Long-standing Overt Ventriculomegaly In Adults Research Articles

Atypical Presentation of Longstanding Overt Ventriculomegaly in Adults: A Case Report

Atypical Presentation of Longstanding Overt Ventriculomegaly in Adults: A Case Report

CSF dynamics in long-standing overt ventriculomegaly in adults

Long-standing overt ventriculomegaly in adults (LOVA) is a form of chronic hydrocephalus and its pathophysiology and treatment remain debated. An analysis of CSF dynamics in this condition has rarely been reported. The aim of this study was to analyze hydrodynamic characteristics of patients with suspected LOVA to discuss its pathophysiological mechanisms and the importance of CSF dynamics analysis for diagnosis and treatment of these patients. This retrospective cohort study, conducted between May 2018 and October 2022, included adult patients aged > 18 years investigated in a department of neurosurgery through a lumbar infusion study for suspicion of LOVA (n = 23). These patients were then compared with a control cohort explored for suspicion of idiopathic normal pressure hydrocephalus (iNPH; n = 30). Clinical symptoms, radiological findings, and hydrodynamic parameters were analyzed. The authors specifically compared two hydrodynamic parameters: resistance to CSF outflow, or Rout, which relies on CSF resorption, and pressure-volume index (PVI), which reflect overall craniospinal compliance. The lumbar infusion study was considered pathological (confirming the diagnosis of chronic hydrocephalus) when at least one of these two parameters was altered. Rout was significantly less frequently increased (cutoff ≥ 12 mm Hg/ml/min) in patients with LOVA (52%) than in those with iNPH (97%; p < 0.001). In contrast, PVI was impaired (cutoff ≤ 25 ml) in both cohorts, i.e., in 61% of patients with LOVA and in 83% of patients with iNPH. Overall, the rate of pathological lumbar infusion study in LOVA (87%) was not statistically different than in iNPH (100%). However, PVI was the only impaired parameter most frequently found in those with LOVA (35%) compared with those with iNPH (3%; p = 0.002). This study suggests that there is a differential CSF dynamics pattern when comparing patients with LOVA versus those with iNPH. A higher proportion of patients with LOVA showed isolated compliance impairment. These findings highlight the utility of CSF dynamics analysis for the evaluation of patients with suspected chronic obstructive hydrocephalus such as LOVA. Future research with larger case series may help define diagnosis and treatment algorithms of chronic obstructive hydrocephalus based on CSF dynamics analysis, in addition to clinical and radiological criteria.

How should we treat long-standing overt ventriculomegaly in adults (LOVA)? A retrospective cohort study

Long-standing overt ventriculomegaly in adults (LOVA) is a heterogenous group of conditions with differing presentations. Few studies have evaluated success rates of available surgical treatments, or ascertained the natural history. There is a need to assess the efficacy of both endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) as first-line treatments. We conducted a retrospective, single-centre study of adults with LOVA at a tertiary neurosurgery centre in England, UK, aiming to identify presentation, management strategy, and outcome following treatment. A total of 127 patients were included (mean age 48.1 years, 61/127 male). Most patients were symptomatic (73.2%, n = 93/127, median symptom duration 10 months). The most common symptoms were gait ataxia, headache, and cognitive decline (52.8%, 50.4%, and 33.9%, respectively). Fourteen patients had papilloedema. Ninety-one patients (71.7%) underwent surgery (84 ETV, 7 VPS). Over a median follow-up of 33.0 months (interquartile range [IQR] 19.0–65.7), 82.4% had a clinical improvement after surgery, and 81.3% had radiological improvement. Clinical improvement rates were similar between ETV and VP shunt groups (82.1% vs 85.7%, p = 0.812). Surgical complication rates were significantly lower in the ETV group than the VP shunt group (4.8% vs 42.9%, p < 0.001). Of the patients treated surgically, 20 (22.0%) underwent further surgery, with 14 patients improving. This study demonstrates the efficacy of ETV as a first-line treatment for LOVA.

