Split cord malformation types I and II: a personal series of 131 patients

Purpose

A personal series of 131 patients with split cord malformation (SCM) operated on is presented.

Methods

Age, gender, symptoms and signs, radiological and operative findings, complications, associated anomalies, outcome, and pathological specimens were analyzed.

Results

There were 88 girls (73 %) and 43 boys (27 %). The female predominance was slightly more remarkable in type I SCMs than in type II SCMs. The presenting symptoms can be summarized as skin lesions, spina bifida aperta, scoliosis or kyphoscoliosis, sphincter disturbance, foot deformities and weakness, and/or atrophy in the lower extremities. The ages of patients with neurological deficits and orthopedic deformities were significantly older than those without deficits (P?=?0.030). The duration of symptoms was longer in the patients with neurological deficits and orthopedic deformities than that in those without deficits (P?=?0.00095). In six patients, composite SCMs were present. Only one patient with a type II SCM did not have an associated spinal cord lesion. A type I SCM was more frequently encountered in patients with spina bifida (P?<?0.0005). Transient postoperative complications were seen in 29 patients (22 %). There was no permanent complication. Retethered cord syndrome developed in five patients with a type I SCM.

Conclusions

The risk of neurological and orthopedic deficits increases with the age of the patient. The risk of permanent deficit after surgery is very low. The whole spine must be examined for additional lesions. All patients should be surgically treated when diagnosed, especially before the development of orthopedic and neurological manifestations, and all associated lesions should also be treated at the same session.     

A comprehensive analysis of early outcomes and complication rates after 769 craniotomies in pediatric patients

Purpose

Perioperative complications following craniotomy in pediatric neurosurgery have received little attention. We analyzed perioperative complications and early outcomes following craniotomy in a large cohort of pediatric patients.

Methods

A retrospective chart review identified 769 operations (27 % epilepsy surgery, 26 % trauma, 21 % tumor, 7 % vascular, 4 % infections, 14 % other, and 88 % supratentorial) in 641 patients <16 years (mean age 8.5 years). We recorded all perioperative complications and functional outcomes 30 days after surgery.

Results

Excluding epilepsy surgery cases, 17.5 % patients had emergency surgery. There were 38 new major neurological deficits (5.0 %; excluding deficits incurred as part of the surgical strategy). New neurological deficits occurred more frequently following operations for brain tumors, when compared to other surgeries (P?<?0.001), and after surgery for infratentorial lesions (P?<?0.001). Local complications occurred in 3.9 %, systemic complications in 2.5 % of patients. Ventricular shunting or endoscopic ventriculostomy was necessary in 87 patients (11.3 %). Surgical mortality was 2.0 % (including moribund patients after trauma or vascular incidence). Preoperative Karnofsky Performance Index (KPI) and the incurrence of new neurological deficits proved the most powerful predictors of functional outcome. Emergency surgery or repeat craniotomies were not correlated with increased rates of local complications.

Conclusions

Craniotomies for pediatric patients carry a low morbidity and mortality. Systemic complications seem to occur less often in the pediatric than in the adult population. Good surgical outcomes require a proper balance between local pediatric neurosurgical care for emergency cases and centralized treatment of more difficult cases.     

Neurological morbidity of surgical resection of pediatric cerebellar astrocytomas

Background

Review of children with low-grade cerebellar astrocytoma (LGCA) prior to 1992 showed a 98 % rate of gross total resection (GTR) but a concerning incidence of permanent neurological dysfunction. The purpose of this study was to determine the rate of GTR of LGCA since 1992 and frequency of neurologic injury.

Methods

Retrospective review of children with LGCA was performed. CT/MR scans were rereviewed to assess extent of resection. Primary outcomes included incidence of GTR and incidence of permanent new neurological deficits. Other outcomes included late effects severity score (LESS), Bloom score for functional status, and educational assessment.

