Onset Of Hydrocephalus Research Articles

Current Trends in the Treatment of Pediatric Hydrocephalus: A Narrative Review Centered on the Indications, Safety, Efficacy, and Long-Term Outcomes of Available Treatment Modalities

The pathophysiologic substrate of pediatric hydrocephalus has not been thoroughly elucidated. Valve-based shunt systems have constituted the main therapeutic option since the late 1950s. The initially used systems were concerning the ventricular system and the atrium. In the 1970s, VA shunts were not the main stay of treatment as the preferred option for the terminal end of the drainage system was the peritoneum. Our review analyzes these valve types and attempts a comparison, based on their functional characteristics. Nowadays, the only available surgical alternative for the treatment of hydrocephalus is ETV. This technique is associated with lower infection rates as well as, on average, a lower re-operation rate. Another term that deserves special mention is related to the outcome of ETV in children who had a medical history of previously incorporated shunts and who were subsequently suffering from shunt malfunction. Well-recognized predictive factors associated with secondary ETV failure include age, early onset of hydrocephalus, and prematurity. Although several attempts have been made in order to establish the optimum surgical treatment management in the different subgroups of patientswho are suffering from shunt dysfunction, there is no universal agreement. Therefore, this review attempts to identify the specific subpopulations of patients in whom the insertion of a drainage system as the preferred treatment modality is associated with an optimum long-term prognosis, compared to ETV, and vice versa. The objective of our study is to analyze the safety, efficacy, and outcomes of drainage devices and ETV in pediatric hydrocephalus patients.

Quality of Life and Motor Function in Children with Spina Bifida and Hydrocephalus: Impact on Family Functioning in Indian Rural Population

Background & Purpose: Spina Bifida means “open spine”, and refers to a common congenital birth defects involving a failure of the neural tube to close or to remain closed during early embryogenesis. A significant complication for infants born with myelomeningocele is the onset of hydrocephalus. In rural Indian populations, where socioeconomic constraints and healthcare accessibility are significant challenges, the correlation between motor function and QoL becomes a critical area of investigation. Thus, the purpose of this study is to explore quality of life and motor function in children with spina bifida and hydrocephalus… its impact on family functioning in Indian rural population. Methodology : 20 Subjects with spina bifida and hydrocephalus, age 6 –10 years, both male and female with mean age of 47.30 months were assessed for their demographic profile according to selection criteria. They were assessed for quality of Life by using PedsQL Infant scoring Scale, Family functioning by using PEDSQLFIS, Motor function by using Gross Motor Function Measure (GMFM), socioeconomic status using Modified Kuppuswamy Socioeconomic Scale. The data was collected and computed in a systematic way and then analysed by using SPSS 20. Results : Correlational analysis was done. Statistically significant correlation was found in duration of operation and GMFM, PedsQL Infant scoring Scale and GMFM. Conclusion : This study shows that children with spina bifida and hydrocephalus in rural India benefit from better quality of life when they have improved motor skills. However, the impact of family dynamics, socioeconomic status, and the length of surgery on their quality of life was less clear. Overall, while good motor function is strongly linked to a better quality of life, other factors also play a role.

Visual disorders in children with shunted hydrocephalus.

The introduction of shunt operations in neurosurgical practice gave a chance for life to these patients and significantly improved its quality. Along with its positive effect, surgical treatment is associated with a number of complications that, together with hydrocephalus itself, affect the subsequent development of shunted patients in the short and long term. In the long term, problems observed in shunted patients are mental retardation, cerebral palsy (CP), epilepsy, hearing and vision disorders. Visual disturbances are common in children with shunted hydrocephalus. These include reduced visual acuity, eye movement disorders, refractive anomalies, visual field defects, reduced color vision and strabismus, optic atrophy. The mechanism of visual disturbances is associated with damage to the oculomotor pathways, optic nerves and optic radiation, dilatation of the lateral ventricles, and damage to the periventricular white matter. Age at onset of hydrocephalus and ventricular dimension are important in the development of visual disturbances and placement of a shunting system can improve visual function. At the same time ophthalmic disturbances may be the first and only sign of shunt dysfunction and rapid normalization of elevated ICP preserves vision. Visual disturbances in patients with shunted hydrocephalus correlate with higher incidence of CP and epilepsy. They determine the children’s social integration and quality of life.

