Basal Ganglia Lesions Research Articles

Overview of The Head CT-Scan in Stroke Patients who was Treated at Muhammadiyah Hospital Palembang

Background: Stroke is a collection of symptoms of neurological deficits due to sudden acute disturbance of brain function, both focal and global, caused by blockage or rupture of arteries and veins and proven by imaging and/or reflex pathology. Using CT-Scan, the description of acute phase stroke can be easier and can determine the appropriate treatment criteria for stroke. Purposes: To determine characteristic Head CT-Scan image of stroke patients hospitalized in Muhammadiyah Palembang Hospital. Methods: This study was conducted in a retrospective descriptive manner and obtained a sample of 41 stroke patients according to the inclusion and exclusion criteria using total sampling. Result: In this study, there were 28 patients (68.2%) with ischemic stroke and 13 patients (31.7%) with hemorrhagic stroke. Head CT-Scan image of ischemic stroke shows basal ganglia lesion (28.5%) and the right hemisphere lesions (57.1%) as the most predominant area. Head CT-Scan image of hemorrhagic stroke (58.3%) had intracerebral hemorrhage with the dominant thalamic lesion area (66.6%) and the right hemisphere (58.3%) had the most lesions with 8 patients (66, 6%) had a midline shift. Stroke patients were treated as experienced by the elderly (90.2%) and were dominated by the female sex (63.4%) and are dominated by clinical symptoms of hemiparesis (29.2%). Conclusion: Stroke patients at Muhammadiyah Palembang hospital dominated by ischemic stroke and on head CT Scan image predominantly shows lesions on the right cerebral hemisphere.

PALLIDOTOMY IN PARKINSONIAN DISEASE: THE RENAISSANCE

ABSTRACT:
 BACKGROUND & OBJECTIVE: To assess the results of pallidotomy in Parkinson’s disease, and its effect on improving the lifestyle of the patients and cost-effectiveness.
 METHODOLOGY: A descriptive study was conducted at Neuro-Spinal & Cancer Care Institute, Karachi from June 2014 to January 2020. Patients who were known case of Parkinson’s disease refractory to medication and developed side effects to medication were included in the study and patients with previous brain surgery, associated brain disorders like Alzheimer’s disease, basal ganglia lesion, brain trauma were excluded. All patients were treated by pallidotomy on the contralateral side. The significance of the difference between groups to compare between the pre-op or post-op treatments was calculated through non-parametric assessment Kruskal-Wallis tests. 
 RESULTS: The mean age of the patients was around 57 years. There were 34(81%) male and 8(19%) female patients’ Maximum number of patients who were more than 45 years, were having a left-sided proportion. More male patients were having a left-sided proportion as compared to female patients. The majority of patients (57.5%) were having dyskinesia as q primary symptom. A significant difference (p-value <0.001) existed in pre & post-operative UPDRS-III scores. A significant difference (p-value <0.001) also existed between on & off medications UPDRS- III (pre-op/post-op) scores.
 CONCLUSION: The result of pallidotomy is promising especially for unilateral pallidotomy to minimize the risk of cognition and speech disorder and long-term follow-up is needed to prove the statement further. Currently, pallidotomy is associated with minimal complications, more effective, and improving the quality of life of Parkinsonian patients.

Domains of Health-Related Quality of Life Are Associated With Specific Deficits and Lesion Locations in Chronic Aphasia

Background Health-related quality of life (HRQL) in stroke survivors is related to numerous factors, but more research is needed to delineate factors related to HRQL in people with aphasia. Objective To examine the relationship between HRQL and demographic factors, impairment-based measures, and lesion characteristics in chronic aphasia. Methods A total of 41 left-hemisphere stroke survivors with aphasia underwent cognitive testing and magnetic resonance imaging. To address relationships with demographic and impairment-based measures, test scores were entered into a principal component analysis (PCA) and multiple linear regression was performed for overall and domain (physical, communication, psychosocial) scores of the Stroke and Aphasia Quality of Life Scale (SAQOL-39g). Independent variables included factor scores from the PCA, motricity, lesion volume, depressed mood, and demographic variables. To address relationships with lesion location, multivariate support vector regression lesion-symptom mapping (SVR-LSM) was used to localize lesions associated with SAQOL-39g scores. Results The PCA yielded 3 factors, which were labeled Language Production, Nonlinguistic Cognition, and Language Comprehension. Multiple linear regression revealed that depression symptoms predicted lower SAQOL-39g average and domain scores. Lower motricity scores predicted lower SAQOL-39g average and physical scores, and lower Language Production factor scores predicted lower communication scores. SVR-LSM demonstrated that basal ganglia lesions were associated with lower physical scores, and inferior frontal lesions were associated with lower psychosocial scores. Conclusions HRQL in chronic left-hemisphere stroke survivors with aphasia relates to lesion location, depression symptoms, and impairment-based measures. This information may help identify individuals at risk for specific aspects of low HRQL and facilitate targeted interventions to improve well-being.

