Signs Of Elevated Intracranial Pressure Research Articles

The Neurocritical Care Examination and Workup.

This article provides an overview of the evaluation of patients in neurocritical care settings and a structured approach to recognizing and localizing acute neurologic emergencies, performing a focused examination, and pursuing workup to identify critical findings requiring urgent management. After identifying and stabilizing imminent threats to survival, including respiratory and hemodynamic compromise, the initial differential diagnosis for patients in neurocritical care is built on a focused history and clinical examination, always keeping in mind critical "must-not-miss" pathologies. A key priority is to identify processes warranting time-sensitive therapeutic interventions, including signs of elevated intracranial pressure and herniation, acute neurovascular emergencies, clinical or subclinical seizures, infections of the central nervous system, spinal cord compression, and acute neuromuscular respiratory failure. Prompt neuroimaging to identify structural abnormalities should be obtained, complemented by laboratory findings to assess for underlying systemic causes. The indication for EEG and lumbar puncture should be considered early based on clinical suspicion. In neurocritical care, the initial evaluation is often fast paced, requiring assessment and management to happen in parallel. History, clinical examination, and workup should be obtained while considering therapeutic implications and the need for lifesaving interventions.

869 Early Signs of Elevated Intracranial Pressure (ICP) on Computed Tomography Correlate With Measured ICP in the Intensive Care Unit and Six-month Outcome Following Moderate to Severe TBI

INTRODUCTION: Identifying patients at risk for increased intracranial pressure (ICP) followingTBI via baseline computed tomography (CT) has not previously been validated in a prospective dataset. METHODS: Data were obtained from the Progesterone for Traumatic Brain Injury, Experimental Clinical Treatment (ProTECTIII) multicenter clinical trial. Baseline CT scans for 881 participants were individually reviewed by a blinded central neuroradiologist. Five signs of elevated ICP were measured (sulcal obliteration, lateral ventricle compression, 3rd ventricle compression, midline shift, and herniation). Associations between signs of increased ICP and outcomes (6-month functional outcome and mortality) were assessed. RESULTS: Sulcal obliteration (p = 0.029) and third ventricular compression (p = 0.039) were associated with elevated ICP. Univariate regression analyses indicated that increasing combinations of the five defined signs of elevated ICP were associated with mortality, poor functional outcome, and time to death. There was also an increased likelihood of mortality if patients required craniotomy (OR = 4.318, 95% Confidence Interval [1.330-16.030]) or hemicraniectomy (OR = 2.993 [1.109-8.482]). On multivariate regression analyses, hemorrhage location was associated with mortality (posterior fossa, OR = 3.208 [1.120-9.188] and basal ganglia, OR = 3.079 [1.178-8.077]). Volume of hemorrhage >30cc was also associated with increased mortality, OR = 3.702 [1.575-8.956]). The proportion of patient hours with sustained ICP = 20 mmHg, and maximum ICP = 20 mmHg, were also directly correlated with increased mortality (OR = 64.99 [7.731-635.51]; and OR = 1.025 [1.004-1.047]). Poor functional outcome was predicted by concurrent presence of all five radiographic signs of elevated ICP (OR = 4.44 [1.514-14.183]) and presence of frontal lobe (OR = 2.951 [1.265-7.067]), subarachnoid (OR = 2.231 [1.067-4.717]), or intraventricular (OR = 2.249 [1.159-4.508]) hemorrhage. Time to death was modulated by total patient days of elevated ICP = 20 mmHg (Effect Size = 3.424 [1.500, 5.439]) in the first two weeks of hospitalization. CONCLUSIONS: ICP-related imaging variables can be prognosticate for moderate-severe TBI.

Early Signs of Elevated Intracranial Pressure on Computed Tomography Correlate With Measured Intracranial Pressure in the Intensive Care Unit and Six-Month Outcome After Moderate to Severe Traumatic Brain Injury.

