August Test 1

1.
Overshooting of target directed movement

a.Ataxia
b.Epilepsy
c. Dysdiadochokinesis
d. Telangiecstasia
e. Dysmetria
2. Which of the following is FALSE with regards to generalised seizure disorders
a. Usually heralded by an aura
b. There is no warning
c. There is bilateral synchronous seizure discharge on EEG
d. The seizure is symmetrical
e. There is always a loss of consciousness
3. What is this describing: Transient loss of muscle tone causing a sudden fall to the floor or
drop of the head.
a. Tonic seizures
b. Absence seizures
c. Tonic-clonic seizures
d. Myoclonic seizures
e. Atonic seizures
4. Febrile convlsions occur in ___% of children between the ages of 6 months and 5 years
a. 10
b. 1
c. 15
d. 20
e. 3
5. A term used to describe seizures which arise form one hemisphere or part of one
hemisphere
a. focal
b. petite
c. generalised
d. myonic
e. tonic
6. What is this describing: Brief, often repetitive jerking movements of the limbs, neck or
trunk.
a. Absence seizures
b. Myoclonic seizures
c. Tonic-clonic seizures
d. Tonic seizures
e. Atonic seizures
7. Cutaneous features consist of ash leaf shaped patches, shagreen patches, & adenoma
sebaceum. Neurological features are; infantile spasms and developmental delay, epilepsy, &
intellectual impairment.
a. Neurofibromatosis type 1
b. Tuberous sclerosis
c. Juvenile myasthenia
d. Neurofibromatosis type 2
e. Sturge Weber syndrome
8. CPK is markedly elevated. Children present with a waddling gait. The average age of
diagnosis is 5.5 years. Death ensues in the late teens or early twenties from respiratory
failure.
a. Dermatomyositis
b. Dystrophia myotonica
c. Becker’s muscular dystrophy
d. Duchenne’s muscular dystrophy
e. Myasthenia gravis
9. What is this describing: Generalised increase in tone
a. Tonic seizures
b. Tonic-clonic seizures
c. Myoclonic seizures
d. Absence seizures
e. Atonic seizures
10. The need to turn prone to rise from a supine to a standing position
a. Arnold Chari’s sign
b. Dandy-Walker’s sign
c. Guillan-Barre’s sign
d. Gower’s sign
e. Becker’s sign
11. Clinical features include corneal clousing, thickened skin, coarse facies, valvular lesions,
thickened skull, hepatosplenomegaly
a. Huntington’s disease
b. Mucopolysaccharidoses
c. Subacute sclerosing panencephalitis
d. Wilson’s disease
e. X-linked adrenoleucodystropy
12. A periodic disorder characterised by paroxysmal headache, often unilateral, and is
characteristically throbbing
a. Torticollis
b. Cluster headache
c. Migraine
d. Sinusitis
e. Tension headache
13. What is this describing: Transient loss of consciousness with abrupt onset and
termination, unaccompanied by motor phenemenon except flickering of eyelids and minor
alteration in motor tone.
a. Absence seizures
b. Tonic seizures
c. Atonic seizures
e. Tonic-clonic seizures
14. Epilepsy has a prevalence of about
a. 1.0%
b. 2.0%
c. 1.5%
d. 0.1%
e. 0.5%
15. In children aged under 5 years, prolonged seizures (> 5 minutes) may be treated using
a. buccal midazolam
b. oral benzodiazepine
c. high dose aspirin
d. sodium valproate
e. digoxin
16. A 5-HT antagonist used in headache prophylaxis. Can cause weight gain and sleepiness.
a. Propranolol
b. Metoclopramide
c. Pizotifen
d. Prochlorperazine
e. Sumatriptan
17. Side effects include rash, neutropaenia, hyponatraemia and ataxia
a. Valproate
b. Gabapentin
c. Carbamazepine
d. Vigabatrin
e. Lamotrigine
18. Occur mostly between 4-6 months. Violent flexor spasms of the head, trunk and limbs,
followed by extension of the arms.
