A patient with granulomatous amoebic encephalitis caused by Balamuthia mandrillaris survived with two excisions and medication

Background Granulomatous amoebic encephalitis (GAE) is a rare central nervous system infection caused by the Balamuthia mandrillaris or Acanthamoeba species. Diagnosis is challenging because of the non-specific clinical presentation, cerebrospinal fluid analysis, and radiological features. There is no effective treatment for GAE to date. Case presentation A 54-year-old male was admitted to hospital after experiencing acute onset of numbness and weakness on his left limb. Due to the initial consideration of intracranial tumor, surgical removal of the right parietal lesion was performed. However, the patient had a headache accompanied by diplopia, difficulty walking and a new lesion was found in the left occipital-parietal lobe two weeks after the first operation. High-throughput next-generation sequencing (NGS) detected the presence of high copy reads of the B. mandrillaris genome sequence in the patient’s blood, cerebral spinal fluid (CSF), and brain tissue. Pathological investigation of the brain tissue showed granulomatous changes and amoebic trophozoite scattered around blood vessels under high magnification. The patient was re-operated due to developing progressive confusion caused by subfalcine herniation of the left cerebral hemisphere. The lesions of the right parietal lobe were obviously decreasing in size after the first surgery, and the lesions of the left occipital lobe and the sunfalcine herniation didn’t ameliorate two months after the second surgery. The patient was transferred to local hospital for continuous treatment with sulfamethoxazole and azithromycin. After five months of the second surgery, the patient showed good recovery with mild headache. Conclusions This is the first report of a patient with B. mandrillaris encephalitis initially confirmed by NGS and have experienced two excisions, responding favorably to the combination of surgeries and medications. Early surgical resection of intracranial lesions combined with drug treatment may offer the chance of a cure.

Right Superior Temporal Gyrus Altered Functional Activity in Patients With Definite Vestibular Migraine

The study aimed to investigate resting-state functional brain activity alterations in patients with definite vestibular migraine (dVM). Seventeen patients with dVM, 8 patients with migraine, 17 health controls (HCs) were recruited. The amplitude of low frequency fluctuation (ALFF), fractional ALFF (fALFF) and regional homogeneity (ReHo) were calculated to observe the changes in spontaneous brain activity. Then brain regions with altered fALFF were selected for seed-based functional connectivity analysis. Compared with HCs, VM patients showed significantly increased ALFF values in the right temporal lobe (Cluster size = 91 voxels, P=0.002, FWE corrected), and significantly increased ReHo values in the right superior temporal gyrus (STG), middle temporal gyrus (MTG) and inferior temporal gyrus (ITG) (Cluster size = 136 voxels, P=0.013, FWE corrected). Compared with patients with migraine, patients with VM showed significantly increased fALFF values in the right parietal lobe (Cluster size = 43 voxels, P=0.011, FWE corrected) and right frontal lobe (Cluster size =36 voxels, P=0.026, FWE corrected), significantly increased ReHo values in the right thalamus (Cluster size = 92 voxels, P=0.043, FWE corrected). Our findings documented that patients with VM showed enhanced spontaneous functional activity in the right temporal lobe (STG, MTG, and ITG) compared with HCs, and increased spontaneous activity in the right parietal lobe-frontal lobe-thalamus compared with patients with migraine. Patients with VM and migraine both had altered brain function, but the regions involved are different.

MRI findings suggestive of alzheimer’s disease in patients with primary open angle glaucoma – a single sequence analysis using rapid 3D T1 spoiled gradient echo

Objective: Primary open-angle glaucoma (POAG) is a degenerative optic neuropathy disease which has somewhat similar pathophysiology to Alzheimer’s disease (AD). This study aims to determine the presence of medial temporal atrophy and parietal lobe atrophy in patients with POAG compared to normal controls using MTA scoring and PCA scoring system on T1-MPRAGE. Methods: 50 POAG patients and 50 normal subjects were recruited and an MRI brain with T1-MPRAGE was performed. Medial temporal lobe and parietal lobe atrophy were by MTA and PCA/Koedam scoring. The score of the PCA and MTA were compared between the POAG group and the controls. Results: There was a significant statistical difference between PCA score in POAG and the healthy control group (p-value = 0.026). There is no statistical difference between MTA score in POAG compared to the healthy control group (p-value = 0.58). Conclusion: This study suggests a correlation between POAG and PCA score. Potential application of this scoring method in clinical diagnosis and monitoring of POAG patients. Advances in knowledge: The scoring method used in Alzheimer’s disease may also be applied in the diagnosis and monitoring of POAG MRI brain, specifically rapid volumetric T1spoiled gradient echo sequence, may be applied in primary open-angle glaucoma assessment

