Progressive multifocal leukoencephalopathy in a patient with systemic sclerosis treated with methotrexate: A case report and literature review

2020 ◽  
Vol 5 (3) ◽  
pp. NP1-NP6 ◽  
Author(s):  
Theodora Simopoulou ◽  
Vana Tsimourtou ◽  
Christina Katsiari ◽  
Marianna Vlychou ◽  
Dimitrios P Bogdanos ◽  
...  
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Jc Virus ◽  
Clinical Manifestations ◽  
Immunosuppressive Drugs ◽  
Daily Activities ◽  
Neurological Deficits ◽  
Slow Recovery ◽  
Immune Mediated

Reactivation of viruses occurs in autoimmune disorders in the setting of certain immunosuppressive drugs. We describe a 54-year-old female with systemic sclerosis and extensive cutaneous calcinosis who had been treated with methotrexate for 18 months and presented with headache and neurological deficits. She was diagnosed with progressive multifocal leukoencephalopathy, a rare disease caused by JC virus. Methotrexate was discontinued and mirtazapine plus mefloquine were added. The patient showed a slow recovery and five years later she had complete resolution of progressive multifocal leukoencephalopathy clinical manifestations. Calcinosis had a limited response to various agents and severely affected daily activities of the patient. This case report, highlights the importance of clinical suspicion for progressive multifocal leukoencephalopathy in every patient with immune-mediated disease, even on weak immunosuppressant, who presents with central nervous system manifestations and also the unmet therapeutic need for systemic sclerosis-associated calcinosis.

Favorable outcome of progressive multifocal leukoencephalopathy in an HIV-infected patient

2021 ◽  
Vol 19 (1) ◽  
pp. 131-134
Author(s):  
E.A. Samotolkina ◽  
◽  
A.V. Pokrovskaya ◽  
E.S. Samotolkina ◽  
D.M. Popova ◽  
...  
Keyword(s):  
Case Report ◽  
Antiretroviral Therapy ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Hiv Infection ◽  
Key Words ◽  
Infected Patient ◽  
Clinical Manifestations ◽  
Favorable Outcome ◽  
Key Words Hiv ◽  
Key Words Hiv Infection

In this article, we report a case of progressive multifocal leukoencephalopathy in an HIV-infected patient receiving antiretroviral therapy. The case is interesting because of its favorable outcome despite severe clinical manifestations of the disease. Key words: HIV infection, case report, progressive multifocal leukoencephalopathy

scholarly journals Progressive multifocal leukoencephalopathy in an HIV patient was diagnosed by 3 times lumbar punctures and 2 times brain biopsies

2020 ◽  
Vol 26 (6) ◽  
pp. 952-956
Author(s):  
Mengyan Wang ◽  
Zhongdong Zhang ◽  
Jinchuan Shi ◽  
Hong Liu ◽  
Binhai Zhang ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
Hiv Positive ◽  
Negative Results ◽  
The Central Nervous System ◽  
Rule Out

AbstractProgressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by JC virus (JCV) and is difficult to diagnose. We report on a male HIV-positive patient with PML finally diagnosed by 3 times lumbar punctures and 2 times brain biopsies. Negative results of JCV-PCR in cerebrospinal fluid (CSF) do not rule out the diagnosis of PML when clinical manifestations and neuroimaging features suspected PML. It is necessary to obtain new CSF and make repeat tests and even perform brain biopsy.

scholarly journals Posterior reversible encephalopathy syndrome (PRES), an acute neurological syndrome due to reversible multifactorial brain edema: a case report

2013 ◽  
pp. 37-42
Author(s):  
Camilla Cicognani ◽  
Chiara Vezzadini ◽  
Stella Battaglia ◽  
Anna F. Marliani ◽  
Roberto Zoni
Keyword(s):  
Case Report ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Clinical History ◽  
Immunosuppressive Drugs ◽  
Hypertensive Encephalopathy ◽  
Neurological Deficits ◽  
Posterior Reversible Encephalopathy ◽  
Missed Diagnosis ◽  
Reversible Encephalopathy ◽  
Clinical Conditions

