Disproportionately enlarged subarachnoid space hydrocephalus (DESH)

Case contributed by Farah Abu Jebbeh
Diagnosis certain

Presentation

The patient presented with dementia, gait disturbance, and urinary incontinence.

Patient Data

Age: 85 years
Gender: Female

There is evidence of ventriculomegaly with Evan index measuring 0.35. Additionally, there is a reduced callosal angle (75°) with bowing of corpus callosum, dilated Sylvian fissures, and tight high convexity sulci (DESH), features suggesting normal pressure hydrocephaly.

Periventricular and subcortical hyperintense foci on T2 and FLAIR images without restricted diffusion or mass-effect suggesting small vessel disease.

No intracerebral hemorrhage (ICH), space-occupying lesion, or shift of midline structures is observed.

The brain stem and cerebellum appear normal.

No tonsillar herniation is noted.

Case Discussion

This patient exhibits classical clinical features of normal pressure hydrocephalus (NPH) with pronounced gait ataxia.

Disproportionately enlarged subarachnoid space hydrocephalus (DESH) is a feature of NPH. There is widespread sulcal enlargement particularly affecting the Sylvian fissures and insular cisterns but the gyri are tightly apposed at the high convexity and this distinguishes DESH from parenchymal atrophy.

NPH may respond to surgical shunting, and some studies suggest that DESH is an indicator of favorable outcome.

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