A multi-center, prospective study on the progression rate of asymptomatic ventriculomegaly with features of idiopathic normal pressure hydrocephalus on magnetic resonance imaging to idiopathic normal pressure hydrocephalus

Kimihira, Luna; Iseki, Chifumi; Takahashi, Yusuke; Sato, Hidenori; Kato, Hiroyuki; Kazui, Hiroaki; Kuriyama, Nagato; Nakajima, Madoka; Miyajima, Masakazu; Endo, Katsuhiro; Kobayashi, Yoshio; Saegusa, Takashi; Takeda, Yoshitaka; Sato, Shunsuke; Tomogane, Yusuke; Baba, Toru; Miyake, Hiroji; Matsumae, Mitsunori; Onozuka, Satoshi; Murai, Hisayuki; Kajimoto, Yoshinaga; Kimura, Teruo; Kobayashi, Masahito; Yamazaki, Masashi; Arai, Hajime; Kato, Takeshi

doi:10.1016/j.jns.2020.117166

Cited by 14 publications

(6 citation statements)

References 17 publications

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“…There are also reports of asymptomatic patients with radiological features of idiopathic NPH. With long-term follow-up, many become symptomatic, consistent with Hakim and Adams' original hypothesis that hydrocephalus develops asymptomatically before causing clinical symptoms 66. Some people with asymptomatic hydrocephalus probably develop Hakim’s triad from underlying neurodegenerative disease that is not shunt-responsive.…”

Section: What Causes Idiopathic Nph?supporting

confidence: 65%

Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide

Carswell

2022

Pract Neurol

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Idiopathic normal pressure hydrocephalus (NPH) was described in 1965 as a syndrome in which hydrocephalus develops but with a normal cerebrospinal fluid (CSF) pressure, causing shunt-responsive gait apraxia, cognitive impairment and urinary incontinence. Not all patients respond to shunting despite having the clinical syndrome with appropriate radiological features. This has led to considerable debate over subsequent decades regarding idiopathic NPH. It is now understood that asymptomatic communicating hydrocephalus can develop in many healthy older people, and that over time this can develop into a symptomatic state that sometimes responds to CSF shunting, but to a variable extent. This review looks at the historical background of NPH, the use of predictive tests, the current state of clinical evidence for the diagnosis and treatment of idiopathic NPH and the possible underlying causes, to provide a contemporary practical guide for assessing patients with the radiological features of idiopathic NPH.

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Section: What Causes Idiopathic Nph?supporting

confidence: 65%

Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide

Carswell

2022

Pract Neurol

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“…Three out of the eight patients with CWH43 alteration had potential iNPH‐related family history, indicating that this variant can either be de novo mutation or that the familiar inheritance is underestimated due to the late onset of the clinical symptoms. Diagnostic criteria of iNPH need to be updated to include also prodromal iNPH, such as asymptomatic ventriculomegaly with features of iNPH on magnetic resonance imaging (AVIM, Kimihira et al , 2020). Identification of genetic variants similar to that now observed with CWH43 will open a window to decipher the underlying pathophysiological mechanisms further, paving the way to better treatments and novel prevention strategies in the future.…”

Section: Figurementioning

confidence: 99%

iNPH—the mystery resolving

2021

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Idiopathic normal pressure hydrocephalus (iNPH) is characterized clinically by degradation of gait, cognition, and urinary continence. INPH is progressive (Andrén et al, 2014), still probably underdiagnosed (Williams et al, 2019) but potentially treatable by CSF diversion (Kazui et al, 2015). Familial aggregation is a strong indicator of genetic regulation in the disease process iNPH (Fig 1). Enlargement of brain ventricles is associated with failed cerebrospinal (CSF) homeostasis by so far mostly unknown mechanisms. A mutation of the cilia gene CFAP43 in iNPH family, confirmed by a knocked‐out mouse model (Morimoto et al, 2019), allelic variation of NME8 (Huovinen et al, 2017), a segmental copy number loss in SFMBT1 in selected iNPH patients (Sato et al, 2016), and current results by Yang et al (2021) indicate that cilia dysfunction is one of the key mechanisms behind iNPH.

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“…However, how these factors and parameters interact and cause the clinical development of iNPH remains unknown. According to previous reports, radiological changes can precede apparent symptoms in patients with iNPH [ 16 – 19 ]. Interestingly, a small group of asymptomatic subjects with iNPH-typical imaging findings in one study showed subclinical declines in verbal fluency and motor regulation, whereas mini-mental state examination (MMSE) and Trail Making Test B (TMT-B) were in the normal range [ 16 ].…”

Section: Introductionmentioning

confidence: 97%

Incidental findings of typical iNPH imaging signs in asymptomatic subjects with subclinical cognitive decline

Engel

Pirpamer

Hofer

et al. 2021

Fluids Barriers CNS

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Background The etiology of idiopathic normal pressure hydrocephalus (iNPH) remains unclear. Little is known about the pre-symptomatic stage. This study aimed to investigate the association of neuropsychological data with iNPH-characteristic imaging changes compared to normal imaging and unspecific atrophy in a healthy population. Methods We extracted data from the community-dwelling Austrian Stroke Prevention Family Study (ASPS-Fam) database (2006–2010). All subjects underwent a baseline and identical follow-up examination after 3–5 years with MR imaging and an extensive neuropsychological test battery (Trail Making Test B, short physical performance balance, walking speed, memory, visuo-practical skills, composite scores of executive function and g-factor). We categorized the subjects into “iNPH”-associated, non-specific “atrophy,” and “normal” based on the rating of different radiological cerebrospinal fluid (CSF) space parameters. We noted how the categories developed over time. We assessed the association of the image categories with the neuropsychological data, different demographic, and lifestyle parameters (age, sex, education, alcohol intake, arterial hypertension, hypercholesterolemia), and the extent of white matter hyperintensities. We investigated whether neuropsychological data associated with the image categories were independent from other parameters as confounders. Results One hundred and thirteen subjects, aged 50–70 years, were examined. The imaging category “iNPH” was only present at follow-up. A third of subjects with “atrophy” at baseline changed to the category “iNPH” at follow-up. More white matter hyperintensities (WMH) were present in later “iNPH” subjects. Subjects with “iNPH” performed worse than “normal” subjects on executive function (p = 0.0118), memory (p = 0.0109), and Trail Making Test B (TMT-B. p < 0.0001). Education, alcohol intake, diabetes, arterial hypertension, and hypercholesterolemia had no effect. Age, number of females, and the extent of white matter hyperintensities were higher in “iNPH” than in “normal” subjects but did not significantly confound the neuropsychological results. Conclusions Apparent asymptomatic subjects with “iNPH” imaging characteristics presented with subclinical cognitive decline and showed worse executive function, memory, and TMT-B results than “normal” subjects. WMH seem to play a role in the etiology before ventriculomegaly. Clinical screening of individuals with incidental iNPH-characteristic imaging and conspicuous results sof these neurocognitive tests needs further validation.

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A multi-center, prospective study on the progression rate of asymptomatic ventriculomegaly with features of idiopathic normal pressure hydrocephalus on magnetic resonance imaging to idiopathic normal pressure hydrocephalus

Cited by 14 publications

References 17 publications

Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide

Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide

iNPH—the mystery resolving

Incidental findings of typical iNPH imaging signs in asymptomatic subjects with subclinical cognitive decline

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