Cerebral Volume Loss Research Articles

MRI findings in glutamic acid decarboxylase associated autoimmune epilepsy.

Glutamic acid decarboxylase (GAD65) has been implicated in a number of autoimmune-associated neurologic syndromes, including autoimmune epilepsy. This study categorizes the spectrum of MRI findings in patients with a clinical diagnosis of autoimmune epilepsy and elevated serum GAD65 autoantibodies. An institutional database search identified patients with elevated serum GAD65 antibodies and a clinical diagnosis of autoimmune epilepsy who had undergone brain MRI. Imaging studies were reviewed by three board-certified neuroradiologists and one neuroradiology fellow. Studies were evaluated for cortical/subcortical and hippocampal signal abnormality, cerebellar and cerebral volume loss, mesial temporal sclerosis, and parenchymal/leptomeningeal enhancement. The electronic medical record was reviewed for relevant clinical information and laboratory markers. A study cohort of 19 patients was identified. The majority of patients were female (84%), with a mean age of onset of 27years. Serum GAD65 titers ranged from 33 to 4415nmol/L (normal < 0.02nmol/L). The most common presentation was medically intractable, complex partial seizures with temporal lobe onset. Parenchymal atrophy was the most common imaging finding (47%), with a subset of patients demonstrating cortical/subcortical parenchymal T2 hyperintensity (37%) or abnormal hippocampal signal (26%). No patients demonstrated abnormal parenchymal/leptomeningeal enhancement. The most common MRI finding in GAD65-associated autoimmune epilepsy is disproportionate parenchymal atrophy for age, often associated with abnormal cortical/subcortical T2 hyperintensities. Hippocampal abnormalities are seen in a minority of patients. This constellation of findings in a patient with medically intractable epilepsy should raise the possibility of GAD65 autoimmunity.

The Spectrum of Movement Disorders in Childhood-Onset Lysosomal Storage Diseases.

Movement disorders are a significant clinical problem in lysosomal storage diseases (LSD) and account for substantial morbidity. The spectrum of movement disorders in childhood-onset LSD, however, remains poorly defined. To define the spectrum of movement disorders in a well-characterized cohort of children with LSD. A retrospective chart review at a single tertiary care center (Boston Children's Hospital, Boston, MA, USA). Patients up to the age of 18 years with a clinical, genetic and/or biochemical diagnosis of an LSD and at least one predefined movement disorder (parkinsonism, dystonia, ataxia, tremor, chorea, myoclonus, ballism, restless leg syndrome) were included. 96 patients were identified and 76 patients had a sufficiently document biochemical and/or genetic diagnosis. Of these, 18 patients met inclusion criteria (mean age: 10.3±5.8 (SD) years, range: 3-18 years; 72% male). The most common LSD associated with a movement disorder was Niemann-Pick disease type C (NPC), followed by several types of neuronal ceroid lipofuscinosis (NCL) and different mucopolysaccharidoses. The most common movement disorder was ataxia followed by rest tremor, dystonia and myoclonus. The other predefined movement disorders were rare. The majority of patients presented with more than one movement disorder. The movement disorder was slowly progressive in all patients. Brain MRI changes included diffuse cerebral volume loss, white matter abnormalities with thinning of the corpus callosum, and cerebellar atrophy. Movement disorders develop in a significant number of LSD patients. Ataxia, often in patients with NPC and NCL, is the most common phenotype but significant heterogeneity exists within and between different LSD.

Distinct magnetic resonance imaging features in a patient with novel RARS2 mutations: A case report and review of the literature