a. Lennox-Gastaut syndrome
b. Juvenile myoclonic epilepsy
c. Benign rolandic epilepsy
d. Petit mal absence seizures
e. Infantile spasms
19. This presents as abnormal muscle fatiguability which improves with rest or
anticholinesterase drugs
a. Juvenile myasthenia
b. Duchenne’s muscular dystrophy
c. Myasthenia gravis
d. Bell’s palsy
e. Charcot-Marie-Tooth disease
20. Presents woith excessive increase in head circumference, separation of skull sutures
bulging of the anterior fontanelle, distention of scalp veins and sun setting of the eyes
a. Spina bifida occulta
b. Myelomeningocele
c. Anencephaly
d. Hydrocephalus
e. Encephalocele
21. A sporadic disorder with a port wine stain in the
distribution of the trigeminal nerve associated with a similar lesion intracranially
a, Tuberous sclerosis
b. Juvenile myasthenia
c. Neurofibromatosis type 2
d. Sturge Weber syndrome
e. Neurofibromatosis type 1
22. The diagnosis of epilepsy requires ___ or more unprovoked seizures
a. 4
b. 3
c. 2
d. 5
e. 1
23. Which of the following is a cause of stroke in childhood?
a. Sickle cell disease
b. SLE
c. Moyamoya disease
d. Endocarditis
e. All of these
24. A disorder of the anterior horn cell
a. Bell’s Palsy
b. Myasthenia gravis
c. Poliomyelitis
d. Duchenne Muscular dystrophy
e. Guillan Barre
25. An autosomal recessive condition. Disorder of DNA repair. The gene ATM has been
identified. There may be mild delay in motor development in infancy and oculomotor
problems.
a. Epilepsy
b. Fredreich’s ataxia
c. Dysdiadochokinesis
d. Ataxia telangiectasia
e. Dysmetria
26. Results from a deletion of chromosome material on the short arm of the X chromosome
(Xp21 site). This site is known to code for a protein called dystrophin.
a. Dermatomyositis
d. Becker’s muscular dystrophy
e. Duchenne’s muscular dystrophy
27. An isolated lower motor neuron paresis of the VIIth cranial nerve. It is probably post
infection with an association with Herpes simplex virus in adults
a. Myasthenia gravis
c. Bell’s palsy
d. Charcot-Marie-Tooth disease
28. Failure of development of most of the cranium and brain. Affected infants are stillborn
or die shortly after birth
a. Encephalocele
b. Anencephaly
c. Hydrocele
d. Myelomeningocele
e. Spina bifida occulta
29. Difficulty in performing repetitive and alternating movements
a. Telangiecstasia
b. Ataxia
c. Epilepsy
d. Dysdiadochokinesis
e. Dysmetria
30. Constitute approximately 90% of migraines
a. With aura
b. Complicated
c. Without aura
d. Cluster
e. Nystigmal
31. The most common cause of epilepsy is
a. neurodegenerative disorders
b. idiopathic
c. cerebral tumour
d. cerebral vascular occlusion
e. neurocutaneous syndromes
32. Presentation is typically 2-3 weeks after an upper respiratory tract infection or
campylobacter gastroenteritis. The prominent feature is an ascending symmetrical
weakness with loss of reflexes and autonomic involvement.
b. Charcot-Marie-Tooth disease
c. Guillan Barre syndrome
d. Duchenne’s muscular dystrophy
e. Juvenile myasthenia
33. Dominantly inherited and cause dby a trinucleotide repeat expansion. Older children can
present with learning difficulties. Adults can develop cataracts and males baldness and
testicular atrophy.