Hypersensitivity to type I interferon as a cause of hydrocephalus development

Ubiquitin specific protease 18 (USP18) serves as a potent inhibitor of Type I interferon (IFN) signaling. Previous studies have shown that Usp18 deficient (homozygous Usp18 gene knockout) mice exhibit hydrocephalus; however, the precise molecular mechanism underlying hydrocephalus development remains elusive. In this study, we demonstrate that mice lacking both type I IFN receptor subunit 1 (Ifnar1) and Usp18 (Ifnar1/Usp18 double knockout mice) are viable and do not display a hydrocephalus phenotype. Moreover, we observed that suppression of USP18 in ependymal cells treated with IFN significantly increased cell death, including pyroptosis, and decreased proliferation. These findings suggest that heightened sensitivity to type I IFN during brain development contributes to the onset of hydrocephalus. Furthermore, they imply that inhibition of IFN signaling may hold promise as a therapeutic strategy for hydrocephalus.

Modulation of Cerebrospinal Fluid Dysregulation via a SPAK and OSR1 Targeted Framework Nucleic Acid in Hydrocephalus.

Hydrocephalus is one of the most common brain disorders and a life-long incurable condition. An empirical "one-size-fits-all" approach of cerebrospinal fluid (CSF) shunting remains the mainstay of hydrocephalus treatment and effective pharmacotherapy options are currently lacking. Macrophage-mediated ChP inflammation and CSF hypersecretion have recently been identified as a significant discovery in the pathogenesis of hydrocephalus. In this study, a pioneering DNA nano-drug (TSOs) is developed by modifying S2 ssDNA and S4 ssDNA with SPAK ASO and OSR1 ASO in tetrahedral framework nucleic acids (tFNAs) and synthesis via a one-pot annealing procedure. This construct can significantly knockdown the expression of SPAK and OSR1, along with their downstream ion channel proteins in ChP epithelial cells, thereby leading to a decrease in CSF secretion. Moreover, these findings indicate that TSOs effectively inhibit the M0 to M1 phenotypic switch of ChP macrophages via the MAPK pathways, thus mitigating the cytokine storm. In in vivo post-hemorrhagic hydrocephalus (PHH) models, TSOs significantly reduce CSF secretion rates, alleviate ChP inflammation, and prevent the onset of hydrocephalus. These compelling results highlight the potential of TSOs as a promising therapeutic option for managing hydrocephalus, with significant applications in the future.

Predicting endoscopic third ventriculostomy success in pediatric shunt dysfunction: a monocentric retrospective case series of 70 consecutive children, systematic review, and meta-analysis.

The outcome of endoscopic third ventriculostomy (ETV) in children who had previously received shunts and who were experiencing shunt dysfunction is still discussed in terms of efficacy (success rate from 40% to 80%) and safety (0%-32.5% of complications). Reported predictive factors of secondary ETV failure are age, early onset of hydrocephalus, and prematurity. The best surgical strategy in the different subgroups of patients with shunt dysfunction is still debated. Therefore, the authors aimed to identify subgroups of patients in whom shunt treatment was associated with favorable outcome of ETV, to define the role of ETV in patients with global rostral midbrain dysfunction syndrome. This study was a monocentric retrospective case series and a meta-analysis of children who had previously received shunts and who underwent secondary ETV for shunt dysfunction between 2012 and 2022. Clinical and MRI features were examined, along with surgical outcome, etiology of hydrocephalus, and preoperative ETV Success Score. Univariate and multivariate analyses were performed to find predictors of outcome of secondary ETV. Youden's J index was calculated on age distribution to find an optimal age cutoff. Systematic review of the literature and a meta-analysis were performed according to the PRISMA statement. Seventy consecutive patients were included. The overall success rate of secondary ETV was 63%. Primary obstructive hydrocephalus, age ≥ 36 months, and the presence of aqueductal obstruction were predictors of ETV success. Multivariate analysis found that age < 36 months, primary inflammatory hydrocephalus, and presence of fourth ventricular obstruction were associated with ETV failure. All patients with global rostral midbrain dysfunction syndrome experienced clinical and radiological improvement after ETV. The meta-analysis showed that postinflammatory etiology and age < 36 months were predictors of ETV failure. ETV is safe and effective for children with obstructive hydrocephalus experiencing shunt dysfunction, notably in cases of primary obstructive hydrocephalus with aqueductal stenosis, and among children whose age was ≥ 36 months who had postinflammatory hydrocephalus.

