{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T16:16:03Z","timestamp":1780503363006,"version":"3.54.1"},"reference-count":15,"publisher":"BMJ","issue":"6","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,4,22]]},"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:p>Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a NOTCH3-related small vessel disease that can mimic idiopathic normal pressure hydrocephalus (iNPH). A woman in her late 70s presented with the classic iNPH triad: progressive gait disturbance with falls, urinary incontinence and cognitive decline. Brain MRI showed ventriculomegaly with periventricular hyperintensities and a narrow callosal angle (60\u00b0), supporting iNPH, but also revealed non-confluent white matter hyperintensities in the anterior temporal poles and external capsules; features strongly suggestive of CADASIL. A large-volume cerebrospinal fluid tap test produced no objective gait improvement. Family history disclosed a relative with similar gait\/psychiatric illness and poor outcomes after shunting. Genetic testing confirmed a heterozygous pathogenic NOTCH3 variant, establishing CADASIL. This case underscores that \u2018NPH-compatible\u2019 imaging and a normal pressure hydrocephalus-like syndrome do not confirm iNPH; clinicians should consider CADASIL when tap test is negative and MRI\/family history suggests small vessel vasculopathy.<\/jats:p>","DOI":"10.1136\/bcr-2025-270748","type":"journal-article","created":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T15:55:22Z","timestamp":1780502122000},"page":"e270748","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Recognising CADASIL in adults with NPH-like syndrome"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4317-6965","authenticated-orcid":false,"given":"Aliakbar","family":"Netterwala","sequence":"first","affiliation":[{"name":"Neurology, St John\u2019s National Academy of Health Sciences, Bengaluru, KA, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3941-8020","authenticated-orcid":false,"given":"Thomas","family":"Mathew","sequence":"additional","affiliation":[{"name":"Dept of Neurology, St John\u2019s National Academy of Health Sciences, Bangalore, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shagun","family":"Bhardwaj","sequence":"additional","affiliation":[{"name":"Neurology, St John\u2019s National Academy of Health Sciences, Bengaluru, KA, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharath Kumar","family":"Goddu Govindappa","sequence":"additional","affiliation":[{"name":"Neuroradiology, St John\u2019s National Academy of Health Sciences, Bengaluru, KA, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,6,3]]},"reference":[{"key":"2026060308551692000_19.6.e270748.1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70127-9"},{"key":"2026060308551692000_19.6.e270748.2","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1212\/WNL.56.5.628","article-title":"MRI hyperintensities of the temporal lobe and external capsule in patients with CADASIL","volume":"56","author":"O\u2019Sullivan","year":"2001","journal-title":"Neurology (ECronicon)"},{"key":"2026060308551692000_19.6.e270748.3","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1212\/CON.0000000000000305","article-title":"Diagnosis and Treatment of Idiopathic Normal Pressure Hydrocephalus","volume":"22","author":"Williams","year":"2016","journal-title":"Continuum (Mount Lawley)"},{"key":"2026060308551692000_19.6.e270748.4","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(10)70104-6"},{"key":"2026060308551692000_19.6.e270748.5","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-008-1044-4"},{"key":"2026060308551692000_19.6.e270748.6","doi-asserted-by":"publisher","DOI":"10.5853\/jos.2015.17.1.7"},{"key":"2026060308551692000_19.6.e270748.7","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2005.102.6.0987"},{"key":"2026060308551692000_19.6.e270748.8","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s44202-024-00135-4","article-title":"Cognitive changes after tap test in patients with normal pressure hydrocephalus: a before-and-after study","volume":"4","author":"Mayorga","year":"2024","journal-title":"Discov Psychol"},{"key":"2026060308551692000_19.6.e270748.9","doi-asserted-by":"crossref","unstructured":"Ishikawa M , Hashimoto M , Kuwana N , et al . 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