LINC-06. OBSERVATION ONLY IN A PATIENT WITH SUSPECTED LOW GRADE GLIOMA. SHOULD NEUROSURGERY ALWAYS BE THE FIRST STEP IN LOW AND MIDDLE INCOME COUNTRIES?

Abstract BACKGROUND Low grade gliomas (LGGs) are the most frequent pediatric brain tumor and they comprise a variety of histologies. Complete surgery is curative but sometimes its location makes it difficult. Recent publications highlight the excellent long-term outcomes of patients with LGGs with complete and incomplete resected tumors. Current strategies are focused on reducing risks of treatment related sequelae. METHOD We describe a patient with a suspected LGG managed by close observation. We describe the case of a 6 year old female with 5 months history of focal onset seizures. During this time a brain MRI was requested and tumor was evidenced. After “tumor diagnosis” was made family visited a handful of private neurosurgeons with a uniformly dismal prognosis and high risk morbidity from procedures offered. When first seen at our Hospital, the clinical history seemed compatible with a LGG and seizures well controlled with antiepileptic drugs. Neurological examination was completely normal. MRI showed a large tumor (7x5x5 cm) hypointense on T1, hyperintense on T2, without contrast enhancement, involving the right temporal lobe white matter, insula, internal capsule, hipoccampus, thalamus and mesencephalus with middle cerebral artery encasement. Interval imaging was proposed and after 4.5 years since diagnosis the tumor has been stable and patient clinically excellent. CONCLUSION Overall survival in pediatric LGGs is excellent and risk of sequelae should always be part of multidisciplinary team considerations. In centers with significant neurosurgical morbidity, biopsy of large tumors that are compatible with LGG may not be required in selected cases.

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The Insular Lobe: Physiopathological and Surgical Considerations

Neurosurgery ◽

10.1097/00006123-200010000-00001 ◽

2000 ◽

Vol 47 (4) ◽

pp. 801-811 ◽

Cited By ~ 129

Author(s):

Hugues Duffau ◽

Laurent Capelle ◽

Manuel Lopes ◽

Thierry Faillot ◽

Jean-Pierre Sichez ◽

...

Keyword(s):

Internal Capsule ◽

Neurological Status ◽

Low Grade ◽

Language Mapping ◽

Motor Mapping ◽

Computed Tomographic ◽

Lenticulostriate Arteries ◽

The Right ◽

Type 3 ◽

Left Insula

Abstract OBJECTIVE Surgery of the insula represents a technical challenge, because of the proximity of the internal capsule to the lenticulostriate arteries and the lack of certainty concerning its functionality. Using intraoperative direct cerebral stimulation, combined with neuronavigation, the authors operated on 12 insular gliomas. On the basis of this experience, the physiopathological and surgical implications are discussed. METHODS A low-grade insular glioma, revealed by seizures, was diagnosed in 12 right-handed patients with a normal neurological status. Preoperative magnetic resonance imaging showed that, according to Yaşargil's classification system, three patients harbored Type 3 lesions and nine patients had Type 5 lesions (10 tumors on the right side and 2 on the left dominant side). All patients underwent surgery using direct cerebral stimulation, under general anesthesia in nine patients (motor mapping) and under local anesthesia in three patients (sensorimotor and language mapping). Ultrasonography and/or neuronavigation was used in all cases. Preoperative angio-computed tomographic scanning showed the lenticulostriate arteries in two patients. RESULTS The internal capsule was systematically detected, and the language areas were identified within the left insula in the awake patients. The lenticulostriate arteries were seen in two patients. Seven patients presented an immediate postoperative deficit; six of them recovered completely within 3 months. Four resections were total, six were subtotal, and two were partial (left insula). CONCLUSION The use of intraoperative direct cerebral stimulation and neuronavigation allows surgery of the insula with minimization of the risk of sequelae, but its use is still limited with regard to the dominant hemisphere, owing to the essential role of this structure in language.

