Callosal Angle Research Articles

The Quest for Predicting Sustained Shunt Response in Normal-Pressure Hydrocephalus: An Analysis of the Callosal Angle's Utility

Diagnosing normal-pressure hydrocephalus (NPH) and selecting patients who will experience a sustained benefit from fluid diversion surgery remains challenging. This study seeks to evaluate the association between the callosal angle (CA) and the long-term postoperative response to ventriculoperitoneal shunt surgery in a different subgroup population than previously studied to assess its generalizability. We studied 72 patients with idiopathic NPH who underwent ventriculoperitoneal shunt surgery and had at least 18 months of follow-up between 2000 and 2016. We recorded their pre- and postoperative symptoms according to the NPH Eide scale and their comorbidities with the Kiefer index. Their CA, as well as Evans' Index, ventricular height, and transependymal signal were measured. Multivariable statistical models were used to determine which factors were associated with postoperative improvement while we controlled for the presence of the NPH triad. Fifty-nine patients (82%) demonstrated a successful response to surgery at their first postoperative follow-up. However, this declined to 54 patients (75%) at 1 year and 45 (62.5%) patients at their last follow-up. When we controlled for the presence of the triad of symptoms, the CA significantly predicted a good, sustained response to surgery; for every degree decrease in the CA, a patient is 4% more likely to experience benefit from surgery. The CA is a useful preoperative prognostic tool for predicting which patients will experience a sustained benefit from surgery. Further studies are required to clarify this disease in the context of old age, comorbidity, and possible concomitant neurodegenerative diseases.

The idiopathic normal-pressure hydrocephalus Radscale: a radiological scale for structured evaluation.

Despite the important role of imaging in diagnosing idiopathic normal-pressure hydrocephalus (iNPH), a structured overall assessment of radiological signs is still lacking. The purpose of this study was to construct a radiological scale, composed of morphological signs of iNPH, and compare it with clinical symptoms. In this prospective, population-based study of iNPH, 168 individuals (93 females) [mean age 75 (range 66-92)years] underwent computed tomography of the brain and a neurological examination with assessment of clinical symptoms according to Hellström's iNPH scale. Two radiologists, blinded to clinical data, independently evaluated and measured eight radiological parameters, i.e. Evans' index, callosal angle, size of temporal horns, narrow high-convexity sulci, dilated Sylvian fissures, focally dilated sulci, peri-ventricular hypodensities and bulging of the lateral ventricular roof. In a linear regression model, all parameters except ventricular roof bulging were significantly associated with clinical iNPH symptoms. The seven remaining parameters were summarized into a total iNPH Radscale score ranging from 0 to 12. There was a significant correlation (r=0.55, P<0.001) between the total iNPH Radscale score and clinical symptoms. The inter-rater agreement for the included radiological parameters was high (intraclass correlation, 0.74-0.97). The iNPH Radscale may become a valuable diagnostic screening tool, allowing a structured radiological assessment. A high iNPH Radscale score together with clinical symptoms should raise suspicion of iNPH, motivating further evaluation for shunt surgery.

Ventriculomegaly in the Elderly: Who Needs a Shunt? A MRI Study on 90 Patients.

In the case of ventriculomegaly in the elderly, it is often difficult to differentiate between communicating chronic hydrocephalus (CCH) and brain atrophy. The aim of this study is to describe the MRI criteria of CCH, defined by a symptomatic patient with ventriculomegaly and that improved after shunt placement. Magnetic resonance imaging was prospectively evaluated in 90 patients with ventriculomegaly. Patients were classified into three groups: patients without clinical signs of CCH (control, n = 47), patients with CCH treated by shunt placement with clinical improvement (responders, n = 36), and patients with CCH treated using a shunt without clinical improvement (nonresponders, n = 7). MRI parameters of the two groups of interest (responders vs. controls) were compared. Compared with controls, Evans' index (p = 0.029), ventricular area (p < 0.01), and volume (p = 0.0001) were higher in the responders. In this group, the callosal angle was smaller (p ≤ 0.0001) and the aqueductal stroke volume (SVa) of CSF was higher (p ≤ 0.0001) than in controls. On the ROC curves, the optimal cut-off values for differentiating between responders and controls were a ventricular area >33.5 cm2, a callosal angle <90.8° and a SVa > 136.5 μL/R-R. In multivariate analysis, responders remained associated with SVa and callosal angle, with a c-statistic of 0.90 (95%CI, 0.83-0.98). On suspicion of CCH, a large ventricular area, a small callosal angle, and an increased aqueductal stroke volume are important MRI arguments that can be associated with the clinical evaluation and dynamic testing of CSF to confirm the indication for a shunt.

