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An Update on Hydrocephalus Research
Abstract
Hydrocephalus is a common neurological disease caused by imbalance in the production and absorption of cerebrospinal fluid (CSF) or obstruction of its circulation pathways. The etiology of the disease is complex, often accompanied by a variety of complication. In recent years, hydrocephalus has been intensively studied. In this paper, the etiology, epidemiology, diagnosis and treatment of hydrocephalus in study on traditional Chinese and western medicine treatment were reviewed in order to provide reference for clinical diagnosis and treatment of hydrocephalus in the future.
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Background and purpose:
Regional cerebral blood flow has previously been studied in patients with idiopathic normal pressure hydrocephalus with imaging methods that require an intravenous contrast agent or expose the patient to ionizing radiation. The purpose of this study was to assess regional CBF in patients with idiopathic normal pressure hydrocephalus compared with healthy controls using the noninvasive quantitative arterial spin-labeling MR imaging technique. A secondary aim was to compare the correlation between symptom severity and CBF.
Materials and methods:
Differences in regional cerebral perfusion between patients with idiopathic normal pressure hydrocephalus and healthy controls were investigated with pseudocontinuous arterial spin-labeling perfusion MR imaging. Twenty-one consecutive patients with idiopathic normal pressure hydrocephalus and 21 age- and sex-matched randomly selected healthy controls from the population registry were prospectively included. The controls did not differ from patients with respect to selected vascular risk factors. Twelve different anatomic ROIs were manually drawn on coregistered FLAIR images. The Holm-Bonferroni correction was applied to statistical analyses.
Results:
In patients with idiopathic normal pressure hydrocephalus, perfusion was reduced in the periventricular white matter (P < .001), lentiform nucleus (P < .001), and thalamus (P < .001) compared with controls. Cognitive function in patients correlated with CBF in the periventricular white matter (r = 0.60, P < .01), cerebellum (r = 0.63, P < .01), and pons (r = 0.71, P < .001).
Conclusions:
Using pseudocontinuous arterial spin-labeling, we could confirm findings of a reduced perfusion in the periventricular white matter, basal ganglia, and thalamus in patients with idiopathic normal pressure hydrocephalus previously observed with other imaging techniques.
Objective
The literature describes various cerebrospinal fluid (CSF) drainage techniques to alleviate posthemorrhagic hydrocephalus in preterm newborns; however, consensus has not been reached. The scope of this study was describing a case series of premature neonates with posthemorrhagic hydrocephalus and assessing the outcomes of different approaches used for CSF diversion.
Methods
A consecutive review of the medical records of neonates with posthemorrhagic hydrocephalus treated with CSF drainage was conducted.
Results
Forty premature neonates were included. Serial lumbar puncture, ventriculosubgaleal shunt, and ventriculoperitoneal shunt were the treatments of choice in 25%, 37.5% and 37.5% of the cases, respectively.
Conclusion
Cerebrospinal fluid diversion should be tailored to each case with preference given to temporary CSF drainage in neonates with lower age and lower birth-weight, while the permanent ventriculoperitoneal shunt should be considered in healthier, higher birth-weight neonates born closer to term.
MRI is not only beneficial in the diagnosis of cerebrospinal fluid (CSF)-related diseases, but also aids in planning the management and post-surgery follow-up of the patients. With recent advances in MRI systems, there are many newly developed sequences and techniques that rapidly enable evaluation of CSF-related disorders with greater accuracy. For a better assessment of this group of disorders, radiologists should follow the developments closely and should be able to apply them when necessary. In this pictorial review, the role of MRI in the evaluation of hydrocephalus, CSF diversion techniques, and other CSF disorders is illustrated.
Teaching Points
• The 3D-SPACE seems to be most efficient technique for evaluation of hydrocephalus and ventriculostomy.
• In complex cases, PC-MRI, 3D-heavily T2W, and/or CE-MRC images may prevent false results of 3D-SPACE.
• MRI is beneficial in the diagnosis and management of hydrocephalus and other CSF-related diseases.
