Specific CT findings in Krabbe disease

Kwan, Eddie; Drace, John E.; Enzmann, Dieter R.

doi:10.2214/ajr.143.3.665

Cited by 23 publications

(17 citation statements)

References 9 publications

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“…ICC was described in older pathological reports and CT‐era papers on infantile Krabbe disease (Fig. k) . We recently reported three patients with otherwise typical infantile Krabbe disease demonstrating calcification in the internal capsule and corona radiata, presumably within areas of abnormal white matter and globoid cell accumulation .…”

Section: Genetic Neurodegenerative Diseasesmentioning

confidence: 72%

“…3k). 83 We recently reported three patients with otherwise typical infantile Krabbe disease demonstrating calcification in the internal capsule and corona radiata, presumably within areas of abnormal white matter and globoid cell accumulation. 84 The radiological and clinical features were otherwise typical of the disease, serving to emphasize that the significance of ICC should be interpreted in the context of all the other radiological and clinical findings.…”

Section: Krabbe Diseasementioning

confidence: 98%

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Intracranial calcification in childhood: a review of aetiologies and recognizable phenotypes

Livingston

Stivaros

Warren

et al. 2013

Develop Med Child Neuro

140

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Intracranial calcification (ICC) is a common finding on neuroimaging in paediatric neurology practice. In approximately half of all cases the calcification occurs in damaged, neoplastic, or malformed brain. For the large number of other disorders in which ICC occurs, no common pathogenetic mechanism can be suggested. Congenital infection, particularly with cytomegalovirus, accounts for a significant proportion of all cases. However, some genetic diseases, in particular Aicardi–Goutières syndrome, Band‐like calcification, and RNASET2‐related disease, may mimic congenital infection; therefore, a full consideration of the radiological and clinical features is necessary before concluding that congenital infection is the cause. In some disorders calcification is a universal finding, in others it is a frequent occurrence, and in some it is only an occasional finding. Characteristic patterns of calcification are seen in a number of conditions, and a systematic approach to the identification and description of radiological findings, taken together in the context of the clinical scenario, allows a diagnosis to be made in many cases. Nonetheless, there remain a number of presumed genetic disorders associated with ICC for which the underlying molecular cause has not yet been identified.

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Section: Genetic Neurodegenerative Diseasesmentioning

confidence: 72%

Section: Krabbe Diseasementioning

confidence: 98%

Intracranial calcification in childhood: a review of aetiologies and recognizable phenotypes

Livingston

Stivaros

Warren

et al. 2013

Develop Med Child Neuro

140

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“…In addition, some calcifications remained purely periventricular in location. In group 2B (patients with OC), from the liter ature, birth asphyxia [19], ischemic episodes [19] and metabolic disease, including Krabbe's [27], MELAS [28] and Kearns Sayre's [29] are recognized causes of BGC as has occurred in our series. Infectious causes of BGC reported in the literature have also included viral encephalitis (measles, chickenpox, coxsackie B) [5,8,19].…”

Section: Discussionmentioning

confidence: 57%

Significance of Basal Ganglia Calcification on Computed Tomography in Children

Legido

Zimmerman²,

Packer

et al. 1988

Pediatr Neurosurg

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We reviewed 6,428 head computed tomography (CT) scans performed on 4,283 children at our institution over a 3-year period and found basal ganglia calcification (BGC) in 48 (1.1%) of the patients. Their mean age at the time of detection was 5.3 years (range: 0.5–20 years); 16 (33%) patients had cancer, 14 (29%) had tuberous sclerosis or congenital infection and 18 (38%) had other medical conditions. All patients with cancer had been treated with radiation therapy, receiving a mean dose of 4,583 cGy (range: 1,800–5,500 cGy) to the diencephalon, and calcifications first became apparent at a median of 10 months after treatment. Other medical conditions included neonatal asphyxia (3), metabolic disease (3) (Kearns Sayre, MELAS, Krabbe’s), congenital anomalies (3), meningitis (2), Fahr’s disease (1) and others (6). Neurologic symptoms were common in children of all groups, but could not be correlated to BGC changes. Calcium and phosphorus metabolism was evaluated in 19 patients and was abnormal in 1. We conclude that BGC on CT in childhood occur primarily as an aftermath of the cancer treatment or in children with generalized neurologic dysfunction. Many children with BGC are delayed in their development, but calcifications are not directly related to specific forms of neurologic dysfunction. Rather, they appear to serve as markers for more extensive brain damage.

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“…4,[9][10][11] However, this observation is not new, and many reports from the CT and early MRI era described individuals with very high-density areas in the periventricular region of the corona radiata, suggestive of calcification. 2,8,[12][13][14][15] In one of the individuals described by Feanny et al, 14 calcification was confirmed on autopsy. One of the cases reported by Percy et al 7 also demonstrated calcification in the deep white matter in areas undergoing degeneration and cyst formation.…”

Section: Discussionmentioning

confidence: 91%

Intracranial calcification in early infantile Krabbe disease: nothing new under the sun

Livingston

Graziano

Pysden

et al. 2011

Develop Med Child Neuro

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We report the cases of three children, one male and two females, with a diagnosis of early infantile Krabbe disease demonstrating intracranial calcification on computed tomography (CT). The pattern of calcification was similar in all individuals and involved the internal capsule and cerebral white matter. The presence of calcification caused some diagnostic confusion in what was otherwise a typical clinical and radiological presentation. This finding is not new and has previously been described in publications from the 1980s and 1990s reporting the CT and magnetic resonance imaging appearances of Krabbe disease. With increasing use of magnetic resonance as the first imaging modality for investigation of neurological disorders, characteristic CT appearances may be forgotten. This report serves as a reminder that Krabbe disease should be included in the differential diagnosis of disorders causing intracranial calcification.

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Specific CT findings in Krabbe disease

Cited by 23 publications

References 9 publications

Intracranial calcification in childhood: a review of aetiologies and recognizable phenotypes

Intracranial calcification in childhood: a review of aetiologies and recognizable phenotypes

Significance of Basal Ganglia Calcification on Computed Tomography in Children

Intracranial calcification in early infantile Krabbe disease: nothing new under the sun

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