Increase in callosal angle and decrease in ventricular volume after shunt surgery in patients with idiopathic normal pressure hydrocephalus.

OBJECTIVE Postoperative decrease in ventricle size is usually not detectable either by visual assessment or by measuring the Evans index in patients with idiopathic normal pressure hydrocephalus (iNPH). The aim of the present study was to investigate whether the angle between the lateral ventricles (the callosal angle [CA]) increases and ventricular volume decreases after shunt surgery in patients with iNPH. METHODS Magnetic resonance imaging of the brain was performed before and 3 months after shunt surgery in 18 patients with iNPH. The CA and Evans index were measured on T1-weighted 3D MR images, and ventricular volume contralateral to the shunt valve was measured with quantitative MRI. RESULTS The CA was larger postoperatively (mean 78°, 95% CI 69°-87°) than preoperatively (mean 67°, 95% CI 60°-73°; p < 0.001). The volume of the lateral ventricle contralateral to the shunt valve decreased from 73 ml (95% CI 66-80 ml) preoperatively to 63 ml (95% CI 54-72 ml) postoperatively (p < 0.001). The Evans index was 0.365 (95% CI 0.35-0.38) preoperatively and 0.358 (95% CI 0.34-0.38) postoperatively (p < 0.05). Postoperative change of CA showed a negative correlation with change of ventricular volume (r = -0.76, p < 0.01). CONCLUSIONS In this sample of patients with iNPH, the CA increased and ventricular volume decreased after shunt surgery. The relative difference was most pronounced for the CA, indicating that this accessible, noninvasive radiological marker should be evaluated further as an indirect method to determine shunt function in patients with iNPH.

Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses

BackgroundVery little is known about the incidence and prevalence of hydrocephalus in patients with mucopolysaccharidoses (MPS). The biggest challenge is to distinguish communicating hydrocephalus from ventricular dilatation secondary to brain atrophy, because both conditions share common clinical and neuroradiological features. The main purpose of this study is to assess the relationship between ventriculomegaly, brain and cerebrospinal fluid (CSF) volumes, aqueductal and cervical CSF flows, and CSF opening pressure in MPS patients, and to provide potential biomarkers for abnormal CSF circulation.MethodsForty-three MPS patients (12 MPS I, 15 MPS II, 5 MPS III, 9 MPS IV A and 2 MPS VI) performed clinical and developmental tests, and T1, T2, FLAIR and phase-contrast magnetic resonance imaging (MRI) followed by a lumbar puncture with the CSF opening pressure assessment. For the analysis of MRI variables, we measured the brain and CSF volumes, white matter (WM) lesion load, Evans’ index, third ventricle width, callosal angle, dilated perivascular spaces (PVS), craniocervical junction stenosis, aqueductal and cervical CSF stroke volumes, and CSF glycosaminoglycans concentration.ResultsAll the scores used to assess the supratentorial ventricles enlargement and the ventricular CSF volume presented a moderate correlation with the aqueductal CSF stroke volume (ACSV). The CSF opening pressure did not correlate either with the three measures of ventriculomegaly, or the ventricular CSF volume, or with the ACSV. Dilated PVS showed a significant association with the ventriculomegaly, ventricular CSF volume and elevated ACSV.ConclusionsIn MPS patients ventriculomegaly is associated with a severe phenotype, increased cognitive decline, WM lesion severity and enlarged PVS. The authors have shown that there are associations between CSF flow measurements and measurements related to CSF volumetrics. There was also an association of volumetric measurements with the degree of dilated PVS.

