Chapter 55B. In Children

Question 1

Question 55B-1 
An 8-year-old male patients with arthritis, rhinorthea, persistent cough, fever and malaise. His neurologic findings are diagnosed as mononeuropathy multiplex. Which is the most likely diagnosis? 
A.  Mixed connective tissue disease 
B.  Behcet's disease 
C.  Wegener's granulomatosis 
D.  Polyarteritis nodosa

Answer 1

Answer 55b-1: C.
Wegener’s granulomatosis presents with
medical complaints which are sinopulmonary
plus neurologic complications which are
cranial neuropathies or peripheral
neuropathies. Mononeuropathy multiplex is a
typical neurologic presentation. (p1105)

Question 2

Question 55B-2: 
Which would be the most likely neurologic complication of cystic fibrosis? 
A.  Cranial neuropathies 
B.  Sleep apnea 
C.  Lethargy and confusion 
D.  Muscle spasms and contractures

Answer 2

Answer 55B-2: C.
The most common neurologic complications
of cystic fibrosis are due to hypoxia and
hypercarbia. Lethargy and confusion are
typical manifestations, and may progress to
coma. (p1106)

Question 3

Question 55B-3: 
Hepatic encephalopathy can be expected to produce which of the following symptoms? 
A.  Tremor 
B.  Confusion 
C.  Anxiety 
D.  Personality change 
E.  All of these

Answer 3

Answer 558-2: C.
The most common neurologic complications
of cystic fibrosis are due to hypoxia and
hypercarbia. Lethargy and confusion are
typical manifestations, and may progress to
coma. (p1106)

Question 4

Question 558-4: 
Which is the most likely presentation of hypothyroidism in the neonatal period? 
A.  Hypotonia 
B.  Arthrogryposis 
C.  Ocular motor palsy 
D.  No clinical findings

Answer 4

Answer 55B-4: D.
Neonates with hypothyroidism are often
asymptomatic, so newborn screening is
needed. Screening is mandatory in most
developed countries. (p 1110)

Question 5

Question 55B-5: 
Which are potential presentations of septooptic dysplasia? 
A.  Normal neurologic function 
B.  Mental retardation 
C.  Spasticity 
D.  Seizures 
E.  Any ofthese

Answer 5

Answer 55B-5:· E.
Any of these can be seen in patients with
septo-optic dysplasia. Patients with mild
forms can have normal intellectual function.
(P1111)

Question 6

Question 55B-6: 
Which is the most common neurol02ic complication of diabetes mellitus in children? 
A. Stroke 
B.  Mononcuropathy multiplex 
C.  Peripheral polyneuropathy 
D.  Cranial neuropathy

Answer 6

Answer 55B-6: C.
Peripheral polyneuropathy is the most
common neurologic complication of PM in
both children and adults. Stroke and cranial
neuropathy are uncommon eyen in children
with DM. (p1112)

Question 7

Question 55B-7: 
A child undergoing intrathecalchemotherapy for leukemia with ara-C develops progressive weakness with areflexia. There are no corticospinal tract signs. Acute polyneuropathy is diagnosed. Which is the most likely cause? 
A.  Neoplastic meningitis 
B.  Ara-C toxicity 
C.  Guillain-Barre syndrome 
D.  Transverse myelitis

Answer 7

Answer 55B-7: B.
Ara-C can produce a host of neurologic
complications, including an acute neuropathy
which resembles GBS, myelopathy. cerebellar
dysfunction, and encephalopathy. (P 1110)

Question 8

Question 55B-8:
A 14-year-old child sustains a head injury during a sporting practice. He loses consciousness briefly, then is confused, but is normal by the time he is seen in the emergency department. His prior history is remarkable for hemophilia. Which would be the most appropriate management approach?
A. CT performed in the ED prior to discharge
B. CT scheduled for the next working day
C. MRl scheduled for the next working day.
D. Discharged home with instructions on monitoring by family

Answer 8

Answer 55B-8: A.
Patients with hemophilia are predisposed to
intracranial bleeding, as well as bleeding
elsewhere in the body. Therefore, if there is
any non-trivial trauma, bleeding has to be
considered, and in this case CT should be
performed prior to the patient leaving the ED
(p 1108)

Question 9

Question 55B-9: 
Sjogren's syndrome can be seen as acomplication of which of the  following conditions? 
1.  SLE 
2.  JRA 
3.  MCTO 
4.  Dermatomyositis

Answer 9

Answer 55B-9: E.
Sjogren’s syndrome can be seen in primary
form or secondary form. The primary fonn is
uncommon in childhood. The listed disorders
can all be causes of secondary Sjogren’s
syndrome. (p1106)

Question 10

Question 55B-10: 
A 12-year-old child presents with headache, behavioral change, then seizures. Examination shows rash, low weight, and hypertension. Which of the following tests confirms the diagnosis of polyarteritis nodosa? 
A.  C-reactive protein 
B.  Low complement levels 
C.  Positive ANCA 
D.  Elevated ESR 
E.  None of these

Answer 10

Answer 55B-10: E.
The clinical presentation is strongly
suggestive or PAN, but there are no specific
diagnostic laboratory tests. All of the listed
tests would be suggestive, but not diagnostic.
(p 1103)