Left Lesion Research Articles

Which components of famous people recognition are lateralized? A study of face, voice and name recognition disorders in patients with neoplastic or degenerative damage of the right or left anterior temporal lobes

We administered to large groups of patients with neoplastic or degenerative damage affecting the right or left ATL, the ‘Famous People Recognition Battery’ (FPRB), in which subjects are required to recognize the same 40 famous people through their faces, voices and names, to clarify which components of famous people recognition are lateralized. At the familiarity level, we found, as expected, a dissociation between a greater impairment of patients with right ATL lesions on the non-verbal (face and voice) recognition modalities and of those with left ATL lesions on name familiarity. Equally expected were results obtained at the naming level, because the worse naming scores for faces and voices were observed in left-sided patients. Less foregone were, for two reasons, results obtained at the semantic level. First, no difference was found between the two hemispheric groups when scores obtained on the verbal (name) and non-verbal (face and voice) recognition modalities were account for. Second, the face and voice recognition modalities showed a different degree of right lateralization. All groups of patients showed, indeed, both at the familiarity and at the semantic level, a greater difficulty in the recognition of voices regarding faces, but this difference reached significance only in patients with right ATL lesions, suggesting a greater right lateralization of the more complex task of voice recognition. A model aiming to explain the greater right lateralization of the more perceptually demanding voice modality of person recognition is proposed.

Clinical Outcome of Intravascular Ultrasound Guided Percutaneous Coronary Intervention of Isolated Ostial Left Anterior Descending Artery Lesions

Purpose: Despite recent advances in interventional cardiology procedures, isolated ostial left anterior descending (LAD) lesions remain a challenge in cardiology. Due to the probability of left main (LM) affection, an LM bifurcation stenting technique may be required in certain individuals. So we evaluated result of Intravascular Ultrasound (IVUS) guided percutaneous coronary intervention (PCI) in isolated LAD coronary artery lesions, either by crossing over the ostium or not, with a focus on major adverse cardiovascular events (MACE).
 Methodology: Our prospective study from January2021 to November 2022 included 79 isolated ostial LAD patients with Ostial LAD stenting (OS) or LM to LAD cross-over (CO) stenting at the National Heart Institute. As per the recommended guidelines, the participants were divided into two groups: the first one had IVUS guided PCI and Group (2) had Angiography guided PCI. The data was collected and statistically analyzed with SPSS 23.0 program.
 Findings: No statistically significant difference was present between the groups regarding socio-demographic or clinical data (P-value > 0.05). It was discovered that an increase in Rotablator use, a decrease in Fluoroscopy time (min), a decrease in Contrast volume (ml), and a decrease in PCI duration (min) in patients who had IVUS guided PCI, with statistically significant difference when compared to other group who had Angiography guided PCI (P-value < 0.05). In addition, it was discovered that an increase in the necessity for a cross-over stenting method in group (1), with no statistically significant difference (P-value > 0.05). High prevalence of mortality and morbidity among IVUS guided PCI patients was present with statistical significance regarding TVR (P-value < 0.05).
 Recommendations: IVUS can offer valuable information on vascular lumen, plaque features, stent deployment, & device failure causes. As a result, IVUS-guided PCI may enhance clinical impact among participants, particularly those with complicated coronary lesions & those at high risk. Further reduction in IVUS’s cost, cardiologists’ education and enhancing IVUS use at PCI should be adapted next.

Mitral Valve Replacement for Enlarged Libman-Sacks Endocarditis in a Patient with Persistent Primary Antiphospholipid Syndrome and Recurrent Stroke: A Case Report.

BACKGROUND Anticardiolipin antibodies in patients with Libman-Sacks endocarditis (LS) are indicative of comorbid antiphospholipid syndrome (APS) and can result in cerebral infarctions. We describe a case of LS and primary APS with recurrent cerebral infarctions despite anticoagulation treatment. The patient underwent surgery for enlarged LS vegetation with high titers of antiphospholipid antibodies. CASE REPORT A 41-year-old Japanese man was admitted to hospital for small cerebral infarction recurrence in a left parietal lesion. At age 35, the patient had suffered multiple cerebral infarctions. He was found to have high serum titers of all 3 antiphospholipid antibodies. Transesophageal echocardiography (TEE) findings were normal. Differential diagnosis ruled out other autoimmune diseases and a clinical diagnosis of primary APS was made. Warfarin anticoagulation was started. When cerebral infarction recurred 6 years after the first episode, serum titers of antiphospholipid antibodies remained high, and TEE showed a 7×8 mm area of mitral vegetation. A TEE results from his first admission revealed a 5×6 mm area of mitral vegetation, which was believed to be related to the current vegetation. As anticoagulation produced no improvement, the mitral valve was replaced with a mechanical valve. Examination of the excised vegetation found it to be consistent with LS. The patient made good progress within 3 years after surgery. CONCLUSIONS LS size can increase despite anticoagulation in cases with high titers of all 3 antiphospholipid antibodies and cerebral infarction. Such patients require ongoing TEE follow-up and surgical treatment should be considered.