Diagnosis of Normal-Pressure Hydrocephalus: Use of Traditional Measures in the Era of Volumetric MR Imaging.

Purpose To assess the diagnostic performance of the callosal angle (CA) and Evans index (EI) measures and to determine their role versus automated volumetric methods in clinical radiology. Materials and Methods Magnetic resonance (MR) examinations performed before surgery (within 1-5 months of the MR examination) in 36 shunt-responsive patients with normal-pressure hydrocephalus (NPH; mean age, 75 years; age range, 58-87 years; 26 men, 10 women) and MR examinations of age- and sex-matched patients with Alzheimer disease (n = 34) and healthy control volunteers (n = 36) were studied. Three blinded observers independently measured EI and CA for each patient. Volumetric segmentation of global gray matter, white matter, ventricles, and hippocampi was performed by using software. These measures were tested by using multivariable logistic regression models to determine which combination of metrics is most accurate in diagnosis. Results The model that used CA and EI demonstrated 89.6%-93.4% accuracy and average area under the curve of 0.96 in differentiating patients with NPH from patients without NPH (ie, Alzheimer disease and healthy control). The regression model that used volumetric predictors of gray matter and white matter was 94.3% accurate. Conclusion CA and EI may serve as a screening tool to help the radiologist differentiate patients with NPH from patients without NPH, which would allow for designation of patients for further volumetric assessment. © RSNA, 2017.

Normal pressure hydrocephalus as a possible reversible cause of dementia, neuroimaging findings

IntroductionNormal pressure hydrocephalus (NPH) occurs in 0.5% of persons over 65 years old. The etiology of NPH is still unknown. Clinically NPH is characterised by cognitive deterioration, gait impairment and urinary incontinence. NPH is a possible reversible cause of dementia. Neuroimaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) allow to assess typical brain changes in this disorder.The objectives are to present the typical findings of NPH on CT and MRI and to demonstrate differences between NPH and central brain atrophy in neuroimaging.ResultsThe imaging features of NPH include: supratentorial ventriculomegaly with callosal angle less than 90o, tight sulci at the vertex and considerable out of proportion enlargement of Sylvian fissures. In case of central brain atrophy there may be a predominance of ventriculomegaly and/or widened sulci without crowding of the gyri at the vertex and callosal angle greater than 90o. In both entities, the decrease of density in periventricular region may be seen: in NPH could be a sign of transependymal oedema or in brain atrophy as an accompanying leukoaraiosis. Additionally, it is possible to assess changes in flow of cerebrospinal fluid (CSF) on MRI: in NPH an increased pulsatile CSF circulation in aqueduct as flow void sign may be observed.ConclusionsCorrect diagnosis of NPH on CT or MRI in relation to clinical data is very important. Treatment with ventriculoperitoneal shunt or third ventriculostomy may partially improve the quality of life in some patients with cognitive impairment due to NPH.Disclosure of interestThe authors have not supplied their declaration of competing interest.

Utility of MRI-based disproportionately enlarged subarachnoid space hydrocephalus scoring for predicting prognosis after surgery for idiopathic normal pressure hydrocephalus: clinical research.

