Temporal Lesions Research Articles

Temporal Trends and Lesion Sets for Persistent Atrial Fibrillation Ablation: A Meta-Analysis With Trial Sequential Analysis and Meta-Regression.

Ablation for persistent atrial fibrillation (PsAF) has been performed for over 20 years, although success rates have remained modest. Several adjunctive lesion sets have been studied but none have become standard of practice. We sought to describe how the efficacy of ablation for PsAF has evolved in this time period with a focus on the effect of adjunctive ablation strategies. Databases were searched for prospective studies of PsAF ablation. We performed meta-regression and trial sequential analysis. A total of 99 studies (15 424 patients) were included. Ablation for PsAF achieved the primary outcome (freedom of atrial fibrillation/atrial tachycardia rate at 12 months follow-up) in 48.2% (5% CI, 44.0-52.3). Meta-regression showed freedom from atrial arrhythmia at 12 months has improved over time, while procedure time and fluoroscopy time have significantly reduced. Through the use of cumulative meta-analyses and trial sequential analysis, we show that some ablation strategies may initially seem promising, but after several randomized controlled trials may be found to be ineffective. Trial sequential analysis showed that complex fractionated atrial electrogram ablation is ineffective and further study of this treatment would be futile, while posterior wall isolation currently does not have sufficient evidence for routine use in PsAF ablation. Overall success rates from PsAF ablation and procedure/fluoroscopy times have improved over time. However, no adjunctive lesion set, in addition to pulmonary vein isolation, has been conclusively demonstrated to be beneficial. Through the use of trial sequential analysis, we highlight the importance of adequately powered randomized controlled trials, to avoid reaching premature conclusions, before widespread adoption of novel therapies.

Imaging of Temporal Bone Mass Lesions: A Pictorial Review.

Tumoral lesions of the temporal bone include benign or malignant tumors and congenital or inflammatory lesions. Temporal bone lesions are difficult to approach. Therefore, making a preoperative diagnosis and considering whether the lesions require treatment are necessary; if they require treatment, then the type of treatment requires consideration. These tumors cannot be observed directly and must be diagnosed based on symptoms and imaging findings. However, the differentiation of temporal bone lesions is difficult because they are rare and large in variety. In this pictorial review, we divided temporal bone lesions by location such as along the facial nerve, along the internal jugular vein, around the endolymphatic sac, in the internal auditory canal/cerebellopontine angle, petrous apex, middle ear, and mastoid, focusing on the imaging findings of temporal bone lesions. Then, we created a diagnostic flowchart that suggested that the systematic separation of imaging findings is useful for differentiation. Although it is necessary to make comprehensive judgments based on the clinical symptoms, patient background, and imaging findings to diagnose temporal bone mass lesions, capturing imaging features can be a useful differentiation method.

Neuropathological hallmarks in autopsied cases with mitochondrial diseases caused by the mitochondrial 3243A>G mutation.

The mitochondrial (m.) 3243A>G mutation is known to be associated with various mitochondrial diseases including mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Their clinical symptoms have been estimated to occur with an increased mitochondrial DNA (mtDNA) heteroplasmy and reduced activity of oxidative phosphorylation (OXPHOS) complexes, but their trends in the central nervous system remain unknown. Six autopsied mutant cases and three disease control cases without the mutation were enrolled in this study. The mutant cases had a disease duration of 1-27 years. Five of six mutant cases were compatible with MELAS. In the mutant cases, cortical lesions including a laminar necrosis were frequently observed in the parietal, lateral temporal, and occipital lobes; less frequently in the frontal lobe including precentral gyrus; and not at all in the medial temporal lobe. The mtDNA heteroplasmy in brain tissue samples of the mutant cases was strikingly high, ranging from 53.8% to 85.2%. The medial temporal lobe was preserved despite an inhospitable environment having high levels of mtDNA heteroplasmy and lactic acid. OXPHOS complex I was widely decreased in the mutant cases. The swelling of smooth muscle cells in the vessels on the leptomeninges, with immunoreactivity (IR) against mitochondria antibody, and a decreased nuclear/cytoplasmic ratio of choroidal epithelial cells were observed in all mutant cases but in none without the mutation. Common neuropathological findings such as cortical laminar necrosis and basal ganglia calcification were not always observed in the mutant cases. A high level of mtDNA heteroplasmy was observed throughout the brain in spite of heterogeneous cortical lesions. A lack of medial temporal lesion, mitochondrial vasculopathy in vessels on the leptomeninges, and an increased cytoplasmic size of epithelial cells in the choroid plexus could be neuropathological hallmarks helpful in the diagnosis of mitochondrial diseases.