Quantitative late gadolinium enhancement cardiac magnetic resonance analysis of the relationship between ablation parameter and left atrial tissue lesion following pulmonary vein isolation.

The impact of ablation parameters on acute tissue lesion formation after pulmonary vein isolation (PVI) has not been sufficiently evaluated in patients with atrial fibrillation. Radiofrequency ablation lesion can be visualized by late gadolinium enhancement cardiac magnetic resonance (LGE-CMR). We sought to quantitatively analyze the relationship between ablation parameter and tissue lesion following PVI at different segments of pulmonary vein (PV) using LGE-CMR. Twenty-one patients with atrial fibrillation who underwent PVI procedure were retrospectively enrolled. All patients underwent LGE-CMR examination within 3 days after radiofrequency ablation. Ablation parameters during PVI were documented, including lesion size index (LSI), force-time integral (FTI), power, contact force, temperature, and time of duration. The ablation point was projected onto 3-dimensional (3D) left atrial shell constructed base on LGE-CMR and corresponding image intensity ratio (IIR) was calculated on the same shell. A tissue lesion point was defined when the LGE-CMR IIR was > 1.2. In total, 1,759 ablation points were analyzed. The ablation parameters and IIRs for each PV segment were significantly different (P < 0.0001). IIRs corresponding to ablation points at posterior of PV tended to be higher than those at non-posterior of PV when similar ablation parameters were applied during ablation. LSI was a better predictor of tissue lesion existence following PVI than FTI, contact force, power, temperature, and duration time at non-posterior wall of PV. The IIR showed positive correlation with LSI at non-posterior wall of PV (non-posterior of right PV, r = 0.13, P = 0.001, non-posterior of left PV, r = 0.26, P < 0.0001). When similar ablation parameters were applied during PVI, the posterior wall of PV had more severe tissue lesion than non-posterior wall of PV. Therefore, it was reasonable to decrease ablation energy at posterior wall of PV. Moreover, LSI was a better index to reflect tissue lesion quality following PVI at non-posterior of PV.

Feasibility of Transcranial Motor Evoked Potentials and Electromyography during MRI-Guided Laser Interstitial Thermal Therapy for Glioblastoma

Intraoperative neuromonitoring (IONM) is routinely used during neurosurgical procedures. Magnetic resonance imaging (MRI)-guided laser interstitial thermal therapy (LITT) is increasingly being used in patients with various brain lesions. Use of IONM (transcranial motor evoked potential [TcMEP] and electromyography [EMG]) during LITT of a brain lesion has not been described previously. In this report, we describe a 70-year-old man who presented with motor weakness in whom imaging revealed a left thalamic lesion. Due to the difficulty in accessing the lesion and proximity to the motor tracts, patient was offered MRI-guided LITT using TcMEP and EMG. The patient underwent satisfactory ablation of the lesion with successful recording of the TcMEP and EMG. Technical nuances related to the set-up and procedure is discussed in this report. No procedure-related complications were encountered. We describe the first report of safety and feasibility of TcMEP and EMG during MRI-guided LITT for left thalamic glioblasatoma. This report paves the way for further prospective investigations regarding the utility of this technique for eloquent brain tumors.

Dosimetric and Volumetric Analysis in Endobronchial Brachytherapy in Lung Carcinoma: A Cross-sectional Study

