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Background The clinical diagnosis of multiple system atrophy (MSA) is challenged by overlapping features with Parkinson's disease (PD) and late-onset ataxias. Additional biomarkers are needed to confirm MSA and to advance the understanding of pathophysiology. Positron emission tomography (PET) imaging of the translocator protein (TSPO), expressed by glia cells, has shown elevations in MSA. Objective In this multicenter PET study, we assess the performance of TSPO imaging as a diagnostic marker for MSA. Methods We analyzed [11 C]PBR28 binding to TSPO using imaging data of 66 patients with MSA and 24 patients with PD. Group comparisons were based on regional analysis of parametric images. The diagnostic readout included visual reading of PET images against clinical diagnosis and machine learning analyses. Sensitivity, specificity, and receiver operating curves were used to discriminate MSA from PD and cerebellar from parkinsonian variant MSA. Results We observed a conspicuous pattern of elevated regional [11 C]PBR28 binding to TSPO in MSA as compared with PD, with "hotspots" in the lentiform nucleus and cerebellar white matter. Visual reading discriminated MSA from PD with 100% specificity and 83% sensitivity. The machine learning approach improved sensitivity to 96%. We identified MSA subtype-specific TSPO binding patterns. Conclusions We found a pattern of significantly increased regional glial TSPO binding in patients with MSA. Intriguingly, our data are in line with severe neuroinflammation in MSA. Glia imaging may have potential to support clinical MSA diagnosis and patient stratification in clinical trials on novel drug therapies for an α-synucleinopathy that remains strikingly incurable. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

OBJECTIVEReports on basal ganglia cavernous malformations (BGCMs) are rare. Here, the authors report on their experience in resecting these malformations to offer insight into this infrequent disease subtype.METHODSThe authors retrospectively reviewed a prospectively managed departmental database of all deep-seated cerebral cavernous malformations (CCMs) treated at Stanford between 1987 and 2019 and included for further analysis those with a radiographic diagnosis of BGCM. Moreover, a systematic literature review was undertaken using the PubMed and Web of Science databases.RESULTSThe departmental database search yielded 331 patients with deep-seated CCMs, 44 of whom had a BGCM (13.3%). Headache was the most common presenting sign (53.5%), followed by seizure (32.6%) and hemiparesis (27.9%). Lesion location involved the caudate nucleus in 21.4% of cases compared to 78.6% of cases within the lentiform nucleus. Caudate BGCMs were larger on presentation and were more likely to present to the ependymal surface (p < 0.001) with intraventricular hemorrhage and hydrocephalus (p = 0.005 and 0.007, respectively). Dizziness and diplopia were also more common with lesions involving the caudate. Because of their anatomical location, caudate BGCMs were preferentially treated via an interhemispheric approach and were less likely to be associated with worsening perioperative deficits than lentiform BGCMs (p = 0.006 and 0.045, respectively). Ten patients (25.6%) were clinically worse in the immediate postoperative period, 4 (10.2%) of whom continued to suffer permanent morbidity at the last follow-up. A long-term good outcome (modified Rankin Scale [mRS] score 0–1) was attained in 74.4% of cases compared to the 69.2% of patients who had presented with an mRS score 0–1. Relative to their presenting mRS score, 89.8% of patients had an improved or unchanged status at the last follow-up. The median postoperative follow-up was 11 months (range 1–252 months). Patient outcomes after resection did not differ among surgical approaches; however, patients presenting with hemiparesis and lesions involving the globus pallidus or posterior limb of the internal capsule were more likely to suffer neurological deficits during the immediate perioperative period. Patients who had undergone awake surgeries were more likely to suffer neurological decline at the early as well as the late follow-up. When adjusting for awake craniotomy as a potential confounder of lesion location, a BGCM involving the posterior limb was predictive of developing early postoperative deficits, but this finding did not persist at the long-term follow-up.CONCLUSIONSSurgery is a safe and effective treatment modality for managing BGCMs, with an estimated long-term permanent morbidity rate of around 10%.

Introduction: Wilson’s disease (WD) is an autosomal recessive disorder involving cellular copper transport. A defect in biliary excretion leads to accumulation of copper in the liver, causing progressive liver injury and cirrhosis. Approximately 1 in 40,000 people have Wilson’s Disease. It affects both men and women equally. Symptoms appear between ages 5 and 35. Case Presentation: This is a case of 17 years old girl came with complained of dysphagia, irritability, breathlessness, weakness in both upper and lower limb at left side for 6 months. She had difficulty in going up and coming down a staircase and in getting up from the squatting position. She could walk with support for the first 6 months of his illness but later, had required crutches. After her admission diagnostic evaluation was done, and in the findings was chest x-ray was done which was normal. ECG showing normal sinus rate and rhythm. Ophthalmology call was done and slit lamp examination was noted which was showing KF ring was present bilaterally. MRI brain suggested symmetrical altered signal intensity lesion in bilateral thalami and lentiform nucleus, midbrain and pons appearing hyper intense on T2/FLAIR sequences in bilateral gangliocapsular region (mainly in putamen) and thalami as well as midbrain and dorsal aspect of pons as described above. A possibility of Wilson’s Disease can be considered, blood test show that Hb was decrease that is -9.7gm%, S.G.O.T was 34.0U/L, S.G.P.T was 32.0U/L, was normal at the time of discharge early ambulation, nutrition, psychological support was given. Therapeutic interventions and outcome: In the present case received syndopa 110/4, BD, orally. Tablet Zinc, OD, orally. Tablet Pan D, OD, orally., inj. Dexamethasone 2 mg I.V, T.D.S., Syrup Sumax, 10ml, BD. Now the patient condition is stable. Conclusion: This presented case of Wilson’s Disease is a rare disease condition. It is an autosomal recessive disease.In this case study, author mainly focus on expert medical management and excellent nursing care which leads to fast recovery of patient. After conversation with patient her response was positive and after nursing management and treatment she was discharged with satisfaction of recovery.

BACKGROUND The literature on white matter anatomy underlying the human orbitofrontal cortex (OFC) is scarce in spite of its relevance for glioma surgery. OBJECTIVE To describe the anatomy of the OFC and of the underlying white matter fiber anatomy, with a particular focus on the surgical structures relevant for a safe and efficient orbitofrontal glioma resection. Based on anatomical and radiological data, the secondary objective was to describe the growth pattern of OFC gliomas. METHODS The study was performed on 10 brain specimens prepared according to Klingler's protocol and dissected using the fiber microdissection technique modified according to U.T., under the microscope at high magnification. RESULTS A detailed stratigraphy of the OFC was performed, from the cortex up to the frontal horn of the lateral ventricle. The interposed neural structures are described together with relevant neighboring topographic areas and nuclei. Combining anatomical and radiological data, it appears that the anatomical boundaries delimiting and guiding the macroscopical growth of OFC gliomas are as follows: the corpus callosum superiorly, the external capsule laterally, the basal forebrain and lentiform nucleus posteriorly, and the gyrus rectus medially. Thus, OFC gliomas seem to grow ventriculopetally, avoiding the laterally located neocortex. CONCLUSION The findings in our study supplement available anatomical knowledge of the OFC, providing reliable landmarks for a precise topographical diagnosis of OFC lesions and for perioperative orientation. The relationships between deep anatomic structures and glioma formations described in this study are relevant for surgery in this highly interconnected area.