Overlapping syndrome of anti-N-methyl-D-aspartate receptor encephalitis and anti-myelin oligodendrocyte glycoprotein inflammatory demyelinating diseases: A distinct clinical entity?

BackgroundThe co-existence of anti-N-methyl-D-aspartate receptor encephalitis (NMDARe) and anti-myelin oligodendrocyte glycoprotein (MOG) antibody disease has sparsely been reported, which needs to be investigated. MethodAmong the patients with NMDARe in Xuanwu Hospital, MOG antibody disease and NMDARe overlapping syndrome (MNOS) were retrospectively identified. We combined our data with those from previously reported cases to characterize this new entity. ResultThere were 45 patients with MNOS with a median onset age of 20. A total of 97.8% of the patients had symptoms of encephalitis; 68.9% of the patients had symptoms of demyelination, including optic neuritis (ON) (37.9%), longitudinally extensive transverse myelitis (LETM) (31.0%) and acute disseminated encephalomyelitis (ADEM) (27.6%). Abnormal signals on magnetic resonance imaging (MRI) usually involved cortical (46.7%), subcortical (31.1%) and basal ganglia (26.7%) lesions, as well as infratentorial (48.9%) and spinal cord (28.9%) lesions. No tumours were found. A total of 62.2% of the patients relapsed, with recurrence rates of 66.7% and 50.0% for those treated with first-line therapy alone and in combination with second-line immunotherapy, respectively. The pathological changes from the biopsy indicated immune-mediated inflammatory demyelination. Although some patients may have residual deficits, 93.3% of the patients became functionally independent. ConclusionThe possibility of MNOS should be considered when patients diagnosed with anti-NMDARe simultaneously or sequentially develop ON, LETM or ADEM. MNOS occurred without tumour association, and inflammatory demyelination may be the pathological change. Steroids combined with second-line immunotherapy can help to reduce high recurrence rates, and most patients will have substantial recovery.

A Case Report of Wernicke's Encephalopathy Associated With Schizophrenia

Introduction: Wernicke's encephalopathy (WE) is a severe neurological syndrome often associated with alcoholism. Clinicians tend to ignore WE in other non-alcoholic clinical settings related to malnutrition and thiamine deficiency, resulting in delayed diagnosis. The diagnosis becomes more difficult when WE is secondary to psychiatric illnesses as symptoms can be masked by the primary disease.Case Presentation: We present a case of a 56-year-old female patient with schizophrenia who was admitted to the hospital for mental and behavioral disorder, without history of alcohol. She presented symptoms of ophthalmoplegia and high muscular tension, and the brain MRI showed symmetric lesions in the bilateral basal ganglia and third ventricle. She responded well to thiamine and was discharged on hospital day 22.Conclusion: The psychiatrists should be on the alert for starvation-induced WE, especially for patients suffering from malnutrition. WE is a preventable and treatable disease, so once suspected of WE, patients ought to take adequate supplements of thiamine immediately.

SLC25A42-associated mitochondrial encephalomyopathy: Report of additional founder cases and functional characterization of a novel deletion.

