{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T21:08:30Z","timestamp":1791666510478,"version":"4.3.4"},"reference-count":52,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","license":[{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,11,1]]},"abstract":"<jats:sec>\n                    <jats:title>OBJECTIVE<\/jats:title>\n                    <jats:p>Enzyme replacement therapy (ERT) with cerliponase alfa (Brineura) has been shown to slow the progression of milestone deterioration in neuronal ceroid lipofuscinosis type 2 (CLN2), an inherited neurodegenerative lysosomal storage disorder. Cerliponase alfa must be administered intraventricularly every 2 weeks, necessitating the placement of ventricular reservoirs and requiring frequent access. Traditionally, positive cerebrospinal fluid (CSF) cultures obtained from implanted ventricular reservoirs are managed with device replacement and antibiotic treatment. The authors sought to establish which circumstances might allow for careful observation without device removal. In this study, the authors report their single-institution experience with the clinical outcomes of positive CSF cultures in 16 CLN2 patients with ventricular reservoirs over 6 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>The authors retrospectively reviewed a cohort of 16 patients with CLN2 disease who had ventricular reservoirs placed for ERT administration. At each ERT infusion, CSF was collected by sterile technique and cultured with both thioglycolate broth and agar plate. This CSF collection was standard practice as per the initial trial protocol for use of ERT in CLN2. Epidemiological and microbiological data, symptomatology, total antibiotic days, removal and replacement of the ventricular reservoir, hospital length of stay, and mortality were analyzed for each patient.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>\n                      In the authors\u2019 cohort, 11 of 16 patients (69%) had at least 1 positive CSF culture. Of the 11 patients with positive cultures, only 3 had their device removed and replaced due to a positive culture with concurrent antibiotic treatment at the authors\u2019 center, and 2 patients subsequently were treated with prophylactic antibiotic infusion. Of 1401 total CSF cultures, 64 (4.56%) were positive. The most common organism grown was\n                      <jats:italic>Cutibacterium acnes<\/jats:italic>\n                      , which typically only grew in broth culture (82%), suggesting low bacterial burden. The other 8 patients with positive cultures remained asymptomatic with no intervention required.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>At the authors\u2019 institution, we have found that in asymptomatic patients with frequently accessed ventricular reservoirs, positive CSF culture with low virulence skin flora was common. Rarely did positive cultures with common skin flora necessitate intervention. The authors\u2019 experience has shown that nonvirulent infection of ventricular reservoirs with low virulence skin flora can be monitored without intervention. This strategy reduces the risks of invasive surgery, prolonged antibiotic courses, and missed infusions.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2025.4.peds24452","type":"journal-article","created":{"date-parts":[[2025,8,23]],"date-time":"2025-08-23T01:54:55Z","timestamp":1755914095000},"page":"649-656","source":"Crossref","is-referenced-by-count":3,"title":["Management of positive cerebrospinal fluid cultures from intraventricular reservoirs of neuronal ceroid lipofuscinosis type 2 patients: one institution\u2019s experience"],"prefix":"10.3171","volume":"36","author":[{"given":"Gianna M.","family":"Fote","sequence":"first","affiliation":[{"name":"Department of Neurological Surgery, University of California, Irvine, Orange, California;"},{"name":"Division of Neurological Surgery, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda","family":"Schafenacker","sequence":"additional","affiliation":[{"name":"Department of Hospital Medicine, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasjit","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of Infectious Disease, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Sherlock","sequence":"additional","affiliation":[{"name":"Division