Summary Background Acute extrapyramidal movement disorders in dialysis patients are rare, inconsistently defined and have uncertain aetiology and prognosis. Aim Define diagnostic criteria, prognosis and risk factors Design and Methods Retrospective case series review of 20 patients (14 female, mean age 62 years) receiving dialysis for a median of 15 (interquartile range 4–35) months who presented with acute parkinsonism (AP = 11) or chorea/athetosis (CA = 9). Results All patients had type 2 diabetes (HbA1c 6.8 ± 1.0) and had received metformin. Lactic acidosis was present in 2 patients at presentation and serum lactate was elevated in 7/15 patients tested. No patient had abnormal copper or thyroid metabolism and 5/8 patients tested returned marginal abnormalities in heavy metal screening. Magnetic resonance imaging (MRI) revealed characteristic bilateral symmetric T2 hyperintensity of the basal ganglia (BG), predominantly putamen and globus pallidus (the lentiform nucleus) and more extensive involvement of the external and internal capsules in patients with AP presentation. Post-mortem demonstrated cytotoxic necrosis of the BG. Therapy included thiamine, intensive dialysis and cessation of metformin. Two patients died acutely, nine recovered and nine had residual symptoms. Median survival did not differ by presentation: AP 24 [95% confidence interval (CI) 21–27] and CA 33 (95% CI 32–35) months, P = 0.21. Conclusions There are two distinct clinical extrapyramidal movement disorders associated with specific diagnostic MRI imaging that support the diagnosis of the extrapyramidal syndromes of chronic kidney disease and dialysis. The associations with diabetes, metformin and metabolic acidosis suggest a common pathogenic mechanism but require additional study. Early recognition and treatment may improve outcomes.

Background Tourette syndrome (TS) is a neurodevelopmental disorder defined by the continual presence of primary motor and vocal tics. Grey matter abnormalities have been identified in numerous studies of TS, but conflicting results have been reported. This study was an unbiased statistical meta-analysis of published neuroimaging studies of TS structures. Methods A voxel quantitative meta-analysis technique called activation likelihood estimation (ALE) was used. The meta-analysis included six neuroimaging studies involving 247 TS patients and 236 healthy controls. A statistical threshold of p Results We found that grey matter volumes were significantly increased in the bilateral thalamus, right hypothalamus, right precentral gyrus, left postcentral gyrus, left inferior parietal lobule, right lentiform nucleus, and left insula of TS patients compared to those of healthy controls. In contrast, grey matter volumes were significantly decreased in the bilateral postcentral gyrus, bilateral anterior cingulate, bilateral insula, left posterior cingulate and left postcentral gyrus of TS patients compared to those of healthy controls. Conclusions Our present meta-analysis primarily revealed significant increases in grey matter volumes in the thalamus and lentiform nucleus, and decreased grey matter volumes in the anterior cingulate gyrus, of TS patients compared to those in healthy controls. Most of these identified regions are associated with cortico-striato-thalamo-cortical circuits. Further studies with larger sample sizes are needed to confirm these changes in grey matter volumes in TS patients.

Objective. To explore altered regional neuronal activity in patients with nonarteritic anterior ischemic optic neuropathy (NAION) and its correlation with clinical performances using the regional homogeneity (ReHo) method, which is based on resting-state functional magnetic resonance imaging (fMRI). Method. Thirty-one patients with NAION (20 males, 11 females) and 31 age- and sex-matched normal controls (NCs) (20 males, 11 females) were enrolled in the study. All patients underwent ophthalmic examination, including eyesight, intraocular pressure measurement, optimal coherence tomography (OCT), visual field analysis, and fMRI scans. After ReHo was calculated, we investigated group differences in results between the patients and NCs. We analyzed the relationship between ReHo values for different brain regions in patients with NAION and intraocular pressure, visual field analysis, and OCT. A receiver operating characteristic (ROC) curve was used to assess the diagnostic ability of the ReHo method. Results. Compared with NCs, patients with NAION exhibited higher ReHo values in the left middle frontal gyrus, left middle cingulate gyrus, left superior temporal gyrus, and left inferior parietal lobule. Additionally, they exhibited lower ReHo values in the right lingual gyrus, left putamen/lentiform nucleus, and left superior parietal lobule. ReHo values in the left superior parietal lobule were negatively correlated with right retinal nerve fiber layer values (r=−0.462, P=0.01). The area under the ROC curve for each brain region indicated that the ReHo method is a credible means of diagnosing patient with NAION. Conclusion. NAION was primarily associated with dysfunction in the default mode network, which may reflect its underlying neural mechanisms.

Recent evidence of gadolinium deposition in the brain has raised safety concerns. Iron oxide nanoparticles are re-emerging as promising alternative MR contrast agents, because the iron core can be metabolized. However, long-term follow up studies of the brain after intravenous iron oxide administration have not been reported thus far. In this study, we investigated, if intravenously administered ferumoxytol nanoparticles are deposited in porcine brains. Methods: In an animal care and use committee-approved prospective case-control study, ten Gottingen minipigs received either intravenous ferumoxytol injections at a dose of 5 mg Fe/kg (n=4) or remained untreated (n=6). Nine to twelve months later, pigs were sacrificed and the brains of all pigs underwent ex vivo MRI at 7T with T2 and T2*-weighted sequences. MRI scans were evaluated by measuring R2* values (R2*=1000/T2*) of the bilateral caudate nucleus, lentiform nucleus, thalamus, dentate nucleus, and choroid plexus. Pig brains were sectioned and stained with Prussian blue and evaluated for iron deposition using a semiquantitative scoring system. Data of ferumoxytol exposed and unexposed groups were compared with an unpaired t-test and a Mann-Whitney U test. Results: T2 and T2* signal of the different brain regions was not visually different between ferumoxytol exposed and unexposed controls. There were no significant differences in R2* values of the different brain regions in the ferumoxytol exposed group compared to controls (p>0.05). Prussian blue stains of the same brain regions, scored according to a semiquantitative score, were not significantly different either between the ferumoxytol exposed group and unexposed controls (p>0.05). Conclusions: Our study shows that intravenous ferumoxytol doses of 5-10 mg Fe/kg do not lead to iron deposition in the brain of pigs. We suggest iron oxide nanoparticles as a promising alternative for gadolinium-enhanced MRI.

