Clinical performance of fixed-pressure Sphera Duo® hydrocephalus shunt.

INTRODUCTION: Cerebral hydrodynamics complications in shunted patients are due to the malfunction of the system. The objective of this retrospective, single-center, single-arm cohort study is to confirm the safety and performance of Sphera® Duo when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cysts. METHODS: Data were generated by reviewing 112 adult patient's charts, who were submitted to a ventriculoperitoneal shunt surgery and followed for one year after surgery. RESULTS: The results show us that 76% of patients had their neurological symptoms improved and that the reoperation rate was 15% in the first year following surgery. DISCUSSION: Sphera Duo® shunt system is an applicable shunt option in routine neurosurgical management of hydrocephalus by several causes. It has presented good results while mitigating effects of overdrainage. Overdrainage is especially important in adults with non-hypertensive hydrocephalus and can cause functional shunt failure, which causes subnormal ICP (particularly in the upright position) and is associated with characteristic neurological symptoms, such as postural headache and nausea. CONCLUSION: Sphera Duo® shunt system is safe when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cyst.

Clinical performance of fixed-pressure Sphera Duo® hydrocephalus shunt / Performance clínica da válvula de pressão fixa Sphera Duo®

Abstract Introduction: Cerebral hydrodynamics complications in shunted patients are due to the malfunction of the system. The objective of this retrospective, single-center, single-arm cohort study is to confirm the safety and performance of Sphera® Duo when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cysts. Methods: Data were generated by reviewing 112 adult patient's charts, who were submitted to a ventriculoperitoneal shunt surgery and followed for one year after surgery. Results: The results show us that 76% of patients had their neurological symptoms improved and that the reoperation rate was 15% in the first year following surgery. Discussion: Sphera Duo® shunt system is an applicable shunt option in routine neurosurgical management of hydrocephalus by several causes. It has presented good results while mitigating effects of overdrainage. Overdrainage is especially important in adults with non-hypertensive hydrocephalus and can cause functional shunt failure, which causes subnormal ICP (particularly in the upright position) and is associated with characteristic neurological symptoms, such as postural headache and nausea. Conclusion: Sphera Duo® shunt system is safe when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cyst.

Resumo Introdução: As complicações da hidrodinâmica cerebral em pacientes com derivação ventriculoperitoneal são frequentemente relacionadas ao malfuncionamento do sistema. O objetivo deste estudo retrospectivo de coorte de centro único é avaliar a segurança e performance clínica do Sistema Sphera® Duo quando utilizado em adultos com hidrocefalia, pseudotumor cerebral ou cistos aracnoides. Métodos: Avaliamos os prontuários de 112 pacientes adultos submetidos a cirurgia de derivação ventriculoperitoneal e acompanhados por 1 ano após a cirurgia. Resultados: O resultado mostra que 76% dos pacientes melhoraram dos sintomas neurológicos e a taxa de reoperação foi de 15% no primeiro ano após a cirurgia. Discussão: O sistema de derivação Sphera Duo® é uma opção de shunt adequada a ser usada no tratamento neurocirúrgico da hidrocefalia por causas diversas. Ele demonstrou bons resultados clínicos enquanto reduziu riscos de hiperdrenagem. A hiperdrenagem é especialmente preocupante e mórbida em pacientes adultos com hidrocefalia não hipertensiva e pode levar a prejuízo clínico e disfunção da válvula, com sintomas de hipotensão craniana, como cefaléia ortostática e náuseas. Conclusão: O sistema de derivação Sphera Duo® é seguro para tratamento da hidrocefalia, pseudotumor cerebri ou cistos aracnóides em adultos.

Association between ELP4 rs986527 polymorphism and the occurrence and development of intracranial arachnoid cyst.