OBJECTIVE The presence of disproportionately enlarged subarachnoid space hydrocephalus (DESH) on brain imaging is a recognized finding of idiopathic normal pressure hydrocephalus (iNPH), but the features of DESH can vary across patients. The aim of this study was to evaluate the utility of MRI-based DESH scoring for predicting prognosis after surgery. METHODS In this single-center, retrospective cohort study, the DESH score was determined by consensus between a group of neurosurgeons, neurologists, and a neuroradiologist based on the preoperative MRI findings of the patients with suspected iNPH. The DESH score was composed of the following 5 items, each scored from 0 to 2 (maximum score 10 points): ventriculomegaly, dilated sylvian fissures, tight high convexity, acute callosal angle, and focal sulcal dilation. The association between the DESH score and improvement of the scores on the modified Rankin Scale (mRS), iNPH Grading Scale (iNPHGS), Mini-Mental State Examination (MMSE), Trail Making Test-A (TMT-A), and Timed 3-Meter Up and Go Test (TUG-t) was examined. The primary end point was improvement in the mRS score at 1 year after surgery, and the secondary outcome measures were the iNPHGS, MMSE, TMT-A, and TUG-t scores at 1 year after surgery. Improvement was determined as improvement of 1 or more levels on mRS, ≥ 1 point on iNPHGS, ≥ 3 points on MMSE, a decrease of > 30% on TMT-A, and a decrease of > 10% on TUG-t. RESULTS The mean DESH score for the 50 patients (mean age 77.6 ± 5.9 years) reviewed in this study was 5.58 ± 2.01. The mean rate of change in the mRS score was -0.50 ± 0.93, indicating an inverse correlation between the DESH score and rate of change in the mRS score (r = -0.749). Patients who showed no improvement in mRS score tended to have a low DESH score as well as low preoperative MMSE and TMT-A scores. There were no differences in the areas of deep white matter hyperintensity and periventricular hyperintensity on the images between patients with and without an improved mRS score (15.6% vs 16.7%, respectively; p = 1.000). The DESH score did differ significantly between patients with and without improved scores on the iNPHGS (6.39 ± 1.76 vs 4.26 ± 1.69, respectively; p < 0.001), MMSE (6.63 ± 1.82 vs 5.09 ± 1.93; p = 0.010), TMT-A (6.32 ± 1.97 seconds vs 5.13 ± 1.93 seconds; p = 0.042), and TUG-t (6.48 ± 1.81 seconds vs 4.33 ± 1.59 seconds; p < 0.001). CONCLUSIONS MRI-based DESH scoring is useful for the prediction of neurological improvement and prognosis after surgery for iNPH.

Feasibility of Simple Linear Measurements to Determine Ventricular Enlargement in Patients With Idiopathic Normal Pressure Hydrocephalus.

To evaluate the feasibility and reproducibility of linear measurements for determining ventricular enlargement in patients with idiopathic normal pressure hydrocephalus (iNPH) and their correlation to ventricular volume (VV). Preoperative brain computed tomography scans were retrospectively evaluated in 36 patients with iNPH. The quantitative markers of Evan index (EI), VV, frontal and occipital horn ratio (FOR), modified cella media index (mCMI), third ventricular width (TVW), temporal horn width (TPH), frontal horn width (FHW), and callosal angle (CA) at the posterior commissure (PC) were independently measured by a neurosurgeon and a radiologist. Intraclass correlation coefficients were calculated to establish inter-rater agreement among the 2 investigators. Pearson correlation coefficients were used to assess the relationship of each linear measurement with total VV. The overall inter-rater agreement among investigators was almost perfect for EI, VV, FOR, mCMI, TVW, substantial for FHW and moderate for TPH, and CA at PC. Pearson correlation coefficients showed excellent correlation between mCMI and VV. Moderate correlation was found between the VV and FHW, TVW, FOR, EI, and CA at PC. Fair correlation was found between the VV and TPH. Simple linear measurements could serve as effective alternative to volumetric analysis to determine ventricular size in patients with iNPH. The quantitative marker of mCMI is more reasonable and accurate than EI, FOR, and other simple linear measurements.

High-Convexity Tightness Predicts the Shunt Response in Idiopathic Normal Pressure Hydrocephalus.