BackgroundPrevious studies have found that elevated copper levels induce oxidation, which correlates with the occurrence of major depressive disorder (MDD). However, the mechanism of abnormal cerebral metabolism of MDD patients remains ambiguous. The main function of the enzyme ATPase copper-transporting alpha (ATP7A) is to transport copper across the membrane to retain copper homeostasis, which is closely associated with the onset of mental disorders and cognitive impairment. However, less is known regarding the association of ATP7A expression in MDD patients.MethodsA total of 31 MDD patients and 21 healthy controls were recruited in the present study. Proton magnetic resonance spectroscopy was used to assess the concentration levels of N-acetylaspartate, choline (Cho), and creatine (Cr) in brain regions of interest, including prefrontal white matter (PWM), anterior cingulate cortex (ACC), thalamus, lentiform nucleus, and cerebellum. The mRNA expression levels of ATP7A were measured using polymerase chain reaction (SYBR Green method). The correlations between mRNA expression levels of ATP7A and/or ceruloplasmin levels and neuronal biochemical metabolite ratio in the brain regions of interest were evaluated.ResultsThe decline in the mRNA expression levels of ATP7A and the increase in ceruloplasmin levels exhibited a significant correlation in MDD patients. In addition, negative correlations were noted between the decline in mRNA expression levels of ATP7A and the increased Cho/Cr ratios of the left PWM, right PWM, and right ACC in MDD patients. A positive correlation between elevated ceruloplasmin levels and increased Cho/Cr ratio of the left PWM was noted in MDD patients.ConclusionsThe findings suggested that the decline in the mRNA expression levels of ATP7A and the elevated ceruloplasmin levels induced oxidation that led to the disturbance of neuronal metabolism in the brain, which played important roles in the pathophysiology of MDD. The decline in the mRNA expression levels of ATP7A and the elevated ceruloplasmin levels affected neuronal membrane metabolic impairment in the left PWM, right PWM, and right ACC of MDD patients.

Minimal hepatic encephalopathy (MHE) is characterized by diffuse abnormalities in cerebral structure, such as reduced cortical thickness and altered brain parenchymal volume. This study tested the potential of gray matter (GM) volumetry to differentiate between cirrhotic patients with and without MHE using a support vector machine (SVM) learning method. High-resolution, T1-weighted magnetic resonance images were acquired from 24 cirrhotic patients with MHE and 29 cirrhotic patients without MHE (NHE). Voxel-based morphometry was conducted to evaluate the GM volume (GMV) for each subject. An SVM classifier was employed to explore the ability of the GMV measurement to diagnose MHE, and the leave-one-out cross-validation method was used to assess classification accuracy. The SVM algorithm based on GM volumetry achieved a classification accuracy of 83.02%, with a sensitivity of 83.33% and a specificity of 82.76%. The majority of the most discriminative GMVs were located in the bilateral frontal lobe, bilateral lentiform nucleus, bilateral thalamus, bilateral sensorimotor areas, bilateral visual regions, bilateral temporal lobe, bilateral cerebellum, left inferior parietal lobe, and right precuneus/posterior cingulate gyrus. Our results suggest that SVM analysis based on GM volumetry has the potential to help diagnose MHE in cirrhotic patients.

Chorea‑hemiballismus is the most common movement disorder, and it commonly results from vascular lesions in the subthalamic nucleus. Rarely, it can result from lacunar infarcts. Here is illustrated a 95‑year‑old male with sudden‑onset left-sided chorea‑hemiballismus of 2 weeks, without any other neurological deficit, who was not a diabetic patient and had no form of acidosis or electrolyte abnormality. Computerized tomogram, serial 32 slides pre‑ and post‑contrast images showed small non-enhancing, hypodense foci within the right frontal subcortical region adjacent to the frontal horn of the right lateral ventricle as well as within the lentiform nucleus. There were no mass effects seen. A diagnosis of hemiballismus from subcortical lacunar infarct was made. The hemiballismus was controlled after 2 weeks of haloperidol and clonazepam therapy, among others, with a substantial reduction in the abnormal movements. Hemiballismus attributable to subcortical lacunar infarcts though rare was presented. Keywords: Hemiballismus, lacunar infarcts, lentiform nucleus, Nigeria, subcortical infarcts, subthalamic nucleus

Introduction: Cognitive decline is the core schizophrenia symptom, which is now well accepted. Holding a role in various aspects of cognition, lentiform nucleus (putamen and globus pallidus) dysfunction contributes to the psychopathology of this disease. However, the effects of lentiform nucleus function on cognitive impairments in schizophrenia are yet to be investigated.Objectives: We aim to detect the fractional amplitude of low-frequency fluctuation (fALFF) alterations in patients with schizophrenia, and examine how their behavior correlates in relation to the cognitive impairments of the patients.Methods: All participants underwent magnetic resonance imaging (MRI) and cognitive assessment (digit span and digit symbol coding tests). Screening of brain regions with significant changes in fALFF values was based on analysis of the whole brain. The data were analyzed between Jun 2020 and Mar 2021. There were no interventions beyond the routine therapy determined by their clinicians on the basis of standard clinical practice.Results: There were 136 patients (75 men and 61 women, 24.1 ± 7.4 years old) and 146 healthy controls (82 men and 64 women, 24.2 ± 5.2 years old) involved in the experiments seriatim. Patients with schizophrenia exhibited decreased raw scores in cognitive tests (p < 0.001) and increased fALFF in the bilateral lentiform nuclei (left: 67 voxels; x = −24, y = −6, z = 3; peak t-value = 6.90; right: 16 voxels; x = 18, y = 0, z = 3; peak t-value = 6.36). The fALFF values in the bilateral lentiform nuclei were positively correlated with digit span-backward test scores (left: r = 0.193, p = 0.027; right: r = 0.190, p = 0.030), and the right lentiform nucleus was positively correlated with digit symbol coding scores (r = 0.209, p = 0.016).Conclusion: This study demonstrates that cognitive impairments in schizophrenia are associated with lentiform nucleus function as revealed by MRI, involving working memory and processing speed.

Acute to subacute onset chorea is commonly due to stroke, metabolic, post-infectious, and autoimmune disorders. Chorea in diabetes (DM) patient is mostly due to hyperglycemia, and few cases were reported due to hypoglycemia. The mechanism at the cellular level is still evolving. The MRI changes in diabetic chorea patients are variably reported. It is important to discuss our patient with hypoglycemic hemichorea because of unique features like low BMI, recently diagnosed DM on sulphonylurea with a history of hypoglycemic episodes, and acute left hemichorea that was controlled with neuroleptics, and her MRI brain showed reversible lentiform nucleus T1 hyperintensity without diffusion restriction and blooming on SWI.

