scholarly journals Probable progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome with immunosuppressant dose reduction following lung transplantation: a case report and literature review

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Kazuhiro Ishii ◽  
Fumiko Yamamoto ◽  
Shinsuke Homma ◽  
Yoshinori Okada ◽  
Kazuo Nakamichi ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Immune Reconstitution ◽  
Brain Mri ◽  
Pulse Therapy ◽  
Brain Lesions ◽  
Cerebral White Matter ◽  
Mri Findings ◽  
Steroid Pulse ◽  
Cell Counts ◽  
Inflammatory Syndrome

Abstract Background Progressive multifocal leukoencephalopathy (PML) is a rapidly developing demyelinating disease in the cerebral white matter and is often caused by JC polyomavirus (JCV). PML after lung transplantation is rare and has a poor prognosis, with no established therapies. Reducing the patient’s immunosuppressant doses, thereby restoring immunity, could be used to treat PML. However, some patients develop immune reconstitution inflammatory syndrome (IRIS) with this treatment, an immune-induced inflammatory response to JCV that results in serious neuronal damage. We herein report a case of a 60-year-old female who suffered from PML 5 years after lung transplantation, had worsened brain lesions thought to be related to PML-IRIS at the time of immunosuppressant reduction, and missed treatment opportunities. Case presentation A 60-year-old female developed PML 5 years after lung transplantation. Fluid-attenuated inversion recovery and diffusion-weighted brain magnetic resonance imaging (MRI) revealed multiple high-signal lesions, mainly in the cerebral white matter. Polymerase chain reaction found 0.32 million copies/mL of JCV in the cerebrospinal fluid. Thus, she was given a diagnosis of PML. Mycophenolate mofetil and tacrolimus dosages were reduced, and CD4-positive cell counts and the blood concentration of each immunosuppressant were monitored. Mefloquine was also orally administered at a daily dose of 275 mg for 3 days and was then administered at a dose of 275 mg per week. Although the patient’s CD4-positive cell counts increased and her immune system recovered, her symptoms and brain MRI findings worsened. We suspected PML progression or a transition to PML-IRIS. Steroid pulse therapy to suppress the inflammatory lesions was not possible but was retrospectively indicated. The patient rapidly began to exhibit akinetic mutism and died 4 months after the onset of neurologic symptoms. Conclusions When neurologic symptoms and abnormal brain MRI findings are noted during immune recovery, it is often difficult to distinguish between progressed PML and PML-IRIS. However, the pathogenesis of brain lesions usually involves inflammation and immune-reactive mechanisms for JCV. Steroid pulse therapy, which can reduce inflammation, should thus be administered in organ transplantation cases with differential diagnoses including PML-IRIS.

Psychological distress in patients suffering from femoral head osteonecrosis, with and without white matter lesions: clinical characteristics and personality traits

2011 ◽  
Vol 26 (S2) ◽  
pp. 877-877
Author(s):  
O. Mouzas ◽  
M.N. Papaliaga ◽  
A. Zibis ◽  
G. Hadjigeorgiou ◽  
N. Angelopoulos ◽  
...  
Keyword(s):  
Psychological Distress ◽  
White Matter ◽  
Femoral Head ◽  
Brain Mri ◽  
General Health Questionnaire ◽  
White Matter Lesions ◽  
Self Report ◽  
Cerebral White Matter ◽  
Mri Findings ◽  
Femoral Head Osteonecrosis

BackgroundThe aim of the present study was to investigate clinical parameters in relation to personality characteristics and psychopathology, in patients with non traumatic osteonecrosis of the femoral head (ONFH), with and without cerebral white matter lesions (WML).MethodsSixty seven patients participated in the study, 48 males and 19 females. The mean age was 37.6 years (SD 10.92, range: 15–61). Seventy five healthy individuals age and sex matched, served as controls. Socio-demographic information, clinical data and brain MRI findings were collected. The following self-report instruments were used: General Health Questionnaire (GHQ-28) and Defense Style Questionnaire (DSQ).ResultsThe odds of being assessed with a psychiatric diagnosis upon interview were three times greater among patients with ONFH compared to healthy controls (p < .006). The presence of cerebral WML and secondary nature of ONFH were associated with intense symptoms of psychological distress and especially with somatic symptoms of depression and social dysfunction (p < .003). A specific personality structure was identified in patients with ONFH.ConclusionsPatients suffering from osteonecrosis of femoral head, especially when white matter lesions are present, often present psychological distress and problematic personality organization. Therefore psychological support and/or psychiatric intervention can be beneficial to these individuals.