Results

Of 50 LGCA, GTR was achieved in 38 (76 %) compared to 43 of 44 (98 %) prior to 1992 (p?<?0.004). Permanent new neurologic deficits from surgery occurred in 16 % compared to 18 % in the prior era (p?=?0.61). For 35 patients operated on by the 2 surgeons in the prior study, 74 % had GTR, with permanent neurological deficits in 8.6 %. At latest follow-up, all patients were alive, 16 % with residual tumor. LESS was two or less (mild or no deficit) in 94 %. Bloom score was one or two (no or mild disability) in 90 %. Eighty-six percent attended normal school.

Conclusions

Less aggressive resection of LGCA in children may reduce postoperative neurologic deficits in the hands of the same surgeons as in the prior study but not overall at our institution. The good long-term outcomes suggest that it may be appropriate to do incomplete resection rather than risk additional neurological deficit.     

The role of ventriculoperitoneal shunt placement in patients of tubercular meningitis with hydrocephalus in poor neurological grade: a prospective study in the pediatric population and review of literature

Purpose

There is still no standard protocol for management of patients of tubercular meningitis (TBM) with hydrocephalus in poor neurological grade. In general, a trial of external ventricular drain (EVD) is an accepted method of treatment to decide whether a particular patient will benefit from shunt surgery. However, recent studies suggest that ventriculoperitoneal (VP) shunt may be undertaken without the trial of an EVD. Our study prospectively evaluates the role of direct VP shunt placement in poor grade patients of TBM with hydrocephalus.

Methods

Twenty-six consecutive pediatric patients of TBM with hydrocephalus in Palur grades III and IV underwent direct VP shunt placement, without prior placement of EVD. Outcome was assessed at the end of 3 months using Glasgow Outcome Score.

Results

The mean age of patients was 3.3 years (range, 4 months to 11 years). Twenty-one (80.8 %) patients were in grade III and five (19.2 %) were in grade IV. Good outcome and mortality in grade IV patients was 20 % (1/5) and 60 % (3/5) respectively; whereas in grade III patients, it was 71.4 % (15/21) and 9.5 % (2/21), respectively. Thirteen patients presented with focal neurological deficit at admission, which persisted in only three patients at 3 months follow up. VP shunt-related complications were observed in six (23.5 %) patients

Conclusions

Despite poor grade at admission, 71.4 % patients in grade III and 20 % patients in grade IV had a good outcome at 3 months follow-up. Direct VP shunt placement is a safe and effective option even in poor grade patients of TBM with hydrocephalus, with a low complication rate.     

Intervertebral disc calcification with neurological symptoms in children: report of conservative treatment in two cases

Purpose

Intervertebral calcifications are rare in the paediatric population. Two cases of children with symptomatic intervertebral calcifications responsible for spinal cord compression and neurological compromise are presented.

Methods

The data of two children treated conservatively for a symptomatic intervertebral calcification responsible for spinal cord compression and neurological compromise were retrospectively reviewed.

Results

Frontal and lateral radiographs are usually sufficient to determine the presence and extent of the calcified cervical disc protrusion. Conservative treatment with antalgics and bracing was applied in both cases. The two patients were completely free of symptoms 4 weeks after initial treatment. Magnetic resonance imaging screening showed a complete vertebral canal clearance at final follow-up.

Conclusion

Despite the lack of significance due to the small number of patients, conservative treatment should be considered in children with moderate neurological symptoms due to calcified disc protrusion.     

Delayed Decompressive Craniectomy Improves the Long-term Outcomes in Hypertensive Rats with Space-occupying Cerebral Infarction

Background and Purpose

No experimental data has been published on the long-term effects of decompressive craniotomy in hypertensive rats with space-occupying cerebral infarction. The aim of the present study was to investigate the efficacy of decompressive craniectomy in a middle cerebral artery occlusion (MCAO) model of hypertensive rats in a prolonged period.

Methods

Totally 92 stroke-prone renovascular hypertensive rats (RHRSP) were subjected to left MCAO by an endovascular occlusion technique. The decompressive craniectomy was performed on 26 RHRSP at 1 and 24 h after MCAO, respectively. Infarct volume, neurological performance, and mortality were evaluated at 1, 2, 4, and 8 weeks after MCAO.