Hydrocephalus in myelomeningocele.

To investigate certain aspects of hydrocephalus in patients with myelomeningocele. We retrospectively analyzed data of 1050 patients with myelomeningocele who underwent surgical treatment between June 1991 and June 2021. These patients were divided into three groups: group 1 consisted of patients who underwent surgery after the first 6h of life, group 2 consisted of patients who underwent surgery within the first 6h, and group 3 consisted of patients who underwent surgery during the fetal period and before 26 6/7weeks of gestation. There were 125, 590, and 335 patients in groups 1, 2, and 3, respectively. In groups 1 and 2, 593 (83%) patients developed hydrocephalus after birth and required ventriculoperitoneal shunt placement in the maternity ward, mainly within the first 4days of life. In contrast, in group 3, 24 (7.2%) patients required surgery to treat hydrocephalus after birth. Hydrocephalus was the primary cause of mortality in groups 1 and 2, with mortality rates of 35% and 10%, respectively. In group 3, the mortality rate was 0.8% and was not related to hydrocephalus. The onset of hydrocephalus is directly related to myelomeningocele closure in neurosurgery.

A Rare Case of Acute Onset of Hydrocephalus in a Nineteen-Year-Old Patient

A Rare Case of Acute Onset of Hydrocephalus in a Nineteen-Year-Old Patient

Ependymal ciliary motion and their role in congenital hydrocephalus

PurposeSince a case of hydrocephalus in humans considered to be caused by ciliary dysfunction was first reported by Greenstone et al. in 1984, numerous papers on the correlation between ciliary function and hydrocephalus have been published.MethodsWe reviewed the published literature on primary ciliary dyskinesia in humans causing hydrocephalus, focusing on articles specifically examining the relation between ciliary function and hydrocephalus and its treatment. In addition, the authors’ experience is briefly discussed.ResultsFull texts of 16 articles reporting cases of human hydrocephalus (including ventriculomegaly) due to defects in ependymal ciliary function or primary ciliary dyskinesia observed in clinical practice were extracted. In recent years, studies on animal models, especially employing knockout mice, have revealed genetic mutations that cause hydrocephalus via ciliary dysfunction. However, a few reports on the onset of hydrocephalus in human patients with primary ciliary dyskinesia have confirmed that the incidence of this condition was extremely low compared to that in animal models.ConclusionIn humans, it is rare for hydrocephalus to develop solely because of abnormalities in the cilia, and it is highly likely that other factors are also involved along with ciliary dysfunction.

Hydrocephalus in primary intradural spinal cord tumors: a systematic review of the literature in the pediatric population.

Hydrocephalus in children with primary intradural spinal cord tumors is exceedingly rare. Herewith, we performed a systematic literature review to address epidemiology, suggested pathophysiological mechanisms, prognostic factors, and treatment of such cases. We performed a systematic review with the best available evidence on cases of pediatric primary intradural tumors of the spinal cord presented with hydrocephalus. The patients were subjected to quantitative analysis on a basis of epidemiological features (age, sex, tumor type and location, clinical presentation, survival, dissemination). The possible pathophysiological theories are discussed in detail. Forty-four studies with a total of 121 patients were included in the study. Astrocytomas were the most frequent tumor (64.5%) type, while most tumors were located in cervical (31.4%) or cervicothoracic region (25.6%). About half of the cases concerned children under 6years of age. The block of subarachnoid CSF (cerebrospinal fluid) pathways from disseminated tumor cells and the neoplastic inflammation caused by tumor elements advocated to be the major pathogenetic mechanisms. Surgical excision of the tumor and hydrocephalus treatment is usually performed. Primary intradural spinal cord tumors should be considered in children with communicative hydrocephalus of unknown etiology. Onset of hydrocephalus after tumor removal is related to higher mortality.