Complications of the Superior Eyelid Endoscopic Transorbital Approach to the Skull Base: Preliminary Experience With Specific Focus on Orbital Outcome.

The endoscopic superior eyelid approach is a relatively novel mini-invasive technique that is currently investigating for skull base cancers. However, questions remain regarding specific approach-related complications when treating different skull base tumors. This study aims to analyze any surgical complications that occurred in our preliminary consecutive experience, with specific focus on orbital outcome. A retrospective and consecutive cohort of patients treated via a superior eyelid endoscopic transorbital approach at the Division of Neurosurgery of the Hospital Clinic in Barcelona was analyzed. Patients features were described in detail. Complications were divided into 2 groups to analyze separately the approach-related complications, and those resulting from tumor removal. The ocular complications were subdivided into early ocular status (<3 weeks), late ocular status (3-8 weeks), and persistent ocular complications. The "Park questionnaire" was used to determine patient's satisfaction related to the transorbital approach. A total of 20 patients (5 spheno-orbital meningiomas, 1 intradiploic Meningioma, 2 intraconal lesions, 1 temporal pole lesion, 2 trigeminal schwannoma, 3 cavernous sinus lesions, and 6 petroclival lesions) were included in the period 2017-2022. Regarding early ocular status, upper eyelid edema was detected in all cases (100%) associated with diplopia in the lateral gaze in 30% of cases, and periorbital edema in 15% of cases. These aspects tend to resolve at late ocular follow-up (3-8 weeks) in most cases. Regarding persistent ocular complications, in one case of intraconal lesion, a limitation of eye abduction was detected (5%). In another patient with intraconal lesion, an ocular neuropathic pain was reported (5%). In 2 cases of petroclival menigioma, who were also treated with a ventriculo-peritoneal shunt, slight enophthalmus was observed as a persistent complication (10%). According to the Park questionnaire, no cosmetic complaints, no head pain, no palpable cranial irregularities, and no limited mouth opening were reported, and an average of 89% of general satisfaction was encountered. The superior eyelid endoscopic transorbital approach is a safe and satisfactory technique for a diversity of skull base tumors. At late follow-up, upper eyelid edema, diplopia, and periorbital edema tend to resolve. Persistent ocular complications are more frequent after treating intraconal lesions. Enophthalmus may occur in patients with associated ventriculo-peritoneal shunt. According to patient's satisfaction, fairly acceptable results are attained.

Focal drug-resistant temporal lobe epilepsy associated with an ipsilateral anterior choroidal artery aneurysm: illustrative case.

The occurrence of both an intracranial aneurysm and epilepsy, especially drug-resistant epilepsy (DRE), is rare. Although the overall incidence of aneurysms associated with DRE is unclear, it is thought to be particularly infrequent in the pediatric population. Surgical ligation of the offending aneurysm has been reported in conjunction with resolving seizure activity, although few cases have cited a combined approach of aneurysm ligation and resection of an epileptogenic focus. We present the case of a 14-year-old female patient with drug-resistant temporal lobe epilepsy and an ipsilateral supraclinoid internal carotid artery aneurysm. Seizure semiology, electroencephalography monitoring, and magnetic resonance imaging all indicated a left temporal epileptogenic focus, in addition to an incidental aneurysm. The authors recommended a combined surgery involving resection of the temporal lesion and surgical clip ligation of the aneurysm. Near-total resection and successful ligation were achieved, and the patient has remained seizure free since surgery at 1 year postoperatively. In patients with focal DRE and an adjacent intracranial aneurysm, a combined surgical approach involving both resection and surgical ligation can be used. Several surgical timing and neuroanesthetic considerations should be made to ensure the overall safety and efficacy of this procedure.

