Disproportionately enlarged subarachnoid space hydrocephalus (DESH) is the characteristic feature of idiopathic normal pressure hydrocephalus. We aimed to characterize the prevalence, development, and association of DESH to cognitive deficit in a large population. We reviewed the data of 1384 subjects eligible for the present study among 1590 participants who underwent magnetic resonance imaging (MRI) in the Ohasama Study, a population-based study in Ohasama, Japan. The participants with Mini-Mental State Examination (MMSE) score < = 25 were assumed to have cognitive deficit and DESH was evaluated by reviewing the MRIs. We assessed the association between DESH, Evans index (EI), and cognitive deficit using multivariate logistic regression models adjusted for relevant confounders. Furthermore, we evaluated the new development of DESH and the deterioration of cognitive function in the participants with DESH. There were nine participants with DESH (0.65%), seven of whom showed cognitive deficit. DESH was significantly associated with cognitive deficit in multivariate regression analyses (odds ratio; 8.50 [95% confidence interval: 1.61–44.88]). In the 669 participants who underwent follow-up MRI, we found four participants newly presenting with DESH; the development of DESH was observed before/after the presence of EI > 0.3. We also found two participants with existing DESH showing no remarkable worsening in MMSE and EI. The present study demonstrated a positive association between the presence of DESH and cognitive deficit. DESH can develop independently of EI > 0.3, and ventricular enlargement in combination with DESH may be an important factor in the worsening of cognitive deficit.
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