Although neuroimaging plays an important role in the diagnosis of idiopathic normal pressure hydrocephalus, its predictive value for response to shunt surgery has not been established. The purpose of the current study was to identify neuroimaging markers that predict the shunt response of idiopathic normal pressure hydrocephalus. Sixty patients with idiopathic normal pressure hydrocephalus underwent presurgical brain MR imaging and clinical evaluation before and 1 year after shunt surgery. The assessed MR imaging features included the Evans index, high-convexity tightness, Sylvian fissure dilation, callosal angle, focal enlargement of the cortical sulci, bumps in the lateral ventricular roof, and deep white matter and periventricular hyperintensities. The idiopathic normal pressure hydrocephalus grading scale total score was used as a primary clinical outcome measure. We used measures for individual symptoms (ie, the idiopathic normal pressure hydrocephalus grading scale subdomain scores, such as gait, cognitive, and urinary scores), the Timed Up and Go test, and the Mini-Mental State Examination as secondary clinical outcome measures. The relationships between presurgical neuroimaging features and postoperative clinical changes were investigated by using simple linear regression analysis. To identify the set of presurgical MR imaging features that best predict surgical outcomes, we performed multiple linear regression analysis by using a bidirectional stepwise method. Simple linear regression analyses demonstrated that presurgical high-convexity tightness, callosal angle, and Sylvian fissure dilation were significantly associated with the 1-year changes in the clinical symptoms. A multiple linear regression analysis demonstrated that presurgical high-convexity tightness alone predicted the improvement of the clinical symptoms 1 year after surgery. High-convexity tightness is a neuroimaging feature predictive of shunt response in idiopathic normal pressure hydrocephalus.

Neuroimaging in normal pressure hydrocephalus.

Normal pressure hydrocephalus (NPH) is a syndrome characterized by the triad of gait disturbance, mental deterioration and urinary incontinence, associated with ventriculomegaly and normal cerebrospinal fluid (CSF) pressure. The clinical presentation (triad) may be atypical or incomplete, or mimicked by other diseases, hence the need for supplementary tests, particularly to predict postsurgical outcome, such as CSF tap-tests and computed tomography (CT) or magnetic resonance imaging (MRI). The CSF tap-test, especially the 3 to 5 days continuous external lumbar drainage of at least 150 ml/day, is the only procedure that simulates the effect of definitive shunt surgery, with high sensitivity (50-100%) and high positive predictive value (80-100%). According to international guidelines, the following are CT or MRI signs decisive for NPH diagnosis and selection of shunt-responsive patients: ventricular enlargement disproportionate to cerebral atrophy (Evans index >0.3), and associated ballooning of frontal horns; periventricular hyperintensities; corpus callosum thinning and elevation, with callosal angle between 40º and 90º; widening of temporal horns not fully explained by hippocampal atrophy; and aqueductal or fourth ventricular flow void; enlarged Sylvian fissures and basal cistern, and narrowing of sulci and subarachnoid spaces over the high convexity and midline surface of the brain. On the other hand, other imaging methods such as radionuclide cisternography, SPECT, PET, and also DTI or resting-state functional MRI, although suitable for NPH diagnosis, do not yet provide improved accuracy for identifying shunt-responsive cases.

Feasibility of radiological markers in idiopathic normal pressure hydrocephalus.

Various radiological markers have been proposed for diagnostics in idiopathic normal pressure hydrocephalus (iNPH). We examined the usefulness of radiological markers in the diagnostics and prediction of shunt response in iNPH. In this retrospective cohort study, we evaluated brain CT or MRI scans of 390 patients with suspected iNPH. Based on a 24-h intraventricular pressure monitoring session, patients were classified into a non-NPH (n = 161) or probable iNPH (n = 229) group. Volumes of cerebrospinal fluid compartments (lateral ventricles, sylvian and suprasylvian subarachnoid spaces and basal cisterns) were visually assessed. Disproportionally enlarged subarachnoid spaces, flow void, white matter changes, medial temporal lobe atrophy and focally dilated sulci were evaluated. Moreover, we measured quantitative markers: Evans' index (EI), the modified cella media index, mean width of the temporal horns and callosal angle. iNPH was more likely in patients with severe volumetric disproportion between the suprasylvian and sylvian subarachnoid spaces than in those without disproportion (OR 7.5, CI 95 % 4.0-14.1, P < 0.0001). Mild disproportion (OR 2.6, CI 95 % 1.4-4.6, P = 0.001) and narrow temporal horns (OR per 1 mm 0.91, CI 95 % 0.84-0.98, P = 0.014) were also associated with an iNPH diagnosis. Other radiological markers had little association with the iNPH diagnosis in the final combined multivariate model. Interestingly, EI was higher in non-NPH than iNPH patients (0.40 vs. 0.38, P = 0.039). Preoperative radiological markers were not associated with shunt response. Visually evaluated disproportion was the most useful radiological marker in iNPH diagnostics. Narrower temporal horns also supported an iNPH diagnosis, possibly since atrophy was more pronounced in the non-NPH than iNPH group.

