scholarly journals Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
T. Nanda

Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease most common in immunodeficient patients. It occurs due to reactivation of the John Cunningham Virus (JCV) and carries a poor prognosis, with a median life expectancy of 6 months. We report a case of a 66-year-old man with a history of HCV related cirrhosis (HCV) and hepatocellular carcinoma (HCC) who was found to have PML in the setting of a negative viral load in the CSF and a CD4+ >200. He initially presented with two weeks of mild confusion and word-finding difficulty concerning for hepatic encephalopathy. An MRI was notable for extensive T2/FLAIR hyperintensity signal in the left temporal lobe. Brain biopsy was positive for JCV. PML is rare in immunocompetent individuals, especially in the setting of a negative viral load. It is possible, however, that transient states of immunosuppression may have been responsible in this case. Although viral load was reported as negative, virus may still have been detected but was below the quantifiable threshold. It is important for clinicians to note that a negative result does not necessarily exclude the possibility of PML, and care should be taken to review lab values on viral load in closer detail.

2021 ◽  
pp. 112067212199053
Author(s):  
Sameera Hettipathirannahelage ◽  
Sidath Wijetilleka ◽  
Hugh Jewsbury

Introduction: Progressive multifocal leukoencephalopathy (PML) is a rare, lethal, demyelinating disease classically seen in profoundly immunosuppressed individuals. It is caused by intracerebral infection by John Cunningham polyomavirus (JCV). We report a rare case of PML in a man with presumed immunocompetence at presentation experiencing bilateral painless visual impairment. Case Description: A 60-year-old man with a 3-week history of bilateral painless visual impairment attended our ophthalmology department. Unusually, he navigated around the room well and was able to read 4 of 13 Ishihara test plates in spite of a best-corrected visual acuity of counting fingers at 1 m bilaterally. Slit lamp examination, routine blood tests and optical coherence tomography (OCT) of the maculae and discs were unremarkable. Diffuse hyperintense white matter lesions on T2-weighted magnetic resonance imaging of the brain and detection of JCV within the parietal lobe tissue obtained by biopsy confirmed PML. Additional investigations identified an underlying hypogammaglobulinaemia, which may have initiated PML. He received intravenous immunoglobulin but passed away 2 months after diagnosis. Conclusions: To our knowledge this case is one of only a handful worldwide to describe PML developing in a patient with presumed immunocompetence at presentation – there was no previous history of recurrent, chronic, or atypical infections. There has only been one other report of visual symptoms presenting as the primary complaint. The case illustrates the importance of ruling out organic, central nervous system pathology in patients presenting with visual loss and normal objective visual function tests such as slit lamp examination and OCT.


2020 ◽  
Vol 26 (6) ◽  
pp. 952-956
Author(s):  
Mengyan Wang ◽  
Zhongdong Zhang ◽  
Jinchuan Shi ◽  
Hong Liu ◽  
Binhai Zhang ◽  
...  

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.


2016 ◽  
Vol 2016 ◽  
pp. 1-2 ◽  
Author(s):  
Chandra Punch ◽  
Christina Schofield ◽  
Penelope Harris

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.


2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Rafael Garcia-Carretero ◽  
Blanca San Jose Montano

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC virus, a polyomavirus that can be reactivated under certain immunosuppressive conditions, such as AIDS, immunomodulatory therapy, and haematological malignancies. However, a few cases of immunocompetent patients have been reported in which no immunodeficiency was present. We describe the case of an 83-year-old immunocompetent man who presented with severe cerebellar symptoms with an MRI scan suggestive of severe demyelinating disease. We were not able to identify any occult immunosuppression or malignancy in our patient.


2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Kimberly A. Silverio ◽  
Shyam A. Patel

Anticancer therapy predisposes patients to infections by the immunosuppression that results from treatment. Although 85% of patients with progressive multifocal leukoencephalopathy (PML) have concurrent HIV/AIDS, PML can also develop in patients after they receive chemotherapy for cancer. The case herein describes a 69-year-old man with history of follicular lymphoma who presented with progressive dysarthria and right-sided paralysis. He received rituximab one year prior to presentation. PET scan suggested no recurrence of lymphoma. Cerebrospinal fluid (CSF) analysis was negative and showed fewer than 500 copies/mL of JC virus. However, brain biopsy showed chromatin margination and viropathic change within oligodendrocytes, confirming PML. He was started on mirtazapine and mefloquine with some clinical improvement. To our knowledge, this is the first case of rituximab-associated PML in a patient with negative JC virus PCR from the CSF. Recognition of PML in the differential of oncology patients with CNS symptoms is an important consideration as we enter the era of targeted therapy and personalized cancer medicine involving biologics. Furthermore, screening of patients for presence of subclinical JC viremia prior to the use of biologics may be an important component of assessing patient candidacy for these agents.


2021 ◽  
Vol 2021 (2b) ◽  
pp. 113-116
Author(s):  
T.A. Litovchenko ◽  
◽  
O.V. Vostrotin ◽  
O.L. Tondiy ◽  
V.V. Lebedynets ◽  
...  

