Degree Of Hydrocephalus Research Articles

Relationship Between Automated Pupillometry Measurements and Ventricular Volume in Patients With Aneurysmal Subarachnoid Hemorrhage.

INTRODUCTION: Pupillometry allows for a standardized assessment of the pupillary light reflex. Acute hydrocephalus (HCP) is a common complication in patients with aneurysmal subarachnoid hemorrhage (aSAH). HCP may affect the pupillary light reflex because of increased intracranial pressure or dilation of the rostral aqueduct. The association between the pupillary light reflex and HCP in aSAH patients has not been clearly established. The objective of this study is to analyze the correlation between the Neurological Pupil index (NPi) and the degree of HCP in aSAH patients. METHODS: The Establishing Normative Data for Pupillometer Assessments in Neuroscience Intensive Care Registry is a prospectively collected database of pupillometry readings in patients admitted to 4 different neurological intensive care units. Patients in the registry with aSAH who had pupillometry assessments within 6 hours of a brain computed tomography were studied. The degree of HCP was quantified using the HCP score, and the relationship between the NPi and HCP was analyzed after controlling for confounders. RESULTS: A total of 43 patients were analyzed (mean age, 54 ± 15 years; 53.2% male; mean HCP score, 5.3 ± 3.8). Thirty-eight patients had HCP. Mean NPi for the right eye was 4.02 (±1.2), and that for the left eye was 3.7 (±1.5). After adjusting for age, sex, race, and sedation, there was no significant correlation between HCP and NPi (right eye: r = 0.12, P = .44; left eye: r = 0.04, P = .8). CONCLUSION: In patients with aSAH, NPi was not correlated with HCP score. A small sample size could be a limitation of this study. Additional studies are needed to characterize the clinical significance of pupillometry in the evaluation of patients with aSAH and HCP.

Clinical Study of Cranioplasty Combined With Ipsilateral Ventriculoperitoneal Shunt in the Treatment of Skull Defects With Hydrocephalus.

To explore the clinical effect and safety of cranioplasty combined with ipsilateral ventriculoperitoneal shunts in the treatment of skull defects with hydrocephalus. The clinical data of 78 patients with skull defects with hydrocephalus were analyzed retrospectively. All patients were treated with cranioplasty and ventriculoperitoneal shunts in 1 stage, including 35 cases of cranioplasty combined with ipsilateral ventriculoperitoneal shunts (ipsilateral operation group) and 43 cases of contralateral operations (contralateral operation group). The incision length (28.97 ± 4.55 cm), operation time (139.00 ± 42.27 minutes), and intraoperative hemorrhage (174.57 ± 79.35 mL) in the ipsilateral operation group were significantly better than those in the contralateral operation group (respectively they were 37.15 ± 5.83 cm, 214.07 ± 34.35 minutes, and 257.21 ± 72.02 mL), and the difference was statistically significant (t = 6.786, 8.656, and 4.815, all P < 0.05). The degree of postoperative hydrocephalus was significantly improved in both groups, but there was no statistically significant difference in the degree of hydrocephalus between the 2 groups (P > 0.05). Among the postoperative complications, there was no statistically significant difference in infection, epilepsy, subdural effusion, titanium plate effusion, or excessive cerebrospinal fluid drainage between the 2 groups (P > 0.05), but the incidence of intracranial hemorrhage in the ipsilateral operation group (2.86%) was significantly lower than that in the contralateral operation group (20.93%, χ2 = 4.138, P = 0.042). The postoperative Glasgow Coma Scale scores of the 2 groups were improved compared with those before the operation (P < 0.05), and there was no statistically significant difference in the postoperative Glasgow Coma Scale scores (P > 0.05). At 6 months after surgery, there was no statistically significant difference in Glasgow Outcome Scale effectiveness between the 2 groups (χ2 = 0.005, P = 0.944). Cranioplasty combined with ipsilateral ventriculoperitoneal shunt has the same therapeutic effect as a contralateral operation, but it has the advantage of a short operation time, less intraoperative trauma, less bleeding, and less risk of intracranial hemorrhage, which is suitable for clinical applications.

