COT-11 Administration of Bevacizumab for patients who failed to complete Stupp regimen after glioblastoma surgery

Stupp regimen is widely used as the standard treatment after glioblastoma surgery, but in some cases treatment must be discontinued for various reasons. We experienced Bevacizumab in two patients who were unable to continue treatment in the Stupp regimen, and report our experience with literature review. First patient is a man in his 60s. Resection of glioblastoma of the left cerebral hemisphere was performed, and postoperatively right hemiparesis and aphasia remained. Irradiation and administration of Temozolomide were performed, but Temozolomide was unable to continue because of side effects. After systemic management, Bevacizumab was administered, and reduction of residual tumor and peripheral edema were observed, and the patient began to speak. After 12 cycles of administration, the tumor regrew, and he died. Second patient is a woman in her 80s. Craniotomy was performed for hemorrhagic infarction of the left cerebral hemisphere, postoperatively, aphasia, right hemiparesis remained, bedridden, and was unable to eat. Four months after initial surgery, a tumor was found in left parietal lobe and was resected. The pathological diagnosis was glioblastoma. For the treatment of recurrence, the patient was unable to be transferred for radiochemotherapy, so the patient was treated with Temozolomide and Bevacizumab. The patient’s condition became better, eat by herself, and could play in rehabilitation facility on the wheelchair. After 12 cycles of bevacizumab, the tumor subsequently enlarged, and died. Although the effect is limited, there are some cases in which Bevacizumab administration could maintain patient’s condition by controlling tumor growth for a certain period of time. From the experience of these patients, it seems that even in patients with postoperative poor Karnofsky Performance Status (KPS)and elderly people, Bevacizumab administration would be an option before transitioning to end-of-life care.

BT-05 A CASE OF GLIOMATOSIS CEREBRI WITH TOTAL SPINAL CORD EXTENSION

INTRODUCTION Although gliomatosis cerebri is no longer a pathological diagnostic name, it is still an important disease state as a gliomatosis cerebri growth pattern. Here we report a case of gliomatosis cerebri originating from the left cerebral hemisphere that developed whole spinal cord. CASE A 10-year-old boy. He has a history of 13q-syndrome and left retinoblastoma. Left eye enucleation and chemotherapy (modified 98A1) have been performed. MRI in July 2017 showed no abnormalities, but in September 2018, he developed epilepsy. MRI revealed a gliomatosis cerebri that spreads extensively in the left cerebral hemisphere. Biopsy revealed anaplastic astrocytoma (MIB-1 LI; 22%, IDH1 / 2; WT, TERT C228T mutation positive) and IMRT (59.4Gy) and temozolomide (Stupp regimen) were performed in December 2018. In June 2019, neck pain developed. Head and neck MRI revealed that the tumor in the head increased lightly, and there was no suspicion of tumor growth in the brainstem, but the tumor progressed to the entire spinal cord. Therefore, radiation therapy was started from the lower brainstem that had not been irradiated last time to the entire spinal cord, and administration of bevacizumab was started. DISCUSSION Based on the single cell origin theory, the left hemisphere tumor and spinal cord tumor should be continuous. Since gliomatosis cerebri is visualized on MRI only after the tumor volume has increased and edema has occurred, it may appear as if there is no tumor in between. The spinal cord MRI was not taken, so it is only speculation, but it seems that tumor cells had probably infiltrated the spinal cord from the beginning, and it seems that it gradually increased because it was not irradiated. Considering the possibility of remote invasion as in this case, it is necessary to consider taking MRI of spinal cord at the first occurrence.

