Diffusion-weighted MRI abnormalities in an outbreak of Streptococcus agalactiae Serotype III, multilocus sequence type 283 meningitis.

PURPOSE: In 2015, an outbreak of group B streptococcal (GBS) infection caused by Streptococcus agalactiae Serotype III, multilocus sequence type 283, related to consuming infected raw freshwater fish, affected more than 200 patients in Singapore. We describe the clinical, laboratory, and neuroimaging features of a subgroup of adults with central nervous system (CNS) infections caused by GBS. MATERIALS AND METHODS: The database of the Singapore Neurologic Infections Program (SNIP), a national multicenter study for surveillance of infectious neurologic disease, was reviewed to select patients with GBS CNS infection during the outbreak. Cases were diagnosed on the basis of clinical features, cerebrospinal fluid (CSF) findings and identification or isolation of Streptococcus agalactiae in the blood or CSF. Demographic, clinical and neuroradiological information was obtained prospectively and retrospectively abstracted. RESULTS: Fourteen patients (6 male, 8 female; median age, 58 years) presented with fever, meningism, headache, encephalopathy, focal neurological deficits, and/or seizures. All except two were previously healthy. Diffusion-weighted imaging (DWI) on admission was abnormal in 13 patients, showing tiny hyperintensities in the subarachnoid space (7 patients), ventricles (6 patients) and brain parenchyma (8 patients); 5 patients had cerebellar abnormalities. CONCLUSION: Among healthy non-pregnant adults infected with Serotype III, multilocus sequence type 283 GBS meningitis linked to eating infected raw freshwater fish, DWI detected small pus collections and unusual cerebellar involvement. A collaborative national surveillance system that includes MRI can be helpful during unusual food-borne zoonotic infectious disease outbreaks. LEVEL OF EVIDENCE: 4 J. Magn. Reson. Imaging 2017;45:507-514.

Hemispheric lateralization abnormalities of the white matter microstructure in patients with schizophrenia and bipolar disorder.

BACKGROUND: Hemispheric lateralization of the brain occurs during development and underpins specialized functions. It is posited that aberrant neurodevelopment leads to abnormal brain lateralization in individuals with psychotic illnesses. Here, we sought to examine whether white matter hemispheric lateralization is abnormal in individuals with the psychotic spectrum disorders of schizophrenia and bipolar disorder. METHODS: We examined the white matter microstructure lateralization in patients with schizophrenia, bipolar disorder with psychotic features and healthy controls by measuring the laterality indices of fractional anisotropy (FA) and mean diffusivity (MD). We also correlated the laterality indices with clinical measures. RESULTS: We included 150 patients with schizophrenia, 35 with bipolar disorder and 77 healthy controls in our analyses. Shared FA lateralization abnormalities in patients with schizophrenia and bipolar disorder were found in the cerebral peduncle and posterior limb of internal capsule, with more extensive abnormalities in patients with bipolar disorder than in those with schizophrenia. The shared MD lateralization abnormalities were more widespread, extending to the subcortical, frontal-occipital, limbic and callosal tracts, with patients with bipolar disorder showing greater abnormalities than patients with schizophrenia. While lateralization was decreased in patients with schizophrenia, the lateralization was reversed in those with bipolar disorder, underpinned by the more pronounced microstructural abnormalities in the right hemisphere. The loss of FA lateralization in patients with schizophrenia was associated with lower quality of life and psychosocial functioning. LIMITATIONS: Owing to the cross-sectional study design, we cannot confirm whether the lateralization abnormalities are neurodevelopmental or a consequence of psychosis onset or chronicity. CONCLUSION: Shared and distinct white matter lateralization abnormalities were found in patients with schizophrenia and bipolar disorder. In distinct regions of abnormalities, the lateralization was attenuated in patients with schizophrenia and reversed in those with bipolar disorder.

The impact of genome wide supported microRNA-137 (MIR137) risk variants on frontal and striatal white matter integrity, neurocognitive functioning, and negative symptoms in schizophrenia.