Traumatic brain injury (TBI) is a leading cause of death and disability in the United States. Early triage and treatment after TBI have been shown to improve outcome. Identifying patients at risk for increased intracranial pressure (ICP) via baseline computed tomography (CT) , however, has not been validated previously in a prospective dataset. We hypothesized that acute CT findings of elevated ICP, combined with direct ICP measurement, hold prognostic value in terms of six-month patient outcome after TBI. Data were obtained from the Progesterone for Traumatic Brain Injury, Experimental Clinical Treatment (ProTECTIII) multi-center clinical trial. Baseline CT scans for 881 participants were individually reviewed by a blinded central neuroradiologist. Five signs of elevated ICP were measured (sulcal obliteration, lateral ventricle compression, third ventricle compression, midline shift, and herniation). Associations between signs of increased ICP and outcomes (six-month functional outcome and death) were assessed. Secondary analyses of 354 patients with recorded ICP monitoring data available explored the relationships between hemorrhage phenotype/anatomic location, sustained ICP ≥20 mm Hg, and surgical intervention(s). Univariate and multi-variate logistic/linear regressions were performed; p < 0.05 is defined as statistically significant. Imaging characteristics associated with ICP in this cohort include sulcal obliteration (p = 0.029) and third ventricular compression (p = 0.039). Univariate regression analyses indicated that increasing combinations of the five defined signs of elevated ICP were associated with death, poor functional outcome, and time to death. There was also an increased likelihood of death if patients required craniotomy (odds ratio [OR] = 4.318, 95% confidence interval [1.330-16.030]) or hemicraniectomy (OR = 2.993 [1.109-8.482]). On multi-variate regression analyses, hemorrhage location was associated with death (posterior fossa, OR = 3.208 [1.120-9.188] and basal ganglia, OR = 3.079 [1.178-8.077]). Volume of hemorrhage >30 cc was also associated with increased death, OR = 3.702 [1.575-8.956]). The proportion of patient hours with sustained ICP ≥20 mm Hg, and maximum ICP ≥20 mm Hg were also directly correlated with increased death (OR = 6 4.99 [7.731-635.51]; and OR = 1.025 [1.004-1.047]), but not with functional outcome. Poor functional outcome was predicted by concurrent presence of all five radiographic signs of elevated ICP (OR = 4.44 [1.514-14.183]) and presence of frontal lobe (OR = 2.951 [1.265-7.067]), subarachnoid (OR = 2.231 [1.067-4.717]), or intraventricular (OR = 2.249 [1.159-4.508]) hemorrhage. Time to death was modulated by total patient days of elevated ICP ≥20 mm Hg (effect size = 3.424 [1.500, 5.439]) in the first two weeks of hospitalization. Sulcal obliteration and third ventricular compression, radiographic signs of elevated ICP, were significantly associated with measurements of ICP ≥20 mm Hg. These radiographic biomarkers were significantly associated with patient outcome. There is potential utility of ICP-related imaging variables in triage and prognostication for patients after moderate-severe TBI.

DECOMPRESSIVE CRANIOTOMY IN MANAGEMENT OF ACUTE CEREBRAL ISCHEMIA

Background: Ischemic stroke is a medical emergency and the most common affection of the central nervous system (CNS). This is the second-leading cause of death worldwide and the first causes of morbidity. Ischemic stroke correspond to 85% of all strokes with a mortality of 10-50%. Large space-occupying infarction accounts for 1-10% of all supratentorial infarction with signs of elevated intracranial pressure (ICP) and brain herniation usually in the second to the fifth day leading to a mortality rate of 53% to 89%. Objective: To evaluate the clinical and radiological outcome of decompressive surgery in the setting of cerebral infarctions, and to study its effect in reducing the overall mortality rates of this fatal condition. Patients and methods: This study is descriptive and analytic clinical trial study for evaluating the clinical and functional outcomes of surgical cases with massive cerebral infarction. Over one year, Between January 2018 and January 2019, at Dar Alshifa Hospital, and Al-Azhar University Hospitals in Cairo. Results: Twenty cases with cerebral infarctions were included in this. In our study, there was 12 males (60%) and 8 females (40%) male: female ratio 1.5:1.The age of the cases ranged from 37 to 77 years. The mean age was 57.65 years. Four cases were less than 50 years (20%), 6 cases between 50 and 60 years (30%), 8 cases between 60 and 70 years (40%), and 2 cases above 70 years (10%). Eighteen cases had middle cerebral artery (MCA) infarctions (90/%), while 2 cases had both MCA and anterior cerebral artery (ACA) infarctions. eleven cases were right sided while 9 cases were having left-sided infarctions. Conclusion: The preoperative clinical condition , higher GCS score at presentation were strongly related to a favorable clinical outcome .Some ICU related complications such as contra lateral infarction, hemorrhagic infarction, pulmonary embolism, pneumonia can worse the patient outcome .Other complication related to the surgery such as CSF leak, wound infection did not effect on patient prognosis. Factors like patients’ age, sex, site of infarction, and preoperative midline shift did not affect the final outcome. A study with larger sample size and longer follow-up is recommended for better confirmation of these facts.