Introduction: High Dose Rate (HDR) brachytherapy plays an important role in the treatment of lung carcinoma. The treatment of lung carcinoma with Endobronchial Brachytherapy Treatment (EBBT) is delivered with three fractions and the effect of EBBT on the Target Volume (TV) after delivering the three fractions in the lung carcinoma needs to be assessed. The TV is covered with the prescribed dose and Organs At Risk (OARs) doses are evaluated. Aim: To assess the doses to OAR nearby the tumour and analyse the effect of the TV, tumour location, and site on the doses to OARs in EBBT in lung carcinoma. Materials and Methods: A cross-sectional study was conducted in the Department of Radiation Oncology, Cancer Research Institute, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University Dehradun, Uttarakhand, India, from January 2018 to December 2020. Thirty patients with lung carcinoma were included in dosimetric and volumetric assessments. A flexible lumencare catheter was inserted into the bronchial lesion. Computed Tomography (CT) scan was acquired and exported to Treatment Planning System (TPS) through Digital Imaging and Communications in Medicine (DICOM) networking system. An optimised treatment plan was generated. The TV and OARs were delineated on the CT scan of the patient. A total of three EBBT sessions were given with a 7 Gy dose per fraction and prescribed the dose at 1.0 cm from the center of the catheter. Doses to OARs and the effects of TV on doses to OARs were evaluated with the help of “Dose Volume Histogram (DVH) tool” in the TPS. Thirty patients, with varying TV and site, were grouped as left lung and right lung tumour lesions and also grouped as TV &lt;22 cc and TV &gt;22 cc for the analysis purpose in this study. The data was entered in Microsoft Office Excel 2007 and analysed in Statistical Package for the Social Sciences (SPSS) version 22.0 statistical analysis software (IBM Corp., Armonk, N.Y., USA) tool. Results: The mean doses to OARs in 1st, 2nd and 3rd EBBT sessions were within their tolerance limit. The mean dose difference between left and right lung tumour site were analysed and found mainly the mean dose to oesophagus and maximum dose to oesophagus, contralateral lung, left coronary artery and descending aorta were significantly higher in left lung compared to right lung with p-value 0.015, 0.027, 0.001, 0.007 and 0.001, respectively. The maximum dose to the contralateral lung and spinal cord were significantly higher in middle-lower bronchial lesion with p-value 0.024 and 0.023, respectively. The mean dose difference between left and right lung tumour volume for TV &lt;22 cc and TV&gt;22 cc was analysed and found mainly for the group TV&gt;22 cc the mean dose to oesophagus and maximum dose to oesophagus, Heart, contralateral lung, left coronary artery and descending aorta were significantly higher in the left lung compared to right lung with p-value 0.002, 0.008, 0.027, 0.003, 0.006 and 0.001, respectively whereas in the TV&lt;22 cc group only the contralateral lung max dose was significantly higher in left lung compared to right lung with p-value 0.046. Conclusion: The OARs doses were increased significantly in left lung compared to right lung carcinoma. The TV was large in the middle-lower bronchial region, therefore, the doses were found higher, and TV in the lower bronchial region is less so the dose was less.

PS-BPR03-4: STAGED RESECTION OF SUSPECTED BILATERAL PHAEOCHROMOCYTOMA IN A PATIENT WITH END-STAGE KIDNEY DISEASE ON HAEMODIALYSIS

Objective: Phaeochromocytoma is a rare finding in the dialysis population. The usual complexities of its management are complicated by diagnostic considerations of anuria and catecholamine variability in relation to dialysis, as well as reduced margins of error in perioperative fluid balance. To date, available case reports detailing management of phaeochromocytoma in dialysis patients have described unilateral lesions. We describe the management of a haemodialysis patient with resistant hypertension secondary to suspected bilateral phaeochromocytoma, to undergo staged surgical resection. We report on the surgical outcomes of the initial staged resection as well as the effect of dialysis timing (pre- and post-dialysis) on laboratory plasma metanephrine measurements. Design and Methods: Case Report A 73-year-old male on home haemodialysis presents with resistant hypertension. He has a background of end-stage kidney disease (ESKD) secondary to hypertensive nephrosclerosis, vascular disease, and Type 2 diabetes mellitus. CT imaging performed for back pain incidentally revealed contrast-enhancing lesions in the left and right adrenal glands, measuring 5.5 cm and 1.4 cm respectively (Figure 1A). PET-CT demonstrated FDG-avid lesions in bilateral adrenal glands with marked activity on PET Gallium Dotatate study, consistent with neuroendocrine tumours (Figure 1B). In the context of resistant hypertension, these were favoured to represent phaeochromocytomas. Plasma normetanephrine levels were consistent with this diagnosis at 6,600pmol/L (Ref &lt; 1,079pmol/L). Blood pressure was not adequately controlled with the original anti-hypertensive regimen (irbesartan, lercanidipine, atenolol, and prazosin), with systolic values of 170–210mmHg, and was altered in the work-up for surgical resection of the lesions. Maximum alpha-receptor blockade was achieved with high-dose phenoxybenzamine (300 mg/day), and a beta-blocker (propranolol) and calcium-channel blocker (nifedipine) were added to attain pre-operative systolic blood pressures of 140–160mmHg with expected postural falls. Preoperative fluid status was adjusted via haemodialysis ultrafiltration, balancing the risks of intraoperative hypotension with anaesthetic considerations of potential pulmonary oedema. Results: The patient underwent laparoscopic resection with initial removal of the left adrenal lesion (Figure 1C), confirmed on histopathology to be phaeochromocytoma (Figure 1D). Blood pressures intraoperatively were maintained with minimal vasopressor support, and postoperatively with no support, consistent with expected outcomes in a staged resection of suspected bilateral phaeochromocytomas. A right adrenalectomy is planned in two months. Conclusion: This is the first reported case of suspected bilateral phaeochromocytoma in an ESKD patient on haemodialysis, and highlights the associated complexities of diagnosis and management. We demonstrate staged resection of bilateral lesions can be a safe approach in this high-risk population.

