Beta-amyloid pathology in human brain microvessel extracts from the parietal cortex: relation with cerebral amyloid angiopathy and Alzheimer's disease.

Several pieces of evidence suggest that blood-brain barrier (BBB) dysfunction is implicated in the pathophysiology of Alzheimer's disease (AD), exemplified by the frequent occurrence of cerebral amyloid angiopathy (CAA) and the defective clearance of Aß peptides. However, the specific role of brain microvascular cells in these anomalies remains elusive. In this study, we validated by Western, ELISA and immunofluorescence analyses a procedure to generate microvasculature-enriched fractions from frozen samples of human cerebral cortex. We then investigated Aß and proteins involved in its clearance or production in microvessel extracts generated from the parietal cortex of 60 volunteers in the Religious Orders Study. Volunteers were categorized as AD (n = 38) or controls (n = 22) based on the ABC scoring method presented in the revised guidelines for the neuropathological diagnosis of AD. Higher ELISA-determined concentrations of vascular Aß40 and Aß42 were found in persons with a neuropathological diagnosis of AD, in apoE4 carriers and in participants with advanced parenchymal CAA, compared to respective age-matched controls. Vascular levels of two proteins involved in Aß clearance, ABCB1 and neprilysin, were lower in persons with AD and positively correlated with cognitive function, while being inversely correlated to vascular Aß40. In contrast, BACE1, a protein necessary for Aß production, was increased in individuals with AD and in apoE4 carriers, negatively correlated to cognitive function and positively correlated to Aß40 in microvessel extracts. The present report indicates that concentrating microvessels from frozen human brain samples facilitates the quantitative biochemical analysis of cerebrovascular dysfunction in CNS disorders. Data generated overall show that microvessels extracted from individuals with parenchymal CAA-AD contained more Aß and BACE1 and less ABCB1 and neprilysin, evidencing a pattern of dysfunction in brain microvascular cells contributing to CAA and AD pathology and symptoms.

Recognition of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) in Two Oligosymptomatic Sisters with Low CADASIL Scale Scores and a Venous Dysplasia: Report of a Novel Greek Family.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) due to mutations of the NOTCH3 gene is the most common cause of inherited cerebral small-vessel disease and one of the genetic causes of migraine with aura. The so-called CADASIL scale has been proposed as a clinical screening tool, and a score of 15 or higher seems useful in identifying patients with high probability of carrying NOTCH3 mutations. We studied a novel Greek family with clinical features compatible with CADASIL. Genetic analysis of NOTCH3 in the 2 living patients revealed the R182C mutation. Both patients had low scores (12 and 14) in the CADASIL scale, probably due to their relatively young age (38 and 37 years, respectively) at which cognitive decline and external capsule involvement have not developed yet. Another unusual feature in the second patient was a venous dysplasia in the parietal lobe. Observations presented here add to the notion that the CADASIL scale, although useful, probably needs a revision, taking into account the patient's age at which the score is calculated.

Contralateral posterior interhemispheric approach to deep medial parietooccipital vascular malformations: surgical technique and results.

OBJECTIVE Deep medial parietooccipital arteriovenous malformations (AVMs) and cerebral cavernous malformations (CCMs) are traditionally resected through an ipsilateral posterior interhemispheric approach (IPIA), which creates a deep, perpendicular perspective with limited access to the lateral margins of the lesion. The contralateral posterior interhemispheric approach (CPIA) flips the positioning, with the midline positioned horizontally for retraction due to gravity, but with the AVM on the upper side and the approach from the contralateral, lower side. The aim of this paper was to analyze whether the perpendicular angle of attack that is used in IPIA would convert to a parallel angle of attack with the CPIA, with less retraction, improved working angles, and no significant increase in risk. METHODS A retrospective review of pre- and postoperative clinical and radiographic data was performed in 8 patients who underwent a CPIA. RESULTS Three AVMs and 5 CCMs were resected using the CPIA, with an average nidus size of 2.3 cm and CCM diameter of 1.7 cm. All lesions were resected completely, as confirmed on postoperative catheter angiography or MRI. All patients had good neurological outcomes, with either stable or improved modified Rankin Scale scores at last follow-up. CONCLUSIONS The CPIA is a safe alternative approach to the IPIA for deep medial parietooccipital vascular malformations that extend 2 cm or more off the midline. Contralaterality and retraction due to gravity optimize the interhemispheric corridor, the surgical trajectory to the lesion, and the visualization of the lateral margin, without resection or retraction of adjacent normal cortex. Although the falx is a physical barrier to accessing the lesion, it stabilizes the ipsilateral hemisphere while gravity delivers the dissected lesion through the transfalcine window. Patient positioning, CSF drainage, venous preservation, and meticulous dissection of the deep margins are critical to the safety of this approach.