Pontocerebellar hypoplasia type 6 (PCH6) is a rare autosomal recessive disease that occurs due to mutations in the mitochondrial arginyl-tRNA synthetase 2 (RARS2) gene. To the best of our knowledge, 23 cases with relatively complete clinical data have been reported thus far. In the present study, a case with PCH6 caused by novel RARS2 mutations is described, in which distinct magnetic resonance imaging (MRI) features were identified. In addition, 23 PCH6 cases found in the literature were reviewed. Early onset hypotonia (43.48%), epileptic seizures (34.78%), encephalopathy (26.08%) and feeding difficulties (17.39%) were common initial symptoms of PCH6. During disease progression, the patients presented refractory epileptic seizures (94.12%), feeding problems (60.87%), severe developmental delay (100%), microcephaly (88.89%) and hyperlactacidemia (76.47%). The clinical features of the present patient were suggestive of PCH6, with early onset epilepsy, feeding difficulties, severe developmental delay, microcephaly, hearing loss and hyperlactacidemia. According to available MRI data from 20 reported cases with PCH6, the characteristic finding in MRI was pontocerebellar dysplasia or progressive cerebral/pontocerebellar atrophy in 16 cases, while 4 cases did not present pontocerebellar hypoplasia, and no basal ganglia involvement was observed in any of the cases. Distinctive MRI features were also identified in the present case, including pontocerebellar preservation after 1 year of age, as well as a high diffusion-weighted imaging signal suggesting intracellular edema in the cerebellar hemispheres, basal ganglia, thalamus and corpus callosum. Progressive loss of cerebral white matter and cortical volume were common features shared by all patients. In conclusion, in the present study, two novel heterozygous mutations were identified in RARS2, namely c.1718C>T(p.Thr573Ile) and c.991A>G (p.Ile331Val). Thus, the present case enriched the phenotypic and genotypic spectrum of the RARS2 mutations.

Imaging of Chronic Concussion.

Conventional imaging findings in patients with cerebral concussion and chronic traumatic encephalopathy are absent or subtle in the majority of cases. The most common abnormalities include cerebral volume loss, enlargement of the cavum of the septum pellucidum, cerebral microhemorrhages, and white matter signal abnormalities, all of which have poor sensitivity and specificity. Advanced imaging modalities, such as diffusion tensor imaging (DTI), blood oxygen level dependent functional MR Imaging (BOLD fMRI), MR spectroscopy, perfusion imaging, positron emission tomography (PET), single photon emission computed tomography (SPECT), and magnetoencephalography detect physiologic abnormalities in symptomatic patients and, although currently in the investigation phase, may become useful in the clinical arena.

P 1 Effect of ocrelizumab on magnetic resonance imaging markers of neurodegeneration in patients with relapsing multiple sclerosis – analysis of the phase III, double-blind, double-dummy, interferon beta-1a- controlled OPERA I and OPERA II studies

Background Ocrelizumab (OCR) is a humanised monoclonal antibody that selectively targets CD20+B cells. The pathogenesis of multiple sclerosis (MS), including neurodegeneration, is thought to be influenced by B cells. Volumetric magnetic resonance imaging (MRI) measurements can be used to assess neurodegeneration. Objective To evaluate the effect of OCR vs interferon beta-1a (IFN β -1a) on brain MRI markers of neurodegeneration in patients with relapsing MS enrolled in two identical Phase III, randomised, double-blind, double-dummy trials (OPERA I and OPERA II). Methods In OPERA I and OPERA II, patients were randomised (1:1) to receive OCR 600 mg via intravenous infusion every 24 weeks or subcutaneous IFN β -1a 44 μ g three-times weekly over 96 weeks. MRI endpoints thought to be related to neurodegeneration included the change in whole brain volume, change in cortical grey matter volume and change in cerebral white matter volume. Results Compared with IFN β -1a, OCR reduced the rate of whole brain volume loss from baseline to Week 96 by 23.5% ( p p = 0.0001), and from Week 24 to Week 96 by 22.8% ( p = 0.0042) and 14.9% ( p = 0.0900) in OPERA I and OPERA II, respectively. OCR-treated patients showed a smaller mean percentage of cortical grey matter volume loss compared with IFN β -1a from baseline to Week 96, with a mean difference of 0.273% ( p = 0.0005) in OPERA I and 0.516% ( p β -1a from baseline to Week 96, with a mean difference of 0.261% ( p = 0.0024); in OPERA II, there was no difference ( p = 0.2748) in cerebral white matter volume loss in patients treated with OCR compared with IFN β -1a from baseline to Week 96. Conclusion The rate of neurodegeneration as measured by whole brain, cortical grey and cerebral white matter volume loss on MRI was reduced by OCR compared with IFN β -1a in patients with relapsing MS over 96 weeks. This study is sponsored by F. Hoffmann-La Roche Ltd.