Effects of Linguistic Distance on Second Language Brain Activations in Bilinguals: An Exploratory Coordinate-Based Meta-Analysis

In this quantitative meta-analysis, we used the activation likelihood estimation (ALE) approach to address the effects of linguistic distance between first (L1) and second (L2) languages on language-related brain activations. In particular, we investigated how L2-related networks may change in response to linguistic distance from L1. Thus, we examined L2 brain activations in two groups of participants with English as L2 and either (i) a European language (European group, n = 13 studies) or (ii) Chinese (Chinese group, n = 18 studies) as L1. We further explored the modulatory effect of age of appropriation (AoA) and proficiency of L2. We found that, irrespective of L1-L2 distance—and to an extent—irrespective of L2 proficiency, L2 recruits brain areas supporting higher-order cognitive functions (e.g., cognitive control), although with group-specific differences (e.g., the insula region in the European group and the frontal cortex in the Chinese group). The Chinese group also selectively activated the parietal lobe, but this did not occur in the subgroup with high L2 proficiency. These preliminary results highlight the relevance of linguistic distance and call for future research to generalize findings to other language pairs and shed further light on the interaction between linguistic distance, AoA, and proficiency of L2.

Crossed Aphasia after Intracranial Hematoma in the Right Temporoparietal Lobe: A Case Report

Purpose: Aphasia in a dextral after right hemisphere injury is called crossed aphasia (CA). We are reporting a first case of transformation of motor aphasia to conduction aphasia after right hemisphere intracerebral hemorrhage (ICH) associated with arteriovenous malformation (AVM) with literature reviews.Methods: A case of a man in his 30s with CA following right hemisphere ICH in the temporal-parietal lobe associated with AVM was reviewed. We analyzed his brain images, initial linguistic characteristics, and changes in aphasia for 8 weeks of follow-up.Results: The initial Paradise Korean Western Aphasia Battery Revised (PK-WAB-R) was evaluated as aphasia quotient (AQ) 72, 64%ile; post evaluation was evaluated AQ 95, 98.9%ile after 8 weeks. The post-test repetition score was the patient score range, which can be attributed to impairment in phonological short-term memory. The patient is diagnosed anomalous CA based on Alexander et al., and we could predict that the language ability originates from both hemispheres based on Nagaraja et al. Considering the appearance of Gerstmann syndrome at the beginning of the onset, we could expect that the function of the dominant parietal lobe is partially crossed as well.Conclusion: Changes in aphasia were reported throughout the initial stage to the end of speech therapy. It is also important to note that literature review of Korean studies was analyzed in this study. It will be necessary to conduct a cognitive test in the early stage of onset to understand the language problems of crossed conduction aphasia to know the characteristics of the cognitive process.

