Cerebral Small Vessel Disease–Intolerability Transient Cognitive Collapse (CSVD-ITCC): A Novel Syndrome of Reversible Cognitive Dysfunction in Context of Severe Cerebral Small Vessel Disease
Taha A-Hakam1, Nazar Elnoor2
1Alyaa Neuroscience Clinical and Research Centre ANCRC, 2Algadaref Teaching Hospital
Objective:

To describe a case series highlighting Cerebral Small Vessel Disease-Intolerability Transient Cognitive Collapse (CSVD-ITCC) as a novel clinical syndrome of transient cognitive impairment in patients with severe CSVD.

Background:

Cerebral small vessel disease (CSVD) is a common cause of chronic cognitive impairment and vascular dementia. The onset and course of cognitive impairment in patients with CSVD usually show no constant pattern. We hypothesize that the episodes described in this series, which we term ITCC, occur due to brain's inability to tolerate additional structural or psychological insults in context of CSVD. While Transient Neurological Attacks (TNAs) have been used to describe similar phenomena, ITCC may more specifically capture cognitive collapse due to intolerability rather than unknown precise mechanisms.

Design/Methods:

This case series included 3 patients aged 78, 55, and 48 years, with normal cognitive background.  All of them presented with severe cognitive impairment that precluded formal cognitive assessment. Two patients were also agitated and violent. One patient (78) presented with acute confusion and agitation after a long airplane and bus trip, while the other two presented with transient right-sided weakness. All had bilateral periventricular white matter hyperintensities (PWMH) graded 3 on the Fazekas Scale on MRI, without diffusion restriction on DWI. Conditions which might affect cognition like infection, electrolyte and metabolic disturbances were all excluded.

Results:

Cognitive recovery occurred within 24 hours, 48 hours, and 72 hours in patients 1, 2, and 3, respectively. Improvement of weakness in patients 2 & 3 together with absence of restriction in DWI confirms diagnosis of TIA. The episodes resolved spontaneously, supporting the hypothesis that severe CSVD make these patients vulnerable to cognitive collapse which is basically transient.

Conclusions:

CSVD-ITCC represents a potential refinement or replacement for the term TNAs, focusing on cognitive collapse due to brain intolerability, thus underscoring the role of CSVD in leading to transient cognitive dysfunction.

10.1212/WNL.0000000000210359
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