Temporal bone manifestation of primary extranodal Rosai–Dorfman disease: a case report

BackgroundRosai–Dorfman disease is a rare benign histiocytic disorder characterized in most cases by painless cervical adenopathy. Less than 10% of extranodal cases involve bony lesions. Primary bone Rosai–Dorfman disease in the absence of nodal disease is extremely rare.Case presentationA 48 year-old Caucasian male presented with progressive right-sided otalgia, tinnitus, vertigo, and hearing loss. A right temporal bone lytic lesion was detected on diagnostic imaging. Resection of the lesion and histopathological examination revealed Rosai–Dorfman disease.ConclusionsRosai–Dorfman disease primary bone lesions are an atypical presentation of a rare disease. This is the second reported case of Rosai–Dorfman disease arising within the temporal bone. This case study reveals that Rosai–Dorfman disease should be considered for patients presenting with inflammatory/lytic lesions of the temporal bone, in cases where infection and malignancy have been excluded.

Extracranial Metastasis of Glioblastoma: A Case Report

Glioblastoma is the most frequent malignant neoplastic pathology among glial neoplasms, extra neural metastases are atypical, presenting in 0.2 to 2% of cases, risk factors include recurrence of the neoplasm, early age of the patient, and prolonged survival. The most frequent localization sites of metastases are lungs, cervical lymph nodes, bone tissue. This article details the case of a 36-year-old patient with no history of previous disease who presented tonic-clonic seizures. In extension tests, a right frontal intracranial tumor was observed. It was decided to perform resection of the tumor with pathology results that indicated a grade IV right frontal glioblastoma, presents a recurrence of the disease, for which reason a surgical reintervention is warranted and subsequent treatment with radiotherapy and chemotherapy one year after the second surgery, a right temporal extracranial lesion is evidenced, which was biopsied and reported as glioblastoma with a component primitively, resection of the tumor mass was chosen with the double scalpel technique and reconstruction with a cervicofacial flap and skin graft. Late post-surgical patient with adequate pain control, without signs of inflammatory response, without evidence of neurological deficit, is discharged.

Menière's Syndrome Associated With Fibrous Dysplasia of the Temporal Bone.

Menière's Syndrome Associated With Fibrous Dysplasia of the Temporal Bone.

Hemichorea secondary to isolated temporal infarction with severe middle cerebral artery stenosis: a case report and review of literature

BackgroundHemichorea typically results from a contralateral subthalamic nuclei (STN) lesion, although it has been reported in the cortex in a minority of cases. However, to our best knowledge, there are no documented cases in literature of hemichorea occurring as a secondary condition to an isolated temporal stroke.Case presentationWe present a case of an elderly female who sustained a sudden onset of hemichorea in her right extremities, predominantly in the distal region, lasting over a period of two days. Brain diffuse weighted image (DWI) demonstrated a high signal in the temporal region, while magnetic resonance angiography (MRA) revealed severe stenosis of the middle cerebral artery. During the symptomatic phase, computed tomography perfusion (CTP) revealed delayed perfusion in the left middle cerebral artery territory, characterized by the time-to-peak (TTP) measure. Based on the results of her medical history and laboratory tests, we were able to rule out the possibility of infectious, toxic, or metabolic encephalopathy. Her symptoms gradually improved with antithrombotic and symptomatic treatment.ConclusionsIt is important to recognize and consider acute onset hemichorea as an initial symptom of stroke to avoid misdiagnosis and delays in appropriate treatment. Further research on temporal lesion that lead to hemichorea is warranted to gain a better understanding of the underlying mechanisms.