Parkinson disease (PD) is a heterogeneous neurodegenerative disorder. Dopamine transporter imaging using 123I-2β-carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl)-nortropane (FP-CIT) and noradrenergic cardiac imaging using 123I-meta-iodobenzylguanidine (MIBG) have been used in combination or separately to study PD patients. Published results regarding uptake of the 2 tracers in each motor subtype are fairly abundant and mostly in agreement. However, data on the intrasubject association between dopaminergic and noradrenergic systems in PD patients are relatively scant and vary. We aimed to assess the intrasubject relationship between striatal dopamine transporter density using a PET tracer and cardiac sympathetic innervation in tremor-dominant subtype (TD) and akinetic-rigid subtype (AR) of PD. This study has a cross-sectional design. Thirty-one patients with early PD (17 TD/14 AR) who underwent both 123I-MIBG cardiac scintigraphy and 18F-FP-CIT PET/CT were retrospectively selected. We assessed the relationship between heart-to-mediastinum ratio (H/M) of 123I-MIBG and specific (striatal)-to-nonspecific (cerebellar) dopamine transporter binding ratio (S/N) measured from 4 separate regions-of-interest (bilateral caudate nuclei and lentiform nuclei) of 18F-FP-CIT in each motor subtype. S/N of all 4 striatal regions were significantly lower in the AR subgroup than in the TD subgroup. H/M was not significantly different. There was a significant intrasubject correlation between H/M and S/N of the lentiform nucleus in AR-PD but no correlation between H/M and any of 4 S/N in TD-PD. Our data suggest a coupled degeneration of nigrostriatal dopaminergic and myocardial sympathetic denervation in AR subtype, but not in TD subtype, of early PD patients. These different results between the 2 motor subtypes likely reflects the heterogeneous pathophysiology of PD.

Background: Schistosomiasis is a helminthic infestation of public health importance. Central nervous system involvement is a severe but under reported complication. Case report: This was a case of neuroschistosomiasis in an eight year old male who lived in a rural community in Enugu, South East Nigeria. Patient had presented with terminal haematuria, headache and neurological complications. Urine microscopy showed many ova of schistosoma haematobium while ultrasound showed urinary bladder base changes that appeared irregular with hyper echoic mucosa and focal mucosal thickening on the right side. Computed tomography of the brain revealed a hyper dense oval area surrounded by an irregular hypo dense area in the left parietal region overlying the left thalamus and lentiform nucleus. Patient showed good clinical response to praziquantel and corticosteroid. Conclusion: Neuroschistosomiasis is a rare complication of schistosomiasis. A high index of suspicion should be entertained in endemic areas as early treatment with praziquantel and steroids has good outcome.

Over the past few decades, it has been recognized that traumatic brain injury (TBI) may result in various movement disorders. However, moderate or mild TBI only rarely causes persistent post-traumatic movement disorders. In the present report, we describe a case of secondary tremor due to a mild head injury with a transitory loss of consciousness. A 26-year-old man developed an isolated rest tremor of the hands and legs without other neurologic signs. The interval between the head trauma and the onset of the symptoms was 4 months. Neuroimaging studies reveled gliosis in the lentiform nucleus. Haloperidol administration resulted in tremor reduction. A rest tremor, similar to essential tremor, can be a rare complication of head trauma. Haloperidol may be an effective and safe treatment modality for post-traumatic tremor. Further studies are needed to clarify the optimal drug for the treatment of post-traumatic tremor.

Neuropsychiatric disease has polygenic determinants but is often precipitated by environmental pressures, including adverse perinatal events. However, the way in which genetic vulnerability and early-life adversity interact remains obscure. We hypothesised that the extreme environmental stress of prematurity would promote neuroanatomic abnormality in individuals genetically vulnerable to psychiatric disorders. In 194 unrelated infants (104 males, 90 females), born before 33 weeks of gestation (mean gestational age 29.7 weeks), we combined Magnetic Resonance Imaging with a polygenic risk score (PRS) for five psychiatric pathologies to test the prediction that: deep grey matter abnormalities frequently seen in preterm infants are associated with increased polygenic risk for psychiatric illness. The variance explained by the PRS in the relative volumes of four deep grey matter structures (caudate nucleus, thalamus, subthalamic nucleus and lentiform nucleus) was estimated using linear regression both for the full, mixed ancestral, cohort and a subsample of European infants. Psychiatric PRS was negatively associated with lentiform volume in the full cohort (β = −0.24, p = 8 × 10−4) and a European subsample (β = −0.24, p = 8 × 10−3). Genetic variants associated with neuropsychiatric disease increase vulnerability to abnormal lentiform development after perinatal stress and are associated with neuroanatomic changes in the perinatal period.

Lacunar strokes are a common type of ischemic stroke. They are known to have long-term cognitive deficits, but the influencing factors are still largely unknown. We investigated if the location of the index lacunar stroke or regional WMH and their change at 1 year could predict the cognitive performance at 1 and 3 years post-stroke in lacunar stroke patients. We used lacunar lesion location and WMH-segmented data from 118 patients, mean age 64.9 who had a brain MRI scan soon after presenting with symptoms, of which 88 had a repeated scan 12 months later. Premorbid intelligence (National Adult Reading Test) and current intelligence [Addenbrooke's Cognitive Exam-Revised (ACE-R)] were measured at 1, 12, and 36 months after the stroke. ANCOVA analyses adjusting for baseline cognition/premorbid intelligence, vascular risk factors, age, sex and total baseline WMH volume found that the recent small subcortical infarcts (RSSI) in the internal/external capsule/lentiform nucleus and centrum semiovale did not predict cognitive scores at 12 and 36 months. However, RSSI location moderated voxel-based associations of WMH change from baseline to 1 year with cognitive scores at 1 and 3 years. WMH increase in the external capsule, intersection between the anterior limb of the internal and external capsules, and optical radiation, was associated with worsening of ACE-R scores 1 and 3 years post-stroke after accounting for the location of the index infarct, age and baseline cognition.