CAPTURING TUBERCULAR MENINGITIS PROGRESSION IN CHILDREN WITH MULTI-SLICED COMPUTED TOMOGRAPHY (MSCT) SCAN: A PROSPECTIVE CASE REPORT

Background: Tubercular meningitis in children poses a diagnostic challenge, either for its detection and follow-up. Neuroimaging is essential in the diagnosis process and early identification of complication or sequelae. Computed tomography (CT) scan is widely available in secondary and tertiary hospitals in Indonesia, which would improve the management of paediatric tubercular meningitis case.&#x0D; Objective: To describe the clinical progression of a child with tubercular meningitis from detection to sequelae at Bethesda Hospital, Yogyakarta.&#x0D; Case description: A 7-year-old boy was admitted to the emergency unit with acute headache, vomiting, and prolonged fever. Tuberculosis transmission indicated from the mother, which on TB treatment. Non-contrast multi-sliced computed tomography (MSCT) scan was performed on the first-day admission, on the 8th day of admission, before and after VP-shunt procedure for life-threatening hydrocephalus and the follow-up. There was an obvious disease progression shown by CT scan from the early stage, the onset of hydrocephalus, deteriorating hydrocephalus, and post-VP-shunt procedure. The periventricular infarct extended over time, concordant with worsening symptoms.&#x0D; Conclusion: Paediatric tubercular meningitis diagnosis relies on accurate neuroimaging studies to identify the specific sign. In the disease progression, the sign as early as slight hyperdensity should lead to meningitis diagnosis, and earlier treatment of hydrocephalus might prevent further debilitating sequelae or even fatal complications of tubercular meningitis.

Retrospective analysis of the clinical and radiological features of 94 consecutive DIPGs patients to investigate the factors determining the development of hydrocephalus and its impact on clinical status and survival.

There is no consensus in the literature about the impact of hydrocephalus on clinical course and overall survival of diffuse intrinsic pontine gliomas (DIPG) patients as well as about its specific treatment. Authors reviewed a series of DIPG patients to investigate factors related to the onset of hydrocephalus, its treatment, and its impact on clinical course and prognosis. A retrospective observational study was performed enrolling pediatric patients affected by DIPG from 2008 to 2018. Clinical and radiological charts were reviewed to find patients' demographic, pathologic and radiologic features in hydrocephalic and non-hydrocephalic patients. In the hydrocephalus cohort, treatment strategy and its effectiveness and complications were analyzed. Ninety-four pediatric patients were enrolled in the study. Patients who developed hydrocephalus showed significantly lesser maximum axial tumor areas than patients without hydrocephalus (respectively 6.5cm2 vs 16.45cm2, p< 0.005). Hydrocephalus developed in 33 patients (35%) with an onset interval of 5.24 ± 1.21months (range 3.2-7.3). The majority of hydrocephalic patients (28 cases, 90%) were treated by ventriculoperitoneal shunt, the remaining 3 patients being treated by endoscopic third ventriculostomy. Mean overall survival was 16.6months ± 20months without significative difference between the groups. The onset of hydrocephalus occurs in the first moths of the disease story and found a negative correlation with tumor maximal axial diameter. Early treatment of hydrocephalus presents a very low complications rate with satisfying clinical outcome, as it allows the patients to continue the neurooncological therapies being a part of the treatment armamentarium instead of a palliative solution.

An MR-based quantitative intraventricular hemorrhage porcine model for MR-guided focused ultrasound thrombolysis.

Intraventricular hemorrhage (IVH) affects approximately 50% of premature births where 50% further develop post-hemorrhagic ventricular dilation (PHVD). Patients face significant impact to long-term development if PHVD is not managed. Unfortunately, there is no accepted treatment to remove the thrombus caused by IVH. This paper describes an acute and chronic IVH model for use with magnetic resonance-guided focused ultrasound (MRgFUS) thrombolysis. A total of 12 pigs (~ 1month in age) were used in the model (eight acute and four chronic). A pre-operative brain MRI was obtained for ventricular targeting. 1.25cm3/kg of autologous blood was injected through a burr hole lateral to the midline and anterior of the coronal suture at a rate of 0.6cm3/min. A craniotomy was performed to simulate a "fontanelle". Post-operative MRI was used to calculate the clot volume. Chronic piglets were recovered, monitored daily with a neurological scoring system (NSS), and MRI scanned for 21days. The clot injection was well tolerated. The average clot size was 3987mm3 (median = 4330mm, standard deviation = 739mm3). Postmortem examination validated the presence of the clot. In the chronic animals, there was an increase in ventricular volume of 30%. Transient neurological impairment immediately followed clot injection and with onset of hydrocephalus in the chronic animals. This model establishes a measurable and targetable IVH clot in an MRI-based neonatal porcine model. The progressive post-hemorrhagic ventricular dilation in the chronic model is a potential alterable outcome from MRgFUS thrombolysis.

