No abstract available.
Leukoencephalopathy, Progressive Multifocal*

Seizures are less frequent in progressive multifocal leukoencephalopathy (PML), because it is considered to be restricted to the white matter. In addition, types of seizures in patients with PML are mostly convulsive, and seizures are usually present at the time of diagnosis or early in the course of disease. We report a case of chronic PML with recurrent complex partial seizures in the absence of motor component.
Humans ; Leukoencephalopathy, Progressive Multifocal ; Seizures

No abstract available.
Head* ; Humans ; Leukoencephalopathy, Progressive Multifocal* ; Lymphoma, T-Cell* ; Neck*

Posttransplantation lymphoproliferative disease (PTLD) is an important form of posttransplant malignancy and is typically associated with Epstein-Barr virus (EBV). Progressive multifocal leukoencephalopathy (PML) is a demyelination disease caused by infection of the John Cunningham (JC) virus. Both PTLD and PML occur in the setting of an immunosuppressive state. Differentiating PTLD from PML is important because PTLD can be treated by reducing immunosuppressant agents or anti-B-cell antibody therapy. We report a case of EBV-related PTLD in a patient with latent JC virus.
Demyelinating Diseases ; Herpesvirus 4, Human ; Humans ; JC Virus ; Leukoencephalopathy, Progressive Multifocal ; Viruses

Acquired Immunodeficiency Syndrome ; DNA ; Diagnostic Tests, Routine ; Humans ; Leukoencephalopathy, Progressive Multifocal ; Polyomavirus

BACKGROUND/AIMS: Inhibition of α4β7 integrin has been shown to be effective for induction and maintenance therapy in patients with ulcerative colitis (UC). We investigated the effects of varying doses of the α4β7 inhibitor abrilumab in Japanese patients with moderate-to-severe UC despite conventional treatments. METHODS: In this randomized, double-blind, placebo-controlled study, 45 UC patients were randomized to abrilumab 21 mg (n=11), 70 mg (n=12), 210 mg (n=9), or placebo (n=13) via subcutaneous (SC) injection for 12 weeks. The double-blind period was followed by a 36-week open-label period, in which all patients received abrilumab 210 mg SC every 12 weeks, and a 28-week safety follow-up period. The primary efficacy variable was clinical remission at week 8 (total Mayo score ≤2 points with no individual subscore >1 point). RESULTS: Clinical remission at week 8 was 4 out of 31 (12.9%) overall in the abrilumab groups versus 0 out of 13 in the placebo group (abrilumab 21 mg, 1/10 [10.0%]; 70 mg, 2/12 [16.7%]; 210 mg, 1/9 [11.1%]). In both the double-blind and open-label periods, fewer patients in the abrilumab groups experienced ≥1 adverse event compared with those in the placebo group. There were no cases of progressive multifocal leukoencephalopathy and no deaths. CONCLUSIONS: Abrilumab 70 mg and 210 mg yielded numerically better results in terms of clinical remission rate at Week 8 than placebo, with the 210 mg dose showing more consistent treatment effects. Abrilumab was well tolerated in Japanese patients with UC.
Asian Continental Ancestry Group ; Colitis, Ulcerative ; Follow-Up Studies ; Humans ; Japan ; Leukoencephalopathy, Progressive Multifocal ; Ulcer

Progressive multifocal leukoencephalopathy (PML) is a fatal disease that is characterized by progressive demyelination of the cerebral white matter due to JC viral infection. We report serial magnetic resonance spectroscopy (MRS) and tractography changes in a patient with PML. After a three-cycle treatment with cidofovir and cytarabine, the patient exhibited remarkable improvements in motor and cognitive functions. Follow-up MRS and tractography revealed regeneration of the white-matter fibers. This is the first consecutive neuroimaging study showing improvements in neurological symptoms using MRS and tractography.
Cytarabine ; Cytosine ; Demyelinating Diseases ; Follow-Up Studies ; Humans ; Leukoencephalopathy, Progressive Multifocal ; Magnetic Resonance Spectroscopy ; Neuroimaging ; Organophosphonates ; Regeneration

Progressive multifocal leukoencephalopathy (PML) is a devastating neurological disease observed nearly exclusively in immunocompromised patients. Recently, the introduction of monoclonal antibodies significantly inhibiting the immune system such as rituximab has led to an increase in PML cases. Although rituximab-based immunochemotherapy remains the standard of treatment for chronic lymphocytic leukemia (CLL), the importance of Bruton’s tyrosine kinase inhibitors such as ibrutinib is steadily increasing. However, long-term experiences regarding possible side effects of these new substances are rare. Here, we report the development of eventually fatal PML possibly associated with ibrutinib therapy for CLL after multiple prior treatment lines, including rituximab. To the best of our knowledge, this is the first study to report such findings. Since the last course of rituximab was applied over 3 years ago, it is conceivable that the strong B cell inhibition by ibrutinib led to PML. With increased awareness of this potential side effect, further clinical studies are certainly warranted to evaluate this possible association.
Antibodies, Monoclonal ; Immune System ; Immunocompromised Host ; JC Virus ; Leukemia, Lymphocytic, Chronic, B-Cell* ; Leukoencephalopathy, Progressive Multifocal* ; Protein-Tyrosine Kinases ; Rituximab

Progressive multifocal leukoencephalopathy (PML) is a rare and fatal disease caused by JC virus. We report a case of PML which developed in a 61-year-old female patient with myasthenia gravis (MG) and thymoma. After 6 years of immunotherapy and chemotherapy she presented with hand weakness followed by progressive decline of consciousness. Serial brain MRI showed rapidly progressive multifocal white matter changes. The JC virus DNA was detected on cerebrospinal fluid. This is a third report of PML in MG.
Brain ; Consciousness ; DNA ; Female ; Hand ; Humans ; Immunotherapy ; JC Virus ; Leukoencephalopathy, Progressive Multifocal ; Middle Aged ; Myasthenia Gravis ; Thymoma

Progressive multifocal leukoencephalopathy (PML) is a rare disease that occurs mainly in immunocompromised patients. Despite the progressive nature of the disease, the changes on MRI during the disease course - which may help in monitoring the disease process - have seldom been reported. Here we describe a patient with polymerase-chain-reaction-proven PML examined using serial diffusion-weighted imaging (DWI) and apparent-diffusion-coefficient mapping. Magnetic resonance spectroscopy (MRS) revealed that the demyelinating process was more active without significant neuronal loss at the newer and advancing edge of a lesion than in the older central part of the lesion. This case shows that MRI findings such as DWI and MRS may improve the diagnosis and the understanding of the pathophysiology of PML.
Diagnosis ; Diffusion* ; Humans ; Immunocompromised Host ; Leukoencephalopathy, Progressive Multifocal* ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy* ; Neurons ; Protons* ; Rare Diseases