Dynamic disequilibrium of macromolecular transport as possible mechanism for hydrocephalus associated with long-term spaceflight

Hydrocephalus associated with long term spaceflight (HALS) for missions lasting over five months is well described but poorly understood. While structural changes of the brain due to microgravitational forces affecting the circulation of cerebrospinal fluid (CSF) have been described as one potential cause, we propose an alternative hypothesis based on dynamic disequilibrium of macromolecular transport across the blood brain barrier. We propose that factors altering physiology under conditions of spaceflight such as microgravity, hypercapnia, venous hypertension, medications, and dietary substances contribute to increased protein load in the ventricles and/or contribute to impairment of transport out of the ventricles that results in HALS. Individual variation in the genetic expression of efflux transporters (p-glycoprotein) has been shown to correlate with the presence and degree of hydrocephalus in animal studies. We describe the evidence behind this concept and propose how these factors can be studied in order to determine the underlying pathogenesis which is imperative in order to cure or prevent HALS.

Hydrocephalus Growth: Definition, Prevalence, Association with Poor Outcome in Acute Intracerebral Hemorrhage.

To propose a novel definition for hydrocephalus growth and to further describe the association between hydrocephalus growth and poor outcome among patients with intracerebral hemorrhage (ICH). We analyzed consecutive patients who presented within 6h after ICH ictus between July 2011 and June 2017. Follow-up CT scans were performed within 36h after initial CT scans. The degree of hydrocephalus were evaluated by the hydrocephalus score of Diringer et al. The optimal increase of the hydrocephalus scores between initial and follow-up CT scan was estimated to define hydrocephalus growth. Poor long-term outcome was defined as a modified Rankin Scale of 4-6 at 3months. Multivariate logistic regression analysis was performed to investigate the hydrocephalus growth for predicting 30-day mortality, 90-day mortality, and poor long-term outcome. A total of 321 patients with ICH were included in the study. Of 64 patients with hydrocephalus growth, 34 (53.1%) patients presented with both concurrent hematoma expansion and intraventricular hemorrhage (IVH) growth. After adjusting for potential confounding factors, hydrocephalus growth independently predicted 30-day mortality, 90-day mortality, and 90-day poor long-term outcome in multivariate logistic regression analysis. Hydrocephalus growth showed higher accuracy for predicting 30-day mortality, 90-day mortality, and poor long-term outcome than IVH growth or hematoma expansion, respectively. Hydrocephalus growth is defined by strongly predictive of short- or long-term mortality and poor outcome at 90days, and might be a potential indicator for assisting clinicians for clinical decision-making.

Relationship between ocular and computed tomography findings in patients with spina bifida

Objectives: Spina bifida is one of the most common congenital diseases in Turkey and around the world. Despite the developing diagnostic and therapeutic methods, abnormal ocular characteristicsin spina bifida patients are still quite common. We investigated the ocular characteristics of spina bifida patients with and without hydrocephalus.Methods: We included 37 patients who were previously referred to the Istanbul Bilim University, Department of Ophthalmology and already had computed tomography (CT) scans. We retrospectively investigated the patients’ ophthalmologic findings (refractive errors, strabismus, and optic disc characteristics) and used their recent CT images to measure the Evans ratios (ERs), which indirectly reflect the grade of hydrocephalus. The patients were divided into three groups according to their ERs (ER ≤ 0.3, 0.3-0.5, and ≥ 0.5), and then the ocular characteristics of these groups were compared. In addition, the patients were divided into three groups according to their ages ( ≤ 1 year, 1-3 years, and ≥ 3 years), and the ERs and rates of refraction defects in these groups were compared.Results: There was no relationship between specific ocular characteristics and ER or between age and ER. However, refraction errors were observed more fr equently as patient age increased.Conclusions: The degree of hydrocephalus does not affect the ocular characteristics of patients with spina bifida, but emmetropization may be deteriorated in these patients.

Long-term cognitive deficits in pediatric low-grade glioma (LGG) survivors reflect pretreatment conditions-report from the German LGG studies.

