Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: An odd presentation of a rare pathology

Abstract Background and Importance: Papillary glioneuronal tumor is a recently known entity in central nervous system tumors. These benign WHO grade I tumors are mostly seen in young adults. Pediatric PGNT is rare and there is no report of these tumors in toddlers. Headache, nausea/vomiting and seizure are most common clinical symptoms. Acute presentation with focal neurological deficits or loss of consciousness are not amongst the expected presentations. These tumors are typically cystic with enhancing mural nodule. Although case with chronic intermittent microhemorrhages are reported in the literature but overt intra-tumoral hemorrhage is an odd radiological presentation with just one reported case in the literature.Clinical presentation: We present an extremely rare case of PGNT presenting with sudden onset hemiparesis and impaired consciousness due to acute intra-tumoral hemorrhage in a toddler which was surgically treated with favorable outcome.Conclusion: PGNTs can also be seen in very young children even in toddlers. Also, it should be kept in mind that these tumors have potential for overt intra-tumoral hemorrhage and acute presentation with focal neurological deficits mimicking more common pathologies which should be considered to plan optimal patient management.

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Spontaneous cervical epidural hematoma: Insight into this occurrence with case examples

Surgical Neurology International ◽

10.25259/sni_15_2021 ◽

2021 ◽

Vol 12 ◽

pp. 79

Author(s):

Brian Fiani ◽

Ryan Jarrah ◽

Nicholas J. Fiani ◽

Juliana Runnels

Keyword(s):

Epidural Hematoma ◽

Clinical Presentation ◽

Treatment Algorithm ◽

Region Of Interest ◽

Sudden Onset ◽

Neurological Deficits ◽

Spinal Epidural Hematoma ◽

Comprehensive Overview ◽

Case Examples ◽

First Case

Background: First characterized in the 19th century, spontaneous spinal epidural hematoma (SSEH) is known as the idiopathic accumulation of blood within the spinal canal’s epidural space, causing symptoms varying from general back pain to complete paraplegia. With varying etiologies, a broad spectrum of severity and symptoms, a time-dependent resolution period, and no documented diagnosis or treatment algorithm, SSEH is a commonly misunderstood condition associated with increasing morbidity. While SSEH can occur at any vertebrae level, 16% of all SSEH cases occur in the cervical spine, making it a region of interest to clinicians. Case Description: Herein, the authors present two case examples describing the clinical presentation of SSEH, while also reviewing the literature to provide a comprehensive overview of its presentation, pathology, and treatment. The first case is a patient with nontraumatic sudden onset neck pain with rapidly progressing weakness. The second case is a patient with painless weakness that developed while taking 325 mg of aspirin daily. Conclusion: Clinicians should keep SSEH in their differential diagnosis when seeing patients with nontraumatic sources of weakness in their extremities. The appropriate steps should be followed to diagnose and treat this condition with magnetic resonance imaging and surgical decompression if there are progressive neurological deficits. There is a continued need for more extensive database-driven studies to understand better SSEHs clinical presentation, etiology, and ultimate treatment.

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Abstract TP424: Re-bleed in Nontraumatic Convexity Subarachnoid Hemorrhage

Stroke ◽

10.1161/str.48.suppl_1.tp424 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

Jennifer Harris ◽

Rachel Forman ◽

James Conners ◽

Rajeev Garg ◽

Sayona John ◽

...