The foramen ovale: a keyhole to the brain? Computer simulations of percutaneous FO punctures

BackgroundThe percutaneous cannulation of the foramen ovale (FO) is implemented in treating trigeminal neuralgia, diagnosing temporal lobe epilepsy and biopsy petroclival lesions. This study dealt with the question whether it is possible to reach intracerebral structures with a puncture beyond the Gasserian Ganglion (GG) without bone destruction or perforating vascularity.MethodsWe considered the FO a natural keyhole and performed computer-simulated punctures through the right and left FO to eight intracerebral structures. Therefore, we took the Hartel and Submandibular (SM) approach as a starting point and planned trajectories with stereotactic planning software by using brain scans of ten patients.ResultsThe simulated punctures with the Hartel approach directly reached the hippocampus (20 out of 20 trajectories), the lateral ventricle (15/20) and the amygdala (2/20). The pons was reached (20/20); however, the pontine vascularity was within the course. The trajectories to the thalamus (13/20) ran through the hippocampus or the mesencephalon. The simulated punctures with the SM approach directly reached the amygdala (18/20), the lateral ventricle (5/20) and the putamen (20/20). The trajectories to the nucleus caudatus (20/20) pierced the hippocampus, the putamen or the maxillary artery. The courses to the thalamus (7/20) ran through the hippocampus or the mesencephalon. The sinus cavernosus could not be reached with the Hartel or SM approach.ConclusionsThis study indicates that a percutaneous approach to the hippocampus, the lateral ventricle, the amygdala and the putamen is possible without harming major vessels or bone destruction. For a possible implementation of these trajectories in a clinical setting, it is necessary to prove these simulated punctures in cadaveric studies.

Risk factors of cognitive impairment post-ischemic stroke

BackgroundStroke is still remarked as one of the major ailments in Indonesia. Cognitive impairment occurs frequently after stroke events and its predictors are needed to identify patients with a high risk of cognitive impairment. However, the researches done in Indonesia still vary in results. This retrospective cohort study evaluated 236 ischemic stroke patients in Bethesda Hospital Yogyakarta from January–November 2020 through medical records. Cognitive impairment was measured by MMSE and CDT, defined as MMSE score le 20 for patients with the educational attainment of 1–6 years; le 24 for the educational attainment of > 6 years and/ or CDT < 4. All data were analyzed with univariate test and Chi-square test, followed by logistic regression.ResultsOf 236 subjects, the frequency of cognitive impairment was 67.4% and the risk factors are age > 80 years old (RR: 1,545; CI 0.136–17.542; p: 0.026), educational attainment ≤ 6 years (RR: 20.016; CI 1.951–222.120; p: 0.012), temporal (RR: 4.238; CI 2.266–7.926; p: 0.007) or multiple site lesion location (RR: 7.522; CI: 3.522–17.532; p: 0.047) and multiple (> 1) lesions (RR: 1,819; CI 0.822–4.022; p: 0.040).ConclusionsThe prevalence of post-stroke cognitive impairment is high, affecting up to 2 out of 3 people in this study. The risk factors of post-stroke cognitive impairment are age > 80 years, educational attainment le 6 years, temporal or multiple site lesion, and > 1 lesion. Strict stroke prevention strategies are needed to halt the development of post-stroke cognitive impairment.

MRI Features and their Association with Outcomes in Children with Anti-NMDA Receptor Encephalitis (S22.003)