SNX27 Deletion Causes Hydrocephalus by Impairing Ependymal Cell Differentiation and Ciliogenesis.

Down's syndrome (DS) in humans and mouse models has been shown previously to confer a high risk for the development of pathological hydrocephalus. Because we have previously described SNX27 as a component that is consistently downregulated in DS, we present here a robust Snx27-deleted mouse model that produces hydrocephalus and associated ciliary defects with complete penetrance. In addition, we find that γ-secretase/Notch modulation may be a candidate drug target in SNX27-associated hydrocephalus such as that observed in DS. Based on these findings, we anticipate that future study will determine whether modulation of a SNX27/Notch/γ-secretase pathway can also be of therapeutic interest to congenital hydrocephalus.

The Role of a Dynamic Craniospinal Compliance in NPH-A Review and Future Challenges.

Despite the normal mean intracranial pressure in normal pressure hydrocephalus, the cerebral ventricles enlarge. Many hypotheses exist as to why, and these have lately been investigated in simulation. These can be grouped into one of two categories: 1) Tissue damage is caused by a transmantle pressure gradient widening the ventricles mechanically. 2) The overall cerebrospinal fluid dynamics are disturbed resulting from various pathologies. This paper reviews the literature regarding the computational simulation models investigating the development of enlarging ventricles in connection with the onset of hydrocephalus. The models are categorized by the underlying hypothesis and their results are contrasted with clinical findings in the field. Finally, open questions are identified for future modeling approaches.

A systematic review of the risks factors associated with the onset and natural progression of hydrocephalus

The purpose of this study was to systematically assess and synthesize the world literature on risk factors for the onset and natural progression of hydrocephalus, thereby providing a basis for policy makers to identify appropriate risk management measures to mitigate the burden of disease in Canada. Evidence for risk factors was limited for both onset and progression. Two meta-analyses that examined a risk factor for onset met the inclusion criteria. One found a significant protective effect of prenatal vitamins among case control studies, but not cohort/randomized controlled trials (RCTs). The second found maternal obesity to be a significant risk factor for congenital hydrocephalus. Significant risk factors among 25 observational studies included: biological (multiple births, maternal parity, common cold with fever, maternal thyroid disease, family history, preterm birth, hypertension, ischemic heart disease, ischemic ECG changes, higher cerebrospinal fluid protein concentration following vestibular schwannoma); lifestyle (maternal obesity, high-density lipoprotein (HDL) cholesterol, maternal diabetes, maternal age), healthcare-related (caesarean section, interhospital transfer, drainage duration following subarachnoid hemorrhage, proximity to midline for craniectomy following traumatic brain injury); pharmaceutical (prenatal exposure to: tribenoside, metronidazole, anesthesia, opioids); and environmental (altitude, paternal occupation). Three studies reported on genetic risk factors: no significant associations were found. There are major gaps in the literature with respect to risk factors for the natural progression of hydrocephalus. Only two observational studies were included and three factors reported. Many risk factors for the onset of hydrocephalus have been studied; for most, evidence remains limited or inconclusive. More work is needed to confirm any causal associations and better inform policy.

Third ventricular colloid cyst and organic hypomania

A colloid cyst is a cyst containing gelatinous material in the brain. Some colloid cysts result in acute onset of hydrocephalus and may lead to sudden death, and colloid cysts of the third ventricle frequently cause psychiatric disturbances leading to erroneous diagnosis of schizophrenia and neurosis. Here, Drs Javed and Dutta describe a case of third ventricle colloid cyst presenting with a seemingly functional psychiatric illness, which was treated neurosurgically and showed complete resolution of symptoms.

Fingerprint changes in CSF composition associated with different aetiologies in human neonatal hydrocephalus: glial proteins associated with cell damage and loss

