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Elevated CSF neopterin level

MedGen UID:
893002
•Concept ID:
C4073152
•
Finding
HPO: HP:0040204

Definition

Increased concentration of neopterin in the cerebrospinal fluid (CSF). [from HPO]

Conditions with this feature

Primary immunodeficiency with post-measles-mumps-rubella vaccine viral infection
MedGen UID:
904009
•Concept ID:
C4225260
•
Disease or Syndrome
Immunodeficiency-44 (IMD44) is an autosomal recessive primary immunodeficiency characterized by increased susceptibility to viral infections and adverse multisystemic reaction to vaccination in some patients. Affected individuals appear to have defects in mitochondrial fission and fusion (summary by Shahni et al., 2015).
See: Condition Record
Primary immunodeficiency with post-measles-mumps-rubella vaccine viral infection

Professional guidelines

PubMed

Cerebrospinal fluid neopterin as a biomarker of treatment response to Janus kinase inhibition in Aicardi-Goutières syndrome.
Han VX, Mohammad SS, Jones HF, Bandodkar S, Crow YJ, Dale RC; AGS-JAKi Study Group
Dev Med Child Neurol 2022 Feb;64(2):266-271. Epub 2021 Aug 20 doi: 10.1111/dmcn.15025. PMID: 34415581
Antiretroviral treatment reduces increased CSF neurofilament protein (NFL) in HIV-1 infection.
Mellgren A, Price RW, Hagberg L, Rosengren L, Brew BJ, Gisslén M
Neurology 2007 Oct 9;69(15):1536-41. doi: 10.1212/01.wnl.0000277635.05973.55. PMID: 17923616
Clinical and immunologic follow-up study of patients with neurocysticercosis after treatment with praziquantel.
Rolfs A, Mühlschlegel F, Jansen-Rosseck R, Martins AR, Bedaque EA, Tamburus WM, Pedretti L, Schulte G, Feldmeier H, Kremsner P
Neurology 1995 Mar;45(3 Pt 1):532-8. doi: 10.1212/wnl.45.3.532. PMID: 7898711

Recent clinical studies

Etiology

Long-term safety and efficacy of mogamulizumab (anti-CCR4) for treating virus-associated myelopathy.
Sato T, Yamauchi J, Yagishita N, Araya N, Takao N, Ohta Y, Inoue E, Takahashi M, Yamagishi M, Suzuki Y, Uchimaru K, Matsumoto N, Hasegawa Y, Yamano Y
Brain 2023 Aug 1;146(8):3181-3191. doi: 10.1093/brain/awad139. PMID: 37093965
Cerebrospinal fluid biomarkers in SARS-CoV-2 patients with acute neurological syndromes.
Chaumont H, Kaczorowski F, San-Galli A, Michel PP, Tressières B, Roze E, Quadrio I, Lannuzel A
Rev Neurol (Paris) 2023 Mar;179(3):208-217. Epub 2022 Nov 30 doi: 10.1016/j.neurol.2022.11.002. PMID: 36610823Free PMC Article
Neurofilament Light in CSF and Plasma Is a Marker of Neuronal Damage in HTLV-1-Associated Myelopathy and Correlates With Neuroinflammation.
Rosadas C, Zetterberg H, Heslegrave A, Haddow J, Borisova M, Taylor GP
Neurol Neuroimmunol Neuroinflamm 2021 Nov;8(6) Epub 2021 Oct 5 doi: 10.1212/NXI.0000000000001090. PMID: 34611039Free PMC Article
Cerebrospinal Fluid Pterins, Pterin-Dependent Neurotransmitters, and Mortality in Pediatric Cerebral Malaria.
Rubach MP, Mukemba JP, Florence SM, Lopansri BK, Hyland K, Simmons RA, Langelier C, Nakielny S, DeRisi JL, Yeo TW, Anstey NM, Weinberg JB, Mwaikambo ED, Granger DL
J Infect Dis 2021 Oct 28;224(8):1432-1441. doi: 10.1093/infdis/jiab086. PMID: 33617646Free PMC Article
Cerebrospinal Fluid Neopterin in Children With Enterovirus-Related Brainstem Encephalitis.
Casas-Alba D, Valero-Rello A, Muchart J, Armangué T, Jordan I, Cabrerizo M, Molero-Luís M, Artuch R, Fortuny C, Muñoz-Almagro C, Launes C
Pediatr Neurol 2019 Jul;96:70-73. Epub 2019 Feb 7 doi: 10.1016/j.pediatrneurol.2019.01.024. PMID: 30935719