According to the traditional understanding of cerebrospinal fluid (CSF) physiology, the majority of CSF is produced by the choroid plexus, circulates through the ventricles, the cisterns, and the subarachnoid space to be absorbed into the blood by the arachnoid villi. This review surveys key developments leading to the traditional concept. Challenging this concept are novel insights utilizing molecular and cellular biology as well as neuroimaging, which indicate that CSF physiology may be much more complex than previously believed. The CSF circulation comprises not only a directed flow of CSF, but in addition a pulsatile to and fro movement throughout the entire brain with local fluid exchange between blood, interstitial fluid, and CSF. Astrocytes, aquaporins, and other membrane transporters are key elements in brain water and CSF homeostasis. A continuous bidirectional fluid exchange at the blood brain barrier produces flow rates, which exceed the choroidal CSF production rate by far. The CSF circulation around blood vessels penetrating from the subarachnoid space into the Virchow Robin spaces provides both a drainage pathway for the clearance of waste molecules from the brain and a site for the interaction of the systemic immune system with that of the brain. Important physiological functions, for example the regeneration of the brain during sleep, may depend on CSF circulation.
The objective of the study was to investigate familial aggregation of primary congenital hydrocephalus in an unselected, nationwide population. Based on the Danish Central Person Register, we identified all children born in Denmark between 1978 and 2008 and their family members (up to third-degree relatives). Information on primary congenital hydrocephalus was obtained from the National Patient Discharge Register. Using binomial log-linear regression, we estimated recurrence risk ratios of congenital hydrocephalus. An alternative log-linear regression model was applied to quantify the genetic effect and the maternal effect. Of 1 928 683 live-born children, 2194 had a diagnosis of idiopathic congenital hydrocephalus (1.1/1000). Of those, 75 (3.4%) had at least one other family member with primary congenital hydrocephalus. Significantly increased recurrence risk ratios of primary congenital hydrocephalus were observed for same-sex twins, first- and second-degree relatives as follows: 34.8 (95% confidence interval: 16.4-74.0), 6.2 (95% confidence interval 4.3-8.9) and 2.2 (95% confidence interval 1.6-3.1), respectively. Recurrence risk ratio for third-degree relatives was 1.5 (95% confidence interval 0.8-2.7). A maternal component was supported by the facts that recurrence risk ratios for opposite-sex twins (37.3, 95% confidence interval 11.9-116.7) were significantly higher than other first-degree relatives and that recurrence risk ratios for maternal half-siblings (8.4, 95% confidence interval 3.7-18.7) were significantly higher than for paternal half-siblings (3.0, 95% confidence interval 0.8-12.2). This population-based study found strong evidence of familial aggregation of primary congenital hydrocephalus, which supports the existence of a genetic component to the aetiology. In addition, the pattern of association suggests that a strong maternal component contributes to the familial aggregation.
We carried out a 4-year study of 159 children (ages 1 month-14 years) with pneumococcal meningitis. The study was divided into two periods: the retrospective period (1998-2000: 107 patients), and the prospective period (2001-2002: 52 patients). About 2/3 of the children were under 2 years of age: 72 (45%) were under 1 year of age and 38 (24%) had meningitis during the second year of life. One-third of the patients had signs of otitis media; convulsions were more frequent in patients under 1 year compared with older patients (34.7 vs. 14.9%; P=0.004); 13/159 children (8.2%) died; 93/159 (58.5%) recovered completely, 12.6% had motor sequelae, 6.9% hydrocephalus, 29.8% sensorineural hearing loss; 140/159 (88%) were treated with third generation cephalosporins, yet only 8.7% of the pneumococci identified were completely penicillin-resistant (> or =1 microg/ml); 119/159 were treated with dexamethasone. Four patients had received an injection of heptavalent vaccine. Antibiotics for 1 week prior to admission, shock, abnormal pupils, leukocytes count <6,000 mm(3), and CSF glucose < or =8.5 mg/dl were significantly associated with poor outcome and/or death in the univariate analysis. No patient with leukocytosis >16,000/mm(3) died. Conclusion. Sequelae are very common in pneumococcal meningitis. Poor outcome was associated with pupillary abnormality and a leukocyte count <6,000/mm(3) on admission. Leukocytosis was protective against poor outcome.