SLC25A42 is the main transporter of coenzyme A (CoA) into mitochondria. To date, 15 individuals have been reported to have one of two bi‐allelic homozygous missense variants in the SLC25A42 as the cause of mitochondrial encephalomyopathy, of which 14 of them were of Saudi origin and share the same founder variant, c.871A > G:p.Asn291Asp. The other subject was of German origin with a variant at canonical splice site, c.380 + 2 T > A. Here, we describe the clinical manifestations and the disease course in additional six Saudi patients from four unrelated consanguineous families. While five patients have the Saudi founder p.Asn291Asp variant, one subject has a novel deletion. Functional analyses on fibroblasts obtained from this patient revealed that the deletion causes significant decrease in mitochondrial oxygen consumption and ATP production compared to healthy individuals. Moreover, extracellular acidification rate revealed significantly reduced glycolysis, glycolytic capacity, and glycolytic reserve as compared to control individuals. There were no changes in the mitochondrial DNA (mtDNA) content of patient fibroblasts. Immunoblotting experiments revealed significantly diminished protein expression due to the deletion. In conclusion, we report additional patients with SLC25A42‐associated mitochondrial encephalomyopathy. Our study expands the molecular spectrum of this condition and provides further evidence of mitochondrial dysfunction as a central cause of pathology. We therefore propose that this disorder should be included in the differential diagnosis of any patient with an unexplained motor and speech delay, recurrent encephalopathy with metabolic acidosis, intermittent or persistent dystonia, lactic acidosis, basal ganglia lesions and, especially, of Arab ethnicity. Finally, deep brain stimulation should be considered in the management of patients with life altering dystonia.

Distribution of multiple sclerosis lesions detected by brain magnetic resonance imaging in Erbil city

Background and objective: Many epidemiological studies and clinical manifestation studies of multiple sclerosis have been done in Iraq. Up to our knowledge, no such observational study to the radiological feature of the multiple sclerosis lesion has been done yet in Erbil in comparison to other worldwide studies. This study aimed to assess the distribution of multiple sclerosis lesions in brain regions detected by magnetic resonance imaging among Erbil population. Methods: This was a cross-sectional study conducted at the College of Medicine, Hawler Medical University, from April 2018 to July 2019. A review of magnetic resonance imaging scans of the brain of 120 patients was done. Special attention was directed toward identifying the variance in multiple sclerosis lesions distribution in the brain regions and their MR signal intensity characteristics. Results: Periventricular lesions were observed in more than 90% of the study sample. The next common was juxtacortical lesions (24.8%), followed by corpus callosum lesions (16.8 %), while brain stem lesions were the least observed proportions. No significant difference was detected in the distribution of multiple sclerosis lesions among ethnicities and genders, except for basal ganglia lesions, which were significantly more common in women (P = 0.016).The magnetic resonance imaging signal intensity of the lesion was significantly variable among disease duration. Conclusion: The T2 hyper intense lesions were most commonly seen in the periventricular region. Juxtacortical and corpus callosum lesions were also frequently observed. The proportions of the brain stem and cerebellum lesions appeared to be lower in comparison to previous studies. Keywords: Multiple Sclerosis; Magnetic Resonance Imaging; Distribution; Lesion.

Neuro-imaging findings in children with developmental delay and cerebral palsy: experience from a tertiary medical center of Bangladesh

Background: Cerebral palsy (CP) is the most common developmental disability in children. Neuro-imaging in CP is widely used investigation. Imaging study can visualize the anatomical location of lesion in brain. The aim of this study was to find out the extent of non-progressive damage in brain among children with CP.
 Methods: This was a cross-sectional study. Data were collected from the out-patient department of Institute of Pediatric Neurodisorders & Autism (IPNA), Bangabandhu Sheikh Mujib Medical University (BSMMU) from December, 2019 to March, 2020.
 Results: Total 88 cases (age up to 14 years) were reviewed. There was male predominance and male to female ratio was 2:1. Computed tomography (CT) scan was done in majority of patients (71 cases) and magnetic resonance imaging (MRI) in 17 cases. Major abnormalities were atrophy in 34.1% cases, ventricular dilatation in 14.8% cases, encephalomalacia in 19.3% cases and basal ganglia lesion in 11.4% cases. Overall in CT scan group, 95.77% cases were abnormal and in MRI group, 88.23% were abnormal.
 Conclusion: Atrophy of the brain was the most common finding in neuro-imaging of patients with CP and developmental disabilities. Neuro-imaging is very useful and recommended for the children with developmental delay and CP cases. It may help to classify the CP and may give a clue for further investigations such as metabolic screening and genetic testing.
 Birdem Med J 2021; 11(2): 112-115

Brain cooling reduces the risk of postneonatal epilepsy in newborns affected by moderate to severe hypoxic-ischemic encephalopathy.