A Simplified Callosal Angle Measure Best Differentiates Idiopathic-Normal Pressure Hydrocephalus from Neurodegenerative Dementia.

Idiopathic normal-pressure hydrocephalus (iNPH) can resemble or occur in combination with other brain disorders frequently present in the elderly such as Alzheimer's disease (AD) and dementia with Lewy bodies (DLB). To study the accuracy of a simplified callosal angle measure in differentiating iNPH from DLB and AD using conventional brain MRI. 76 patients (24 iNPH, 30 DLB, 22 AD) and 40 healthy controls served as discovering cohort. The callosal angle measure was obtained on standard coronal brain MRI images crossing the corpus callosum midpoint. 41 patients (21 iNPH and 20 DLB/AD) were used as independent validation cohort. A set of other conventional MRI markers of iNPH was also evaluated. iNPH showed a significantly decreased mean callosal angle value compared to both disease groups and controls (iNPH = 109±9; DLB = 136.9±8.2; AD = 135.4±11.3; Controls = 138.5±5.2; p < 0.00001). Using a cut off angle of 123, derived by the mean -3SD of the control group, an accuracy of 96% (sensitivity 100% , specificity 95.4% ) was obtained. By ROC analysis, the area under the curve was 0.99 (95% CI: 0.97-1). The measure was consistent (intra-rater: r = 0.94) and reproducible (inter-rater: r = 0.89). In the validation cohort, this cut off angle value discriminated iNPH from DLB/AD with 97.5% accuracy. None of the conventional MRI signs reached the same accuracy. This simplified callosal angle measure represents an accurate, reproducible, and easy marker of iNPH.

Simple and reproducible linear measurements to determine ventricular enlargement in adults.

Background:Recent studies have suggested that Evan's Index (EI) is not accurate and instead endorse volumetric measurements. Our aim was to evaluate the reproducibility of linear measurements and their correlation to ventricular volume.Methods:Using magnetic resonance (MR) images of 30 patients referred for normal pressure hydrocephalus (NPH), EI, frontal-occipital horn ratio (FOR), third ventricular width and height, frontal horn width (FHW), and callosal angle (CA) at the foramen of Monro and the posterior commissure (PC) were independently measured by residents in neurosurgery and radiology, a neurosurgeon and radiologist, and a medical student. Intraclass correlation coefficients (ICC) were calculated to establish inter-rater agreement among the reviewers. Pearson's correlation coefficients were done to assess the relationship of the linear measurements with total ventricular volume. Kappa analyses were performed to assess the degree of agreement between cutpoints determined by the ROC analysis for the linear measurements and reviewers’ gestalt impression about ventricular size with volumetric abnormality.Results:The overall inter-rater agreement among reviewers was almost perfect for EI (ICC = 0.913), FOR (ICC = 0.830), third ventricular width, FHW (ICC = 0.88), and CA at PC (ICC = 0.865), substantial for temporal horn width (ICC = 0.729) and CA at foramen of Monro (ICC = 0.779), and moderate for third ventricular height (ICC = 0.496). EI, FOR, third ventricular width, temporal horn width, and CA at PC measures correlated with total ventricular volume. There was fair-to-almost-perfect agreement of the individual reviewer's gestalt responses of abnormatility with volumetric abnormality. Gestalt responses were better for more senior raters.Conclusion:Linear measurements are reliable and reproducible methods for determining ventricular enlargement.

Association of lipocalin-type prostaglandin D synthase with disproportionately enlarged subarachnoid-space in idiopathic normal pressure hydrocephalus.

