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Background: Re-irradiation (ReRT) is an effective treatment modality in appropriately selected patients with recurrent/progressive high-grade glioma (HGG). The literature is limited regarding recurrence patterns following ReRT, which was investigated in the current study. Methods: Patients with available radiation (RT) contours, dosimetry, and imaging-based evidence of recurrence were included in the retrospective study. All patients were treated with fractionated focal conformal RT. Recurrence was detected on imaging with magnetic resonance imaging (MRI) and/ or amino-acid positron emission tomography (PET), which was co-registered with the RT planning dataset. Failure patterns were classified as central, marginal, and distant if >80%, 20–80%, or

Grading of gliomas is a piece of critical information related to prognosis and survival. Classifying glioma grade by semantic radiological features is subjective, requires multiple MRI sequences, is quite complex and clinically demanding, and can very often result in erroneous radiological diagnosis. We used a radiomics approach with machine learning classifiers to determine the grade of gliomas. Eighty-three patients with histopathologically proven gliomas underwent MRI of the brain. Whenever available, immunohistochemistry was additionally used to augment the histopathological diagnosis. Segmentation was performed manually on the T2W MR sequence using the TexRad texture analysis softwareTM, Version 3.10. Forty-two radiomics features, which included first-order features and shape features, were derived and compared between high-grade and low-grade gliomas. Features were selected by recursive feature elimination using a random forest algorithm method. The classification performance of the models was measured using accuracy, precision, recall, f1 score, and area under the curve (AUC) of the receiver operating characteristic curve. A 10-fold cross-validation was adopted to separate the training and the test data. The selected features were used to build five classifier models: support vector machine, random forest, gradient boost, naive Bayes, and AdaBoost classifiers. The random forest model performed the best, achieving an AUC of 0.81, an accuracy of 0.83, f1 score of 0.88, a recall of 0.93, and a precision of 0.85 for the test cohort. The results suggest that machine-learning-based radiomics features extracted from multiparametric MRI images can provide a non-invasive method for predicting glioma grades preoperatively. In the present study, we extracted the radiomics features from a single cross-sectional image of the T2W MRI sequence and utilized these features to build a fairly robust model to classify low-grade gliomas from high-grade gliomas (grade 4 gliomas).

Rationale: Prolonged undernutrition may arise out of depression and lead to Wernicke's encephalopathy if timely diagnosis and intervention are missed. Wernicke's encephalopathy is potentially treatable, and appropriate treatment may revert clinical depression and cognitive dysfunction to some extent. Patient's concern: A 69-year-old female who had been taking escitalopram for one year developed tremor, ophthalmoplegia, ataxia, progressive cognitive decline, and convulsions. Diagnosis: Non-alcoholic Wernicke's encephalopathy and hypomagnesemia due to psychogenic anorexia. Interventions: High dose intravenous thiamine and magnesium were supplemented. Outcomes: The patient showed remarkable improvement in neurological complications and even in depressive features. Lessons: Wernicke's encephalopathy should not be ignored in the treatment of depression.

The persistent primitive olfactory artery is a very rare arterial variant with a little over a score of cases reported in literature so far. We present 3 cases illustrating this entity, which were incidentally diagnosed while imaging for other indications.

BACKGROUND Surgery is indicated for large or symptomatic lesions in patients with limited brain metastasis (BM), followed by adjuvant stereotactic radiosurgery (A-SRS) to the cavity. Emerging evidence suggests promising role of neoadjuvant SRS (NA-SRS) before surgery with potentially lesser risk of leptomeningeal disease (LMD) and radionecrosis (RN). Hippocampal avoidance whole brain radiotherapy (HA-WBRT) results in better neurocognitive outcomes than standard WBRT, and use of simultaneous integrated boost (SIB) to surgical cavity can improve the local control. Absence of high-quality evidence forms the basis of current study comparing these three treatment strategies. OBJECTIVES Primary endpoint is 1-year event-free survival (EFS) a composite endpoint comprising any local failure, LMD, DBF, symptomatic RN, or death as events. Other endpoints include individual endpoints and longitudinal neuro-cognitive function and quality-of-life assessment. METHODS Target population includes adults with newly diagnosed BM (≤3 lesions) with life expectancy >1 year and one target lesion needing surgery. Patients will be randomized (1:1:1) to A-SRS (control arm) or one of two test arms (NA-SRS or HA-WBRT-SIB). In A-SRS arm, patients will receive single fraction (16-20Gy) or hypofractionated-SRS (24-27Gy/3 fractions or 30-32.5Gy/5 fractions) based on volume and location of cavity and other intact BM. In the test arms, patients will be allocated to either NA-SRS group (single/multi-fraction) followed by surgery within 2 weeks or HA-WBRT (30Gy/10 fractions) with SIB to cavity plus other intact BM (40-50Gy/10 fractions) combined with memantine within 6 weeks of surgery. A sample size of 168 patients is required to prove the superiority of test arms individually compared to the control arm with assumption of 1-year EFS of 43% versus 25% with a hazard ratio of 0.6 (two-sided alpha=0.05, power=80%, and 15% attrition rate). DISCUSSION The study will generate level 1 evidence investigating the role of NA-SRS or HA-WBRT-SIB compared to A-SRS in limited brain metastases.

