[show abstract][hide abstract] ABSTRACT: Glioblastoma multiforme is the most common malignant primary brain tumor in adults and generally considered to be universally fatal. Glioblastoma multiforme accounts for 12% to 15% of all intracranial neoplasms and affects 2 to 3 adults per every 100,000 in the United States annually. In children glioblastoma multiforme accounts for only approximately 7% to 9% of central nervous system tumors. The mean survival rate in adults after diagnosis ranges from 12 to 18 months with standard therapy and 3 to 6 months without therapy. The prognosis in children is better compared to adult tumor onset with a mean survival of approximately 4 years following gross total surgical resection and chemotherapy. There have been few advances in the treatment of glioblastoma multiforme in the past 40 years beyond surgery, radiotherapy, chemotherapy, and corticosteroids. For this reason a restrictive calorie ketogenic diet, similar to that used in children to control drug resistant seizure activity, has been advanced as an alternative adjunctive treatment to help prolonged survival. This article reviews the science of tumor metabolism and discusses the mechanism of calorie restriction, cellular energy metabolism, and how dietary induced ketosis can inhibit cancer cell's energy supply to slow tumor growth.
Journal of child neurology 05/2013; · 1.59 Impact Factor
[show abstract][hide abstract] ABSTRACT: INTRODUCTION: This study aims to evaluate the capability of magnetic resonance imaging (MRI) susceptibility weighted images (SWI) in depicting retinal hemorrhages (RH) in abusive head trauma (AHT) compared to the gold standard dilated fundus exam (DFE). METHODS: This is a retrospective, single institution, observational study on 28 patients with suspected AHT, who had a DFE and also underwent brain MRI-SWI as part of routine diagnostic protocol. Main outcome measures involved evaluation of patients to determine whether the RH could be identified on standard and high-resolution SWI sequences. RESULTS: Of the 21 subjects with RH on DFE, 13 (62 %) were identified by using a standard SWI sequence performed as part of brain MRI protocols. Of the 15 patients who also underwent an orbits SWI protocol, 12 (80 %) were positive for RH. None of the seven patients without RH on of DFE had RH on either standard or high-resolution SWI. Compared with DFE, the MRI standard protocol showed a sensitivity of 75 % which increased to 83 % for the orbits SWI protocol. CONCLUSIONS: Our study suggests the usefulness of a tailored high-resolution orbits protocol to detect RH in AHT.
[show abstract][hide abstract] ABSTRACT: Acute encephalitis remains one of the contemporary challenges of critical care medicine. The diagnosis is difficult and sometimes unconfirmed, and encephalitis remains without clear evidence-based therapies or even therapeutic goals for the prevention of high neurologic sequelae. This article provides a framework for pediatric intensivists to guide the diagnosis and management of patients with suspected encephalitis. It provides an in-depth review of the most common causes of encephalitis in children. The article promotes early recognition, appropriate testing and empiric treatment, and management of the expected complications of acute encephalitis.
Critical care clinics 04/2013; 29(2):259-77. · 1.72 Impact Factor
[show abstract][hide abstract] ABSTRACT: We report on tacrolimus-associated posterior reversible encephalopathy syndrome with the previously unreported finding of leptomeningeal enhancement occurring separate from the site of parenchymal magnetic resonance signal abnormality. Recognition of this atypical finding as a noninfectious cause of leptomeningeal enhancement may assist those caring for patients affected by posterior reversible encephalopathy syndrome.
[show abstract][hide abstract] ABSTRACT: Seizures may frequently occur during tuberculous meningitis. We describe a patient with an apparent generalised tonic-clonic seizure, initially not associated with any magnetic resonance imaging (MRI) abnormality, which was the presenting symptom of tuberculous meningitis. Follow-up MRI, performed after gadolinium administration, showed signs of meningeal involvement. Seizures may be the presenting symptoms of tuberculous meningitis even in the absence of evident intracerebral lesions on MRI. Therefore, contrast-enhanced brain MRI should be performed in the diagnostic workup for each first seizure, especially in patients with a clinical suspicion of CNS infectious disease. The term "heraldic seizure", indicating a subset of acute symptomatic seizures presenting at the onset of a brain/systemic injury or preceding the full clinical manifestation of a cerebral insult, may be helpful to classify these seizures retrospectively, based initially on unknown aetiology.
Epileptic disorders: international epilepsy journal with videotape 08/2012; 14(3):329-33. · 1.17 Impact Factor
[show abstract][hide abstract] ABSTRACT: Agenesis of the internal carotid artery (ICA) is a rare congenital anomaly occurring in less than 0.01% of the population, often incidentally discovered in pediatric populations. We recognized a high incidence of additional congenital malformations in children with ICA agenesis.
Our study reports nine cases of ICA agenesis and co-existent malformations and discusses implications of the association.
We conducted a retrospective chart review of nine children evaluated at our institution with imaging findings of ICA agenesis.