BackgroundIn hydrocephalus an imbalance between production and absorption of cerebrospinal fluid (CSF) results in fluid accumulation, compression and stretching of the brain parenchyma. In addition, changes in CSF composition have a profound influence on the development and function of the brain and together, these can result in severe life-long neurological deficits. Brain damage or degenerative conditions can result in release of proteins expressed predominantly in neurons, astroglia, or oligodendroglia into the brain interstitial fluid, CSF and blood. Determination of such products in the CSF might be of value in diagnosing cause, aetiology and/or assessing the severity of the neurological damage in patients with hydrocephalus. We therefore analysed CSF from human neonates with hydrocephalus for these proteins to provide an insight into the pathophysiology associated with different aetiologies.MethodsCSF was collected during routine lumbar puncture or ventricular tap. Samples were categorized according to age of onset of hydrocephalus and presumed cause (fetal-onset, late-onset, post-haemorrhagic or spina bifida with hydrocephalus). Glial fibrillary acidic protein (GFAP), myelin basic protein (MBP), vimentin and 2′ , 3′-cyclic nucleotide 3′-phosphodiesterase (CNPase) were analysed through Western blotting of hydrocephalic CSF samples (n = 17) and compared with data from CSF of normal infants without neurological deficits (n = 8).ResultsGFAP was significantly raised only in CSF from post-haemorrhagic hydrocephalus while MBP was significantly raised in post-haemorrhagic and in spina bifida with hydrocephalus infants. Vimentin protein was only detected in some CSF samples from infants with late-onset hydrocephalus but not from other conditions. Surprisingly, CNPase was found in all neonatal CSF samples, including normal and hydrocephalic groups, although it was reduced in infants with late onset hydrocephalus compared with normal and other hydrocephalic groups.ConclusionsApart from CNPase, which is an enzyme, the markers investigated are intracellular intermediate filaments and would be present in CSF only if the cells are compromised and the proteins released. Raised GFAP observed in post-haemorrhagic hydrocephalus must reflect damage to astrocytes and ependyma. Raised MBP in post-haemorrhagic and spina bifida with hydrocephalus indicates damage to oligodendrocytes and myelin. Vimentin protein detected in some of the late-onset hydrocephalic samples indicates damage to glial and other progenitors and suggests this condition affects periventricular regions. The presence of CNPase in all CSF samples was unexpected and indicates a possible novel role for this enzyme in brain development/myelination. Less CNPase in some cases of late-onset hydrocephalus could therefore indicate changes in myelination in these infants. This study demonstrates differential glial damage and loss in the developing human neonatal hydrocephalic brain associated with different aetiologies.

Role of the subcommissural organ in the pathogenesis of congenital hydrocephalus in the HTx rat

The present investigation was designed to clarify the role of the subcommissural organ (SCO) in the pathogenesis of hydrocephalus occurring in the HTx rat. The brains of non-affected and hydrocephalic HTx rats from embryonic day 15 (E15) to postnatal day 10 (PN10) were processed for electron microscopy, lectin binding and immunocytochemistry by using a series of antibodies. Cerebrospinal fluid (CSF) samples of non-affected and hydrocephalic HTx rats were collected at PN1, PN7 and PN30 and analysed by one- and two-dimensional electrophoresis, immunoblotting and nanoLC-ESI-MS/MS. A distinct malformation of the SCO is present as early as E15. Since stenosis of the Sylvius aqueduct (SA) occurs at E18 and dilation of the lateral ventricles starts at E19, the malformation of the SCO clearly precedes the onset of hydrocephalus. In the affected rats, the cephalic and caudal thirds of the SCO showed high secretory activity with all methods used, whereas the middle third showed no signs of secretion. At E18, the middle non-secretory third of the SCO progressively fused with the ventral wall of SA, resulting in marked aqueduct stenosis and severe hydrocephalus. The abnormal development of the SCO resulted in the permanent absence of Reissner's fibre (RF) and led to changes in the protein composition of the CSF. Since the SCO is the source of a large mass of sialilated glycoproteins that form the RF and of those that remain CSF-soluble, we hypothesize that the absence of this large mass of negatively charged molecules from the SA domain results in SA stenosis and impairs the bulk flow of CSF through the aqueduct.

Delayed Positivization of Cerebral Angiography in Reversible Cerebral Vasoconstriction Syndrome (RCVS) Presenting with Recurrent Subarachnoid Haemorrhage

Benign angiopathy of the central nervous system is a clinical syndrome with evidence of reversible cerebral vasoconstriction (RCVS). Haemorrhagic stroke, either subarachnoid or intracerebral is an unusual presentation of RCVS. We describe a case of RCVS presenting with a subarachnoid haemorrhage (SAH), with rebleeding and onset of hydrocephalus during the first week, and, notably, delayed evidence of typical angiographic features after two negative prior exams. Normalization of the angiographic vasculitic-like lesions was documented at month +6. Repeated cerebral angiograms are mandatory to exclude this kind of disease, and the uncommon presentation of this case reinforces this concept.