Results

The mortality was reduced from 52.5% in controls to 7.7% and 23.1% in the rats underwent craniectomy at 1 and 24 h after MCAO, respectively (P < 0.05, respectively). All of the treated rats presented smaller infarct volume from 1 week to 8 weeks and better neurological performance at 4–8 weeks after MCAO compared to the controls (P < 0.05, respectively). The craniectomy at early stage was more effective than that at late stage in reducing infarct volume and improving neurological performances at 1 and 2 weeks (P < 0.05, respectively). However, there was no significant difference in infarct volume and neurological scores between the treated groups of rats at 4 and 8 weeks after MCAO (P > 0.05).

Conclusions

Although the early craniectomy is more effective than delayed craniectomy in improving short-term outcome, the latter has the similar beneficial effects as early craniectomy on long-term outcome in hypertensive rats with space-occupying cerebral infarction.     

Gamma Knife radiosurgery for arteriovenous malformations in pediatric patients

Objective

The authors present the results of Gamma Knife stereotactic radiosurgery performed in a series of children with arteriovenous malformations (AVMs).

Methods

Between June 2005 and January 2014, 75 patients 18 years old or younger received Gamma Knife radiosurgery for AVMs. Of these, 58 patients were eligible for further analysis. The median age of the population was 12 years; 41 % presented with hemorrhage, 34 % with neurological insult, and 24 % patients were diagnosed incidentally. The median AVM volume was 3.5 cm³. The median radiosurgery-based AVM score (RSBAVMS) was 0.86. The median follow-up period was 32 months.

Results

Single session Gamma Knife radiosurgery resulted in complete AVM obliteration in 40 (68.9 %) patients. There were 35 (60.3 %) excellent outcome (complete obliteration with no new deficits) in this series. During the follow-up period, nine (15.51 %) patients experienced new deficits and three (5.1 %) patients experienced intracranial hemorrhage. The annual rate of developing new deficits and hemorrhage was calculated as 5.45 and 1.8 %, respectively. Volume, gender, RSBAVMS, and nidus type factor were factors associated with excellent outcome.

Conclusions

Radiosurgery was successful in majority of patients with minimal morbidity. Gamma Knife radiosurgery for AVMs can be a safe and successful method in pediatric patients.     

Postoperative Anticoagulation in Patients with Mechanical Heart Valves Following Surgical Treatment of Subdural Hematomas

Background

Thromboembolic events and anticoagulation-associated bleeding events represent frequent complications following cardiac mechanical valve replacement. Management guidelines regarding the timing for resuming anticoagulation therapy following a surgically treated subdural hematoma (SDH) in patients with mechanical valves remains to be determined.

Objective

To determine optimal anticoagulation management in patients with mechanical heart valves following treatment of SDH.

Methods

Outcomes were retrospectively reviewed for 12 patients on anticoagulation therapy for thromboembolic prophylaxis for mechanical cardiac valves who underwent surgical intervention for a SDH at the Johns Hopkins Hospital between 1995 and 2010.

Results

The mean age at admission was 71 years. All patients had St. Jude’s mechanical heart valves and were receiving anticoagulation therapy. All patients had their anticoagulation reversed with vitamin K and fresh frozen plasma and underwent surgical evacuation. Anticoagulation was withheld for a mean of 14 days upon admission and a mean of 9 days postoperatively. The average length of stay was 19 days. No deaths or thromboembolic events occurred during the hospitalization. Average follow-up time was 50 months, during which two patients had a recurrent SDH. No other associated morbidities occurred during follow-up.

Conclusion

Interruptions in anticoagulation therapy for up to 3 weeks pose minimal thromboembolic risk in patients with mechanical heart valves. Close follow-up after discharge is highly recommended, as recurrent hemorrhages can occur several weeks after the resumption of anticoagulation.     