BackgroundDisease and treatment contribute to cognitive late effects following pediatric low-grade glioma (LGG). We analyzed prospectively collected neuropsychological data of German pediatric LGG survivors and focused on the impact of hydrocephalus at diagnosis, neurofibromatosis type 1 (NF1) status, and extent of surgery.MethodsWe used the Neuropsychological Basic Diagnostic screening tool based on the Cattell–Horn–Carroll model for intelligence and the concept of cross-battery assessment at 2 and 5 years from diagnosis for 316 patients from the German pediatric LGG study and LGG registry (7.1 years median age; 45 NF1; cerebral hemispheres 16%, supratentorial midline 39%, infratentorial 45%). Hydrocephalus was classified radiologically in 137 non-NF1 patients with infratentorial tumors (95/137 complete/subtotal resection).ResultsPatients with NF1 versus non-NF1 exhibited inferior verbal short-term memory and visual processing (P < .001–.021). In non-NF1 patients, infratentorial tumor site and complete/subtotal resection were associated with sequelae in visual processing, psychomotor speed, and processing speed (P < .001–.008). Non-NF1 patients without surgical tumor reduction and/or nonsurgical treatment experienced similar deficits. Degree of hydrocephalus at diagnosis had no further impact. Psychomotor and processing speed were impaired comparably following chemo-/radiotherapy (P < .001–.021). Pretreatment factors such as NF1 or tumor site were relevant at multivariate analysis.ConclusionsAll pediatric LGG survivors are at risk to experience long-term cognitive impairments in various domains. Even surgical only management of cerebellar LGG or no treatment at all, that is, biopsy only/radiological diagnosis did not protect cognitive function. Since pattern and extent of deficits are crucial to tailor rehabilitation, neuropsychological and quality of survival assessments should be mandatory in future LGG trials.

Unruptured brain arteriovenous malformations and hydrocephalus: Case series and review of the literature

ObjectiveHydrocephalus is an uncommon presentation of unruptured brain arteriovenous malformations (AVMs). The goal of this case series and literature review is to present possible pathological mechanisms, management strategies and outcomes in patients with hydrocephalus due to unruptured AVMs. MethodsThree consecutive patients with hydrocephalus caused by unruptured AVMs as well as all cases previously reported in the literature were retrospectively reviewed to determine clinical symptoms, AVM location, nidus size, venous drainage, mechanism of hydrocephalus, level/cause of obstruction, and degree of hydrocephalus. Management of hydrocephalus, AVM treatment, and follow-up length were evaluated. ResultsOf 350 patients unruptured AVMs, 3 presented with hydrocephalus (0.8%). In the literature review we found an additional 22 patients for a total of 25 cases. Eighteen patients had mechanical obstruction by the draining vein or the AVM nidus, usually at the level of the aqueduct (52%). Impaired cerebrospinal fluid resorption secondary to venous congestion led to hydrocephalus in 7 patients. Ten patients were treated for both the AVM and hydrocephalus, 13 patients underwent treatment of either hydrocephalus, or the AVM alone. Treatment of hydrocephalus, with or without associated treatment of the brain AVM, resulted in improvement of symptoms in 92% of patients. No rupture of the AVM was reported at follow-up. ConclusionsThe most common cause of hydrocephalus in unruptured brain AVMs is mechanical obstruction by the draining vein if it is located in a strategic position. Treatment of hydrocephalus alone or with associated treatment of the AVM is safe and effective.

Targeting Germinal Matrix Hemorrhage-Induced Overexpression of Sodium-Coupled Bicarbonate Exchanger Reduces Posthemorrhagic Hydrocephalus Formation in Neonatal Rats.

BackgroundGerminal matrix hemorrhage (GMH) is a leading cause of mortality and lifelong morbidity in preterm infants. Posthemorrhagic hydrocephalus (PHH) is a common complication of GMH. A sodium‐coupled bicarbonate exchanger (NCBE) encoded by solute carrier family 4 member 10 gene is expressed on the choroid plexus basolateral membrane and may play a role in cerebrospinal fluid production and the development of PHH. Following GMH, iron degraded from hemoglobin has been linked to PHH. Choroid plexus epithelial cells also contain iron‐responsive element‐binding proteins (IRPs), IRP1, and IRP2 that bind to mRNA iron‐responsive elements. The present study aims to resolve the following issues: (1) whether the expression of NCBE is regulated by IRPs; (2) whether NCBE regulates the formation of GMH‐induced hydrocephalus; and (3) whether inhibition of NCBE reduces PHH development.Methods and Results GMH model was established in P7 rat pups by injecting bacterial collagenase into the right ganglionic eminence. Another group received iron trichloride injections instead of collagenase. Deferoxamine was administered intraperitoneally for 3 consecutive days after GMH/iron trichloride. Solute carrier family 4 member 10 small interfering RNA or scrambled small interfering RNA was administered by intracerebroventricular injection 24 hours before GMH and followed with an injection every 7 days over 21 days. NCBE expression increased while IRP2 expression decreased after GMH/iron trichloride. Deferoxamine ameliorated both the GMH‐induced and iron trichloride–induced decrease of IRP2 and decreased NCBE expressions. Deferoxamine and solute carrier family 4 member 10 small interfering RNA improved cognitive and motor functions at 21 to 28 days post GMH and reduced cerebrospinal fluid production as well as the degree of hydrocephalus at 28 days after GMH.ConclusionsTargeting iron‐induced overexpression of NCBE may be a translatable therapeutic strategy for the treatment of PHH following GMH.

