Cognitive impairment due to widespread enlarged perivascular spaces

Abstract

Perivascular spaces, also known as Virchow-Robin spaces, are usually considered as a normal, asymptomatic finding. However, this finding can occasionally demonstrate an atypical appearance and can be symptomatic. We report herein a rare case of cognitive impairment associated with extremely enlarged perivascular spaces. A 68-year-old Japanese woman visited our hospital with a 1-year history of progressive memory impairment. In addition to temporal disorientation and short-term memory impairment, neuropsychological testing showed frontal lobe-related symptoms such as slowed thinking processes, reduced verbal fluency, attention deficit, and reduced working memory. Magnetic resonance imaging of the brain showed widespread enlarged perivascular spaces almost symmetrically in the subcortical white matter of bilateral hemispheres, prominently in bilateral insulas, and frontal opercula. On 99mTc-ethyl cysteinate dimer single photon emission computed tomography, hypoperfusion was apparent in bilateral insulas and frontal opercula where enlarged periventricular spaces were prominent, whereas cerebral perfusion was preserved in areas where enlargement of perivascular spaces was mild or absent. Because symptoms were consistent with the distribution of the enlarged perivascular spaces and hypoperfusion in the brain, cognitive impairment due to enlarged perivascular spaces was diagnosed. Clinicians should note enlarged perivascular spaces as a potential cause of neurological deficits including cognitive impairment.

The etiology and evolution of magnetic resonance imaging-visible perivascular spaces: Systematic review and meta-analysis

“…Several case reports have confirmed the mostly stable nature of these structures ( Ogawa et al, 1995 ; Sawada et al, 1999 ; Flors et al, 2010 ; Tsutsumi et al, 2011 ; Sankararaman et al, 2013 ; Tseng et al, 2013 ; Taniguchi et al, 2017 ), some of them with follow-up periods of up to 7 years ( Manara et al, 2010 ). However, a subset of these MVPVS can enlarge over time ( Mehta et al, 2013 ; Gopinath et al, 2018 ; Yamaguchi et al, 2021 ). This can result in compression of adjacent CNS structures with concomitant neurological disorders such as non-communicating hydrocephalus ( Papayannis et al, 2003 ), necessitating surgical decompression ( Rocha et al, 2013 ; Rivet et al, 2017 ).…”

“…Typical locations of MVPVS are in the basal ganglia along lenticulostriate vessels, centrum semiovale, and midbrain (ponto-mesencephalic junction) ( Kwee and Kwee, 2007 ). Less frequent MVPVS locations are: insula ( Yamaguchi et al, 2021 ), hippocampus ( Zhu et al, 2011 ), anterior temporal lobe ( McArdle et al, 2020 ), corpus callosum ( Manara et al, 2010 , 2011 ), mesencephalon-thalamic junction ( Salzman et al, 2005 ), and cerebellum ( Alqahtani et al, 2014 ). Although many agree that MVPVS should be an imaging component of cerebral vessel disease ( Francis et al, 2019 ), emerging data suggest that it can also be a feature of metabolic ( Manara et al, 2011 ), neurodegenerative ( Charidimou et al, 2017 ), and neuroinflammatory diseases ( Granberg et al, 2020 ).…”

Case Report: Focal leptomeningeal disease, atypical cancer of unknown primary site in Peru

“…10,11 Regarding the physiopathology of this disease, it has been postulated that the perivascular spaces with the perivascular channels act as a bridge through angiotropic mechanisms. 9,12,13 In addition, the association of dural thickening and enhancement has been reported, as a radiological expression of tumor involvement and venous congestion secondary to the perivascular involvement of the disease. 9,11,14 The neurological symptoms associated with this condition have been described as less severe than those associated with classic leptomeningeal disease.…”

Case Report: Focal leptomeningeal disease, atypical cancer of unknown primary site

“…10,11 Regarding the physiopathology of this disease, it has been postulated that the perivascular spaces with the perivascular channels act as a bridge through angiotropic mechanisms. 9,12,13 In addition, the association of dural thickening and enhancement has been reported, as a radiological expression of tumor involvement and venous congestion secondary to the perivascular involvement of the disease. 9,11,14 The neurological symptoms associated with this condition have been described as less severe than those associated with classic leptomeningeal disease.…”