Sleep mediates the association between homocysteine and oxidative status in mild cognitive impairment

Tremendous progress has been made over the last few years in understanding how sleep and amyloid-β (Aβ) cooperate to speed up the progression of Alzheimer’s disease (AD). However, it remains unknown whether sleep deficits also interact with other risk factors that exacerbate the pathological cascade of AD. Based on evidence showing that higher levels of homocysteine (HCY) and sleep loss increase oxidative damage, we here investigate whether the relationship between HCY and total antioxidant capacity (TAC) is mediated by changes in objective sleep in healthy older (HO, N = 21) and mild cognitive impairment (MCI, N = 21) subjects. Results revealed that reduced TAC levels in MCI was significantly correlated with increased HCY, shorter sleep duration, lower sleep efficiency, and reduced volume of temporal regions. However, only the HCY-TAC association showed diagnostic value, and this relationship was mediated by poorer sleep quality in MCI patients. We further showed that HCY-related cerebral volume loss in MCI depended on the serial relationship between poorer sleep quality and lower TAC levels. These findings provide novel insights into how impaired sleep may contribute to maintain the relationship between HCY and oxidative stress in prodromal AD, and offer empirical foundations to design therapeutic interventions aimed to weaken this link.

DISPROPORTIONATE INCREASE IN VENTRICULAR RELATIVE TO SULCAL CEREBROSPINAL FLUID VOLUME AND COGNITIVE FUNCTIONING: THE SMART-MR STUDY

Global cerebral volume loss is associated with neurodegenerative diseases but also with normal aging. To understand their etiology studies aim to pinpoint specific brain tissue regions that are affected early in the disease process or investigate disproportionate volume loss of specific brain tissue regions. Here, we take a different perspective by examining the disproportionate increase in ventricular relative to sulcal cerebrospinal fluid (CSF) and the association with age and cognitive functioning. Within the SMART-MR study a 1.5T MRI was performed in 1232 patients with vascular disease (mean age 58±10 years, 80% male). Automated brain segmentation was used to quantify brain tissue volumes, CSF and lesions. The ventricular-sulcal CSF (VS) ratio was calculated by dividing ventricular volume by sulcal CSF volume. Z-scores for the domains memory and executive functioning were calculated. Multiple linear regression analysis was used to estimate the associations of VS ratio with age and with memory performance and executive functioning. The mean ventricular and sulcal CSF volumes were 31±15ml and 276±43ml, respectively. The VS ratio was 0.11±0.05. Pearson correlation coefficients between the natural log-transformed VS ratio and total brain volume, intracranial volume, brain parenchymal fraction, log-deep WMH, log-periventricular WMH, and age were 0.02 (p=0.52), 0.18, -0.40, 0.21, 0.33, and 0.35, respectively (p-values <0.0001). The VS ratio increased with older age (p<0.0001) independent of sex, total WMH, and ICV. It was also significantly associated with poorer memory performance (b of Z-score=-1.29; 95% CI -2.41 to -0.17) and poorer executive functioning (b of Z-score=-1.72; 95% CI -2.78 to -0.67), adjusted for age, sex, log-WMH, and ICV. In this population, a disproportionate increase in ventricular volume relative to sulcal CSF is associated with older age and with poorer memory and executive functioning. Future studies should investigate to what extent this is a biomarker of accelerated cognitive aging.

GRIN2B encephalopathy: novel findings on phenotype, variant clustering, functional consequences and treatment aspects

BackgroundWe aimed for a comprehensive delineation of genetic, functional and phenotypic aspects of GRIN2B encephalopathy and explored potential prospects of personalised medicine.MethodsData of 48 individuals with de novo GRIN2B variants...

Baicalin protects neonatal rat brains against hypoxic-ischemic injury by upregulating glutamate transporter 1 via the phosphoinositide 3-kinase/protein kinase B signaling pathway.