Selective Digestive Tract Decontamination Decreases Time on Ventilator in Guillain–Barré Syndrome

Background

Ventilator-associated pneumonia (VAP) occurs in more than half of mechanically ventilated patients with Guillain–Barré syndrome (GBS) and is associated with prolonged mechanical ventilation (MV). We investigated the impact of selective decontamination of the digestive tract (SDD), an intervention that reduces hospital acquired infections in ICU patients, on duration of MV in GBS and neurological outcome at 6 months.

Methods

We performed a retrospective study in mechanically ventilated GBS patients in the Netherlands. We compared patients treated with and without SDD. Main outcomes were duration of MV and the ability to walk independently at 6 months. Statistical comparison was done with logistic and ordinal regression analyses.

Results

We included 124 GBS patients on MV at 2 weeks after first symptoms (SDD, n = 54 and non-SDD, n = 70). The median duration of MV without SDD was 42 days (interquartile range, IQR 25–77 days) versus 29 days with SDD (IQR 17–45 days). Median duration of MV for all included patients was 35 days. The adjusted odds ratio (OR) for duration of MV > 35 days in the SDD versus the non-SDD cohort was 0.37 (95% CI 0.17–0.77). SDD did not affect neurological recovery after 6 months from first symptoms. VAP occurred in 12% (95% CI 2–22%) in the SDD cohort and in 47% (95% CI 35–59%) in the non-SDD cohort.

Conclusions

SDD in mechanically ventilated GBS patients reduced the time on the ventilator, probably by preventing VAP, but did not affect neurological recovery after 6 months.     

Torticollis as a first sign of posterior fossa and cervical spinal cord tumors in children

Background

Torticollis, despite being well-known neurological manifestation, is often underestimated as a first symptom of the abnormalities of posterior cranial cavity and cervical spinal cord.

Objectives

The purpose of this study is to analyze the occurrence of acquired torticollis in children as a herald sign of the tumors of the cervical spinal cord or of the posterior fossa.

Methods

Clinical records of 54 cases treated for the tumor of the cervical spinal cord or posterior fossa (including congenital ones) were retrospectively reviewed. The following data were calculated: the occurrence of the torticollis as a first sign of tumors, the duration time from the onset of the symptoms to diagnosis, the concurrence of other pathological symptoms, and the diminishing of symptoms of the torticollis following treatment.

Results

In 12/54 (22.2 %) torticollis was first sign of central nervous system tumor and in all of them preceded other neurological symptoms. The time from the onset of torticollis to establishing diagnosis ranged from 2 to 52 weeks (9.6 weeks on average). Eleven of twelve patients were treated surgically—in 10 of them, torticollis disappeared in the postoperative course.

Conclusions

Torticollis may be a herald sign of the tumor of the cervical spinal cord or the posterior fossa. Those pathologies should be considered in the differential diagnosis of the torticollis, particularly if accompanied by other symptoms of the focal pathology of central nervous system. Awareness of this fact may shorten the time to establish the proper diagnosis. Torticollis necessitates exclusion of the posterior fossa and spinal cord tumor.     

Neurological complication of non Hodgkin lymphoma in childhood: experience from a single center in Turkey

Purpose

Lymphomas are the third most common childhood malignant disease after leukemia and central nervous system (CNS) tumors. Early diagnosis of these complications will reduce mortality and morbidity. In this study we aimed to review the neurological complications of childhood non Hodgkin Lymphoma (NHL).

Patients and methods

Forty four children with NHL between 2006 and 2012 were investigated retrospectively and 14 cases with neurological complications were identified.

