ATI RN
Pediatric Neurology Questions Questions
Question 1 of 5
Small vessel childhood primary angiitis of the CNS (SVcPACNS) is usually presented with
Correct Answer: C
Rationale: SVcPACNS presents with cognitive dysfunction , per studies, from diffuse small vessel inflammation. Stroke , seizur, personality chang, and social loss are less typical. Cognitive impact makes C the correct answer.
Question 2 of 5
The MOST common cause of viral meningoencephalitis are
Correct Answer: B
Rationale: Enterovirusare the most common viral meningoencephalitis cause (~80%), per CDC, due to fecal-oral spread (e.g., coxsackie). Arbovirus, HSV-1 , VZV , and mumps are less frequent. Enterovirus prevalence make B the correct answer.
Question 3 of 5
Idiopathic intracranial hypertension, also known as pseudotumor cerebri, is a clinical syndrome that mimics brain tumors and is characterized by the following EXCEPT
Correct Answer: C
Rationale: Pseudotumor cerebri featurhigh ICP , normal CSF , papilledema , and normal ventricl. Slightly decreased ventricular size is not typical; ventriclare normal or slit-like, not reduced. This imaging nuance makes C the exception and correct answer.
Question 4 of 5
A 4-year-old female has experienced progressive loss of ambulation for a 2-year period. On examination, the child is apathetic and uninterested in her surroundings. She has horizontal nystagmus and optic atrophy. Her voice is dysarthric. She is hypotonic, and her deep tendon reflexare absent. A sibling died at age 6 with a similar history. Motor nerve conduction velocitishow marked slowing, and CT shows diffuse symmetric attenuation of cerebral and cerebellar white matter. The most likely diagnosis is
Correct Answer: B
Rationale: Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by arylsulfatase A deficiency, leading to sulfatide accumulation in myelin, which matchthis case. Progressive ambulation loss, apathy, nystagmus, optic atrophy, dysarthria, hypotonia, absent reflexes, and slowed nerve conduction are classic signs of MLD, typically onset in late infantile form (1-4 years). Diffuse white matter attenuation on CT reflects demyelination. A siblings similar death suggests autosomal recessive inheritance, common in MLD. Multiple sclerosis is relapsing-remitting, rare in young children, and shows focal lesions, not diffuse attenuation. GM2 gangliosidosis (Tay-Sachs) presents earlier with seizurand cherry-red spots, not nystagmus or slow conduction. Neuronal ceroid lipofuscinosis (D in original) has seizurand vision loss but not hypotonia or conduction slowing. Pelizaeus-Merzbacher involvspasticity, not hypotonia, and is X-linked, less likely with a female sibling affected. MLDs clinical and imaging fit make B the correct answer.
Question 5 of 5
Causof megalocephaly include all of the following EXCEPT
Correct Answer: A
Rationale: Megalocephaly (large head) results from chronic subdural effusions , hydrocephalus , Canavan disease (D in original), congenital CMV , and familial traits , all increasing brain or skull size via fluid, tissue, or genetics. Thalassemia , a hemoglobinopathy, causmarrow hyperplasia and skull thickening (e.g., frontal bossing), but not true megalocephaly, which involvbrain or CSF volume. Subdurals expand skull space, hydrocephalus enlargventricles, and familial casreflect benign inheritance. Thalassemias skeletal focus, not brain enlargement, makes A the exception and correct answer, per differential diagnostic criteria.