BACKGROUND Neonatal hypoxic-ischemic encephalopathy is still a significant cause of neonatal death and neurodevelopmental disabilities, such as cerebral palsy, mental delay, and epilepsy. After the introduction of therapeutic hypothermia, the prognosis of hypoxic-ischemic encephalopathy has improved, with reduction of death and disabilities. However, few studies evaluated whether hypothermia affects rate and severity of postneonatal epilepsy. We evaluated rates, characteristics and prognostic markers of postneonatal epilepsy in infants with moderate to severe hypoxic-ischemic encephalopathy treated or not with therapeutic hypothermia. METHODS We analyzed clinical data, EEG recordings, cerebral Magnetic Resonance Imaging (MRI) and outcome in 23 cooled and 26 non-cooled asphyxiated neonates (≥36 weeks' gestation), admitted from 2004 to 2012. RESULTS Among 49 neonates 11 (22%) had postneonatal epilepsy, of which 9 (18%) were non-cooled and 2 (4%) were cooled (P=0.05). Six of 11 infants (55%) had West syndrome, 4 (36%) had focal epilepsy and 1 (9%) had Lennox-Gastaut Syndrome. At multiple logistic regression analysis MRI pattern significantly correlated with postneonatal epilepsy (OR 0.19, 95% CI 0.04-0.88, P=0.03). Extensive lesions in basal ganglia and thalami plus cortical and white matter were associated with postneonatal epilepsy. CONCLUSIONS Only perinatal asphyxia with extensive lesions in basal ganglia and thalami plus cortical and white matter lesion conveys a high risk for early and severe postneonatal epilepsy. Moreover, therapeutic hypothermia is associated with a decrease of the risk of developing postneonatal epilepsy.

Evaluation of the Anatomical Locations of Stroke Events From Computed Tomography Scan Examinations in a Tertiary Facility in Ghana.

IntroductionStroke events are leading causes of mortalities globally and currently increasing alarmingly in low- and middle-income nations including Ghana, thus overburdening national healthcare delivery sectors. This trend is predicted to ultimately have an impact on the socio-economic development of these countries, thus gaining the attention of policy-makers and implementers. This study was therefore conducted to evaluate the anatomical locations of stroke events from CT scan examinations and the possibly associated variables to assist in managing this non-communicable pandemic.MethodsAll computed tomography (CT) scans performed for stroke events at the Cape Coast Teaching Hospital from June 2016 to June 2020 were retrieved and reviewed for this study. The socio-demographics and the presence of hypertensive risk factor were also retrieved. Data were then collated, grouped, coded, inputted, and used for analysis. Chi-square test of independence was employed for assessing possible associations, and logistic regression analysis was performed to predict the anatomical locations of stroke events using sex and hypertension. Statistical significance level was specified at p ≤ 0.05.ResultsA total of 1,750 stroke cases were recorded during the study period, comprising 1,237 (70.7%) ischemic strokes and 513 (29.3%) hemorrhagic strokes. Majority (54.3%) of the patients were males. The average age of participants was 62.46±14.74 years. Basal ganglia (43.0%), parietal lobe (26.7%), and frontal lobe (6.9%) were the commonest anatomical locations. The elderly (≥ 60 years) were significantly affected at the basal ganglia (p=0.006), parietal lobe (p=0.005), frontal lobe (p=0.013), temporal lobe (p=0.048), and cerebellum (p=0.049). Basal ganglia lesions were significantly recorded in men, whereas lesions located at the pons were significantly seen in females. The regression model revealed that the risk of stroke at the pons increased by 2.155-folds in males (p=0.043; 95% CI=1.026-4.528). Generally, gender and hypertension were not significant predictors of stroke lesion locations.ConclusionsThe basal ganglia area, which falls under the middle cerebral artery territory, was the commonest anatomical location for stroke events in our setting. Knowing the anatomical locations of these stroke events has an impact on the type of interventions needed, especially at the early stages of these stroke events. CT perfusion, CT angiography, and magnetic resonance imaging (MRI) with MR angiography (MRA) when available can further assist in determining the exact cause so that urgent interventions such as endovascular treatments can be offered.

