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Questions and Answers:

 

1.      The most common site of intracranial calcifications in the pediatric population is:

a.       Cerebellum

b.      Periventricular region

c.       Pineal gland

d.      Thalamus

e.       Falx cerebri

 

Answer: c. In the pediatric population, they are typically seen in the pineal gland and choroid plexus. Pineal calcifications <1cm, appear as dots and tend to be benign in 40% of individuals less than 20 years old. Whereas pineal calcifications >1cm in patients younger than 9 years should be regarded as pathologic and warrant further investigation.

   

2.      A blush-like pattern calcifications in the bilateral basal ganglia in a child is typically seen in:

a.       Tuberous sclerosis

b.      Raine syndrome

c.       Krabbe’s disease

d.      Sturge-Weber  syndrome

e.       Neurofibromatosis

 

Answer: c. Krabbe’s disease is an autosomal recessive demyelinating disorder affecting infants due to galactocerebroside b-galactosidase deficiency leading to accumulation of abnormal lipids. A symmetrical blush-like pattern of calcifications in the bilateral basal ganglia is characteristic.

 

3.      A 3 day-old boy was admitted to the neonatal intensive care unit after an episode of tonic-clonic seizure. His mother denied prenatal care. Non-contrast CT was showed both sub-ependymal and periventricular calcifications with cerebral volume loss. What is the most likely diagnosis?

a.       Tuberous sclerosis

b.      Congenital CMV

c.       Congenital hypothyroidism

d.      Neurofibromatois

e.       Hypoglycemia-induced seizure

 

Answer: b. Congenital Cytomegalovirus (CMV): Incidence is 0.2-2.4% of live births. Intracranial calcifications are seen in 77% of those with imaging abnormalities typically in the sub-ependymal and periventricular regions of the brain along with brain atrophy.

   

4.      Among all the intra-axial brain tumors, which of the following is mostly associated with intracranial calcifications?

a.       Oligodendroglioma

b.      Pilocytic astrocytoma

c.       Dysembryonic neuroectodermal tumors

d.      Medulloblastoma

e.       Metastasis

 

Answer: a. Oligodendrogliomas exhibit the highest frequency of calcifications (up to 90%). Calcifications can be scattered dots or clumped together to form large nodules, located centrally or peripherally and frequently in the frontal lobes.

   

5.      A 50-year-old female patient presented with dry cough, fatigue, and shortness of breath. On physical exam she has tender reddish bumps on the skin. Review of the systems revealed seizures and depression. CXR showed enlarged hilar lymph nodes and CT scan of the brain showed small calcified masses in the leptomeninges and periventricular white matter. What is the most likely diagnosis?

a.       Hypothyroidism

b.      Hyperparathyroidism

c.       Hemochromatosis

d.      Chronic lead toxicity

e.       Sarcoidosis

 

Answer: e. Sarcoidosis affects many organs including the CNS, lungs and skin. On non-contrast CT scan of the brain, small calcified granulomas can be seen in the leptomeninges, periventricular white matter, pons, hypothalamus, pituitary stalk, and optic chiasm with varying degrees of edema.