Lacunar strokes are a common type of ischemic stroke. They are associated with long-term disability, but the factors affecting the dynamic of the infarcted lesion and the brain imaging features associated with them, reflective of small vessel disease (SVD) severity, are still largely unknown. We investigated whether the distribution, volume and 1-year evolution of white matter hyperintensities (WMH), one of these SVD features, relate to the extent and location of these infarcts, accounting for vascular risk factors. We used imaging and clinical data from all patients [n = 118, mean age 64.9 (SD 11.75) years old] who presented to a regional hospital with a lacunar stroke syndrome within the years 2010 and 2013 and consented to participate in a study of stroke mechanisms. All patients had a brain MRI scan at presentation, and 88 had another scan 12 months after. Acute lesions (i.e., recent small subcortical infarcts, RSSI) were identified in 79 patients and lacunes in 77. Number of lacunes was associated with baseline WMH volume (B = 0.370, SE = 0.0939, P = 0.000174). RSSI volume was not associated with baseline WMH volume (B = 3.250, SE = 2.117, P = 0.129), but predicted WMH volume change (B = 2.944, SE = 0.913, P = 0.00184). RSSI location was associated with the spatial distribution of WMH and the pattern of 1-year WMH evolution. Patients with the RSSI in the centrum semiovale (n = 33) had significantly higher baseline volumes of WMH, recent and old infarcts, than patients with the RSSI located elsewhere [median 33.69, IQR (14.37 50.87) ml, 0.001 ≤ P ≤ 0.044]. But patients with the RSSI in the internal/external capsule/lentiform nucleus experienced higher increase of WMH volume after a year [n = 21, median (IQR) from 18 (11.70 31.54) ml to 27.41 (15.84 40.45) ml]. Voxel-wise analyses of WMH distribution in patients grouped per RSSI location revealed group differences increased in the presence of vascular risk factors, especially hypertension and recent or current smoking habit. In our sample of patients presenting to the clinic with lacunar strokes, lacunar strokes extent influenced WMH volume fate; and RSSI location and WMH spatial distribution and dynamics were intertwined, with differential patterns emerging in the presence of vascular risk factors. These results, if confirmed in wider samples, open potential avenues in stroke rehabilitation to be explored further.

Highlights • Cohort study of neonatal encephalopathy using continuous glucose monitoring. • Higher glucose on day 1 associated with widespread changes in brain microstructure. • Lower glucose not associated with brain microstructural changes. • No changes in MR spectroscopy found related to higher or lower glucose., Objective To identify how alterations in glucose levels are associated with regional brain injury in neonatal encephalopathy. Methods This was a prospective cohort study of 102 newborns with neonatal encephalopathy, with continuous glucose monitoring for 72 h. 97 (95%) completed 72 h of therapeutic hypothermia. Brain imaging around day 5 of life included diffusion tensor imaging and MR spectroscopy. Regions of interest were placed for both DTI and MR spectroscopy, and tractography of the optic radiation and corticospinal tract were evaluated. Linear regression models related each MR metric with minimum and maximum glucose values during each day of life, adjusting for 5-minute Apgar scores and umbilical artery pH. Results Higher maximum glucose levels on the first day of life were associated with widespread changes in mean diffusivity in the anterior and posterior white matter, splenium of the corpus callosum, lentiform nucleus, pulvinar nucleus of the thalamus, posterior limb of the internal capsule, and optic radiations, thus including regions traditionally associated with hypoxia–ischemia or hypoglycemia. No associations were found between lower minimum glucose levels and DTI changes in any regions tested, or between glucose levels and MR spectroscopy. Conclusions In this cohort of neonatal encephalopathy with therapeutic hypothermia, higher maximal glucose on the first day of life was associated with widespread microstructural changes, but lower minimum glucose levels were not associated with changes in any of the regions tested. Long-term follow-up will determine if imaging findings translate to long-term outcomes.

In this study we used a combination of measures including regional cerebral blood flow (rCBF) and heart rate variability (HRV) to investigate brain–heart correlates of longitudinal baseline changes of chronic low back pain (cLBP) after osteopathic manipulative treatment (OMT). Thirty-two right-handed patients were randomised and divided into 4 weekly session of OMT (N = 16) or Sham (N = 16). Participants aged 42.3 ± 7.3 (M/F: 20/12) with cLBP (duration: 14.6 ± 8.0 m). At the end of the study, patients receiving OMT showed decreased baseline rCBF within several regions belonging to the pain matrix (left posterior insula, left anterior cingulate cortex, left thalamus), sensory regions (left superior parietal lobe), middle frontal lobe and left cuneus. Conversely, rCBF was increased in right anterior insula, bilateral striatum, left posterior cingulate cortex, right prefrontal cortex, left cerebellum and right ventroposterior lateral thalamus in the OMT group as compared with Sham. OMT showed a statistically significant negative correlation between baseline High Frequency HRV changes and rCBF changes at T2 in the left posterior insula and bilateral lentiform nucleus. The same brain regions showed a positive correlation between rCBF changes and Low Frequency HRV baseline changes at T2. These findings suggest that OMT can play a significant role in regulating brain–heart interaction mechanisms.

Dysphagia is common in patients with middle cerebral artery (MCA) infarctions and associated with malnutrition, pneumonia, and mortality. Besides bedside screening tools, brain imaging findings may help to timely identify patients with swallowing disorders. We investigated whether the Alberta stroke program early CT score (ASPECTS) allows for the correlation of distinct ischemic lesion patterns with dysphagia. We prospectively examined 113 consecutive patients with acute MCA infarctions. Fiberoptic endoscopic evaluation of swallowing (FEES) was performed within 24 h after admission for validation of dysphagia. Brain imaging (CT or MRI) was rated for ischemic changes according to the ASPECT score. 62 patients (54.9%) had FEES-proven dysphagia. In left hemispheric strokes, the strongest associations between the ASPECTS sectors and dysphagia were found for the lentiform nucleus (odds ratio 0.113 [CI 0.028–0.433; p = 0.001), the insula (0.275 [0.102–0.742]; p = 0.011), and the frontal operculum (0.280 [CI 0.094–0.834]; p = 0.022). A combination of two or even all three of these sectors together increased relative dysphagia frequency up to 100%. For right hemispheric strokes, only non-significant associations were found which were strongest for the insula region. The distribution of early ischemic changes in the MCA territory according to ASPECTS may be used as risk indicator of neurogenic dysphagia in MCA infarction, particularly when the left hemisphere is affected. However, due to the exploratory nature of this research, external validation studies of these findings are warranted in future. Electronic supplementary material The online version of this article (10.1007/s00455-020-10204-0) contains supplementary material, which is available to authorized users.

Objective: To explore the clinical manifestation, diagnosis, therapy, and mechanism of hemichorea associated with non-ketotic hyperglycemia (HC-NH) so as to enhance awareness and avoid misdiagnosis or missed diagnosis of the disease. Methods: A case of HC-NH was reported and reviewed in terms of the clinical features, diagnosis and treatment. Results: Hemichorea associated with non-ketotic hyperglycemia is a rare complication of diabetes mellitus, which is commonly seen in elderly women with poorly-controlled diabetes. The condition is characterized by non-ketotic hyperglycemia, unilateral involuntary choreiform movements, and contralateral basal ganglia hyper-intensity by T1-weighted MR imaging or high density on CT scans. Blood glucose control is the basal treatment, in combination with dopamine receptor antagonists and benzodiazepine sedative, in controlling hemichorea. Conclusion: In clinical practice, the possibility of unilateral chorea should be considered for diabetic patients with poor blood glucose control.