<h3>Objective:</h3> To correlate MRI brain lesions with clinical outcomes in pediatric anti-NMDA receptor encephalitis (pNMDARE). <h3>Background:</h3> Up to 50% of MRIs in NMDARE are reportedly abnormal. However, data is limited on range and types of abnormalities, and also how abnormal MRIs associate with outcomes in pNMDARE. <h3>Design/Methods:</h3> This was a multi-center retrospective cohort study with ten institutions. NMDARE was defined as positive CSF NMDA-receptor antibody and at least one neuropsychiatric symptom. Patients with prior HSV encephalitis were excluded. Outcomes were assessed by the modified Rankin Score (mRS) at 1 year with good (mRS ≤ 2) and poor (mRS ≥ 3) outcomes and also for full recovery (mRS=0) versus incomplete recovery (mRS&gt;0). Statistical analyses were performed using SAS 16.0 (Cary, NC). <h3>Results:</h3> A total of 169 patients with pNMDARE were included, with one-year mRS available in 134/169 (79%). Abnormal MRIs were found in 59/169 (35%), and were associated with an increased likelihood of intubation and ICU admission (chi-square, p = 0.038 and p=0.045, respectively). The most common T2 FLAIR lesion locations were frontal (29/59, 49%), temporal (28/59, 47%), parietal (19/59, 32%), and hippocampal lesions (11/59, 19%). MRI enhancement was seen in 34/165 (21%) and MRI brain atrophy was seen in 4% (6/168). MRI features that predicted incomplete recovery at one year (mRS &gt; 0) was the presence of T2 frontal lobe lesions (chi-square, p = 0.0427). G-tube placement and intubation also predicted incomplete recovery. On multivariable logistic regression analysis using backward selection, one-year mRS was associated with: intubation (OR 0.232, 95%CI 0.103–0.526) and T2 frontal lesions on MRI (OR 0.373, 95% 0.148–0.938). <h3>Conclusions:</h3> Abnormal frontal lesions on MRI along with intubation may associate with one-year mRS in pNMDARE. MRIs may be a helpful tool for prognostication, which will be examined in future studies. <b>Disclosure:</b> The institution of Dr. Gombolay has received research support from CDC. The institution of Dr. Gombolay has received research support from NIH. Dr. Brenton has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cycle Pharmaceuticals. The institution of Dr. Brenton has received research support from NIH/NINDS. Dr. Brenton has received personal compensation in the range of $500-$4,999 for serving as a Grant Reviewer with Department of Defense. Dr. Brenton has received personal compensation in the range of $500-$4,999 for serving as a Expert Interview with MDEdge. Dr. Brenton has received personal compensation in the range of $0-$499 for serving as a Grant Reviewer with NIH. Dr. Yang has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Neurology Live. Dr. Yang has received research support from United Mitochondrial Disease Foundation. The institution of Dr. Stredny has received research support from Pediatric Epilepsy Research Foundation. The institution of Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. The institution of Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi-Genzyme. Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Ogborn-Mihm Trial Lawyers. The institution of Dr. Kammeyer has received research support from Rocky Mountain Multiple Sclerosis Center. The institution of Dr. Otten has received research support from CDC. Dr. Vu has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Syneos Health. Dr. Santoro has received personal compensation for serving as an employee of UCB. Dr. Santoro has received personal compensation for serving as an employee of Cycle Pharma. Dr. ROBLES LOPEZ has nothing to disclose. Dr. Christiana has nothing to disclose. The institution of Dr. Steriade has received research support from NIH. The institution of Dr. Steriade has received research support from Dorris Duke Fund to Retain Clinician Scientists. Miss Morris has received personal compensation in the range of $10,000-$49,999 for serving as a Research Assistant/Specialist with Emory University, School of Medicine, Department of Pediatrics, Division of Neurology. The institution of Dr. Gorman has received research support from Pfizer. The institution of Dr. Gorman has received research support from Roche / Genetech . Dr. Moodley has nothing to disclose. Dr. Hardy has nothing to disclose. Dr. Kornbluh has nothing to disclose. Ilana Kahn has nothing to disclose. Dr. Sepeta has received personal compensation in the range of $500,000-$999,999 for serving as a Grant PI with Nih. Dr. Sepeta has a non-compensated relationship as a Special volunteer with Nih that is relevant to AAN interests or activities. Dr. Yeshokumar has nothing to disclose.

A Rare Familial Case of MELAS in a 66-Year-Old Female and Her 33-Year-Old Son (P12-5.005)

<h3>Objective:</h3> Mitochondrial encephalopathy, lactic acidosis, and stroke like episodes (MELAS) is a mitochondrial disorder presenting most commonly between 2 to 40 years of age, with 75% of cases before age 20. Rare cases have been reported past age 60. We report on a case of a family presenting with late onset MELAS in the same year. <h3>Background:</h3> A 66-year-old female with a past medical history of sensorineural hearing loss, type 2 diabetes, hypertension and hyperlipidemia presented with word finding difficulty and right sided neglect. MRI demonstrated a DWI lesion in the left temporal lobe consistent with infarct. Vessel imaging, echocardiogram, and cardiac monitor were unremarkable, and she was discharged on clopidogrel. She returned 11 days later with worsening aphasia and MRI revealed extension of her stroke into the left occipital lobe and right cerebellum. It was then discovered her previously healthy 33-year-old son had been diagnosed with MELAS three months prior. The son had presented with subtle speech difficulties and was found to have a right temporal DWI and T2 hyperintense lesion on MRI. CSF and serum studies were unremarkable for infectious and autoimmune causes. He was discharged following improvement of symptoms but returned the following day after having generalized seizure. MRI revealed expansion of the lesion into the right parietal lobe. MR Spectroscopy showed an elevated lactate peak leading to a presumptive diagnosis of MELAS. He was started on L-Arginine and CoQ10. In both cases, genetic testing revealed a pathogenic MT-TL1 m.3243A&gt;G variant consistent with MELAS (9.7% heteroplasmy in mother and 31% heteroplasmy in son). <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> This case demonstrates low heteroplasmy can lead to a later presentation of MELAS requiring MELAS to be on the differential even in people older than 60. <b>Disclosure:</b> Dr. Iordanova has nothing to disclose. Dr. Wong has nothing to disclose. Dr. Cheng has nothing to disclose.