Cervical Nerve Root Cavernoma – Case Report and Literature Review

Spinal cavernous angiomas are uncommon vascular malformations in the spine accounting for 5%-12% of all spinal vascular lesions. When present in an intradural extra-medullary location, these usually present with radicular pain and neurological deficits due to mass effect (myelopathy). Herein, we present an atypical presentation of cavernous angioma in a 54-year-old man with tinnitus, headache and sensorineural hearing loss. We have also reviewed 51 cases of intradural extramedullary cavernous angiomas including our case with respect to demographic and clinical profile. A 54-year-old man presented with tinnitus in the left ear and occipital headache with neck pain and slight weakness of left-hand grip along with atrophy of thenar muscles. His pure tone audiometry (PTA) test reveled mild left sensorineural hearing loss. Magnetic resonance imaging (MRI) of cervical spine showed T2WI heterogeneously hyperintense left intradural extramedullary lesion at C7 vertebral body level. It was avidly enhancing with contrast. The patient underwent C7 laminectomy with a midline durotomy and complete excision of the lesion under neuromonitoring with sacrifice of the C8 sensory root. His symptoms improved following the surgery. The diagnosis of a cavernoma in an unusual location in the presence of cranial nerve dysfunction needs a high degree of diagnostic suspicion. Most of these cavernomas have a nerve root origin or attachment. The optimal treatment is microsurgical en bloc resection which leads to an effective resolution of both the symptoms.

Non-Small Cell Lung Cancer Presenting as Anterior Nasal Mass and Epistaxis.

Lung malignancy presentation with an uncommon metastatic site is a diagnostic challenge and often associated with poor prognosis. Nasal cavity is a rare metastatic site for any type of lung cancer. We report an unusual case of poorly differentiated adenosquamous carcinoma of the lung with widespread metastasis presenting as a right vestibular nasal mass with epistaxis. A 76-year-old male patient with chronic obstructive pulmonary disease and 80 pack-year smoking history presented with spontaneous epistaxis. He reported a new rapidly growing right-sided nasal vestibular mass first noticed 2 weeks prior. Physical examination showed fleshy mass with crusting in right nasal vestibule along with a left nasal domus mass. Imaging revealed an ovoid mass in the right anterior nostril and a large mass in the right upper lobe of the lung (RULL) along with thoracic vertebral sclerotic metastasis and large left frontal lobe hemorrhagic lesion with severe vasogenic edema. Positron emission tomography scan showed large right upper lobe mass and suspected to be the primary malignancy along with widespread metastasis. Biopsy of the nasal lesion revealed poorly differentiated non-small cell carcinoma with squamous and glandular features. The diagnosis of very poorly differentiated adenosquamous carcinoma of the lung with widespread metastasis was made. In conclusion, unusual metastatic sites with unknown primary lesions require a thorough diagnostic workup involving biopsy and extensive imaging. Lung cancer with unusual metastatic sites is inherently aggressive and associated with poor prognosis. Multidisciplinary treatment modalities should be employed keeping in view the functional status and comorbidities of the patient.

Stereo-EEG tailored resection in a child with presumed perinatal post-stroke epilepsy: The complex organization of epileptogenic zone.

