Progressive multifocal leukoencephalopathy in common variable immunodeficiency: mitigated course under mirtazapine and mefloquine.

Kurmann, Rebekka; Weisstanner, Christian; Kardas, Piotr; Hirsch, Hans H; Wiest, Roland; Lämmle, Bernhard; Furrer, Hansjakob; Du Pasquier, Renaud; Bassetti, Claudio; Sturzenegger, Matthias; Krestel, Heinz Eric (2015). Progressive multifocal leukoencephalopathy in common variable immunodeficiency: mitigated course under mirtazapine and mefloquine. Journal of neurovirology, 21(6), pp. 694-701. Springer 10.1007/s13365-015-0340-4

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Demonstration of survival and outcome of progressive multifocal leukoencephalopathy (PML) in a 56-year-old patient with common variable immunodeficiency, consisting of severe hypogammaglobulinemia and CD4+ T lymphocytopenia, during continuous treatment with mirtazapine (30 mg/day) and mefloquine (250 mg/week) over 23 months. Regular clinical examinations including Rankin scale and Barthel index, nine-hole peg and box and block tests, Berg balance, 10-m walking tests, and Montreal Cognitive Assessment (MoCA) were done. Laboratory diagnostics included complete blood count and JC virus (JCV) concentration in cerebrospinal fluid (CSF). The noncoding control region (NCCR) of JCV, important for neurotropism and neurovirulence, was sequenced. Repetitive MRI investigated the course of brain lesions. JCV was detected in increasing concentrations (peak 2568 copies/ml CSF), and its NCCR was genetically rearranged. Under treatment, the rearrangement changed toward the archetype sequence, and later JCV DNA became undetectable. Total brain lesion volume decreased (8.54 to 3.97 cm(3)) and atrophy increased. Barthel (60 to 100 to 80 points) and Rankin (4 to 2 to 3) scores, gait stability, and box and block (7, 35, 25 pieces) and nine-hole peg (300, 50, 300 s) test performances first improved but subsequently worsened. Cognition and walking speed remained stable. Despite initial rapid deterioration, the patient survived under continuous treatment with mirtazapine and mefloquine even though he belongs to a PML subgroup that is usually fatal within a few months. This course was paralleled by JCV clones with presumably lower replication capability before JCV became undetectable. Neurological deficits were due to PML lesions and progressive brain atrophy.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology
04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Infectiology
04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic and Interventional Neuroradiology

UniBE Contributor:

Kurmann, Rebekka; Weisstanner, Christian; Wiest, Roland; Lämmle, Bernhard; Furrer, Hansjakob; Bassetti, Claudio; Sturzenegger, Matthias and Krestel, Heinz Eric

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1538-2443

Publisher:

Springer

Language:

English

Submitter:

Annelies Luginbühl

Date Deposited:

28 May 2015 07:59

Last Modified:

31 Oct 2015 01:30

Publisher DOI:

10.1007/s13365-015-0340-4

PubMed ID:

25916731

BORIS DOI:

10.7892/boris.68972

URI:

https://boris.unibe.ch/id/eprint/68972

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