Giant central nervous system tuberculoma in pediatric patients: surgical case series

Central nervous system tuberculoma is rare and challenging situation. Clinical records of patients with pathologically proven tuberculoma were retrospectively reviewed. Clinical presentation, lesion location, radiological characteristics, perioperative and surgical management, and outcome is summarized and analyzed. Eight patients were included and there was one girl. Age ranged from 3 to 14 years with mean age 9.8 years. Clinical duration ranged from 20 days to 2 years, and 3 patients had previous lung tuberculosis with anti-TB treatment. The lesion was in cerebellum in 6 cases, including 1 involving basal ganglia and 1 involving thalamus. The lesion was in basal ganglia, thalamus, and third ventricle in 1 case, and in T12-L1 spinal cord in another. Cerebellar lesion was resected via paramedian suboccipital approach in 5 patients, basal ganglia lesion via trans-cortical frontal horn approach in 2 patients, and intra-spinal lesion via trans-laminar approach in 1 patient. Follow-up ranged from 10 to 24 months. Of the 8 patients, 6 returned to normal life. One patient had cerebellar lesion resected and the thalamic lesion reduced in size after anti-TB treatment. One patient died from TB spreading. Our data showed that most patients can be successfully treated by resection of the lesion. Low T2 signal, ring shaped enhancement and peripheral edema strongly suggest tuberculoma. Empirical anti-TB treatment should be initiated perioperatively.

Dementia and Parkinson-like syndrome with basal ganglia lesion in neuromyelitis optica spectrum disorders

ABSTRACT Brain lesions in neuromyelitis optica spectrum disorders (NMOSD) are generally located at sites of high anti-aquaporin 4 (AQP4) expression. Clinical features of NMOSD associated with basal ganglia damage in sites not enriched with AQP4 remain unknown. Here we describe the case of an 82-year-old woman who developed dementia and bradykinesia for 5 weeks. Brain magnetic resonance imaging revealed obvious basal ganglia abnormalities. Test for serum anti-AQP4 antibody was positive, and she was diagnosed with NMOSD. Our case showed that NMOSD associated with dementia and/or Parkinson-like syndrome with basal ganglia lesions could be another clinical presentation in NMOSD.

Burden of Depression Among Survivors of Ischemic Stroke of Southern Punjab

Background; Depression is one of the most frequent neuropsychiatric disturbances after ischemic stroke. The frequency of depression in stroke patients has varied widely in different populations. Post stroke depression is an important factor limiting recovery and rehabilitation in acute stroke patients. This study was done to ascertain frequency of depression among patients with ischemic stroke in our local population. Objective; To assess the frequency of depression in patients with ischemic stroke in Pakistan. Material and Methods; Consecutive 150 patients fulfilling inclusion criteria was enrolled and this descriptive study was conducted at department of Psychiatry and Neurology OPD, Nishtar Hospital, Multan using non – probability convenient sampling technique. All patients were assessed by single psychiatrist on PHQ-9 scale, for Depression. Results; Of these 150 study cases, 94 (62.7 %) were male patients while 56 (37.3 %) were female patients. Mean age of our study cases was 54.69 ± 7.18 years (with minimum age of our study cases was 44 years while maximum age was 71 years). Of these 150 study cases, 71 (47.3 %) belonged to rural areas and 79 (52.7 %) belonged to urban areas. Monthly family income up to Rs. 50000 was noted in 89 (59.3%) while more than Rs. 50000 was noted in 61 (40.7%). Of these 150 study cases, 83 (55.3%) were illiterate and 67 (44.7%) were literate. Of these 150 study cases, site of lesion in basal ganglia was 61 (40.7%), subcortical in 56 (37.3%) and cortical in 33 (22.0%) and Depression was noted in 77 (51.3%). Conclusion; Very high frequency of depression was observed in our study among patients having ischemic stroke. Depression was significantly associated with gender, occupation, educational level, prolonged duration of illness and site of lesion. Clinicians treating stroke patients should anticipate depressive symptoms and diagnose them to treat them in early stages. Keywords; Ischemic stroke, depression, frequency. DOI: 10.7176/JMPB/69-01 Publication date: March 31 st 2021

