Periventricular Calcification

Periventricular Calcification

Susan I. Blaser, MD, FRCPC



  • TORCH, General

    • CMV, Congenital

    • Toxoplasmosis, Congenital

    • Herpes Encephalitis, Congenital

    • HIV, Congenital

    • Rubella, Congenital

  • Tuberous Sclerosis Complex

Less Common

  • Neurocysticercosis

  • Tuberculosis

  • Ventriculitis (Chronic)

  • Germinal Matrix Hemorrhage

Rare but Important

  • Radiation and Chemotherapy

  • Pseudo-TORCH

    • Aicardi-Goutières Syndrome

    • Coats-Plus Syndrome


Key Differential Diagnosis Issues

  • Look for associations

    • Brain destruction

    • Malformations

    • Other loci of calcification

    • History

Helpful Clues for Common Diagnoses

  • TORCH, General

    • Classic acronym for congenital infections

      • Caused by transplacental transmission of pathogens

      • TOxoplasmosis, Rubella, Cytomegalovirus, Herpes

      • All cause parenchymal Ca++

      • Most can cause lenticulostriate mineralization, vasculopathy

      • Some (CMV) cause migrational defects

      • Some (syphilis, herpes) cause meningitis, meningoencephalitis

      • Some (e.g., CMV) cause germinolytic cysts

      • Others (e.g., rubella, HSV) cause striking lobar destruction/encephalomalacia

    • Congenital HIV, syphilis also considered part of TORCH

    • Consider congenital HIV if bilateral symmetric basal ganglia C++ identified in child > 2 months old!

    • If congenital infection is diagnostic consideration, obtain NECT to detect Ca++

  • CMV, Congenital

    • Most common cause of intrauterine infection in USA

    • Timing of infection predicts pattern of damage

    • Hypomyelination

    • Cortical gyral anomalies

    • Microcephaly

    • Symmetric periventricular Ca++ in 30-70%

  • Toxoplasmosis, Congenital

    • Periventricular & scattered Ca++

    • Hydrocephalus (colpocephaly-like)

  • Herpes Encephalitis, Congenital

    • Calcification pattern varies in HSV2

      • Asymmetric periventricular

      • Scattered periventricular and deep gray

      • Subcortical white matter & cortex

      • Calcification pronounced in foci of hemorrhagic ischemia

      • Like rubella, rare cause of “stone brain”

    • Brain atrophy or cystic encephalomalacia

      • Focal or diffuse

  • HIV, Congenital

    • Vertical HIV infection

    • Basal ganglia Ca++, atrophy

    • Consider congenital HIV if bilateral symmetric basal ganglia C++ identified in child > 2 months old!

  • Rubella, Congenital

    • Periventricular and scattered

    • Scattered or hazy basal ganglia Ca++

    • Rare “stone brain”

      • Extensive gyral calcification & gliosis

    • Micro-infarcts

  • Tuberous Sclerosis Complex

    • Look for cutaneous markers of TS

    • Subependymal nodules

      • Variable-sized periventricular calcifications

    • Cortical tubers also calcify

Helpful Clues for Less Common Diagnoses

  • Neurocysticercosis

    • Best clue: Dot inside cyst

    • Usually convexity subarachnoid space

    • Also gray-white junction, intraventricular

    • Nodular calcified (healed) stage

      • Shrinks to small Ca++ puncta or nodule

  • Tuberculosis

    • Best diagnostic clue: Basal meningitis and pulmonary TB

    • Acute

      • Typically basal meningitis

      • ± Localized CNS tuberculoma

    • Chronic

      • Residual pachymeningeal

      • ± Localized Ca++

    • “Target sign”

      • Calcification surrounded by enhancing rim (not specific)

  • Ventriculitis (Chronic)

    • Areas of prior hemorrhagic infarction prone to dystrophic calcification

  • Germinal Matrix Hemorrhage

    • Occasional ependymal, germinal matrix calcific foci

Helpful Clues for Rare Diagnoses

  • Radiation and Chemotherapy

    • History!

    • Mineralizing microangiopathy

  • Pseudo-TORCH

    • Aicardi-Goutières Syndrome

      • “Mendelian mimic of congenital infection”

      • Multifocal punctate calcifications

      • Variable locations including periventricular white matter, basal ganglia, dentate nuclei

      • Elevated CSF interferon (IFN-α)

      • TREX1 mutations in some

    • Coats-Plus Syndrome

      • a.k.a., cerebroretinal microangiopathy with calcifications and cysts (CRMCC)

      • Ocular coats: Retinal telangiectasia & exudate

      • CNS small blood vessel calcification

      • Extensive thalamic and gyral calcification

      • Defects of bone marrow & integument

      • Growth failure


1. Briggs TA et al: Cerebroretinal microangiopathy with calcifications and cysts (CRMCC). Am J Med Genet A. 146A(2):182-90, 2008

2. Crow YJ et al: Aicardi-Goutières syndrome: an important Mendelian mimic of congenital infection. Dev Med Child Neurol. 50(6):410-6, 2008

3. Rice G et al: Clinical and molecular phenotype of Aicardi-Goutieres syndrome. Am J Hum Genet. 81(4):713-25, 2007

4. Linnankivi T et al: Cerebroretinal microangiopathy with calcifications and cysts. Neurology. 67(8):1437-43, 2006

5. Abdel-Salam GM et al: Aicardi-Goutières syndrome: clinical and neuroradiological findings of 10 new cases. Acta Paediatr. 93(7):929-36, 2004

6. Malinger G et al: Fetal cytomegalovirus infection of the brain: the spectrum of sonographic findings. AJNR Am J Neuroradiol. 24(1):28-32, 2003

7. Numazaki K et al: Intracranial calcification with congenital rubella syndrome in a mother with serologic immunity. J Child Neurol. 18(4):296-7, 2003

8. Tanaka F et al: Association of osteopontin with ischemic axonal death in periventricular leukomalacia. Acta Neuropathol. 100(1):69-74, 2000

Image Gallery

Coronal NECT shows classic findings of TORCH. Note linear periventricular Ca++ image with scattered Ca++ foci within cortex image in this deaf child, suggesting prior intrauterine CMV exposure.

Sagittal T2WI MR shows a thick cortex with small gyri, hyperintense white matter image, and a thin layer of calcification image in the same 18 month old deaf toddler.

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Aug 7, 2016 | Posted by in NEUROLOGY | Comments Off on Periventricular Calcification

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