Open-aqueduct LOVA, LIAS, iNPH: a comparative clinical-radiological study exploring the “grey zone” between different forms of chronic adulthood hydrocephalus

PurposeThe definition of chronic adult hydrocephalus encompasses different pathological entities with overlapping characteristics, including long-standing overt ventriculomegaly in adults (LOVA), late-onset idiopathic aqueductal stenosis (LIAS) and idiopathic normal pressure hydrocephalus (iNPH). The aim of our study was to identify preoperative clinical and radiological features peculiar of these diseases providing some pathophysiology inferences on these forms of hydrocephalus.MethodsClinical and radiological preoperative records, type of surgical treatment and clinical outcome of patients with chronic adult hydrocephalus who were surgically treated between 2013 and 2019 were retrospectively reviewed. Univariate and multivariate analyses were performed to evaluate the contribution of each variable to the differential diagnosis.ResultsIn total, 105 patients were included: 18 with LOVA, 23 with LIAS and 64 with iNPH. On multivariate analysis, an enlarged cisterna magna and a more severe ventriculomegaly were associated with the diagnosis of LOVA, while an older age and DESH with iNPH. LIAS patients tend to have an higher prevalence of raised ICP symptoms. Based on that, a clinical and radiological scoring system was developed to distinguish between iNPH and no iNPH cases. A precise cut-off value with a sensitivity of 95.1% and a specificity of 90.6% was identified.ConclusionsLOVA, LIAS and iNPH are different forms of chronic adulthood hydrocephalus and present different and peculiar clinical and radiological features, with an impact on the treatment and outcome prediction. The implementation of a clinical-radiological score for differential diagnosis may help the differentiation. Further studies are warranted.

Characteristics and Treatment of Young Adult-Onset Hydrocephalus

Certain congenital or developmental anomalies of adult-onset hydrocephalus often share clinical symptoms with idiopathic normal pressure hydrocephalus(iNPH). These anomalies include long-standing overt ventriculomegaly in adults(LOVA), persistent Break's pouch cyst(PBC)and panventriculomegaly(PaVM). Certain patients with adult-onset hydrocephalus might have congenital or secondary etiologies, such as late-onset idiopathic aqueductal stenosis(LIAS), and syndrome of hydrocephalus in young and middle-aged adults(SHYMA). Some of these conditions have unknown etiologies, and the definitions of LOVA differ between Japan and overseas. Adult-onset hydrocephalus usually presents with chronic onset, with younger patients tending to have headaches and older patients tending to have iNPH symptoms. In cases where the third ventricle floor bulging, endoscopic third ventriculostomy(ETV)is often performed. However, the arachnoid membrane of the pontine cistern might be strong, and the brain stem might be shifted to the clivus due to the enlarged fourth ventricle. For treating such patients, experienced hands might be needed. At present, it is necessary to carefully examine the cerebrospinal fluid dynamics in each patient and select the best test and treatment.

Surgical Treatment of Long-Standing Overt Ventriculomegaly in Adults (LOVA): A Comparative Case Series between Ventriculoperitoneal Shunt (VPS) and Endoscopic Third Ventriculostomy (ETV).

Background: Long-standing overt ventriculomegaly in adults (LOVA) is an uncommon type of adult chronic hydrocephalus. In recent years, conflicting case series described different outcomes after treatment of LOVA with endoscopic third ventriculostomy (ETV) or ventriculoperitoneal shunt (VPS). The aim of this study is to report a single institutional surgical experience of patients with LOVA in order to evaluate the clinical outcome of those patients treated with one or, sometimes, both surgical procedures, analyzing the main clinical features of these patients, before and after surgery. Methods: We conducted a retrospective study on 31 patients with diagnosis of LOVA, who were treated in our University Hospital between December 2010 and October 2020. We reported gender, age, clinical presentation, surgical treatment, and clinical outcome according to the Kiefer index (KI). Evans’ index, head circumference, aqueductal stenosis and expanded/destroyed sella turcica were assessed on preoperative MRI. Results: The most common clinical manifestation was gait disturbances (100%) followed by urinary incontinence in 23 (74.2%) patients and cognitive deficits in 22 (71%) patients. On preoperative MRI, the overall mean Evans’s Index was 0.49, whereas the overall mean head circumference was 57.3 cm. Twenty-three patients (74.2%) had obliterated cortical sulci, 20 (64.5%) patients had aqueductal stenosis, and 22 (71%) patients had an expanded/destroyed sella turcica on preoperative MRI. Fifteen (48.4%) patients underwent ETV and sixteen (51.6%) were treated with VPS as first surgical procedure. Four (26.6%) out of fifteen patients treated with ETV required a subsequent VPS. The overall median age of patients was 64 (IQR: 54.5–74) and the overall median follow-up was 57 months (IQR 21.5–81.5). Overall morbidity was 22.5%. Mean recovery index (RI), according to KI, was 3.8 ± 4.3 and 2.2 ± 5.6 (p = 0.05) at last follow-up in patients treated with ETV and VPS, respectively. Conclusions: The choice of surgical treatment of LOVA remains under discussion. Although EVT is a tempting option for patients with LOVA, conversion to VP shunt is not uncommon.

