Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system (CNS) due to John Cunningham (JC) virus reactivation most often in immunocompromised patients. The brainstem and the anterior corpus callosum are uncommon locations for white matter lesions. We present a case of PML in a 40-year-old female presenting to the emergency department for a tonic seizure with transient postictal confusion. The inpatient workup revealed low cluster of differentiation cell counts (CD3 and CD4), transaminitis, positive drug screen, and abnormal electroencephalogram (EEG). The computed tomogram (CT) of the head and magnetic resonance image (MRI or MR) of the brain showed evidence of subcortical and periventricular white matter lesions in the right hemisphere extending into the brainstem and the left frontal lobe. The hospital course consisted of supportive measures, seizure treatment along with prophylaxis, and human immunodeficiency virus (HIV) management along with prophylactic antibiotics. The patient was discharged with appropriate medications and outpatient referrals. Overall, this case describes some key points. It highlights particular imaging characteristics of PML in the setting of inadequately treated HIV. For example, white matter lesions cross the anterior corpus callosum rather than the splenium, as in the \"barbell\" sign. In addition, the lesions extend inferiorly along the ipsilateral corticospinal tract into the midbrain and pons. This could be one of the first cases to capture both of these features given the rarity of their concomitant occurrence.

A progresszív multifokális leukoencephalopathiát a John Cunningham-vírus reaktiválódása okozza, amely szinte kizárólag immunhiányos betegeknél fordul elő. A betegség tüneteit elsősorban a demyelinisatiós gócok lokalizációja határozza meg; a betegség a kezdeti szakaszban tünetszegény lehet, és a neurológiai tünetek csak később jelennek meg. Diagnosztikájában elsősorban a képalkotó vizsgálatok és a vírus-DNS liquorból történő kimutatása játszik fontos szerepet. Specifikus terápiája nem ismert, a cél az immunrendszer működésének helyreállítása. Kazuisztikánkban egy pszichiátriai osztályon észlelt páciens kórtörténetét ismertetjük, akinek esetében AIDS-hez köthető jobb féltekei progresszív multifokális leukoencephalopathia képe igazolódott. Korai differenciáldiagnosztikai nehézséget jelentettek a páciensnél észlelhető patológiás személyiségjegyek, illetve az élethelyzeti nehézségek és a párkapcsolati veszteség talaján kialakult krízisállapot. Esetünkkel szeretnénk felhívni a figyelmet az immunhiányos betegeknél jelentkező pszichiátriai tünetek fontosságára. Orv Hetil. 2024; 165(33): 1295–1302.

Progressive multifocal leukoencephalopathy (PML), a severe demyelinating disease of the central nervous system, is caused by the reactivation of the polyomavirus JC virus (JCV). It favors the cerebrum and typically occurs in patients with immunodeficiencies, with a progressive course and fatal outcome in the majority of cases. However, the cerebellar form of PML, characterized by isolated posterior fossa lesions, such as those in the cerebellum or brainstem at disease onset, is rare, and reports of its occurrence in peritoneal dialysis (PD) patients are lacking. In this paper, we describe a rare case of a cerebellar form of PML in a PD patient. A 64-year-old man undergoing PD was referred to our hospital for anorexia, nausea, and vomiting in the past month. He had finger-to-nose test abnormalities, gaze-directed nystagmus, and scanning speech. He was diagnosed with the cerebellar form of PML based on his progressive cerebellar symptoms, the typical magnetic resonance imaging findings, and the presence of JCV-DNA in the cerebrospinal fluid polymerase chain reaction test. He developed nocturnal delirium, aggravated disquiet, and died of pneumonia on the 69th day. Clinicians should consider the cerebellar form of PML as a differential diagnosis if PD patients develop progressive cerebellar symptoms.

Opportunistic viral infections in individuals with severe immunodeficiency can lead to fatal conditions such as progressive multifocal leukoencephalopathy (PML), for which treatment options are limited. These infections pose significant risks, especially when co-infections with other viruses occur. We describe a combined therapy approach using directly isolated allogeneic Human Polyomavirus 1 (also known as BKV) and Epstein-Barr virus (EBV) specific cytotoxic T-cells for the treatment of PML in conjunction with identified EBV in the cerebrospinal fluid (CSF) of a male patient infected with human immunodeficiency virus (HIV). A 53-year-old HIV-positive male, recently diagnosed with PML, presented with rapidly worsening symptoms, including ataxia, tetraparesis, dysarthria, and dysphagia, leading to respiratory failure. The patient developed PML even after commencing highly active antiretroviral therapy (HAART) 3 months prior. Brain magnetic resonance imaging (MRI) revealed multifocal demyelination lesions involving the posterior fossa and right thalamus suggestive of PML. In addition to the detection of human polyomavirus 2 (also known as JCV), analysis of CSF showed positive results for EBV deoxyribonucleic acid (DNA). His neurological condition markedly deteriorated over the following 2 months. Based on MRI, there was no evidence of Immune Reconstitution Inflammatory Syndrome contributing to this decline. The patient did not have endogenous virus-specific T-cells. We initiated an allogeneic, partially human leukocyte antigen-matched transfer of EBV and utilizing the cross-reactivity between BKV and JCV-BKV specific T-cells. This intervention led to notable neurological improvement and partial resolution of the MRI lesions within 6 weeks. Our case of a patient with acquired immune deficiency syndrome demonstrates that PML and concurrent EBV co-infection can still occur despite undergoing HAART treatment. This innovative experimental therapy, involving a combination of virus-specific T-cells, was demonstrated to be an effective treatment option in this patient.