We have created the immunodeficient SRG rat, a Sprague-Dawley Rag2/Il2rg double knockout that lacks mature B cells, T cells, and circulating NK cells. This model has been tested and validated for use in oncology (SRG OncoRat®). The SRG rat demonstrates efficient tumor take rates and growth kinetics with different human cancer cell lines and PDXs. Although multiple immunodeficient rodent strains are available, some important human cancer cell lines exhibit poor tumor growth and high variability in those models. The VCaP prostate cancer model is one such cell line that engrafts unreliably and grows irregularly in existing models but displays over 90% engraftment rate in the SRG rat with uniform growth kinetics. Since rats can support much larger tumors than mice, the SRG rat is an attractive host for PDX establishment. Surgically resected NSCLC tissue from nine patients were implanted in SRG rats, seven of which engrafted and grew for an overall success rate of 78%. These developed into a large tumor volume, over 20,000 mm3 in the first passage, which would provide an ample source of tissue for characterization and/or subsequent passage into NSG mice for drug efficacy studies. Molecular characterization and histological analyses were performed for three PDX lines and showed high concordance between passages 1, 2 and 3 (P1, P2, P3), and the original patient sample. Our data suggest the SRG OncoRat is a valuable tool for establishing PDX banks and thus serves as an alternative to current PDX mouse models hindered by low engraftment rates, slow tumor growth kinetics, and multiple passages to develop adequate tissue banks.

Cysticercosis, an infection caused by the larval stage of tapeworm Taenia solium, is the most common parasitic disease of the human nervous system and the single most common cause of acquired epileptic seizures in the developing world. Here, we describe the stormy course of a 67-year-old female with neurocysticercosis (NCC) having a recurrent encephalitic presentation. She went through the most severe spectrum of this disease, namely NCC encephalitis and disseminated cysticercosis and had a classical starry sky brain in neuroimaging. In contrary to the popular practice of avoiding antihelminthic drugs in such extreme presentation, as a desperate measure, we had to use albendazole in this case, which showed clinical and radiological improvement.

Acute neurovascular events, though rare, can complicate pregnancy and postpartum period. It is important to be aware of these clinical conditions for reducing maternal and fetal morbidity and mortality. In this review, we present a few important neurovascular emergencies and their imaging manifestations by various imaging modalities-computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA)-which presented at our institution in the peripartum period.

Diffuse spinal arachnoiditis in neurobrucellosis is a rare manifestation. We report a boy aged 17, presenting with hearing impairment and recurrent vomiting for 18 months, weight loss for 12 months, dysphagia, dysarthria, hypophonia for 6 months, and gait unsteadiness for 5 months. He had bilateral 5th (motor) to 12th cranial nerve palsy, wasting and weakness of limbs, fasciculations, absent tendon reflexes, and positive Babinski’s sign. Cerebrospinal fluid (CSF) showed raised protein and pleocytosis. Magnetic resonance imaging (MRI) showed extensive enhancing exudates in cisterns and post-contrast enhancement of bilateral 5th, 6th, 7th, and 8th nerves. Spine showed clumping with contrast enhancement of the cauda equina roots and encasement of the cord with exudates. Serum and CSF were positive for anti-Brucella antibodies. He showed significant improvement with antibiotics. At 4 months follow-up, MRI demonstrated near complete resolution of cranial and spinal arachnoiditis. It is important to recognize such rare atypical presentations of neurobrucellosis.

Neurotropism and infiltration by Mycobacterium leprae of peripheral nerves causing neuropathy are well established, but reports of central nervous system (CNS) damage are exceptional. We report CNS magnetic resonance imaging (MRI) abnormalities of the brain and spinal cord as well as lesions in nerve roots and plexus in leprosy patients. Eight patients aged between 17 and 41 years underwent detailed clinical, histopathological, and MRI evaluation. All had prominent sensory-motor deficits with hypopigmented and hypo/anesthetic skin patches and thickened peripheral nerves. All demonstrated M. Leprae DNA in affected peripheral nerve tissue. All received multidrug therapy (MDT). Two patients had brainstem lesions with enhancing facial nuclei and nerves, and one patient had a lesion in the nucleus ambiguus. Two patients had enhancing spinal cord lesions. Follow-up MRI performed in four cases showed resolution of brainstem and cord lesions after starting on MDT. Thickened brachial and lumbosacral plexus nerves were observed in six and two patients, respectively, which partially resolved on follow-up MRI in the two cases who had reimaging. The site and side of the MRI lesions corresponded with the location and side of neurological deficits. This precise clinico-radiological correlation of proximal lesions could be explained by an immune reaction in the gray matter corresponding to the involved peripheral nerves, retrograde axonal and gray matter changes, or infection of the CNS and plexus by lepra bacilli. Further study of the CNS in patients with leprous neuropathy is needed to establish the exact nature of these CNS MRI findings.

