Neurologic Disorders Flashcards

0
Q
Patient with symptoms of craniopharyngioma, what would you expect to find in the CT scan?
​a. attenuated pituitary stalk
​b. suprasellar calcification
​c. Sellar erosion
​d. (di ko maalala)
A

B. suprasellar calcification

NTP: Brain Tumors: Craniopharyngioma (WHO grade I) is a common tumor of childhood, accounting for 7–10% of all childhood tumors. The adamantinomatous variant of craniopharyngioma predominates in childhood. These tumors are solid with cystic components and occur within the suprasellar region. They are minimally invasive, adhere to adjacent brain parenchyma, and engulf normal brain structures. MRI demonstrates the solid tumor with cystic structures containing fluid of intermediate density. CT may show calcifications associated with the solid and cystic wall components.

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1
Q

Most common brain tumor

A

Astrocytoma

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2
Q

a. Simple febrile seizure
b. Complex febrile seizure
c. Meningitis
d. Epilepsy

A

b. Complex febrile seizure

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3
Q

At the ER, a child who presented with generalized tonic clonic seizure was given Diazepam IV and was then admitted. At the wards, the patient again presented with GTC and given Diazepam. After 1 hour, the doctor noted weakness on the right extremity.

a. Complex Febrile Seizure
b. Simple Febrile Seizure

A

no answer given

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4
Q

Patient, 5 year old, presented with gradually violent behavior, irritable, eventually associated with generalized tonic clonic seizures, and brawny skin. What is the diagnosis? The child initially well after a week now presented with cognitive dysfunction, behavioral changes, generalized tonic clonic seizures then myoclonic seizures, choreoathetosis. On PE, there was note of brawny discoloration of the skin, optic neuritis, chorioretinitis. On EEG, there was note of burst activity with normal waves

a. Myoclonic seizure
b. ADEM
c. Rett Syndrome
d. SSPE

A

D. SSPE

Nelson’s 18th: Initial clinical manifestations include: personality changes, aggressive behavior, impaired cognitive function. Myoclonic seizures soon dominate, etc. EEG- bursts of high voltage slow waves interspersed with normal background in the early stages.

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5
Q
GCS score of no eye opening, incomprehensible speech, flexed arms 
A. 2 
B. 4 
C. 6 
D. 8
A
C NTP19 Ch 62 Pediatric Emergency and resuscitation Table 62-3   -- GLASGOW COMA SCALE 
EYE OPENING (TOTAL POSSIBLE POINTS 4) 
Spontaneous               4
To voice                      3
To pain                        2
None                            1
VERBAL RESPONSE (TOTAL POSSIBLE POINTS 5) 
OLDER CHILDREN 
Oriented                       5
Confused                     4
Inappropriate               3
Incomprehensible        2
None                            1

INFANTS AND YOUNG CHILDREN
Appropriate words; smiles, fixes, and follows 5
Consolable crying 4
Persistently irritable 3
Restless, agitated 2
None 1

MOTOR RESPONSE (TOTAL POSSIBLE POINTS 6) 
Obeys                  6     
Localizes pain      5     
Withdraws            4     
Flexion                 3     
Extension             2     
None                    1

Adapted and modified from Teasdale G, Jennett B: Assessment of coma and impaired consciousness: a practical scale, Lancet 2:81–84, 1974.

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6
Q
How many weeks before pregnancy do you start folic acid supplementation? 
A. 2 weeks 
B. 4 weeks 
C. 6 weeks 
D. 8 weeks
A

B Ch 90
The Fetus Folic Acid supplementation decreases the incidence and recurrence of (NTDs). Because the neural tube closes within the 1st 28 days of conception, periconceptional supplementation is needed for prevention. It is recommended that women without a prior history of a NTD ingest 400 µg/day of folic acid throughout their reproductive years. Women with a history of a prior pregnancy complicated by an NTD or a 1st-degree relative with an NTD should have preconceptional counseling and should ingest 4 mg/day of supplemental folic acid beginning at least 1 mo before conception.

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7
Q
Autosomal dominant disease with café au lait spots 
A. von recklinghausen 
B. tuberous sclerosis 
C. 
D.
A

A NTP19 Ch 644
Hyperpigmented lesions Neurofibromatosis Type 1 (Von Recklinghausen Disease)
The café-au-lait spot is the most familiar cutaneous hallmark of the autosomal dominant neurocutaneous syndrome known as neurofibromatosis type 1 (neurofibromin gene)

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8
Q

Mother delivered an anencephalic child
A. The chance of the next child being anencephalic is 45%
B. This will not affect future pregnancies.
C.
D. This can be prevented by the intake of folic acid before pregnancy.