Although genome wide association studies have highlighted MicroRNA 137 (MIR137) as a novel susceptibility gene for schizophrenia, the mechanisms by which MIR137 risk variants mediate the neurobiology of schizophrenia are not clear. Based on extant data linking MIR 137 gene with structural brain anomalies and functional brain activations in schizophrenia, we hypothesized that MIR137 risk variants rs1625579 and rs1198588 would be associated with reduced fractional anisotropy in frontostriatal brain regions, impaired neurocognitive functioning and worse psychotic symptoms in schizophrenia patients compared with healthy controls. A total of 147 Chinese participants (84 patients with DSM-IV diagnosis of schizophrenia (SCZ) and 63 healthy controls (HC)) were genotyped using blood samples and underwent diffusion tensor imaging. Neurocognitive domains and psychotic symptoms were assessed using The Brief Assessment of Cognition Battery for Schizophrenia and Positive and Negative Syndrome Scale respectively. We found significant diagnosis-genotype interactions in the right orbitofrontal regions (rs1625579: F = 5.44, P = 0.021; rs1198599: F = 7.55, P = 0.005), left striatum (rs1625579: F = 8.09, P=0.007; rs1198599: F=9.56, P = 0.002), and negative symptoms (rs1625579: t = 2.45, P = 0.016; rs1198588: t = 2.29, P = 0.024). Specifically, SCZ carrying the risk TT genotype had worse negative symptoms and decreased FA in the fronto-striatal regions compared to G and A allele carriers for rs1625579 and rs1198588 respectively, and worse attention and processing speed compared with G-allele for rs1625579. Our findings suggested that the MI137 risk variants were associated with decreased fronto-striatal brain white matter integrity which may underlie poorer attention, processing speed, and greater negative symptoms in schizophrenia.

Cognitive deficits in mild Parkinson's disease are associated with distinct areas of grey matter atrophy.

BACKGROUND AND OBJECTIVES: The neuroanatomical substrates underlying cognitive impairment in Parkinson's disease (PD) remain poorly understood. To address this gap, we compared the grey matter atrophy patterns in PD patients with mild cognitive impairment (PD-MCI) with PD patients having no cognitive impairment (PD-NCI), and examined relationships between atrophic regions and cognitive performance in specific domains. METHODS: 90 non-demented PD patients (64.95±7.54 years, Hoehn and Yahr=1.88±0.39) were classified using formal diagnostic criteria as PD-MCI (n=23) or PD-NCI (n=67). Grey matter volume differences were examined using voxel-based morphometry on structural MRI, and multivariate linear regressions were employed to assess the relationships between cognitive performance in specific domains and atrophic regions. RESULTS: Patients with PD-MCI had lower global cognition scores compared with PD-NCI (Mini Mental State Examination: 26.9 vs. 28.4, p=0.011; Montreal Cognitive Assessment: 24.5 vs. 27.0, p<0.001). The PD-MCI group demonstrated significantly poorer performance on executive function, attention, memory and language abilities. Patients with PD-MCI had reductions in grey matter volumes in the left insular, left superior frontal and left middle temporal areas compared to PD-NCI. Multiple regressions controlling for age, education and cardiovascular risk factors revealed significant positive correlations between left insular atrophy and executive-attention dysfunction. CONCLUSIONS: Domain specific cognitive impairment in mild PD is associated with distinct areas of grey matter atrophy. These regions of atrophy are demonstrable early in the disease course and may serve as a biomarker for dementia in PD.

Corpus callosum morphology in first-episode and chronic schizophrenia: combined magnetic resonance and diffusion tensor imaging study of Chinese Singaporean patients.

BACKGROUND: Abnormalities in the corpus callosum have been reported in patients with schizophrenia for over 30 years but the influence of inter-individual differences and illness characteristics remains to be fully elucidated. AIMS: To examine the influence of individual and illness characteristics on the corpus callosum in Chinese Singaporean patients with schizophrenia. METHOD: Using magnetic resonance and diffusion tensor imaging, mean corpus callosum area, volume and fractional anisotropy were investigated in 120 Chinese Singaporean patients (52 with chronic and 68 with first-episode schizophrenia) and compared with data from 75 matched healthy controls. RESULTS: Both area and volume were significantly reduced in patients relative to controls but no significant differences in corpus callosum existed between genders in either patients or controls. Differences in area and volume of the corpus callosum were greatest in patients whose condition was chronic relative to patients with a first episode and controls. Anterior callosum in patients, regardless of chronicity, was no different to that of controls. CONCLUSIONS: Morphological abnormalities in the corpus callosum may increase with illness progression.

