{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,7,22]],"date-time":"2024-07-22T22:33:57Z","timestamp":1721687637747},"reference-count":29,"publisher":"Georg Thieme Verlag KG","issue":"04","license":[{"start":{"date-parts":[[2022,9,20]],"date-time":"2022-09-20T00:00:00Z","timestamp":1663632000000},"content-version":"vor","delay-in-days":1754,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Asian J Neurosurg"],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:p>Subependymal giant cell astrocytomas (SEGAs) are histologically benign tumors most frequently associated with tuberous sclerosis complex (TSC). Despite their benign histopathological appearance, they may cause unfavorable outcomes due to their intraventricular location. Rarely, SEGA may be associated with hyperproteinorrhachia (high levels of proteins in the cerebrospinal fluid [CSF]), which causes malresorptive, communicating hydrocephalus; certainly, this scenario makes shunt obstruction likely in this patient population. In this report, we illustrate the case of hyperproteinorrhachia in an SEGA patient with known TSC, who presented repeatedly with shunt failure from proteinaceous shunt obstruction. Subsequent surgical resection of the main intraventricular lesion resulted in a dramatic drop in the CSF protein levels and has since prevented further shunt failures. Different treatment concepts and possible pathophysiology are discussed and the pertinent literature is reviewed.<\/jats:p>","DOI":"10.4103\/ajns.ajns_231_16","type":"journal-article","created":{"date-parts":[[2017,10,3]],"date-time":"2017-10-03T12:54:35Z","timestamp":1507035275000},"page":"746-750","source":"Crossref","is-referenced-by-count":4,"title":["Subependymal giant cell astrocytoma: Associated hyperproteinorrhachia causing shunt failures and nonobstructive hydrocephalus - Report of successful treatment with long-term follow-up"],"prefix":"10.1055","volume":"12","author":[{"given":"Ekkehard","family":"Kasper","sequence":"additional","affiliation":[{"name":"Department of Surgery, Beth Israel Deaconess Medical Center, Division of Neurosurgery, Harvard Medical School, Boston, MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yosef","family":"Laviv","sequence":"additional","affiliation":[{"name":"Department of Surgery, Beth Israel Deaconess Medical Center, Division of Neurosurgery, Harvard Medical School, Boston, MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammed-Adeeb","family":"Sebai","sequence":"additional","affiliation":[{"name":"Department of Surgery, Beth Israel Deaconess Medical Center, Division of Neurosurgery, Harvard Medical School, Boston, MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ning","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William","family":"Butler","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2022,9,20]]},"reference":[{"key":"key-10.4103\/1793-5482.215770-1","first-page":"657","volume-title":"Tuberous sclerosis","author":"Curatolo","year":"2008","journal-title":"Lancet"},{"key":"key-10.4103\/1793-5482.215770-2","first-page":"184","volume-title":"Tuberous sclerosis complex","author":"Rodrigues","year":"2012","journal-title":"An Bras Dermatol"},{"key":"key-10.4103\/1793-5482.215770-3","first-page":"1345","volume-title":"The tuberous sclerosis complex","author":"Crino","year":"2006","journal-title":"N Engl J Med"},{"key":"key-10.4103\/1793-5482.215770-4","first-page":"217","volume-title":"Biological behavior and tumorigenesis of subependymal giant cell astrocytomas","author":"Kim","year":"2001","journal-title":"J Neurooncol"},{"key":"key-10.4103\/1793-5482.215770-5","first-page":"115","volume-title":"Tuberous sclerosis complex","author":"Sparagana","year":"2000","journal-title":"Curr Opin Neurol"},{"key":"key-10.4103\/1793-5482.215770-6","first-page":"185","volume-title":"Subependymal giant cell astrocytoma.Significance and possible cytogenetic implications of an immunohistochemical study","author":"Bonnin","year":"1984","journal-title":"Acta Neuropathol"},{"key":"key-10.4103\/1793-5482.215770-7","first-page":"106","volume-title":"Recurrent subependymal giant-cell astrocytoma in the absence of tuberous sclerosis.Case report","author":"Halmagyi","year":"1979","journal-title":"J Neurosurg"},{"key":"key-10.4103\/1793-5482.215770-8","first-page":"1457","volume-title":"Subependymal giant cell tumors in tuberous sclerosis complex","author":"Goh","year":"2004","journal-title":"Neurology"},{"key":"key-10.4103\/1793-5482.215770-9","first-page":"125","volume-title":"Epidemiology of tuberous sclerosis","author":"Osborne","year":"1991","journal-title":"Ann NY Acad Sci"},{"key":"key-10.4103\/1793-5482.215770-10","first-page":"563","volume-title":"The burden of subependymal giant cell astrocytomas associated with tuberous sclerosis complex: Results of a patient and caregiver survey","author":"Skalicky","year":"2015","journal-title":"J Child Neurol"},{"key":"key-10.4103\/1793-5482.215770-11","first-page":"1562","volume-title":"Subependymal giant cell astrocytomas in patients with tuberous sclerosis complex: Considerations for surgical or pharmacotherapeutic intervention","author":"Wheless","year":"2014","journal-title":"J Child Neurol"},{"key":"key-10.4103\/1793-5482.215770-12","first-page":"115","volume-title":"On the treatment of