of Neurological Surgery, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bianca","family":"Romero","sequence":"additional","affiliation":[{"name":"Division of Neurological Surgery, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Himstead","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, University of California, Irvine, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordan","family":"Davies","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, University of California, Irvine, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Pedroza","sequence":"additional","affiliation":[{"name":"Division of Neurological Surgery, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justin","family":"So","sequence":"additional","affiliation":[{"name":"School of Medicine, University of California, Irvine, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendi","family":"Gornick","sequence":"additional","affiliation":[{"name":"Department of Infectious Disease, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Arrieta","sequence":"additional","affiliation":[{"name":"Department of Infectious Disease, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond Y.","family":"Wang","sequence":"additional","affiliation":[{"name":"Division of Metabolic Disorders, Children\u2019s Hospital of Orange County CHOC, Orange, California; and"},{"name":"Department of Pediatrics, University of California, Irvine School of Medicine, Irvine, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joffre","family":"Olaya","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, University of California, Irvine, Orange, California;"},{"name":"Division of Neurological Surgery, Children\u2019s Hospital of Orange County CHOC, Orange, California;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"2","key":"b1","series-title":"Biochim Biophys Acta","first-page":"496","article-title":"Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis","volume":"1429","author":"Rawlings ND","year":"1999","unstructured":"Rawlings ND, Barrett AJ. Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis. Biochim Biophys Acta. 1999;1429(2):496\u2013500."},{"issue":"3","key":"b2","series-title":"Clin Genet","first-page":"213","article-title":"The clinical and genetic epidemiology of neuronal ceroid lipofuscinosis in Newfoundland","volume":"74","author":"Moore SJ","year":"2008","unstructured":"Moore SJ, Buckley DJ, Macmillan A,  The clinical and genetic epidemiology of neuronal ceroid lipofuscinosis in Newfoundland. Clin Genet. 2008;74(3):213\u2013222."},{"issue":"1","key":"b3","series-title":"Orphanet J Rare Dis","first-page":"19","article-title":"Molecular epidemiology of childhood neuronal ceroid-lipofuscinosis in Italy","volume":"8","author":"Santorelli FM","year":"2013","unstructured":"Santorelli FM, Garavaglia B, Cardona F,  Molecular epidemiology of childhood neuronal ceroid-lipofuscinosis in Italy. Orphanet J Rare Dis. 2013;8(1):19."},{"issue":"4","key":"b4","series-title":"Am J Med Genet","first-page":"347","article-title":"Late infantile neuronal ceroid lipofuscinosis: quantitative description of the clinical course in patients with CLN2 mutations","volume":"112","author":"Steinfeld R","year":"2002","unstructured":"Steinfeld R, Heim P, Von Gregory H,  Late infantile neuronal ceroid lipofuscinosis: quantitative description of the clinical course in patients with CLN2 mutations. Am J Med Genet. 2002;112(4):347\u2013354."},{"issue":"11","key":"b5","series-title":"Drugs","first-page":"1247","article-title":"Cerliponase alfa: first global approval","volume":"77","author":"Markham A","year":"2017","unstructured":"Markham A. Cerliponase alfa: first global approval. Drugs. 2017;77(11):1247\u20131249."},{"issue":"1","key":"b6","series-title":"Orphanet J Rare Dis","first-page":"221","article-title":"Presymptomatic treatment of classic late-infantile neuronal ceroid lipofuscinosis with cerliponase alfa","volume":"16","author":"Schaefers J","year":"2021","unstructured":"Schaefers J, van der Giessen LJ, Klees C,  Presymptomatic treatment of classic late-infantile neuronal ceroid lipofuscinosis with cerliponase alfa. Orphanet J Rare Dis. 2021;16(1):221."