Study of the effect of anaesthesia onthecanine ultrasonographic optic nerve sheath diameter.

To investigate the effects of anaesthetic duration and serial anaesthetic events on optic nerve sheath diameter in a population of dogs without intracranial disease using point-of-care ultrasonography. Client-owned dogs requiring advanced head imaging were prospectively enrolled. Exclusion criteria included signs of elevated intracranial pressure, glaucoma and optic nerve disease. Using a transpalpebral technique, two optic nerve sheath diameter measurements were recorded for each eye at three timepoints: following premedication, after induction within 7minutes and before discontinuing isoflurane. Mixed model analysis was used to characterise optic nerve sheath diameter behaviour and investigate the effects of anaesthetic duration, bodyweight and anaesthetic protocol, age and sex. Fourteen dogs of various ages, breeds and bodyweights were enrolled. A positive linear relationship was detected between body weight and optic nerve sheath diameter. In 12 of 14 dogs, the optic nerve sheath diameter increased from measurements taken after premedication when compared to measurements taken after induction within 7minutes. In a subset of patients, measurements subsequently decreased when anaesthetic duration exceeded 120 minutes. Age, side, sex, final body temperature, blood pressure and anaesthetic protocol did not significantly affect optic nerve sheath diameter. No significant association was noted between optic nerve sheath diameter and end-tidal carbon dioxide after induction and before discontinuing isoflurane. When using point-of-care ultrasound, a transient increase in optic nerve sheath diameter occurs between premedication and within 7 minutes following induction, regardless of bodyweight. This should be taken into consideration when serial monitoring is performed.

Craniofrontonasal dysplasia: hypertelorism correction in late presenting patients.

Craniofrontonasal dysplasia (CFND) is a rare congenital craniofacial syndrome characterized by single suture synostosis, hypertelorism, other clinical facial features, and abnormalities in the upper extremities. There are only a few studies in the applicable literature that address hypertelorism management for CFND patients and outcomes and complication rates. A retrospective study was performed on consecutive late presenting CFND patients referred to our hospital with substantially completed craniofacial skeleton growth, who underwent hypertelorism correction between 2007 and 2019 following intracranial pressure screening, and who received at least 1 year of follow-up care. None of the patients in this study underwent prior craniofacial surgery. Only those patients with a confirmed mutation of the EFNB1 gene were included in this study. All patients in this study underwent hypertelorism correction by facial bipartition or box osteotomy. A total of ten late presenting CFND patients (all female) were treated at our hospital during the study period. None of the patients presented signs of elevated intracranial pressure. The average patient age at hypertelorism correction was 13.4 ± 7.68 years of age. Major complications, defined as complications requiring a return to the operating room, were limited to infection of the frontal bone, which required partial bone removal, and cerebrospinal fluid (CSF) leak, which was completely resolved by insertion of a lumbar shunt for a 7-day period. The absence of elevated intracranial pressure enables hypertelorism correction in late presenting CFND patients via facial bipartition or box osteotomy without the need for additional operations that provide for cranial expansion.

Prediction of Postoperative Risk of Raised Intracranial Pressure After Spontaneous Skull Base Cerebrospinal Fluid Leak Repair.