Seven children (78%) had congenital aortic or cardiac anomalies including coarctation (4), hypoplastic left heart (1), tetralogy of Fallot (1), and muscular ventricular septal defect (VSD) (1). Four children were diagnosed with an inherited disorder: Alagille syndrome (1), PHACE syndrome (1), VACTERL association (1), and methylenetetrahydrofolate reductase (MTHFR) gene variant (1). Additional congenital anomalies are also described.
In the setting of ICA agenesis, we report a robust association with congenital aortic and cardiac anomalies, as well as a broad spectrum of additional anatomical abnormalities that can occur in the setting of known genetic syndromes or as isolated findings. Knowledge of the natural history of ICA agenesis and associated anomalies will guide optimal care for these children.
[show abstract][hide abstract] ABSTRACT: To establish whether 18FFluorodeoxyglucose (FDG) positron emission computerised tomography (FDG-PET/CT) might reveal active disease in patients with myositis.
We studied 12 patients with active myositis (2 polymyositis, 10 dermatomyositis). The controls consisted of 14 randomly chosen subjects without muscle disease. FDG uptake was expressed as the ratio of maximum proximal muscle to liver standardised uptake value. Magnetic resonance of the thigh and pelvic floor muscles was performed on a 1.0 or 1.5T scanner using a surface coil. Oedema (1= present, 0=absent) was assessed by fat suppressed sequences in 17 muscles and a score (0-17) calculated by adding the separate scores. Muscle strength was evaluated in 12 muscle groups by manual muscle test and graded according to the extended Medical Research Council scale (0-5).
FDG uptake in proximal muscles was significantly higher in patients with myositis (median 0.58, interquartile range 0.52) than in those without (median 0.30, interquartile range 0.09; p<0.001 Mann-Whitney U-test). FDG muscle uptake in patients with myositis did not correlate with disease duration, creatine kinase levels, muscle strength, or magnetic resonance scores.
FDG-PET/CT can reveal FDG uptake by affected muscles of patients with myositis and might potentially be useful to assess myositis activity.
Clinical and experimental rheumatology 05/2012; 30(4):570-3. · 2.66 Impact Factor
[show abstract][hide abstract] ABSTRACT: We report a patient showing isolated phonological agraphia after an ischemic stroke involving the left supramarginal gyrus (SMG). In this patient, we investigated the effects of focal repetitive transcranial magnetic stimulation (rTMS) given as theta burst stimulation (TBS) over the left SMG, corresponding to the Brodmann area (BA) 40. The patient and ten control subjects performed a dictational words and nonwords writing task before, and 5 and 30 min after they received excitatory intermittent TBS (iTBS) over the left BA 40, the right hemisphere homologous to BA 40, the Wernicke's area, or the primary visual cortex. ITBS over the left SMG lead to a brief facilitation of phonological non-words writing to dictation. This case study report illustrates that rTMS is able to influence, among other language functions, the phonological loading processes during the written language production in stroke patients.
Brain and Language 01/2012; 120(3):422-6. · 3.39 Impact Factor
[show abstract][hide abstract] ABSTRACT: Hepatic encephalopathy (HE) is an uncommon complication of total parenteral nutrition (TPN). Cytotoxic edema has not been reported in children with TPN-related HE. We describe a case of TPN-related HE presenting with diffuse cytotoxic edema which reversed after liver transplantation. J Neuroimaging 2012;XX:1-3.
Journal of neuroimaging: official journal of the American Society of Neuroimaging 01/2012; · 3.36 Impact Factor
[show abstract][hide abstract] ABSTRACT: Little attention has been given to the fetal-type posterior circle of Willis (FTP) in the literature; also symptomatic basilar artery (BA) hypoplasia has been rarely reported. We aimed to illustrate that the association of a hypoplastic vertebrobasilar system (VBS) with the FTP may lead to posterior circulation ischemia. Magnetic resonance imaging and three-dimensional time-of-flight magnetic resonance angiography were performed in 88 consecutive patients with ischemic stroke or TIA in the VBS. Thirteen patients were identified with either stroke or TIA in the context of a hypoplastic VBS and a fetal origin of the posterior cerebral arteries. All patients had unilateral or bilateral FTP, hypoplastic BA and at least one hypoplastic vertebral artery. Transcranial color-coded duplex revealed decreased flow velocity and increased pulsatility index along the BA. A hypoplastic VBS may be accompanied by the FTP and its simultaneous occurrence can predispose to ischemic events in the posterior circulation.
[show abstract][hide abstract] ABSTRACT: We report a case of acute disseminated encephalomyelitis preceding measles virus infection. Brain magnetic resonance imaging revealed signal intensity abnormalities in the basal ganglia and cortex consistent with acute disseminated encephalomyelitis. Fever and the first Koplik spots appeared 8 and 10 days later, respectively. This case supports the hypothesis that the immune-mediated demyelinating process may occur before the symptomatic phase of a viral infection. Therefore, children without history of infectious disorders should also have acute disseminated encephalomyelitis included in the differential considerations.
Journal of child neurology 07/2011; 26(12):1590-2. · 1.59 Impact Factor
[show abstract][hide abstract] ABSTRACT: NF2 is an autosomal dominant disorder with neuroectodermal dysplasia. Most patients present with characteristic clinical tumors during or beyond the adolescent age group. The diagnosis is mainly clinical. Vasculopathy is rarely associated with NF2. Vascular complication as the presenting symptom in NF-2 is unknown. We report a case of a 2-year-old child with no prior family history of neurofibromatosis presenting with ataxia and brain-stem stroke.