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P040 An unusual case of giant cell arteritis

Rheumatology ◽

10.1093/rheumatology/keab247.037 ◽

2021 ◽

Vol 60 (Supplement_1) ◽

Author(s):

Jessica Ellis ◽

Keziah Austin ◽

Sarah Emerson

Keyword(s):

Inflammatory Markers ◽

Illicit Drugs ◽

Unusual Case ◽

Temporal Arteritis ◽

White Cell Count ◽

Healthcare Providers ◽

Temporal Artery ◽

Low Grade ◽

Temporal Artery Biopsy ◽

The Right

Abstract Background/Aims A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods The case is discussed below. Results Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure J. Ellis: None. K. Austin: None. S. Emerson: None.

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LGG-18. EVEROLIMUS TREATMENT IN PEDIATRIC PATIENTS AFFECTED BY LOW-GRADE GLIOMAS (pLGG) NON-TSC, BRAF v600-WT

Neuro-Oncology ◽

10.1093/neuonc/noaa222.400 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii369-iii369

Author(s):

Antonella Cacchione ◽

Evelina Miele ◽

Maria Chiara Lodi ◽

Andrea Carai ◽

Giovanna Stefania Colafati ◽

...

Keyword(s):

Adverse Event ◽

Disease Progression ◽

Mapk Pathway ◽

Brain Mri ◽

Mammalian Target Of Rapamycin ◽

Low Grade ◽

Duration Of Treatment ◽

Tumor Biopsy ◽

Low Grade Gliomas ◽

Personalized Approach

Abstract BACKGROUND MAPK pathway is the hallmark of pediatric low grade gliomas (pLGGs); hyperactivation of mTOR (mammalian target of rapamycin) might be a suitable biomarker for therapeutic response. We investigated the feasibility of Everolimus, mTOR inhibitor, in patients affected by pLGGs. METHODS Patients 1 to 18 years old, diagnosed with pLGG, with a positive tumor biopsy for mTOR/phospho-mTOR and radiological and / or clinical disease progression, treated at Bambino Gesù Children’s Hospital in Rome were evaluated. Tumor DNA methylation analysis was performed in 10 cases. Exclusion criteria included: Tuberous Sclerosis patients, Sub Ependymal Giant Astrocytoma. Everolimus was administered orally at a dose of 2.5 mg or 5 mg daily based on body weight. Patients were evaluated with brain MRI every 4, 8 and 12 months after treatment start and every six months thereafter. RESULTS 16 patients were enrolled from September 2014 and 2019. The median age was 7.5 years old. All patients had at least one adverse event. Events rated as severe (grade 3/4) were reported in 6 patients. Stomatitis was the most frequent adverse event. One patient discontinued treatment due to grade 4 toxicity (ulcerative stomatitis and fatigue). The median duration of treatment was 21 months (4–57 months). Brain MRI evaluations have showed disease stability in 11 patients, partial response in 2 patients and disease progression in 3 patients. CONCLUSIONS Everolimus has proven to be well tolerated and effective treatment in terms of disease stability in patients with pLGGs. It’s also an excellent example of chemo-free personalized approach.

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Gasperini Syndrome: A Case Report and Systematic Review and Proposing a New Definition

Case Reports in Neurology ◽

10.1159/000510845 ◽

2020 ◽

Vol 12 (3) ◽

pp. 433-439

Author(s):

Riwaj Bhagat ◽

Siddharth Narayanan ◽

Marwa Elnazeir ◽

Thong Diep Pham ◽

Robert Paul Friedland ◽

...

Keyword(s):

Systematic Review ◽

Brain Mri ◽

Cranial Nerves ◽

Clinical Manifestations ◽

The Other ◽

Neurological Deficits ◽

Brainstem Stroke ◽

Neurological Features ◽

The Right ◽

Core Features

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.