Clinical case of arteriovenous malformation

Arteriovenous malformation (AVM) is a congenital malformation of cerebral vessels, associated with an abnormal connection of arteries and veins. The altered locations of the cerebral vascula-ture form a conglomerate that is a direct arteriovenous shunting without intermediate capillaries. The incidence of the disease is 4 cases per 100 thousand population. Clinically, AVM debuts mainly at the age of 20 to 40, and is accompanied by intracranial hemorrhages in 50% to 60% of patients; mortality is 35%. In 27% to 70% of individuals, AVM are manifested by epileptic seizures of various focal origins. In diagnostics, X-ray computed tomography is used to detect hemorrhage in case of a ruptured malformation; magnetic resonance imaging, including angiography, to assess the AVM node, afferent vessels and venous drainage. Currently, the only radical treatment for AVM is surgical removal of the malformation. This is feasible for small and medium-sized AVMs of cerebral vessels (36% to 50% of patients), since removal of large AVMs and AVMs located in functionally important areas, even microsurgically, is associated with a high risk of death and disability. A clinical case of a 30-year-old young patient with an acute onset of the disease characterized by sudden loss of consciousness and an episode of seizures is presented. Based on the data of clinical and instrumental examination, an AVM of the left parietal lobe with parenchymal-ventricular hemorrhage was revealed. On the day of admission to the hospital in Ryazan, a surgical intervention was performed: external ventriculostomy on the left side. After 4 months the second surgical intervention, removal of AVM of the left parietal region using neurophysiological control was carried out at Burdenko Neurosurgical Hospital in Moscow, Russia. The successful surgical approach has completely eliminated the life-threatening pathology. Subsequently, the patient underwent rehabilitation in a neurological hospital at the place of residence. CONCLUSION: The topic of AVM is of great practical interest not only for neurosurgeons, but also for neurologists and radiologists, since timely diagnosis and treatment effectiveness directly depend on the coordinated work of these specialists.

Altered Processing of Visual Stimuli in Vestibular Migraine Patients Between Attacks: A Combined VEP and sLORETA Study

Objective: Vestibular migraine (VM) is one of the most common causes of recurrent vertigo, but the neural mechanisms that mediate such symptoms remain unknown. Since visual symptoms and photophobia are common clinical features of VM patients, we hypothesized that VM patients have abnormally sensitive low-level visual processing capabilities. This study aimed to investigate cortex abnormalities in VM patients using visual evoked potential (VEP) and standardized low-resolution brain electromagnetic tomography (sLORETA) analysis.Methods: We employed visual stimuli consisting of reversing displays of circular checkerboard patterns to examine “low-level” visual processes. Thirty-three females with VM and 20 healthy control (HC) females underwent VEP testing. VEP components and sLORETA were analyzed.Results: Patients with VM showed significantly lower amplitude and decreased latency of P1 activation compared with HC subjects. Further topographic mapping analysis revealed a group difference in the occipital area around P1 latency. sLORETA analysis was performed in the time frame of the P1 component and showed significantly less activity (deactivation) in VM patients in the frontal, parietal, temporal, limbic, and occipital lobes, as well as sub-lobar regions. The maximum current density difference was in the postcentral gyrus of the parietal lobe. P1 source density differences between HC subjects and VM patients overlapped with the vestibular cortical fields.Conclusion: The significantly abnormal response to visual stimuli indicates altered processing in VM patients. These findings suggest that abnormalities in vestibular cortical fields might be a pathophysiological mechanism of VM.

Distinct Brain Mechanisms for Conflict Adaptation within and across Conflict Types

Cognitive conflict, like other cognitive processes, shows the characteristic of adaptation, that is, conflict effects are attenuated when immediately following a conflicting event, a phenomenon known as the conflict adaptation effect (CAE). One important aspect of CAE is its sensitivity to the intertrial coherence of conflict type, that is, behavioral CAE occurs only if consecutive trials are of the same conflict type. Although reliably observed behaviorally, the neural mechanisms underlying such a phenomenon remains elusive. With a paradigm combining the classic Simon task and Stroop task, this fMRI study examined neural correlates of conflict adaptation both within and across conflict types. The results revealed that when the conflict type repeated (but not when it alternated), the CAE-like neural activations were observed in dorsal ACC, inferior frontal gyrus (IFG), superior parietal lobe, and so forth (i.e., regions within typical task-positive networks). In contrast, when the conflict type alternated (but not when it repeated), we found CAE-like neural deactivations in the left superior frontal gyri (i.e., a region within the typical task-negative network). Network analyses suggested that the regions of ACC, IFG, superior parietal lobe, and superior frontal gyrus can be clustered into two antagonistic networks, and the ACC–IFG connection was associated with the within-type CAE. This evidence suggests that our adaptation to cognitive conflicts within a conflict type and across different types may rely on these two distinct neural mechanisms.