Results

The most common symptom was alteration of the consciousness (10 patients, 71.4 %) followed by convulsion (5 patients, 35.7 %), and hallucination (4 patients, 28.5 %); headache, eye pain, neurogenic bladder, speech disability and facial paralysis, and hemiplegia, were less common and each of them was seen in 1 (7.1 %) of the patients. The neurological complications were mostly seen in children with precursor T lymphoblastic lymphoma followed by anaplastic large cell lymphoma. The complications were secondary to medications (Eight patients) infection (two patients); CNS relapse (two patients); or CNS involvement of the primary disease (two patients). Chemotherapy-related neurologic complications were secondary to intrathecal methotrexate, l-asparaginase, vincristine, and ifosfamide

Conclusion

Advanced disease and PTLL subtype can be suggested as predictors of neurological complication. The survival rates of neurological complications are fairly good unless it is secondary to involvement of the primary disease. In patients with drug-induced neurological complications, the treatment can be safely re-administered after controlling the neurological complications. Therefore, clinicians managing children with NHL must be informative about neurological complications.     

Intra-Arterial Thrombolysis Within Three Hours of Stroke Onset in Middle Cerebral Artery Strokes

Background and Purpose

The Prolyse in Acute Cerebral Thromboembolism II (PROACT II) trial showed improved outcomes in patients with proximal middle cerebral artery (MCA) occlusions treated with intra-arterial (IA) thrombolysis within 6 h of stroke onset. We analyzed outcomes of patients with proximal MCA occlusions treated within 3 h of stroke onset in order to determine the influence of time-to-treatment on clinical and angiographic outcomes in patients receiving IA thrombolysis.

Methods

Thirty-five patients from three academic institutions with angiographically demonstrated proximal MCA occlusions were treated with IA thrombolytics within 3 h of stroke onset. Outcome measures included outcomes at 30–90 day follow-up, recanalization rates, incidence of symptomatic intracranial hemorrhage, and mortality in the first 90 days. The endpoints were compared to the IA treated and control groups of the PROACT II trial.

Results

The median admission National Institutes of Health Stroke Scale (NIHSS) score was 16 (range 4–24). The mean time to initiation of treatment was 106 min (range 10–180 min). Sixty-six percent of patients treated, had a modified Rankin Scale (mRS) score of 2 or less at 1–3 month follow-up compared to 40% in the PROACT II trial. The recanalization rate was 77% (versus 66% in PROACT II). The symptomatic intracranial hemorrhage rate was 11% (versus 10% in PROACT II) and the mortality rate was 23% (versus 25% in PROACT II).

Conclusion

Time-to-treatment is just as important in IA thrombolysis as it is in IV thrombolysis, both for improving clinical outcomes and recanalization rates as well.     

Value of Transcranial Doppler,Perfusion-CT and Neurological Evaluation to Forecast Secondary Ischemia after Aneurysmal SAH

Introduction

This study was conducted to prospectively evaluate the diagnostic value of detailed neurological evaluation, transcranial Doppler sonography (TCD) and Perfusion-CT (PCT) to predict delayed vasospasm (DV) and delayed cerebral infarction (DCI) within the following 3 days in patients with aneurysmal subarachnoid hemorrhage (SAH).

Methods

A total of 61 patients with aneurysmal SAH were included in the study. All patients were amenable for neurological evaluation throughout the critical phase to develop secondary ischemia after SAH. The neurological status was assessed three times a day according to a detailed examination protocol. Mean flow velocities (MFV) in intracranial vessel trunks were measured daily by TCD. Native CT and PCT were routinely acquired at 3-day intervals and, in addition, whenever it was thought to be of diagnostic relevance. The predictive values of abnormal PCT and accelerations in TCD (MFV > 140 cm/s) to detect angiographic DV and DCI within the following 2 days were calculated and compared to the predictive value of delayed ischemic neurological deficits (DIND).

Results

The accuracy of TCD and PCT to predict DV or DCI was 0.65 and 0.63, respectively. In comparison, DIND predicted DV or DCI with an accuracy of 0.96. Pathological PCT findings had a higher sensitivity (0.93) and negative predictive value (0.98) than TCD (0.81 and 0.96).

Conclusion

Neurological assessment at close intervals is the most accurate parameter to detect DV and DCI in the following 3 days. However, DIND may not be reversible. The routine acquisition of PCT in addition to daily TCD examinations seems reasonable, particularly in patients who are not amenable to a detailed neurological examination since it has a higher sensitivity and negative predictive value than TCD and leaves a lower number of undetected cases of vasospasm and infarction.     

