A case of progressive multifocal leukoencephalopathy with chronic renal failure, whose JC virus in cerebrospinal fluid disappeared after mefloquine-mirtazapine dual therapy.

An 83-year-old man with chronic renal failure was referred to our hospital because of subacute progressive right hemiparesis. A brain MRI showed high-intensity lesions in bilateral middle cerebellar peduncles and white matter of the left frontal lobe on T2-weighted images. The lesions increased gradually, so we suspected a brain tumor because 1H-MRS images showed elevated Cho and decreased NAA, and also pathologic findings of the brain biopsy suggested glioblastoma. However, JC virus (JCV) in cerebrospinal fluid was revealed highly positive by PCR. So we reconsidered pathologically and finally found bizarre astrocytes which were infected with JCV in immunohistochemical studies and we diagnosed progressive multifocal leukoencephalopathy at last. Then we medicated with mefloquine and mirtazapine, and the JCV in cerebrospinal fluid disappeared, without new MRI lesions. This is a rare case in respect of the background of the patient and the clinical course.

[Patient with chronic sarcoid myopathy without other organ involvement].

We herein report the patient of a 69-year-old woman who presented with the chronic myopathic form of sarcoid myopathy. She had experienced slowly progressive limb muscle weakness for three years. She was found to be thin, but otherwise normal, on a physical examination. Neurologically, proximal muscles are predominantly involved without any sensory or other focal deficits. Electromyography revealed myopathic motor unit potentials exhibiting spontaneous discharge. Muscle biopsy demonstrated extensive connective tissue and few residual muscle fibers with a hint of granuloma formation. Repeated sectioning of the muscle biopsy revealed noncaseatious granuloma with a multinucleated giant cell, confirming the diagnosis. The findings of all imaging studies, including a systemic PET (positron emission tomography) scan, were unremarkable. Without careful pathological observation with repeated sectioning, this patient would have been misdiagnosed with limb-girdle muscular dystrophy