Keyword(s):

Subarachnoid Hemorrhage ◽

Clinical Outcome ◽

Clinical Presentation ◽

Clinical Symptoms ◽

Severe Headache ◽

Neurological Deficits ◽

Amyloid Angiopathy ◽

Good Clinical Outcome ◽

Cerebral Vasoconstriction ◽

The Mean

Introduction: Re-bleeding can have significant adverse consequences, but has not been well described in isolated nontraumatic convexity subarachnoid hemorrhage (cSAH), and therefore we sought to report the clinical spectrum of re-bleed in this population. Methods: With IRB approval, we retrospectively reviewed consecutive nontraumatic cSAH admitted to our institution from August 1, 2006 to June 1, 2016. cSAH was defined as radiographic hemorrhage isolated to the convexities, without cisternal component. Trauma was excluded. Data was abstracted on demographics, medical history, neuroimaging, etiology, and clinical presentation. Good clinical outcome was defined as modified rankin score of 0-2 at 3 months. Results: Among 93 cSAH patients, re-bleed occurred in 9 (10%) patients. The mean age of patients with re-bleed was 55.6 years (range, 24-68) and 8 (89%) were women. The majority 6 (67%) were Hispanic. Most patients 8 (89%) presented with severe headache. The etiology of cSAH was reversible cerebral vasoconstriction syndrome (RCVS) in 6 (67%), amyloid angiopathy in 1 (11%), cerebral venous thrombosis (CVT) in 1 (11%), and post-procedural related in 1 (11%). Initial re-bleeding was new remote subarachnoid hemorrhage in 1 (11%) and new intracerebral hemorrhage in 8 (89%). Clinical symptoms of initial re-bleeding included isolated worsening headache in 2 (22%), focal neurological deficits in 4 (44%), and no clinical change in 3 (33%). Time from initial neuroimaging documenting cSAH to CT documenting re-bleed was 40 hours (range, 5 - 74), and re-bleed occurred within 60 hrs in most patients (89%). Two patients had subsequent re-bleeding (due to CVT and RCVS) documented at 1.9 and 11.8 hours after the initial re-bleed CT. Good clinical outcome was seen in 7 (78%) . Conclusion: Re-bleeding was seen in 10% of cSAH patients and in the majority of patients occurred early, within 2.5 days. Clinicians should be aware of this potential complication of cSAH.

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Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: an odd presentation of a rare pathology

British Journal of Neurosurgery ◽

10.1080/02688697.2020.1800588 ◽

2020 ◽

pp. 1-6

Author(s):

Amin Tavallaii ◽

Ehsan Keykhosravi ◽

Hamid Rezaee

Keyword(s):

Glioneuronal Tumor ◽

Acute Presentation ◽

Papillary Glioneuronal Tumor

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COVID-19 and neurology perspective

Hormone Molecular Biology and Clinical Investigation ◽

10.1515/hmbci-2020-0069 ◽

2021 ◽

Vol 42 (1) ◽

pp. 69-75

Author(s):

Shivani Singh ◽

Ashok Kumar Ahirwar ◽

Priyanka Asia ◽

Niranjan Gopal ◽

Kirti Kaim ◽

...

Keyword(s):

Neurological Disorders ◽

Clinical Presentation ◽

Sudden Onset ◽

Natural Immunity ◽

The Novel ◽

Specific Antibodies ◽

Initial Symptoms ◽

Diet And Exercise ◽

Short Span ◽

Very High

Abstract COVID-19 caused by SARS CoV2 (The novel corona virus) has already taken lives of many people across the globe even more than anyone could have imagined. This outbreak occurred in China and since then it is expanding its devastating effects by leaps and bounds. Initially it appeared to be an outbreak of pneumonia but soon it was found to be much more than that and the infectivity was found to be very high. This is the reason that it has taken whole globe in its trap and become a pandemic in such a short span of time. Death is occurring because it is a new virus and human body has no specific antibodies for it. Presently there is no approved vaccine so everyone is susceptible but people with co-morbidities appear to be in more risk and the best way for protection is social distancing and increasing one’s natural immunity by taking healthy diet and exercise. When a person is infected the clinical presentation ranges from asymptomatic to severe ARDS, sudden onset of anosmia, headache, cough may be the initial symptoms. This review is focused on immunopathology and effect of COVID-19 on neurological disorders and also the neurological manifestations and the treatment.