Longstanding overt ventriculomegaly in adults (LOVA) with patent aqueduct: surgical outcome and etiopathogenesis of a possibly distinct form of chronic hydrocephalus

PurposeLongstanding overt ventriculomegaly in adults (LOVA) represents a form of chronic adulthood hydrocephalus with symptomatic manifestation in late adulthood. Based on the patency of the aqueduct, two different subcohorts of LOVA can be distinguished. Surgical treatments of this condition are also debated. Therefore, we analyzed preoperative characteristics and clinical outcome after different surgical treatments in a subgroup of LOVA patients with a patent aqueduct.MethodsEighteen LOVA patients with a patent aqueduct consecutively treated at our institution between July 2013 and December 2019 were analyzed for this study. Median age was 70 years. Preoperative radiological and clinical features, surgical procedures (ventriculo-peritoneal shunt or endoscopic third ventriculostomy), and outcomes were collected. Successful outcome was qualitatively defined as an improvement or a halt of progression of the presenting symptoms at follow-up, and quantitatively by changes in mRS and iNPHGS scales.ResultsTwelve patients underwent an ETV as a primary treatment, while 6 underwent VPS. A total of 22.2% of them were lost to follow-up. Median follow-up time was 38 months. Six patients (66.7%) in the ETV cohort achieved a successful outcome after treatment, with a complication rate of 11.1%. Two patients underwent rescue VPS after ETV failure with a good outcome. Four patients (100%) underwent primary VPS and achieved a satisfactory outcome after treatment, with a reported complications rate of 25%.ConclusionLOVA with patent aqueduct represents, in our opinion, a distinct clinical form of chronic hydrocephalus. For this subgroup, as well as for other forms of LOVA, ETV remains an acceptable first-line treatment option considering the good results, and the low complication rate, obtained in those patients and the hypothesis that hydrocephalus is due to an “intracisternal” obstruction.

Long-Standing Overt Ventriculomegaly in Adults (LOVA): Diagnostic Aspects, CSF Dynamics with Lumbar Infusion Test and Treatment Options in a Consecutive Series with Long-Term Follow-Up

Long-standing overt ventriculomegaly in adults is a chronic form of hydrocephalus without a clear pathophysiological description and a consensus about the treatment. We present the results of endoscopic third ventriculostomy (ETV) in a consecutive series with a mean follow-up of 79 ± 23 months, highlighting how the preoperative lumbar infusion test could facilitate understanding the pathophysiology of the disease. We retrospectively collected data regarding clinical assessment, neuroradiological findings, and preoperative lumbar infusion tests in 22 symptomatic patients. In the majority of cases, patients reported imbalance and gait disorders, and 8 subjects had headaches. The preoperative lumbar infusion test demonstrated a mean opening pressure of 13.95 ± 2.88 mm Hg, with plateau values ranging from 22 to 39 mm Hg. The resistance to outflow was 11.21 ± 2.00 mm Hg/mL/min. After the procedure, all patients reported improvement or halted progression in their presenting symptoms, whereas no significant reduction was demonstrated in Evans' index. One subject underwent a second ETV procedure after more than 2 years because of the failure of the endoscopic approach. A progressive exhaustion of brain compliance plays an important role in explaining the dichotomy between severe ventriculomegaly and mild clinical symptoms in patients with long-standing overt ventriculomegaly in adults. The role of the aqueductal stenosis as a diagnostic criterion might be reconsidered. The preoperative infusion test data support this observation. Preoperative assessment should include not only clinical and neuroradiological evaluation but also the study of cerebrospinal fluid dynamics. ETV should be considered the treatment of choice because of its safety and efficacy. Long-term follow-up is mandatory.