scholarly journals Extensive immune reconstitution inflammatory syndrome in Fingolimod-associated PML: a case report with 7 Tesla MRI data

2019 ◽  
Author(s):  
Tim Sinnecker ◽  
Jeffrie Hadisurya ◽  
Tilman Schneider-Hohendorf ◽  
Nicholas Schwab ◽  
Karsten Wrede ◽  
...  
Keyword(s):  
Immune Reconstitution Inflammatory Syndrome ◽  
Immune Reconstitution ◽  
Rare Complication ◽  
7 Tesla ◽  
Mri Findings ◽  
Early Discontinuation ◽  
Case Presentation ◽  
Ultrahigh Field Mri ◽  
7 Tesla Mri ◽  
Inflammatory Syndrome

Abstract Background Progressive multifocal leukoencephalopathy (PML) is a rare complication of patients treated with fingolimod. Case presentation Routine MRI eventually led to diagnosis of asymptomatic early PML that remained stable after discontinuation of fingolimod. As blood lymphocyte counts normalized, signs of immune reconstitution inflammatory syndrome (IRIS) and renewed MS activity developed. Both, advanced laboratory and ultrahigh field MRI findings elucidated differences between PML and MS. Conclusions In our case, early discontinuation of fingolimod yielded a good outcome, lymphocyte counts reflected immune system activity, and paraclinical findings helped to differentiate between PML-IRIS and MS.

scholarly journals The predictive power of MRI criteria in the diagnosis of immune reconstitution inflammatory syndrome by the comparison of classification using the decision tree method and ROC analysis

2021 ◽  
Vol 12 (3) ◽  
pp. 16-25
Author(s):  
E. G. Bakulina ◽  
G. V. Kataeva ◽  
T. N. Trofimova
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Decision Tree ◽  
Immune Reconstitution Inflammatory Syndrome ◽  
Immune Reconstitution ◽  
Roc Analysis ◽  
Brain Mri ◽  
Predictive Values ◽  
The Central Nervous System ◽  
Inflammatory Syndrome

Introduction. Immune reconstitution inflammatory syndrome involving the central nervous system (CNS-IRIS) is a dangerous complication in HIV-infected patients on antiretroviral therapy (ART). The radiologic features of this syndrome have been little studied and are presented in isolated works. The diagnosis is difficult because there are no generally accepted criteria for IRIS. Our study is devoted to radiology of IRIS. Based on the results of brain MRI, together with clinical and laboratory data, MRI criteria for IRIS were formulated.Purpose and goals. To determine the prognostic value of MRI signs of CNS-IRIS using in a cohort of HIV-positive patients with neurological symptoms.Materials and methods. The analysis includes data from 68 HIV-infected patients who underwent brain MRI. In 14 of them were diagnosed IRIS with involvement of the central nervous system. To determine the diagnostic efficiency of the formulated MRI criteria, the STATISTICA program was used, decision trees were built, and a ROC analysis was performed.Results. Five decision tree models were built with different predictive values. The models took into account the categorical predictors (MRI criteria) in different order and quantity. The best performance has model #5, which can be considered a clinically useful predictive model.Conclusion. Brain MRI is an essential diagnostic step in HIV-infected patients on ART. It is necessary to expand the indications and conditions for radiological studies of the brain in patients with suspected immune reconstitution inflammatory syndrome.

scholarly journals Methadone-Induced Encephalopathy: A Case Series and Literature Review

2020 ◽  
Author(s):  
Maryam Haghighi-Morad ◽  
Zahra Naseri ◽  
Nazila Jamshidi ◽  
Hossein Hassanian-Moghaddam ◽  
Nasim Zamani ◽  
...  
Keyword(s):  
White Matter ◽  
Brain Mri ◽  
Case Series ◽  
Neurological Deficits ◽  
Cerebral White Matter ◽  
High Signal ◽  
Mri Findings ◽  
Caudate Nuclei ◽  
Delayed Onset ◽  
Signal Abnormality

