Pathogenesis of idiopathic Normal Pressure Hydrocephalus: A review of knowledge

Bräutigam, Konstantin; Vakis, Antonis; Tsitsipanis, Christos

doi:10.1016/j.jocn.2018.10.147

Cited by 58 publications

(43 citation statements)

References 30 publications

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“…Indeed, concomitant, biopsy proven hallmarks of AD have been reported in up to 68% of iNPH patients, and might negatively influence shunt-response [9]. Moreover, Parkinson-like, extrapyramidal motor disorders related to dopaminergic system dysfunction are also frequently reported in these patients [10,11]. Lastly, evidence has shown that iNPH is not simply the result of impaired CSF circulation, but that it has a complex pathogenesis involving brain vascular autoregulation deficits and decreased cerebral metabolism [12,13].…”

Section: Introductionmentioning

confidence: 99%

“…Positron emission tomography (PET) is a noninvasive functional imaging technique which is routinely used in clinical practice in the evaluation of patients affected by neurodegenerative diseases, including dementias and parkinsonian syndromes. With a portfolio of several radiotracers which are capable of assessing different cerebral metabolic functions or brain protein depositions, PET has been used in NPH patients to determine several cerebral functions: (1) the amyloid burden with different radiotracers i.e., 2-(4 -[ 11 The aim of this systematic review is to assess the role of PET imaging with different radiotracers in iNPH patients according to published literature.…”

Section: Introductionmentioning

confidence: 99%

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Usefulness of Brain Positron Emission Tomography with Different Tracers in the Evaluation of Patients with Idiopathic Normal Pressure Hydrocephalous

Mattoli

Treglia

Calcagni

et al. 2020

IJMS

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Idiopathic normal pressure hydrocephalus (iNPH) is the only form of dementia that can be cured by surgery. Its diagnosis relies on clinical and radiological criteria. Identifying patients who can benefit from surgery is challenging, as other neurological diseases can be concomitant or mimic iNPH. We performed a systematic review on the role of positron emission tomography (PET) in iNPH. We retrieved 35 papers evaluating four main functional aspects with different PET radiotracers: (1) PET with amyloid tracers, revealing Alzheimer’s disease (AD) pathology in 20–57% of suspected iNPH patients, could be useful in predictions of surgical outcome. (2) PET with radiolabeled water as perfusion tracer showed a global decreased cerebral blood flow (CBF) and regional reduction of CBF in basal ganglia in iNPH; preoperative perfusion parameters could predict surgical outcome. (3) PET with 2-Deoxy-2-[18F]fluoroglucose ([18F]FDG ) showed a global reduction of glucose metabolism without a specific cortical pattern and a hypometabolism in basal ganglia; [18F]FDG PET may identify a coexisting neurodegenerative disease, helping in patient selection for surgery; postsurgery increase in glucose metabolism was associated with clinical improvement. (4) Dopaminergic PET imaging showed a postsynaptic D2 receptor reduction and striatal upregulation of D2 receptor after treatment, associated with clinical improvement. Overall, PET imaging could be a useful tool in iNPH diagnoses and treatment response.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Usefulness of Brain Positron Emission Tomography with Different Tracers in the Evaluation of Patients with Idiopathic Normal Pressure Hydrocephalous

Mattoli

Treglia

Calcagni

et al. 2020

IJMS

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“…It appears to be sporadic and is characterized by ventricular enlargement without significant elevations of intracranial pressure. However, etiology and pathophysiology is likely mixed between brain/vessel compression and disrupted CSF dynamics . Some cases of NPH are secondary to proximal causes such as subarachnoid or intraventricular hemorrhage, meningitis, head trauma, or chronic aqueduct stenosis, where the pressure normalizes by unknown mechanisms .…”

Section: Csf Plays Key Roles In Supporting Brain Healthmentioning

confidence: 99%

Brain Ventricular System and Cerebrospinal Fluid Development and Function: Light at the End of the Tube

2020

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The brain ventricular system is a series of connected cavities, filled with cerebrospinal fluid (CSF), that forms within the vertebrate central nervous system (CNS). The hollow neural tube is a hallmark of the chordate CNS, and a closed neural tube is essential for normal development. Development and function of the ventricular system is examined, emphasizing three interdigitating components that form a functional system: ventricle walls, CSF fluid properties, and activity of CSF constituent factors. The cellular lining of the ventricle both can produce and is responsive to CSF. Fluid properties and conserved CSF components contribute to normal CNS development. Anomalies of the CSF/ventricular system serve as diagnostics and may cause CNS disorders, further highlighting their importance. This review focuses on the evolution and development of the brain ventricular system, associated function, and connected pathologies. It is geared as an introduction for scholars with little background in the field.