Arteries and Veins of the Sylvian Fissure and Insula: Microsurgical Anatomy.

We present a vascular anatomical study of the arteries and veins of the sylvian fissure and insula.A good knowledge of the sylvian fissure, the insula, and their vascular relationship would seem mandatory before performing surgery in this area, whatever the type of surgery (aneurysms, arteriovenous malformations, insular tumors).We start with the sylvian fissure and insula morphology, followed by the MCA description and its perforators, with special attention paid to the insular perforators. We demonstrate that the long insular perforators penetrating in the superior part of the posterior short gyrus and long gyri vascularize, respectively, the corticonuclear and corticospinal fasciculi. We particularly insist too on three anatomical constants regarding the vascularization of the insula, already described in the literature: The superior periinsular sulcus is the only sulcus on the lateral surface of the brain without an artery along its axis; the superior branch of the MCA supplies the anterior insular pole and both the anterior and middle short gyri in 100 % of cases; in at least 90 % of cases, the artery that supplied the central insular sulcus continued on to become the central artery.We end with the anatomical study of the veins and cisterns.

The effect of flavanol-rich cocoa on cerebral perfusion in healthy older adults during conscious resting state: a placebo controlled, crossover, acute trial.

RATIONALE: There has recently been increasing interest in the potential of flavanols, plant-derived compounds found in foods such as fruit and vegetables, to ameliorate age-related cognitive decline. Research suggests that cocoa flavanols improve memory and learning, possibly as a result of their anti-inflammatory and neuroprotective effects. These effects may be mediated by increased cerebral blood flow (CBF), thus, stimulating neuronal function. OBJECTIVES: The present study employed arterial spin labelling functional magnetic resonance imaging to explore the effect of a single acute dose of cocoa flavanols on regional CBF. METHODS: CBF was measured pre- and post-consumption of low (23 mg) or high (494 mg) 330 ml equicaloric flavanol drinks matched for caffeine, theobromine, taste and appearance according to a randomized counterbalanced crossover double-blind design in eight males and ten females, aged 50-65 years. Changes in perfusion from pre- to post-consumption were calculated as a function of each drink. RESULTS: Significant increases in regional perfusion across the brain were observed following consumption of the high flavanol drink relative to the low flavanol drink, particularly in the anterior cingulate cortex and the central opercular cortex of the parietal lobe. CONCLUSIONS: Consumption of cocoa flavanol improves regional cerebral perfusion in older adults. This provides evidence for a possible acute mechanism by which cocoa flavanols are associated with benefits for cognitive performance.

Working memory network plasticity after anterior temporal lobe resection: a longitudinal functional magnetic resonance imaging study.

Working memory is a crucial cognitive function that is disrupted in temporal lobe epilepsy. It is unclear whether this impairment is a consequence of temporal lobe involvement in working memory processes or due to seizure spread to extratemporal eloquent cortex. Anterior temporal lobe resection controls seizures in 50-80% of patients with drug-resistant temporal lobe epilepsy and the effect of surgery on working memory are poorly understood both at a behavioural and neural level. We investigated the impact of temporal lobe resection on the efficiency and functional anatomy of working memory networks. We studied 33 patients with unilateral medial temporal lobe epilepsy (16 left) before, 3 and 12 months after anterior temporal lobe resection. Fifteen healthy control subjects were also assessed in parallel. All subjects had neuropsychological testing and performed a visuospatial working memory functional magnetic resonance imaging paradigm on these three separate occasions. Changes in activation and deactivation patterns were modelled individually and compared between groups. Changes in task performance were included as regressors of interest to assess the efficiency of changes in the networks. Left and right temporal lobe epilepsy patients were impaired on preoperative measures of working memory compared to controls. Working memory performance did not decline following left or right temporal lobe resection, but improved at 3 and 12 months following left and, to a lesser extent, following right anterior temporal lobe resection. After left anterior temporal lobe resection, improved performance correlated with greater deactivation of the left hippocampal remnant and the contralateral right hippocampus. There was a failure of increased deactivation of the left hippocampal remnant at 3 months after left temporal lobe resection compared to control subjects, which had normalized 12 months after surgery. Following right anterior temporal lobe resection there was a progressive increase of activation in the right superior parietal lobe at 3 and 12 months after surgery. There was greater deactivation of the right hippocampal remnant compared to controls between 3 and 12 months after right anterior temporal lobe resection that was associated with lesser improvement in task performance. Working memory improved after anterior temporal lobe resection, particularly following left-sided resections. Postoperative working memory was reliant on the functional capacity of the hippocampal remnant and, following left resections, the functional reserve of the right hippocampus. These data suggest that working memory following temporal lobe resection is dependent on the engagement of the posterior medial temporal lobes and eloquent cortex.

