TY - JOUR
T1 - Performance of PML diagnostic criteria in natalizumab-associated PML
T2 - data from the Dutch-Belgian cohort
AU - Wijburg, Martijn T
AU - Warnke, Clemens
AU - Barkhof, Frederik
AU - Uitdehaag, Bernard M J
AU - Killestein, Joep
AU - Wattjes, Mike P
PY - 2019/1
Y1 - 2019/1
N2 - OBJECTIVE: To test the current progressive multifocal leukoencephalopathy (PML) diagnostic criteria by applying them to patients previously diagnosed with natalizumab (NTZ)-associated PML in a real-world clinical setting.METHODS: Patients from the Dutch-Belgian NTZ-PML cohort (n=28) were reviewed at the time of first diagnostic work-up and during follow-up, using the PML diagnostic criteria as proposed in a consensus statement from the American Academy of Neurology.RESULTS: At first diagnostic work-up, 18 patients (64.3%) met the criteria for high diagnostic certainty for PML ('definite PML' or 'probable PML'). During follow-up, this increased to 20 patients (71.4%) as JC virus DNA was detected in cerebrospinal fluid of two additional patients. Nonetheless, 28.6% of patients were still classified as 'possible PML' or 'not PML' (6 (21.5%) and 2 (7.1%) patients, respectively) despite a very high suspicion for PML based on lesion evolution and signs of PML-immune reconstitution inflammatory syndrome on MRI, and development of compatible symptoms.CONCLUSIONS: The current case definition of PML has low sensitivity for diagnosis of NTZ-PML in a real-world clinical setting in which MRI is frequently used for PML screening. This may delay diagnosis and appropriate management of PML, and may complicate a valid estimation of PML incidence during NTZ therapy.
AB - OBJECTIVE: To test the current progressive multifocal leukoencephalopathy (PML) diagnostic criteria by applying them to patients previously diagnosed with natalizumab (NTZ)-associated PML in a real-world clinical setting.METHODS: Patients from the Dutch-Belgian NTZ-PML cohort (n=28) were reviewed at the time of first diagnostic work-up and during follow-up, using the PML diagnostic criteria as proposed in a consensus statement from the American Academy of Neurology.RESULTS: At first diagnostic work-up, 18 patients (64.3%) met the criteria for high diagnostic certainty for PML ('definite PML' or 'probable PML'). During follow-up, this increased to 20 patients (71.4%) as JC virus DNA was detected in cerebrospinal fluid of two additional patients. Nonetheless, 28.6% of patients were still classified as 'possible PML' or 'not PML' (6 (21.5%) and 2 (7.1%) patients, respectively) despite a very high suspicion for PML based on lesion evolution and signs of PML-immune reconstitution inflammatory syndrome on MRI, and development of compatible symptoms.CONCLUSIONS: The current case definition of PML has low sensitivity for diagnosis of NTZ-PML in a real-world clinical setting in which MRI is frequently used for PML screening. This may delay diagnosis and appropriate management of PML, and may complicate a valid estimation of PML incidence during NTZ therapy.
KW - JC virus
KW - diagnosis
KW - multiple sclerosis
KW - natalizumab
KW - progressive multifocal leukoencephalopathy
UR - http://www.scopus.com/inward/record.url?scp=85058682533&partnerID=8YFLogxK
U2 - https://doi.org/10.1136/jnnp-2018-318261
DO - https://doi.org/10.1136/jnnp-2018-318261
M3 - Article
C2 - 30100552
SN - 0022-3050
VL - 90
SP - 44
EP - 46
JO - Journal of neurology, neurosurgery, and psychiatry
JF - Journal of neurology, neurosurgery, and psychiatry
IS - 1
ER -