Background: The essential features of Posterior Reversible Encephalopathy Syndrome (PRES) are headache, mental changes, seizures, visual symptoms and often arterial hypertension. Brain RMN typically shows cortico-sottocortical parieto-occipital edema, with a bilateral and symmetric distribution. PRES develops in clinical conditions as hypertensive encephalopathy, preeclampsia/ eclampsia, autoimmune diseases, after transplantation, infections and as an adverse effect of immunosuppressive drugs or chemotherapy. It usually completely reverses with treatment, although permanent sequelae are possible in case of delayed or missed diagnosis. Case report: We describe the case of a transsexual (M!F) and tetraplegic patient, admitted for neck and low back pain. She suddenly developed headache, confusion, seizures and severe hypertension with normal blood tests. RMN showed multiple cortico-sottocortical areas of vasogenic and citotoxic edema in temporo-occipital, parietal, frontal, and cerebellar regions. Soon after the beginning of the antihypertensive therapy, clinical recovery was observed, as well as the disappearance of edema at RMN. Discussion and conclusions: Although PRES is usually associated with definite pathological conditions, it is not always the case, as was for the patient here described, who had no predisposing factors in her past clinical history, and presented hypertension only in the acute phase of the syndrome. Since, moreover, PRES usually presents with acute non specific features and it can be misdiagnosed with other serious diseases, the clinician will be helped by the knowledge of this syndrome to promptly start diagnostic workup and treatments, and avoid permanent neurological deficits.

scholarly journals Relapsing polychondritis presenting 2 years after systemic sclerosis with pulmonary arterial hypertension

2021 ◽  
Vol 2 (2) ◽  
pp. 121-123
Author(s):  
Li Su ◽  
Na Zhang ◽  
Hui Wang ◽  
Zhenwen Yang ◽  
Wei Wei
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Relapsing Polychondritis ◽  
Immunosuppressive Drugs ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Immune Mediated ◽  
Endothelin A Receptor ◽  
Pulmonary Arterial ◽  
Phosphodiesterase Type

Abstract Relapsing polychondritis (RPC) is a systemic immune-mediated disease characterized by recurrent and progressive inflammation of cartilaginous tissues. 64% of RPC patients concurrent with other autoimmune disorders, there are very few reports about the concomitant RPC patients with systemic sclerosis (SSc). Herein we report a case of RPC in a 50-year-old female following SSc with pulmonary arterial hypertension (PAH) 2 years ago. She was treated with corticosteroids, immunosuppressive drugs, oral endothelin-A receptor antagonist and phosphodiesterase type 5 inhibitors. Her ocular and auricular symptoms disappeared quickly. The hemodynamic parameters were also significantly improved after treatment. To our knowledge, this is the first RPC complicated with SSc-PAH reported.

scholarly journals JC Virus Granule Cell Neuronopathy as AIDS-Presenting Illness

2018 ◽  
Vol 45 (4) ◽  
pp. 466-469 ◽  
Author(s):  
Simon Grandjean Lapierre ◽  
Xin Dang ◽  
Danielle Gilbert ◽  
Sylvie Lauzier ◽  
Igor J. Koralnik ◽  
...  
Keyword(s):  
Human Immunodeficiency Virus ◽  
Virus Infection ◽  
Human Immunodeficiency Virus Infection ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Granule Cell ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Clinical Manifestations ◽  
Immunodeficiency Virus ◽  
Granule Cell Neuronopathy

AbstractJC virus is the etiological agent of progressive multifocal leukoencephalopathy, a white matter demyelinating disease that mostly affects immunocompromised patients. JC virus can also infect neurons and meningeal cells and cause encephalitis, meningitis and granule cell neuronopathy. We report a patient with JC virus granule cell neuronopathy, without concomitant progressive multifocal leukoencephalopathy, presenting as inaugural acquired immune deficiency syndrome-related illness. This patient’s human immunodeficiency virus infection remained undiagnosed for several months after neurological symptoms onset. We review JC virus pathophysiology, clinical manifestations, treatment and prognosis, and emphasize the importance of considering human immunodeficiency virus infection and related opportunistic infections in the differential diagnosis of new-onset isolated cerebellar disease.

scholarly journals Rituximab-Associated Inflammatory Progressive Multifocal Leukoencephalopathy