},{"issue":"5","key":"b7","series-title":"Childs Nerv Syst","first-page":"853","article-title":"The use of a smartphone-assisted ventricle catheter guide for Ommaya reservoir placement\u2014experience of a retrospective bi-center study","volume":"34","author":"Ozerov S","year":"2018","unstructured":"Ozerov S, Thomale UW, Schulz M, Schaumann A, Samarin A, Kumirova E. The use of a smartphone-assisted ventricle catheter guide for Ommaya reservoir placement\u2014experience of a retrospective bi-center study. Childs Nerv Syst. 2018;34(5):853\u2013859."},{"key":"b8","series-title":"A case series. Mol Genet Metab Rep","first-page":"100718","article-title":"Real world effectiveness of cerliponase alfa in classical and atypical patients","volume":"27","author":"Espitia Segura OM","year":"2021","unstructured":"Espitia Segura OM, Hern\u00e1ndez Z, Mancilla NI, Naranjo RA, Tavera L. Real world effectiveness of cerliponase alfa in classical and atypical patients. A case series. 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J Child Neurol. 2021;36(8):635\u2013641."},{"issue":"6","key":"b10","series-title":"J Child Neurol","first-page":"468","article-title":"Cerliponase alfa for the treatment of atypical phenotypes of CLN2 disease: a retrospective case series","volume":"36","author":"Wibbeler E","year":"2021","unstructured":"Wibbeler E, Wang R, Reyes E,  Cerliponase alfa for the treatment of atypical phenotypes of CLN2 disease: a retrospective case series. J Child Neurol. 2021;36(6):468\u2013474."},{"key":"b11","series-title":"Eur J Paediatr Neurol","first-page":"17","article-title":"Cerliponase alfa changes the natural history of children with neuronal ceroid lipofuscinosis type 2: the first French cohort","volume":"30","author":"Estublier B","year":"2021","unstructured":"Estublier B, Cano A, Hoebeke C,  Cerliponase alfa changes the natural history of children with neuronal ceroid lipofuscinosis type 2: the first French cohort. Eur J Paediatr Neurol. 2021;30:17\u201321."},{"issue":"20","key":"b12","series-title":"N Engl J Med","first-page":"1898","article-title":"Study of intraventricular cerliponase alfa for CLN2 disease","volume":"378","author":"Schulz A","year":"2018","unstructured":"Schulz A, Ajayi T, Specchio N,  Study of intraventricular cerliponase alfa for CLN2 disease. N Engl J Med. 2018;378(20):1898\u20131907."},{"issue":"1","key":"b13","series-title":"J Neurosurg Pediatr","first-page":"115","article-title":"A survival analysis of ventricular access devices for delivery of cerliponase alfa","volume":"29","author":"Craven CL","year":"2022","unstructured":"Craven CL, Gissen P, Bower R, Lee L, Aquilina K, Thompson DNP. A survival analysis of ventricular access devices for delivery of cerliponase alfa. 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Clin Transl Sci. 2021;14(5):1810\u20131821."},{"issue":"2","key":"b15","series-title":"Clin Transl Sci","first-page":"635","article-title":"Clinical pharmacokinetics and pharmacodynamics of cerliponase alfa, enzyme replacement therapy for cln2 disease by intracerebroventricular administration","volume":"14","author":"Kim A","year":"2021","unstructured":"Kim A, Grover A, Hammon K,  Clinical pharmacokinetics and pharmacodynamics of cerliponase alfa, enzyme replacement therapy for cln2 disease by intracerebroventricular administration. Clin Transl Sci. 2021;14(2):635\u2013644."},{"issue":"12","key":"b16","series-title":"Ann Clin Transl Neurol","first-page":"2437","article-title":"Neurofilament light is a treatment-responsive biomarker in CLN2 disease","volume":"6","author":"Ru Y","year":"2019","unstructured":"Ru Y, Corado C, Soon RK,  Neurofilament light is a treatment-responsive biomarker in CLN2 disease. Ann Clin Transl Neurol. 2019;6(12):2437\u20132447."},{"key":"b17","series-title":"Clin Immunol","first-page":"68","article-title":"Immunogenicity to cerliponase alfa intracerebroventricular enzyme replacement therapy for CLN2 disease: results from a phase 1\/2 study","volume":"197","author":"Cherukuri A","year":"2018","unstructured":"Cherukuri A, Cahan H, de Hart G,  Immunogenicity to cerliponase alfa intracerebroventricular enzyme replacement therapy for CLN2 disease: results from a phase 1\/2 study. Clin Immunol. 2018;197:68\u201376."