A relationship between idiopathic intracranial hypertension and spontaneous skull base cerebrospinal fluid (CSF) leaks has been proposed, by which CSF leak decreases intracranial pressure (ICP) and masks the symptoms and signs of elevated ICP. These patients are at risk of developing papilledema, symptoms of elevated ICP, or a recurrent CSF leak after CSF leak repair. The objective of this study was to assess whether radiographic signs of raised ICP on preoperative magnetic resonance or computed venography (MRI or CTV) are predictors of postoperative papilledema, recurrence of CSF leak, or need for CSF shunt surgery. We performed a retrospective review of systematically collected demographics, fundus examination, and presurgical brain MRI and magnetic resonance venography/computed tomography venography (MRV/CTV) in patients seen at 1 institution between 2013 and 2019 with spontaneous skull base CSF leak repair. Patients were divided into 2 groups depending on whether they developed papilledema, recurrent CSF leak, or required CSF shunting (Group 1) or not (Group 2). Fifty-seven patients were included, among whom 19 were in Group 1. There was no difference in demographic characteristics or clinical features between patients in Group 1 and Group 2. Controlling for other imaging features, bilateral transverse venous sinus stenosis (TVSS) on preoperative imaging increased the odds of being in Group 1 by 4.2 times (95% confidence interval [CI], 1.04-21.2, P = 0.04), optic nerve tortuosity decreased the odds of being in Group 1 by 8.3 times (95% CI: 1.4-74.6, P = 0.02). Imaging of the intracranial venous system with MRV or CTV is warranted before repair of spontaneous CSF leak, as bilateral TVSS is an independent risk factor for postoperative papilledema, CSF leak recurrence, or need for a CSF shunting procedure.

Medulloblastoma in adults - reviewing the literature from a surgeon's point of view.

Medulloblastoma is a common primary brain tumor in children but it is a rare cancer in adult patients. We reviewed the literature, searching PubMed for articles on this rare tumor entity, with a focus on tumor biology, advanced neurosurgical opportunities for safe tumor resection, and multimodal treatment options. Adult medulloblastoma occurs at a rate of 0.6 per one million people per year. There is a slight disparity between male and female patients, and patients with a fair skin tone are more likely to have a medulloblastoma. Patients present with cerebellar signs and signs of elevated intracranial pressure. Diagnostic efforts should consist of cerebral MRI and MRI of the spinal axis. Cerebrospinal fluid should be investigated to look for tumor dissemination. Medulloblastoma tumors can be classified as classic, desmoplastic, anaplastic, and large cell, according to the WHO tumor classification. Molecular subgroups include WNT, SHH, group 3, and group 4 tumors. Further molecular analyses suggest that there are several subgroups within the four existing subgroups, with significant differences in patient age, frequency of metastatic spread, and patient survival. As molecular markers have started to play an increasing role in determining treatment strategies and prognosis, their importance has increased rapidly. Treatment options include microsurgical tumor resection and radiotherapy and, in addition, chemotherapy that respects the tumor biology of individual patients offers targeted therapeutic approaches. For neurosurgeons, intraoperative imaging and tumor fluorescence may improve resection rates. Disseminated disease, residual tumor after surgery, lower radiation dose, and low Karnofsky performance status are all suggestive of a poor outcome. Extraneural spread occurs only in very few cases. The reported 5-year-survival rates range between 60% and 80% for all adult medulloblastoma patients.

Resident Perspectives

Resident Perspectives

Decompressive craniectomy in malignant middle cerebral artery infarctions: outcome of 25 cases

BackgroundLarge space-occupying middle cerebral artery (MCA) infarctions with signs of elevated intracranial pressure and brain herniation have overall mortality rates of 80%. Several studies have suggested that decompressive surgery reduces mortality and improves outcome of these patients.AimThe aim of this study was to evaluate the clinical and radiological outcome of decompressive craniectomy in the setting of malignant MCA infarctions.Patients and methodThis is a prospective study conducted on patients presenting with manifestations of malignant MCA infarction. All cases were operated upon by decompressive craniectomy and duroplasty within 6 h of presentation or deterioration in Glasgow Coma Scale (GCS). Functional outcome was assessed in terms of mortality and modified Rankin Scale (mRS) for neurologic disability. Radiological outcome was assessed by comparing the midline shift in the preoperative CT and 24 to 96 h postoperatively.ResultsTwenty-five patients were included in this study. The mean age was 64.4 years. The mean preoperative GCS score was 8.14. The mean preoperative midline shift was 7.9 mm. All cases showed radiological improvement in the early follow-up period. Good functional outcome based on mRS was achieved in 64% and poor outcome in 36% of cases. The overall mortality in this study was 28%.ConclusionEarly decompressive hemicraniectomy had a proven role in reduction of mortality and improving functional outcome in cases with malignant MCA infarction. Among other factors, timing of surgery and the preoperative clinical condition were factors that affected the final outcome.

