PEDIATRIC NEUROLOGY
Question # 01:
A 12-year-old
female is brought to the ED because she has altered consciousness, headache and
fever of 1020F for 2 days. You examined her and you found no
meningeal signs, but she requires repeated tactile stimulation to maintain
arousal, she is not oriented except to person, makes frequent paraphasic
errors, and can recall just one of three objects after a distracting task. The
rest of the neurologic examination is unremarkable.
Lumbar
puncture was done and CSF analysis shows a red blood cell count of 25
cells/hpf; white blood cell count of 120 cells/hpf with a lymphocytic predominance;
glucose of 70 mg/dL; and protein of 95 mg/dL.
What is
the best treatment to be initiated at this time?
A.
Iduroxidine
B. Ceftriaxone
C.
Vidarabine
D.
Solumedrol
E.
Acyclovir
Question # 02:
A 2 days old full-term infant has been evaluated for irritability and microcephaly.
He is a product of uncomplicated pregnancy, labor and delivery for a healthy
mother. Head ultrasonography and CT scan showed enlarged posterior horns of the
lateral ventricles and punctuate calcifications which are both periventricular and
diffuse. On neurological examination, he has increased tone and hyperreflexia.
A dilated funduscopic exam shows a large area of absent retina with hyperpigmented
margins.
What is the most
likely diagnosis?
A. Congenital cytomegalovirus infection
B. Congenital toxoplasmosis
C. Congenital herpes simplex type 1
D. Congenital rubella
E. Congenital syphilis
Question # 03:
You have
been asked to see a 5-dayold baby girl in the NICU for possible seizures. She
was small for gestational age and born at 32 weeks gestation. On day 2 of life,
she has had feeding difficulties and has received phototherapy for jaundice. On
your exam, she is micro cephalic, has hepatosplenomegaly, and a purpuric rash.
You
ordered head ultrasound which shows ventriculomegaly. Her blood labs elevated
aminotransferases, direct and indirect hyperbilirubinemia, thrombocytopenia,
and hemolytic anemia.
Which
test is most likely to confirm this patient’s diagnosis?
A. Bone
marrow biopsy
B. Head
CT scan
C.
Ophthalmologic examination
D. Rapid
plasma reagin (RPR) test
E. Urine
CMV culture
Question # 04:
A 13-year-old female came to the
office with 2 days history of right facial weakness and she is unable to close
her right eye. She has trouble sucking from a straw and also, she has drooling
from her right side of the mouth. She denies paresthesias, ear pain or
hyperacusis. On skin examination you note the following:
The most likely infectious agent causing the
above symptoms is:
A. Herpes Simplex Virus Type 1
B. Borrelia burgdorferi
C. Rickettsia rickettsii
D. Mycoplasma pneumonia
E. Tuberculosis
Question # 05:
The best treatment option for the patient
in question # 04 is:
A.
Acyclovir
B.
Forscarnet
C.
Doxycycline
D.
Azithromycin
E.
Fluconazole
CHECK ANSWERS TOMORROW AT 10 AM PLEASE
Question # 01 Correct Answer is: E
ReplyDeleteThe clinical presentation is indicative of herpes simplex encephalitis (HSE), Approximately one-third of cases occurring in patients between the ages of 6 months and 20 years. HSE can occur as a primary infection or a reactivation of the herpes simplex virus (HSV). Patients present with a variety of non-specific findings, including fever, headache, altered consciousness, dysphasia, seizures, and/or focal neurologic deficits.
CSF analysis could be normal in 5–10%. In majority of cases, CSF analysis shows an elevated white blood cell count with a lymphocytic predominance and an elevated protein. Red blood cells are often present but are non-diagnostic. PCR form the CSF has a sensitivity of 94% and
specificity of 98% in confirming the diagnosis. EEG, CT, and MRI are supportive studies in the diagnosis. Treatment consists of intravenous acyclovir 10 mg/kg every 8 h (30 mg/kg/d) for 14–21 days.
Idoxuridine was the first antiviral drug used to treat HSE; however, it proved to be both ineffective and toxic. Acyclovir was demonstrated to be superior to vidarabine in a head-to-head trial.