A 65-year-old woman presented to the emergency department with sudden onset of left-sided weakness, headache and vomiting. A cerebral CT showed an acute intracerebral haemorrhage involving the right caudate nucleus and lentiform nucleus with mild midline shift and intraventricular extension. CT angiography did not reveal aneurysm or other vascular anomaly. Conventional cerebral angiography demonstrated a 3 mm right medial lenticulostriate branch aneurysm, arising from the right anterior cerebral artery (ACA). Endovascular treatment was performed from the left internal carotid via the anterior communicating artery into the right ACA. Complete occlusion was achieved with injection of N-butyl-2-cyanoacrylate. The patient had neurological rehabilitation during hospitalisation followed by outpatient physical therapy. Two years later, clinical follow-up demonstrated excellent recovery.

Basal ganglia germinomas are rare and patients are classically young Oriental males presenting with gradually progressive hemiparesis. Although early treatment with chemo-radiotherapy can be curative and significantly improve the quality of life, medical attention is often delayed. A young Chinese male experienced a 6-month history of right hemiparesis with magnetic resonance imaging findings of hemicerebral atrophy and lentiform nucleus microhemorrhage, highly suggestive and early signs of basal ganglia germinomas. No further imaging was performed until 2 years later when he was admitted for acute neurological deterioration and a repeat scan revealed a large infiltrative tumor pathologically confirmed to be a pure germinoma.

Introduction Hyperglycemia may present with neurological symptoms. This rare condition is called non-ketotic hemichorea-hemiballismus (HCHB). When investigating the causes of neurological symptoms in these cases, magnetic resonance imaging (MRI) imaging typically has a hyperintense appearance in T1 sections and a variable intense appearance in T2. Case report A 68-year-old female patient was admitted to the emergency department with speech disorder. Blood sugar elevation and cranial tomography revealed hyperdense appearance in the left caudate nucleus and lentiform nucleus. The patient was hospitalized with a preliminary diagnosis of HCHB. Magnetic resonance in hyperintense T1W sequences in the nucleus caudatus hood and corpus on the left, putamen, globus pallidus, Significant hypointense signal changes were observed in T2W sequences. The patient was admitted to the hospital for treatment and his blood sugar regulation was restored. Conclusion HCHB is a rare condition in emergency. After the regulation of blood sugar, neurological picture regression is observed. Blood sugar levels should be checked and appropriate treatment should be given in patients presenting with neurological symptoms.

Background Studies detailing the anatomy of the basal forebrain (BF) from a neurosurgical perspective are missing. Objective To describe the anatomy of the BF and of the anterior perforated substance (APS), the BF emphasizing surgical useful anatomical relationship between surface landmarks and deep structures. Methods White matter fiber microdissection was performed on 5 brain specimens to analyze the topographic anatomy of the APS and expose layer-by-layer fiber tracts and nuclei of the BF. Results The APS, as identified anatomically, surgically, and neuroradiologically, has clear borders measured 23.3 ± 3.4 mm (19-27) in the mediolateral and 12.5 ± 1.2 mm (11-14) in the anteroposterior directions. A detailed stratigraphy of the BF was performed from the APS up to basal ganglia and thalamus allowing identification and dissection of the main components of the BF (septum, nucleus accumbens, amygdala, innominate substance) and its white matter tracts (band of Broca, extracapsular thalamic peduncle, ventral amygdalohypothalamic fibers). The olfactory trigone together with diagonal gyrus and the APS proper is a relevant superficial landmark for the basal ganglia (inferior to the nucleus accumbens, lateral to the caudate head, and medial to the lentiform nucleus). Conclusion The findings in our study supplement available anatomic knowledge of APS and BF, providing reliable landmarks for precise topographic diagnosis of BF lesions and for intraoperative orientation. Surgically relevant relationships between surface and deep anatomic structures are highlighted offering thus a contribution to neurosurgeons willing to perform surgery in this delicate area.

Chronic obstructive pulmonary disease (COPD) affects a large population and is closely associated with cognitive impairment. However, the mechanisms of cognitive impairment in COPD patients have not been unraveled. This study investigated the change in patterns of intrinsic functional hubs using a degree centrality (DC) analysis. The connectivity between these abnormal hubs with the remaining brain was also investigated using functional connectivity (FC). Nineteen stable patients with COPD and 20 normal controls(NC) underwent functional magnetic resonance imaging (MRI) examinations and clinical and neuropsychologic assessments. We measured the voxel-wise DC across the whole brain gray matter and the seed-based FC between these abnormal hubs in the remaining brain matter; the group difference was calculated. A partial correlation analysis was performed to assess the relationship between the abnormal DC and clinical variables in COPD patients. Compared to NC, the patients with COPD exhibited significantly decreased DC in the right lingual gyrus (LG), bilateral supplementary motor area (SMA), and right paracentral lobule (PCL). A further seed-based FC analysis found that COPD patients demonstrated significantly decreased FC between these abnormal hubs in several brain areas, including the left cerebellum anterior lobe, left lingual gyrus, left fusiform gyrus, right insula, right inferior frontal gyrus, limbic lobe, cingulate gyrus, left putamen, lentiform nucleus, right precuneus, and right paracentral lobule. A partial correlation analysis showed that the decreased DC in the right PCL was positively correlated with the FEV1 and FEV1/FVC, and the decreased DC in the SMA was positively correlated with naming and pH in COPD patients. This study demonstrates that there are intrinsic functional hubs and connectivity alterations that may reflect the aberrant information communication in the brain of COPD patients. These findings may help provide new insight for understanding the mechanisms of COPD-related cognitive impairment from whole brain functional connections. Electronic supplementary material The online version of this article (10.1007/s11682-019-00130-7) contains supplementary material, which is available to authorized users.