Stepping outside the box: an adolescent with a new-onset seizure.

First-time seizures are frightening to children and their families, but the practice parameter recommends minimal evaluation in the emergency department (ED) for the child who presents in a neurologically normal state. We report a 12-year-old girl with seizure whose evaluation in the ED included a computed tomographic scan, largely because of parental anxiety. Computed tomography demonstrated a cerebral cavernous hemangioma or cavernoma. Because of the high recurrence risk of seizures with cavernomas, she was discharged from the ED with a prescription for an antiepileptic drug.

Antioxidant and Nrf2 inducing activities of luteolin, a flavonoid constituent in Ixeris sonchifolia Hance, provide neuroprotective effects against ischemia-induced cellular injury.

Ixeris sonchifolia Hance is an herb distributed in northeastern part of China and has been used by natives to invigorate circulation. In the present study, bioactivity-guided fractionation of I. sonchifolia Hance extract was performed with the aim to isolate and identify the compounds underlying the potential protective effects against ischemia brain injury. Among the four fractions isolated from the herb extract, the ethyl acetate fraction was found to scavenge DPPH radicals, induce ARE-dependent transcriptional activity and upregulate Nrf2 protein levels. The isolation work focused on this fraction revealed the presence of two categories of compounds: flavonoids and sesquiterpene lactones. Among the five isolated flavonoids, luteolin was evaluated to possess direct and indirect antioxidant activities by scavenging free radicals and inducing the upregulation of ARE-dependent phase II enzymes. Concomitant with the findings from the cell-based assays, in the middle cerebral artery occlusion-induced ischemia rat model, administration of luteolin at 4 mg/kg displayed neuroprotective effects by reducing infarct area and inhibiting neuronal cell death. In summary, the obtained results suggest that flavonoids in I. sonchifolia Hance, in particular luteolin, contribute at least partly to the neuroprotective effects against ischemia-induced cellular injury and can be potentially developed for treatment of ischemia-reperfusion induced diseases.

Brain metastases and multiple cerebral aneurysms from cardiac myxoma: case report and review of the literature.

Cardiac myxoma, the most common benign cardiac tumour, can determine brain metastases or multiple cerebral aneurysms, but very few cases of both complications have been reported. We discuss the therapeutic management in the case of a patient, operated for a cardiac myxoma, who presented three intracerebral tumours and five cerebral microaneurysms.

Schizophrenia with preserved insight is associated with increased perfusion of the precuneus.

BACKGROUND: Preserved insight into illness has been suggested to be predictive of outcome in patients with schizophrenia. We aimed to investigate the functional substrate underlying preserved insight in these patients. METHODS: We recruited patients with paranoid schizophrenia and healthy controls matched for age and sex. Patients were grouped according to preserved or impaired insight into illness using the Scale to assess Unawareness of Mental Disorder (SUMD). Whole-brain technetium-99m ethyl cysteinate dimer single photon emission computed tomography regional cerebral blood flow was compared at the voxel level between the 2 groups using a statistical parametric map (voxel-level significance of p < 0.001, uncorrected; cluster level significance of p < 0.05, uncorrected). RESULTS: We enrolled 31 right-handed patients with schizophrenia and 18 controls in our study. Twenty-one (67.7%) patients had preserved insight. The 2 groups did not differ significantly in demographic and clinical characteristics or in treatment. Compared with controls, the whole group of patients showed bilateral frontotemporal hypoperfusions, with no statistical difference between patients with preserved or impaired insight for these areas. Patients with preserved insight showed significantly increased perfusion of the bilateral precuneus relative to those with impaired insight. LIMITATIONS: Patients with subtypes other than paranoid schizophrenia have to be investigated to assess whether involvement of the precuneus in patients with preserved insight can be identified across the full spectrum of subtypes and symptoms of schizophrenia. Moreover, our study concerned only the central dimension (awareness of mental disorder) of 1 scale (SUMD); other dimensions of insight could be studied. CONCLUSION: Our results show that schizophrenia with preserved insight is associated with greater perfusion of the precuneus, a brain area known to be involved in self- consciousness, suggesting a compensatory mechanism of fronto-temporal impairment.