Usefulness of brain FDG PET/CT imaging in pediatric patients with suspected autoimmune encephalitis from a prospective study

Purpose Early diagnosis and treatment are of paramount importance for pediatric patients with autoimmune encephalitis (AE). The aim is to evaluate the usefulness of FDG PET/CT in pediatric patients with suspected AE from a prospective study. Methods The prospective study was conducted over a period of 23.5 months from May 14, 2019, to April 30, 2021. All patients (< 18-year-old) were hospitalized at the department of pediatric neurology and met the criteria of clinical suspected AE. The children underwent the tests of blood samplings, CSF, EEG, MRI, and 18F-FDG PET/CT. The criteria for FDG PET/CT diagnosis of AE were large lobar hypometabolism with or without focal hypermetabolism found on PET/CT. The clinical final diagnosis of AE includes seropositive and seronegative AE based on the diagnostic criteria. Results One hundred four pediatric inpatients (57 boys, 47 girls) were included, of which 58 children were diagnosed with AE (seropositive, 16; seronegative, 42), 45 children were diagnosed with non-AE, and one boy remained indeterminate diagnosis. Large lobar hypometabolism was found in 61 children, of which 54 (88.5%) children were finally diagnosed with AE. The sensitivity, specificity, and accuracy of FDG PET/CT for diagnosis of AE were 93.1%, 84.4%, and 89.3%, respectively, with a positive predictive value of 88.5% and a negative predictive value of 90.5%. The most common involved with hypometabolism was the parietal lobe, followed by occipital and frontal lobes, finally the temporal lobe on PET/CT in children with AE. Conclusion Brain FDG PET/CT imaging has high specificity, sensitivity, and accuracy for diagnosis of AE in clinical suspected AE children. Trial registration. Clinical Trials.gov. NCT02969213. Registered 17 October 2016.

Sneddon’s Syndrome as rare and underdiagnosed cause of stroke: about two cases / A Síndrome de Sneddon como causa rara e subdiagnosticada de AVC: cerca de dois casos

Case 1. Female, 27 years old, with migraine with aura. Use of combined contraceptive therapy. She presented visual and cutaneous complaints for eight months, using prednisone 60mg/day due to the hypothesis of systemic vasculitis. On examination, right temporal hemianopsia, normal fundus of the eye, Raynaud ‘s phenomenon with necrosis stitches in fingers and livedo were noted. Brain MRI with diffusion restriction in the left occipital lobe, hypersignal foci in the parietal lobe, and head of the left caudate nucleus. Reticular Livedo (RL) compatible biopsy. Anticardiolipin IgM (13.3 U/mL), ESR (47mm/h in the 1st hour and 52mm/h in the 2nd hour), and VDRL 1:8 reagents. Normal CSF. Initially treated with warfarin, she has been using rivaroxaban for two years, remaining asymptomatic. Case 2. Female, 39 years old, with a report of sudden dysarthria and right hemiparesis, with a previous episode of vertigo. A lumbosacral spine MRI was performed, presenting no alterations. Normal CSF. Antiphospholipid antibodies: Beta-2-glycoprotein I IgM 21 UR/mL and IgG 9 UR/mL, lupus anticoagulant 0.98, anticardiolipin IgG 9.4 GPL and IgM 31 MPL. Cranial MRI showed old ischemic gaps and hyperintense areas on T2/FLAIR with diffusion restriction affecting the left paramedian portion of the pons, related to acute stroke. Presence of livedo in the distal region of the limbs. In current use of warfarin 7.5mg/day, presenting remission of symptoms. Discussion: Sneddon Syndrome (SS) is a non-inflammatory thrombotic vasculopathy characterized by the concomitant presence of livedo reticularis and stroke. Neurological manifestations are present in up to 77% of cases. It is more prevalent in women between 20 and 42 years old. Treatment is aimed at preventing potentially serious, disabling, and even fatal new vascular events. Final Comments: SS is a rare syndrome that probably results from a series of acquired or congenital hemostatic abnormalities that preferentially involve the cutaneous and cerebral vascular beds. Although it has no specific biomarker, there are reports associating SS with antiphospholipid antibodies and VDRL reactor. Clinical presentation, laboratory findings and skin biopsy associated with an excellent response to anticoagulant treatment were fundamental in the diagnostic elucidation.