Periventricular Calcification



Periventricular Calcification


Susan I. Blaser, MD, FRCPC



DIFFERENTIAL DIAGNOSIS


Common



  • 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


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 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



SELECTED REFERENCES

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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