A

D NTP19 Ch 585.4 Myelomeningocoele
Myelomeningocele represents the most severe form of dysraphism, a so-called aperta or open form, involving the vertebral column and spinal cord, which occurs with an incidence of approximately 1/4,000 live births. The cause of myelomeningocele is unknown, but as with all neural tube closure defects including anencephaly, a genetic predisposition exists; the risk of recurrence after one affected child is 3-4% and increases to 10% with 2 prior affected children. Both epidemiologic evidence and the presence of substantial familial aggregation of anencephaly, myelomeningocele, and craniorachischisis indicate heredity, on a polygenic basis, as a significant contributor to the etiology of NTDs. Nutritional and environmental factors have a role in the etiology of myelomeningocele as well. Folate is intricately involved in the prevention and etiology of NTDs. Folate functions in single-carbon transfer reactions and exists in many chemical forms. Folic acid (pteroylmonoglutamic acid), which is the most oxidized and stable form of folate, occurs rarely in food but is the form used in vitamin supplements and in fortified food products, particularly flour. Most naturally occurring folates (food folate) are pteroylpolyglutamates, which contain 1-6 additional glutamate molecules joined in a peptide linkage to the γ-carboxyl of glutamate. Folate coenzymes are involved in DNA synthesis, purine synthesis, generation of formate into the formate pool, and amino acid interconversion; the conversion of homocysteine to methionine provides methionine for the synthesis of S-adenosyl-methionine (SAM-e, an agent important for in vivo methylation). Mutations in the genes encoding the enzymes involved in homocysteine metabolism include 5,10 methylenetetrahydrofolate reductase (MTHFR), cystathionine β-synthase, and methionine synthase. An association between a thermolabile variant of MTHFR and mothers of children with NTDs might account for up to 15% of preventable NTDs. Maternal periconceptional use of folic acid supplementation reduces the incidence of NTDs in pregnancies at risk by at least 50%. To be effective, folic acid supplementation should be initiated before conception and continued until at least the 12th wk of gestation, when neurulation is complete. The mechanisms by which folic acid prevents NTDs remain poorly understood.

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9
Q

Most common brain tumor

A

Astrocytoma

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10
Q
  1. A child was brought because of persistent crying after a fall. He had an episode of losing consciousness and had a seizure. But after that, he was crying again. PE , Neuro exam was Normal. The child has…
    a. Breath holding
    b. Pallid spells
    c. Head trauma ??
A

B.
p. 2010
Pallid spells are typically initiated by a painful experience such as FALLING and striking the head or a sudden startle. The child stops breathing, rapidly loses consciousness, becomes pale and hypotonic, and may have a tonic seizure.

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11
Q

True of febrile seizures

a. Phenobarbital as prophylaxis for recurrence
b. Oral Diazepam is effective & safe in reducing risk of recurrence of febrile seizure for the duration of illness

A

a. Phenobarbital as prophylaxis for recurrence – false
b. Oral Diazepam is effective & safe in reducing risk of recurrence of febrile seizure for the duration of illness – true
p. 1994

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12
Q

Complex Febrile Seizure, which is not true

a. repeated episodes in a day
b. more than 5 minutes
c. focal seizure activity
d. focal findings

A

b – not true p. 1994

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13
Q
  1. Normal CSF glucose except
    a. Viral
    b. abscess
    c. fungal
    d. subdural empyema
A

b. fungal p. 2039 table 594-1

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14
Q

A10-month old boy was brought to PER due to seizures. He had a 2-day history of fever (38C) and colds, not associated with increased sleeping time. Normal neurological examination. What is the probable diagnosis?

a. Febrile seizure
b. Inborn error of metabolism
c. CNS infection
d. Metabolic imbalance

A

a febrile seizures

Seizures associated with fever in the absence of CNS infection, acute electrolyte imbalance or history of afebrile seizures in a young child.
CPG: 3months-6 years, T 37.8C
Nelson: 9mo-5 years, T>=39C

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15
Q
Which of the following would be the first logical step in the work-up of this patient?
a Cranial CT scan or ultrasound
b EEG
c Serum electrolytes
d CSF analysis
A

D csf analysis
Suspicion of CNS infection
Neonates with clinical sepsis neonatorum
Children 18 months with clinical signs of meningitis
Suspicion of SAH
Suspicion of acute demyelinating disorder
Suspicion of carcinomatous meningitis

Therapeutic

16
Q

1 year and 3-month old boy was brought to the PER because of fever and decreased in sensorium. He has been having cough and fever for 3 days. The following are probable differential diagnosis, except:

a. Viral encephalitis
b. Bacterial meningitis
c. TB meningitis
d. None of the above

A

c tb meningitis
viral-acute Colds, headache, behavioral change
bacterial- acute-subacute, Bacterial infection in body -
tb-chronic 2weeks cough and fever
fungal- chronic, immunocompromised