular junction (NMJ). Anti-Ach-R antibodies bind to components of the NMJ resulting in dysfunction of these proteins and disturbance of neuromuscular transmission.Thymoma may be present as an etiologic factor, but other malignant tumors can rarely be found in the background. (Quite contrary, there are often malignant tumors in the etiology of Eaton-Lambert syndrome).
NEU - 6.20
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
B) 1st and 3rd answers are correct
EXPLANATION
Pyridostigmin and neostigmin are acetylcholinesterase inhibitors, however urapidil is an antihypertensive, nimesulid is a non-steroid anti-inflammatory drug.
NEU - 6.21
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
D) only 4th answer is correct
EXPLANATION
The common symptom is the muscle weakness (sensory disturbance can only be found in GBS, abnormal reflex can be seen in ALS - pyramidal lesion). Only a small fraction of ALS is hereditary.
NEU - 6.22
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
B) 1st and 3rd answers are correct
EXPLANATION
Typical liquor finding: cell-protein dissociation (the total protein content of the CSF is increased, while the cell count is normal or only slightly elevated).
NEU - 6.23
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
C) 2nd and 4th answers are correct
EXPLANATION
In acute Guillain-Barré syndrome plasma exchange therapy and B vitamins can be used, depending on the severity of the clinical picture.
NEU - 6.24
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
E) all of the answers are correct
EXPLANATION
Acute Guillain-Barré syndrome (GBS) may include all of the symptoms listed here. GBS begins typically with distal type of paraesthesia and hypotonic paresis that may ascend in the following days. Lower cranial nerve symptoms are present in almost half of the GBS cases (dysphagia, diplegia facialis).