Non epileptogenic Rasmussen`s encephalitis

Introduction: Rasmussen's encephalitis (RE) is a very rare chronic inflammatory unilateral encephalopathy with still unknown etiology. It predominantly affects children under the age of 10, but can also affect adolescents and adults. Current evidence on possible etiologies are divided in two main theories. The first one is based on brain inflammation caused by a reaction to a foreign antigen. The second one is based on an autoimmune disease that is limited to a single hemisphere of the brain. The common presentation is intractable and frequent focal motor seizures, often associated with progressive neurological decline, and progressive unilateral focal cortical atrophy. Progression of the inflammatory process in MRI is considered a good biomarker in RE. Objectives: Report a rare case of a female infant presenting atypical manifestations of uncommon symptoms without seizures caused by Rasmussen's encephalitis. Case Report: A ten-year-old right-handed female presented to the emergency department with sudden onset of abnormal involuntary movements of the right upper and lower limbs, face paresia of the same side and loss of attention without loss of consciousness. The involuntary contractions were brief, random, irregular, and worsened with activity, along with a positive milkmaid grip sign. She also displayed reduced movement speed and hyperactive biceps, triceps, patellar and ankle jerk reflexes. Associated with the motor abnormalities, the girl presented with a recent but progressive decrease in cognitive functions, which caused a decline in school performance and impaired reasoning, and affective blunting. She had no sign of previous neuropsychomotor development abnormality. She was taken to a quaternary hospital where a MRI and a CT of the brain were performed. MRI showed atrophy of the left cerebral hemisphere, predominantly in the operculum and head of the caudate nucleus, hypersignal in the insular cortex and adjacent white matter. CT presented with hypoattenuation of the left frontal lobe and parieto-occipital white matter associated with atrophy of the head of the caudate nucleus of the same side and enlargement of the adjacent fissures and sulcus. A subsequent PET-CT showed a left cerebral hemisphere hypometabolism, predominantly in the basal ganglia, temporal, frontal and insular lobes. Functional evaluation detected diffuse unilateral disorganization of brain electric activity, but no epileptic paroxysmal discharges were found on the electroencephalogram. After hospitalization, human immunoglobulin at the dose 2g/kg/day was used for five days. The girl remained stable, with no complications. After sixteen days, she was discharged with a rehabilitation program and follow-up care.

Occlusion of Extracranial-Intracranial Bypass Anastomosis-Associated Aneurysms Following Contralateral High-Flow Extracranial-Intracranial Bypass in a Patient with Impaired Cerebrovascular Reserve

BACKGROUND AND IMPORTANCE Extracranial-intracranial (EC-IC) bypass anastomosis-associated aneurysms are rare sequelae of cerebral revascularization surgery. Although treatment paradigms are not well defined, clipping, trapping with revision bypass, and donor vessel ligation represent the most common microsurgical approaches. CLINICAL PRESENTATION A 53-yr-old male presented with cognitive decline, left extremity weakness, and left visual field blurriness. Computed tomographic angiography of head/neck demonstrated bilateral cervical internal carotid artery occlusion and magnetic resonance imaging of brain showed a small right parieto-occipital lobe infarct. The patient's symptoms worsened despite aggressive medical management. Therefore, a right superficial temporal artery to middle cerebral artery (STA-MCA) bypass was performed for flow augmentation. Follow-up digital subtraction angiography (DSA) approximately 1 yr after surgery noted 2 new aneurysms adjacent to the patent STA-MCA anastomosis. Perfusion imaging at that time showed persistently reduced blood flow in the left cerebral hemisphere. A left STA-MCA bypass was performed, and intraoperative blood flow measurements showed this to be a high-flow bypass. Follow-up DSA 4 mo later demonstrated involution of the right STA-MCA bypass and occlusion of the anastomosis-associated aneurysms with increased perfusion of the right cerebral hemisphere via collateral blood flow from the patent high-flow left STA-MCA bypass. At 7 mo following left STA-MCA bypass, the patient's neurological examination remained stable and perfusion imaging showed improved blood flow in the left cerebral hemisphere. CONCLUSION We present a unique case in which a high-flow left EC-IC bypass with robust contralateral collateral blood flow was associated with subsequent occlusion of a right EC-IC bypass and 2 anastomosis-associated aneurysms in a patient with bilateral impaired cerebrovascular reserve.

Limb apraxia in aphasic patients

ABSTRACT Limb apraxia is usually associated with left cerebral hemisphere damage, with numerous case studies involving aphasic patients. The aim of this study was to verify the occurrence of limb apraxia in aphasic patients and analyze its nature. This study involved 44 healthy volunteers and 28 aphasic patients matched for age and education. AH participants were assessed using a limb apraxia battery comprising subtests evaluating lexical-semantic aspects related to the comprehension/production of gestures as well as motor movements. Aphasics had worse performances on many tasks related to conceptual components of gestures. The difficulty found on the imitation of dynamic gesture tasks also indicated that there were specific motor difficulties in gesture planning. These results reinforce the importance of conducting limb apraxia assessment in aphasic patients and also highlight pantomime difficulties as a good predictor for semantic disturbances.