Natalizumab (NAT)-treated relapsing remitting multiple sclerosis (RRMS) patients have a marked progressive multifocal leukoencephalopathy (PML) risk, with an overall incidence of 3.4 per 1000 NAT treated patients (95%− CI 3.08 to 3.74).1 In HIV-positive PML patients a supratentorial manifestation occurred in 93.8%. An infratentorial PML manifestation is less frequent, occurring in 11–58.3% of the HIV-positive PML patients.2 ,3 Within the infratentorial brain structures, the pons is most commonly affected.2 Irie et al reported a infratentorial PML manifestation in one patient with renal failure. They found an overall survival of ≤4 months after onset of infratentorial PML symptoms.4 Up to now no clinical studies regarding NAT-PML with a predominant infratentorial manifestation have been published. Our study aimed to investigate differences in clinical data and disease course between NAT-PML patients with or without initial infratentorial involvement.
Methods
NAT-PML patients treated in two German university hospitals (Bochum n=21, Hannover n=3,) until 1 November 2013 were included in this retrospective study (ethic committees: Bochum 4566–13, Hannover: 2100−2013). Treatment of NAT-PML was standardised including plasma exchange, mirtazapine and mefloquine treatment. Patients were followed for 14.6±12.4 months (mean±SD), basic characteristics and clinical data were collected by medical records. Extended Disability Status Scale (EDSS) and Karnofsky Index (KI) were performed at onset, after the immune reconstitution inflammatory syndrome (IRIS), and after 6 months post-NAT-PML diagnosis, 1,5-Tesla MRI scans were obtained. Compared with our preceding work,5 we focused on the speciality of infratentorial PML manifestations and included a larger patient population. Statistical analysis was performed using SPSS …
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Note: Materials may be edited for content and length