OBJECTIVE: The association between ELP4 rs986527 polymorphism and the occurrence and development of intracranial arachnoid cyst was studied in this paper. METHODS: Eighty-five patients diagnosed with intracranial arachnoid cysts by cerebral computed tomography scan were selected. Sixty-three healthy volunteers for medical examination in hospitals served as controls. The cognition, depressive symptoms, and the likelihood of headache, dizziness, head trauma history, dementia, depression, and epilepsy were assessed. ELP4 genotypes and its allele frequency were determined by PCR, endonuclease restriction analysis, and gel electrophoresis. RESULTS: ELP4 rs986527 had three genotypes: TT, TC, and CC. The intracranial arachnoid cyst group showed no statistically significant difference in genotype frequencies compared with healthy controls. There was no significant correlation between ELP4 rs986527 polymorphism and location of intracranial arachnoid cyst. TC and C genotype frequencies were associated with a higher incidence of clinical symptoms than TT genotype frequencies, and C allele frequencies were associated with a significantly higher incidence of clinical symptoms compared with T allele frequencies. There was no significant difference in TNF-α and IL-1ß levels between TT/TC/CC genotypes before treatment. After treatment, the levels of TNF-α and IL-1ß were significantly decreased in different genotypes, and the decrease in CC was the greatest. The frequency of TT and TC genotypes was higher than that of CC genotypes. CONCLUSION: ELP4 rs986527 polymorphism affected the incidence of clinical symptoms and the levels of TNF-α and IL-1ß in patients with intracranial arachnoid cysts.

Spinal Arachnoid Cysts: Presentation, management and pathophysiology.

OBJECTIVE: Evaluation of the presentation and outcomes associated with surgical marsupialisation of spinal arachnoid cysts and formulation of a putative hypothesis explaining their pathogenesis. PATIENTS AND METHODS: Cases were identified from electronic and theatre records at a single centre. All patients underwent pre-operative assessment and radiographic evaluation with subsequent spinal multidisciplinary discussion. Following surgery patients were reviewed at 6, 12 weeks, 6-months and beyond. RESULTS: A total of 17 patients with dorsal thoracic arachnoid cysts with a mean age at time of surgery of 58 years with a male to female ratio of 1.8:1 were identified. Paraesthesia (76%), neuropathic pain (76%), weakness (47%) and unsteadiness (53%) were the commonest presenting complaints. Abnormal gait (76%), altered sensation (71%) and weakness (47%) were the most commonly observed signs. Average cyst volume was observed to be 2570 mm3 (sd ±1682, range 544 to 7644 mm3), spanning a median of 2 thoracic levels, with a resultant reduction of cord volume of 33% (sd 12%). A syrinx was associated with 35% of SAC. All cases underwent marsupialisation of the arachnoid cyst. Six months following surgery all patients experienced improvement in at least of one their presenting symptoms and or clinical signs. Weakness, gait and paraesthesia were most likely to improve following surgery. Only 29% of cases had resolution of neuropathic pain, with 13% of the rest reporting an improvement in the sensitivity component of their pain. Clinical improvements correlated with an average 45% (sd 18%) volume increase in previously compressed cord. CONCLUSION: Intradural arachnoid cysts commonly present with paraesthesia, neuropathic pain and gait disturbance. Marsupialisation of the SAC heralds immediate and long-term improvement in symptoms. Cysts putatively arise within a dissection in the septum posticum and give rise to both dynamic and static compression of cord parenchyma secondary to the complex CSF flow dynamics within the thoracic spine.

WNT1-associated osteogenesis imperfecta with atrophic frontal lobes and arachnoid cysts.

A rare form of osteogenesis imperfecta (OI) caused by Wingless-type MMTV integration site family 1 (WNT1) mutations combines central nervous system (CNS) anomalies with the characteristic increased susceptibility to fractures. We report an additional case where arachnoid cysts extend the phenotype, and that also confirms the association of intellectual disabilities with asymmetric cerebellar hypoplasia here. Interestingly, if the cerebellum is normal in this disorder, intelligence is as well, analogous to an association with similar delays in a subset of patients with sporadic unilateral cerebellar hypoplasia. Those cases typically appear to represent vascular disruptions, and we suggest that most brain anomalies in WNT1-associated OI have vascular origins related to a role for WNT1 in CNS angiogenesis. This unusual combination of benign cerebellar findings with effects on higher functions in these two situations raises the possibility that WNT1 is involved in the pathogenesis of the associated sporadic cases as well. Finally, our patient reacted poorly to pamidronate, which appears ineffective with this form of OI, so that a lack of improvement is an indication for molecular testing that includes WNT1.

Rare Case of Convexity Meningioma Growing into Arachnoid Cyst.

BACKGROUND: Meningioma growing into an arachnoid cyst is an extremely rare event. Only 3 cases are reported in the literature. In 2 of them, an operative procedure in or near the arachnoid cyst preceded tumor growth. CASE DESCRIPTION: We report a case of a patient requiring marsupialization of an arachnoid cyst of the middle cranial fossa. On follow-up, 3 years postoperatively he showed no signs of recurrence or tumor growth. One year later, the fourth year after surgery on the cyst, he presented with large tumor growth into the former cyst's cavity. Pathologic workup after resection revealed an atypical meningioma (World Health Organization grade II). CONCLUSIONS: We discuss the possible pathogenesis in light of the scarce published literature, as well as the differential diagnosis of this rapidly growing tumor.