Levodopa and the feedback process on set-shifting in Parkinson's disease.

OBJECTIVE: To study the interaction between levodopa and the feedback process on set-shifting in Parkinson's disease (PD). METHODS: Functional magnetic resonance imaging (fMRI) studies were performed on 13 PD subjects and 17 age-matched healthy controls while they performed a modified card-sorting task. Experimental time periods were defined based on the types of feedback provided. PD subjects underwent the fMRI experiment twice, once during "off" medication (PDoff) and again after levodopa replacement (PDon). RESULTS: Compared with normal subjects, the cognitive processing times were prolonged in PDoff but not in PDon subjects during learning through positive outcomes. The ability to set-shift through negative outcomes was not affected in PD subjects, even when "off" medication. Intergroup comparisons showed the lateral prefrontal cortex was deactivated in PDoff subjects during positive feedback learning, especially following internal feedback cues. The cortical activations were increased in the posterior brain regions in PDoff subjects following external feedback learning, especially when negative feedback cues were provided. Levodopa replacement did not completely restore the activation patterns in PD subjects to normal although activations in the corticostriatal loops were restored. CONCLUSION: PD subjects showed differential ability to set-shift, depending on the dopamine status as well as the types of feedback cues provided. PD subjects had difficulty performing set-shift tasks through positive outcomes when "off" medication, and showed improvement after levodopa replacement. The ability to set-shift through negative feedback was not affected in PD subjects even when "off" medication, possibly due to compensatory changes outside the nigrostriatal dopaminergic pathway.

Genome-wide supported psychosis risk variant in ZNF804A gene and impact on cortico-limbic WM integrity in schizophrenia.

Genome-wide association, case association genetic and meta-analytic studies have highlighted ZNF804A as a robust genome-wide supported susceptibility gene for schizophrenia (SCZ). In view of the possible involvement of ZNF804A gene in early neurodevelopment and cellular processes including oligodendrocyte proliferation and differentiation, we examined the effect of ZNF804A on brain WM (WM) integrity in patients with SCZ. Based on extant data in healthy controls (HC), we hypothesized that ZNF804A risk variant rs1344706 is associated with lower fractional anisotropy (FA) in brain regions within cortico-limbic circuits, namely frontal, parietal, medial temporal lobes, and cingulate gyri in SCZ. A total of 200 Chinese participants (125 patients with DSM-IV diagnosis of SCZ and 75 controls) were genotyped using blood samples, a subset of 153 participants (89 patients with DSM-IV diagnosis of SCZ and 64 controls) underwent structural magnetic resonance imaging and diffusion tensor imaging (DTI). There are significant effects of diagnosis (left cingulate gyrus: Adjusted F(1,149) = 9.36, P = 0.003) and diagnosis-genotype interactions (left parietal lobe: Adjusted F(1,147) = 7.39, P = 0.007; right parietal lobe: Adjusted F(1,147) = 6.95, P = 0.009; right medial temporal lobe: Adjusted F(1,147) = 8.79, P = 0.004; left cingulate gyrus: Adjusted F(1,147) = 8.02, P = 0.005). Specifically, we found that patients with SCZ who are risk T homozygotes have lower FA in bilateral parietal lobes, and left cingulate gyrus compared with G carriers. Compared with risk T homozygotes in HC, patients with SCZ who are risk T homozygotes have decreased FA in bilateral parietal lobes, and left cingulate gyrus as well as right medial temporal lobe. Our findings suggest that ZNF804A risk variant influence WM integrity involving cortico-limbic brain regions in SCZ and highlight the importance of investigating the impact of genome-wide supported risk factors on intermediate phenotypes with potential to shed light on the neurobiology of SCZ.

Practice enhancements with FastStroke ColorViz analysis in acute ischemic stroke.