subependymal giant cell astrocytomas and associated hydrocephalus in tuberous sclerosis","author":"Di","year":"1995","journal-title":"Pediatr Neurosurg"},{"key":"key-10.4103\/1793-5482.215770-13","first-page":"241","volume-title":"Persistent communicating hydrocephalus in adult tuberous sclerosis patients: A possible therapeutic role for everolimus","author":"Laviv","year":"2015","journal-title":"Acta Neurochir (Wien)"},{"key":"key-10.4103\/1793-5482.215770-14","first-page":"83","volume-title":"Effective everolimus treatment of inoperable, life-threatening subependymal giant cell astrocytoma and intractable epilepsy in a patient with tuberous sclerosis complex","author":"Perek-Polnik","year":"2012","journal-title":"Eur J Paediatr Neurol"},{"key":"key-10.4103\/1793-5482.215770-15","first-page":"30","volume-title":"Sudden death in epilepsy due to an isolated subependymal giant cell astrocytoma of the septum pellucidum","author":"Prahlow","year":"1995","journal-title":"Am J Forensic Med Pathol"},{"key":"key-10.4103\/1793-5482.215770-16","first-page":"21","volume-title":"A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex","author":"Harter","year":"2014","journal-title":"J Neurosurg Pediatr"},{"key":"key-10.4103\/1793-5482.215770-17","first-page":"1801","volume-title":"Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis","author":"Krueger","year":"2010","journal-title":"N Engl J Med"},{"key":"key-10.4103\/1793-5482.215770-18","first-page":"125","volume-title":"Efficacy and safety of everolimus for subependymal giant cell astrocytomas associated with tuberous sclerosis complex (EXIST-1): A multicentre, randomised, placebo-controlled phase 3 trial","author":"Franz","year":"2013","journal-title":"Lancet"},{"key":"key-10.4103\/1793-5482.215770-19","first-page":"574","volume-title":"Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma","author":"Krueger","year":"2013","journal-title":"Neurology"},{"key":"key-10.4103\/1793-5482.215770-20","first-page":"932","volume-title":"Increased intracranial pressure caused by increased protein content in the cerebrospinal fluid; an explanation of papilledema in certain cases of small intracranial and intraspinal tumors, and in the Guillain-Barre syndrome","author":"Gardner","year":"1954","journal-title":"N Engl J Med"},{"key":"key-10.4103\/1793-5482.215770-21","first-page":"185","volume-title":"New concepts in the pathogenesis of hydrocephalus","author":"Krishnamurthy","year":"2014","journal-title":"Transl Pediatr"},{"key":"key-10.4103\/1793-5482.215770-22","first-page":"73","volume-title":"Non-obstructive hydrocephalus associated with intracranial schwannomas: Hyperproteinorrhachia as an etiopathological factor?","author":"Bloch","year":"2003","journal-title":"Acta Neurochir (Wien)"},{"key":"key-10.4103\/1793-5482.215770-23","first-page":"1188","volume-title":"Clinical and neuroimaging characteristics of hydrocephalus associated with vestibular schwannoma","author":"Tanaka","year":"2003","journal-title":"J Neurosurg"},{"key":"key-10.4103\/1793-5482.215770-24","first-page":"144","volume-title":"A review of the symptoms and signs of acoustic neurofibromata","author":"Edwards","year":"1951","journal-title":"Brain"},{"key":"key-10.4103\/1793-5482.215770-25","first-page":"68","volume-title":"Communicating hydrocephalus and vestibular schwannomas: Etiology, treatment, and long-term follow-up","author":"Al","year":"2013","journal-title":"J Neurol Surg B Skull Base"},{"key":"key-10.4103\/1793-5482.215770-26","first-page":"639","volume-title":"Physical properties of cerebrospinal fluid of relevance to shunt function 1: The effect of protein upon CSF viscosity","author":"Brydon","year":"1995","journal-title":"Br J Neurosurg"},{"key":"key-10.4103\/1793-5482.215770-27","first-page":"306","volume-title":"Subacute hydrocephalus after experimental subarachnoid hemorrhage: Its prevention by intrathecal fibrinolysis with recombinant tissue plasminogen activator","author":"Brinker","year":"1992","journal-title":"Neurosurgery"},{"key":"key-10.4103\/1793-5482.215770-28","first-page":"759","volume-title":"Phase 1 trial of prevention of hydrocephalus after intraventricular hemorrhage in newborn infants by drainage, irrigation, and fibrinolytic therapy","author":"Whitelaw","year":"2003","journal-title":"Pediatrics"},{"key":"key-10.4103\/1793-5482.215770-29","first-page":"443","volume-title":"Soluble tissue antigens in human brain tumor and cerebrospinal fluid","author":"Hass","year":"1966","journal-title":"Arch Neurol"}],"container-title":["Asian Journal of Neurosurgery"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.4103\/ajns.AJNS_231_16.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,9,20]],"date-time":"2022-09-20T22:54:15Z","timestamp":1663714455000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.4103\/ajns.AJNS_231_16"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12]]},"references-count":29,"journal-issue":{"issue":"04","published-online":{"date-parts":[[2022,9,20]]},"published-print":{"date-parts":[[2017,12]]}},"URL":"https:\/\/doi.org\/10.4103\/ajns.ajns_231_16","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["1793-5482","2248-9614"],"issn-type":[{"value":"1793-5482","type":"print"},{"value":"2248-9614","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,12]]}}}