Only a few studies have focused on tailored resection in post-stroke epilepsy, in which hemispherectomy and hemispherotomy are the most recognized treatments. We describe the case of a patient with drug-resistant, presumed perinatal, post-stroke epilepsy and moderate right hemiparesis. The seizures were stereotyped, both spontaneous and induced by sudden noises and somatosensory stimuli. Considering the discordant anatomic-electro-clinical data - left perisylvian malacic lesion with electrical onset over the left mesial fronto-central leads - and the patient's functional preservation, SEEG was performed. SEEG revealed sub-continuous abnormalities in the perilesional regions. Several seizures were recorded, with onset over the premotor area, rapidly involving the motor and insular-opercular regions. We decided for a combined surgical approach, SEEG-guided radiofrequency thermocoagulation, on the fronto-mesial structure but also on the central operculum, followed by resective surgery including only the fronto-mesial structures. The SEEG allowed to localize the epileptogenic zone far away from the anatomical lesion but connected to part of it. A combined surgical approach tailored on SEEG results allowed a good outcome (Engel Ib) without additional deficits.

Intra-Operative Supplementary Motor Area Aphosia During Awake Craniotomy a Case Report

Introduction: Language function is complex, involving association between Broca’s motor speech area, Wernicke’s sensory speech area and various interconnected cortical and sub-cortical regions. For lesions in eloquent areas, awake craniotomy with intra-operative neurological monitoring of motor and language function, aids in maximal safe resection of lesion with minimal neurological deficit. Case presentation: We present a case of 40-year-old patient with left frontal lobe lesion involving motor and speech area who underwent awake craniotomy under scalp block and titrated sedation. Though resection was in safe zone as marked both by neuro-navigation and direct electrical stimulation, patient developed aphasia intra-operatively. The aphasia resolved post-operatively with speech therapy over two weeks. Resection in Supplementary motor area (SMA) in the dominant hemisphere may be the likely cause of aphasia in this patient, resulting in reversible SMA syndrome. Conclusion: SMA syndrome must be considered as differential diagnosis of deficit during awake craniotomy when resection is in SMA. Keywords: Aphasia, Supplementary motor area, Awake craniotomy, Eloquent areas

Gait asymmetry in stroke patients with unilateral spatial neglect

The recovery of independent gait represents one of the main functional goals of the rehabilitative interventions after stroke but it can be hindered by the presence of unilateral spatial neglect (USN). The aim of the paper is to study if the presence of USN in stroke patients affects lower limb gait parameters between the two body sides, differently from what could be expected by the motor impairment alone, and to explore whether USN is associated to specific gait asymmetry. Thirty-five stroke patients (right or left lesion and ischemic or hemorrhagic etiology) who regained independent gait were assessed for global cognitive functioning and USN. All patients underwent a gait analysis session by using a wearable inertial system, kinematic parameters were computed. Enrolled patients presented altered motion parameters. Stroke patients with USN showed specific asymmetries in the following parameters: stance phase, swing phase, and knee range of motion. No differences in the clinical scores were found as the presence of USN. The presence of USN was associated with a specific form of altered gait symmetry. These findings may help clinicians to develop more tailored rehabilitative training to enhance gait efficacy of patients with motor defects complicated by the presence of selected cognitive impairments. Overview of the experiment setup. The workflow shows: diagnosis of unilateral spatial neglect by the neuropsychologist, sensors placement, gait analysis protocol and evaluation of the gait asymmetry together with the statistically significant features.

Patterns of placental injury in various types of fetal congenital heart disease.

Fetal blood circulation may be modified in congenital heart disease (CHD). This retrospective analysis was performed to study whether the type of CHD is associated with specific placental pathology. Three types of CHD based on presumed proportion of placental and systemic blood distribution in fetal circulation were analyzed: Group 1: 89 cases with low placental blood content (hypoplastic left heart syndrome, transposition of great arteries, coarctation of aorta), Group 2: 71 placentas with intermediate placental and systemic blood content due to increased intracardiac blood mixing (tetralogy of Fallot, truncus arteriosus, double inlet/outlet ventricle), and Group 3: 24 placentas with high placental blood content (tricuspid or pulmonary atresia, Ebstein anomaly). Frequencies of 27 independent clinical and 47 placental phenotypes of 184 placentas in those three groups were statistically compared. The most advanced gestational age at delivery, and large vessel (global) fetal vascular malperfusion (FVM) were most common in Group 1, while macerated stillbirths, neonatal mortality, abnormal amniotic fluid volume (oligohydramnios or polyhydramnios), other congenital anomalies, distal villous lesions of FVM, placental edema and amnion nodosum were most common in Groups 2 and 3, although the frequencies of placental lesions were statistically not significant. Left heart obstructive lesions potentially associated with brain maldevelopment show increase in lesions of global FVM (in aggregate and individually fetal vascular ectasia, stem vessel obliteration and intramural fibrin deposition) as may be seen in umbilical cord compromise. CHD with increased intracardiac blood mixing or with right heart defects is associated with average preterm gestational age at delivery and placental lesions of distal villous FVM, villous edema and amnion nodosum.

