INNV-25. RITUXIMAB MONOTHERAPY FOR TREATMENT OF RARE LOW GRADE PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA; A CASE REPORT AND LITERATURE REVIEW

Primary central nervous system lymphoma (PCNSL) is a specific variant of non-Hodgkin's lymphoma confined to the brain, leptomeninges, spinal cord, and/or eyes. The majority of PCNSL is diffuse large B-cell lymphoma (DLBCL), however, a small percentage are categorized as low-grade lymphomas (LGL). Compared to high-grade and aggressive DLBCL, LGL are indolent; allowing for targeted and less neurotoxic first-line treatments. There is currently no consensus for LGL treatment. A patient at our institution was diagnosed with LGL without extra-CNS involvement 11/2018 and then, successfully treated with rituximab monotherapy. This 65 year-old, immunocompetent woman presented with one month of right leg weakness and numbness. Brain MRI demonstrated a subtly enhancing infiltrative left thalamic lesion extending into the left frontal lobe with surrounding edema. Flow cytometry on spinal fluid showed rare monotypic B-cells. Brain biopsy results showed an atypical predominantly perivascular lymphoid infiltrate. These atypical small lymphoid cells had mature-appearing nuclear chromatin, absent nucleoli, and uniformly expressed CD79a and CD20 with variable PAX-5 expression, lacked CD56 and CD117 expression, and had plasmacytic differentiation; thus consistent with marginal zone lymphoma, a type of indolent B-cell lymphoma. There was no evidence of extra-CNS involvement on PET scan, bone marrow biopsy, or ocular exam. She had normal cognitive functioning on neuropsychological testing. Interestingly, SPEP showed a monoclonal IgM immunoglobulin of kappa-light chain-type that became a monoclonal protein in the gamma fraction. Additionally, CT abdomen initially showed splenomegaly that resolved on repeat imaging a year later. Radiation therapy was deferred due to high risk. Weekly rituximab 500mg/m2 was initiated for 4 doses, then monthly for 4 doses. Therapy was well tolerated and she noted clinical improvement plus there was positive response on brain imaging. Repeat ophthalmology exam and CT body without any evidence of cancer. She is now 29 months progression-free since completing rituximab.

Pediatric thalamic incidentalomas: an international retrospective multicenter study

OBJECTIVE Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain incidentalomas. Thalamic lesions have unique characteristics such as deep location, surgical complexity, and proximity to eloquent neuronal structures. Currently, the natural course of incidental thalamic lesions is unknown. Therefore, the authors present their experience in treating such lesions. METHODS A retrospective, international multicenter study was carried out in 8 tertiary pediatric centers from 5 countries. Patients were included if they had an incidental thalamic lesion suspected of being a tumor and were diagnosed before the age of 20 years. Treatment strategy, imaging characteristics, pathology, and the outcome of operated and unoperated cases were analyzed. RESULTS Overall, 58 children (23 females and 35 males) with a mean age of 10.8 ± 4.0 years were included. The two most common indications for imaging were nonspecific reasons (n = 19; e.g., research and developmental delay) and headache unrelated to small thalamic lesions (n = 14). Eleven patients (19%) underwent early surgery and 47 were followed, of whom 10 underwent surgery due to radiological changes at a mean of 11.4 ± 9.5 months after diagnosis. Of the 21 patients who underwent surgery, 9 patients underwent resection and 12 underwent biopsy. The two most frequent pathologies were pilocytic astrocytoma and WHO grade II astrocytoma (n = 6 and n = 5, respectively). Three lesions were high-grade gliomas. CONCLUSIONS The results of this study indicate that pediatric incidental thalamic lesions include both low- and high-grade tumors. Close and long-term radiological follow-up is warranted in patients who do not undergo immediate surgery, as tumor progression may occur.