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“…The pathophysiological basis of NPH remains unclear. Advanced age, vascular risk factors, and alterations in CSF dynamics have been recognized as potentially contributory . In addition, frequent co‐occurrence of degenerative neuropathology (typically Alzheimer's disease) in patients with NPH has burgeoned the concept of “neurodegenerative NPH.” Glymphatic dysfunction (and thence impaired waste clearance) has been observed in patients with NPH, and may help to explain both the alteration in CSF dynamics and frequent association with neurodegenerative pathology (through shared disease substrates, namely impaired waste clearance) …”

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confidence: 99%

“…Familial segregation in NPH has on occasion been observed, suggesting that genetic factors may be at play . In this study, Morimoto et al performed whole‐exome sequencing (WES) on a Japanese family with multiple individuals affected by NPH, with the aim of identifying a disease‐related gene.…”

mentioning

confidence: 99%

Ciliary Dysfunction: The Hairy Explanation of Normal Pressure Hydrocephalus?

Mulroy

Latorre

Magrinelli

et al. 2019

Movement Disord Clin Pract

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Normal pressure hydrocephalus (NPH) is a poorly understood entity characterized clinically by the (often incomplete) triad of gait impairment, cognitive decline, and urinary dysfunction, alongside cerebral ventricular enlargement without obstruction to cerebrospinal fluid (CSF) flow, in the absence of elevated CSF pressure or previous insults causing secondary hydrocephalus (e.g., meningitis, subarachnoid hemorrhage). 1 Diagnosis relies on identification of typical clinicoradiological features, supported by specialized diagnostic testing (typically CSF drainage procedures). 1 The pathophysiological basis of NPH remains unclear. Advanced age, vascular risk factors, and alterations in CSF dynamics have been recognized as potentially contributory. 2 In addition, frequent co-occurrence of degenerative neuropathology (typically Alzheimer's disease) in patients with NPH has burgeoned the concept of "neurodegenerative NPH." 3 Glymphatic dysfunction (and thence impaired waste clearance) has been observed in patients with NPH, and may help to explain both the alteration in CSF dynamics and frequent association with neurodegenerative pathology (through shared disease substrates, namely impaired waste clearance). 4,5 Familial segregation in NPH has on occasion been observed, suggesting that genetic factors may be at play. 2 In this study, 6 Morimoto et al. performed whole-exome sequencing (WES) on a Japanese family with multiple individuals affected by NPH, with the aim of identifying a disease-related gene. Affected patients presented with the typical triad of cognitive decline, gait imbalance, and urinary incontinence and manifested classic neuroimaging and CSF pressure findings. Chronic respiratory tract infections and infertility were common accompanying symptoms. WES, performed on 3 individuals (1 affected, 2 unaffected), identified 72 variants in the affected individual only. After filtering out simple missense variants, 13 deleterious variants remained as candidates. Sanger sequencing of these in 5 family members (2 affected, 3 unaffected) identified a likely causative nonsense variant (chr10:105893468C>T) in the CFAP43 gene, which cosegregated with the disease in the pedigree. Next, using CRISPR/cas9 technology, they generated a CFAP43 knockout mouse. These mice demonstrated

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Pathogenesis of idiopathic Normal Pressure Hydrocephalus: A review of knowledge

Cited by 58 publications

References 30 publications

Usefulness of Brain Positron Emission Tomography with Different Tracers in the Evaluation of Patients with Idiopathic Normal Pressure Hydrocephalous

Usefulness of Brain Positron Emission Tomography with Different Tracers in the Evaluation of Patients with Idiopathic Normal Pressure Hydrocephalous

Brain Ventricular System and Cerebrospinal Fluid Development and Function: Light at the End of the Tube

Ciliary Dysfunction: The Hairy Explanation of Normal Pressure Hydrocephalus?

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