Baicalin is a flavonoid compound extracted from Scutellaria baicalensis root. Recent evidence indicates that baicalin is neuroprotective in models of ischemic stroke. Here, we investigate the neuroprotective effect of baicalin in a neonatal rat model of hypoxic-ischemic encephalopathy. Seven-day-old pups underwent left common carotid artery ligation followed by hypoxia (8% oxygen at 37°C) for 2 hours, before being injected with baicalin (120 mg/kg intraperitoneally) and examined 24 hours later. Baicalin effectively reduced cerebral infarct volume and neuronal loss, inhibited apoptosis, and upregulated the expression of p-Akt and glutamate transporter 1. Intracerebroventricular injection of the phosphoinositide 3-kinase/protein kinase B (PI3K/Akt) inhibitor LY294002 30 minutes before injury blocked the effect of baicalin on p-Akt and glutamate transporter 1, and weakened the associated neuroprotective effect. Our findings provide the first evidence, to our knowledge that baicalin can protect neonatal rat brains against hypoxic-ischemic injury by upregulating glutamate transporter 1 via the PI3K/Akt signaling pathway.

Congenital Lymphocytic Choriomeningitis Virus in a Member of a Twin Pregnancy

AbstractWe report a male infant, twin A of a dichorionic pregnancy, with a fetal ultrasound and subsequent MRI at 29 weeks of gestation revealing a small cranium (26 cm, &lt; 3rd percentile for gestation) and marked ex vacuo ventriculomegaly. Twin B had normal ultrasound and brain MRI. Newborn exam was unremarkable and his weight and head circumference were both at 5th percentile. Placental pathology findings included focal acute vasculitis and chronic villitis. Postnatal brain MRI showed significant cerebral volume loss with extensive large cystic parietal encephalomalacia and diffuse cerebral cortical dysplasia (polymicrogyria). Common congenital infectious diseases investigation on maternal serum was normal. Twin A was admitted for bronchiolitis and possible seizure at 3 months of age. At this time, he had microcephaly and was not tracking light. Ophthalmology exam showed markedly abnormal macula with diffuse pigment changes and scarring that extended between the full arcades bilaterally. Congenital lymphocytic choriomeningitis virus (LCMV) infection was confirmed with negative IgM titer and highly positive IgG titer of greater than 1:256. Serologic tests for LCMV should be considered in infants who have central nervous system and retinal involvement but negative TORCH studies.

Neuroimaging of chronic alcohol misuse.

Alcohol is one of the most commonly abused substances worldwide. It results in a wide range of diseases and disorders affecting many organ systems. Alcohol-related nutritional deficiencies and electrolyte disturbance leave chronic abusers at risk of a range of demyelinating conditions to which the radiologist and clinician should always be alert. These include Wernicke's encephalopathy, Korsakoff's syndrome, Marchiafava-Bignami disease and osmotic demyelination. Cerebral volume loss is also a commonly encountered neuroimaging phenomenon in chronic alcohol abusers. Neuroimaging with CT and MR, with a focus on FLAIR and diffusion-weighted MR sequences, play an important role in the diagnosis and often monitoring of these conditions. We present an educational review of these entities in terms of their clinical features, neuropathology and imaging features along with a case example of each condition.

The effect of methylphenidate intake on brain structure in adults with ADHD in a placebo-controlled randomized trial

Based on animal research several authors have warned that the application of methylphenidate, the first-line drug for the treatment of attention-deficit/hyperactivity disorder (ADHD), might have neurotoxic effects potentially harming the brain. We investigated whether methylphenidate application, over a 1-year period, results in cerebral volume decrease. We acquired structural MRIs in a double-blind study comparing methylphenidate to placebo. Global and regional brain volumes were analyzed at baseline, after 3 months and after 12 months using diffeomorphic anatomic registration through exponentiated lie algebra. We included 131 adult patients with ADHD into the baseline sample, 98 into the 3-month sample (54 in the methylphenidate cohort and 44 in the placebo cohort) and 76 into the 1-year sample (37 in the methylphenidate cohort and 29 in the placebo cohort). Methylphenidate intake compared with placebo did not lead to any detectable cerebral volume loss; there was a trend toward bilateral cerebellar grey matter increase. Detecting possible neurotoxic effects of methylphenidate might require a longer observation period. There is no evidence of grey matter volume loss after 1 year of methylphenidate treatment in adult patients with ADHD.

