Periventricular Calcification

Periventricular Calcification
Susan I. Blaser, MD, FRCPC
DIFFERENTIAL DIAGNOSIS
Common
  • TORCH, General
    • Congenital CMV
    • Congenital Toxoplasmosis
    • Congenital Herpes Encephalitis
    • Congenital HIV
    • Congenital Rubella
  • 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
ESSENTIAL INFORMATION
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 calcifications
      • 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 calcifications identified in child > 2 months old
    • If congenital infection is diagnostic consideration, obtain NECT to detect calcifications
  • Congenital CMV
    • Most common cause of intrauterine infection in USA
    • Timing of infection predicts pattern of damage
    • Hypomyelination
    • Cortical gyral anomalies
    • Microcephaly
    • Symmetric periventricular calcifications in 30-70%
  • Congenital Toxoplasmosis
    • Periventricular and scattered calcifications
    • Hydrocephalus (colpocephaly-like)
  • Congenital Herpes Encephalitis
    • Calcification pattern varies in HSV2
      • Asymmetric periventricular
      • Scattered periventricular and deep gray
      • Subcortical white matter and cortex
      • Calcification pronounced in foci of hemorrhagic ischemia
      • Like rubella, rare cause of “stone brain”
    • Brain atrophy or cystic encephalomalacia
      • Focal or diffuse
  • Congenital HIV
    • Vertical HIV infection
    • Basal ganglia calcifications
    • Atrophy
    • Consider congenital HIV
      • If bilateral symmetric basal ganglia calcifications present and child is > 2 months old
  • Congenital Rubella
    • Periventricular and scattered calcifications
    • Scattered or hazy basal ganglia calcifications
    • Rarely “stone brain”
      • Extensive gyral calcification
      • Gliosis
    • Micro-infarcts
  • Tuberous Sclerosis Complex
    • a.k.a. Bourneville disease
    • Classic triad
      • Mental retardation
      • Epilepsy
      • Adenoma sebaceum
    • Look for cutaneous markers of tuberous sclerosis
    • 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
      • Shrunken calcified nodule
  • Tuberculosis
    • Best diagnostic clue
      • Basal meningitis
      • Pulmonary tuberculosis
    • Acute
      • Typically basal meningitis
      • ± localized CNS tuberculoma
    • Chronic
      • Residual pachymeningeal
      • ± localized calcifications
    • “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
      • Ocular coats: Retinal telangiectasia and exudate
      • CNS small blood vessel calcification
      • Extensive thalamic and gyral calcification
      • Defects of bone marrow and integument
      • Growth failure
Image Gallery
Coronal NECT shows classic findings of TORCH. Note the linear periventricular Ca++ image with scattered Ca++ foci within the 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 10, 2016 | Posted by in PEDIATRICS | Comments Off on Periventricular Calcification

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