Acute Corticosteroid Responsive Meningoencephalitis with Cerebral Vasculitis after COVID-19 Infection in a Thirteen-Year-Old Case Report.

Various neurologic manifestations have already been described in children during or after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections. The central nervous system disorders reported in children are mainly encephalopathies during multisystem inflammatory syndrome. We present here an acute meningoencephalitis with cerebral vasculitis associated to a coronavirus disease 2019 (COVID-19) infection in a 13-year-old girl with a 1-year clinical, electroencephalogram (EEG), and magnetic resonance imaging (MRI) follow-up. A 13-year-old girl presented acute symptoms of consciousness impairment, frontal headache, hyperthermia, and aphasia, with moderate lymphopenia (900/mm3), elevated C-reactive protein (17 mg/L), cerebrospinal fluid (CSF) pleocytosis (15 cells/mm3), slow background with frontal focalization on EEG, a left frontal ischemic lesion, leptomeningeal enhancement, and bilateral limbic fluid-attenuated inversion recovery hyperintensity on cerebral MRI. Reverse transcription-polymerase chain reaction for SARS-CoV-2 was positive in nasopharyngeal swab and COVID serology was positive for immunoglobulin (Ig) M and G, whereas extensive autoimmune antibody investigation was negative except for a positive low titer of anti-myelin oligodendrocyte glycoprotein in CSF and blood. The diagnosis of probable encephalitis associated to cerebral vasculitis after COVID infection was suggested and steroids pulse were started. She recovered within a few days. Six months later, she had moderate clinical sequels including persistent intermittent headaches, an isolated spatial deficit, and focal spikes on the EEG without argument for epilepsia. A teenager without previous medical history presented with acute encephalitis with leptomeningitis and vasculitis after a recent COVID-19 infection. Steroids pulse therapy allowed clinical improvement. Cerebral MRI and EEG helped diagnosis, follow-up of the encephalitis, and evolution after treatment.

Retinoblastoma with Isolated Parotid Gland Metastasis: A Report of 2 Cases with Review of Literature.

Retinoblastoma, the most common primary intraocular malignant neoplasm in young children, show frequent metastasis to lymph nodes, central nervous system and bones. However, retinoblastoma metastasizing to parotid gland is very rare. We here present 2 cases of isolated parotid gland metastasis in two children with unilateral retinoblastoma. Case 1 was a one-year-old child presenting with left globe lesion which was diagnosed as Retinoblastoma. Post chemotherapy, enucleation was done which on histopathology examination showed retinoblastoma with scleral invasion and anterior chamber seeding. On 3 month follow up, patient presented with left parotid swelling. He underwent Fine needle aspiration cytology (FNAC) and Core needle biopsy which confirmed retinoblastoma metastasizing to parotid following which superficial parotidectomy was done as it was a solitary hotspot on PET-CT followed by localized radiotherapy and chemotherapy. While case 2, a 6-year-old child presented with left parotid swelling with a history of undergoing enucleation 3 months earlier in an outside hospital; Enucleation slides were reviewed in addition to patient undergoing FNAC and Biopsy from parotid swelling, all of which were consistent with Retinoblastoma metastasizing to parotid; This patient was lost to follow up; Although rare with only a few case reports in literature, both the pathologist and the clinician should be aware of retinoblastoma metastasizing to parotid, which when diagnosed and treated promptly has overall better prognosis in the cases reported so far.

Recurrent Brainstem Lesions as a Manifestation of MOGAD

Objective NA. Background MOG antibody-associated disease (MOGAD) is an inflammatory and demyelinating disease of the central nervous system. Brainstem and cerebellar involvement in MOGAD is relatively common and typically occurs in conjunction with lesions in other parts of the central nervous system such as the optic nerve, supratentorial white matter, or spine. Isolated attacks of the brainstem and cerebellum are rare–especially in a relapsing form. Design/Methods A case report. Results We present the case of a 60 year-old female who developed new onset weakness and incoordination of her left arm and leg. Her brain MRI revealed restricted diffusion and edema involving the left cerebellar peduncle. She was diagnosed with a stroke and discharged home on anti-platelet therapy. One month later, she developed a left facial droop and was diagnosed with a Bell's palsy. She received oral steroids and had improvement of her facial droop, though it returned a few weeks after completion of her oral steroids. A repeat brain MRI at that time revealed enlargement of the left cerebellar lesion with extension into the left pons. She was again treated with oral steroids and her symptoms improved. She presented again 3 months later with dysarthria and pseudobulbar affect with her brain MRI revealing diffuse T2/FLAIR hyperintensities and edema throughout the bilateral pons and cerebellar peduncles. She received 3 days of high-dose IV methylprednisolone with marked improvement. She ultimately had serum MOG antibody testing performed which returned positive (titer of 1:1000). She was initiated on rituximab, and was clinically stable for over 24 months. Conclusions Although brainstem involvement in MOGAD usually occurs in conjunction with other lesions, our case demonstrates that relapsing attacks isolated to the brainstem and cerebellum are within the clinical spectrum of MOGAD.