Abstract Background: Accidental ingestion or consumption of supra-therapeutic doses of methadone can result in neurological sequelae in humans. We aimed to determine the neurological deficits of methadone-poisoned patients admitted to a referral poisoning hospital using brain magnetic resonance (MR) and diffusion weighted (DW) imaging. Methods: In this retrospective study, brain MRIs of the patients admitted to our referral center due to methadone intoxication were reviewed. Methadone intoxication was confirmed based on history, congruent clinical presentation, and confirmatory urine analysis . Each patient had an MRI with Echo planar T1, T2, FLAIR, and DWI and apparent deficient coefficient (ADC) sequences without contrast media. Abnormalities were recorded and categorized based on their anatomic location and sequence. Results: Ten patients with abnormal MRI findings were identified. Eight had acute- and two had delayed-onset encephalopathy. Imaging findings included bilateral confluent or patchy T2 and FLAIR high signal intensity in cerebral white matter, cerebellar involvement, and bilateral occipito-parietal cortex diffusion restriction in DWI. Internal capsule involvement was identified in two patients while abnormality in globus pallidus and head of caudate nuclei were reported in another. Bilateral cerebral symmetrical confluent white matter signal abnormality with sparing of subcortical U-fibers on T2 and FLAIR sequences were observed in both patients with delayed-onset encephalopathy. Conclusions : Acute- and delayed-onset encephalopathies are two rare adverse events detected in methadone-intoxicated patients. Brain MRI findings can be helpful in detection of methadone-induced encephalopathy.

Neurosyphilis and neuro-AIDS

2020 ◽  
pp. 6100-6109
Author(s):  
Hadi Manji
Keyword(s):  
Spinal Cord ◽  
Immune Reconstitution ◽  
Brain Lesions ◽  
Low Grade ◽  
Syphilis Infection ◽  
Highly Active ◽  
Tabes Dorsalis ◽  
The Central Nervous System ◽  
Inflammatory Syndrome ◽  
The Brain

Invasion of the central nervous system occurs early in the course of syphilis infection. Neurosyphilis causes a meningitis, a myeloradiculopathy due to pachymeningitis, gummatous (granulomatous) cord and brain lesions; endarteritis may cause infarction and a low-grade meningoencephalitis affecting the brain results in dementia (general paralysis of the insane) and in the spinal cord, a sensory ataxic syndrome (tabes dorsalis). The introduction of highly active antiretroviral therapies has greatly reduced the frequency of these complications in patients with access to these treatments. However, newer complications are now increasingly recognized such as neurological immune reconstitution inflammatory syndrome, a compartmentalization syndrome (cerebrospinal fluid escape). This chapter looks at these and other important issues regarding the background, diagnosis, treatment, and outlook for neurosyphilis and neuro-AIDS.

Frequency and Distribution of Asymptomatic Brain Lesions in Patients with β-Thalassemia Intermedia

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 5141-5141
Author(s):  
Mehran Karimi ◽  
Sezaneh Haghpanah ◽  
Mohammad Hadi Bagheri ◽  
Mohammad Reza Bordbar ◽  
Parisa Pishdad ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Brain Mri ◽  
Univariate Analysis ◽  
White Matter Lesions ◽  
Brain Lesions ◽  
Cerebral White Matter ◽  
Blood Transfusions ◽  
Thalassemia Intermedia ◽  
Cross Sectional ◽  
Number Of Patients

Abstract Abstract 5141 We aimed to determine the frequency of asymptomatic brain lesions in a group of patients with β-thalassemia intermedia (β-TI) and to evaluate correlation of asymptomatic brain lesions with splenectomy, thrombocytosis, blood transfusions and clinical parameters. Ninety five neurologically intact patients with β-TI were randomly enrolled in this cross-sectional study. Diffusion Weighted Imaging (DWI) brain MRI was performed in every patient to detect cerebral white matter lesions (WML). We found an overall frequency of 15 (15. 8%) for WMLs, 14 (23. 7%) in splenectomized and 1 (2. 8%) in nonsplenectomized patients. The presence of WML was significantly associated with splenectomy (P= 0. 008) and thrombocytosis (P=0. 009). However, after adjustment for splenectomy, thrombocytosis was not significantly associated with the presence of WML (P>0. 05). The number of patients with regular blood transfusions and normal MRI was not significantly higher compared to those with abnormal findings (52. 5% vs 26. 7%; P=0. 092). In untransfused patients, hydroxyurea (HU) administration was associated with a lower incidence of WML (P<0. 001). Although in univariate analysis either splenectomy or thrombocytosis showed significant correlation with the presence of single or multiple WMLs, thrombocytosis by itself did not significantly contribute in developing asymptomatic brain lesions. The lack of significant correlation between lesions and regular blood transfusions could be related to the treatment with HU in untransfused patients, which increased fetal hemoglobin levels and improved the morphology and the pathological indices of the red blood cells. Larger prospective studies are suggested for evaluation of the correlation of these factors with developing asymptomatic brain lesions accurately. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Fingolimod-associated PML with mild IRIS in MS