2016 ◽  
Vol 2016 ◽  
pp. 1-2 ◽  
Author(s):  
Chandra Punch ◽  
Christina Schofield ◽  
Penelope Harris
Keyword(s):  
Case Report ◽  
Immune System ◽  
Lower Extremity ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Poor Prognosis ◽  
Immune Reconstitution ◽  
Jc Virus ◽  
The Other ◽  
Inflammatory Syndrome ◽  
Hiv Negative

Progressive multifocal leukoencephalopathy (PML) is a rare disease of the immunosuppression that results from neurotropic invasion of the JC virus which leads to demyelination of oligodendrocytes. Immune reconstitution inflammatory syndrome (IRIS), on the other hand, is a condition of inflammation that develops as the immune system reconstitutes. This case report describes a case of a 35-year-old HIV-negative male who presented with three weeks of right lower extremity paresthesias as well as right upper extremity apraxia. He was diagnosed with PML complicated by IRIS secondary to Rituximab, which he had completed four months prior to presentation. Despite the condition’s poor prognosis, the patient recovered with only minor deficits.

scholarly journals Systemic Sclerosis: From Pathophysiology to Novel Therapeutic Approaches

Biomedicines ◽  
2022 ◽  
Vol 10 (1) ◽  
pp. 163
Author(s):  
Devis Benfaremo ◽  
Silvia Svegliati ◽  
Chiara Paolini ◽  
Silvia Agarbati ◽  
Gianluca Moroncini
Keyword(s):  
Immune System ◽  
Systemic Sclerosis ◽  
Plasma Cells ◽  
Clinical Manifestations ◽  
Therapeutic Targets ◽  
Tissue Level ◽  
Internal Organs ◽  
Immune Mediated ◽  
Chronic Disorder ◽  
Substantial Progress

Systemic sclerosis (SSc) is a systemic, immune-mediated chronic disorder characterized by small vessel alterations and progressive fibrosis of the skin and internal organs. The combination of a predisposing genetic background and triggering factors that causes a persistent activation of immune system at microvascular and tissue level is thought to be the pathogenetic driver of SSc. Endothelial alterations with subsequent myofibroblast activation, excessive extracellular matrix (ECM) deposition, and unrestrained tissue fibrosis are the pathogenetic steps responsible for the clinical manifestations of this disease, which can be highly heterogeneous according to the different entity of each pathogenic step in individual subjects. Although substantial progress has been made in the management of SSc in recent years, disease-modifying therapies are still lacking. Several molecular pathways involved in SSc pathogenesis are currently under evaluation as possible therapeutic targets in clinical trials. These include drugs targeting fibrotic and metabolic pathways (e.g., TGF-β, autotaxin/LPA, melanocortin, and mTOR), as well as molecules and cells involved in the persistent activation of the immune system (e.g., IL4/IL13, IL23, JAK/STAT, B cells, and plasma cells). In this review, we provide an overview of the most promising therapeutic targets that could improve the future clinical management of SSc.

scholarly journals A case report of simultaneous PML-IRIS during corticosteroids tapering in a patient with an anti-synthetase syndrome

F1000Research ◽  
2013 ◽  
Vol 2 ◽  
pp. 283 ◽  
Author(s):  
Guillaume Martin-Blondel ◽  
David Brassat ◽  
Hervé Dumas ◽  
Emmanuelle Uro-Coste ◽  
Daniel Adoue ◽  
...  
Keyword(s):  
Case Report ◽  
Viral Load ◽  
Rheumatic Disease ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Immune Reconstitution Inflammatory Syndrome ◽  
Immune Reconstitution ◽  
Jc Virus ◽  
Immunocompromised Patient ◽  
Pcr Assay ◽  
Inflammatory Syndrome

We report a case of simultaneous progressive multifocal leukoencephalopathy-associated immune reconstitution inflammatory syndrome (PML-IRIS) during corticosteroid tapering in a patient with an anti-synthetase syndrome. We describe the challenges associated with the diagnosis and the management of this emerging inflammatory neurological condition in this immunocompromised patient with a severe rheumatic disease. We highlight that, in the setting of IRIS, the low-level of the JC virus viral load requires a sensitive PCR assay before excluding PML.

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