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Published in: Journal of Medical Case Reports 1/2021

Open Access 01-12-2021 | Magnetic Resonance Imaging | Case report

Uncertainty of leukoencephalopathies: a case report

Authors: Mohamed A. Taha, T. Scott Diesing

Published in: Journal of Medical Case Reports | Issue 1/2021

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Abstract

Background

Adult-onset leukoencephalopathies are a group of heterogeneous disorders characterized by white matter abnormalities. Leukoencephalopathy is usually encountered in children, but here we report a case with adult-onset leukoencephalopathy. Also, we explore this concept of uncertainty in medicine by discussing the approach to this case that has multiple possible etiologies.

Case presentation

A 70-year-old Caucasian male presented with a subacute onset of cognitive impairment and mood disturbances associated with behavioral changes. Neuroimaging demonstrated high-intensity lesions involving cerebral white matter. Progressive dementia and cognitive decline followed. Multiple possible etiologies are discussed based on the patient presentation and risk factors.

Conclusion

Adult-onset leukoencephalopathy can become a diagnostic challenge. Certain approaches need to be developed to explore the uncertainty of such conditions and to improve diagnostic yield.
Literature
1.
go back to reference Koralnik IJ. Progressive multifocal leukoencephalopathy revisited: has the disease outgrown its name? Ann Neurol. 2006;60(2):162–73.CrossRef Koralnik IJ. Progressive multifocal leukoencephalopathy revisited: has the disease outgrown its name? Ann Neurol. 2006;60(2):162–73.CrossRef
3.
go back to reference Marzocchetti A, Di Giambenedetto S, Cingolani A, Ammassari A, Cauda R, De Luca A. Reduced rate of diagnostic positive detection of JC virus DNA in cerebrospinal fluid in cases of suspected progressive multifocal leukoencephalopathy in the era of potent antiretroviral therapy. J Clin Microbiol. 2005;43(8):4175–7.CrossRef Marzocchetti A, Di Giambenedetto S, Cingolani A, Ammassari A, Cauda R, De Luca A. Reduced rate of diagnostic positive detection of JC virus DNA in cerebrospinal fluid in cases of suspected progressive multifocal leukoencephalopathy in the era of potent antiretroviral therapy. J Clin Microbiol. 2005;43(8):4175–7.CrossRef
4.
go back to reference Rabinovici GD, Wang PN, Levin J, Cook L, Pravdin M, Davis J, DeArmond SJ, Barbaro NM, Martindale J, Miller BL, Geschwind MD. First symptom in sporadic Creutzfeldt-Jakob disease. Neurology. 2006;66(2):286–7.CrossRef Rabinovici GD, Wang PN, Levin J, Cook L, Pravdin M, Davis J, DeArmond SJ, Barbaro NM, Martindale J, Miller BL, Geschwind MD. First symptom in sporadic Creutzfeldt-Jakob disease. Neurology. 2006;66(2):286–7.CrossRef
5.
go back to reference Collie DA, Sellar RJ, Zeidler M, Colchester AC, Knight R, Will RG. MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol. Clin Radiol. 2001;56(9):726–39.CrossRef Collie DA, Sellar RJ, Zeidler M, Colchester AC, Knight R, Will RG. MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol. Clin Radiol. 2001;56(9):726–39.CrossRef
6.
go back to reference Steinhoff BJ, Zerr I, Glatting M, Schulz-Schaeffer W, Poser S, Kretzschmar HA. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol . 2004;56(5):702–8.CrossRef Steinhoff BJ, Zerr I, Glatting M, Schulz-Schaeffer W, Poser S, Kretzschmar HA. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol . 2004;56(5):702–8.CrossRef
7.
go back to reference Foutz A, Appleby BS, Hamlin C, Liu X, Yang S, Cohen Y, Chen W, Blevins J, Fausett C, Wang H, Gambetti P. Diagnostic and prognostic value of human prion detection in cerebrospinal fluid. Ann Neurol. 2017;81(1):79–92.CrossRef Foutz A, Appleby BS, Hamlin C, Liu X, Yang S, Cohen Y, Chen W, Blevins J, Fausett C, Wang H, Gambetti P. Diagnostic and prognostic value of human prion detection in cerebrospinal fluid. Ann Neurol. 2017;81(1):79–92.CrossRef
8.
go back to reference Muayqil T, Gronseth G, Camicioli R. Evidence-based guideline: diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology. 2012;79(14):1499–506.CrossRef Muayqil T, Gronseth G, Camicioli R. Evidence-based guideline: diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology. 2012;79(14):1499–506.CrossRef
9.