Cerebral infarction developing in a patient without cancer with a markedly elevated level of mucinous tumor marker.

Previous studies have shown the possible role of mucin in cerebral infarction associated with coagulation abnormalities in patients with cancer, particularly adenocarcinoma. We report a 42-year-old woman who developed motor aphasia and cerebral infarction in the left frontal lobe and right parietal lobe. A mucinous tumor marker, CA125 level, was markedly elevated at 1750 U/mL (normal, <36 U/mL), and the D-dimer level was 6.0 µg/mL (normal, <1 µg/mL). She had adenomyosis and no malignancy was revealed. The CA125 and the D-dimer levels became normal after treatment of adenomyosis. Our findings suggest for the first time that marked elevation of mucinous tumor marker level may cause cerebral infarction even in benign conditions.

Microsurgical retrieval of an endovascular microcatheter trapped during Onyx embolization of a cerebral arteriovenous malformation.

OBJECTIVE: Cerebral arteriovenous malformations (AVMs) are vascular lesions that are amenable to various treatment modalities including stereotactic radiosurgery, fractionated radiotherapy, endovascular embolization, microsurgical obliteration or combined modality treatment. A potential complication of endovascular therapy with embolization material is microcatheter entrapment. We report on a patient for whom surgery was combined with endovascular embolization to obliterate an AVM and retrieve an entrapped endovascular microcatheter. PARTICIPANT: A 52-year-old woman suffered a left parietal hemorrhage from an AVM. She underwent staged endovascular embolization of the lesion using Onyx material. During the second stage of the embolization, the microcatheter (Marathon Flow Directed Microcatheter; eV3 Neurovascular, Inc., Irvine, CA, USA) was retained in the Onyx plug. It was decided to section the microcatheter at the groin and proceed with microsurgical obliteration of the AVM, with removal of the entrapped microcatheter remnant. INTERVENTION: The AVM was dissected circumferentially allowing the meticulous obliteration of the feeding vessels. A single remaining feeding vessel originating from the distal anterior cerebral artery was identified and suspected to contain the entrapped microcatheter. The location was confirmed using stereotactic guidance (BrainLab, Munich, Germany) and the vessel was then sectioned allowing complete removal of the AVM. The microcatheter (102 cm) was then extracted cranially using gentle traction. CONCLUSION: This demonstrates the first incidence of microcatheter removal after procedural entrapment in Onyx embolization material.

Primary angiitis of the central nervous system mimicking tumor-like lesion--case report.

A 60-year-old man presented with a rare case of primary angiitis of the central nervous system mimicking a tumor-like lesion and manifesting as slight disorientation, left hemiparesis, and motor aphasia. Computed tomography showed multiple low density lesions in the left frontal lobe, brain stem, and right parietal lobe. Magnetic resonance images revealed a slightly enhanced mass lesion in the right parietal lobe with surrounding brain edema. Serum, cerebrospinal fluid, and other image examinations did not show any abnormal findings, so surgical removal of the right parietal mass was performed. Histological examination revealed that the mass consisted of hemorrhagic infarction without cellular atypia. Proliferations of endothelial cells in small and medium arteries, and infiltration of macrophages in the perivascular space were detected in the infarction tissues. The histological diagnosis was primary angiitis of the central nervous system.

Cortical hyperexcitability and mechanism of medication-overuse headache.