Central Nervous System Tuberculosis

Central nervous system tuberculosis (CNS-TB) takes three clinical forms: meningitis (TBM), intracranial tuberculoma, and spinal arachnoiditis. TBM predominates in the western world and presents as a subacute to chronic meningitis syndrome with a prodrome of malaise, fever, and headache progressing to altered mentation and focal neurologic signs, followed by stupor, coma, and death within five to eight weeks of onset. The CSF formula typically shows a lymphocytic pleocytosis, and low glucose and high protein concentrations. Diagnosis rests on serial samples of CSF for smear and culture, combined with CSF PCR. Brain CT and MRI aid in diagnosis, assessment for complications, and monitoring of the clinical course. In a patient with compatible clinical features, the combination of meningeal enhancement and any degree of hydrocephalus is strongly suggestive of TBM. Vasculitis leading to infarcts in the basal ganglia occurs commonly and is a major determinant of morbidity and mortality. Treatment is most effective when started in the early stages of disease, and should be initiated promptly on the basis of strong clinical suspicion without waiting for laboratory confirmation. The initial 4 drug regimen (isoniazid, rifampin, pyrazinamide, ethambutol) covers the possibility of infection with a resistant strain, maximizes antimicrobial impact, and reduces the likelihood of emerging resistance on therapy. Adjunctive corticosteroid therapy has been shown to reduce morbidity and mortality in all but late stage disease.

Analysis of the treatment strategies and surgical complications of hydrocephalus after decompressive craniectomy

Objectives To investigate the reasonable operation time and operation approaches of the hydrocephalus treatment after decompressive craniectomy and to count its complication characteristics. Methods From January 2009 to February 2015, the clinical data of 54 patients treated with cranioplasty and ventricular peritoneal (V-P) shunt at the same time (n=21) or different time (n=33) at the Department of Neurosurgery, Shandong Jiaotong Hospital and the Emergency Surgery, Qilu Hospital of Shandong University were analyzed retrospectively. The operation time of both procedures and the operation related complications were analyzed and discussed. Results The surgical complication rate was 32% (17/54). The occurrence rates of complications of cranioplasty and V-P shunt groups over the same period were higher than those of the staging operation group (57% vs. 15%; P<0.05). The incidence of postoperative infection of the synchronous operation group was significantly higher than that of the staging operation group (P<0.02). The incidence of postoperative complication in patients with flap bulging was higher than that in patients with flap depression (40% vs. 16%, P<0.05). There were no statistical significance in the effects of successively order, skull defect area, and degree of hydrocephalus on complications of the cranioplasty and V-P shunt between the 2 groups, however, 13 patients did not treated with V-P shunt because of their hydrocephalus were improved significantly after the first cranioplasty. Conclusions Because of its lower incidence of complications, the staging cranioplasty and V-P shunt might be more beneficial to the treatment of patients, especially for those with obvious flap bulging. Because the order of 2 procedures did not have any effect on postoperative complications and no need to perform V-P shunt after partial cranioplasty, therefore, it was recommended that patients without flap bulging should conduct cranioplasty first. Key words: Decompression, Surgery; Hydrocephalus; Neurosurgical procedures; Postoperative complications

An experiment model of obstructive hydrocephalus in the rats: injecting the astrocytes into the lateral ventricle