Endoscopic third ventriculostomy compared to Ventriculoperitoneal shunt as a first line treatment for long standing overt ventriculomegaly in adults (LOVA): which treatment should be offered?

Endoscopic third ventriculostomy compared to Ventriculoperitoneal shunt as a first line treatment for long standing overt ventriculomegaly in adults (LOVA): which treatment should be offered?

The surgical results of endoscopic third ventriculostomy in long-standing overt ventriculomegaly in adults with papilledema

ObjectiveLongstanding overt vetriculomegaly in adults (LOVA) is a type of chronic hydrocephalus presumed to begin during infancy, which manifests in adults after a long and slow clinical course. Only a quite small number of LOVA case series have been published, controversies regarding optimal management still exist. The authors describe a series of symptomatic LOVA patients with papilledema treated successfully using endoscopic third ventriculostomy (ETV) at a single institution. Patients and methodsIn the past 7 years, 4 LOVA patients with papilledema were surgically treated using ETV. Clinical features and neuroimaging of all patients were carefully reviewed retrospectively. Changes of the third ventricle transverse diameter, Evan’s ratio, frontal occipital horn ratio after operation were measured. ResultThere were two males and two females, with a mean age of 24 (21–29) on first presentation. Presentation symptoms were visual problems (4 cases), headaches (3 cases), hemidysesthesia(1 case), and poor mobility(1 cases). Papilledema and increased intracranial pressure were identified in all 4 cases. The mean follow-up period of this series was 5 years (range 4–6 years). All patients reported improved vision function 3 months and experienced other symptom relief accompanying with normalized intracranial pressure after ETV and did not require any further surgical intervention. ConclusionsEndoscopic third ventriculostomy provides an effective treatment for LOVA patients with papilledema, which can improve the symptoms of LOVA and relive papilledema. The fundoscopy is of great value in making decisions related to surgical intervention for LOVA patients.

LOVA: the role of endoscopic third ventriculostomy and a new proposal for diagnostic criteria

The diagnostic criteria and management of this condition continue to be a topic of debate in the literature. The term definitive symptomatic LOVA (longstanding overt ventriculomegaly in adults) is applied to patients that fulfil a series of diagnostic criteria,which are reviewed in our paper. The main subject of our study is to propose a rearrangement of the criteria for the diagnosis of this entity and adjusted treatment strategies. We present our data evaluating the role of endoscopy in this pathology. This descriptive and analytical study comprised 27 adult patients with LOVA who underwent endoscopic third ventriculostomy (ETV). Data regarding age, gender and onset of symptoms, a previously implanted shunt, clinical examination and radiological findings at the time of diagnosis, and the intracranial pressure (ICP) recordings in some cases, were carefully retrospectively reviewed. ETV success was determined based on clinical state and radiological criteria. ETV was performed in all 27 cases. Mean follow up period was 4.2 years (6 months to 8 years). 2/27 patients (7.40%) were lost to follow-up. The overall success rate for ETV was 76% (21/25 patients). ETV resulted in shunt independency in two of the three patients with a previous shunt. Based on our results, we believe that ETV must be considered as the treatment of choice for patients with symptomatic LOVA.

Surgical treatment of long-standing overt ventriculomegaly in adults (LOVA)

BackgroundLongstanding overt ventriculomegaly in adults (LOVA) is characterised by chronic hydrocephalus presumed to begin during infancy, but arresting before becoming clinically detectable. Later in life clinical features of hydrocephalus ensue, typically in the 5th or 6th decades. Only a relatively small number of LOVA case series have been published, and ambiguity remains regarding optimal management. This case series describes a series of patients with LOVA treated successfully at a single neurosurgical institution using endoscopic third ventriculostomy (ETV).MethodsA series of 14 patients were diagnosed with LOVA using established clinical and radiological criteria. All patients underwent an ETV and their clinical conditions were followed up for up to 5 years post-operatively.ResultsFourteen patients (100 %) reported either improvement or halt of progression in their presenting symptoms 3 months after ETV; 93 % of patients (n = 13) did not require any further surgical intervention. One patient (7 %) reported deterioration in symptoms beyond 3 months post-operatively, which necessitated further surgery (ventriculoperitoneal shunt). These promising outcomes after ETV are mirrored in numerous other LOVA case series. Other works have analysed the value of CSF shunting procedures in LOVA, with mixed results. A direct, prospective comparison of outcomes after shunt procedures and ETV, with a specific focus on LOVA patients, is yet to be completed. A minority of patients fail to respond, or develop recurrence of symptoms, months or years after initial surgical intervention.ConclusionsETV is an attractive option for surgical treatment of LOVA. After surgical treatment for LOVA, long-term follow-up should be considered to screen for late recurrence of the condition.