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Recurrent Sphenocavernous Meningioma

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0041-1725941 ◽

2021 ◽

Author(s):

Mizuho Inoue ◽

Mohamed Labib ◽

Alexander Yang ◽

A. Samy Youssef

Keyword(s):

Tumor Resection ◽

Intensity Modulated Radiotherapy ◽

Residual Tumor ◽

World Health ◽

Neurological Deficits ◽

Falciform Ligament ◽

Total Resection ◽

Anterior Clinoid Process ◽

Who Grade ◽

Health Organization

AbstractA case of a recurrent sphenocavernous meningioma is presented. The patient is a 42-year-old male who presented with an episode of transient right-sided numbness. A magnetic resonance imaging (MRI) revealed a large left sphenocavernous meningioma. The patient underwent a frontotemporal craniotomy for tumor resection. Near total resection was achieved with minimal residual in the left cavernous sinus (CS) and orbital apex. The pathology was consistent with meningioma, World Health Organization (WHO) grade I. A follow-up MRI was done 9 months after surgery and showed a growth of the residual tumor, which was treated with intensity modulated radiotherapy. Tumor growth was detected on serial imaging over a 4-year period. Surgical resection was offered. A left frontotemporal craniotomy with pretemporal transcavernous approach was performed. The bone flap was reopened and the dura was opened in a Y-shaped fashion. The roof of the optic canal was drilled off, and the falciform ligament was opened to decompress the optic nerve. The tumor was disconnected from the anterior clinoid region (the anterior clinoid process was eroded by the tumor) and reflected off the wall of the lateral CS. Tumor was adherent to the V2 fascicles (the lateral CS wall was resected in the first surgery) and was sharply dissected off. Gross total resection was achieved. The pathology was consistent with meningioma, WHO grade I. The patient had an unremarkable postoperative course without any new neurological deficits.The link to the video can be found at: https://youtu.be/KVBVw_86JqM.

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Papillary glioneuronal tumor: A clinicopathological and immunohistochemical study of two cases

Neuropathology ◽

10.1111/j.1440-1789.2006.00687.x ◽

2006 ◽

Vol 26 (3) ◽

pp. 243-248 ◽

Cited By ~ 23

Author(s):

Li Chen ◽

Yue-Shan Piao ◽

Qing-Zhong Xu ◽

Xiao-Ping Yang ◽

Hong Yang ◽

...

Keyword(s):

Immunohistochemical Study ◽

Glioneuronal Tumor ◽

Papillary Glioneuronal Tumor

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Clinical Presentation and Autoimmune Characteristics of Very Young Children at the Onset of Type 1 Diabetes Mellitus

Journal of Pediatric Endocrinology and Metabolism ◽

10.1515/jpem.2010.180 ◽

2010 ◽

Vol 23 (11) ◽

Cited By ~ 1

Author(s):

Bizzarri Carla ◽

Benevento Danila ◽

Ciampalini Paolo ◽

Patera Ippolita Patrizia ◽

Schiaffini Riccardo ◽

...

Keyword(s):

Diabetes Mellitus ◽

Type 1 Diabetes ◽

Young Children ◽

Type 1 Diabetes Mellitus ◽

Clinical Presentation ◽

Very Young Children

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LGG-50. INTEGRATED MOLECULAR AND CLINICAL ANALYSIS OF 1,000 PEDIATRIC LOW-GRADE GLIOMAS UNCOVERS NOVEL SUBGROUPS FOR CLINICAL RISK STRATIFICATION

Neuro-Oncology ◽

10.1093/neuonc/noaa222.428 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii375-iii376

Author(s):

Uri Tabori ◽

Scott Ryall ◽

Michal Zapotocky ◽

Julie Bennett ◽

Liana Nobre ◽

...