Journal of neuroimaging: official journal of the American Society of Neuroimaging 06/2011; · 3.36 Impact Factor
[show abstract][hide abstract] ABSTRACT: Primary central nervous system vasculitis (PCNSV) is a rare primary vasculitis limited to the brain and spinal cord. It can affect any age group, but has a predilection for subjects aged 40 to 60 years without clear gender predominance. Clinical manifestations are nonspecific, including headache, non-focal neurological features and, less frequently, focal neurological signs. Brain biopsy is the diagnostic gold standard, but may be falsely negative when unaffected tissue is sampled. In addition, brain biopsy carries a small but significant risk of serious complications. Imaging procedures are a key part of the workup of PCNSV patients. They can be used to document the extent and type of lesions, to gauge response to treatment, and sometimes as surrogates for brain biopsy. Magnetic resonance is extremely sensitive but non-specific. The most common findings are multiple bilateral ischaemic lesions often involving white and grey matter. Conventional or magnetic resonance angiography (MRA) typically shows segmental narrowing and dilation in multiple cerebral arteries. However, atypical findings have also been described both with magnetic resonance and angiography. This review discusses the state-of-the-art of current imaging techniques in the workup of PCNSV patients and highlights future prospects.
[show abstract][hide abstract] ABSTRACT: OBJECTIVE: Our aim was to review the emergent neuroimaging findings of alcohol-related CNS nontraumatic disorders. Alcohol (ethanol) promotes inflammatory processes, increases DNA damage, and creates oxidative stress. In addition, the accompanying thiamine deficiency may lead to Wernicke encephalopathy. Associated changes in serum osmolarity may lead to acute demyelination. CONCLUSION: Alcohol-related encephalopathies can be life-threatening conditions but can be prevented or treated, if recognized.
American Journal of Roentgenology 12/2010; 195(6):1378-84. · 2.90 Impact Factor
[show abstract][hide abstract] ABSTRACT: A 13 month old with a history of significant pulmonary disease related to reflux, global developmental delay, macrocephaly, and recent decrease in weight gain velocity was referred for further evaluation. MRI demonstrated an area of bilateral symmetrical hyperintensity in the central tegmental tract on T2-weighted and diffusion-weighted images in more than two axial slices (Figs. 1 and 2, arrows). When evaluating a large population of children, the highest percentages of symmetrical CTT alterations in absence of other abnormalities have been described in association with cerebral palsy (30%), developmental delay (15%) and febrile seizures (15%) . Congenital metabolic disorders, Gorlin syndrome, post-chemotherapy syndrome, hypotonia, and craniosynostosis are less frequently seen, being found in 5% of patients . Degeneration of the white matter tracts, edema and gliosis might account for these abnormalities. Although the pathogenesis of CTT alteration is not clearly understood, radiologists should be familiar with the pathological entities associated with CTT.
[show abstract][hide abstract] ABSTRACT: A 50-year-old epileptic woman affected by hydrocephalus due to aqueductal stenosis was admitted to the hospital because of headache, dysarthria, and mild lateral pulsion. Cranial computed tomography (CT) revealed left cerebellar hemorrhage. Subsequent magnetic resonance imaging (MRI) of the brain demonstrated an enlarged ventricular system unchanged from prior studies. Flow-sensitive phase-contrast (PC) cine magnetic resonance imaging showed flow absence through the aqueduct and flow pulsations through the third ventricle floor in systole and diastole, consistent with spontaneous third ventricle patency. On stationary tissue images, the third ventricle floor and the mamillary body were displaced downward at systole and upward at diastole. Stenosis of the cerebral aqueduct of Sylvius was also shown. Retrospective evaluation of the sagittal T2-weighted images disclosed flow void at the level of the third ventricle floor. On-off movements of the third ventricle floor could account for chronic hydrocephalus persistence by an en valve mechanism. Spontaneous third ventriculostomy (STV) was diagnosed on the basis of these findings. STV is a rare condition occurring in chronic obstructive hydrocephalus patients as a result of longstanding pulsations against the walls of the third ventricle, the floor in particular. We reviewed the literature to establish which imaging and clinical findings could improve the identification of STV in patients affected by obstructive hydrocephalus.
Clinical neurology and neurosurgery 11/2010; 112(9):817-20. · 1.30 Impact Factor
[show abstract][hide abstract] ABSTRACT: We report a patient with clinical and neuroimaging findings of spontaneous intracranial hypotension (SIH) who developed cerebral venous thrombosis (CVT). An association between SIH and CVT has rarely been observed. Anticoagulation therapy was administered. The clinical course was subsequently complicated by a large subdural hematoma that required neurosurgical evacuation. The present report indicates that SIH should not be always considered a benign condition, especially when associated with CVT and subdural fluid collections. Furthermore, clinicians should be aware of the potential risks of anticoagulant therapy in patients with SIH and CVT.