Clinical characteristics of acute encephalopathy with acute brain swelling: A peculiar type of acute encephalopathy

ObjectivesAcute encephalopathy has been observed with acute brain swelling (ABS) that is characterized by rapid progression to whole-brain swelling. The objective of this study was to describe the clinical characteristics of ABS. MethodsWe encountered four patients with ABS and retrospectively investigated their clinical data with a medical chart review. ResultsThree patients had seizure clustering or status epilepticus in the clinical course. Signs of elevated intracranial pressure (ICP) appeared 3–9 h after the first convulsive attack in three patients. In all patients, signs of brainstem involvement appeared 1–8 h after signs of elevated ICP. Mild hyponatremia that progressed after signs of elevated ICP appeared was noted in three patients. Brain CT revealed mild brain swelling in the initial phase, which rapidly progressed to whole-brain swelling. No focal abnormalities were detected on brain MRI in one patient. Continuous electroencephalography was initially normal, but in two patients, high-amplitude slow waves appeared with rapid changes before signs of brainstem involvement. Although recovery was achieved without sequelae in two patients, outcome was fatal for the other two. ConclusionsThe pathogenesis of ABS has yet to be clarified, but clinical features in our patients are not consistent with any established subtypes of acute encephalopathy. Therefore, we believe that ABS should be recognized as a new type of acute encephalopathy.

Virtual Surgical Planning for Correction of Delayed Presentation Scaphocephaly Using a Modified Melbourne Technique.

Late treatment of scaphocephaly presents challenges including need for more complex surgery to achieve desired head shape. Virtual surgical planning for total vault reconstruction may mitigate some of these challenges, but has not been studied in this unique and complex clinical setting. A retrospective chart review was conducted for patients with scaphocephaly who presented to our institution between 2000 and 2014. Patients presenting aged 12 months or older who underwent virtual surgical planning-assisted cranial vault reconstruction were included. Patient demographic, intraoperative data, and postoperative outcomes were recorded. Pre- and postoperative anthropometric measurements were obtained to document the fronto-occipital (FO) and biparietal (BP) distance and calculate cephalic index (CI). Virtual surgical planning predicted, and actual postoperative anthropometric measurements were compared. Five patients were identified who fulfilled inclusion criteria. The mean age was 50.6 months. One patient demonstrated signs of elevated intracranial pressure preoperatively. Postoperatively, all but one needed no revisional surgery (Whitaker score of 1). No patient demonstrated postoperative evidence of bony defects, bossing, or suture restenosis. The mean preoperative, simulated, and actual postoperative FO length was 190.3, 182, and 184.3 mm, respectively. The mean preoperative, simulated, and actual postoperative BP length was 129, 130.7, and 131 mm, respectively. The mean preoperative, simulated, and actual postoperative CI was 66, 72, and 71.3, respectively. Based on our early experience, virtual surgical planning using a modified Melbourne technique for total vault remodeling achieves good results in the management of late presenting scaphocephaly.

Imaging in Neurocritical Care Practice.

The use of neuroimaging in conjunction with serial neurological examinations is a core component of modern neurocritical care practice. Although there is a growing role for other neuromonitoring techniques, the ability to quickly and accurately interpret images in the context of a patient's clinical status arguably remains the indispensable skill for neurocritical care practitioners. Due to its rapid acquisition time and excellent ability to detect intracerebral hemorrhage (ICH), cerebral edema, and signs of elevated intracranial pressure, computed tomography (CT) remains the most useful neuroimaging technique for intensive care unit (ICU) patients. An emergent head CT is obtained to inform most time-sensitive decisions that arise in the neurological ICU (NICU). CT features also figure prominently in prognostic scores for common NICU conditions such as traumatic brain injury (TBI), ICH, and subarachnoid hemorrhage (SAH). Among patients who are sufficiently stable to leave the ICU and lie flat for an extended period, magnetic resonance imaging provides much more detailed, high-contrast images which can aid in the detection of ischemia, diffuse axonal injury, and neuroprognostication. Though primarily used in neurocritical care research, nuclear medicine imaging techniques have some clinical applications, particularly in ancillary testing for brain death. Finally, as in the field of critical care as a whole, formal and point-of-care ultrasound studies are increasingly utilized in the NICU, and are an important tool in the neurointensivist's armamentarium. We review here the common applications of imaging in the neurocritical care setting. As ICU patients are frequently unstable and their risk of clinical decompensation increases substantially during transport away from the ICU, guidelines and recommendations for maximizing patient safety during transport to radiology studies are also explored.

