CASE 1041 Published on 30.07.2001

Dilated Virchow-Robin spaces and ectodermal dysplasia

Section

Neuroradiology

Case Type

Clinical Cases

Authors

R.N.Sener

Patient

8 years, male

Categories

No Area of Interest ; Imaging Technique MR

No Procedure ; No Special Focus ;

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Clinical History

Learning difficulties. Ectodermal dysplasia.

Imaging Findings

The patient had learning difficulties. Abnormal teeth, and dystrophic nails were noted, consistent with ectodermal dysplasia. A brain MRI study was performed.

Discussion

A number of recent papers dealt with a combination of dilated Virchow-Robin spaces, and ectodermal dysplasia. Virchow-Robin spaces are the perivascular spaces lined by ependymal/leptomeningeal cells. Small Virchow-Robin spaces, less than 2mm, can be found on MRI in almost all age groups. However, if these are larger and numerous, the condition may reflect some sort of a degeneration of the brain parenchyma, at least a manifestation of atrophy of the white matter. Extremely dilated Virchow-Robin spaces (cystic dilatation) has been found in three patients with ectodermal dysplasia and facial abnormalities, however, another patient was reported without clinical correspondence. An association with Joubert's syndrome has also been reported. In the current patient, Virchow-Robin spaces were wide up to 5 mm, especially in the centrum semiovale. Examination of the brain by MRI is suggested in patients with ectodermal dysplasia.

Differential Diagnosis List

Dilated Virchow-Robin spaces and ectodermal dysplasia

Final Diagnosis

Dilated Virchow-Robin spaces and ectodermal dysplasia

References

[1] Lynch SA, Hall K, Precious S, Wilkies AO, Hurst JA. Two further cases of Sener syndrome: frontonasal dysplasia and dilated Virchow-Robin spaces.
J Med Genet. 2000 Jun;37(6):466-70. (PMID: 10928859)

[2] Bacheschi LA, Magalhaes ACA, Mathias SC. Multiple cystic lesions in white matter without clinical manifestations (unidentified black holes). Neuroradiology. (suppl)1995;37:246-7.

[3] Sener RN. Polycystic brain (cerebrum polycystica vera) associated with ectodermal dysplasia: a new neurocutaneous syndrome.
Pediatr Radiol. 1994;24(2):116-8. (PMID: 8078711)

[4] Sener RN. A patient with ectodermal dysplasia, Joubert's syndrome, and brain cysts.
Comput Med Imaging Graph. 1998 Jul-Aug;22(4):349-51. (PMID: 9840666)

Case information

URL:	https://www.eurorad.org/case/1041
DOI:	10.1594/EURORAD/CASE.1041
ISSN:	1563-4086

Figure 1

Dilated Virchow-Robin spaces

T2-weighted image reveals numerous dilated Virchow-Robin spaces.

T2-weighted image at a lower section shows dilated Virchow-Robin spaces, some of which are as wide as 5 mm. (a few of these, and up to 2 mm is considered normal).

At a lower section, again numerous dilated Virchow-Robin spaces are seen in longitudinal orientation.

Figure 2

Dilated Virchow-Robin spaces

Heavily T1-weighted image (turbo inversion recovery) reveals the dilated Virchow-Robin spaces.

T1-weighted image at lower section again reveals multiple dilated Virchow-Robin spaces.

T1-weighted image at the region of basal ganglia suprisingly reveals a number of almost normal Virchow-Robin spaces. In normal individuals similar sized Virchow-Robin spaces at this region is commonly seen.

T1-weighted image at the region of the temporal lobes and cerebellum reveals a number of Virchow-Robin spaces. Those in the cerebellum are indicated by arrow.