Objective To establish the animal model of obstructive hydrocephalus in rat by injecting lateral ventricle astrocytes from rat. Methods Three different concentrations of 20 μl astrocytes suspension with 5×107, 5×108, 5×109 cells per milliliter were injected into the right lateral ventricle of group A, B, C through stereotactic puncture, respectively, while 20 μl sterile phosphate buffer saline (PBS) solution was injected into the right lateral ventricle of control group. The magnetie resonance imaging (MRI) was performed to obtain T2 weighted brain images at 7, 14, 21, 28, 35 days after injection. Measure the maximum transverse diameter of lateral ventricle through coronary position images to determine the degree of hydrocephalus. Results The mean value of maximum transverse diameter in the control group, group A, group B and group C was (0.11±0.01), (1.01±0.78), (1.83±0.23), (1.89±0.21) mm at 35th day after injection, respectively. Compared with group A, group B and group C, the maximum transverse diameter of control group was statistically different(P 0.05). Conclusion The animal model of obstructive hydrocephalus in rat can be successfully established by injecting lateral ventricle astrocytes. Key words: Hydrocephalus; Rats; Models, animal; Astrocytes

Can sonographic measurement of optic nerve sheath diameter be used to detect raised intracranial pressure in patients with tuberculous meningitis? A prospective observational study.

CNS Tuberculosis can manifest as meningitis, arachnoiditis and a tuberculoma. The rupture of a tubercle into the subarachnoid space leads to Tuberculosis Meningitis (TBME); the resulting hypersensitivity reaction can lead to an elevation of the intracranial pressure and hydrocephalus. While bedside optic nerve sheath diameter (ONSD) ultrasonography (USG) can be a sensitive screening test for elevated intracranial pressure in adult head injury, little is known regarding ONSD measurements in Tuberculosis Meningitis.Objectives:The aim of this study was to determine whether patients with TBME had dilation of the optic nerve sheath, as detected by ocular USG performed in the emergency department (ED).Materials and Methods:We conducted a prospective, observational study on adult ED patients with suspected TBME. Patients underwent USG measurements of the optic nerve followed by MRI. The ONSD was measured 3 mm behind the globe in each eye. MRI evidence of basilar meningeal enhancement and any degree of hydrocephalus was suggestive of TBME. Those patients without evidence of hydrocephalus subsequently underwent a lumbar puncture to confirm the diagnosis. Exclusion criteria were age less than 18 and obvious ocular pathology. In total, the optic nerve sheath diameters of 25 adults with confirmed TBME were measured. These measurements were compared with 120 control patients.Results:The upper limit of normal ONSD was 4.37 mm in control group. Those patients with TBME had a mean ONSD of 5.81 mm (SD 0.42). These results confirm that patients with tuberculosis meningitis have an ONSD in excess of the control data (P < 0.001).Conclusion:The evaluation of the ONSD is a simple non-invasive and potentially useful tool in the assessment of adults suspected of having TBME.

Intraventricular ganglioglioma prognosis and hydrocephalus: The largest case series and systematic literature review

Ganglioglioma is mostly found in cerebral parenchymal, and it is rarely located in the ventricular system. To date, ganglioglioma within the ventricular system has been reported in only 10 cases. Its prognosis and relationship with complicating hydrocephalus are unclear. A total of 7 cases with intraventricular ganglioglioma diagnosed by the surgical pathology examination from June 2004 to April 2011 in our center were retrospectively analyzed. The clinical data were collected from the clinical medical records, and the tumor site, size and basement of tumor were analyzed. Follow up was performed to obtain the clinical outcomes. The 7 cases included 5 males and 2 females, with disease onset at 23.6 ± 14.9 years old. Epilepsy as the initial symptom was observed in 1 case. Reduced hearing, dizziness and weakness of both lower limbs were found in 1 case. Intracranial hypertension were detected in 5 cases, including 1 case complicating by decreased visual acuity. Tumors were located in the lateral ventricle in 5 cases, while 2 cases in the third ventricle. Hydrocephalus was observed in 5 cases, including 2 cases with severe hydrocephalus, and both underwent ventriculoperitoneal shunting. Total resection of tumors was performed in 5 cases, and 2 cases underwent gross total resection. The mean duration of follow-up was 28.7 months (8-90 months). Intracranial hypertension in all cases disappeared. Even radiotherapy post-surgery, one case with GTR relapsed 1 year later. However, the other 6 cases didn't relapse. Ganglioglioma in ventricular system is extremely rare, mainly with the symptoms of intracranial hypertension or seizure. The degree of hydrocephalus is closely related to the site of tumor's basement. The prognosis is good after total resection. The patients with GTR should be followed-up.