MRI Brain Volume Measurements in Infantile Neuronal Ceroid Lipofuscinosis.

Infantile neuronal ceroid lipofuscinosis is a devastating neurodegenerative storage disease caused by palmitoyl-protein thioesterase 1 deficiency, which impairs degradation of palmitoylated proteins (constituents of ceroid) by lysosomal hydrolases. Consequent lysosomal ceroid accumulation leads to neuronal injury, resulting in rapid neurodegeneration and childhood death. As part of a project studying the treatment benefits of a combination of cysteamine bitartrate and N-acetyl cysteine, we made serial measurements of patients' brain volumes with MR imaging. Ten patients with infantile neuronal ceroid lipofuscinosis participating in a treatment/follow-up study underwent brain MR imaging that included high-resolution T1-weighted images. After manual placement of a mask delineating the surface of the brain, a maximum-likelihood classifier was applied to determine total brain volume, further subdivided as cerebrum, cerebellum, brain stem, and thalamus. Patients' brain volumes were compared with those of a healthy population. Major subdivisions of the brain followed similar trajectories with different timing. The cerebrum demonstrated early, rapid volume loss and may never have been normal postnatally. The thalamus dropped out of the normal range around 6 months of age; the cerebellum, around 2 years of age; and the brain stem, around 3 years of age. Rapid cerebral volume loss was expected on the basis of previous qualitative reports. Because our study did not include a nontreatment arm and because progression of brain volumes in infantile neuronal ceroid lipofuscinosis has not been previously quantified, we could not determine whether our intervention had a beneficial effect on brain volumes. However, the level of quantitative detail in this study allows it to serve as a reference for evaluation of future therapeutic interventions.

Polymer-induced central nervous system complications following vascular procedures: spectrum of iatrogenic injuries and review of outcomes

Polymer substances are commonly applied as surface coatings on endovascular catheters and vascular devices. Adverse effects related to their use have been reported, although the overall clinical significance and appropriate methods of detection of these complications have been unclear. In this analysis, we systematically reviewed clinical and diagnostic features in 32 patients (age, 36-87years; mean, 59years) in whom intracranial polymer reactions were documented following vascular interventions. Associated neuroradiologic and neuropathologic findings were variable and included cerebral vasculitis or vasculopathy (63%), abscess or granuloma formation (38%), ischemic infarcts (28%), parenchymal hematomas (28%), white matter change (25%), and/or chemical meningitis (22%). Location(s) of polymer reactions varied and included sites adjacent to and/or downstream from instrument insertion or implantation. Presenting clinical signs included focal neurologic deficits (41%), headache (22%), constitutional symptoms (19%), meningitis (16%), seizure and/or involuntary movements (9%), coma (6%), and syncope (3%). Adverse outcomes included stroke (31%), death (28%), delayed communicating hydrocephalus (9%), steroid dependency (9%), steroid complications (6%), and cerebral volume loss (3%). In some cases, these complications necessitated increased cost and length of medical care. In this review, we highlight the diverse features of polymer-induced reactions involving the central nervous system and summarize distinct diagnostic patterns that may enable earlier premortem detection of these lesions in the postprocedural clinical setting. Further work in this area is necessary to identify additional etiologic, preventative and therapeutic strategies. These data have potentially broad implications pertaining to the safety, efficacy, standards of use, storage, manufacturing, and regulation of new and emerging vascular devices and polymer nanotechnologies.

Neuroimaging features of Cornelia de Lange syndrome.