According to the Committee on Medical Internal Radiation Dose (MIRD), the absorbed dose for radiopharmaceuticals is obtained by multiplying cumulated activity in source organs by S-values (average absorbed dose rate to target organ per unit activity present in a source organ). S-values are calculated using Monte Carlo simulation from anthropomorphic body or organ models (computational phantoms). In 1999, MIRD Pamphlet No. 15 included S-values for several head and brain structures calculated from a stylized phantom of an adult head and brain. MIRD head and brain phantom was designed with simple geometrical surfaces. This phantom is not completely realistic but it has been the standard to estimate absorbed dose in head and brain structures since its publication. The aim of this work was to use the voxelized Zubal/Evans phantom to calculate S-values of cortical and subcortical structures for the most widely used radionuclides in PET brain studies, and to compare them to MIRD S-values. An input file on Monte Carlo N-Particle (MCNP6) was written from publicly available input decks made by Evans. S-values were calculated for 18F, 11C, and 15O. Caudate nucleus, cerebral cortex, cerebral white matter, cerebellum, lentiform nucleus, and thalamus were considered as source organs. 12 structures (including source organs) were considered as source targets. In order to compare S-values of Zubal/Evans and MIRD, ratios between them were calculated. Results showed that S-values for 15O were higher than 11C and 18F in all cases. The S-values were higher for self-absorbed dose (when the organ source and the organ target were the same). A ratio of 0.22–10.25 was found between S-values from Zubal/Evans and MIRD phantoms. The target organ with the highest ratio was the spinal cord when the cerebellum was the source organ. The highest ratios were obtained with 15O. The best agreement was found when the thalamus was the source organ. We have concluded that while small differences were found in some organs, bigger differences were found in others. These differences could be due to variances in the volume and shape of the computational phantoms, as well as advances in the Monte Carlo simulations.

BackgroundIn the first weeks after liver transplantation about 30% of the patients develop a posttransplant encephalopathy. A posttransplant encephalopathy comprises metabolic-toxic caused symptoms such as disorientation, confusion, hallucinations, cognitive dysfunction and seizures. We hypothesize that alterations of cerebral metabolites before liver transplantation predispose posttransplant encephalopathy development after liver transplantation.Methods31 patients with chronic liver disease underwent magnetic resonance spectroscopy (MRS) before liver transplantation to assess glutamine/glutamate (Glx), myo-Inositol (mI), choline (Cho), creatine/phosphocreatine- and N-acetyl-aspartate/N-acetyl-aspartate-glutamate concentrations in the thalamus, lentiform nucleus and white matter. Of these, 14 patients underwent MRS additionally after liver transplantation. Furthermore, 15 patients received MRS only after liver transplantation. Patients' data were compared to 20 healthy age adjusted controls.ResultsPatients showed significantly increased Glx and decreased mI and Cho concentrations compared to controls before liver transplantation (p≤0.01). The MRS values before liver transplantation of patients with posttransplant encephalopathy showed no significant difference compared to patients without posttransplant encephalopathy. Patients after liver transplantation showed increased Glx concentrations (p≤0.01) compared to controls, however, patients with and without posttransplant encephalopathy did not differ. Patients with posttransplant encephalopathy who underwent MRS before and after liver transplantation showed a significant mI increase in all three brain regions (pConclusionPatients with and without posttransplant encephalopathy showed no significant difference in cerebral metabolites before liver transplantation. However, the paired sub-analysis indicates that the extent of cerebral metabolite alterations in patients with liver cirrhosis might be critical for the development of posttransplant encephalopathy after liver transplantation.

Objective: To examine whether subacute stroke patients would exhibit abnormal dynamic characteristics of brain activity relative to healthy controls (HC) and to investigate whether the altered dynamic regional indexes were associated with clinical behavior in stroke patients. Methods: The dynamic amplitude of low-frequency fluctuations (dALFF) and dynamic regional homogeneity (dReHo) in 42 subacute stroke patients and 55 healthy controls were compared. Correlation analyses between dALFF and dReHo in regions showing significant intergroup differences and clinical scores (i.e., the National Institutes of Health Stroke Scale, Fugl-Meyer assessment and lesion volume size) were conducted in stroke patients. Receiver operating characteristic (ROC) curve analysis was used to determine the potential value of altered dynamic regional indexes to identify stroke patients. Results: Significantly dALFF in the bilateral cerebellum posterior lobe (CPL), ipsilesional superior parietal lobe, ipsilesional inferior temporal gyrus (ITG), the midline supplementary motor area (SMA), ipsilesional putamen and lentiform nucleus were detected in stroke patients compared to HC. Relative to the HC group, the stroke patients showed significant differences in dReHo in the contralesional rectal gyrus, contralesional ITG, contralesional pons, ipsilesional middle frontal gyrus (MFG). Significant correlations between dALFF variability in midline SMA and Fugl-Meyer assessment (FMA) scores or between dReHo variability in the ipsilesional MFG and FMA scores were detected in stroke patients. Furthermore, the ROC curve revealed that dynamic ALFF at SMA and ReHo at ipsilesional MFG might have the potential to distinguish stroke patients. Conclusion: The pattern of intrinsic brain activity variability is altered in stroke patients compared with HC, and dynamic ALFF/ReHo might be potential tools to assess stroke patients' motor function.

Attention deficit hyperactivity disorder (ADHD) occurs more frequently and manifests with greater symptom severity in men than in women. Although studies have implicated basal ganglia dysfunction, the functional connectivity (FC) of the dorsal striatum (DS), particularly in terms of sex differences, has not been fully investigated in ADHD. Here, using resting state fMRI data of a large sample of adults (n = 744; 395 women; 22–36 years) curated from the Human Connectome Project, we performed seed-based correlations for caudate and lentiform nucleus (LN) FC. ADHD symptom severity was quantified with the Achenbach Adult Self-Report ADHD total score as well as inattention and hyperactivity subscores. Imaging data were processed with published routines and evaluated at a corrected threshold. Men showed significantly higher ADHD total score than women. In men, inattention was negatively associated with LN FC with the right superior frontal gyrus. In women, inattention was negatively associated with caudate FC with the right inferior parietal gyrus and positively with LN FC with the left inferior frontal gyrus, and hyperactivity was positively associated with LN FC with a cluster in the dorsal anterior cingulate cortex and supplementary motor area. Sex differences in most of these connectivity patterns were confirmed by slope tests. Further, k-means clustering of FC's identified 3 groups each in men and in women. In men, group 1 showed higher inattention and hyperactivity than both group 2 and 3, and group 2 showed higher inattention than group 3. In women, group 1 showed higher inattention and hyperactivity than group 3 and higher inattention than group 2, and group 2 showed higher hyperactivity than group 3. These findings together suggest sex differences in DS FC as neural markers of ADHD and potentially of ADHD subtypes, with men and women each showing altered FC predominantly in the executive control and ventral attention/saliency networks.