},{"issue":"10","key":"b18","series-title":"J Proteome Res","first-page":"3787","article-title":"Proteomic analysis of brain and cerebrospinal fluid from the three major forms of neuronal ceroid lipofuscinosis reveals potential biomarkers","volume":"16","author":"Sleat DE","year":"2017","unstructured":"Sleat DE, Tannous A, Sohar I,  Proteomic analysis of brain and cerebrospinal fluid from the three major forms of neuronal ceroid lipofuscinosis reveals potential biomarkers. J Proteome Res. 2017;16(10):3787\u20133804."},{"issue":"1","key":"b19","series-title":"Sci Rep","first-page":"15229","article-title":"Untargeted metabolite profiling of cerebrospinal fluid uncovers biomarkers for severity of late infantile neuronal ceroid lipofuscinosis (CLN2, Batten Disease)","volume":"8","author":"Sindelar M","year":"2018","unstructured":"Sindelar M, Dyke JP, Deeb RS,  Untargeted metabolite profiling of cerebrospinal fluid uncovers biomarkers for severity of late infantile neuronal ceroid lipofuscinosis (CLN2, Batten Disease). Sci Rep. 2018;8(1):15229."},{"key":"b20","series-title":"Pediatr Neurol","first-page":"23","article-title":"Intracerebroventricular delivery as a safe, long-term route of drug administration","volume":"67","author":"Cohen-Pfeffer JL","year":"2017","unstructured":"Cohen-Pfeffer JL, Gururangan S, Lester T,  Intracerebroventricular delivery as a safe, long-term route of drug administration. Pediatr Neurol. 2017;67:23\u201335."},{"issue":"1","key":"b21","series-title":"J Neurooncol","first-page":"139","article-title":"Safety of Ommaya reservoirs in children with brain tumors: a 20-year experience with 5472 intraventricular drug administrations in 98 patients","volume":"120","author":"Peyrl A","year":"2014","unstructured":"Peyrl A, Chocholous M, Azizi AA,  Safety of Ommaya reservoirs in children with brain tumors: a 20-year experience with 5472 intraventricular drug administrations in 98 patients. J Neurooncol. 2014;120(1):139\u2013145."},{"issue":"3","key":"b22","series-title":"Mol Genet Metab","first-page":"184","article-title":"Best practices for the use of intracerebroventricular drug delivery devices","volume":"124","author":"Slavc I","year":"2018","unstructured":"Slavc I, Cohen-Pfeffer JL, Gururangan S,  Best practices for the use of intracerebroventricular drug delivery devices. 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Clin Genet. 2008;74(3):213\u2013222."},{"issue":"1","key":"b3","series-title":"Orphanet J Rare Dis","first-page":"19","article-title":"Molecular epidemiology of childhood neuronal ceroid-lipofuscinosis in Italy","volume":"8","author":"Santorelli FM","year":"2013","unstructured":"Santorelli FM, Garavaglia B, Cardona F,  Molecular epidemiology of childhood neuronal ceroid-lipofuscinosis in Italy. Orphanet J Rare Dis. 2013;8(1):19."},{"issue":"4","key":"b4","series-title":"Am J Med Genet","first-page":"347","article-title":"Late infantile neuronal ceroid lipofuscinosis: quantitative description of the clinical course in patients with CLN2 mutations","volume":"112","author":"Steinfeld R","year":"2002","unstructured":"Steinfeld R, Heim P, Von Gregory H,  Late infantile neuronal ceroid lipofuscinosis: quantitative description of the clinical course in patients with CLN2 mutations. Am J Med Genet. 2002;112(4):347\u2013354."},{"issue":"11","key":"b5","series-title":"Drugs","first-page":"1247","article-title":"Cerliponase alfa: first global approval","volume":"77","author":"Markham A","year":"2017","unstructured":"Markham A. Cerliponase alfa: first global approval. Drugs. 2017;77(11):1247\u20131249."},{"issue":"1","key":"b6","series-title":"Orphanet J Rare Dis","first-page":"221","article-title":"Presymptomatic treatment of classic late-infantile neuronal ceroid lipofuscinosis with cerliponase alfa","volume":"16","author":"Schaefers J","year":"2021","unstructured":"Schaefers J, van der Giessen LJ, Klees C,  Presymptomatic treatment of classic late-infantile neuronal ceroid lipofuscinosis with cerliponase alfa. Orphanet J Rare Dis. 2021;16(1):221."},{"issue":"5","key":"b7","series-title":"Childs Nerv Syst","first-page":"853","article-title":"The use of a smartphone-assisted ventricle catheter guide for Ommaya reservoir placement\u2014experience of a retrospective bi-center study","volume":"34","author":"Ozerov S","year":"2018","unstructured":"Ozerov S, Thomale UW, Schulz M, Schaumann A, Samarin A, Kumirova E. The use of a smartphone-assisted ventricle catheter guide for Ommaya reservoir placement\u2014experience of a retrospective bi-center study. Childs Nerv Syst. 2018;34(5):853\u2013859."