Comparative characteristic of neuropsychological disorders and morphological brain changes in elderly men and women with chronic brain ischemia

52 elderly women and 36 matched by age and education men with initiatory stages of chronic brain ischemia (CBI) have been studied using comparative investigation of neuropsychological and neuroimaging findings. The results revealed that the clock-drawing test was made by men with CBI better than by women that surpassed men by the indicators of attention and memory. The magnetic resonance imaging has shown that elderly men with CBI were characterized by more severe degree of hydrocephalus in comparison with women that surpassed men by the average rate of periventricular leucoaraiosis. The interrelations between the severity of cognitive disorders and the degree of hydrocephalus have been revealed in men with CBI as against women.

Presurgical neuropsychological and behavioral evaluation of children with posterior fossa tumors

Brain tumors are associated with behavioral and neuropsychological effects. Most available data are focused on the posttreatment neurological and cognitive deficits of these patients. The aim of the present study was to investigate the pretreatment neuropsychological and behavioral impairment in children with posterior fossa tumors. The authors studied 24 children with posterior fossa tumors who were between 4 and 15 years of age, and who were surgically treated at the authors' institute. During the period prior to the tumor excision, neuropsychological and behavioral assessments were performed. A control group of age-matched children was also studied. The children's executive functions were assessed using the short form of the Wechsler Intelligence Scale for Children (WISC). For the assessment of visuospatial functions, spatial memory, and visuomotor integration skills, the Bender-Gestalt Test (BGT) was used. For assessment of the visual perception and visual memory, the authors used the Benton Visual Retention Test (BVRT). Furthermore, parents or caregivers completed the Child Behavior Checklist (CBCL). The WISC revealed no significant difference between patients and the control group. The CBCL revealed significant somatic concerns compared with the measure's norms. Furthermore, the patients differed in aggressiveness, somatic concerns, anxiety symptoms, internalizing of problems, and total problems. In the BGT and the BVRT results, no significant difference was observed between patients and the control group. Furthermore, no significant correlation was found between neuropsychological scores and sex, age at diagnosis, histological diagnosis, presence of hydrocephalus, degree of hydrocephalus, tumor size, and tumor location. Children with posterior fossa tumors suffer more frequently from somatic concerns, aggressiveness, anxiety, and internalizing disorders compared with controls. No difference was found with respect to intelligence scores.

Variability among pediatric neurosurgeons in the threshold for ventricular shunting in asymptomatic children with hydrocephalus

The thresholds for shunting CSF in children with asymptomatic hydrocephalus are unclear; there are neither guidelines nor sufficient research to determine what degree of hydrocephalus should be treated. The authors hypothesize that 1) pediatric neurosurgeons currently have high thresholds for recommending treatment for these children, but 2) there is significant variability among these treatment thresholds. The authors surveyed attendees of the Joint Pediatric Neurosurgery Section meeting in Spokane, Washington, in December 2008, regarding their treatment thresholds for 22 clinical scenarios. Each participant was provided an illustration of 5 imaging studies (3 slices each) showing progressively larger ventricles. For each scenario, respondents were asked to indicate the minimum ventricular size they would treat, if any. Responses were quantified from 1 to 6 from smaller to larger, with 6 being no treatment, and a mean theoretical treated ventricular size (MTTVS) was calculated for each scenario. Respondents were relatively conservative in recommending treatment, with MTTVSs of 3.7-6.0; in 13 scenarios, the MTTVS was greater than 5.0 (larger than the largest presented ventricular size). For scenarios in which a mean frontooccipital ratio could be calculated, the value ranged from 0.55 to 0.67 (moderate to severe hydrocephalus). Although there were clear majority responses for each scenario, there was also significant variability. There were no patterns of association with the respondent's age, training, board certification, or type or location of practice. This study demonstrates that pediatric neurosurgeons' thresholds for treating asymptomatic children with hydrocephalus are generally high, but there is also significant variability.