OBJECTIVE
Different CSF diversion procedures (ventriculoperitoneal, ventriculoatrial, and lumboperitoneal shunting) have been utilized for the treatment of idiopathic normal pressure hydrocephalus. More recently, endoscopic third ventriculostomy has been suggested as a reasonable alternative in some studies. The purpose of this study was to perform a systematic review and meta-analysis to assess overall rates of favorable outcomes and adverse events for each of these treatments. An additional objective was to determine the outcomes and complication rates in relation to the type of valve utilized (fixed vs programmable).METHODS
Multiple databases (PubMed, Ovid MEDLINE In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid Embase, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, and Scopus) were searched for studies involving patients with idiopathic ventriculomegaly, no secondary cause of hydrocephalus, opening pressure < 25 mm Hg on high-volume tap or drainage trial, and age > 60 years. Outcomes included the proportion of patients who showed improvement in gait, cognition, and bladder function. Adverse events considered in the analysis included postoperative ischemic/hemorrhagic complications, subdural fluid collections, seizures, need for revision surgery, and infection.RESULTSA total of 33 studies, encompassing 2461 patients, were identified. More than 75% of patients experienced improvement after shunting, without significant differences among the different techniques utilized. Overall, gait improvement was observed in 75% of patients, cognitive function improvement in more than 60%, and improvement of incontinence in 55%. Adjustable valves were associated with a reduction in revisions (12% vs 32%) and subdural collections (9% vs 22%) as compared to fixed valves.CONCLUSIONS
Outcomes did not differ significantly among different CSF diversion techniques, and overall improvement was reported in more than 75% of patients. The use of programmable valves decreased the incidence of revision surgery and of subdural collections after surgery, potentially justifying the higher initial cost associated with these valves.
INTRODUCTION: Hydrocephalus occurs in 85% of patients with tuberculous meningitis (TBM). Ventriculoperitoneal shunt (VPS) insertion is first-line treatment for relieving increased intercranial pressure. VPS obstruction secondary to increased protein levels in cerebrospinal fluid (CSF) is a known complication.
OBJECTIVE: To ascertain if there is a difference in protein levels 1) between cranial and lumbar CSF, and to quantify levels associated with VPS obstruction, and 2) obtained from lumbar puncture vs. ventricular CSF.
METHOD: A 30-year retrospective analysis was undertaken. CSF protein levels were statistically analysed to determine correlation between these levels and VPS obstruction.
RESULTS: Of 214 children and 376 adults who underwent VPS insertion for TBM, respectively 27.5% and 25.5% sustained blocked VPS. The mean protein level in CSF collected from the non-obstructed group was 1.76 g/l, compared to 2.94 g/l in the obstructed group. The mean CSF protein level from intraoperative ventricular vs. lumbar puncture samples in the VPS obstruction group was respectively 2.471 g/l and 2.474 g/l.
CONCLUSION: Patients with increased protein levels in CSF are at a high risk of VPS blockage. In these patients, temporary measures should be employed until CSF protein levels decrease.
Purpose Hydrocephalus is caused by an imbalance of production and absorption of cerebrospinal fluid (CSF) or obstruction of its pathways, resulting in ventricular dilatation and increased intracranial pressure. Imaging plays a crucial role in the diagnosis, differential diagnosis and planning of treatment. Methods This review article presents the different types of hydrocephalus und their typical imaging appearance, describes imaging techniques, and discusses differential diagnoses of the different forms of hydrocephalus. Results and Conclusion Imaging plays a central role in the diagnosis of hydrocephalus. While magnetic resonance (MR) imaging is the first-line imaging modality, computed tomography (CT) is often the first-line imaging test in emergency patients. Key points · Occlusive hydrocephalus is caused by obstruction of CSF pathways.. · Malabsorptive hydrocephalus is caused by impaired CSF absorption.. · The MR imaging protocol should always include sagittal high-resolution T2-weighted images.. · When an inflammatory etiology is suspected, imaging with contrast agent administration is necessary.. Citation Format · Langner S, Fleck S, Baldauf J et al. Diagnosis and Differential Diagnosis of Hydrocephalus in Adults. Fortschr Röntgenstr 2017; DOI: 10.1055/s-0043-108550.
© Georg Thieme Verlag KG Stuttgart · New York.
