Normal tension glaucoma in CSF-shunted normal pressure hydrocephalus patients. An extended follow-up.
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SUBMITTER: Gallina P
PROVIDER: S-EPMC9829667 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
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Normal tension glaucoma in CSF-shunted normal pressure hydrocephalus patients. An extended follow-up.
Gallina Pasquale P Savastano Alfonso A Buzzi Matilde M Angelini Lucia L Miele Alba A Rizzo Stanislao S Scollato Antonio A Caini Saverio S Porfirio Berardino B
Eye (London, England) 20220425 1
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Project description:CSF shunting with adjustable valve is the treatment of idiopathic normal pressure hydrocephalus. The opening pressure valve setting is left to the neurosurgeon's experience. Aqueductal CSF stroke volume by phase-contrast magnetic resonance measures the CSF passing through the Sylvian aqueduct and it changes with intracranial hydrodynamics. We sought to identify a window of stroke volume differences associated with the best clinical outcome and lowest rate of complications. The records of 69 patients were reviewed. At every clinical check, stroke volume, opening pressure valve, clinical outcome, and CSF overdrainage were analyzed. The correlation between stroke volume differences and negative outcome was also analyzed. The median follow-up was 2.3 years (range 0.3-10.4 years). The odds of negative outcome between two consecutive checks significantly increased by 16% (95%CI 4-28%, p = 0.006). Taking the lowest risk group as reference, the odds ratio of negative outcome was 1.16 (95%CI 0.51-2.63, p = 0.726) for SV differences less than - 37.6 µL, while it was 1.96 (95%CI 0.97-3.98, p = 0.062) for stroke volume changes above + 23.1 µL. Maintaining stroke volume values within a definite range might help maximize clinical benefit and avoid the risk of CSF overdrainage.
| S-EPMC8007697 | biostudies-literature
Project description:BackgroundThe aim of this study was to examine in patients with idiopathic and neurodegenerative normal pressure hydrocephalus (NPH) if motor and cognitive performance as well as changes in biomarkers in cerebrospinal fluid (CSF) evolve differently.Methods41 patients with a typical clinical and MR-/CT-morphological presentation of NPH divided into an Alzheimer-negative (AD-, n = 25) and an Alzheimer-positive (AD+, n = 16) group according to neurodegenerative biomarkers (S100 protein, neuron-specific enolase, β-amyloid 1-42, Tau protein, phospho-Tau, protein-level and CSF pressure) in CSF. Follow-up of cognitive and gait functions before and after a spinal tap of 40-50 ml CSF of up to 49 months. Clinical, motor, neuropsychological and CSF biomarkers were analyzed using a repeated multifactorial analysis of variance (ANOVA) with post-hoc testing.ResultsGait and neuropsychological performance and CSF biomarkers evolved differently between the AD- and AD+ patients. In particular, the AD+ patients benefited from the spinal tap regarding short-term memory. In contrast, gait parameters worsened over time in the AD+ patients, although they showed a relevant improvement after the first tap.ConclusionsThe results substantiate the recently reported association between a tap-responsive NPH and CSF changes of Alzheimer disease. Furthermore, they suggest that the AD changes in CSF manifest in an age-related fashion in AD- patients presenting with NPH.
| S-EPMC10483764 | biostudies-literature
Project description:PurposeTo characterize features of glaucoma associated with a TANK binding kinase 1 (TBK1) gene duplication, which is among the most common molecularly defined causes of normal tension glaucoma (NTG).DesignRetrospective observational case series.MethodsWe conducted a retrospective case series, by reviewing medical records of 7 members of a pedigree with NTG caused by TBK1 gene duplications. Clinical features of these patients at diagnosis, throughout management, and at latest follow-up were identified, including age, intraocular pressure (IOP), central corneal thickness (CCT), optic nerve head appearance, and mean deviation (MD) assessed with Humphrey visual field (HVF) testing protocols.ResultsAt initial diagnosis, the mean age was 35 ± 7 years, IOP was 16 ± 2.1 mm Hg, cup-to-disc (C/D) ratio was 0.9 ± 0.08, and MD assessed via HVF 30-2 and/or 24-2 testing protocols was -9.0 ± 8.9 (range: -1.8 to -27) dB in the 14 study eyes. At initial diagnosis, 4 of 14 eyes (28%) had no visual field defect, 4 (28%) had early visual field defects, and 6 (43%) had severe visual field defects. Patients had a mean follow-up of 21.5 ± 9.0 years and experienced an average reduction of IOP by 28%. Four of 12 eyes (33%) had stable visual fields throughout follow-up, while 8 eyes (67%) had slow-to-moderate progression. The 30-2 and/or 24-2 HVF tests had an average change in MD of -0.53 ± 0.26 dB/year. No eyes had rapid progression with an MD >1.0 dB/year. At final follow-up, the mean IOP was 11.5 ± 2.9, and C/D ratio was 0.94 ± 0.4. At final follow-up, 3 of 14 eyes (21%) had early visual field defects, 4 (29%) had moderate visual field defects, and 7 (50%) had severe visual field defects. Six of 14 eyes (43%) met criteria for legal blindness.ConclusionsWe provide the first report of the clinical features and long-term clinical course in a family of NTG patients with TBK1 gene duplications. TBK1-associated glaucoma exhibits classic features of NTG. Patients present with severe disease at a relatively early age and most (67%) have slow-to-moderate progression of their visual field defects. The rate of visual field change appears correlated with the magnitude of IOP, suggesting that it may be advantageous to set extremely low IOP targets for some patients with TBK1-associated glaucoma.