The present study was conducted to determine the effect of acute (1 h) and chronic (daily dose for 30 days) paracetamol administration on the development of cortical spreading depression (CSD), CSD-evoked cortical hyperaemia and CSD-induced Fos expression in cerebral cortex and trigeminal nucleus caudalis (TNC). Paracetamol (200 mg/kg body weight, intraperitonealy) was administered to Wistar rats. CSD was elicited by topical application of solid KCl. Electrocorticogram and cortical blood flow were recorded. Results revealed that acute paracetamol administration substantially decreased the number of Fos-immunoreactive cells in the parietal cortex and TNC without causing change in CSD frequency. On the other hand, chronic paracetamol administration led to an increase in CSD frequency as well as CSD-evoked Fos expression in parietal cortex and TNC, indicating an increase in cortical excitability and facilitation of trigeminal nociception. Alteration of cortical excitability which leads to an increased susceptibility of CSD development can be a possible mechanism underlying medication-overuse headache.

Shared neural activity in panic disorder and undifferentiated somatoform disorder compared with healthy controls.

OBJECTIVE: In previous studies, some brain areas, including parahippocampal gyrus, were suggested to be associated with panic disorder. Both panic disorder and somatoform disorders are associated with anxiety. This study sought to determine if there are shared neural activity underlying panic disorder and undifferentiated somatoform disorder. METHOD: Sixteen nonmedicated patients with panic disorder, 16 nonmedicated patients with undifferentiated somatoform disorder, and 10 healthy subjects were scanned between February 2005 and August 2006. Diagnoses were made according to the Korean version of the Structured Clinical Interview for DSM-IV Axis I Disorders, Research Version, Patient/Non-Patient Edition. Regional cerebral perfusion was measured by 99 m-Tc-ethyl cysteinate dimer single photon emission computed tomography (SPECT). Using statistical parametric mapping analysis, we compared the SPECT images between the groups. RESULTS: Significant hyperperfusion was found at the left superior temporal gyrus and the left supramarginal gyrus in the panic disorder patients when compared to the controls (family-wise error [FWE], P < .001). The somatoform disorder patients showed hyperperfusion in the left hemisphere at the superior temporal gyrus, inferior parietal lobule, middle occipital gyrus, precentral gyrus, postcentral gyrus, and, in the right hemisphere, at the superior temporal gyrus when compared to the controls (false discovery rate [FDR], P < .001). In contrast, significant hypoperfusion was found at the right parahippocampal gyrus in each of panic disorder (FWE, P = .001) and somatoform disorder (FWE, P < .001) groups compared to healthy controls. However, no significant differences were found in regional cerebral perfusion between the 2 disorder groups. CONCLUSIONS: Both panic disorder and undifferentiated somatoform disorder showed hyperperfusion in the left superior temporal gyrus and hypoperfusion in the right parahippocampal gyrus, which suggests that the 2 disorders are likely to share neural activity.

[Removal of an arteriovenous malformation near the pyramidal tract using the neuronavigation system: a case report].

A 20-year-old female presented with sudden severe headaches. Computed tomography showed an intraventricular hemorrhage and slightly dilated lateral ventricles. Her neurological examinations revealed no abnormalities. Magnetic resonance (MR) images and cerebral angiography revealed arteriovenous malformation (AVM) in the right parietal lobe. The size of the nidus was 1.8 cm, and it was located in the deep white matter. It was fed by middle cerebral arteries and drained by the vein of Galen. Diffusion tensor tractography (DTT) demonstrated that the right pyramidal tract was situated close to the nidus. The AVM was surgically resected completely, without any complications, using intraoperative neuronavigation image guidance. Neuronavigation images combined with DTT are useful for surgical resection of AVM that is located close to the pyramidal tract.

Neuropsychological improvement in patients with cervical spondylotic myelopathy after posterior decompression surgery.

Patients with cervical spondylotic myelopathy sometimes complain of cognitive dysfunction, which may be coincidence. However, cognitive dysfunction may be related to disorders of the cervical spine and/or spinal cord. This study investigated cognitive dysfunction in patients with cervical spinal disorders. A total of 79 patients with cervical spondylotic myelopathy (40 women and 39 men, mean age 61.2 years) underwent cervical laminoplasty between January 2006 and July 2007. Ten of these 79 patients (7 women and 3 men, mean age 65.2 years) complained of moderate to severe memory disturbances. These 10 patients underwent neuroimaging studies and a battery of neuropsychological tests consisting of the mini-mental state examination, Kohs Block Design Test, Miyake Memory Test, Benton Visual Retention Test (BVRT), and "kana-hiroi" test before and 3 months after surgery. Brain magnetic resonance imaging showed no organic brain lesions in the 10 patients, but single photon emission computed tomography demonstrated reduced regional cerebral blood flow in the posterior cortical areas in eight patients before surgery. Neuropsychological test scores showed statistically significant improvement after surgery in the Kohs Block Design Test and the BVRT, which measure visuospatial perception and reflect the function of the parietal and/or occipital lobes (p < 0.05). The practice effect may have contributed to the neuropsychological improvements, but this study suggests that cervical spinal disorders may affect cognitive functions and that surgical treatment can ameliorate such effects.