Overlapping and Distinguishing Features Among MS, NMOSD, MOGAD and Other Atypical Demyelinating Syndromes of the Central Nervous System (P11-3.016)

<h3>Objective:</h3> To describe and identify the clinical-demographic, imaging and serological overlapping and distinguishing features among MS, NMOSD, MOGAD, and atypical demyelinating syndromes (ADS) in an attempt to define whether ADS-suggestive diagnostic indicator(s) emerge. <h3>Background:</h3> The era of the enlarging spectrum of MS, NMOSD, MOGAD, and a subgroup of patients with ADS overlapping features has resulted in diagnostic and therapeutic challenges. Since there is a significant diagnostic gap in some patients due to these overlapping features, further research is warranted to describe better diagnostic markers. <h3>Design/Methods:</h3> A total of 204 MS patients fulfilling the McDonald 2017 criteria, 36 AQP4-IgG+ NMOSD patients, 21 double-seronegative NMOSD patients fulfilling the 2015 diagnostic criteria for NMOSD, 15 MOG-IgG+ patients, and 80 ADS patients not fulfilling any of the aforementioned diagnostic criteria were included. Clinical, serological, and neuroimaging findings of the patients were retrospectively evaluated. <h3>Results:</h3> Positive oligoclonal bands (OCBs) in the CSF were found to be significantly higher in the MS and ADS groups than in both seropositive and seronegative NMOSD groups (all p&lt;0.001). The MS group had the highest average number of periventricular (PV) lesions(≥3 lesions) compared with the AQP4+NMOSD and MOGAD groups(no lesions, P&lt;0.001) and the ADS and seronegative NMOSD groups(1–2 lesions, P&lt;0.001). Temporal PV lesions and ovoid PV lesions were significantly higher in MS patients than in ADS patients(both p&lt;0.001). Juxtacortical/cortical (JC/C) lesions were significantly higher in the MS group than in the ADS group(p&lt;0.001). <h3>Conclusions:</h3> Differential features of overlapping demyelinating disorders revealed imaging features such as the presence of temporal PV, ovoid PV, and JC/C lesions, which are highly suggestive of MS, whereas the absence of these imaging features, absence of all diagnostic criteria as well as negative antibody serology but positive OCBs are more common in ADS. Further research and molecular diagnostic markers are required to elaborate on these results and fill this diagnostic gap. <b>Disclosure:</b> Bade Gulec has nothing to disclose. Samet Cam has nothing to disclose. Dr. Bilge has nothing to disclose. Melih Tutuncu has nothing to disclose. Sabahattin Saip, 6729 has nothing to disclose. Ugur Uygunoglu has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche Turkey . The institution of Ugur Uygunoglu has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. The institution of Ugur Uygunoglu has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen Idec/Gen Pharma of Turkey. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi-Genzyme. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen - TR. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abdi Ibrahim Ilac - TR. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen - TR. Dr. Siva has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck Serono . The institution of Dr. Siva has received research support from Turkish MS Society. The institution of Dr. Siva has received research support from The Scientific and Technological Research Council Of Turkey - Health Sciences Research Grants.