The Eye as a Window to the Brain: Prominent Retinal Vasculopathy Points to Neuro-Behcet Diagnosis for an Undifferentiated Solitary Brain Lesion

Objective To report a perplexing case of Behcet disease (BD) presenting as a focal parenchymal lesion that reached a diagnosis after noting a prominent retinal vasculopathy, highlighting the importance of ophthalmologic evaluation in undifferentiated CNS disease. Background BD can have variable systemic manifestations driven by a vasculitis, including oral or genital ulcers, pulmonary aneurysms, and uveitis. Neurologic involvement is present in less than 10% of patients, most commonly as a meningoencephalitis. Design/Methods We present the case of a woman who developed asymmetric sub-acute sensorineural hearing loss at age 31 followed by transient right facial weakness at age 40, and most recently presented with right facial numbness and arm weakness at age 47. Brain MRI revealed a left frontal enhancing lesion with associated T2/FLAIR hyperintensity extending from the periventricular to the juxtacortical area with a thin rim of a reduced diffusion. CSF and serum studies were negative for inflammation, infection and malignancy except for elevated ESR and CRP. Brain biopsy revealed non-specific gliosis. Persistent enhancement on MRI was noted over 3 months, with spontaneous clinical improvement. Patient endorsed insidious vision changes over recent years, and visual testing was performed. Results Dilated ophthalmic examination demonstrated striking peripheral attenuation and sclerosis of retinal vasculature, with evidence of nonperfusion and skip lesions on retinal fluorescein angiography (FA). Findings of occlusive retinal peripheral vasculopathy suggested an underlying vasculitis as the etiology of the brain lesion and prior deficits, raising the likelihood of BD. Patient was homozygous for HLA-B*51, further supporting this diagnosis even with lack of mucosal ulcers and negative history of pathergy. Conclusions Neurologic manifestations of BD can be diverse including retinal occlusive vasculopathy; ulcers are not universally present. Ophthalmologic examination, even when minimally symptomatic, can inform the diagnosis of CNS lesions. Patient was started on Prednisone, Infliximab and Methotrexate, achieving disease remission.

MPC-20 CLINICAL AND MOLECULAR FEATURES OF GLIOBLASTOMAS ARISING IN THE SETTING OF LI FRAUMENI SYNDROME

Abstract Introduction Li Fraumeni Syndrome (LFS) is a rare autosomal dominant tumor predisposition syndrome caused by germline alterations of the TP53. Brain tumors are included in LFS core cancers. Choroid plexus carcinomas and medulloblastomas tend to present in pediatric LFS patients, while gliomas tend to present in adult LFS patients. There are few previous reports about gliomas arising in the setting of LFS. Here, we report clinical and molecular features of three cases of glioblastoma arising in the setting of LFS. Results Mean age at diagnosis was 40 years (range: 32-45). Two patients were male. Two patients had medical history of multiple tumors and family history of brain tumors. Magnetic resonance imaging of each patients revealed a left cerebellar peduncle lesion, a right parietal lobe lesion and two lesions in the pons and right parietal lobe, respectively. Pathological diagnoses of all cases were glioblastomas, IDH-wildtype. All patients underwent chemoradiotherapy after surgery. Mean progression free survival was 10.4 months (range: 5.1-17.0). All patients were admitted to palliative care mean 17.3 months (range: 14.4-21.4) after diagnosis. All patients had heterozygous germline mutations of TP53. Genetic analysis of tumor tissues revealed TP53 mutation and PDGFRA amplification in all patients. In all cases, mutation spots of TP53 in tumor-derived DNA were consistent with those of germ line mutations. In all cases, copy number alteration of TP53 gene in tumor-derived DNA were not detected. EGFR amplification, the combination of gain of chromosome 7 and loss of chromosome 10 (7+/10-) and mutations of IDH, H3F3A, BRAF genes and TERT gene promoter were not detected in all tumor-derived DNA. Conclusion We experienced three cases of glioblastoma, IDH-wildtype arising in the setting of LFS. Clinical courses of all cases were poor. Notably, all tumor tissues harbored TP53 mutation and PDGFRA amplification, while no tumor tissues harbored TERTp mutation, EGFR amplification and 7+/10-.

