Neurology - Neuromuscular diseases 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.
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.