Neither ceftriaxone, nor solumedrol would be appropriate treatments, as both the clinical presentation and CSF studies suggest viral encephalitis.
Suggested Reading
Whitley RJ, Kimberlin DW. Herpes simplex: encephalitis children and adolescents. Semin Pediatr Infect Dis. 2005; 16:17–23.
Question # 02: Correct Answer is: B
Congenital toxoplasmosis (CT) is a rare disorder despite the ubiquitous presence of the parasite in the environment and high incidence of sero-positivity in the population. Domestic cats are the reservoir and human transmission occurs from oral ingestion of ova in fecal contaminated dirt or litter boxes.
Infected mothers during pregnancy will transmit the congenital infection to their babies in 50–80%, via hemaotgenous spread of the parasite to the placenta and fetus. Only 5% of children will have neurological sequelae, approximately 35% develop chorioretinitis by age 12 with many lesions being subclinical.
Treatment is not known to prevent chorioretinitis or neurologic injury but this is debated. The calcifications in CT in this case is diffuse involving the cortex and may also be periventricular with hydrocephalus, but calcification in congenital CMV involve the periventricular region only. There is no evidence that maternal treatment prevents congenital toxoplasmosis. The prognosis for a child affected as above is poor.
Suggested Reading
Gilbert R, Tan H, Cliffe S, Guy E, and Stanford M. Symptomatic toxoplasma infection due to congenital and postnatally acquired infection. Arch Dis Child 2006; 91:495–8.
Question # 03: Correct Answer is: E
ReplyDeleteCytomegalovirus is one of the herpesviridae family. Infected immune-competent adults are asymptomatic; on the other hand, congenital infection is the most prevalent infection-related cause of congenital neurologic impairment. Vertical transmission—during pregnancy through the placenta, during delivery through cervical secretions or blood, or postnatally through breast milk—occurs in 30% of cases; however, not all infected fetuses are affected. Symptomatic Infants at birth (10%) are typically small for gestational age with feeding problems, ocular problems (chorioretinitis, retinal scars, optic atrophy, and central vision loss), sensorineural hearing loss, organomegaly, thrombocytopenia, jaundice, microcephaly, seizures, and abnormal neurologic examinations.
Neuroimaging may reveal periventricular cysts and calcifications, ventriculomegaly, vasculitis, hydranencephaly, and/or neuronal migration abnormalities. Perinatal mortality for symptomatic newborns ranges from 2–30%, with survivors having poor neuro-developmental prognoses. Infected infants who are asymptomatic at birth are still at risk for
neurodevelopmental issues, such as learning disabilities and attention deficit hyperactivity disorder. In addition, 5–15% of these infants will develop sensori-neural hearing loss, which is typically bilateral and progressive. If CMV infection is suspected in utero, an amniocentesis should be performed to confirm vertical transmission. Fetal ultrasound or MRI can help to predict the risk that the fetus will be affected at birth. Urine culture or CMV-DNA testing by PCR can help in confirming congenital infection within 3 weeks of birth. Infected newborns
should be further evaluated with a complete blood count, platelet count, transaminase level, and bilirubin levels (direct and indirect) and should have both an ophthalmologic examination and auditory brainstem evoked responses.
Symptomatic newborns should be treated with gancyclovir 6 mg/kg/dose twicw a day for 6 weeks, as this improves hearing outcomes. Treatment of asymptomatic newborns is not currently recommended.
Suggested Reading
Coll O, et al. Guidelines on CMV congenital infection. J Perinat Med. 2009;37:433–45.
Question # 04: Correct Answer is: B
Question # 05: Correct Answer is: C
The skin manifestation is the target sign of the Lyme disease which is caused by the tick-transmitted spirochete, B. burgdorferi. Clinical manifestations may include erythema migrans, lymphocytic meningitis, cranial neuritis, and polyradiculitis. Diagnosis includes an ELISA on serum for anti-B. burgdorferi antibodies. If serum is positive, then Western blot is
completed. Treatment options include oral doxycycline, ampicillin, and cefuroxime axetil.