暂无摘要。

John Cunningham virus (JCV) is most commonly acquired in childhood and is often asymptomatic throughout life. However, in the case of primary or secondary immunosuppression, it is known to cause progressive multifocal leukoencephalopathy (PML) in the central nervous system. Hereby, we describe a rare case of PML in a patient without known factors of immunosuppression or use of immunomodulation. A 53-year-old female patient was presented with progressive left-side weakness and tremors in the left hand over a period of two months. The patient was diagnosed with PML based on history, examination, cerebrospinal fluid markers, histopathology, and brain magnetic resonance imaging at presentation. Despite detailed examination, nothing was found in the patient to cause an immunosuppressed state. Therapy was started with mirtazapine with significant neurological improvement.To our knowledge, PML in immunocompetent patient with bening prognosis is a very rare condition. There is also no effective treatment. Our case is a complicated example of this condition.

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A John Cunningham-vírus (JCV) leginkább gyermekkorúakat fertőz, majd gyakran egész életben tünetmentes marad. Elsődleges vagy másodlagos immunszuppresszió esetén azonban ismert, hogy progresszív multifokális leukoencephalopathiát (PML) okoz a központi idegrendszerben. Ismertetjük a PML ritka esetét egy olyan betegnél, akinél nem ismert immunszuppressziót okozó tényező vagy immunmoduláció alkalmazása. Az 53 éves nőbeteg két hónapja tartó progresszív bal oldali gyengeséggel és a bal kéz remegésével jelentkezett. A betegnél jelentkezése után a kórelőzmény, a fizikális vizsgálat, a liquormarkerek, a szövettani vizsgálat és az agyi mágneses rezonanciás képalkotás alapján PML-t diagnosztizáltunk. A részletes vizsgálat ellenére a betegnél nem találtunk semmi olyat, ami immunszupprimált állapotot okozhatott volna. Mirtazapinterápiát kezdtünk, ami jelentős neurológiai javulást eredményezett. Ismereteink szerint a PML immunkompetens betegben benignus prognózissal nagyon ritka állapot. Nincs is hatékony kezelés. Esetünk ennek az állapotnak egy bonyolult példája.

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The differential diagnoses of ring-enhancing lesions of the brain parenchyma is broad, but complete ring-enhancing lesions often indicate a neoplastic or infectious process. We present a case of a 70-year-old female with a history of multiple sclerosis (MS) who was not on current disease-modifying therapy (DMT) and was found to have a ring-enhancing lesion that mimicked a high-grade glioma. The patient underwent gross total resection, and histopathologic and molecular analysis revealed a diagnosis of progressive multifocal leukoencephalopathy (PML). A subsequent medical workup on the patient was unrevealing aside from mild lymphopenia. This is a unique case that highlights both an unusual clinical presentation and radiographic appearance of PML. There is a known associated increased risk of PML with the use of some DMTs for MS. However, this case raises the question of the possibility of developing PML years after interferon beta-1a therapy in a patient without overt immunosuppression.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by reactivation of the human polyomavirus 2 (HPyV-2). PML is associated with a high morbidity and mortality rate and there is currently no standard curative therapy. We report short-term immunologic response and long-term clinical outcomes in a patient diagnosed with follicular lymphoma (FL) who developed PML. Diagnosis of PML was established conclusively based on findings from a brain biopsy. The patient was treated with recombinant interleukin 2 (IL-2) and showed rapid clinical improvement. HPyV-2-specific T-cells were tracked longitudinally and correlation with clinical status, viral load, and radiographic imaging was documented. After the progression of the patient\'s FL, which required an allogeneic bone marrow transplant, the patient prophylactically received human leukocyte antigen-matched donor-derived HPyV-2 T-cells to prevent the recurrence of the PML as part of a clinical trial. Twelve years after the initial diagnosis of PML, he did not develop a relapse of his PML, supporting data that therapies that increase HPyV-2-specific T-cells, including IL-2, may be effective in the management of PML.

UNASSIGNED: Progressive multifocal leukoencephalopathy is a rare manifestation in itself. Although many immunosuppressive states are associated with the disease, its occurrence in the setting of chronic lymphocytic leukaemia treated with chemotherapy is seldom reported to date.
UNASSIGNED: A 67-year-old woman with known chronic lymphocytic leukaemia who was previously receiving chlorambucil treatment was identified as having progressive multifocal leukoencephalopathy; her prognosis is currently good.
UNASSIGNED: Although a rare disease in an immunocompromised setting, progressive multifocal leukoencephalopathy often leads to a grave outcome. However, the authors describe a case with a good prognosis to date.
UNASSIGNED: Progressive multifocal leukoencephalopathy should be in differentials in immunocompromised patients with dementia. Given that the later prognosis of the disease is unpredictable, an earlier diagnosis would be better for immunological reconstitution.

Progressive multifocal leukoencephalopathy (PML) is a rare fetal disease that has been uprising since the 1980s. Accurate diagnosis can be challenging and requires a thorough clinical suspicion, particularly among individuals who do not have HIV infection. Further diagnostics studies including cerebrospinal fluid analysis are required for DNA polymerase chain reaction (PCR) and if negative, more invasive tests like Brain biopsy are required. Herein, we describe a rare case of a 64-year-old female with a history of discoid lupus for 30 years who was not on any medications and presented to the hospital multiple times with different neurological deficits. The initial diagnosis consistently pointed toward a stroke until a critical turning point when a cerebrospinal fluid sample tested positive for John Cunningham (JC) virus DNA. Unfortunately, by the time the disease was identified, it had already progressed significantly, resulting in the unfortunate demise of the patient. To our knowledge, this represents the second reported case of PML in a patient with discoid lupus who lacks other commonly observed risk factors for the disease. This finding underscores the significance of maintaining clinical attentiveness within this specific patient population.