NCMP-08. CHALLENGING DIAGNOSIS AND MANAGEMENT OF PCNSL IN AN IMMUNOSUPPRESSED PATIENT ON MYCOPHENOLATE MOFETIL FOR MYASTHENIA GRAVIS

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi148-vi149
Author(s):  
Ornella Bricoune ◽  
Sepideh Mokhtari ◽  
Arnold Etame ◽  
Timothy Robinson ◽  
yolanda Pina ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Myasthenia Gravis ◽  
Brain Mri ◽  
B Cell Lymphoma ◽  
Rapid Progression ◽  
Periventricular White Matter ◽  
Dramatic Improvement ◽  
Immunosuppressed Patient ◽  
Radiographic Findings ◽  
Csf Analysis

Abstract INTRODUCTION Primary CNS lymphoma (PCNSL) typically presents with non-focal neurologic symptoms including disorientation, poor balance and memory and singel or multifocal periventricular MRI lesions. Deviations in characteristic findings can delay diagnosis and require additional diagnostic tests. OBSERVATION A 68-year-old man with a recent zoster infection and acetylcholine receptor antibody positive myasthenia gravis (MG) on Mycophenolate Mofetil for 22 years presented at another institution with left eye vision changes, and focal neurological deficits. A brain MRI showed an enhancing lesion within his left medulla extending to the cerebellum. Cerebrospinal fluid (CSF) analysis was positive for EBV and negative for malignancy. He was diagnosed with VZV vasculopathy and discharged home on IV Acyclovir for 14 days and a 5-day course of oral prednisone 60 mg. Three months later, a repeat brain MRI showed multiple new enhancing lesions bilaterally along the periventricular white matter with involvement of the corpus callosum with several lesions in peripheral locations of the cerebrum, cerebellum, and brainstem. He presented to local ER with intermittent encephalopathy, acute left eye vision blurriness and was started on steroids. He was transferred to our institution and had CSF analysis which was positive for EBV and negative for malignancy. Due to rapid progression of his symptoms, he underwent gross total resection of the left frontal lesion which showed EBV-induced diffuse large B-cell lymphoma (DLBCL). His Mycophenolate Mofetil was discontinued and he had a dramatic improvement in his left eye vision and cognitive deficits within 24 hours after one dose of Rituximab IV 500 mg/m2. DISCUSSION In the setting of periventricular lesions and EBV positivity on CSF, EBV-induced DLBCL should be highly considered. CONCLUSION Misdiagnosis or delay in diagnosis of PCNSL due to the presence of atypical features in disease presentation and radiographic findings could lead to progression of PCNSL.

Variable Presentation and Reduced Penetrance in Autosomal Dominant Acute Necrotizing Encephalopathy Related to RANBP2 Variant

2021 ◽  
Author(s):  
Daniel R. Carvalho ◽  
Carlos E. Speck-Martins ◽  
Bernardo J. A. F. Martins ◽  
Ana Paula Izumi ◽  
Alessandra La Rocque-Ferreira
Keyword(s):  
Autosomal Dominant ◽  
Phenotypic Variability ◽  
Brain Mri ◽  
Central Nervous System Infection ◽  
Rapid Progression ◽  
Periventricular White Matter ◽  
Incomplete Penetrance ◽  
Healthy Children ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing

AbstractAcute necrotizing encephalopathy (ANE) is clinically characterized by fever, acute alteration of consciousness, seizures, and rapid progression to coma within days of onset of a viral illness occurring in healthy children without evidence of central nervous system infection. Brain magnetic resonance imaging (MRI) shows multiple symmetrical lesions affecting primarily the thalami but also brain stem, putamina, periventricular white matter, and cerebellum. Most cases of ANE are sporadic and nonrecurrent. However, a missense variant in RANBP2 has been identified in some families with recurrent ANE (OMIM # 608033), also named autosomal dominant ANE (ADANE). Clinical manifestation, clinical course, and brain MRI imaging findings of six affected members of two distinct families with ADANE were described. Sequencing revealed heterozygous c.1754C > T variant in RANBP2 (p.Thr585Met) in affected and asymptomatic family members. Only few ADANE families have been reported and it is the first description in South America. Differential diagnosis of Leigh disease and acute disseminated encephalomyelitis is discussed. Our report reinforces incomplete penetrance of ADANE and intrafamilial phenotypic variability of outcome.

scholarly journals Electroencephalography in systemic lupus erythematosus patients with neuropsychiatric manifestations

2020 ◽  
Vol 32 (1) ◽  
Author(s):  
Howaida E. Mansour ◽  
Reem A. Habeeb ◽  
Noran O. El-Azizi ◽  
Heba H. Afeefy ◽  
Marwa A. Nassef ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Epileptiform Activity ◽  
Brain Mri ◽  
Periventricular White Matter ◽  
Normal Brain ◽  
Systemic Lupus ◽  
Specific Test ◽  
Eeg Abnormalities ◽  
Neuropsychiatric Manifestations