Frequency and phenotype of thalamic aphasia

Background Aphasia is a recognized presenting symptom of thalamic lesions. Little is known regarding its frequency and phenotype. We examined the frequency of thalamic aphasia following Isolated Acute unilateral ischemic Lesions in the Thalamus (IALT) with respect to lesion location. Furthermore, we characterized thalamic aphasia according to affected language domains and severity. Methods Fifty-two patients with IALT were analyzed [44% female, median age: 73 years (IQR: 60–79)]. Lesion location was determined using 3-Tesla magnetic resonance imaging and categorized as anterior, posterior, paramedian or inferolateral. Standardized language assessment was performed using the validated Aphasia checklist (ACL) directly after symptom onset. Aphasia was defined as an ACL sum score of < 135 (range: 0–148). Results Of 52 patients, 23 (44%) fulfilled the ACL diagnostic criteria for aphasia, including nearly all lesion locations and both sides. The average ACL sum score was 132 ± 11 (range: 98–147). Aphasia was characterized by deficits within domains of complex understanding of speech and verbal fluency. Patients with left anterior IALT were most severely affected, having significantly lower ACL scores than all other patients (117 ± 13 vs. 135 ± 8; p < 0.001). In particular, aphasia in patients with left anterior IALT was characterized by significantly worse performance in the rating of verbal communication, verbal fluency, and naming (all p ≤ 0.001). Conclusion Aphasia occurs in almost half of patients with focal thalamic lesions. Thalamic aphasia is not confined to one predefined thalamic lesion location, but language deficits are particularly pronounced in patients with left anterior IALT presenting with a distinct pattern.

RARE-05. ANAPLASTIC ASTROCYTOMA AND OLIGODENDROGLIOMA PRESENTING AS SIMULTANEOUS PRIMARY BRAIN TUMORS IN A PEDIATRIC PATIENT: THE FIRST KNOWN REPORT OF A RARE CONDITION

Multiple metastatic brain tumors and multiple histologically identical primary brain tumors have been reported in adults and children. The concurrent presence of multiple histologically distinct primary brain tumors is rare without prior chemotherapy, radiotherapy, or phacomatosis. Exceedingly rare are simultaneous histologically distinct primary brain tumors in children without therapeutic or genetic predisposition, with only five previously reported cases. Gliomas are the most common primary brain tumor in adults and children. Gliomas encompass a heterogenous group characterized by astrocytic, oliogodendroglial, and ependymomal histology, further subdivided into pathologic World Health Organization (WHO) grades I to IV. In children, grade I astrocytomas are the most common, with grade III astrocytomas (anaplastic astrocytomas) and oligodendrogliomas being rare. We report a case of simultaneous anaplastic astrocytoma and grade II oligodendroglioma in a pediatric patient. A previously healthy 6-year-old female presented with persistent headaches. Initial magnetic resonance imaging (MRI) demonstrated non-enhancing right frontal lobe and left peri-thalamic lesions without mass affect. Serial MRIs showed progression of both lesions, prompting frontal lobe biopsy, which revealed a grade II oligodendroglioma. The patient was started on standard low-grade glioma chemotherapy, which was stopped due to an allergic reaction. Following chemotherapy cessation, both lesions increased in size, and the peri-thalamic lesion demonstrated new heterogenous enhancement. The patient underwent gross-total resection of the peri-thalamic lesion and repeat biopsy of the frontal lobe lesion. Pathology confirmed the frontal lobe lesion to be a grade II oligodendroglioma and revealed the peri-thalamic lesion to be an anaplastic astrocytoma. The tumors were additionally proven molecularly distinct. The patient responded well to cranial radiotherapy and standard high-grade glioma chemotherapy. To the best of the authors’ knowledge, this is the first case of simultaneous anaplastic astrocytoma and oligodendroglioma in a child, increasingly unique given the histological and molecular rarity of the tumors in pediatric patients.

Overview of The Head CT-Scan in Stroke Patients who was Treated at Muhammadiyah Hospital Palembang

Background: Stroke is a collection of symptoms of neurological deficits due to sudden acute disturbance of brain function, both focal and global, caused by blockage or rupture of arteries and veins and proven by imaging and/or reflex pathology. Using CT-Scan, the description of acute phase stroke can be easier and can determine the appropriate treatment criteria for stroke. Purposes: To determine characteristic Head CT-Scan image of stroke patients hospitalized in Muhammadiyah Palembang Hospital. Methods: This study was conducted in a retrospective descriptive manner and obtained a sample of 41 stroke patients according to the inclusion and exclusion criteria using total sampling. Result: In this study, there were 28 patients (68.2%) with ischemic stroke and 13 patients (31.7%) with hemorrhagic stroke. Head CT-Scan image of ischemic stroke shows basal ganglia lesion (28.5%) and the right hemisphere lesions (57.1%) as the most predominant area. Head CT-Scan image of hemorrhagic stroke (58.3%) had intracerebral hemorrhage with the dominant thalamic lesion area (66.6%) and the right hemisphere (58.3%) had the most lesions with 8 patients (66, 6%) had a midline shift. Stroke patients were treated as experienced by the elderly (90.2%) and were dominated by the female sex (63.4%) and are dominated by clinical symptoms of hemiparesis (29.2%). Conclusion: Stroke patients at Muhammadiyah Palembang hospital dominated by ischemic stroke and on head CT Scan image predominantly shows lesions on the right cerebral hemisphere.