In acute ischemic stroke (AIS), large vessel occlusion (LVO) and the status of pial collaterals are important factors in decision-making for further treatment such as endovascular therapy. Multiphasic CT Angiogram (mCTA) is the mainstay of AIS imaging, allowing detection of LVO, evaluation of intracranial arterial dynamics, and quantification of pial collaterals. However, thorough mCTA evaluation entails scrutiny of multiple image datasets, individually and then simultaneously, which can be time-consuming, causing a potential delay in treatment. ColorViz (FastStroke, GE Healthcare, Milwaukee, Wisconsin) is a novel CT application which combines mCTA information into a single color-coded dataset for quick, unequivocal evaluation of pial collaterals. In our practice, ColorViz is both time-saving and increases the diagnostic accuracy of LVO and pial collaterals as well as medium vessel, multivessel and posterior circulation occlusions. In this article, we discuss the practical aspects of ColorViz in patients presenting with AIS.

Detecting the Uncommon Imaging Manifestations of Posterior Reversible Encephalopathy Syndrome (PRES) in Adults: a Comprehensive Illustrated Guide for the Trainee Radiologist.

Posterior reversible encephalopathy syndrome (PRES) has traditionally been described as a reversible leukoencephalopathy with a distinct pattern of posteriorly distributed vasogenic oedema involving the subcortical regions of parietal and occipital lobes. PRES commonly occurs in the setting of hypertensive emergencies, pre-eclampsia/eclampsia, impaired renal function, and immunosuppressive therapy. The various clinical presentations of PRES include encephalopathy, seizures, headache, visual, and focal neurological deficits. As knowledge of this entity grows, the range of clinical, and radiological features is seen to be much broader than originally described. The brain oedema may not always be posteriorly distributed and the syndrome may not be uniformly reversible. Of special note are some uncommon imaging features (unilateral cerebral involvement, and isolated posterior fossa involvement) and also some uncommon complications (haemorrhage, cytotoxic oedema, and vasoconstriction). These red herrings may lead to potential diagnostic challenges and pitfalls especially for trainee radiologists, who often read these scans in an emergency setting. Early and accurate diagnosis is crucial for prompt optimum management, thereby avoiding residual morbidity. This review article focusses on the atypical radiological features of PRES in adults with extensive case-based imaging examples. A brief description of the pathophysiology, clinical, and classic radiological features of PRES has also been included. A tabulated summary of potential mimics with diagnostic pearls is provided to highlight pertinent take home points and to serve as an easy guide for day-to-day clinical practice.

Ipsilateral stroke in a patient with horizontal gaze palsy with progressive scoliosis and a subcortical infarct.

BACKGROUND AND PURPOSE: horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare congenital disorder caused by mutation in the ROBO3 gene. It is characterized by absent horizontal eye movements with progressive scoliosis developing in childhood and adolescence. To our knowledge, both diffusion tensor imaging evaluation in HGPPS patients who present with stroke and truncating stop codon mutation in the ROBO3 gene have yet to be reported. SUMMARY OF CASE: we present a man with HGPPS who experienced a left pure motor stroke as a result of a left corona radiata infarct on diffusion-weighted imaging. Diffusion tensor imaging tractography confirmed the presence of uncrossed corticospinal tracts, accounting for the ipsilateral deficit. He was also found to possess a novel ROBO3 stop codon mutation on genetic testing. CONCLUSIONS: patients with HGPPS may present with stroke symptoms on the ipsilateral side of the infarct in view of uncrossed corticospinal tracts. Truncating mutation in ROBO3 may provide additional pathophysiologic insights.

Cortical and subcortical white matter abnormalities in adults with remitted first-episode mania revealed by Tract-Based Spatial Statistics.

OBJECTIVES: Abnormalities of brain white matter have been noted in structural magnetic resonance imaging and diffusion tensor imaging (DTI) studies of bipolar disorder, but there are fewer investigations specifically examining white matter integrity early in the course of illness. In this study, we employed DTI to elucidate white matter changes in adult patients with remitted first-episode mania and hypothesized that first-episode mania was associated with decreased fractional anisotropy in cortical (frontal) and subcortical (thalamus, striatum) white matter as well as white matter tracts (cingulum, corpus callosum). METHODS: Diffusion tensor images were acquired from 16 patients with remitted first-episode mania and 16 healthy controls matched for age, gender, handedness, and years of education. Fractional anisotropy and radial and axial diffusivities were analyzed using Tract-Based Spatial Statistics. RESULTS: Patients had lower fractional anisotropy and higher radial diffusivity in the left anterior frontal white matter, right posterior thalamic radiation, left cingulum, and bilateral sagittal striatum. In addition, increased radial diffusivity was found in the left corpus callosum. CONCLUSION: Our findings highlighted that white matter abnormalities were present by the time of remission of first-episode mania. The widespread occurrence of these white matter abnormalities both in first-episode mania and chronic bipolar disorder suggested that disruption of white matter cortical-subcortical networks as well as projection, associative, and commissural tracts is a hallmark of the illness.