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QOL-09. WHOLE-BRAIN WHITE MATTER NETWORK CONNECTIVITY IS DISRUPTED BY PEDIATRIC BRAIN TUMOR TREATMENT

Neuro-Oncology ◽

10.1093/neuonc/noaa222.673 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii432-iii432

Author(s):

Adeoye Oyefiade ◽

Kiran Beera ◽

Iska Moxon-Emre ◽

Jovanka Skocic ◽

Ute Bartels ◽

...

Keyword(s):

White Matter ◽

Pediatric Brain Tumor ◽

Pediatric Brain Tumors ◽

Magnetic Resonance Images ◽

Long Term Effects ◽

Brain White Matter ◽

The Right ◽

Pediatric Brain ◽

Betweeness Centrality

Abstract INTRODUCTION Treatments for pediatric brain tumors (PBT) are neurotoxic and lead to long-term deficits that are driven by the perturbation of underlying white matter (WM). It is unclear if and how treatment may impair WM connectivity across the entire brain. METHODS Magnetic resonance images from 41 PBT survivors (mean age: 13.19 years, 53% M) and 41 typically developing (TD) children (mean age: 13.32 years, 51% M) were analyzed. Image reconstruction, segmentation, and node parcellation were completed in FreeSurfer. DTI maps and probabilistic streamline generation were completed in MRtrix3. Connectivity matrices were based on the number of streamlines connecting two nodes and the mean DTI (FA) index across streamlines. We used graph theoretical analyses to define structural differences between groups, and random forest (RF) analyses to identify hubs that reliably classify PBT and TD children. RESULTS For survivors treated with radiation, betweeness centrality was greater in the left insular (p < 0.000) but smaller in the right pallidum (p < 0.05). For survivors treated without radiation (surgery-only), betweeness centrality was smaller in the right interparietal sulcus (p < 0.05). RF analyses showed that differences in WM connectivity from the right pallidum to other parts of the brain reliably classified PBT survivors from TD children (classification accuracy = 77%). CONCLUSIONS The left insular, right pallidum, and right inter-parietal sulcus are structurally perturbed hubs in PBT survivors. WM connectivity from the right pallidum is vulnerable to the long-term effects of treatment for PBT.

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Recurrence of Primary Breast Lymphoma Presenting as Bilateral Vitreoretinal Lymphoma

Ocular Oncology and Pathology ◽

10.1159/000515560 ◽

2021 ◽

pp. 1-5

Author(s):

Karishma Habbu ◽

Roshan George ◽

Miguel Materin

Keyword(s):

Brain Mri ◽

B Cell Lymphoma ◽

Intrathecal Chemotherapy ◽

Primary Breast Lymphoma ◽

Blurred Vision ◽

Breast Lymphoma ◽

Central Nervous System Penetration ◽

Vitreoretinal Lymphoma ◽

The Right ◽

Vitreous Biopsy

Purpose: This report describes a case of relapsed primary breast lymphoma (PBL) presenting as vitreoretinal lymphoma (VRL). Methods: We describe the clinical and hematopathologic findings in a patient with relapsed PBL involving the vitreous of both eyes. Results: A 59-year-old woman was treated for PBL with systemic and intrathecal chemotherapy 5 years prior to presentation. Three years later, she presented to an outside clinic with blurred vision in both eyes and bilateral vitritis. She was referred to our clinic with concern for ocular lymphoma. On presentation, the patient’s best-corrected visual acuity was 20/40 in the right eye and 20/25 in the left eye with 3+ vitreous cells in the right eye and 2+ vitreous cells in the left eye. Vitreous biopsy of the right eye revealed CD5-negative/CD10-negative B-cell lymphoma cells on flow cytometry. She had no evidence of disease on brain MRI, lumbar puncture, bone marrow biopsy, or full-body CT scans. She was treated with a regimen of rituximab, methotrexate, procarbazine, and vincristine for central nervous system penetration as well as multiple intraocular injections of methotrexate and rituximab with improvement in vision and ocular inflammation bilaterally. Conclusion: Relapsed PBL can present as bilateral VRL.