BackgroundIdiopathic normal pressure hydrocephalus (iNPH) is a treatable cause of dementia, gait disturbance, and urinary incontinence in elderly patients with ventriculomegaly. Its unique morphological feature, called disproportionately enlarged subarachnoid-space hydrocephalus (DESH), may also be a diagnostic feature. Lipocalin-type prostaglandin D synthase (L-PGDS) is a major cerebrospinal fluid (CSF) protein produced by arachnoid cells, and its concentration in the CSF is reportedly decreased in iNPH. L-PGDS acts as a prostaglandin D2-producing enzyme and behaves as a chaperone to prevent the neurotoxic aggregation of amyloid beta (Aβ) implicated in Alzheimer’s disease, a major comorbidity of iNPH. The aim of this study was to confirm the L-PGDS decrease in DESH-type iNPH and to clarify its relationship with clinico-radiological features or other CSF biomarkers.MethodsWe evaluated 22 patients (age: 76.4 ± 4.4 y; males: 10, females: 12) referred for ventriculomegaly without CSF pathway obstruction, and conducted a CSF tap test to determine the surgical indication. CSF concentrations of L-PGDS, Aβ42, Aβ40, and total tau (t-tau) protein were determined using enzyme-linked immunosorbent assays. Clinical symptoms were evaluated by the iNPH grading scale, mini-mental state examination, frontal assessment battery (FAB), and timed up and go test. The extent of DESH was approximated by the callosal angle, and the severity of parenchymal damage was evaluated by the age-related white matter change (ARWMC) score.ResultsL-PGDS and t-tau levels in CSF were significantly decreased in DESH patients compared to non-DESH patients (p = 0.013 and p = 0.003, respectively). L-PGDS and t-tau showed a significant positive correlation (Spearman r = 0.753, p < 0.001). Among the clinico-radiological profiles, L-PGDS levels correlated positively with age (Spearman r = 0.602, p = 0.004), callosal angle (Spearman r = 0.592, p = 0.004), and ARWMC scores (Spearman r = 0.652, p = 0.001), but were negatively correlated with FAB scores (Spearman r = 0.641, p = 0.004).ConclusionsOur data support the diagnostic value of L-PGDS as a CSF biomarker for iNPH and suggest a possible interaction between L-PGDS and tau protein. In addition, L-PGDS might work as a surrogate marker for DESH features, white matter damage, and frontal lobe dysfunction.

髄液中リポカリン型プロスタグランジンD合成酵素 (L-PGDS) 濃度は特発性正常圧水頭症 (iNPH) における狭脳梁角, 白質障害, 前頭葉機能低下と相関する

特発性正常圧水頭症 (iNPH) の形態的基準である脳室拡大に付随して, DESH (disproportionately enlarged subarachnoid-space hydrocephalus) と呼ばれる髄液不均等分布が重要視されている. 一方, くも膜細胞で産生されるリポカリン型プロスタグランジンD合成酵素 (L-PGDS) はiNPHの生物学的マーカーとして有力候補である. 今回, 診断目的で得る髄液中のL-PGDS濃度を評価したところ, DESH症例においてL-PGDSが低値であること, また, L-PGDSが低値であるほど, 脳梁角が狭く, 白質障害および前頭葉機能障害が軽度であることが示された.

The callosal angle measured on MRI as a predictor of outcome in idiopathic normal-pressure hydrocephalus

Different neuroimaging biomarkers have been studied to find a tool for prediction of response to CSF shunting in idiopathic normal-pressure hydrocephalus (iNPH). The callosal angle (CA) has been described as useful in discriminating iNPH from ventricular dilation secondary to atrophy. However, the usefulness of the CA as a prognostic tool for the selection of shunt candidates among patients with iNPH is unclear. The aim of this study was to compare the CA in shunt responders with that in nonresponders and clarify whether the CA can serve as a predictor of the outcome. Preoperative MRI brain scans were evaluated in 109 patients who had undergone shunt surgery for iNPH during 2006-2010. Multiplanar reconstruction was performed interactively to obtain a coronal image through the posterior commissure, perpendicular to the anterior-posterior commissure plane. The CA was measured as the angle between the lateral ventricles on the coronal image. The patients were examined clinically before surgery and at 12 months postoperatively. Shunt responders had a significantly smaller mean preoperative CA compared with nonresponders: 59° (95% CI 56°-63°) versus 68° (95% CI 61°-75°) (p < 0.05). A CA cutoff value of 63° showed the best prognostic accuracy. The preoperative CA is smaller in patients whose condition improves after shunt surgery and may be a useful tool in the selection of shunt candidates among patients with iNPH.