},{"key":"b8","series-title":"A case series. Mol Genet Metab Rep","first-page":"100718","article-title":"Real world effectiveness of cerliponase alfa in classical and atypical patients","volume":"27","author":"Espitia Segura OM","year":"2021","unstructured":"Espitia Segura OM, Hern\u00e1ndez Z, Mancilla NI, Naranjo RA, Tavera L. Real world effectiveness of cerliponase alfa in classical and atypical patients. A case series. Mol Genet Metab Rep. 2021;27:100718."},{"issue":"8","key":"b9","series-title":"J Child Neurol","first-page":"635","article-title":"Development of the \"Hamburg Best Practice Guidelines for ICV-Enzyme Replacement therapy (ERT) in CLN2 Disease\" based on 6 years treatment experience in 48 patients","volume":"36","author":"Schwering C","year":"2021","unstructured":"Schwering C, Kammler G, Wibbeler E,  Development of the \"Hamburg Best Practice Guidelines for ICV-Enzyme Replacement therapy (ERT) in CLN2 Disease\" based on 6 years treatment experience in 48 patients. J Child Neurol. 2021;36(8):635\u2013641."},{"issue":"6","key":"b10","series-title":"J Child Neurol","first-page":"468","article-title":"Cerliponase alfa for the treatment of atypical phenotypes of CLN2 disease: a retrospective case series","volume":"36","author":"Wibbeler E","year":"2021","unstructured":"Wibbeler E, Wang R, Reyes E,  Cerliponase alfa for the treatment of atypical phenotypes of CLN2 disease: a retrospective case series. J Child Neurol. 2021;36(6):468\u2013474."},{"key":"b11","series-title":"Eur J Paediatr Neurol","first-page":"17","article-title":"Cerliponase alfa changes the natural history of children with neuronal ceroid lipofuscinosis type 2: the first French cohort","volume":"30","author":"Estublier B","year":"2021","unstructured":"Estublier B, Cano A, Hoebeke C,  Cerliponase alfa changes the natural history of children with neuronal ceroid lipofuscinosis type 2: the first French cohort. Eur J Paediatr Neurol. 2021;30:17\u201321."},{"issue":"20","key":"b12","series-title":"N Engl J Med","first-page":"1898","article-title":"Study of intraventricular cerliponase alfa for CLN2 disease","volume":"378","author":"Schulz A","year":"2018","unstructured":"Schulz A, Ajayi T, Specchio N,  Study of intraventricular cerliponase alfa for CLN2 disease. N Engl J Med. 2018;378(20):1898\u20131907."},{"issue":"1","key":"b13","series-title":"J Neurosurg Pediatr","first-page":"115","article-title":"A survival analysis of ventricular access devices for delivery of cerliponase alfa","volume":"29","author":"Craven CL","year":"2022","unstructured":"Craven CL, Gissen P, Bower R, Lee L, Aquilina K, Thompson DNP. A survival analysis of ventricular access devices for delivery of cerliponase alfa. 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Clin Transl Sci. 2021;14(5):1810\u20131821."},{"issue":"2","key":"b15","series-title":"Clin Transl Sci","first-page":"635","article-title":"Clinical pharmacokinetics and pharmacodynamics of cerliponase alfa, enzyme replacement therapy for cln2 disease by intracerebroventricular administration","volume":"14","author":"Kim A","year":"2021","unstructured":"Kim A, Grover A, Hammon K,  Clinical pharmacokinetics and pharmacodynamics of cerliponase alfa, enzyme replacement therapy for cln2 disease by intracerebroventricular administration. Clin Transl Sci. 2021;14(2):635\u2013644."},{"issue":"12","key":"b16","series-title":"Ann Clin Transl Neurol","first-page":"2437","article-title":"Neurofilament light is a treatment-responsive biomarker in CLN2 disease","volume":"6","author":"Ru Y","year":"2019","unstructured":"Ru Y, Corado C, Soon RK,  Neurofilament light is a treatment-responsive biomarker in CLN2 disease. Ann Clin Transl Neurol. 2019;6(12):2437\u20132447."},{"key":"b17","series-title":"Clin Immunol","first-page":"68","article-title":"Immunogenicity to cerliponase alfa intracerebroventricular enzyme replacement therapy for CLN2 disease: results from a phase 1\/2 study","volume":"197","author":"Cherukuri A","year":"2018","unstructured":"Cherukuri A, Cahan H, de Hart G,  Immunogenicity to cerliponase alfa intracerebroventricular enzyme replacement therapy for CLN2 disease: results from a phase 1\/2 study. 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