Gyriform restricted diffusion in adults: looking beyond thrombo-occlusions.

Gyriform restricted diffusion (GRD) refers to hyperintense signal involving the cerebral cortex on diffusion-weighted images (DWI) with corresponding hypointensity on apparent diffusion coefficient (ADC) images. These changes are commonly seen following a vascular occlusion, reflecting the limitation of water molecule movement across cell membranes (restricted diffusion) due to the failure of Na+/K+-ATPase pumps (cytotoxic oedema). However, GRD can occur in several other neurological conditions as well. A thorough understanding of these conditions and their anatomic predilection plays a critical role in identifying and differentiating them from vascular thrombo-occlusion, with impact towards appropriate clinical management. This review highlights the less commonly encountered, non-stroke causes of GRD in adults with case-based examples. A tabulated chart of the patterns of cortical and subcortical involvement associated with these aetiologies is provided for a quick, pattern-based reference for daily radiological reporting.

Pearls and Pitfalls in the Magnetic Resonance Diagnosis of Dural Sinus Thrombosis: A Comprehensive Guide for the Trainee Radiologist.

Dural sinus thrombosis (DST) is a potentially fatal neurological condition that can be reversed with early diagnosis and prompt treatment. Non-enhanced CT scan is often the first imaging investigation in patients presenting with acute neurological symptoms; however, its poor sensitivity in detecting DST is a major drawback. Magnetic resonance (MR) imaging offers multiple advantages such as excellent contrast resolution and unenhanced venography possibilities, making it the mainstay in the non-invasive diagnosis of DST. However, physiological variations, evolution of thrombi, and incorrect selection/application of MR techniques can lead to false positive and false negative interpretations impacting patient management and outcome. This article discusses the MR techniques useful to diagnose DST and describes pitfalls, with troubleshooting methods, to ensure an accurate diagnosis. We have used multiple diagrammatic illustrations and MR images to highlight pertinent take-home points and to serve as an easy guide for day-to-day clinical practice.

Adult-onset diffuse leukoencephalopathy with axonal spheroids and pigmented glia presenting with acute stroke-like symptoms: A rare clinical scenario.

Adult-onset diffuse leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a rare progressive degenerative white matter disease caused by mutations in the colony-stimulating factor-1 receptor gene. Patients commonly present in the 4th or 5th decade with variable clinical presentations including behavioral changes, dementia, parkinsonism, and motor dysfunctions, eventually leading to death within a few years. Although the disease is typically hereditary, sporadic cases are known to occur. The classic MRI features of ALSP include T2 hyperintensities in the frontal and parietal white matter, scattered foci of restricted diffusion in the white matter, age-advanced cerebral involutional changes, thinning and signal changes in the corpus callosum, absence of infratentorial involvement and lack of enhancement. CT commonly shows tiny calcifications in the corpus callosum and deep white matter. We report a unique case of sporadic ALSP that initially presented as young stroke with acute onset of left-sided hemiparesis and no preceding history of cognitive decline. However, subsequent cognitive and behavioral changes lead to the consideration of an alternative diagnosis. Stroke-like symptoms is a very rare primary presentation of this disease entity. We have highlighted the classic MRI and CT features that helped to guide its diagnosis in our patient and prompted early corroborative genetic testing.

Acute subdural haemorrhage complicating cerebral venous thrombosis in a patient with protein C deficiency.

Cerebral venous thrombosis (CVT) directly causing subdural haemorrhage (SDH) is a rare entity. We present a case of an 18-year-old female patient who presented with severe occipital headache. Neuroimaging showed acute SDH and CVT. She was eventually discovered to have underlying protein C deficiency. She was treated with anticoagulation and made an uneventful recovery. We aim to highlight the epidemiology, risk factors and aetiopathogenesis of CVT. We have included a literature review of previously described 13 case studies/reports describing SDH associated with CVT and a brief discussion of the dilemmas associated with management.