Cornelia de Lange syndrome is a rare genetic disease characterized by distinctive facial dysmorphia and dwarfism. Multiple organ system involvement is typical. Various central nervous system (CNS) aberrations have been described in the pathology literature; however, the spectrum of neuroimaging manifestations is less well documented. To present neuroimaging findings from a series of eight patients with Cornelia de Lange syndrome. The CT/MR database at a single academic children's hospital was searched for the terms "Cornelia," "Brachmann" and "de Lange." The search yielded 18 exams from 16 patients. Two non-CNS and six exams without available images were excluded. Ten exams from eight patients were evaluated by a board-certified neuroradiologist. All patients had skull base dysplasia, most with an unusual coronal basioccipital cleft (7/8). All brain MR exams showed microcephaly, volume loss and gyral simplification (5/5). Six patients had an absent massa intermedia. Four patients had small globe anterior segments; three had optic pathway hypoplasia. Basilar artery fenestration was present in two patients; vertebrobasilar hypoplasia was present in one patient. The inner ear vestibules were dysplastic in two patients. One patient had pachymeningeal thickening. Spinal anomalies included scoliosis, segmentation anomalies, endplate irregularities, basilar invagination, foramen magnum stenosis and tethered spinal cord. Typical imaging manifestations of Cornelia de Lange syndrome include skull base dysplasia with coronal clival cleft, cerebral and brainstem volume loss, and gyral simplification. Membranous labyrinth dysplasia, anterior segment and optic pathway hypoplasia, basilar artery fenestration, absent massa intermedia and spinal anomalies may also be present.

Twenty-year brain magnetic resonance imaging follow-up study in Systemic Lupus Erythematosus: Factors associated with accrual of damage and central nervous system involvement

To evaluate the long-term progression of cerebral MRI abnormalities in patients with longstanding SLE, 30 patients (age 53.5±11.3) underwent brain MRI at baseline (b-MRI) and after 19.4±3.7years of follow-up (fu-MRI). Two neuroradiologists visually analyzed the MRIs comparing: 1) white matter hyperintensities (WMHIs), 2) cerebral volume, and 3) parenchymal defects; these outcomes were also built in a modified MRI scoring system (mMSS) to estimate the cumulative parenchymal damage. The independent risk factors for accrual of MRI brain damage, as well as the association between MRI abnormalities and the development of new neuropsychiatric (NP) manifestations classified according to the 1999 ACR case definition were also analyzed. Twenty-three patients (76.7%) showed worsening of mMSS; 19 (63.3%) had increased number and volume of WMHIs, 8 (26.7%) had significant cerebral volume loss, and 6 (20%) showed new ischemic parenchymal lesions. Only 6 patients had normal MRI. Antimalarial agents (p=0.006; OR 0.08) were protective against worsening of WMHIs. High cumulative dose of corticosteroids (p=0.026; OR 8.8) and dyslipidemia (p=0.044; OR 10.1) were associated with increased mMSS and cerebral volume loss, respectively. Higher mMSS score at baseline was independently associated with worsening of WMHIs (p=0.001; OR 5.7) and development of new NP events (p=0.019; OR 2.0); higher load of deep WMHIs at b-MRI (p=0.018; OR 2.0) was independently associated with stroke risk. This study shows that MRI brain damage in SLE patients progresses independently from NP involvement as effect of potentially modifiable risk factors and it is associated with increased risk of new NP events.

Correlations between homocysteine and grey matter volume in patients with Alzheimer's disease.

Previous studies have reported that elevated total homocysteine levels are associated with cognitive dysfunction. However, few studies have examined the radiological markers of associated neuropathology in Alzheimer's disease (AD). We hypothesized that elevated levels of homocysteine are associated with cerebral grey matter volume loss. We compared the grey matter in a high homocysteine group and a normal homocysteine group using an optimized voxel-based morphometry. The study included 79 patients with AD who were divided into two groups: a high homocysteine group and a normal homocysteine group. The participants underwent brain magnetic resonance imaging using a standardized protocol and neurocognitive evaluation. Homocysteine tests and other routine laboratory examinations for dementia assessment were carried out in all patients. There was no significant difference in grey matter volume between the patients with high homocysteine levels and those with normal homocysteine levels. A multiple regression analysis also revealed that the levels of homocysteine were not associated with the grey matter volume in patients with AD. Homocysteine levels were not correlated significantly with Mini-Mental State Examination, Global Deterioration Scale, or Clinical Dementia Rating. Our results showed that elevated homocysteine levels are not associated with reduced cerebral grey matter volume in AD. Larger samples will be needed to assess potential correlations between homocysteine and neuroanatomical pathology in the future.