Rosiglitazone Attenuates Cerebral Vasospasm and Provides Neuroprotection in an Experimental Rat Model of Subarachnoid Hemorrhage

Background

Glutamate and oxidative stress play important roles after subarachnoid hemorrhage (SAH). The ability to modulate glutamate transporter 1 (GLT-1) and the antioxidative effect of rosiglitazone have been demonstrated. We investigated the neuroprotective effect of rosiglitazone after SAH.

Methods

SAH was induced by double blood injection. The rats were randomly divided into sham, SAH + vehicle, and SAH + rosiglitazone groups and treated with dimethyl sulfoxide, dimethyl sulfoxide, and 6 mg/kg of rosiglitazone, respectively, at 2 and 12 h after SAH induction and then daily for 6 days. Cerebrospinal fluid dialysates were collected 30 min before SAH induction and then daily for 7 days for glutamate measurement. Mortality, body weight, and neurological scores were also measured daily. On day 7 after SAH, the wall thickness and the perimeter of the basilar artery (BA), neuron variability, GLT-1 levels, glial fibrillary acidic protein (GFAP) expression and activity, and malondialdehyde, superoxide dismutase, and catalase activities were also evaluated.

Results

Rosiglitazone improved survival (relative risk = 0.325) and neurological functions and reduced neuronal degeneration (5.7 ± 0.8 vs. 10.0 ± 0.9; P < 0.001) compared with the SAH + vehicle group. Rosiglitazone also lowered glutamate levels by 43.5-fold and upregulated GLT-1 expression by 1.5-fold and astrocyte activity by 1.8-fold compared with the SAH + vehicle group. The increase in BA wall thickness was significantly attenuated by rosiglitazone, whereas the perimeter of the BA was increased. In addition, rosiglitazone abated the 1.9-fold increase in malondialdehyde levels and the 1.6-fold increase in catalase activity after SAH.

Conclusion

Rosiglitazone reduced SAH mortality, neurological deficits, body weight loss, GFAP loss, and cerebral vasospasm by preventing the neurotoxicity induced by glutamate and oxidative stress.     

Clinical features and long-term outcomes of intraspinal ependymomas in pediatric patients

Purpose

The aim of this study was to discuss the clinical manifestations, radiological features, treatment, and long-term outcomes of intraspinal ependymomas (Word Health Organization grade II) in pediatric patients.

Methods

The data of 15 pediatric patients who underwent microsurgery for intraspinal grade II ependymomas were retrospectively reviewed. Pre- and postoperative magnetic resonance imaging was performed in all patients. The diagnosis of grade II ependymomas was based on pathology. All the follow-up data were obtained during office visits.

Results

There were ten males and five females, with a mean age of 13.7?±?3.4 years. Four tumors were located in the cervical cord, six in the cervicothoracic cord, four in the thoracic cord, and one in the conus-cauda region, respectively. The most common symptom was motor deficits. Gross total resection (GTR) of the tumor was achieved in 12 cases, and subtotal resection (STR) was achieved in three cases. Regrowth of the residual tumor was observed in two STR cases during a mean follow-up period of 44.8 months. STR was performed again in one case due to clinical progression. At the last follow-up, 12 patients experienced an improvement in the neurological function and three patients maintained their preoperative status.

Conclusions

Pediatric intraspinal grade II ependymomas are amenable to surgical resection before neurological deficits deteriorate. GTR is the best treatment of choice, and the outcome is favorable. Due to uncertain therapeutic efficacy and possible radiation-induced toxicity, postoperative radiotherapy should be considered carefully for cases of STR.     

Outcome of Poor-Grade Subarachnoid Hemorrhage as Determined by Biomarkers of Glucose Cerebral Metabolism

Purpose

The aim of this study was to determine if the measurement of blood biomarkers of glucose cerebral metabolism, performed with retrograde jugular catheter, could predict the outcome of poor-grade aneurysmal subarachnoid hemorrhage (aSAH) patients.