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Delayed post-hypoxic leukoencephalopathy: Case report

European Psychiatry ◽

10.1016/j.eurpsy.2017.01.1654 ◽

2017 ◽

Vol 41 (S1) ◽

pp. s839-s839 ◽

Cited By ~ 1

Author(s):

M. Solerdelcoll Arimany ◽

M. Garriga ◽

E. Parellada

Keyword(s):

Neuropsychiatric Symptoms ◽

Brain Mri ◽

Clinical History ◽

Good Prognosis ◽

Mri Findings ◽

The Mean ◽

Competing Interest ◽

Weighted Sequences ◽

Direct Toxicity ◽

Progressive Cognitive Impairment

IntroductionDelayed post-hypoxic leukoencephalopathy (DPHL) is an underrecognized syndrome of delayed demyelination, where patients manifest neuropsychiatric symptoms after a period of 2–40 days of apparent recovery from a cerebral hypo-oxygenation episode.ObjectivesWe report a case of a patient who successfully recovered from an overdose of heroin, but then suffered a delayed abrupt neurological deterioration.AimsTo improve assessment and recognition of DPHL.MethodsAn adequate retrospective collection of clinical data and nonsystematic review of the literature was performed.ResultsA 43-year-old male with schizoaffective disorder who attempted suicide with an overdose of heroin, was successfully revived and return to his previously mental status, but 3 weeks after, he abruptly developed progressive cognitive impairment with akinetic mutism and ataxia. He was admitted to our acute psychiatric unit after brain CT and chemistry analyses were unremarkable. Brain MRI showed diffusely symmetric hyperintensity in the white matter (WM), pronominally the periventricular WM, on FLAIR and T2 weighted sequences. At 16 weeks postoverdose, he presented improvement both cognitive and motor symptoms, lasting deficits in frontal-executive functions.DiscussionDPHL is characterized by similar clinical and neuroimaging features regardless of the initial insult. The mean lucid interval coincides with the replacement half-life for myelin related lipids and proteins. Prolonged mild-to-moderate hypo-oxygenation of WM is thought to disrupt myelin turnover. It appears probable that these were responsible for DPHL in our patient rather than a direct toxicity.ConclusionDPHL can be diagnosed when clinical history, laboratory assessments and MRI findings are concordant. DPHL requires extensive support care and carries a relatively good prognosis.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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A Case of Atypical Bartonellosis in a 4-Year-Old Immunocompetent Child

Microorganisms ◽

10.3390/microorganisms9050950 ◽

2021 ◽

Vol 9 (5) ◽

pp. 950

Author(s):

Chiara Sodini ◽

Elena Mariotti Zani ◽

Francesco Pecora ◽

Cristiano Conte ◽

Viviana Dora Patianna ◽

...

Keyword(s):

Clinical Laboratory ◽

Bartonella Henselae ◽

Delayed Diagnosis ◽

Mitral Valve Regurgitation ◽

Low Grade ◽

Systemic Involvement ◽

Mild Disease ◽

Precise Diagnosis ◽

Major Illness ◽

The Right

In most cases, infection due to Bartonella henselae causes a mild disease presenting with a regional lymphadenopathy frequently associated with a low-grade fever, headache, poor appetite and exhaustion that spontaneously resolves itself in a few weeks. As the infection is generally transmitted by cats through scratching or biting, the disease is named cat scratch disease (CSD). However, in 5–20% of cases, mainly in immunocompromised patients, systemic involvement can occur and CSD may result in major illness. This report describes a case of systemic CSD diagnosed in an immunocompetent 4-year-old child that can be used as an example of the problems that pediatricians must solve to reach a diagnosis of atypical CSD. Despite the child’s lack of history suggesting any contact with cats and the absence of regional lymphadenopathy, the presence of a high fever, deterioration of their general condition, increased inflammatory biomarkers, hepatosplenic lesions (i.e., multiple abscesses), pericardial effusion with mild mitral valve regurgitation and a mild dilatation of the proximal and medial portion of the right coronary artery, seroconversion for B. henselae (IgG 1:256) supported the diagnosis of atypical CSD. Administration of oral azithromycin was initiated (10 mg/kg/die for 3 days) with a progressive normalization of clinical, laboratory and US hepatosplenic and cardiac findings. This case shows that the diagnosis of atypical CSD is challenging. The nonspecific, composite and variable clinical features of this disease require a careful evaluation in order to achieve a precise diagnosis and to avoid both a delayed diagnosis and therapy with a risk of negative evolution.