Discrepancies in the late auditory potentials of post-anoxic patients: Watch out for focal brain lesions, a pilot retrospective study

AimsLate auditory evoked potentials, and notably mismatch negativity (MMN) and P3 responses, can be used as part of the multimodal prognostic evaluation in post-anoxic disorders of consciousness (DOC). MMN response preferentially stems from the temporal cortex and the arcuate fasciculus. Situations with discrepant evaluations, for example MMN absent but P3 present, are frequent and difficult to interpret. We hypothesize that discrepant MMN-/P3+ results could reflect a higher prevalence of lesions in MMN generating regions. This study presents correlations between neurophysiological and neuroradiological results. MethodsThis retrospective study was conducted on 38 post-anoxic DOC patients. Brain lesions were analyzed on 3T MRI both anatomically and through computation of the local arcuate fasciculus fractional anisotropy values on Diffusion Tensor Imaging sequences. Neurophysiological data and outcome were also analyzed. ResultsOur cohort included 8 MMN-/P3+, 7 MMN+/P3+, 21 MMN-/P3− and 2 MMN-/P3+ patients, assessed at a median delay of 20.5 days since cardiac arrest. Our results show that MMN-/P3+ patients tended to have fewer temporal and basal ganglia lesions than MMN-/P3− patients, and more than MMN+/P3+ patients (p-values for trend: p = 0.02 for temporal and p = 0.02 for basal ganglia lesions). There was a statistical difference across groups for mean fractional anisotropy values in the arcuate fasciculus (p = 0.008). The percentage of patients regaining consciousness at three months in MMN-/P3+ patients was higher than in MMN-/P3− patients and lower than in MMN+/P3+ patients. ConclusionThis study suggests that discrepancies in late auditory evoked potentials may be linked to focal post-anoxic brain lesions, visible on brain MRI.

Teaching Neuro Images : Infantile herpes simplex encephalitis

A 7-month-old girl developed fever, unconsciousness, and left oral clonic seizures. EEG showed background slowing and periodic lateralized epileptiform discharges (PLED) over right central-temporal regions. Brain MRI revealed a right temporal pole T2 hyperintense lesion (figure 1). CSF PCR was positive for herpes simplex virus 1. Five days after starting treatment with acyclovir (45 mg/kg/day) and phenobarbital (7.5 mg/kg/day), the patient was alert and without seizures, and EEG activity improved. Two weeks later, MRI showed a right temporal-polar malacic area (figure 2), and PCR was negative. Although seen in many diseases,1,2 PLED suggest herpes simplex encephalitis in the appropriate clinical context.

Application of Radiomics to the Differential Diagnosis of Temporal Bone Skull Base Lesions: A Pilot Study.

Temporal bone skull base pathologies represent a complex differential because they can be radiographically obscure and difficult to diagnose without biopsy. Radiomics involves the use of mathematical quantification of imaging data beyond simple intensity, size, and location to inform diagnosis and prognosis. We examined the feasibility of using radiomic parameters to help predict temporal bone tumor type. A total of 117 radiomic parameters were analyzed from 5 magnetic resonance imaging sequences (T1 without contrast, T1 with contrast, T2, fluid-attenuated inversion recovery, apparent diffusion coefficient [ADC]) for each tumor. Statistical analysis was used to delineate known primary, metastatic/secondary, and lymphoma lesions using radiomics. The mean tumor volumes for the 14 primary, 12 secondary, and 8 lymphoma lesions were 2.98 ± 2.11, 3.28 ± 2.31, and 12.16 ± 7.1cm3, respectively (P= 0.2). No significant differences in mean intensity values for any sequence helped distinguish tumors (P > 0.05), but 6 radiomic parameters were significantly correlated with diagnostic accuracy. Discriminant analysis using a stepwise algorithm generated a model where radiomic parameters for T1 cluster prominence, ADC dependence nonuniformity, T1 with contrast zone percentage, and ADC informational measure of correlation 2 achieved the best predictive model (P= 0.0001). These significant characteristics were often indirect measures of tumor heterogeneity on different magnetic resonance imaging sequences. These data suggest that quantitative measures of tumor heterogeneity can be discriminatory of pathology and might be integrated into clinical workflow. Although this pilot study requires further validation, these data support the exploration of radiomics in temporal bone radiographic diagnostics.