Methods

This study was conducted in 68 poor-grade aSAH patients. A total of 4,024 blood samples obtained from jugular and radial catheters were analyzed for glucose, lactate, and oxygen content every 8 h for 10 ± 0.5 days. Metabolic ratio (MR) and lactate–oxygen index (LOI) were obtained by ratios using arterio-jugular differences. Functional outcome was evaluated at 12 months with the Glasgow Outcome Scale.

Results

Outcome was unfavorable in 40 patients. In this group of patients, the MR was significantly lower (p < 0.0001) and the LOI was significantly higher (p = 0.0001) than in the group with favorable outcome. The MR cutoff value, below which the patients are likely to have an unfavorable outcome, was determined to be 3.35. More interestingly, the data obtained in this study demonstrated that the patients achieving an unfavorable outcome were distinguished from those with a favorable outcome by having at least three events of MR inferior to 3.35 (sensitivity = 90 %, specificity = 82.1 %). Moreover, in patients who developed cerebral vasospasm, we observed a significant decrease in the MR.

Conclusion

Our data provide additional support to the view that the MR is a reliable marker for predicting the outcome of poor-grade aSAH patients. Prospective studies are needed to confirm its value in multimodal monitoring.     

Effect of Interferon-β on Neuroinflammation, Brain Injury and Neurological Outcome After Experimental Subarachnoid Hemorrhage

Introduction

Aneurysmal subarachnoid hemorrhage (SAH) has a poor outcome, particularly attributed to progressive injury after the initial incident. Several studies suggest a critical role for inflammation in lesion progression after SAH. Our goal was to test whether treatment with anti-inflammatory interferon-β, which has shown promise as a therapeutic agent in experimental ischaemic stroke, can protect the brain after SAH.

Methods

SAH was induced in adult male Wistar rats by puncturing the intracranial bifurcation of the right internal carotid artery. Treatment effects of daily interferon-β (n = 16) or vehicle (n = 14) injections were serially evaluated with multiparametric MRI and behavioral tests from day 0 to 7, in compliance with recent recommendations for pre-clinical drug testing. Outcome measures included neurological status, brain lesion volume, blood–brain barrier (BBB) leakage, and levels of inflammatory markers.

Results

In animals that survived up to 7 days post-SAH, we found no significant differences between vehicle- and interferon-β-treated animals with respect to final neurological score (14.3 ± 1.0 vs. 13.0 ± 2.2), brain lesion size on T₂-weighted MR images (59 ± 83 vs. 124 ± 99 mm³), BBB leakage (0.26 ± 0.05 vs. 0.22 ± 0.08 contrast-induced relative MR signal change), upregulation of brain RNA for cytokines, chemokines and cell adhesion molecules, and increased neutrophil activation.

Conclusions

In contrast to previously published findings in experimental ischemic stroke models, interferon-β has no clear efficacy to protect the brain after SAH. In line with recent highlighting of the significance of negative findings, our data currently do not recommend clinical testing of interferon-β to prevent neurological damage in SAH patients.     

Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases

Background

Cavernous malformations (CMs) of the central nervous system (CNS) are angiographically occult vascular lesions that affect approximately 0.5 % of the general population, and one quarter of all CMs occurs in children.

Methods

We retrospectively analyzed demographic, clinical, radiological, management, and follow-up data of 36 pediatric patients with CMs from a single institution.