Abstract Background Neuropsychiatric manifestations are frequently reported in systemic lupus erythematosus (SLE) patients. This study was done to describe electroencephalographic (EEG) findings in SLE patients with neuropsychiatric manifestation (NPSLE). Results Among 60 SLE patients, there were 50 females (83.3%) and 10 males (16.7%). EEG abnormalities were reported in 12 patients out of 30 (40%) with NPSLE, while all patients with non-NPSLE (n = 30) had no EEG abnormalities; diffuse slowing (20%) was the most common abnormalities, followed by generalized epileptiform activity (13.3%), and lastly temporal epileptiform activity (6.7%). Seizure was the most reported neuropsychiatric disorder in 13 patients (43.3%); 8 of them had abnormal EEG (61.5%). Periventricular white matter lesion (23.3%) followed by infarction (13.3%) were the most common MRI brain findings among 53.3% of NPSLE group. Half of the cases with EEG abnormality had normal brain MRI. SLEDAI score and ACL IgM positivity were higher in the NPSLE group than the non-NPSLE group. EEG is not a sensitive or specific test for detecting NPSLE with sensitivity (37.5%) and specificity (57.1%). Conclusion Not all patients with NPSLE must have abnormal brain MRI or EEG. EEG is a useful assistant tool in the assessment of different manifestations of NPSLE, but it cannot be used as a screening test alone and must be supplemented by neuroimaging studies.

Recurrence of Primary Breast Lymphoma Presenting as Bilateral Vitreoretinal Lymphoma

2021 ◽  
pp. 1-5
Author(s):  
Karishma Habbu ◽  
Roshan George ◽  
Miguel Materin
Keyword(s):  
Brain Mri ◽  
B Cell Lymphoma ◽  
Intrathecal Chemotherapy ◽  
Primary Breast Lymphoma ◽  
Blurred Vision ◽  
Breast Lymphoma ◽  
Central Nervous System Penetration ◽  
Vitreoretinal Lymphoma ◽  
The Right ◽  
Vitreous Biopsy

<b><i>Purpose:</i></b> This report describes a case of relapsed primary breast lymphoma (PBL) presenting as vitreoretinal lymphoma (VRL). <b><i>Methods:</i></b> We describe the clinical and hematopathologic findings in a patient with relapsed PBL involving the vitreous of both eyes. <b><i>Results:</i></b> A 59-year-old woman was treated for PBL with systemic and intrathecal chemotherapy 5 years prior to presentation. Three years later, she presented to an outside clinic with blurred vision in both eyes and bilateral vitritis. She was referred to our clinic with concern for ocular lymphoma. On presentation, the patient’s best-corrected visual acuity was 20/40 in the right eye and 20/25 in the left eye with 3+ vitreous cells in the right eye and 2+ vitreous cells in the left eye. Vitreous biopsy of the right eye revealed CD5-negative/CD10-negative B-cell lymphoma cells on flow cytometry. She had no evidence of disease on brain MRI, lumbar puncture, bone marrow biopsy, or full-body CT scans. She was treated with a regimen of rituximab, methotrexate, procarbazine, and vincristine for central nervous system penetration as well as multiple intraocular injections of methotrexate and rituximab with improvement in vision and ocular inflammation bilaterally. <b><i>Conclusion:</i></b> Relapsed PBL can present as bilateral VRL.

The Clinical Value of Periventricular White Matter Hyperintensity on MRI in Sudden Sensorineural Hearing Loss

2021 ◽  
pp. 000348942110189
Author(s):  
Jung Woo Lee ◽  
Deoksu Kim ◽  
Seokhwan Lee ◽  
Sung-Won Choi ◽  
Soo-Keun Kong ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Multivariate Analysis ◽  
White Matter ◽  
Brain Mri ◽  
Sudden Sensorineural Hearing Loss ◽  
White Matter Hyperintensity ◽  
Control Group ◽  
Periventricular White Matter ◽  
Clinical Value ◽  
Hearing Gain

