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Intracranial calcifications in childhood: Part 2

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Abstract

This article is the second of a two-part series on intracranial calcification in childhood. In Part 1, the authors discussed the main differences between physiological and pathological intracranial calcification. They also outlined histological intracranial calcification characteristics and how these can be detected across different neuroimaging modalities. Part 1 emphasized the importance of age at presentation and intracranial calcification location and proposed a comprehensive neuroimaging approach toward the differential diagnosis of the causes of intracranial calcification. Pathological intracranial calcification can be divided into infectious, congenital, endocrine/metabolic, vascular, and neoplastic. In Part 2, the chief focus is on discussing endocrine/metabolic, vascular, and neoplastic intracranial calcification etiologies of intracranial calcification. Endocrine/metabolic diseases causing intracranial calcification are mainly from parathyroid and thyroid dysfunction and inborn errors of metabolism, such as mitochondrial disorders (MELAS, or mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes; Kearns–Sayre; and Cockayne syndromes), interferonopathies (Aicardi–Goutières syndrome), and lysosomal disorders (Krabbe disease). Specific noninfectious causes of intracranial calcification that mimic TORCH (toxoplasmosis, other [syphilis, varicella-zoster, parvovirus B19], rubella, cytomegalovirus, and herpes) infections are known as pseudo-TORCH. Cavernous malformations, arteriovenous malformations, arteriovenous fistulas, and chronic venous hypertension are also known causes of intracranial calcification. Other vascular-related causes of intracranial calcification include early atherosclerosis presentation (children with risk factors such as hyperhomocysteinemia, familial hypercholesterolemia, and others), healed hematoma, radiotherapy treatment, old infarct, and disorders of the microvasculature such as COL4A1- and COL4A2-related diseases. Intracranial calcification is also seen in several pediatric brain tumors. Clinical and familial information such as age at presentation, maternal exposure to teratogens including viruses, and association with chromosomal abnormalities, pathogenic genes, and postnatal infections facilitates narrowing the differential diagnosis of the multiple causes of intracranial calcification.

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Authors and Affiliations

  1. Division of Neuroradiology, Department of Radiology, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA, 19104, USA

    Fabricio Guimarães Gonçalves, Sara Reis Teixeira, César Augusto Pinheiro Ferreira Alves, Savvas Andronikou & Arastoo Vossough

  2. Neuroradiology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

    Luca Caschera

  3. Department of Pathology and Laboratory Medicine, Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA

    Angela Nicole Viaene

  4. Neuroradiology Unit, Pediatric Neuroradiology Section, ASST Spedali Civili, Brescia, Italy

    Lorenzo Pinelli

  5. Department of Radiology, Great Ormond Street Hospital, London, UK

    Kshitij Mankad

  6. Division of Neurology, Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Xilma Rosa Ortiz-Gonzalez

  7. Department of Neurology, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA

    Xilma Rosa Ortiz-Gonzalez

  8. Department of Radiology, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA

    Savvas Andronikou & Arastoo Vossough

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  1. Fabricio Guimarães Gonçalves
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Gonçalves, F.G., Caschera, L., Teixeira, S.R. et al. Intracranial calcifications in childhood: Part 2. Pediatr Radiol 50, 1448–1475 (2020). https://doi.org/10.1007/s00247-020-04716-y

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