Keyword(s):

Risk Stratification ◽

Clinical Presentation ◽

Mapk Pathway ◽

Clinical Analysis ◽

Genetic Alterations ◽

Low Grade ◽

Who Grade ◽

Low Grade Gliomas ◽

Younger Age ◽

Risk Categories

Abstract Pediatric low-grade gliomas (pLGG) are primarily driven by genetic alterations in the RAS/MAPK pathway, most commonly involving BRAF of NF1. Despite their molecular convergence, pLGG often show unexplained variability in their clinical outcome. To address this, we molecularly characterized a cohort of >1,000 clinically annotated pLGG. 84% of cases harbored a detectable driver mutation. The remaining 16% of patients nonetheless showed RAS/MAPK pathway up-regulation at the RNA level. The clinical presentation and outcome of pLGG appeared highly variable and linked to the alteration type: re-arrangement or SNV. Re-arrangement-driven tumors were diagnosed at a younger age (6.6 versus 10.9 years, p<0.0001), enriched for WHO grade I histology (88% versus 66%, p<0.0001), infrequently progressed (27% versus 46%, p<0.0001), and rarely resulted in death (3 versus 13%, p<0.0001) as compared to SNV-driven tumors. These included the rarest molecular drivers of pLGG, for which we now have the clinicopathologic features of including MYB, MYBL1, FGFR2 fusions, FGFR1-TACC1, FGFR1 SNVs, IDH1 p.R132H, and H3.3 p.K27M. Utilizing this information, we suggest novel risk categories of pLGG that effectively predicted patient outcome. Low-risk tumors progressed infrequently and rarely succumbed to their disease (10-year PFS of 71% and OS of 98%). Intermediate-risk pLGG had a 10-year PFS and OS of 35% and 90%, respectively. High risk pLGG almost invariably progressed (10-year PFS of 0%) and these patients often succumbed to their disease (10-year OS of 41%). These data highlight the biological and clinical differences between pLGG subtypes and offers molecular based risk stratification to these cancers.

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Hyperperfusion Syndrome: Toward a Stricter Definition

Neurosurgery ◽

10.1227/01.neu.0000088738.80838.74 ◽

2003 ◽

Vol 53 (5) ◽

pp. 1053-1060 ◽

Cited By ~ 126

Author(s):

◽

Shelagh B. Coutts ◽

Michael D. Hill ◽

William Y. Hu ◽

Garnette R. Sutherland

Keyword(s):

Carotid Artery ◽

Intracerebral Hemorrhage ◽

Carotid Endarterectomy ◽

Clinical Presentation ◽

Imaging Techniques ◽

Neurological Deficits ◽

Acute Hemorrhage ◽

Motor Weakness ◽

Hyperperfusion Syndrome ◽

Angioplasty And Stenting

Abstract OBJECTIVE Hyperperfusion syndrome is a rare and potentially devastating complication of carotid endarterectomy or carotid artery angioplasty and stenting. With the advent of new imaging techniques, we reviewed our experience with this phenomenon. METHODS This report is a retrospective review of 129 consecutive cases of carotid endarterectomy performed between June 1, 2000, and May 31, 2002, and 44 consecutive cases of carotid artery angioplasty and stenting performed between January 1, 1997, and May 31, 2002. We specifically searched for examples of patients who developed postprocedural nonthrombotic neurological deficits that typified the hyperperfusion syndrome. RESULTS Seven cases of hyperperfusion syndrome occurred, four after endarterectomy (3.1% of carotid endarterectomy cases) and three after stenting (6.8% of stenting cases). The cases of hyperperfusion were classified as presenting with 1) acute focal edema (two cases with stroke-like presentation, attributable to edema immediately after revascularization), 2) acute hemorrhage (two cases of intracerebral hemorrhage immediately after stenting and one case immediately after endarterectomy), or 3) delayed classic presentation (two cases with seizures, focal motor weakness, and/or late intracerebral hemorrhage at least 24 hours after endarterectomy). CONCLUSION Hyperperfusion syndrome may be more common and more variable in clinical presentation than previously appreciated.

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