Early Outcomes and Complications of Endovascular Treatment of Cerebral Arteriovenous Malformations in Pediatric Patients

Introduction: Arteriovenous malformations (AVMs) are the commonest cause of hemorrhagic stroke in children. Endovascular embolization is a feasible treatment modality, but cure rates are heterogenous from one series to another. We aimed to describe the immediate obliteration rates and periprocedural complications of embolization of pediatric AVMs. Methods: Between 2011 and 2019, participants below 18 years of age with AVMs treated by the same neurosurgeon at a single center were included. The clinical features, immediate angiographic results, and periprocedural complications were retrospectively collected from the clinical records. Results: Thirty-four embolization sessions were performed on 20 children (12 females with a mean age of 13). Intracranial hemorrhage was the most common presentation (75%), and the majority were frontal (30%) and basal ganglia (30%) lesions. An immediate complete angiographic obliteration was achieved in 9 patients (45%) with low-grade lesions (Spetzler-Martin grade I and II). NBCA was the most common embolic agent used (52.9%). Complications were reported in 3 (8.8%) out of 34 sessions. Two of them were intraoperative perforations with clinical consequences. A slight cortical hemorrhage during the procedure was observed in 1 patient without clinical repercussions. Discussion: This single-surgeon single-center experience suggests that endovascular treatment is a safe and efficient treatment for pediatric AVMs. Pediatric prognostic scores for a suitable selection of candidates are needed. Further studies are required to validate these results.

Hyponatremia, osmotic demyelination syndrome, and the importance of the patient’s history

Central pontine myelinolysis (CPM), first described in 1959, is a symmetrical non-inflammatory demyelinating disease with loss of oligodendrocytes that occurs most often following a rapid correction of severe hyponatremia (i.e., <120 mmol/L). It presents as a biphasic disease with initial seizure or encephalopathy, followed by clinical improvement and subsequent rapid deterioration with bulbar dysfunction, oculomotor dysfunction, various degree of paresis, and even locked in syndrome. Its occurrence is rare (≈0.6% of severe hyponatremia), it is diagnosed clinically and confirmed with brain imaging, ideally with magnetic resonance image, and it is reversible in approximately half the patients. Lesions are classically identified in the pons but extra pontine lesions (in basal ganglia, cerebellar white matter, thalamus, and hippocampus) have also been identified. The most commonly accepted molecular mechanism involves brain cell volume regulation with a rapid shift of osmole following brain edema which establishes during the chronic hyponatremic phase. For these reasons, osmotic demyelination syndrome (ODS) is a better term. The most identified risk factor is severe hyponatremia, but other electrolyte abnormalities can contribute, in particular, if the patient is an alcoholic or malnourished. This diagnosis should also be suspected in post-op patients with nausea and headache non-responsive to antiemetic and analgesic drugs. An essential step is an appropriate medical history, a list of medications, physical examination, and basic initial lab tests with the goal of identifying possible easily reversible causes of hyponatremia. Correction of severe hyponatremia with neurological symptoms should be done using rapid boluses of hypertonic saline solution in rapid succession with goals of increasing serum sodium by 5-6 mEq/L in the first two hours, which should be stopped if the level has risen by 10 mEq/L in the first five hours, and with the overall correction goal not to exceed 15-20 mEq/L in 48 hrs. This method has been shown safe in all hospital settings studied. Serial measurements of electrolyte levels and neurological examinations are recommended, as are correction of all electrolyte abnormalities, in
 particular magnesium and potassium. Thiamine should be given to all patients with chronic alcohol use who present with hyponatremia and encephalopathy.

Brain Magnetic Resonance Imaging Characteristics of Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis and Their Clinical Relevance: A Single-Center Study in China.