Long term neuropsychological outcome and management of ‘decompensated’ longstanding overt ventriculomegaly in adults

Objective. We aimed to study a group of patients presenting with symptomatic longstanding overt ventriculomegaly in adults (LOVA) requiring treatment (endoscopic third ventriculostomy), and to study their long term neuropsychological status and functionality. Methods. Twenty patients were treated with endoscopic third ventriculostomy on presentation and were studied with neuropsychological assessments including the repeatable battery for the assessment of neuropsychological status and the hospital anxiety and depression scores. Pre- and post-operative MRI studies were used to assess the patency of the endoscopic third ventriculostomy (ETV) along with clinical outcome scores. Results. A variety of presenting symptoms were experienced including headache (90%), worsening imbalance, attacks of loss of consciousness and cognitive decline. Mean age of patients was 52 years (range, 17–78) with mean head circumference of 60.9 cm. ETV was successful in controlling symptoms in 89% of patients with three patients requiring shunts (gravitational valves). There were no subdurals after surgery. All patients did universally badly in all aspects of the neuropsychological studies without showing any undue anxiety or depression. Conclusion. LOVA patients can decompensate at any time in their adult life with acute symptoms, which are generally well controlled with ETV and with few complications. Sadly, the long-term effects of carrying ‘asymptomatic’ or compensated hydrocephalus since childhood are of a significant cognitive cost to these patients.

Membranous Structures Affecting the Success of Endoscopic Third Ventriculostomy in Adult Aqueductus Sylvii Stenosis

The purpose of the present study was to observe Liliequist's membrane (LM) and membranous structures located in the prepontine cistern via 3-Tesla magnetic resonance imaging (MRI) with 3D driven equilibrium radio frequency reset pulse (DRIVE) sequence and multiplanar reformat (MPR) images and to evaluate the success of endoscopic third ventriculostomy (ETV) by assessing these membranes in adult aqueduct stenosis. 29 patients (17 female, 12 male) with primary aqueductus sylvii stenosis were included in the study. 19 patients were diagnosed as long-standing overt ventriculomegaly in adults (LOVA) and patients had severe ventriculomegaly, macrocephalus, and aqueduct stenosis on MR imaging. 10 patients were diagnosed as aqueduct stenosis presented with acute onset of hydrocephalus with symptoms of raised ICP. All patients in the study group were analyzed with conventional and cine MRI before and after treatment. We performed 3D DRIVE sequence and MPR at 3-T MR equipment to determine the membranous structures in 3 dimensions. We correlated the success of the procedure considering the preoperative, postoperative MRI and intraoperative images. 5 patients (26.3%) with LOVA and 2 patients (20%) with aqueduct stenosis, in total 7 patients (24.1%), did not respond to ETV. Cerebrospinal fluid (CSF) flow was blocked by membranous structures located in the prepontine cistern in 4 of 8 patients. In 2 patients, CSF through the stoma was blocked either by the LM or closed tuber cinerum. In 1 patient insufficient CSF flow was observed through the stoma and the LM accompanying prepontine membranes.Totally closed membranes were observed in the prepontine cistern in 5 patients (17.24%) according to the postoperative MRI. LM was verified in all patients intraoperatively that were also demonstrated in the preoperative MRI. 3D sequences with MPR may help to observe not only the LM but also other membranes located through the prepontine cistern, which may be the reason of failed ETV.