a. Duchenne’s muscular dystrophy
b. Dermatomyositis
e. Dystrophia myotonica
34. Side effects include restriction of visual fields and sedation
a. Lamotrigine
b. Valproate
c. Carbamazepine
d. Gabapentin
e. Vigabatrin
35. A seizure lasting 30 minutes or repeated seizures for 30 minutes without recovery of
consciousness
a. telangiectasia
b. status epilepticus
c. Friedrich's fit
d. SUDEP
e. ataxia
36. Children who experience complex febrile seizures (i.e. focal, prolonged or repeated in
the same illness) have a _____ risk of subsequent epilepsy
a. 20-30%
b. 4-12%
c. 30-40%
d. 10-20%
e. 1-5%
37. Due to binding of antibody to acetylcholine receptors on the post-junctional synaptic
membrane. Presentation is usually after 10 years of age with opthalmoplegia and ptosis,
aloss of facial expression and difficulty chewing.
a. Juvenile myasthenia
b. Charcot-Marie-Tooth disease
c. Duchenne’s muscular dystrophy
d. Myasthenia gravis
e. Bell’s palsy
38. Features include the accumulation of copper which may cause changes in behaviour and
additional involuntary movements or a mixture of neurological and hepatic symptoms
b. Wilson’s disease
d. X-linked adrenoleucodystropy
e. Lysosomal storage disorders
39. Small areas of visual loss
a. Fortification spectra
b. Opthalmoplegia
c. Hemianopia
d. Scotoma
e. Aura
40. The most common type, may result in strange warning feelings or aura with smell and
taste abnormalities. Lip smacking, plucking at one's clothing and automatisms may be seen.
a. Temporal lobe seizures
b. Frontal seizures
c. Occipital seizures
d. Parietal lobe seizures
e. Spastic seizures
41. Comprises 15% of all childhood epilepsies. EEG shows
sharp focal waves from the centrotemporal area.
A, Benign occipital epilepsy
b. Lennox-Gastaut syndrome
c. Juvenile myoclonic epilepsy
d. Benign rolandic epilepsy
e. Petit mal absence seizures
42. Protrusion of the meninges, spinal cord, and nerve roots through a gap in the spine,
accompanied by paralysis of the legs and urinary incontinence
a. Myelomeningocele
b. Encephalocele
c. Anencephaly
d. Spina bifida occulta
e. Hydrocele
43. Associated with neurological phenomena such as opthalmoplegia, hemiparesis,
paraesthesiae or hemidysaesthesia
a. Complicated
b. Without aura
c. Cluster
d. With aura
e. Nystigmal
44. A delayed response in adolescence to a previous measles infection causing neurological
regression with a characteristic EEG
b. X-linked adrenoleucodystropy
d. Wilson’s disease
e. Lysosomal storage disorders
45. Consists of a downward displacement of the cerebellar tonsils and the medulla through
the foramen magnum, sometimes causing hydrocephalus
a. Spina bifida occulta
b. Encephalocele
c. Arnold Chari malformation
d. Dandy Walker malformaton
e. Anencephaly
46. Presentation is usually with acute onset of headache pain, neck stiffness and
occasionally fever. A CT scan of the head usually identified blood in the CSF.
a. Myelomeningocele
b. Subdural haematoma
c. Subarachnoid haemorrhage
d. Extradural haemorrhage
e. Encephalocele
47. Onset is in the first decade with distal atrophy and pes cavus. The legs are more affected
than the arms.
b. Guillan Barre syndrome
c. Duchenne’s muscular dystrophy
d. Charcot-Marie-Tooth disease
48. Results from tearing of the veins as they cross the subdural space. May be caused by
shaking or direct trauma. Occasionally seen following a fall from a considerable height.
a. Subarachnoid haemorrhage
b. Encephalocele
c. Subdural haematoma
e. Myelomeningocele
49. Involve the motor cortex. May lead to clonic movements, which may travel proximally
(Jacksonian march)
a. Spastic seizures
b. Temporal lobe seizures
d. Parietal lobe seizures
e. Frontal seizures
50. The cause is often an aneurysm or arteriovenous malformation. Retinal haemorrhage is
usually present.