Objective: To characterize the magnetic resonance imaging (MRI) features of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis and explore their clinical relevance.Methods: Patients with anti-LGI1 encephalitis who underwent MRI at our center were included in this study. Baseline and follow-up MRI characteristics were evaluated, and relationships between lesion location and clinical symptoms were analyzed. The extent of signal abnormalities within the lesion overlap region was measured and correlated with modified Rankin Scale scores and serum antibody titer.Results: Seventy-six patients were enrolled, of which 57 (75%) were classified as MR positive. Brain lesions were located in medial temporal lobe (MTL) (89%) and basal ganglia (BG) (28%). Hippocampus and amygdala were lesion hubs with more than 50% lesion overlap. BG lesions were found in 30% of patients with faciobrachial dystonic seizure (FBDS) and only 7% of patients without FBDS (p = 0.013). Meanwhile, MTL lesions were more commonly observed in patients with memory impairment (70 vs. 0%, p = 0.017). MRI features included hyperintensity and edema at baseline, as well as hypointensity and atrophy at follow-up. Correlations between signal intensity of lesion hubs (including hippocampus and amygdala) and modified Rankin Scale scores were found on T2 (r = 0.414, p < 0.001) and diffusion-weighted imaging (r = 0.456, p < 0.001).Conclusion: MTL and BG are two important structures affected by anti-LGI1 encephalitis, and they are associated with distinctive symptoms. Our study provided evidence from Chinese patients that BG lesions are more commonly observed in patients with FBDS, potentially suggesting BG localization. Furthermore, in addition to supporting diagnosis, MRI has the potential to quantify disease severity.

Behavior Disorder and Social Function Impairment in Children with Basal Ganglia Germ Cell Tumors.

PurposeBasal ganglia intracranial germ cell tumors (iGCTs) can specifically destroy the basal ganglia network, leading to several cognitive, learning, behavioral, and social impairments. This study aimed to investigate the behavior and social disorders of patients with basal ganglia iGCTs.Patients and MethodsWe recruited 30 newly diagnosed iGCTs patients (and their parents) for the current study. The Child Behavior Checklist/6–18 was used to evaluate emotional and behavioral problems. The Conner’s Parent Rating Scales was used to assess symptoms of hyperactivity/impulsivity and conduct problems. The health-related quality of life (HRQoL) was assessed using the Pediatric Quality of Life Inventory 4.0 Generic Core Scale. Performance status was assessed using the Lansky play-performance scale and Karnofsky performance scale. The effects of basal ganglia lesions on these scores were examined.ResultsPatients with basal ganglia iGCTs (n = 10) had more behavioral problems (attention problems, aggressive behavior, learning problems, hyperactivity index), social function impairment, anxiety/depression, and poorer HRQoL compared to patients with non-basal ganglia iGCTs (n = 20). There was no significant difference in the Lansky play-performance/Karnofsky performance scale scores.ConclusionThis study demonstrates the effects of basal ganglia lesions on behavioral and emotional outcomes, social functions, and HRQoL of patients with iGCTs. The results may help to understand the function of basal ganglia and provide evidence for the benefit of early psychological intervention to improve the treatment for this rare disease.

Performance on the Rey-Osterrieth complex figure test and the correlation with the magnetic resonance imaging brain lesion volume in multi-infract versus small vessel disease dementia

Background/Aim. Regarding several cognitive domains, including visuospatial and visuoconstructional abilities, little is known about the differences between vascular dementia (VaD) subtypes, even in the most common sub-types, such as multi-infarct dementia (MID) and subcortical ischemic small vessel disease dementia (SSVD). This paper aimed to identify the differences between the performances on the Rey-Osterrieth Complex Figure (ROCF) test in MID and SSVD and correlate the ROCF scores in both groups with magnetic resonance imaging (MRI) ischemic lesion load. Methods. Sixty VaD patients with matching severity of dementia, age, and education were included in this study: 32 with SSVD and 28 with MID ac-cording to the NINDS-AIREN (National Institute of Neuro-logical Disorders and Stroke and Association Internationale pour la Recherch? et l'Enseignement en Neurosciences) neuroradiological criteria. A quantitative scoring system was performed. ROCF was given to all subjects in three test conditions: copy, immediate recall after 3 minutes, and delayed recall after 45 min. Magnetic resonance imaging (MRI) of the is-chemic brain volumes of anterior and posterior lesions, left and right hemispheric lesions, left and right-sided basal ganglia lesions, and total lesion load (TLL) were calculated in both groups. Results. The MID group was more impaired than SSVD on ROCF copy (p = 0.008), immediate recall (p= 0.005) and delayed recall (p = 0.001). There were significant correlations between ROCF copy score and the TLL (p &lt; 0.05) and posterior brain lesion volume (p&lt; 0.05) in the MID group. Conclusion. The importance of visuospatial, visuoconstructional deficit and impairment of visual memory is disregarded in VaD subtypes. These impairments are more severe in MID than SSVD and the deficit of ROCF copying in MID patients correlates with posterior and total MRI lesion volume.