Clinical impact of the callosal angle in the diagnosis of idiopathic normal pressure hydrocephalus

The utility of measuring the corpus callosal angle (CA) for the diagnosis of idiopathic normal pressure hydrocephalus (INPH) was investigated. Three-dimensional magnetic resonance imaging (MRI) was performed in 34 INPH patients, 34 Alzheimer's disease (AD) patients, and 34 normal control (NC) subjects. Measurement of the CA on the coronal MR images of the posterior commissure perpendicular to the anteroposterior commissure plane was performed for all subjects. The CA of the INPH group (mean +/- SD, 66 +/- 14 degrees) was significantly smaller than those of the AD (104 +/- 15 degrees) and NC (112 +/- 11 degrees) groups. When using the threshold of the mean - 2SD value of the NC group (= 90 degrees), an accuracy of 93%, sensitivity of 97%, and specificity of 88% were observed for discrimination of INPH from AD patients. Measuring the CA helps in differentiating INPH patients from AD and normally aged subjects.

Dementia paralytica: deterioration from communicating hydrocephalus.

Five patients suffering from dementia paralytica who failed to improve or deteriorated after one or several high dosage courses of penicillin, had pneumoencephalographic patterns suggesting communicating hydrocephalus. Measurements of the ventricular index, ratio of cella media to width of the temporal horn, and the callosal angle differed from that in seven cases of dementia paralytica with associated cerebral atrophy. An isotope cisternogram in three cases with communicating hydrocephalus further confirmed a blockage of the cerebrospinal fluid (CSF) at the parasagittal subarachnoid space. Three patients exhibited the full syndrome of gait apraxia, incontinence, and pyramidal tract signs associated with a severe degree of dementia. Shunting of the CSF in three cases was followed by immediate improvement in two, one in a longlasting way. No active parenchymal inflammation was observed in any of three brain biopsy samples taken during surgery, except for leptomeningeal fibrosis in one. Chronic leptomeningitis in dementia paralytica may impair subarachnoid CSF absorption with subsequent communicating hydrocephalus. Progression or inadequate responses after therapeutic dose of penicillin in dementia paralytica should prompt investigation for this complication as an alternative, effective treatment could be offered.

Ventricular size following head injury: A clinico-radiological study

A retrospective study of the records of 93 patients who had sustained a severe closed head injury was carried out to determine the relationship, if any, between the severity of the injury, the size of the cerebral ventricles and the residual disability. Ventricular size was expressed as a ventricular index. The callosal angle was also measured to see whether this was of value in the recognition of post-traumatic external obstructive hydrocephalus. A normal range of ventricular indices and callosal angles was obtained from a review of the air encephalograms of 56 patients matched for age and sex with the head injury group, and whose air studies were considered to be normal. The findings on the head injury group were subjected to detailed statistical analysis. The study showed that there was no statistically significant relationship between severity of injury, measured by duration of unconsciousness, and ventricular size expressed as a ventricular index. There was a relationship between ventricular size and residual disability as graded in this study. If age was taken into consideration, the relationship between ventricular size and disability was closer. It was not possible to predict residual disability of individual patients from ventricular size alone due to the wide range of values in any disability grade. Failure of air to pass from the basal cisterns over the cerebral convexities is a widely accepted sign of extraventricular obstruction. The shapes of the ventricles, the ventricular index and a callosal angle of 110 degrees or less provided supporting evidence of obstruction in this study. Recognition of an obstructive element in ventricular dilatation following head injury is important, since in a small carefully selected group of patients a ventricular shunting operation may favourably affect recovery.