Many inborn errors of metabolism cause generalized convulsions in the newborn period. Prominent hiccups, persistent generalized seizures, and lethargy rapidly leading to coma are featurof

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Pediatric Neurology Test Questions Questions

Question 1 of 5

Many inborn errors of metabolism cause generalized convulsions in the newborn period. Prominent hiccups, persistent generalized seizures, and lethargy rapidly leading to coma are featurof

Correct Answer: C

Rationale: Nonketotic hyperglycinemia presents with hiccups, seizures, and coma from glycine accumulation disrupting neurotransmission. Propionic acidemia and maple syrup urine disease cause acidosis and odor, not hiccups. Leigh and adrenoleukodystrophy are later-onset. Glycinunique signs make C the correct answer.

Question 2 of 5

Café-au-lait maculare not specific for NF-1; they may be seen in the following EXCEPT

Correct Answer: B

Rationale: Café-au-lait maculoccur in NF-1, Noonan , Fanconi , Gaucher , and Hurler syndromes, tied to genetic or metabolic defects. Ataxia telangiectasia featurtelangiectasia, not macules. This phenotypic distinction make B the exception and correct answer.

Question 3 of 5

Prematurity is a major cause of

Correct Answer: A

Rationale: Prematurity commonly causspastic diplegia , from periventricular leukomalacia in preterm infants (~32-36 weeks), affecting legs most. Quadriplegia and hemiplegia link to broader insults; athetoid and dyskinetic to basal ganglia injury. Diplegias preterm tie makes A the correct answer.

Question 4 of 5

The MOST common focal presentation of arterial ischemic stroke is

Correct Answer: A

Rationale: Hemiparesis is the most common focal sign of childhood arterial ischemic stroke, per studi(e.g., ICH), from MCA territory infarction. Visual , speech , sensory , and balance deficits are less frequent. Motor predominance makes A the correct answer.

Question 5 of 5

The diagnosis of diffuse CNS infections depends on examination of cerebrospinal fluid (CSF) obtained by lumbar puncture (LP). The cerebrospinal fluid (CSF) protein may reach 3,000 (mg/dL) in

Correct Answer: B

Rationale: Tuberculous meningitis can elevate CSF protein to 3,000 mg/dL from chronic inflammation and exudate. Bacterial peaks lower (~500-1,000); fungal and amebic rarely exceed 1,000; empyema is focal. TBs severity make B the correct answer.

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