Defective Somatosensory Inhibition and Plasticity Are Not Required to Develop Dystonia.

Dystonia may have different neuroanatomical substrates and pathophysiology. This is supported by studies on the motor system showing, for instance, that plasticity is abnormal in idiopathic dystonia, but not in dystonia secondary to basal ganglia lesions. The aim of this study was to test whether somatosensory inhibition and plasticity abnormalities reported in patients with idiopathic dystonia also occur in patients with dystonia caused by basal ganglia damage. Ten patients with acquired dystonia as a result of basal ganglia lesions and 12 healthy control subjects were recruited. They underwent electrophysiological testing at baseline and after a single 45-minute session of high-frequency repetitive somatosensory stimulation. Electrophysiological testing consisted of somatosensory temporal discrimination, somatosensory-evoked potentials (including measurement of early and late high-frequency oscillations and the spatial inhibition ratio of N20/25 and P14 components), the recovery cycle of paired-pulse somatosensory-evoked potentials, and primary motor cortex short-interval intracortical inhibition. Unlike previous reports of patients with idiopathic dystonia, patients with acquired dystonia did not differ from healthy control subjects in any of the electrophysiological measures either before or after high-frequency repetitive somatosensory stimulation, except for short-interval intracortical inhibition, which was reduced at baseline in patients compared to control subjects. The data show that reduced somatosensory inhibition and enhanced cortical plasticity are not required for the clinical expression of dystonia, and that the abnormalities reported in idiopathic dystonia are not necessarily linked to basal ganglia damage. © 2020 International Parkinson and Movement Disorder Society.

Basal Ganglia Intracranial Mixed Germ Cell Tumor With Predominance of Yolk Sac Component

Abstract Introduction/Objective Primary intracranial germ cell tumors (ICGCTs) represent a rare heterogenous group of neoplasms with an overall incidence of 3% of all pediatric brain tumors. ICGCTs are classified into germinomatous and non-germinomatous tumors and approximately 25% are mixed, and contain more than one histologic component. Most ICGCTs are located in suprasellar and pineal regions with some occurring in rare locations such as basal ganglia, thalamus and fourth ventricle. Methods In our case, A 9-year-old Asian-American male presented with headaches and a few weeks history of unilateral weakness. Imaging showed a left basal ganglia lesion with midline shift. Serum AFP was elevated at 17,974.5ng/ml. Results Histology revealed a biphasic tumor with a predominant yolk-sac component composed of sheets of pleomorphic cells with prominent nucleoli, focally arranged in a reticular pattern with hyaline globules, increased mitoses and foci of necrosis. A lesser component display features of germinoma, in the form of sheets and nests of large cells with clear vacuolated cytoplasm separated by fibrous septa with lymphocytic infiltrates. Tumor cells in yolk sac component were positive for AFP, while PLAP, CD117, D240 stained germinoma component. After receiving 6 courses of chemotherapy and radiation, MRI did not show any evidence of residual disease. Conclusion Pathogenesis of ICGCT is thought to be either from primordial germ cells that have migrated aberrantly during embryonic development or from pluripotent embryonic cells that have been entrapped into lateral mesoderm due to misfolding and misplacement and then undergo malignant transformation. The outcomes of ICGCTs differ among histological subtypes. Highest survival rates are seen in patients with germinomas or mature teratomas (10- year survival rate &amp;gt;90% in both groups. The lowest survival rates are seen in choriocarcinoma, embryonal carcinoma and yolk sac tumor.