Unusual central nervous system tuberculosis debut in children: stroke

Dear Editor: We have read the interesting report by du Plessis et al. on unusual forms of spinal tuberculosis [1]. The prognosis of central nervous system (CNS) tuberculosis depends on both early diagnosis and treatment. An early diagnosis continues to be a challenge because of the usual insidious debut, coming on gradually or almost imperceptibly [2–4]. We report a 5-year-old girl, pertaining to a low socioeconomic level, who was admitted because of sudden left-sided weakness making walking impossible. No other clinical symptom or sign was present. Examination was unremarkable apart from left hemiparesis; funduscopic examination was normal. An early CT scan showed right large-vessel arterial ischemic stroke together with a combination of basilar meningeal enhancement and a mild degree of hydrocephalus [ventricular size index (VSI) >30%] without parenchymal signs different from infarct. Ventricular enlargement on CT scan was calculated by VSI as the relation between bifrontal diameter over the frontal horn diameter. Pressure and biochemical and microbiological features, as well as a smear of CSF, were all irrelevant. CSF antigen tests were negative for neisseria meningococcus type B, streptococcus pneumonia, and hemophilus influenzae. Chest X-ray was normal. A later brain magnetic resonance image with diffusion weighted image and apparent diffusion coefficient disclosed any tuberculous parenchymal lesion but showed a T2-weighted image of the right middle cerebral artery infarct maximally involving the cortex. Quadruple antituberculosis therapy with isoniazid, rifampicin, ethambutol, and pyrazinamide were started. Steroids were used as adjunctive therapy for 3 weeks and then gradually tapered over a period of 7 days. The patient had not received BCG. A Mantoux test (dilution 10 units of purified protein derivative) was negative after 48 and 72 h, but the diagnosis was bacteriologically confirmed by detection of Mycobacterium tuberculosis in the CSF using the polymerase chain reaction (PCR). PCR for herpes virus, varicella-zoster virus, cytomegalovirus, adenovirus, enterovirus, parvovirus, coxsackievirus, and influenza virus were all negative. At our institution, a Mantoux response of >10 mm is considered positive for children without BCG. The case was notified and contact tracing was performed, with a possible source for her illness being discovered. CSF culture also confirmed the diagnosis, and the organism was fully sensitive to the above treatment. Other additional investigations were all normal: mycoplasmal, cryptococcus, clamydia, routine hematologic and biochemical studies, lactate in both blood and CSF, immunoglobin G index, antinuclear and anti-DNA antibodies to native DNA, electroencephalography, electrocardiography, echocardiography, Doppler imaging of the carotid arteries and transcranial Doppler imaging, cocaine in urine, homocystinuria, hypercoaguable state, coagulopathy and hemoglobinopathy, sickle cell anemia, and drug-induced thrombosis. She continued ethambutol treatment for 2 months and will continue on rifampicin, pyrazinamide, and isoniazid for 12 months. After a follow-up of 4 months, mild left hemicorporal weakness persisted. No other clinical findings were present. This case illustrates several practical points relevant to facilitate both early diagnosis and treatment of atypical Childs Nerv Syst (2008) 24:539–540 DOI 10.1007/s00381-008-0582-z