Impaired fMRI activation in patients with primary brain tumors.

To characterize peritumoral BOLD contrast disorders, 25 patients referred for resection of primary frontal or parietal neoplasms (low-grade glioma (LGG) (n=8); high-grade glioma (HGG) (n=7); meningioma (n=10)) without macroscopic tumoral infiltration of the primary sensorimotor cortex (SM1) were examined preoperatively using BOLD fMRI during simple motor tasks. Overall cerebral BOLD signal was estimated using vasoreactivity to carbogen inhalation. Using bolus of gadolinium, cerebral blood flow (CBF), cerebral blood volume (CBV), and mean transit time (MTT) were estimated. In a 1cm(3) region-of-interest centered on maximal T-value in SM1 contralateral to movements, interhemispheric asymmetry was evaluated using interhemispheric ratios for BOLD and perfusion parameters. During motor tasks contralateral to the tumor, ipsitumoral sensorimotor activations were decreased in HGG and meningiomas, correlated to the distance between the tumor and SM1. Whereas CBV was decreased in ipsitumoral SM1 for HGG, it remained normal in meningiomas. Changes in basal perfusion could not explain motor activation impairment in SM1. Decreased interhemispheric ratio of the BOLD response to carbogen was the best predictor to model the asymmetry of motor activation (R=0.51). Moreover, 94.9+/-4.9% of all motor activations overlapped significant BOLD response to carbogen inhalation.

Huge supratentorial extraventricular anaplastic ependymoma presenting with massive calcification--case report.

A 15-year-old boy presented with an anaplastic supratentorial ependymoma causing massive intratumoral calcification, without contributory medical and family history, and manifesting as persistent headache for 2 months. Physical examination found no neurological deficit except for visual defect in the right lower quadrant, with intact visual acuity. Blood examination showed no abnormalities. Cranial computed tomography revealed a huge calcified mass in the left parietooccipital lobe, with extensive perilesional brain edema. Cranial radiography showed diffuse and symmetrical thinning of the calvarial bone. Magnetic resonance imaging confirmed the tumor as an assembly of medullated masses with extraventricular location, 7 x 6.5 x 6.5 cm in diameter, and appearing as heterogeneous intensity on both T(1)- and T(2)-weighted images with inhomogeneous enhancement except for the central cores. The patient underwent tumor resection. Intraoperative findings revealed that the cortical veins overlying the tumor were reddish and moderately engorged. The hypervascular tumor, entirely extraventricular in location, was totally resected without neurological deterioration. Histological examination revealed that the tumor was highly cellular with hyperchromatic nuclei and cell atypia. Necrosis, mitotic figures, and perivascular pseudorosette formations were frequently seen. Immunohistochemical study showed positive staining for glial fibrillary acidic protein, S-100 protein, vimentin, and epithelial membrane antigen, but negative for synaptophysin. The MIB-1 labeling index was 26.5%. The findings were compatible with anaplastic ependymoma (World Health Organization classification grade 3). Ependymoma should be included in the differential diagnosis of a supratentorially located, extraventricular mass with massive intratumoral calcification.

Congenital duplication of the superior sagittal sinus and parietal encephalocele after vacuum extraction delivery.

A newly-born infant with a congenital dural and bony defect and an associated short-segmented duplication of the superior sagittal sinus suffered from herniation and infarction of parietal brain tissue secondary to vacuum extraction. This ultimately led to the formation of a subgaleal cerebrospinal fluid (CSF) collection. Initial operative closure of the encephalocele was performed by attaching a galeal flap to the periostium surrounding the congenital defect. As the bony defect developed characteristics of a growing fracture later on, dural repair, transplantation of a split-bone flap and, finally, the insertion of a ventriculoperitoneal shunt became necessary. This case affirms that stringent indication and cautious usage of vacuum-assisted delivery is strongly recommended, especially in view of the possibility that undetected congenital cranial, vascular and/or cerebral alterations may be present.