Results

The mean age of the children at first presentation and at operation was 8.7 and 9.6 years, respectively. However, a bimodal age distribution was found with peak under 4 years and above 12 years. Seizure was the most common single presenting symptom (38.9 %), and 61.1 % of patients had at least one seizure before the admission. Focal neurological deficits (410.7 %), intracranial hypertension (27.8 %), and headache (2.8 %) were the other manifestations. Acute/subacute hemorrhage was evident at presentation in 63.9 %. The patients under 6 years of age were found to have significantly more giant cavernomas (69 vs 20 %; p?=?0.011), and more overt hemorrhages (81 vs 47 %; p?=?0.065) at diagnosis than those patients above 12 years. Surgery was performed in 31 patients (32 CMs), with 26 total and 6 incomplete resections. Mean follow-up duration was 6.9?±?4.1 years. Of all patients, 63.8 % had excellent and 30.5 % had good clinical outcomes, and also 90.9 % of the epileptic patients were seizure-free (Engel Class I) at the last follow-up.

Conclusions

Younger children tend to harbor larger CMs and present with hemorrhage more frequently than older ones. Microsurgical resection should be the treatment of choice in symptomatic and accessible CMs.     

Interfrontal encephalocele: a rare feature of forehead in hydrocephalic myelomeningocele patients. Clinical feature, probable mechanisms, and management

Objective

Myelomeningocele is a complex central nervous system malformation mostly associated with other neural and extraneural anomalies. A rare special feature of the forehead and skull was observed in myelomeningocele. Here, we present several patients with myelomeningocele, severe hydrocephalus, and interfrontal encephalocele accompanied by metopic suture widening and abnormally shaped frontal bones.

Methods

Five children with this feature were enrolled in this series. The age, sex, location of myelomeningocele sac, neurological deficits, hydrocephalus and history of shunt surgery, and follow-up period were evaluated.

Results

Patients were aged from 1 to 12 months (mean, 6 months). All patients were male. Most sacs were located in lumbar and lumbosacral areas. Neurological deficits varying from only sphincter problem to paraplegia were found in all patients. Hydrocephalus was found in all children that needed a shunt procedure. Asymptomatic Chiari malformation, interfrontal encephalocele, and corpus callosum agenesis were evident in five, five, and three patients, respectively.

Conclusion

Anterior fontanel anomalies known as interfrontal encephalocele associated with myelomeningocele have been reported before. The patients have an open metopic suture extending widely to the nasal radix producing hypertelorism accompanied by interfrontal herniation of frontal lobes. The associated hydrocephalus exaggerates the anomaly. Treatment of accompanying hydrocephalus is advised to decrease the severity of frontal lobe herniation. Some patients may need frontal bone reconstruction surgery to provide cosmetic correction at the place of the midline frontal bone defect.     

Pediatric brain tumors in Nigeria: clinical profile, management strategies, and outcome

Introduction

Although modern neuroimaging has facilitated early care of brain tumors in children worldwide, there are, however, few published reports on clinical profile, treatment, and outcome of brain tumors in children from our subregion.

Purpose

We aimed to retrospectively study the clinical profile and outcome of pediatric brain tumors in a tertiary referral center from a developing country.

Methods

Forty pediatric patients with histologically verified brain tumors managed by the authors over a 13-year period (May1994–April 2006) were studied. Patients' data from clinical, radiological, and pathology records were analyzed using the statistical package for social sciences version 16.

Results

The mean age was 9.75 years (range 1–15 years). Twenty-two males, 18 females. Common presenting symptoms were headaches (23 patients, 57.5 %) and seizures (15 patients, 37.5 %). Hyperreflexia (72.5 %) and focal motor deficits (62.5 %) were the most common neurologic signs. The mean interval from onset of symptoms to neurosurgical diagnosis was 13.4 months (95 % CI). All patients had tumor resection, while 11 (27.5 %) patients received adjuvant radiotherapy. Hydrocephalus occurred in 19 (47.5 %) patients and was associated with early presentation (X²?=?10.65, p?<?0.01). Low-grade astrocytoma (25 %) and medulloblastoma (25 %) were the most common tumors. Survival at 1 and 5 years were 56 and 47 %, respectively.

Conclusion

Focal motor signs and elevated intracranial pressure are the salient presenting features of brain tumors in children seen in Nigeria. Those of them with hydrocephalus are likely to present early. The outcome for pediatric brain tumors remains poor.