| S-EPMC7282998 | biostudies-literature
Project description:ImportanceGlaucoma has been dichotomically classified as open or closed angle, and accordingly, distinct therapies have been administered. In this study, the issue of narrow-angle normal-tension glaucoma (NTG), which may be an intermediate-stage or hybrid-stage disease entity, was addressed.ObjectiveTo determine whether anterior chamber (AC) angle width plays any role in NTG progression.Design, setting, and participantsRetrospective analysis of prospectively collected data at Seoul National University Hospital between January 2004 and December 2009. Fifty-two eyes of narrow-angle NTG and 52 wide-angle NTG eyes matched for age, untreated intraocular pressure, and mean deviation of visual field. Nonindentation gonioscopy was used to grade AC angles: narrow angle was defined as a partially invisible (invisible in ≥90° and <180°) pigmented posterior trabecular meshwork, and wide angle was defined as a fully visible pigmented posterior trabecular meshwork. Data were analyzed in September 2017.Main outcomes and measuresOptic disc/retinal nerve fiber layer defect and visual field progression.ResultsOf the narrow-angle NTG cohort, the mean (SD) age was 49.5 (9.1) years and 15 individuals (28.8%) were women; of the wide-angle NTG cohort, the mean (SD) age was 48.7 (9.5) years and 19 (36.5%) were women. All participants were Korean. Over the course of the mean (SD) 7.6 (0.4)-year follow-up period, 25 of 52 narrow-angle eyes (48.1%) and 13 of 52 wide-angle eyes (25.0%) showed structural progression (odds ratio [OR], 2.78; 95% CI, 1.21-6.37; P = .02). Meanwhile, 21 of 52 narrow-angle eyes (40.3%) and 9 of 52 wide-angle eyes (17.3%) showed functional progression (OR, 3.24; 95% CI, 1.31-8.00; P = .009). The cumulative probability of both structural and functional progression was significantly greater in the narrow-angle than in the wide-angle group (mean [SD] 5-year survival rates, 0.56 [0.07] vs 0.83 [0.05]; P = .006 and 0.60 [0.07] vs 0.87 [0.05]; P = .007, respectively). The baseline diurnal intraocular pressure's SD was approximately 1.38-times greater in the narrow-angle than in the wide-angle group (1.8 [0.6] vs 1.3 [0.3] mm Hg; mean difference, 0.52; 95% CI, 0.32-0.72; P < .001). For the follow-up intraocular pressure fluctuation, the narrow-angle group showed an approximately 1.75-times greater SD (2.1 [0.5] vs 1.2 [0.3] mm Hg; mean difference, 0.93; 95% CI, 0.77-1.09; P < .001).Conclusions and relevanceNarrow-angle NTG showed a greater probability of disease progression than did wide-angle NTG. Further studies determining whether augmented or differentiated treatment strategies would be beneficial for patients with narrow-angle NTG are warranted.
| S-EPMC6439780 | biostudies-literature
Project description:Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible neurological disease, causing motor and cognitive dysfunction and dementia. iNPH and Alzheimer's disease (AD) share similar molecular characteristics, including amyloid deposition, t-tau and p-tau dysregulation; however, the disease is under-diagnosed and under-treated. The aim was to identify a panel of sphingolipids and proteins in CSF to diagnose iNPH at onset compared to aged subjects with cognitive integrity (C) and AD patients by adopting multiple reaction monitoring mass spectrometry (MRM-MS) for sphingolipid quantitative assessment and advanced high-resolution liquid chromatography-tandem mass spectrometry (LC-MS/MS) for proteomic analysis. The results indicated that iNPH are characterized by an increase in very long chains Cer C22:0, Cer C24:0 and Cer C24:1 and of acute-phase proteins, immunoglobulins and complement component fragments. Proteins involved in synaptic signaling, axogenesis, including BACE1, APP, SEZ6L and SEZ6L2; secretory proteins (CHGA, SCG3 and VGF); glycosylation proteins (POMGNT1 and DAG1); and proteins involved in lipid metabolism (APOH and LCAT) were statistically lower in iNPH. In conclusion, at the disease onset, several factors contribute to maintaining cell homeostasis, and the protective role of very long chains sphingolipids counteract overexpression of amyloidogenic and neurotoxic proteins. Monitoring specific very long chain Cers will improve the early diagnosis and can promote patient follow-up.