Pediatric Pseudotumor Cerebri Syndrome.

Idiopathic intracranial hypertension, otherwise known as primary pseudotumor cerebri syndrome (PTCS), most frequently occurs in obese women of childbearing age. However, children may be affected as well. This review will address recent findings regarding demographics, diagnosis, and treatment of pediatric PTCS. Prepubertal children with primary PTCS have an equal sex distribution and less frequent obesity compared with adult patients. However, female gender and obesity are risk factors for primary PTCS in postpubertal children. Compared with adults, children with PTCS more frequently present with ocular motility deficits and more often have associated medical conditions that increase the risk of developing PTCS. Visual field testing may be unreliable, and the optimal modality to monitor visual function is unknown. MRI shows signs of elevated intracranial pressure (ICP) in children with PTCS similar to that of adults. It has now been established that elevated ICP in children ≤18 years old is greater than 25 cm H20 in nonobese, nonsedated children, and greater than 28 cm H2O in the remainder. Optical coherence tomography (OCT) may be used to distinguish pseudopapilledema from papilledema, monitor response to treatment in preverbal children, and identify patients with PTCS at risk for permanent visual loss. However, the precise role of OCT in the management of pediatric PTCS remains to be determined.

From Symptom to Diagnosis—The Prediagnostic Symptomatic Interval of Pediatric Central Nervous System Tumors in Austria

BackgroundChildren with central nervous system (CNS) tumours may present with a multitude of symptoms, ranging from elevated intracranial pressure to focal neurological deficit. In everyday practice, some signs may be misleading, thereby causing prolonged prediagnostic symptomatic intervals. Prediagnostic symptomatic intervals are longer for pediatric brain tumors than for other childhood malignancies. This study evaluated prediagnostic symptomatic intervals and parental and diagnostic intervals for pediatric patients with CNS tumours in Austria. It also considered socioeconomic factors. MethodsPatients ≤ 19 years of age treated at the Medical University of Vienna and diagnosed during the years 2008 to 2013 were included. Patients diagnosed incidentally or by screening were excluded. ResultsTwo hundred twelve consecutive patients were included in the study. They reflected the expected spectrum of CNS tumors. Patients presented with a median of five symptoms at diagnosis, most frequently with signs of elevated intracranial pressure. The median prediagnostic symptomatic interval was 60 days (0 days to seven years), the median parental interval was 30 days (0 days to 6.7 years), and the median diagnostic interval was three days (0 days to 6.5 years). In spinal tumors alone (n = 7), the median prediagnostic symptomatic interval was 70 days (ten days to seven years), and three of seven patients had a prediagnostic symptomatic interval longer than 320 days. Young age, higher tumor grade, and ataxia were associated with a shorter prediagnostic symptomatic interval. Localization in the supratentorial midline, histology of craniopharyngioma, and endocrine symptoms prolonged the prediagnostic symptomatic interval. There was a clear trend for longer prediagnostic symptomatic interval in non-native speakers. ConclusionsResults are comparable to other industrialized countries. However, long delays in diagnosis of central nervous system tumors still occur, urging increased awareness.

Facial Paralysis and Hearing Loss: A Rare Manifestation of Prostate Cancer Metastases

Dural prostate metastases (DPM) are a rare manifestation of metastatic prostate cancer seen in approximately one to six percent of cases. Presenting symptoms may include signs of elevated intracranial pressure, headache, altered mental status, or cranial nerve palsies. Hearing loss, sensory changes, dysarthria, and dysphagia are rare symptoms in DPM that were present in our patient. We present a case of a 58-year-old male with a known diagnosis of adenocarcinoma of the prostate presenting with symptoms of acute exacerbation of chronic obstructive pulmonary disease (COPD), sub-acute right-sided hearing loss, and right-sided facial paralysis. Over the course of hospitalization, his neurological symptoms worsened and he developed dysarthria, dysphagia, facial numbness, and worsening back pain. He also appeared more withdrawn and lethargic. The symptoms prompted a neurological evaluation and a magnetic resonance imaging (MRI) revealed multiple areas of bone marrow signal abnormality compatible with osseous metastatic disease. There was extensive smooth dural thickening as well as focal nodular thickening, both consistent with dural metastases. The patient was treated with corticosteroids and external beam radiation therapy (EBRT) with improvement in his back pain and facial paralysis. He died two weeks after completing EBRT. Although rare, DPM should be suspected in males over 50 years of age presenting with neurological symptoms. An MRI with gadolinium is most helpful in delineating the presence and extent of dural and calvarial involvement. Corticosteroids and EBRT have been shown to improve neurological function in up to 67% of patients. However, median survival post-radiation remains approximately three months.