Headache is very common and affects almost everyone at some point. It is one of the most common disorders that leads patients to see their physician. All different forms have the nociception via trigeminal nerve fibers in common. Beside the clinical course headaches are classified as either primary or secondary, with the latter having an identifiable structural or biochemical cause. Imaging has a low diagnostic yield in primary headache but play an important role in the differential diagnosis of secondary forms. An overview of different forms of secondary headache is given, outlining diagnostic procedures and the morphologic imaging features of each syndrome. Key points: • Headache can be differentiated in primary and secondary forms.• Imaging plays an important role in differential diagnosis of secondary forms.• Imaging should be performed in patients with concomitant systemic or neurologic symptoms. Citation Format: • Langner S, Kirsch M, Hosten N Radiological Diagnosis and Differentialdiagnosis of Headache. Fortschr Röntgenstr 2015; DOI: 10.1055/s-0034-1399140.
© Georg Thieme Verlag KG Stuttgart · New York.
Imaging of acute neurologic disease in the emergency department can be challenging because of the wide range of possible causes and the overlapping imaging appearance of many of these entities on nonenhanced computed tomography (CT). The key to formulating a succinct, pertinent differential diagnosis includes characterizing the pattern of abnormalities on CT and identifying key features that suggest a particular diagnosis. This article divides neurologic emergencies into 5 scenarios based on the CT findings, including subarachnoid hemorrhage, intraparenchymal hemorrhage, vasogenic edema without and with underlying mass lesion, and acute hydrocephalus. Specific common or important diagnoses in each category are discussed.
Copyright © 2015 Elsevier Inc. All rights reserved.
Assessment of ventricular size is essential in clinical management of hydrocephalus and other neurological disorders. At present, ventricular size is assessed using indices derived from the dimensions of the ventricles rather than the actual volumes. In a population of 22 children with congenital hydrocephalus and 22 controls, the authors evaluated the relationship between ventricular volume and linear indices in common use, such as the frontooccipital horn ratio, Evans' index, and the bicaudate index. Ventricular volume was measured on high-resolution anatomical MR images. The frontooccipital horn ratio was found to have a stronger correlation with both absolute and relative ventricular volume than other indices. Further analysis of the brain volumes found that congenital hydrocephalus produced a negligible decrease in the volume of the brain parenchyma.
Background In a previous study we found significantly decreased N-acetyl aspartate (NAA) and total N-acetyl (tNA) groups in the thalamus of patients with idiopathic normal pressure hydrocephalus (iNPH) compared with healthy individuals (HI). No significant difference between the groups could be found in the frontal deep white matter (FDWM).
Objective The primary aim of this study was to investigate if these metabolites in the thalamus were normalised after shunt surgery. The secondary aim was to investigate postoperative metabolic changes in FDWM.
Subjects and methods Fourteen patients with iNPH, mean age 74 years, and 15 HI, also mean age 74 years, were examined. Assessment of a motor score (MOSs) was performed before and after shunt surgery. Absolute quantitative 1H-MR spectroscopy (1.5 T, volumes of interest 2.5–3 ml) was performed on the patients in the FDWM and in the thalamus, before and 3 months after shunt surgery, and also once on the HI. The following metabolites were analysed: tNA, NAA, total creatine, total choline (tCho), myo-inositol (mIns), glutamate and lactate concentrations. MRI volumetric calculations of the lateral ventricles were also performed.
Results At 3 months postoperatively, we found no significant changes of tNA or NAA in the thalamus. In contrast, in the FDWM, there was a significant increase of tCho (p=0.01) and a borderline significant decrease of mIns (p=0.06). 12/14 patients were shunt responders (motor function). Median reduction of the lateral ventricle was 16%. A weak correlation between MOS and ventricular reduction was seen.
Conclusions Normalisation of thalamic tNA and NAA could not be detected postoperatively. The increased tCho and decreased mIns in the FDWM postoperatively might relate to clinical improvement.
Cysts and cystic-appearing intracranial masses have a broad imaging and pathologic spectra. The authors review the pathologic findings, origin, radiologic appearance, and differential diagnosis of many different intracranial cysts. A diagnostic algorithm based on most common anatomic locations is presented that helps narrow the differential diagnosis.
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