Deliberate termination of life of newborns with spina bifida

De Jong is to be complimented on his scholarly and lucid discussion of the many ethical dilemmas accompanying the management of children with spina bifida. He focuses particularly on evolving issues pertaining to the ethical and legal standards of practice in the Netherlands, but many aspects are relevant in all cultures and countries. The complex treatment decisions faced by all health professions dealing with parents and relatives of children with myelodysplasia are acute. However, they are never as acute as for paediatric neurosurgeons who usually, together with the obstetrician and delivering paediatrician, are the first to make these life-affecting decisions. De Jong argues compellingly that the correct ethical choice is to operate on all children whatever the level of the myelomeningocoele, degree of hydrocephalus and neurological dysfunction. He rightfully refutes the ability of any medical professional to judge the present and to prognosticate on the future quality of life. He convincingly rejects the concept of active euthanasia on the basis of the unreliability of estimating pain and suffering in neonates. In a first-world setting, where medical and surgical resources are available to all, I think to deny patients the right to life clearly violates their human rights . Especially since, as de Jong points out, virtually all our clinical methods of predicting outcome are unreliable. Emotional issues of religion and culture, both of professionals and parents, make objective decision-making impossible. Therefore, in countries where resources and access are available, the ethically defendable approach is to close the myelomeningocoele at birth and treat these patients actively in all regards. As has been argued elsewhere, it would be inconceivable not to treat a young teenager who sustained an acute spinal cord injury. Traumatic paraplegics have the same concomitant urological and neurological dysfunction asmyelomeningocoele children, but the same ethical debate surrounding the management of these patients does not arise. De Jong also points out that the medical professional’s attitude toward the quality of life of patients with myelomeningocele may have an undue influence on parents’ decision regarding the termination of pregnancy. Since prenatal imaging and ultrasound have improved, such decisions have become more frequent. The effect of overly pessimistic perceptions by obstetricians, paediatricians and paediatric neurosurgeons on such decisions should therefore be kept in mind. It clearly behoves all who deal with these children to be sufficiently informed about all aspects of myelodysplasia as the literature on the subject is huge. Parents whose child has been prenatally diagnosed with a myelomeningocoele should be objectively, not subjectively, informed. Where appropriate, they should be placed in contact with the many resource centres and support groups available. Only then can consent for termination of pregnancy be considered to be truly “informed”. Differing ethical dilemmas arise in countries where not enough resources are available to patients both in hospital and at home; where it is not possible to have urological follow-up or shunt assessments; where there is no access to wheelchairs or intermittent catheterisation ,which is often the case in rural settings. Children born into poor socioeconomic environments are commonly one of many siblings. Myelomeningocoele children often then ”usurp” all the meagre resources of the household so that the care of Childs Nerv Syst (2008) 24:55–56 DOI 10.1007/s00381-007-0489-0

Hidrocefalia derivada na infância: um estudo clínico-epidemiológico de 243 observações consecutivas

This study aimed to evaluate the epidemiological and clinical data of 243 children with shunted hydrocephalus, followed-up during 1 to 27 years, as well as to identify the risk factors for psychomotor disturbances, epilepsy and death. Postoperative meningitis and mechanical problems were the most frequent complications (22.3% and 30.7%, respectively). There was an average of 1.47 shunt per patient. Severe motor disturbances occurred in 34.3%, cognitive deficits in 58.5% and epilepsy in 43.6%. Motor disturbances correlated positively with the degree of hydrocephalus. The majority of patients with hydrocephalus due to meningitis presented cognitive deficits and a significant incidence of epilepsy. Death occurred in 52 patients and there was a higher percentage of brain tumours in this group, as well as the replacement of the first shunt device due to precocious postoperative meningitis.

Posttraumatic epilepsy: neuroradiologic and neuropsychological assessment of long-term outcome.

We sought to detect the incidence and the risk factors of posttraumatic epilepsy (PTE) in rehabilitation patients; to define the influence of PTE for late clinical and functional outcome; and to assess the cognitive and behavioral features of the patients with PTE. Patients were examined with (a) cognitive and behavioral examinations, which included a clinical interview and psychometric tests performed by an expert clinical psychologist; (b) single-photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI); and (c) functional evaluation including the Glasgow Outcome Scale (GOS) and the Functional Independence Measure (FIM). Of the 143 patients examined in this study, in 27 (19%), seizures developed after a mean time from trauma of 11.9 +/- 8.6 months. The occurrence of PTE was significantly correlated with the hypoperfusion in temporal lobes (p < 0.004), the degree of hydrocephalus (p < 0.04), the evidence of intracerebral hematoma (p < 0.01), and operative brain injury (p < 0.001). Patients with epilepsy showed a significantly higher incidence of personality disorders than did patients without epilepsy. The uninhibited behavior, irritability, and agitated and aggressive behavior were significantly more frequent and severe in PTE patients. The psychometric tests intended to explore memory, language, intelligence, attention, and spatial cognition did not show any significant difference between those with and without epilepsy. PTE also was significantly correlated with a worse functional outcome 1 year after the trauma. The degrees of hydrocephalus and of hypoperfusion in the temporal lobes are significant risk factors for late PTE. Another main finding of our study is the absence of influence of epilepsy on cognitive disorders; its influence on neurobehavioral disorders and functional outcome is discussed.