Imaging Features of Idiopathic Intracranial Hypertension in Children.

Magnetic resonance imaging (MRI) signs of elevated intracranial pressure and idiopathic intracranial hypertension have been well characterized in adults but not in children. The MRIs of 50 children with idiopathic intracranial hypertension and 46 adults with idiopathic intracranial hypertension were reviewed for optic nerve head protrusion, optic nerve head enhancement, posterior scleral flattening, increased perioptic cerebrospinal fluid, optic nerve tortuosity, empty or partially empty sella, tonsillar herniation, enlargement of Meckel's cave meningoceles, and transverse venous sinus stenosis(TSS). Compared to adolescents (11-17 years, n = 40) and adults (>17 years, n = 46), prepubescent children (<11 years, n = 10) had lower frequencies of scleral flattening (50% vs 89% and 85%, P = .02), increased perioptic cerebrospinal fluid (60% vs 84% and 89%, P = .08), optic nerve tortuosity (20% vs 46% and 59%, P = .07), empty or partially empty sella (56% vs 78% and 93%, P = .007), and TSS (67% vs 93% and 96%, P = .04). Children with idiopathic intracranial hypertension have similar MRI findings as adults, but they are less frequent in prepubescent children.

Desmoplastic astrocytoma: new insights into its clinical profile, diagnosis, and treatment

Desmoplastic astrocytoma (DA) is a rare intracranial tumor which usually affects pediatric patients. The aim of this study is to describe the clinical features and management of DA based on a joint analysis of the cases reported in the scientific literature. A thorough review was carried out, gathering those pathologically proven DAs reported since the first description of this entity. Two new own cases were included in order to illustrate this review. Epidemiological, clinical, radiological, therapeutic, and follow-up data were analyzed with the software SPSS version 20. A total of 52 DAs were recorded. Most cases occurred in the first 2years of life, although older patients were also reported. Patients mainly presented symptoms and signs of elevated intracranial pressure. According to their radiological features, we were able to classify DAs in four main groups, with distinct differential diagnosis and prognosis. After treatment, 14.2% of patients presented persistent neurological impairment and the mortality rate was close to 10%. DAs can be diagnosed at any age from birth to adolescence. These neoplasms can show up a wider range of radiological morphologies than previously thought. Surgery represents the treatment of choice for DAs, although chemotherapy can also be useful in the setting of recurrence or progression of the disease. Those DAs lacking classic radiological features, especially type 4 tumors, were linked with a poorer clinical outcome.