Response to correspondence regarding 5-year follow up of the Amaze Randomized Controlled trial of ablation treatment for arrhythmia as an adjunct to cardiac surgery.

We thank Dr Garcia-Villarreal [1] for his comment on our article in which he questions the rate of return to stable sinus rhythm in the Amaze trial [2]. We should like to point out that the Amaze trial [3] was not an evaluation of the complete Cox-maze procedure, but an evaluation of concomitant atrial fibrillation surgery as it is done in the real world. Amongst our findings, we discovered that a large variety of lesion sets were being used and that many surgeons did not complete the full biatrial lesion set in their practice. We also found that there appears to be an ablation ‘dose–response’ in achieving return to sinus rhythm, with the greatest step-up achieved when the left atrial lesion set includes the mitral isthmus lesion. We therefore agree with Dr Garcia-Villarreal that concomitant atrial fibrillation surgery should ideally be as complete as possible, and there may be an argument for concentrating such surgery in the hands of interested and experienced surgeons. Despite this, over 5 years later, we have anecdotal evidence that less complete choices of lesion sets continue to be made. We agree that the experience of surgeons devoted to atrial fibrillation should help in maximizing return to sinus rhythm and may also aid in achieving better atrial function after surgery, which is at least as important for clinical patient outcomes as electrical sinus rhythm restoration (study registration ISRCTN82731440) [4].

Characteristics of language and communication after subcortical lesions in the left and right hemisphere

<h3>Research Objectives</h3> The present case comparison investigates to our knowledge for the first time, whether the dichotomy of specific linguistic and communicative characteristics following left and right hemisphere cortical lesions can also be demonstrated after subcortical damage. Classically, left hemisphere cortical lesions of language-relevant areas cause aphasia (Raymer & Rothi, 2018), with cardinal symptoms on a microlinguistic level (e.g. phonologic and semantic errors). Damage to right-hemisphere cortical areas typically results in Cognitive Communication Disorders (CCDs) (Blake, 2018). Symptoms of CCDs are mainly evident on a macrolinguistic level (e.g. tangentiality, verbosity). <h3>Design</h3> Systematic single-case comparison. <h3>Setting</h3> Neurological rehabilitation clinic. <h3>Participants</h3> Two persons with comparable subcortical lesions after postacute left (Patient A) and right (Patient B) basal ganglia hemorrhage stroke. Patient A: Anomic aphasia (Huber et al., 1983), fluent spontaneous speech, short, syntactically simple sentences, word-retrieval problems. Patient B: CCD (Quinting et al., 2020), fluent spontaneous speech, complex, partially paragrammatic syntax. <h3>Interventions</h3> Participants were assessed for communicative-pragmatic ability using the ANELT (Blomert et al., 1994; Rubi-Fessen et al., 2021). Responses were analyzed with regard to linguistic and communicative-pragmatic aspects. A linguistic evaluation was performed by computer-assisted software 'Aachen spontaneous speech analysis' (ASPA, Huber et al., 2005). <h3>Main Outcome Measures</h3> A-scale of the ANELT and linguistic parameters of ASPA. <h3>Results</h3> The results of the ANELT-A-scale were comparable for both patients (Patient A: 84/100, Patient B: 81/100 points). On a microlinguistic level, Patient A showed phonematic paraphasias; ASPA analyses revealed deviations from normative data from non-brain-damaged speakers (Hussmann et al., 2012) concerning all parameters except for type-token-ratio. Patient B´s reactions were syntactically complex and less coherent, indicating problems on a macrolinguistic level. However, ASPA analyses showed results slightly below average concerning percentage words and type-token-ratio. <h3>Conclusions</h3> Typical linguistic-communicative characteristics of cortical left- and right-hemispheric lesions may appear equivalently in left- and right hemispheric subcortical damage. With comparably good communicative effectiveness in the ANELT, these characteristics can also be shown quantitatively by specific patterns in spontaneous speech parameters in ASPA analyses. <h3>Author(s) Disclosures</h3> All authors report no conflicts of interest.