Neuropsychological Changes After Endoscopic Third Ventriculostomy for Long-Standing Overt Ventriculomegaly in Adults -Case Report-

A 39-year-old woman presented with long-standing overt ventriculomegaly in adults (LOVA) manifesting as gradually worsening headache. Past history included treatment for myeloschisis at birth. Intelligence quotient (IQ) was 115 and preoperative psychological assessment using the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) showed normal scores in all domains. However, scores for constructional ability were slightly low. Computed tomography revealed severe ventriculomegaly involving the lateral and third ventricles, and magnetic resonance imaging showed aqueductal stenosis. Endoscopic third ventriculostomy was performed under a diagnosis of LOVA. Postoperatively, the headache resolved and the RBANS showed improvements in memory and constructional ability. Detailed evaluation of cognitive function provides a good indicator in the treatment of hydrocephalus with normal IQ.

Longstanding overt ventriculomegaly in adults: pitfalls in treatment with endoscopic third ventriculostomy

The recently described condition of longstanding overt ventriculomegaly in adults (LOVA) has not been defined in terms of the need for intervention, timing of intervention, and ideal treatment. The purpose of this review was to evaluate the role of endoscopic third ventriculostomy (ETV) in the treatment of LOVA. Data collected in six patients with LOVA who had undergone ETV were reviewed retrospectively in terms of the definition of treatment success, rates of success, complications, and outcome. All six patients presented with headache disorders. In all patients, triventricular hydrocephalus had been diagnosed as aqueductal stenosis, and head circumference measurements were above the 98th percentile. All six had undergone successful ETV as documented by the free flow of cerebrospinal fluid into the basal cisterns, which remained open throughout the follow-up period. After the procedure, one patient experienced a mild degree of difficulty with short-term memory. Five patients remained symptomatic or had symptoms requiring further treatment 3 months to 3 years after ETV. Four patients received ventriculoperitoneal shunts, and one underwent venous stenting for high intracranial pressure after successful ETV. In two patients in whom aqueductal stenosis had been diagnosed, the sylvian aqueduct was patent after the procedure. In LOVA patients who present with headaches, ETV may not lead to improvement in the headaches. Despite the presence of triventricular hydrocephalus, closure of the aqueduct may be a secondary phenomenon, and flow through the aqueduct may be reestablished after ETV. If intracranial hypertension persists after successful ETV, its cause may be increased venous sinus pressure.

Gravitational Shunts in Longstanding Overt Ventriculomegaly in Adults

A new entity of chronic hydrocephalus was introduced recently in the international literature: longstanding overt ventriculomegaly in adults. Previous experience with this disorder has demonstrated that shunt therapy for such patients involves a considerable risk of overdrainage. In the present study, we aimed to clarify whether this risk could be avoided by use of gravitational shunts. A total of 26 adults (age range, 17-75 yr) with macrocephaly and progressive hydrocephalus symptoms underwent implantation of either an adjustable Codman Hakim gravity-assisted shunt (Codman Medos, LeLocle, Switzerland) plus an Aesculap-Miethke ShuntAssistant (Miethke KG, Kleinmachnow, Germany) or a nonadjustable gravitational shunt (Aesculap-Miethke gravity-assisted valve). The follow-up period averaged 29 +/- 13 months (range, 6-48 mo). Significant sustained clinical improvement was achieved in 87% of patients. In more than 90% of patients, Evans index decreased postoperatively by less than 10%. No correlation was documented between the degree of ventricle width reduction and clinical improvement. Only two patients developed subdural hematoma, which was caused by insufficient hydrostatic pressure compensation owing to errors in estimation of intraperitoneal pressure. Unlike conventional differential pressure shunts, gravitational shunts can be used in the treatment of high-risk patients with longstanding overt ventriculomegaly in adults. Significant risk of overdrainage can be avoided. Gravitational shunts offer a viable alternative to endoscopic third ventriculostomy, provided the choice and adjustment of the shunt opening pressure is based on a correct assessment of the hydrostatic pressure to be compensated for.

Neuropsychophysiological findings in a case of long-standing overt ventriculomegaly (LOVA)

Long-standing overt ventriculomegaly in adults (LOVA) is a clinical entity characterized by chronic hydrocephalus with infant onset, slow evolution and clinical disturbances during adulthood. Few cases are reported in literature describing the evident contrast between the severity of hydrocephalus and the relatively spared neurological functioning and cognitive aspects. The authors describe a 59-year-old man with congenital hydrocephalus complaining of persistent gait impairment. Neurological examination showed a mild paraparesis, severe higher cortical function impairment but relatively sparing of daily living activity. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a very remarkable ventriculomegaly compressing the brain cortex but sparing the cerebellum and the brainstem. Brain Single Photon Emission Computer Tomography (SPECT) showed a prevalent cerebellar perfusion as well. Neuropsychological testing was consistent with severe cognitive deterioration and attention disorders. Language and praxis functions seemed to be preserved. Auditory oddball ERPs (P300) showed morphological abnormalities especially of late components. This case report demonstrates in vivo the level of adaptation to which human brain can reach under chronic mechanic stress conditions. The striking poor cerebral parenchyma representation and the relatively spared language and praxic abilities account for a functional reorganization of residual structures due to the neural plasticity.