Neurosyphilis Presenting as Syndrome of Limbic Encephalitis Mimicking Herpes Simplex Virus Neuro-Infection Diagnosed Using CXCL13 Point-of-Care Assay—Case Report

The syndrome of limbic encephalitis is a severe clinical condition with heterogenous aetiopathogenesis. A common pathogen causing the infectious syndrome of limbic encephalitis is herpes simplex virus (HSV), but rare cases caused by Treponema pallidum have also been reported. We present the case of a 46-year-old man who presented with sudden onset of headaches, nausea, vomiting, and short-term loss of consciousness with clonic convulsions and subsequent disorientation and aphasia. Examination of the cerebrospinal fluid (CSF) revealed lymphocytic pleocytosis and magnetic resonance of the brain revealed bilateral temporal lesions. Clinical, radiologic, and biochemical examinations of CSF suggested encephalitis caused by HSV. However, the positivity of CXCL-13 chemokine in the CSF by a rapid point-of-care assay suggested active spirochetal infection and led to further serologic investigation. The definitive diagnosis of neuro-syphilis was concluded by positive intrathecal synthesis of immunoglobulins against Treponema pallidum. Penicillin therapy led to a rapid improvement, and the patient was discharged home after three weeks. Due to memory problems and irritability, after eighteen months, he came for a follow-up neurological and psychological examination. The psychological examination revealed a significant deficit in executive functions and behavioural changes. Neurosyphilis should be considered in the differential diagnosis of limbic encephalitis with lymphocytic pleocytosis in cerebrospinal fluid, and CXCL-13 may help to achieve diagnosis.

Cerebral Toxoplasmosis in a Previously Fit Individual – Pitfalls in Management – A Case Report.

Most people with toxoplasmosis infection are asymptomatic. Cerebral toxoplasmosis in HIV patient represents poor prognostic determinant, but treatable if early treatment is initiated. A case of delayed diagnosis of cerebral toxoplasmosis is presented. A 27-year-old man presented with one-sided facial numbness and treated as Bell’s Palsy. Later he developed slurred speech. Contrast enhanced CT brain showed left frontal temporal hypodense lesion, leptomeningeal enhancement and perilesional oedema. Steroid was started. Four days later, he presented with decreased responsiveness. Retroviral was detected and diagnosis changed to opportunistic brain infection. He died after 3 days. Toxoplasmosis IgG antibodies turned out positive. In diagnosing cerebral toxoplasmosis, clinical presentation and examination are as important as laboratory testing and radiological imaging.

Music perception in acquired prosopagnosia

BackgroundAcquired prosopagnosia is often associated with other deficits such as dyschromatopsia and topographagnosia, from damage to adjacent perceptual networks. A recent study showed that some subjects with developmental prosopagnosia also have congenital amusia, but problems with music perception have not been described with the acquired variant. ObjectiveOur goal was to determine if music perception was also impaired in subjects with acquired prosopagnosia, and if so, its anatomic correlate. MethodWe studied eight subjects with acquired prosopagnosia, all of whom had extensive neuropsychological and neuroimaging testing. They performed a battery of tests evaluating pitch and rhythm processing, including the Montréal Battery for the Evaluation of Amusia. ResultsAt the group level, subjects with anterior temporal lesions were impaired in pitch perception relative to the control group, but not those with occipitotemporal lesions. Three of eight subjects with acquired prosopagnosia had impaired musical pitch perception while rhythm perception was spared. Two of the three also showed reduced musical memory. These three reported alterations in their emotional experience of music: one reported music anhedonia and aversion, while the remaining two had changes consistent with musicophilia. The lesions of these three subjects affected the right or bilateral temporal poles as well as the right amygdala and insula. None of the three prosopagnosic subjects with lesions limited to the inferior occipitotemporal cortex exhibited impaired pitch perception or musical memory, or reported changes in music appreciation. ConclusionTogether with the results of our previous studies of voice recognition, these findings indicate an anterior ventral syndrome that can include the amnestic variant of prosopagnosia, phonagnosia, and various alterations in music perception, including acquired amusia, reduced musical memory, and subjective reports of altered emotional experience of music.