Hirayama disease is generally considered to be a sporadic disorder, except for a few reports of familial occurrence. In this study, we describe eight patients from four families with cervical flexion induced myelopathy (CFIM)/Hirayama disease (HD) and intra-familial phenotypic variations. All underwent clinical and electrophysiological evaluation, while seven of them had contrast MR imaging of cervical spine in flexion. There was significant intra-familial variability: distal bimelic form in four patients, classical monomelic form in three and proximo-distal form in one. Irrespective of the clinical phenotype, MRI showed characteristic dynamic changes of posterior dural detachment with prominent epidural enhancement extending variably from C3 vertebral level to dorsal spine in six patients. One patient with 28 years of illness, had only lower cervical cord atrophy without dynamic changes while another patient demonstrated forward dural displacement with epidural enhancement even after 38 years of disease duration.

Bruns syndrome is characterized by attacks of sudden severe headache, vomiting, and vertigo precipitated due to abrupt movements of the head due to presence of mobile deformable intraventricular lesion causing episodic obstructive hydrocephalus. Proposed underlying mechanism is intermittent or positional CSF obstruction resulting from ball-valve mechanism. Most common etiologies are NCC and intraventricular tumors. Here we present an unusual case of Bruns syndrome that was initially MRI negative.

Hirayama disease (HD)/cervical flexion-induced myelopathy (CFIM) is a lower motor neuron disease conventionally affecting a single upper extremity. We describe three men progressing after a long stable period to develop severe spastic paraparesis and bladder disturbances as a protracted implication of HD. The age at onset was 20, 24, and 15 years, while the age at presentation was 27, 41, and 57 years, respectively. The second phase of disease progression occurred after 4, 13, and 28 years of stationary period. All had CFIM with characteristic magnetic resonance imaging features as observed during progressive stages. The anterior dural shift extended variably from C4 to D4 levels with a median value of 5 mm and was maximum at C6 to C7 levels, pushing the cord anteriorly causing compression. This study emphasizes the need to recognize this unusual subgroup of HD and mandates long-term follow-up with timely intervention in arresting the progression/improving the deficits.

Myxoid adrenal cortical neoplasms are rare. To the best of our knowledge, no such case has been reported in pediatric or infantile age group till now. Here we report a case of non-functional myxoid adrenocortical adenoma (ACA) in a 7-month-old girl, who presented with a large mass in the abdomen. Microscopically, the tumor was composed of alveolar clusters of cells with focal pseudoglandular architecture in a background of abundant alcian blue positive myxoid matrix. Compressed rim of adrenal tissue was identified at periphery. The patient was put on a close follow-up in view of scarce literature on the subject. She has been doing fine without any recurrences. Myxoid adrenal cortical tumors expand the differential diagnoses of a myxoid neoplasm in retroperitoneum.

Sacrococcygeal teratoma is the most common congenital neoplasm in neonates. We came across a post term (42 weeks) newborn baby girl delivered by normal vaginal route. The baby presented with a large soft, cystic mass over the sacrococcygeal region. Radiological examination showed a soft tissue mass with variegated appearance. Complete excision of the mass was done. Histopathological examination revealed the mass to be a mature sacrococcygeal teratoma with the extremely uncommon finding of a complete adrenal gland within the teratoma sac. Sacrococcygeal teratoma is a component of a continuum with other tumors including fetiform teratoma, fetus in fetu, parasitic and conjoint twins.

Abstract 4076 Poster Board III-1011 The reactivation of fetal hemoglobin has been shown to ameliorate symptoms of sickle cell disease. Previous work from our laboratory demonstrated that histone deacetylase inhibitors such as Trichostatin A and sodium butyrate activate γ-globin gene expression via p38 MAPK signaling. Using a transient luciferase genetic reporter system, the trans-factors cAMP response element binding protein 1 (CREB1) and activating transcription factor-2 (ATF-2) were shown to trans-activate the Gγ-globin CRE, located upstream at nucleotide -1222. To study this regulatory element in a native chromatin conformation, we cloned the Gγ-globin promoter (-1500 to +36) into pGL4.17 Luc2/neo to produce the pGγLuc2 reporter construct. In addition, three mutant plasmids were created by site directed mutagenesis including the -1225 G/A mutation (pGγLuc2m1), -1227 AC/TG mutations (pGγLuc2m2) and a scrambled G-CRE, 5'-TCTATGTA-3' (pGγLuc2m3S). Each reporter along with the empty vector control (pGL4.17 Luc2/neo) was transfected into K562 cells and five stable lines including KLuc2, KGγLuc2, KGγLuc2(m1), KGγLuc2(m2) and KGγLuc2(m3s) were established using the G418 selectable marker. Luciferase activity in KGγLuc2 was 700-fold higher than the promoterless Kluc2 stable line. When the G-CRE element was mutated, we observed a 70 to 80 % decrease (p Disclosures: No relevant conflicts of interest to declare.