go back to reference Lynch DS, de Rodrigues Brandão Paiva A, Zhang WJ, Bugiardini E, Freua F, Tavares Lucato L, Macedo-Souza LI, Lakshmanan R, Kinsella JA, Merwick A, Rossor AM. Clinical and genetic characterization of leukoencephalopathies in adults. Brain. 2017;140(5):1204–11.CrossRef Lynch DS, de Rodrigues Brandão Paiva A, Zhang WJ, Bugiardini E, Freua F, Tavares Lucato L, Macedo-Souza LI, Lakshmanan R, Kinsella JA, Merwick A, Rossor AM. Clinical and genetic characterization of leukoencephalopathies in adults. Brain. 2017;140(5):1204–11.CrossRef
10.
go back to reference Baumann N, Turpin JC. Adult-onset leukodystrophies. J Neurol. 2000;247(10):751–9.CrossRef Baumann N, Turpin JC. Adult-onset leukodystrophies. J Neurol. 2000;247(10):751–9.CrossRef
11.
go back to reference Kotil K, Kalayci M, Köseoglu T, Tugrul A. Myelinoclastic diffuse sclerosis (Schilder’s disease): report of a case and review of the literature. Br J Neurosurg. 2002;16(5):516–9.CrossRef Kotil K, Kalayci M, Köseoglu T, Tugrul A. Myelinoclastic diffuse sclerosis (Schilder’s disease): report of a case and review of the literature. Br J Neurosurg. 2002;16(5):516–9.CrossRef
12.
go back to reference Kayser MS, Titulaer MJ, Gresa-Arribas N, Dalmau J. Frequency and characteristics of isolated psychiatric episodes in anti-N-methyl-d-aspartate receptor encephalitis. JAMA Neurol. 2013;70(9):1133–9.CrossRef Kayser MS, Titulaer MJ, Gresa-Arribas N, Dalmau J. Frequency and characteristics of isolated psychiatric episodes in anti-N-methyl-d-aspartate receptor encephalitis. JAMA Neurol. 2013;70(9):1133–9.CrossRef
13.
go back to reference Bataille B, Delwail V, Menet E, Vandermarcq P, Ingrand P, Wager M, Guy G, Lapierre F. Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 2000;92(2):261–6.CrossRef Bataille B, Delwail V, Menet E, Vandermarcq P, Ingrand P, Wager M, Guy G, Lapierre F. Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 2000;92(2):261–6.CrossRef
14.
go back to reference Bühring U, Herrlinger U, Krings T, Thiex R, Weller M, Küker W. MRI features of primary central nervous system lymphomas at presentation. Neurology. 2001;57(3):393–6.CrossRef Bühring U, Herrlinger U, Krings T, Thiex R, Weller M, Küker W. MRI features of primary central nervous system lymphomas at presentation. Neurology. 2001;57(3):393–6.CrossRef
15.
go back to reference Boogerd W, vd Sande JJ, Moffie D. Acute fever and delayed leukoencephalopathy following low dose intraventricular methotrexate. J Neurol Neurosurg Psychiatry. 1988;51(10):1277–83.CrossRef Boogerd W, vd Sande JJ, Moffie D. Acute fever and delayed leukoencephalopathy following low dose intraventricular methotrexate. J Neurol Neurosurg Psychiatry. 1988;51(10):1277–83.CrossRef
16.
go back to reference Kim JY, Kim ST, Nam DH, Lee JI, Park K, Kong DS. Leukoencephalopathy and disseminated necrotizing leukoencephalopathy following intrathecal methotrexate chemotherapy and radiation therapy for central nerve system lymphoma or leukemia. J Korean Neurosurg Soc . 2011;50(4):304.CrossRef Kim JY, Kim ST, Nam DH, Lee JI, Park K, Kong DS. Leukoencephalopathy and disseminated necrotizing leukoencephalopathy following intrathecal methotrexate chemotherapy and radiation therapy for central nerve system lymphoma or leukemia. J Korean Neurosurg Soc . 2011;50(4):304.CrossRef
17.
go back to reference Raghavendra S, Nair MD, Chemmanam T, Krishnamoorthy T, Radhakrishnan VV, Kuruvilla A. Disseminated necrotizing leukoencephalopathy following low-dose oral methotrexate. Eur J Neurol. 2007;14(3):309–14.CrossRef Raghavendra S, Nair MD, Chemmanam T, Krishnamoorthy T, Radhakrishnan VV, Kuruvilla A. Disseminated necrotizing leukoencephalopathy following low-dose oral methotrexate. Eur J Neurol. 2007;14(3):309–14.CrossRef
18.
go back to reference Klir GJ, Smith RM. On measuring uncertainty and uncertainty-based information: recent developments. Ann Math Artif Intell. 2001;32(1–4):5–33.CrossRef Klir GJ, Smith RM. On measuring uncertainty and uncertainty-based information: recent developments. Ann Math Artif Intell. 2001;32(1–4):5–33.CrossRef
Metadata
Title
Uncertainty of leukoencephalopathies: a case report
Authors
Mohamed A. Taha
T. Scott Diesing
Publication date
01-12-2021
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2021
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-021-03089-6

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