Case reportEfficacy of mefloquine to progressive multifocal leukoencephalopathy initially presented with parkinsonism
Introduction
Progressive multifocal leukoencephalopathy (PML) is a rare CNS disease usually occurring in immunocompromised host, such as AIDS, hematological malignancy, autoimmune disease, and patients with multiple sclerosis treated by natalizumab [1]. Some few cases were reported in immunocompetent hosts [2]. In AIDS related PML, long term survival without real neurological improvement has been reported in patients treated with highly active antiretroviral therapy (HAART). Few cases of improvement with cidofovir or cytosine arabinoside have been described in AIDS related or non-AIDS related PML, but in larger trials in AIDS related PML, no clinical benefit was found. Recently, it was reported that anti-malaria drug, mefloquine was effective for inhibiting the replication of JC virus in experimental conditions [3]. We have tried to treat the patient with PML without AIDS, and found its efficacy. It stopped the progression of the disease and resulted in partial improvement of neurological signs as well as MRI findings.
Another rare feature of the patient was initial presenting symptom was parkinsonism [4], though patients with PML may show pathological involvement of the basal ganglia at autopsy.
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Case report
A 60-year-old man presented with 4-month history of tremor and clumsiness affecting the left hand and was diagnosed as hemiparkinsonism. He developed mild left hemiparesis which developed 2 weeks before hospitalization. Previous history disclosed bilateral hilar lymphoadenopathy 7 years ago, and sarcoidosis was suspected without treatment. At admission, he was alert. Cranial nerves were intact. There was mild left hemiparesis with moderate hyperreflexia without Babinski sign. Mild
Discussion
Parkinsonism as an initial manifestation of PML is rarely documented [4]. The pathological mechanism underlying parkinsonism is due to the lesion of basal ganglia as well as the lesion of white matter of cerebral cortex, indicating the disturbance of corticostriatal pathway. The involvement of corticostriatal pathway without direct involvement of basal ganglia and midbrain is conceivable in our case.
Occurrence of PML in immunocompetent patient is rarely reported. The underlying disease might be
Conclusion
A patient who initially presented signs with unilateral parkinsonism had PML diagnosed by brain biopsy. He was treated by anti-malaria drug of mefloquine, which stopped the progression of the disease and resulted in partial improvement of the neurological signs as well as MRI findings. Further study should be undertaken, and a randomized trial in necessary before giving recommendation to evaluate the efficacy of mefloquine for the treatment of PML.
Conflicts of interest
The authors of this manuscript report no disclosures or conflicts of interest.
Acknowledgements
We thank Dr. Shuji Kishida, Division of Neurology, Tokyo Metropolitan Cancer and Infectious Disease Center for helpful suggestions and Dr. Michio Nakamichi at Department of Virology 1, National Institute of Infectious Diseases for assaying the PCR of JC virus DNA in CSF.
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