This article discusses the development of progressive multifocal leukoencephalopathy after a kidney transplantation on the background of immunosuppressive therap. It’s the example of a clinical case. Based on the literature review, it is known that progressive multifocal leukoencephalopathy is a progressive demyelinating disease of the central nervous system, which is caused by reactivation of the latent JC virus; such reactivation usually occurs in immunodeficient conditions. In the clinical case, the appearance of this disease was demonstrated and its severe and potentially fatal multifocal lesion of the white matter of the brain was proved. The disease was developed on the background of immunosuppressive therapy after a history of kidney transplantation. The understanding of the clinical course of progressive multifocal leukoencephalopathy, the absence of specific neurological manifestations and course were expanded. It is noted that MRI results in addition to the clinical picture and anamnestic data help to establish the diagnosis of PML and make a differential diagnosis. Progressive multifocal leukoencephalopathy in transplant recipients receiving immunosuppressive therapy have an unfavorable prognosis.


2019 ◽  
Vol 7 (1) ◽  
pp. 181
Author(s):  
Akshay K. Barde ◽  
Pankaj Soni

Aspergillus is a fungus found in the environment. In an immunocompetent person, inhalation of spores may cause localized infection. In immune compromised patients, these fungi can cause life-threatening invasive infections which has high morbidity and mortality. Invasive aspergillosis has a poor prognosis. Intracranial aspergillosis is an extremely rare manifestation of invasive aspergillosis in immunocompetent individuals. A case of 60-year-old immunocompetent male is reported who had multiple Aspergillus brain abscess.


Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 78
Author(s):  
Anne Bryden ◽  
Natalie Majors ◽  
Vinay Puri ◽  
Thomas Moriarty

This study examines an 11-year-old boy with a known history of a large previously asymptomatic arachnoid cyst (AC) presenting with acute onset of right facial droop, hemiplegia, and expressive aphasia. Shortly after arrival to the emergency department, the patient exhibited complete resolution of right-sided hemiplegia but developed headache and had persistent word-finding difficulties. Prior to symptom onset while in class at school, there was an absence of reported jerking movements, headache, photophobia, fever, or trauma. At the time of neurology consultation, the physical exam showed mildly delayed cognitive processing but was otherwise unremarkable. The patient underwent MRI scanning of the brain, which revealed left convexity subdural hematohygroma and perirolandic cortex edema resulting from ruptured left frontoparietal AC. He was evaluated by neurosurgery and managed expectantly. He recovered uneventfully and was discharged two days after presentation remaining asymptomatic on subsequent outpatient visits. The family express concerns regarding increased anxiety and mild memory loss since hospitalization.


2021 ◽  
pp. sextrans-2020-054887
Author(s):  
Silvia Achia Nieuwenburg ◽  
Ricardo Jamie Sprenger ◽  
Maarten Franciscus Schim van der Loeff ◽  
Henry John Christiaan de Vries

ObjectivesHIV-positive men who have sex with men (MSM) may be at a higher risk of repeat syphilis, have different clinical manifestations and have a different serological response to treatment compared with HIV-negative MSM. The objective of this study was to assess whether HIV-negative and HIV-positive MSM with infectious syphilis (primary, secondary or early latent) differed in history of previous syphilis episodes, disease stage and non-treponemal titre of initial and repeat episodes, and the titre response 6 and 12 months after treatment. Furthermore, determinants associated with an inadequate titre response after treatment were explored.MethodsThis retrospective analysis used data of five longitudinal studies (four cohorts; one randomised controlled trial) conducted at the STI clinic in Amsterdam, the Netherlands. Participants were tested for syphilis and completed questionnaires on sexual risk behaviour every 3–6 months. We included data of participants with ≥1 syphilis diagnosis in 2014–2019. Pearson’s χ² test was used to compare HIV-negative and HIV-positive MSM in occurrence of previous syphilis episodes, disease stage of initial and repeat syphilis episode and non-treponemal titre treatment responses.ResultsWe included 355 participants with total 459 syphilis episodes. HIV-positive MSM were more likely to have a history of previous syphilis episodes compared with HIV-negative MSM (68/90 (75.6%) vs 96/265 (36.2%); p<0.001). Moreover, HIV-positive MSM with repeat syphilis were less often diagnosed with primary syphilis (7/73 (9.6%) vs 36/126 (28.6%)) and more often diagnosed with secondary syphilis (16/73 (21.9%) vs 17/126 (13.5%)) and early latent syphilis (50/73 (68.5%) vs 73/126 (57.9%)) (p=0.005). While not significantly different at 12 months, HIV-negative MSM were more likely to have an adequate titre response after 6 months compared with HIV-positive MSM (138/143 (96.5%) vs 66/74 (89.2%); p=0.032).ConclusionsIn repeat syphilis, HIV infection is associated with advanced syphilis stages and with higher non-treponemal titres. HIV infection affects the serological outcome after treatment, as an adequate titre response was observed earlier in HIV-negative MSM.


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