Magnetic resonance imaging provides evidence of glymphatic drainage from human brain to cervical lymph nodes.

Pre-clinical research in rodents provides evidence that the central nervous system (CNS) has functional lymphatic vessels. In-vivo observations in humans, however, are not demonstrated. We here show data on CNS lymphatic drainage to cervical lymph nodes in-vivo by magnetic resonance imaging (MRI) enhanced with an intrathecal contrast agent as a cerebrospinal fluid (CSF) tracer. Standardized MRI of the intracranial compartment and the neck were acquired before and up to 24-48 hours following intrathecal contrast agent administration in 19 individuals. Contrast enhancement was radiologically confirmed by signal changes in CSF nearby inferior frontal gyrus, brain parenchyma of inferior frontal gyrus, parahippocampal gyrus, thalamus and pons, and parenchyma of cervical lymph node, and with sagittal sinus and neck muscle serving as reference tissue for cranial and neck MRI acquisitions, respectively. Time series of changes in signal intensity shows that contrast enhancement within CSF precedes glymphatic enhancement and peaks at 4-6 hours following intrathecal injection. Cervical lymph node enhancement coincides in time with peak glymphatic enhancement, with peak after 24 hours. Our findings provide in-vivo evidence of CSF tracer drainage to cervical lymph nodes in humans. The time course of lymph node enhancement coincided with brain glymphatic enhancement rather than with CSF enhancement.

Spontaneous Repeated Disappearance and Recurrence of Multiple Spinal Intradural Arachnoid Cysts in a Child.

BACKGROUND: Spinal intradural arachnoid cysts are rare in the pediatric population. We present a rare case of intradural spinal arachnoid cysts that spontaneously and repeatedly disappeared and reoccurred. CASE DESCRIPTION: A 2-year-and-8-months-old boy presenting with lower extremity weakness was found to have spinal intradural arachnoid cysts in cervical and thoracolumbar regions at separate times. Although spontaneous disappearance of both lesions was observed, surgical treatment was finally performed for the symptomatic recurrent thoracolumbar lesion. CONCLUSIONS: To the best of our knowledge, this is the first report of spontaneously disappearing and recurring spinal arachnoid cysts.

Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon.

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral ("no-no") involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

An Unusual Presentation of Creutzfeldt-Jakob Disease and an Example of How Hickam's Dictum and Ockham's Razor Can Both Be Right.

Patients can have more than one neurological problem, and sorting out acute from chronic disease can be challenging. The authors report a middle-aged patient who presented with ataxia, right hemiparesis, and abnormal nystagmus. Magnetic resonance imaging (MRI) showed a Chiari and an arachnoid cyst with brainstem compression that appeared to explain his abnormal examination. Shortly after admission, he was noted to have intermittent abnormal behavior and confusion. History from family revealed significant acute and chronic psychiatric problems that appeared to explain his abnormal mental status; this delayed the diagnosis of intermittent complex partial seizures. The multitude of various symptoms resulted in a delay of the final diagnosis of Creutzfeldt-Jakob disease (CJD), which in retrospect explained the entire new physical examination, seizures, and mental status changes.

The association of arachnoid cysts and focal epilepsy: Hospital based case control study.

OBJECTIVE: Arachnoid cysts (ACs) are common findings in brain MRI. Our aim was to examine frequency and distribution of ACs in patients with focal epilepsy, compared to healthy control subjects, and to investigate the association of AC and electro-clinical features of focal epilepsy. PATIENTS AND METHODS: We performed a retrospective case-control study, using data from 180 patients that underwent video-EEG monitoring between 2009 and 2012, and of 114 healthy controlled subjects. Analysis of electro-clinical data and structural MRI images was conducted. RESULTS: A significantly higher proportion of ACs in the focal epilepsy group (19/180; 10.5%) compared to healthy control subjects (3/114; 2.6%) (p=0.012) was identified. Significant congruence of semiological features or interictal and ictal EEG with AC localization was identified in only one MRI nonlesional patient with temporal cyst localization. CONCLUSION: ACs are seen more often in patients with focal epilepsy. Explicit association between focal epilepsy and AC is possible but exceptional. More likely, focal epilepsy and AC share a common etiological ancestor but represent distant and distinct entities.