Severe Traumatic Brain Injury (TBI) with Late Cognitive and Functional Decline

A 73-year-old- gentleman admitted to hospital for evaluation of gradual functional and cognitive decline over past 6 months. He had history of traumatic brain injury at age of 9 when a heavy carton fell accidently on his head. Unfortunately, patient did not undergo any surgery as there was no advance Neurosurgery available those days. Limited history of the mechanism of injury was available from sister who was 4 years younger than patient. Patient sustained right sided hemiplegia requiring a walking stick for mobilisation and remained cognitively well. No previous scans were available. A CT angiogram was organised which revealed marked ex-vacuo dilatation of the left lateral ventricle with associated porencephalic cyst formation with chronic mass effect and mild right sided midline shift. Ventriculomegaly involving the right lateral, third and fourth ventricles was likely related to cerebral, cerebellar and brainstem volume loss. A giant aneurysm of right cavernous internal carotid artery (ICA) measuring 30 mm × 29 mm causing impression on the surrounding area was also seen. Adjacent to the left anterior clinoid process, a metallic fragment was noted - in absence of prior surgical/endovascular intervention, this was likely a post-traumatic fragment. The left internal carotid artery was completely occluded and there was no definite left anterior cerebral artery A1 segment evident. A neurosurgical opinion was sought to consider shunt for possible communicating hydrocephalus. However, patient was deemed not suitable for surgery as patient`s cognitive decline was attributed to cerebrovascular disease rather than possible communicating hydrocephalus as well as likely poor outcome of surgery. Patient ended up in a high level of care nursing home. TBI has a wide range of presentation but a combination of large hydrocephalus, chronic thrombosis and ICA aneurysm with late cognitive decline is uncommon (Figures 1-3).

Gyral Calcification in an Adult Masquerading as Subarachnoid Hemorrhage

An 85-year-old female with a medical history significant for coronary artery disease, status post pacemaker placement, and a prior stroke presented to an outside emergency department (ED) with complaints of lethargy, diaphoresis, dysarthria, and an episode of transient loss of consciousness at her nursing home. There was no history of trauma and she was not on any anticoagulants. A computed tomography (CT) scan was performed and read by the ED physician as subarachnoid hemorrhage (SAH) in the left sylvian fissure, and the patient was emergently transferred to our hospital for further management with a tentative diagnosis of SAH. Upon arrival to our intensive care unit, she was following commands, oriented to self, dysarthric but with fluent speech. Cranial nerve examination was within normal limits except for the presence of rightsided upper motor neuron–type facial nerve palsy. Careful evaluation of the CT scan showed the presence of a serpentine hyperdensity along the left perisylvian cortex consistent with dystrophic calcification from prior ischemic insult rather than an SAH, along with chronic microvascular ischemic changes, old lacunar infarcts, and age-appropriate cerebral parenchymal volume loss (Figure 1). CT angiography performed as part of a

More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders.

Although Langerhans cell histiocytosis (LCH) is a familiar entity to most radiologists and to pediatric radiologists in particular, it is but one of a group of disorders caused by the overproduction of histiocytes, a subtype of white blood cells. Other less familiar diseases in this category are Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG), Rosai-Dorfman disease (RDD), and hemophagocytic lymphohistiocytosis (HLH). This review describes the classification system, clinical manifestations, and pathophysiology of each disease, with particular attention to differential radiographic findings, including typical locations of involvement and varying appearances at radiography, computed tomography, magnetic resonance imaging, ultrasonography, and nuclear medicine imaging. Although LCH has a wide variety of manifestations and appearances, classic imaging findings include vertebra plana, skull lesions with a beveled edge, the "floating tooth" sign, bizarre lung cysts, and an absent posterior pituitary bright spot with infundibular thickening. The classic imaging findings of ECD are a perirenal rind of soft tissue and patchy long bone osteosclerosis. RDD has more nonspecific imaging findings, including lymphadenopathy (most commonly cervical) and intracranial lesions. Imaging findings in HLH are broad, with the most common abnormalities being hepatosplenomegaly, cerebral volume loss, and periventricular white matter abnormalities. JXG can manifest at imaging, but radiology does not play a major role in diagnosis. Familiarity with these disorders and their associated imaging findings facilitates correct and timely diagnosis. Imaging also features prominently in the assessment of treatment response.