a. Subarachnoid haemorrhage
b. Myelomeningocele
c. Encephalocele
e. Subdural haematoma
51. Congenital deformity of the foot usually marked by a
curled shape or twisted position of the ankle and heel and toes
a, Arnold Chari malformation
c. Becker’s deformity
d. Talipes
e. Dandy-Walker malformation
52. Diagnosis is made by observing improvement following the administration of
intravenous edrophonium and can be further confirmed by testing for acetylcholine
receptor antibodies. Treatments include neostigmine or pyridostigmine.
c. Charcot-Marie-Tooth disease
d. Bell’s palsy
53. Presents with worsening ataxia, distal wasting in the legs, absent lower limb reflexes,
pes cavus and dysarthria. There is impairment of joint position and vibration sense.
a. Fredreich’s ataxia
b. Ataxia telangiectasia
c. Dysmetria
d. Epilepsy
e. Dysdiadochokinesis
54. Which of the following is FALSE?
a. Anti-epileptic drug therapy can usually be discontinued after 2 years free of seizures
b. Phenytoin has predictable pharmacokinetics
c. Carbamazepine can make myoclonic seizures worse
d. Not all seizures require anti-epileptic drug therapy
e. Carbamazepine can make absence sizures worse
55. A symmetrical headache of gradual onset, often described as tightness, a band or
pressure.
a. Migraine
b. Torticollis
c. Tension headache
d. Sinusitis
e. Cluster headache
56. Usually associated with a skull fracture. There may be focal neurological signs with
dilatation of the ipsilateral pupil and paresis of the contralateral limbs. Diagnosis is
confirmed with a CT scan.
a. Subarachnoid haemorrhageEncephalocele
b. Subdural haematoma
c. Extradural haemorrhage
d. Myelomeningocele
57. What is this describing: Rhythmical contraction of muscle groups following a generalised
increase in muscle tone.
a. Absence seizures
b. Tonic-clonic seizures
c. Tonic seizures
e. Atonic seizures
58. Cause distortion of vision
b. Parietal lobe seizures
d. Spastic seizures
e. Frontal seizures
59. Failure of fusion of the vertebral arch. Often an incidental finding on X-ray, but there
may be an overlying skin lesion such as a tuft of hair
a. Myelomeningocele
b. Anencephaly
c. Hydrocele
d. Spina bifida occulta
e. Encephalocele
60. Which of the following is NOT characteristic of a headache due to a space occupying
lesion?
a. Papilloedema
b. Visual field defects
c. Torticollis
d. Abnormal gait
e. Eased on lying down
61. There is extrusion of the brain and meninges through a
midline skull defect
a, Anencephaly
b. Hydrocele
c. Encephalocele
d. Myelomeningocele
e. Spina bifida occulta
62. Bilateral acoustic neuromata are the predominant feature and present with deafness
and sometimes a cerebellopontine angle syndrome
b. Tuberous sclerosis
c. Juvenile myasthenia
d. Neurofibromatosis type 2
63. This disorder is usually autosomal recessive and due to degeneration of the anterior
horn cells, leading to progressive weakness and wasting of skeletal muscles
a. Poliomyelitis
b. Spinal muscular atrophy
c. Guillan Barre
d. Duchenne Muscular dystrophy
64. Treatment for this is with acyclovir. Hypertension should be excluded. There is an
association with coarctation of the aorta.
c. Bell’s palsy
d. Myasthenia gravis
e. Charcot-Marie-Tooth disease
65. Constitute approximately 10% of migraines
a. Nystigmal
b. Without aura
c. Cluster
d. With aura
e. Complicated
66. Which of the following is NOT a typical sign of Werdnig- Hoffman disease?
a. Increased foetal movements during pregnancy
b. Absent deep tendon reflexes
c. Intercostal recession
d. Lack of antigravity power in hip flexors
e. Fasciculation of the tongue
67. Cause contralateral dysaesthesias (altered sensation), or distorted body image.
b. Parietal lobe seizures
c. Spastic seizures
d. Frontal seizures
e. Occipital seizures
68. Some functional dystrophin is produced. Average age of onset is 11 years. Leads to
inability to walk in the late twenties and death in the 40’s, although this is very variable.