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Provocation and prediction of visual peripersonal neglect-like symptoms in preoperative planning and during awake brain surgery

Acta Neurochirurgica ◽

10.1007/s00701-021-04822-2 ◽

2021 ◽

Author(s):

Viktória Tamás ◽

Gabriella Sebestyén ◽

Szilvia Anett Nagy ◽

Péter Zsolt Horváth ◽

Ákos Mérei ◽

...

Keyword(s):

Preoperative Planning ◽

Right Hemisphere ◽

Superior Temporal Gyrus ◽

Low Grade ◽

Navigated Transcranial Magnetic Stimulation ◽

Resonance Imaging ◽

Parietal Area ◽

Routine Magnetic Resonance Imaging ◽

Visuospatial Functions ◽

The Right

AbstractNeglect is a severe neuropsychological/neurological deficit that usually develops due to lesions of the posterior inferior parietal area of the right hemisphere and is characterized by a lack of attention to the left side. Our case is a proven right-handed, 30-year-old female patient with a low-grade glioma, which was located in the temporo-opercular region and also in the superior temporal gyrus of the right hemisphere. Upon presurgical planning, the motor, language, and visuospatial functions were mapped. In order to achieve this, the protocol for routine magnetic resonance imaging and navigated transcranial magnetic stimulation has been expanded, accordingly.

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A case report of unilateral conductive hearing loss as a consequence of malignant sinonasal melanoma: the importance of completing a full clinical history and examination

The Egyptian Journal of Otolaryngology ◽

10.1186/s43163-020-00057-7 ◽

2020 ◽

Vol 36 (1) ◽

Author(s):

Victoria Blackabey ◽

Olivia Kenyon ◽

Rishi Talwar

Keyword(s):

Hearing Loss ◽

Case Report ◽

Clinical Symptoms ◽

Symptomatic Relief ◽

Clinical History ◽

Histological Diagnosis ◽

Unusual Presentation ◽

Sinus Surgery ◽

Cancer Management ◽

The Right

Abstract Background Sinonasal melanoma is a rare head and neck tumour. It is associated with a poor prognosis, high rates of loco-regional recurrence and distant metastasis. Treatment of the disease is therefore complicated, and because of limited data regarding the cancer, management is frequently tailored to the individual patient. We describe an unusual presentation of sinonasal melanoma with relevant histology, radiology and clinical photography. Case presentation The case report describes the presentation of a 64-year-old man to the Ear, Nose and Throat department with progressive right-sided hearing loss. A thorough history highlighted other clinical symptoms including unilateral nasal obstruction and epistaxis. Clinical examination showed a right middle ear effusion with a polypoidal lesion in the right nasal cavity. Relevant imaging demonstrated a destructive process that required further assessment. An endoscopic sinus procedure was performed to obtain histological diagnosis as well as providing symptomatic relief. Histology confirmed malignant mucosal melanoma. The patient underwent maxillectomy and orbital exenteration (due to further progression of disease) at a tertiary centre with a plan for subsequent immunotherapy. This however has been delayed due to further surgery to excise a metastatic lesion to the right femur. Conclusions This case report highlights the importance of a thorough clinical history and examination. An unusual presentation of a sinonasal tumour can easily be missed leading to a significant delay in treatment. The case report also describes the use of functional endoscopic sinus surgery in order to obtain histological diagnosis and to debulk the tumour, providing symptomatic relief. The current literature regarding management will be discussed as well as current developments guiding future treatment.

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