Aims: In Wilson disease (WD), T2/T2*-weighted (T2*w) MRI frequently shows hypointensity in the basal ganglia that is suggestive of paramagnetic deposits. It is currently unknown whether this hypointensity is related to copper or iron deposition. We examined the neuropathological correlate of this MRI pattern, particularly in relation to iron and copper concentrations. Methods: Brain slices from nine WD and six control cases were investigated using a 7T-MRI system. High resolution T2*w images were acquired and R2* parametric maps were reconstructed using a multi-gradient recalled echo sequence. R2* was measured in the globus pallidus (GP) and the putamen. Corresponding histopathological sections containing the lentiform nucleus were examined using Turnbull iron staining, and double staining combining Turnbull with immunohistochemistry for macrophages or astrocytes. Quantitative densitometry of the iron staining as well as copper and iron concentrations were measured in the GP and putamen and correlated to R2* values. Results: T2*w hypointensity in the GP and/or putamen was apparent in WD cases and R2* values correlated with quantitative densitometry of iron staining. In WD, iron and copper concentrations were increased in the putamen compared to controls. R2* was correlated with the iron concentration in the GP and putamen whereas no correlation was observed for the copper concentration. Patients with more pronounced pathological severity in the putamen displayed increased iron concentration, which correlated with an elevated number of iron-containing macrophages. Conclusions: T2/T2*w hypointensity observed in vivo in the basal ganglia of WD patients is related to iron rather than copper deposits.

A stroke is a sudden loss of brain function which happens due to the disruption of cerebral blood flow. It is characterized by expeditious development of clinical symptoms during the first few minutes or hours after which the symptoms progress and in some patients lead to death. In more than 80% of patients with stroke, the cause is vascular insufficiency and this type of stroke is called ischemic. It is an emergent condition which needs accelerated management so that optimum care and prognosis can be reached. Evaluation of the ischemic stroke is managed with noncontrast computed tomography in most cases, thanks to its widespread availability. A detailed literature search through the Medline and Ebsco databases was performed, using the following terms: (computed tomography) AND (acute ischemic stroke) AND (early signs). We selected original investigations, meta-analyses and reviews from the past 10 years, with accessible full-texts, in the English language, as well as significant reference lists of articles which could contribute to the readers' comprehension. After a detailed review of the literature, we have segregated and described the following early signs in acute ischemic stroke: insular ribbon sign, obscuration of the lentiform nucleus, hyperdense artery sign, hemispherical sulcal effacement and focal hypoattenuation. These signs are related to the consequences of cellular hypoperfusion and cytotoxic edema. The aim of this paper was to review the literature regarding the role of computed tomography in patients with early signs of acute ischemic stroke and to serve as an educational material that illustrates those findings.

Background: Prevalence of cerebrovascular diseases (CVD) is rising in India along with its morbid impact. Till now there is no study from Eastern India to reflect the current scenario of cerebrovascular diseases in this part of world. This study destined to know and analyse several epidemiologic factors behind cerebrovascular diseases in Eastern India. Methods: 108 patients who were admitted with symptoms of stroke were analysed based on several parameters. They were investigated, treated and their outcomes were followed. Results: Correlation between clinical findings and radiological confirmation was much more in case of infarction (65%) compared to haemorrhagic cases (22%). Superior division of the middle cerebral artery was the commonest site get involved in infarction cases and internal capsule area is the most commonly involved area in lacunar infarction almost one third of haemorrhagic patients had intraventricular extension. Most common association with this was thalamic bleeding although most common site of haemorrhage was lentiform nucleus. Among the patients with hemorrhage with intraventricular extension 50% were thalamic bleed and 50% were non thalamic bleed (mainly lentiform nucleus). In hospital mortality rate was found to be highest in non-thalamic group (63%). Overall mortality in non-thalamic bleed was higher though not statistically significant. Conclusions: Haemorrhagic CVD is much more prevalent in this part of world compared to western population. Clinico radiologic correlation is much more in ischaemic infarction hypertension and smoking are two strong associations in cerebrovascular diseases.

Introduction: Wilson's Disease referred to as hepatolenticular degeneration with excessive copper retention involving liver and brain. Hepatic manifestations predominate in children and neuropsychiatric manifestations predominate in adolescents, adults.Case presentation: This is a case report of 24 years old female with long standing difficulty in walking and speech. Computerized tomography imaging showed bilateral symmetrical hypodensity in lentiform nucleus of basal ganglia, thalamus due to copper deposition and slit lamp examination showed Kayser-Fleischer (KF) ring surrounding limbus corneae.Conclusion: Professional counselling and support in addition to drug treatment provide help to patient and her family to overcome their problems and improve the treatment outcome.KYAMC Journal Vol. 5, No.-1, Jul 2014, Page 488-490

Highlights • CT perfusion reveals thalamic hypoperfusion in acute anterior circulation stroke. • This indirect phenomenon is referred to as ipsilateral thalamic diaschisis (ITD). • Quantitative analysis indicates that ITD is a non-binary phenomenon. • ITD is associated with lesion extent and involvement of the lentiform nucleus. • Stroke outcome was not associated with ITD parameters., Purpose Ipsilateral thalamic diaschisis (ITD) refers to the phenomenon of thalamic hypoperfusion or hypometabolism due to a distant cerebral injury. To further investigate the characteristics and spectrum of ITD, we analyzed quantitative measurements of thalamic hypoperfusion in acute anterior circulation stroke. Methods We selected consecutive patients with large-vessel occlusion (LVO) anterior circulation stroke and available CT perfusion (CTP) examination on admission who underwent endovascular thrombectomy. Thalamic perfusion parameters on CTP were tested between ipsi- and contralesional thalamus and ischemic territory. Values were compared with thresholds from CTP analysis software. Associations of thalamic perfusion parameters with acute imaging and clinical data were determined in uni- and multivariate logistic regression analyses. Results Ninety-nine patients were included. All perfusion parameters indicated significant non-ischemic hypoperfusion of the thalamus, not reaching the levels of ischemia in the middle cerebral artery territory due to LVO (all p

We aimed to determine patterns of α-synuclein (α-syn) pathology in multiple system atrophy (MSA) using 70-µm-thick sections of 20 regions of the central nervous system of 37 cases with striato-nigral degeneration (SND) and 10 cases with olivo-ponto-cerebellar atrophy (OPCA). In SND cases with the shortest disease duration (phase 1), α-syn pathology was observed in striatum, lentiform nucleus, substantia nigra, brainstem white matter tracts, cerebellar subcortical white matter as well as motor cortex, midfrontal cortex, and sensory cortex. SND with increasing duration of disease (phase 2) was characterized by involvement of spinal cord and thalamus, while phase 3 was characterized by involvement of hippocampus and amygdala. Cases with the longest disease duration (phase 4) showed involvement of the visual cortex. We observed an increasing overlap of α-syn pathology with increasing duration of disease between SND and OPCA, and noted increasingly similar regional distribution patterns of α-syn pathology. The GBA variant, p.Thr408Met, was found to have an allele frequency of 6.94% in SND cases which was significantly higher compared with normal (0%) and other neurodegenerative disease pathologies (0.74%), suggesting that it is associated with MSA. Our findings indicate that SND and OPCA show distinct early foci of α-syn aggregations, but increasingly converge with longer disease duration to show overlapping patterns of α-syn pathology.