| S-EPMC8347603 | biostudies-literature
Project description:Background and purposeCSF volumes in the basal cistern and Sylvian fissure are increased in both idiopathic normal pressure hydrocephalus and Alzheimer disease, though the differences in these volumes in idiopathic normal pressure hydrocephalus and Alzheimer disease have not been well-described. Using CSF segmentation and volume quantification, we compared the distribution of CSF in idiopathic normal pressure hydrocephalus and Alzheimer disease.Materials and methodsCSF volumes were extracted from T2-weighted 3D spin-echo sequences on 3T MR imaging and quantified semi-automatically. We compared the volumes and ratios of the ventricles and subarachnoid spaces after classification in 30 patients diagnosed with idiopathic normal pressure hydrocephalus, 10 with concurrent idiopathic normal pressure hydrocephalus and Alzheimer disease, 18 with Alzheimer disease, and 26 control subjects 60 years of age or older.ResultsBrain to ventricle ratios at the anterior and posterior commissure levels and 3D volumetric convexity cistern to ventricle ratios were useful indices for the differential diagnosis of idiopathic normal pressure hydrocephalus or idiopathic normal pressure hydrocephalus with Alzheimer disease from Alzheimer disease, similar to the z-Evans index and callosal angle. The most distinctive characteristics of the CSF distribution in idiopathic normal pressure hydrocephalus were small convexity subarachnoid spaces and the large volume of the basal cistern and Sylvian fissure. The distribution of the subarachnoid spaces in the idiopathic normal pressure hydrocephalus with Alzheimer disease group was the most deformed among these 3 groups, though the mean ventricular volume of the idiopathic normal pressure hydrocephalus with Alzheimer disease group was intermediate between that of the idiopathic normal pressure hydrocephalus and Alzheimer disease groups.ConclusionsThe z-axial expansion of the lateral ventricle and compression of the brain just above the ventricle were the common findings in the parameters for differentiating idiopathic normal pressure hydrocephalus from Alzheimer disease.
| S-EPMC7960331 | biostudies-literature
Project description:ObjectiveTo assess whether gait, neuropsychological, and multimodal MRI parameters predict short-term symptom reversal after cerebrospinal fluid (CSF) tap test in idiopathic normal pressure hydrocephalus (iNPH).MethodsThirty patients (79.3 ± 5.9 years, 12 women) with a diagnosis of probable iNPH and 46 healthy controls (74.7 ± 5.4 years, 35 women) underwent comprehensive neuropsychological, quantitative gait, and multimodal MRI assessments of brain morphology, periventricular white-matter microstructure, cortical and subcortical blood perfusion, default mode network function, and white-matter lesion load. Responders were defined as an improvement of at least 10% in walking speed or timed up and go test 24 h after tap test. Univariate and multivariable tap test outcome prediction models were evaluated with logistic regression and linear support vector machine classification.ResultsSixteen patients (53%) respondedpositively to tap test. None of the gait, neuropsychological, or neuroimaging parameters considered separately predicted outcome. A multivariable classifier achieved modest out-of-sample outcome prediction accuracy of 70% (p = .028); gait parameters, white-matter lesion load and periventricular microstructure were the main contributors.ConclusionsOur negative findings show that short-term symptom reversal after tap test cannot be predicted from single gait, neuropsychological, or MRI parameters, thus supporting the use of tap test as prognostic procedure. However, multivariable approaches integrating non-invasive multimodal data are informative of outcome and may be included in patient-screening procedures. Their value in predicting shunting outcome should be further explored, particularly in relation to gait and white-matter parameters.
| S-EPMC9363476 | biostudies-literature
Project description:Pseudo-continuous arterial spin labeling (pCASL) measurements were performed in 20 patients with idiopathic normal pressure hydrocephalus (iNPH) to investigate whether cerebral blood flow (CBF) increases during the first 24 hours after a cerebrospinal fluid tap test (CSF TT). Five pCASL magnetic resonance imaging (MRI) scans were performed. Two scans were performed before removal of 40 mL CSF, and the other three at 30 minutes, 4 hours, and 24 hours, respectively after the CSF TT. Thirteen different regions of interest (ROIs) were manually drawn on coregistered MR images. In patients with increased CBF in lateral and frontal white matter after the CSF TT, gait function improved more than it did in patients with decreased CBF in these regions. However, in the whole sample, there was no significant increase in CBF after CSF removal compared with baseline investigations. The repeatability of CBF measurements at baseline was high, with intraclass correlation coefficients of 0.60 to 0.90 for different ROIs, but the median regional variability was in the range of 5% to 17%. Our results indicate that CBF in white matter close to the lateral ventricles plays a role in the reversibility of symptoms after CSF removal in patients with iNPH.
| S-EPMC4269752 | biostudies-literature