Gravitational shunt management of long-standing overt ventriculomegaly in adult (LOVA) hydrocephalus

Recently a new subtype of chronic hydrocephalus was described: long-standing overt ventriculomegaly in adults (LOVA). Experience to date has indicated that shunt therapy was contraindicated, due to over-drainage. Therefore we investigated whether this problem could be overcome using gravitational shunts. Thirty macrocephalic adults (17-72 years of age), suffering from progressive hydrocephalus were managed with two different gravitational shunts. The post-operative observation period was 5-87 months. Only two patients developed hygromas, and only one of these required surgical shunt revision. Eighty-seven percent of patients had a long-lasting clinical improvement. Ventricular size was only slightly reduced in 29 patients. There was no correlation between reduction in ventricular size and clinical improvement. Contrary to clinical guidelines issued to date, we demonstrate that LOVA can be treated reliably with gravitational shunts, making them a genuine alternative to endoscopic third ventriculostomy (ETV).

Pathophysiology of long-standing overt ventriculomegaly in adults

Long-standing overt ventriculomegaly in adults (LOVA) is a unique form of hydrocephalus that develops during childhood and manifests symptoms during adulthood. The aim of the present study was to analyze the specific pathophysiological characteristics of LOVA. The specific diagnostic criteria for LOVA include severe ventriculomegaly in adults that is associated with macrocephalus measuring more than two standard deviations in head circumference and/or neuroradiological evidence of a significantly expanded or destroyed sella turcica. Twenty patients who fulfilled these criteria, 14 males and six females, were retrospectively studied. These patients' ages at diagnosis ranged from 15 to 61 years (mean 39.4 years). All had symptoms and/or signs indicating that hydrocephalus first occurred at birth or during infancy in the absence of any known underlying disease. The authors performed a pathophysiological study that included specific variations of magnetic resonance (MR) imaging, such as fluid-attenuated inversion recovery and cardiac-gated cine-mode imaging; intracranial pressure (ICP) monitoring; three-dimensional computerized tomography (CT) scanning; and other techniques. Hydrocephalus was caused by aqueductal stenosis in all patients. Severe ventriculomegaly involving the lateral and third ventricles was associated with a marked expansion or destruction of the sella turcica in 17 cases. Cardiac-gated cine-MR imaging did not reveal any significant movements of cerebrospinal fluid in the aqueduct. Three-dimensional CT ventriculography confirmed that the expanded third ventricle protruded into the sella and, sometimes, extended a diverticulum. Fourteen patients revealed symptoms and signs that indicated increased ICP with prominent pressure waves. Dementia or mental retardation was seen in 11 patients, gait disturbance in 12, and urinary incontinence in eight; all three of these symptoms were observed in seven patients. Thirteen patients experienced visual disturbance. Nine patients underwent ventriculoperitoneal shunt implantation as the initial treatment, leading to postoperative subdural hematoma in all seven cases in which a differential pressure valve was used. Nine patients, three of whom were initially treated by shunt placement, underwent a neuroendoscopic procedure, mainly for third ventriculostomy. Postoperatively, ICP returned to normal, and marked to-and-fro pulsatile movements at the site of ventriculostomy were recognized on cine-MR imaging in patients treated endoscopically. However, the ventriculomegaly was little improved. Consequently, all patients eventually demonstrated improvement in response to either a shunt equipped with a pressure-programmable valve or an endoscopic procedure; however, depression appeared in six patients, who required psychiatric consultation or medication. Such remarkably decreased intracranial compliance but relatively high ICP dynamics are the pathophysiological characteristics of LOVA. The therapeutic regimen should be determined based on the individual's specific pathophysiological makeup.