2017 ◽  
Vol 5 (1) ◽  
pp. e415 ◽  
Author(s):  
Shuhei Nishiyama ◽  
Tatsuro Misu ◽  
Yukiko Shishido-Hara ◽  
Kazuo Nakamichi ◽  
Masayuki Saijo ◽  
...  
Keyword(s):  
White Matter ◽  
Immune Reconstitution ◽  
Jc Virus ◽  
Brain Mri ◽  
Subcortical White Matter ◽  
Precentral Gyrus ◽  
Fluid Attenuated Inversion Recovery ◽  
Viral Inclusions ◽  
Inflammatory Syndrome

Objective:To clarify the clinical, neuropathologic, and virologic characteristics of progressive multifocal leukoencephalopathy (PML) and its immune reconstitution inflammatory syndrome (IRIS) in a patient with fingolimod-treated MS.Methods:A case study.Results:A 34-year-old patient with MS using fingolimod for 4 years had a gradual progression of right hemiparesis and aphasia with a new subcortical white matter lesion in the precentral gyrus by initial MRI. Blood tests were normal, except for lymphopenia (160 cells/μL). One month after the cessation of fingolimod, brain MRI depicted a diffusely exacerbated hyperintensity on fluid-attenuated inversion recovery and diffusion-weighed imaging in the white matter with punctate gadolinium enhancement, suggesting PML-IRIS. A very low level of JC virus (JCV)-DNA (15 copies/mL) was detected in the CSF as judged by quantitative PCR. Brain tissues were biopsied from the left frontal lesion, which showed some small demyelinated foci with predominant loss of myelin-associated glycoprotein with infiltrations of lymphocytes and macrophages, but clear viral inclusion was not observed with hematoxylin-eosin staining. JCV-DNA was uniquely detectable in an active inflammatory demyelinating lesion by in situ hybridization, possibly suggesting an early phase of PML. DNA extracted from the brain sample was positive for JCV-DNA (151 copies/cell). It took 3 months to normalize the blood lymphocyte count. The patient was treated with 1 g of IV methylprednisolone for 3 days and a weekly oral dose (375 mg) of mefloquine, and her symptoms gradually improved.Conclusion:Low CSF JCV-DNA and unfound viral inclusions initially made her diagnosis difficult. The clinical course of fingolimod-associated PML may be associated with mild immune reconstitution.

scholarly journals Acute Kidney Injury, Recurrent Seizures, and Thrombocytopenia in a Young Patient with Lupus Nephritis: A Diagnostic Dilemma

2016 ◽  
Vol 2016 ◽  
pp. 1-8
Author(s):  
Hector Alvarado Verduzco ◽  
Anjali Acharya
Keyword(s):  
Blood Pressure ◽  
Acute Kidney Injury ◽  
Lupus Nephritis ◽  
Kidney Biopsy ◽  
Brain Mri ◽  
Kidney Injury ◽  
Uncontrolled Hypertension ◽  
Cerebral White Matter ◽  
Mri Findings ◽  
Class Iv

Introduction. Posterior reversible encephalopathy syndrome (PRES) is a constellation of clinical and radiologic findings. Fluctuations in blood pressure, seizures, and reversible brain MRI findings mainly in posterior cerebral white matter are the main manifestations. PRES has been associated with multiple conditions such as autoimmune disorders, pregnancy, organ transplant, and thrombotic microangiopathy (TMA). Case Presentation. A 22-year-old woman with history of Systemic Lupus Erythematous complicated with chronic kidney disease secondary to lupus nephritis class IV presented with recurrent seizures and uncontrolled hypertension. She was found to have acute kidney injury and thrombocytopenia. Repeat kidney biopsy showed diffuse endocapillary and extracapillary proliferative and membranous lupus nephritis (ISN-RPS class IV-G+V) and endothelial swelling secondary to severe hypertension but no evidence of TMA. Brain MRI showed reversible left frontal and parietal lesions that resolved after controlling the blood pressure, making PRES the diagnosis. Conclusion. PRES is an important entity that must be recognized and treated early due to the potential reversibility in the early stages. Physicians must have high suspicion for these unusual presentations. We present a case where performing kidney biopsy clinched the diagnosis in our patient with multiple confounding factors.

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