Defective Gpsm2/Gαi3 signalling disrupts stereocilia development and growth cone actin dynamics in Chudley-McCullough syndrome.

Mutations in GPSM2 cause Chudley-McCullough syndrome (CMCS), an autosomal recessive neurological disorder characterized by early-onset sensorineural deafness and brain anomalies. Here, we show that mutation of the mouse orthologue of GPSM2 affects actin-rich stereocilia elongation in auditory and vestibular hair cells, causing deafness and balance defects. The G-protein subunit Gαi3, a well-documented partner of Gpsm2, participates in the elongation process, and its absence also causes hearing deficits. We show that Gpsm2 defines an ∼200 nm nanodomain at the tips of stereocilia and this localization requires the presence of Gαi3, myosin 15 and whirlin. Using single-molecule tracking, we report that loss of Gpsm2 leads to decreased outgrowth and a disruption of actin dynamics in neuronal growth cones. Our results elucidate the aetiology of CMCS and highlight a new molecular role for Gpsm2/Gαi3 in the regulation of actin dynamics in epithelial and neuronal tissues.

Intracranial Intra-arachnoid Diverticula and Cyst-like Abnormalities of the Brain.

Primary intracranial cystic or cyst-like lesions include intra-arachnoid, epidermoid, dermoid, and choroid plexus cysts. Differentiation of these cystic lesions can usually be accomplished by imaging studies alone; however, some cysts are similar in appearance and require histopathology for definitive diagnosis. Clinical signs often reflect the location of the cysts within the intracranial cavity rather than the type of cyst. If clinical signs are significant and progressive, surgical removal is warranted and may be successful, although cystic contents could be harmful if allowed to contact surrounding brain parenchyma or meninges.

Quistes aracnoideos. Evolución histórica del concepto y teorías fisiopatológicas / Arachnoid cysts. Historical evolution of the concept and pathophysiological theories

Los quistes aracnoideos del sistema nervioso central son colecciones de líquido similar o igual al cefalorraquídeo, de naturaleza benigna, revestidas de una membrana aracnoidea y de origen congénito o secundarias a traumatismo, hemorragia o infección. Descritos por primera vez por Bright en 1931, su definición y posible fisiopatología ha sufrido repetidas modificaciones hasta nuestros días. El objetivo de este trabajo es presentar un resumen de este proceso

Arachnoid cysts of central nervous system are benign collections filled with a liquid that is equal o similar to cerebrospinal fluid, coated with a single layer or flattened arachnoid cells membrane, congenital or secondary to trauma, meningitis or hemorrhage. First described by Bright in 1931, its definition and possible pathophysiology has been debated to date. Our objective is to present a summary of this process

Visualization of Pulsatile CSF Motion Around Membrane-like Structures with both 4D Velocity Mapping and Time-SLIP Technique.

PURPOSE: We compared the depiction of pulsatile CSF motion obtained by 4-dimensional phase-contrast velocity mapping (4D-VM) with that by time-spatial labeling inversion pulse (time-SLIP) technique in the presence of membrane structures. MATERIALS AND METHODS: We compared the 2 techniques using a flow phantom comprising tubes with and without a thin rubber membrane and applied the techniques to 6 healthy volunteers and 2 patients to analyze CSF dynamics surrounding thin membrane structures, such as the Liliequist membrane (LM), or the wall of an arachnoid cyst. RESULTS: Phantom images exhibited propagation of the flow and pressure gradient beyond the membrane in the tube. In contrast, fluid labeled by the time-SLIP technique showed little displacement from the blockage of spin travelling by the membrane. A similar phenomenon was observed around the LM in healthy volunteers and the arachnoid cyst wall in a patient. CONCLUSION: Four-dimensional phase-contrast velocity mapping permitted visualization of the propagation of CSF pulsation through the intracranial membranous structures. This suggests that 4D-VM and the time-SLIP technique provide different information on flow and that both techniques are useful for classifying the pathophysiological status of CSF and elucidating the propagation pathway of CSF pulsation in the cranium.

[Arachnoid cysts. Historical evolution of the concept and pathophysiological theories]. / Quistes aracnoideos. Evolución histórica del concepto y teorías fisiopatológicas.

Arachnoid cysts of central nervous system are benign collections filled with a liquid that is equal o similar to cerebrospinal fluid, coated with a single layer or flattened arachnoid cells membrane, congenital or secondary to trauma, meningitis or hemorrhage. First described by Bright in 1931, its definition and possible pathophysiology has been debated to date. Our objective is to present a summary of this process.