Enterovirus-related rhombencephalitis and myelitis in the third trimester of pregnancy: A case report highlighting clinico-radiological findings at diagnosis and follow-up.

Rhombencephalitis (RE) refers to inflammatory diseases involving the brainstem and cerebellum. Although RE is a rare entity, it is associated with high morbidity and mortality. The management of such patients is often challenging in terms of identifying the etiology and defining prognosis. Infections, autoimmune and paraneoplastic conditions are commonly implicated. Patients with RE often present with a biphasic illness with an initial flu-like syndrome followed by brainstem dysfunction. CSF pleocytosis, abnormal brain MRI findings, isolation of organism or molecular (PCR/antigen) detection in CSF/blood cultures/stool samples and nasal/rectal swabs help in arriving at a definitive or probable diagnosis. Prompt aggressive treatment with antibacterial and antiviral drugs and/or immunoglobulins along with supportive therapy is crucial for avoiding a poor outcome. We present a case report of a 28-year old female patient who developed RE and myelitis in the third trimester of pregnancy. We aim to highlight the highly suggestive radiological findings which corroborated with the clinical diagnosis of enterovirus infection. The patient's radiological follow-up and neurological sequalae are also described. To the best of our knowledge, ours is the first report which describes the MRI features of this clinical scenario in the third trimester of pregnancy, and also the subsequent clinico-radiological follow up.

Neurocysticercosis Diagnosed by Taenia solium PCR on Brain Biopsy.

Neurocysticercosis is a common cause for brain lesions and adult-onset epilepsy in endemic countries. However, diagnosis is challenging in the absence of typical radiologic or histopathologic features. In this case report, we present a case of a 35-year-old male with a new-onset seizure and a rim-enhancing temporal lobe lesion. Radiologic features were nonspecific, and brain biopsy was performed. Histologic features showed only nonspecific granulomatous inflammation, and the diagnosis of neurocysticercosis was confirmed only with polymerase chain reaction (PCR) testing on brain biopsy tissue demonstrating PCR products consistent with Taenia solium. This case highlights the diagnostic role of PCR in such clinical situations whereby the diagnosis is unclear after initial routine evaluation.

Severe hypoglycemia associated with an illegal sexual enhancement product adulterated with glibenclamide: MR imaging findings.

PURPOSE: To describe the magnetic resonance (MR) imaging findings associated with severe hypoglycemia after consumption of an illegal sexual enhancement product (Power 1 Walnut) adulterated with glibenclamide, an oral hypoglycemic agent used to treat diabetes mellitus. MATERIALS AND METHODS: Institutional review board approval was obtained for this retrospective study. Records in eight male patients with severe hypoglycemia of unknown cause, without prior treatment for diabetes, and with positive blood toxicology results for glibenclamide were reviewed. MR imaging included diffusion-weighted imaging and, in some patients, MR angiography, dynamic contrast material-enhanced perfusion MR imaging, and MR spectroscopy. RESULTS: In seven patients, there were hyperintense abnormalities on diffusion-weighted and T2-weighted images in the hippocampus and cerebral cortex, sparing the subcortical white matter and cerebellum. Three patients had abnormalities of the splenium of the corpus callosum, and one had widespread involvement, including the caudate nucleus, basal ganglia, and internal capsule bilaterally. In three patients, unilateral cortical involvement, which did not conform to the typical cerebral arterial territories, was noted. In one patient, perfusion MR imaging showed slightly increased relative cerebral blood volume, and MR spectroscopy revealed no evidence of abnormal lactate in the affected cerebral cortex. CONCLUSION: Diffusion-weighted MR imaging findings in patients with severe hypoglycemia showed typical lesions in the hippocampus and cerebral cortex, but the caudate nucleus and basal ganglia were involved in only the most severely affected patient. The splenium of the corpus callosum and internal capsule were also abnormal in three patients, and unilateral cortical lesions could be distinguished from acute ischemic stroke by the pattern of involvement and MR angiographic, perfusion, and spectroscopic findings.