c. Dystrophia myotonica
d. Dermatomyositis
e. Becker’s muscular dystrophy
69. Occur mostly between 1-3 years. Multiple seizure types but mostly astatic seizures, tonic
seizures, and atypical absences. Also neurodevelopmental arrest or regression.
a. Benign rolandic epilepsy
b. Petit mal absence seizures
c. Lennox-Gastaut syndrome
d. Juvenile myoclonic epilepsy
e. Infantile spasms
70. Side effects include weight gain and hair loss
a. Valproate
b. Lamotrigine
c. Gabapentin
d. Vigabatrin
e. Carbamazepine
71. Most common between 4 - 10 years of age. Tonic-clonic seizures in sleep, or awareness
of abnormal feelings in the tongue and distortion of the face.
a. Infantile spasms
c. Petit mal absence seizures
d. Benign rolandic epilepsy
e. Juvenile myoclonic epilepsy
72. A disorder of neuromuscular transmission
a. Duchenne Muscular dystrophy
c. Poliomyelitis
d. Guillan Barre
e. Bell’s Palsy
73. This is a systemic illness, possibly due to an angiopathy. Usual onset is between 5 and 10
years. There is a characteristic violaceous (heliotrope) rash to the eyelids, and periorbital
oedema. Leads to symmetrical muscle weakness.
c. Dermatomyositis
74. Most common in adolescence - adulthood. Tonic-clonic seizures and absences may
occur, mostly shortly after walking. A typical history is throwing drinks or cornflakes about in
the morning.
a. Lennox-Gastaut syndrome
b. Juvenile myoclonic epilepsy
c. Infantile spasms
e. Benign rolandic epilepsy
75. The most common hereditary motor sensory neuropathy (usually dominantly inherited).
b. Guillan Barre syndrome
c. Charcot-Marie-Tooth disease
d. Juvenile myasthenia
76. Describes incoordination of movement, speech and posture
a. Dysmetria
b. Ataxia
c. Telangiecstasia
d. Epilepsy
e. Dysdiadochokinesis
77. Occur mostly between 4 - 12 years. Stare momentarily and stop moving, may twitch
their eyelids or a hand minimally. Lasts no longer than 30 seconds.
a. Infantile spasms
c. Benign rolandic epilepsy
78. Most common between 1 and 14 years. In younger children it is characterised by periods
of unresponsiveness, eye deviation and vomiting. In older children it is characterised by
headaches and visual disturbances.
a. Petit mal absence seizures
b. Benign rolandic epilepsy
c. Lennox-Gastaut syndrome
d. Benign occipital epilepsy
79. The seeing of zigzag lines
a. Aura
b. Hemianopia
c. Fortification spectra
d. Opthalmoplegia
e. Scotoma
80. In order to make the diagnosis, two or more of the following must be present: six or
more café au lait spots > 5mm in size before puberty or >15 mm after puberty; > 1
neurofibroma, axillary freckles, optic glioma, Lisch nodule, bony lesions from sphenoid
dysplasia, first degree relative with the condition
b. Neurofibromatosis type 1
c. Tuberous sclerosis
d. Juvenile myasthenia
Key
1. E
2. A
3. E
4. E
5. A
6. B
7. B
8. D
9. A
10. D
11. B
12. C
13. A
14. E
15. A
16. C
17. C
18. E
19. C
20. D
21. D
22. C
23. E
24. C
25. D
26. E
27. C
28. B
29. D
30. C
31. B
32. C
33. E
34. E
35. B
36. B
37. A
38. B
39. D
40. A
41. D
42. A
43. A
44. C
45. C
46. C
47. D
48. C
49. E
50. A
51. D
52. E
53. A
54. B
55. C
56. C
57. B
58. C
59. D
60. E
61. C
62. D
63. B
64. C
65. D
66. A
67. B
68. E
69. C
70. A
71. D
72. B
73. C
74. B
75. C
76. B
77. D
78. D
79. C
80. B

August Test 1

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1.

Overshooting of target directed movement

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