Macrocephaly with dilated Virchow-Robin spaces: a new clinical entity?

Dilatation of VRS has been associated with aging process and is present in various disease conditions (e.g. metabolic, vascular, inflammatory, neoplastic).

Macrocephaly combined with dilated Virchow-Robin spaces has recently been discussed as a new clinical entity.

We describe two male patients two and five years of age with macrocephaly and dilated perivascular spaces on magnetic resonance imaging.

These patients had a mild muscle hypotonia and mild psychomotoric retardation. Biochemical and metabolic tests were normal. The cerebral MRI showed curvilinear periventricular areas that are isointense to CSF on all imaging sequences, in one case particularly in the region of the trigones; as a classical appearance for dilated perivascular spaces. In one case there was also an enlargement of the inner and outer CSF spaces as a sign for brain atrophy.

In several publications an association between macrocephaly and dilated perivascular spaces in patients with mild psychomotoric retardation has been described.

These data may indicate that the dilatation of perivascular spaces reflects an underlying brain pathology. Due to the perivascular location a vascular pathology has to be considered. The role of VRS as drainage pathways of interstitial fluid in the brain and their immunologic function may be of relevance.

Long-term-follow-up and repeated MRI examinations are required for further characterisation of the supposed new entity with combined macrocephaly and dilated perivascular spaces and its potential impact.