Diagnosis

CSF neopterin and beta-2-microglobulin as inflammation biomarkers in newborns with hypoxic-ischemic encephalopathy.
Carreras N, Arnaez J, Valls A, Agut T, Sierra C, Garcia-Alix A
Pediatr Res 2023 Apr;93(5):1328-1335. Epub 2022 Apr 6 doi: 10.1038/s41390-022-02011-0. PMID: 35388137
Neurofilament Light in CSF and Plasma Is a Marker of Neuronal Damage in HTLV-1-Associated Myelopathy and Correlates With Neuroinflammation.
Rosadas C, Zetterberg H, Heslegrave A, Haddow J, Borisova M, Taylor GP
Neurol Neuroimmunol Neuroinflamm 2021 Nov;8(6) Epub 2021 Oct 5 doi: 10.1212/NXI.0000000000001090. PMID: 34611039Free PMC Article
Chemotherapy-induced autoimmune-mediated encephalitis during germinoma treatment.
Yamada N, Yamasaki K, Yamamoto N, Kuki I, Sakuma H, Hara J
Brain Dev 2021 Oct;43(9):967-971. Epub 2021 Jun 3 doi: 10.1016/j.braindev.2021.05.010. PMID: 34092404
Cerebrospinal Fluid Pterins, Pterin-Dependent Neurotransmitters, and Mortality in Pediatric Cerebral Malaria.
Rubach MP, Mukemba JP, Florence SM, Lopansri BK, Hyland K, Simmons RA, Langelier C, Nakielny S, DeRisi JL, Yeo TW, Anstey NM, Weinberg JB, Mwaikambo ED, Granger DL
J Infect Dis 2021 Oct 28;224(8):1432-1441. doi: 10.1093/infdis/jiab086. PMID: 33617646Free PMC Article
Clinical Use of CSF Neurotransmitters.
Rodan LH, Gibson KM, Pearl PL
Pediatr Neurol 2015 Oct;53(4):277-86. Epub 2015 Jun 11 doi: 10.1016/j.pediatrneurol.2015.04.016. PMID: 26194033

Therapy

Long-term safety and efficacy of mogamulizumab (anti-CCR4) for treating virus-associated myelopathy.
Sato T, Yamauchi J, Yagishita N, Araya N, Takao N, Ohta Y, Inoue E, Takahashi M, Yamagishi M, Suzuki Y, Uchimaru K, Matsumoto N, Hasegawa Y, Yamano Y
Brain 2023 Aug 1;146(8):3181-3191. doi: 10.1093/brain/awad139. PMID: 37093965
One-year-old boy with refractory Listeria monocytogenes meningitis due to persistent hypercytokinemia.
Kaneko S, Hatasaki K, Ueno K, Fujita S, Igarashi N, Kuroda M, Wada T
J Infect Chemother 2022 Dec;28(12):1682-1686. Epub 2022 Sep 5 doi: 10.1016/j.jiac.2022.08.027. PMID: 36067911
Intrathecal dexamethasone therapy for febrile infection-related epilepsy syndrome.
Horino A, Kuki I, Inoue T, Nukui M, Okazaki S, Kawawaki H, Togawa M, Amo K, Ishikawa J, Ujiro A, Shiomi M, Sakuma H
Ann Clin Transl Neurol 2021 Mar;8(3):645-655. Epub 2021 Feb 5 doi: 10.1002/acn3.51308. PMID: 33547757Free PMC Article
Cerebrospinal fluid levels of neopterin are elevated in delirium after hip fracture.
Hall RJ, Watne LO, Idland AV, Raeder J, Frihagen F, MacLullich AM, Staff AC, Wyller TB, Fekkes D
J Neuroinflammation 2016 Jun 29;13(1):170. doi: 10.1186/s12974-016-0636-1. PMID: 27357281Free PMC Article
CXCL13 and neopterin concentrations in cerebrospinal fluid of patients with Lyme neuroborreliosis and other diseases that cause neuroinflammation.
Hytönen J, Kortela E, Waris M, Puustinen J, Salo J, Oksi J
J Neuroinflammation 2014 Jun 11;11:103. doi: 10.1186/1742-2094-11-103. PMID: 24920219Free PMC Article