The Resection of a Thalamic Pilocytic Astrocytoma Through the Transchoroidal Fissure, Transcallosal Approach: 2-Dimensional Operative Video

Surgical resection is the primary treatment of pilocytic astrocytomas and total removal can be curative. However, these lesions occur in critical areas, such as the thalamus, being surrounded by critical life neurovascular structures, which imposes a surgical challenge.1-5 Exhaustive acquisition and meticulous interpretation of preoperative radiological exams; reliable surgical orientation based on profound microneurosurgical anatomic knowledge and judicious discernment of the neuroanatomic distortions on the surface and deep-seated structures inflicted by the neuropathological entity; embracing and comprehensive application of the vast scope of available intraoperative guidance imaging and neurophysiological monitoring; in alliance with the mastered carefully microsurgical technique supported by endoscopic visualization are the keystones to the pursed duet “cure with quality of life” in the treatment of these lesions. We present the case of a 17-yr-old young lady with a progressive motor deficit in her right hemibody for over 2 yr. Her radiological investigation demonstrated a left thalamic lesion displacing the projection fibers (corticospinal tract) within the internal capsule laterally. The patient consented to the surgical procedure. The surgical strategy, intraoperative findings, and microsurgical and endoscopic technique, as well as the postoperative radiological and clinical evaluation are presented. The patient gave her informed consent for the publication of the case.

Case Report: Thalamomesencephalic stroke in a patient with HIV

We present a 41-year-old HIV-positive female patient complaining of complete right palpebral ptosis, diplopia, and inability to balance herself. On examination, the right eye was able to move laterally and downwards. The motor exam showed left hemiparesis (4/5) on upper and lower limbs, bilateral Babinski sign with left hemiataxia without the sensory disorder. CT scan and magnetic resonance imaging angiography demonstrated an ischemic infarct on the right paramedian branch of the posterior cerebral artery territory. This patient did not present clinical manifestations of the thalamic lesion. To our knowledge, this is the first reported case of a young patient presenting a unilateral thalamomesencephalic ischemic stroke secondary to HIV vasculitis with bilateral Babinski signs and without thalamic signs in the medical literature.

Giant central nervous system tuberculoma in pediatric patients: surgical case series

Central nervous system tuberculoma is rare and challenging situation. Clinical records of patients with pathologically proven tuberculoma were retrospectively reviewed. Clinical presentation, lesion location, radiological characteristics, perioperative and surgical management, and outcome is summarized and analyzed. Eight patients were included and there was one girl. Age ranged from 3 to 14 years with mean age 9.8 years. Clinical duration ranged from 20 days to 2 years, and 3 patients had previous lung tuberculosis with anti-TB treatment. The lesion was in cerebellum in 6 cases, including 1 involving basal ganglia and 1 involving thalamus. The lesion was in basal ganglia, thalamus, and third ventricle in 1 case, and in T12-L1 spinal cord in another. Cerebellar lesion was resected via paramedian suboccipital approach in 5 patients, basal ganglia lesion via trans-cortical frontal horn approach in 2 patients, and intra-spinal lesion via trans-laminar approach in 1 patient. Follow-up ranged from 10 to 24 months. Of the 8 patients, 6 returned to normal life. One patient had cerebellar lesion resected and the thalamic lesion reduced in size after anti-TB treatment. One patient died from TB spreading. Our data showed that most patients can be successfully treated by resection of the lesion. Low T2 signal, ring shaped enhancement and peripheral edema strongly suggest tuberculoma. Empirical anti-TB treatment should be initiated perioperatively.

Bilateral thalamic lesion presenting as Broca’s type subcortical aphasia in cerebral venous thrombosis: index case report

A 35-year-old man with a history of multiple substances abuse (alcohol, tobacco and cannabis) presented with acute, severe, holocranial headache associated with nausea and few episodes of vomiting followed by acute onset Broca-type aphasia with intact comprehension from next day, without any other focal neurodeficits, seizure or altered sensorium. Neurological examination was marked by Broca-type aphasia and failure in convergence reaction bilaterally, rest unremarkable. Brain imaging revealed lesions in bilateral thalamus, while magnetic resonance venography showed multiple flow voids in posterior part of superior sagittal sinus and bilateral transverse sinus. A diagnosis of cerebral venous sinus thrombosis was made. Subsequent investigations revealed decreased levels of protein C, protein S and antithrombin III. The patient was started on anticoagulation to which his headache and aphasia recovered completely after 8 and 12 days of therapy, respectively. He is being continued on anticoagulation and is following-up with us for past 4 months uneventfully.