Macrocephaly and right arm disuse in a 7-month-old boy

B.Desmoplastic infantile ganglioglioma The patient underwent gross total resection, and final pathology was consistent with desmoplastic infantile ganglioglioma (DIG). Intraoperatively, the tumor body was firmly attached to the dura and had an associated cystic nodule (Fig. 2A). Pathological examination of the specimen demonstrated tumor mainly in the leptomeninges, which was well-demarcated from the adjacent brain (Fig. 2B). The leptomeningeal component consisted primarily of desmoplastic areas and scattered foci with different cell types. Mitotic figures were very rare and only very minimal necrosis was visualized. The adjacent brain demonstrated mainly perivascular infiltrate, with few individual tumor cells infiltrating the cortex. Accordingly, the Ki-67 immunolabeling was low (1–5%). As the patient underwent gross total surgical resection (Fig. 2C, D) with no residual enhancement on MRI (Fig. 2E), no further adjuvant therapy was necessary. DIG is a very rare childhood tumor almost always presenting before 2 years of age [[1]Tamburrini G. Colosimo Jr., C. Giangaspero F. et al.Desmoplastic infantile ganglioglioma.Child’s Nerv Syst: ChNS. 2003; 19: 292-297Crossref PubMed Scopus (72) Google Scholar]. Its etiology is unknown, although is likely prenatal in origin. Common presenting signs and symptoms include macrocephaly, hemiparesis, and/or signs of elevated intracranial pressure [[2]Blount J.P. Bauer D.F. Desmoplastic infantile gangliogliomas.in: Tonn J.-C. Westphal M. Rutka J.T. Oncology of CNS Tumors. Springer, Berlin Heidelberg2010: 477-481Crossref Scopus (2) Google Scholar]. CT scan and MRI demonstrate an aggressive-appearing tumor, which can appear similar to primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, pleomorphic xanthoastrocytoma, or ganglioglioma [[3]Alexiou G.A. Stefanaki K. Sfakianos G. et al.Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature.Pediatr Neurosurg. 2008; 44: 422-425Crossref PubMed Scopus (22) Google Scholar]; therefore the diagnosis cannot definitively be made with imaging alone. The mainstay of treatment is surgical resection, which is considered curative [[1]Tamburrini G. Colosimo Jr., C. Giangaspero F. et al.Desmoplastic infantile ganglioglioma.Child’s Nerv Syst: ChNS. 2003; 19: 292-297Crossref PubMed Scopus (72) Google Scholar]. Chemotherapy is reserved for cases of brain invasion, or when partial resection is followed by radiographic evidence of non-operable disease progression. Radiotherapy may be used as a last resort in children over 5 years of age when other approaches have failed. The prognosis of both complete and partially resected DIG is excellent. Partial resection is often followed by disease stability or spontaneous regression, with a median disease-free survival of 8 to 30 years reported [[3]Alexiou G.A. Stefanaki K. Sfakianos G. et al.Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature.Pediatr Neurosurg. 2008; 44: 422-425Crossref PubMed Scopus (22) Google Scholar]. There are rare reports of local recurrence and/or metastasis, usually associated with deep-seated, particularly suprasellar tumor locations. Surveillance MRIs are generally recommended until stable disease has persisted for some time. Lifetime surveillance does not appear to be necessary as there are no reports of long-term recurrence [[2]Blount J.P. Bauer D.F. Desmoplastic infantile gangliogliomas.in: Tonn J.-C. Westphal M. Rutka J.T. Oncology of CNS Tumors. Springer, Berlin Heidelberg2010: 477-481Crossref Scopus (2) Google Scholar]. Macrocephaly and right arm disuse in a 7-month-old boyJournal of Clinical NeuroscienceVol. 34PreviewA 7-month-old boy presented with macrocephaly (head circumference of 52 cm, 2.5 standard deviations above the 98th percentile). An examination of his cranial contour demonstrated prominent dilated scalp vessels, an elevated and full anterior fontanelle, and mild splaying of the coronal and sagittal sutures. He maintained his right hand in a fisted position, and would not reach towards objects or faces with his right hand; his right upper extremity was also significantly hypertonic. A neurological examination of his cranial nerves and other limbs was otherwise normal. Full-Text PDF

Clinical characteristics and outcome of patients with primary central nervous system lymphoma

目的探讨原发中枢神经系统淋巴瘤(PCNSL)的临床特征、治疗方案以及预后相关因素。方法回顾性总结北京协和医院自1999年6月至2012年6月收治的37例PCNSL患者临床资料，对患者临床特征、治疗以及预后相关因素进行分析。结果全部37例PCNSL患者中位发病年龄57(17~78)岁，男女比为2.7:1。以颅内高压和智能障碍为最常见临床表现，起病至诊断中位时间1.5(1~24)个月。肿瘤累及部位以大脑半球为主，且多灶病变更为常见。36例经治患者无进展生存(PFS)时间为18.0 (95%CI 9.1~26.9)个月，总生存(OS)时间为36.0 (95%CI 21.7~50.3)个月，3年累计OS率为46.9%。放化疗联合治疗与单纯化疗组PFS时间[16.0 (95%CI 8.9~23.1)个月对26.0(95% CI 2.9~49.1)个月]及OS时间[36.0 (95%CI 2.3~69.7)个月对29.0 (95%CI 23.4~34.6)个月]比较，差异均无统计学意义(P=0.401、0.866)。结论PCNSL预后不佳，化放疗联合并不能显著改善患者生存，其最佳治疗方案应需进一步探索。