Prognosis

CSF neopterin and beta-2-microglobulin as inflammation biomarkers in newborns with hypoxic-ischemic encephalopathy.
Carreras N, Arnaez J, Valls A, Agut T, Sierra C, Garcia-Alix A
Pediatr Res 2023 Apr;93(5):1328-1335. Epub 2022 Apr 6 doi: 10.1038/s41390-022-02011-0. PMID: 35388137
Lessons from the Cerebrospinal Fluid Analysis of HTLV-1-Infected Individuals: Biomarkers of Inflammation for HAM/TSP Development.
Freitas NL, Gomes YCP, Souza FDS, Torres RC, Echevarria-Lima J, Leite ACCB, Lima MASD, Araújo AQC, Silva MTT, Espíndola OM
Viruses 2022 Sep 29;14(10) doi: 10.3390/v14102146. PMID: 36298702Free PMC Article
Neurofilament Light in CSF and Plasma Is a Marker of Neuronal Damage in HTLV-1-Associated Myelopathy and Correlates With Neuroinflammation.
Rosadas C, Zetterberg H, Heslegrave A, Haddow J, Borisova M, Taylor GP
Neurol Neuroimmunol Neuroinflamm 2021 Nov;8(6) Epub 2021 Oct 5 doi: 10.1212/NXI.0000000000001090. PMID: 34611039Free PMC Article
Chemotherapy-induced autoimmune-mediated encephalitis during germinoma treatment.
Yamada N, Yamasaki K, Yamamoto N, Kuki I, Sakuma H, Hara J
Brain Dev 2021 Oct;43(9):967-971. Epub 2021 Jun 3 doi: 10.1016/j.braindev.2021.05.010. PMID: 34092404
Intrathecal dexamethasone therapy for febrile infection-related epilepsy syndrome.
Horino A, Kuki I, Inoue T, Nukui M, Okazaki S, Kawawaki H, Togawa M, Amo K, Ishikawa J, Ujiro A, Shiomi M, Sakuma H
Ann Clin Transl Neurol 2021 Mar;8(3):645-655. Epub 2021 Feb 5 doi: 10.1002/acn3.51308. PMID: 33547757Free PMC Article

Clinical prediction guides

Long-term safety and efficacy of mogamulizumab (anti-CCR4) for treating virus-associated myelopathy.
Sato T, Yamauchi J, Yagishita N, Araya N, Takao N, Ohta Y, Inoue E, Takahashi M, Yamagishi M, Suzuki Y, Uchimaru K, Matsumoto N, Hasegawa Y, Yamano Y
Brain 2023 Aug 1;146(8):3181-3191. doi: 10.1093/brain/awad139. PMID: 37093965
CSF neopterin and beta-2-microglobulin as inflammation biomarkers in newborns with hypoxic-ischemic encephalopathy.
Carreras N, Arnaez J, Valls A, Agut T, Sierra C, Garcia-Alix A
Pediatr Res 2023 Apr;93(5):1328-1335. Epub 2022 Apr 6 doi: 10.1038/s41390-022-02011-0. PMID: 35388137
Elevated Cerebrospinal Fluid Anti-CD4 Autoantibody Levels in HIV Associate with Neuroinflammation.
Cheng D, Luo Z, Fu X, Stephenson S, Di Germanio C, Norris PJ, Fuchs D, Ndhlovu LC, Li QZ, Zetterberg H, Gisslen M, Price RW, Peng S, Jiang W
Microbiol Spectr 2022 Feb 23;10(1):e0197521. Epub 2022 Jan 5 doi: 10.1128/spectrum.01975-21. PMID: 34985329Free PMC Article
Cerebrospinal Fluid Pterins, Pterin-Dependent Neurotransmitters, and Mortality in Pediatric Cerebral Malaria.
Rubach MP, Mukemba JP, Florence SM, Lopansri BK, Hyland K, Simmons RA, Langelier C, Nakielny S, DeRisi JL, Yeo TW, Anstey NM, Weinberg JB, Mwaikambo ED, Granger DL
J Infect Dis 2021 Oct 28;224(8):1432-1441. doi: 10.1093/infdis/jiab086. PMID: 33617646Free PMC Article
Significance of CSF total neopterin and biopterin in inflammatory neurological diseases.
Furukawa Y, Nishi K, Kondo T, Tanabe K, Mizuno Y
J Neurol Sci 1992 Aug;111(1):65-72. doi: 10.1016/0022-510x(92)90113-y. PMID: 1402999

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