Objectives: To assess the clinical value of periventricular white matter hyperintensity (PWMH) found on brain magnetic resonance imaging (MRI) in patients with sudden sensorineural hearing loss (SSNHL). Methods: In this prospective study, 115 patients who were diagnosed with SSNHL aged between 55 and 75 years were analyzed. All subjects underwent brain MRI and were divided into a PWMH and control groups, depending on the presence of PWMH on MRI. PWMH was subdivided into 3 groups according to severity. Pure-tone average results and hearing gain were compared between the 2 groups before treatment and 2 months after treatment. Hearing improvement was assessed using Sigel’s criteria. Results: A total of 106 patients (43 in the PWMH group and 63 in the control group) finally completed the 2-month follow-up. Average hearing gain in the PWMH group was significantly higher than in the control group (34.8 ± 20.3 and 25.9 ± 20.3, respectively, P = .029). PWMH score 1 showed significantly better hearing levels and hearing gain compared to PWMH score 3 and the control group. Multivariate analysis revealed that younger age, better initial hearing level, and the presence of PVWM score 1 were associated with good recovery. Conclusions: The presence of PWMH score 1 on brain MRI in patients with SSNHL was associated with better treatment response and was a good prognostic factor in a multivariate analysis while the hearing recovery in more severe PWMH (scores 2, 3) was not different from the control group.

Primary diffuse large B cell lymphoma of the optic chiasm in an immunocompetent patient

2021 ◽  
Vol 14 (7) ◽  
pp. e243307
Author(s):  
Orlando De Jesus ◽  
Christian Rios-Vicil ◽  
Frances M Gómez-González ◽  
Román Vélez
Keyword(s):  
B Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Brain Mri ◽  
B Cell Lymphoma ◽  
Optic Chiasm ◽  
Immunocompetent Patient ◽  
Primary Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Primary lymphoma of the visual pathway is rare, especially at the chiasm. Very few cases have been reported. The lesion is frequently confused with an optic–hypothalamic glioma. A 55-year-old man was found disoriented at his home by a friend and evaluated with a brain MRI which demonstrated an expansile mass located at the optic chiasm and hypothalamus level. The principal differential was a high-grade hypothalamic glioma due to the contrast enhancement. A biopsy of the chiasmal lesion was performed. Histological diagnosis of the lesion was compatible with a diffuse large B cell lymphoma. He was started on methotrexate and rituximab; however, his clinical course kept deteriorating, and he died 64 days after his presentation. All prior cases of primary lymphoma of the chiasm are reviewed.

Manifestation of cognitive impairments after a prior coronavirus infection COVID-19 in a patient with ApoE ε4 genotype

2021 ◽  
Vol 26 (2) ◽  
pp. 25-29
Author(s):  
M. S. Novikova ◽  
V. V. Zakharov ◽  
N. V. Vakhnina
Keyword(s):  
Cognitive Impairment ◽  
Mild Cognitive Impairment ◽  
Small Vessel Disease ◽  
Brain Mri ◽  
Periventricular White Matter ◽  
Everyday Activity ◽  
Apoe Ε4 ◽  
Coronavirus Infection ◽  
Novel Coronavirus

Nowadays, the novel coronavirus infection (COVID-19) pandemic is one of the most important global health problems. There is increasing evidence that COVID-19 affects central and peripheral nervous system as well. The paper presents a clinical case of a 47 old patient with the ApoE ε4 haplotype and family history of Alzheimer’s disease who developed cognitive impairment after acute COVID-19. Before the infection the patient has no cognitive complaints and preserved everyday activity. After novel coronavirus infection, which was observed in mild form, the patient had started to complain on constant excessive forgetfulness. Neuropsychological assessment confirmed the presence of pre-mild cognitive impairment of predominantly single domain amnestic type. However, brain MRI showed only subtle periventricular white matter changes usually attributed to small vessel disease. Memory complaints were observed for 3 months of follow up despite intensive cognitive training, optimization of lifestyle and therapy with choline alphoscerate. Probable links between coronavirus infectious and cognitive impairment manifestation are discussed. There is data that ApoE ε4 haplotype is associated with increase of microglia mediated neuro-inflammation and it can be significant for accelerating of progression of neurodegenerative diseases after COVID-19. Further follow up of the patient is necessary for determination of nosological diagnosis explaining manifested predominantly amnestic type pre-mild cognitive impairment.

scholarly journals Lactic acidosis: a unique presentation of diffuse large B-cell lymphoma

2019 ◽  
Vol 12 (10) ◽  
pp. e230277 ◽  
Author(s):  
Turab Jawaid Mohammed ◽  
Rohit Gosain ◽  
Rajeev Sharma ◽  
Pallawi Torka
Keyword(s):  
Lactic Acidosis ◽  
B Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
B Cell Lymphoma ◽  
Intrathecal Methotrexate ◽  
Dramatic Improvement ◽  
Metabolic Encephalopathy ◽  
Large B Cell Lymphoma ◽  
Large B Cell

An elderly man in the seventh decade of life was brought to the hospital with worsening mental status. Blood tests revealed anaemia and thrombocytopenia with elevated lactate dehydrogenase and serum lactate levels. CT scan showed bulky thoracic and abdominal lymphadenopathy with splenomegaly. A positron emission tomography scan confirmed the above and in addition, revealed bilateral adrenal involvement. Bone marrow biopsy revealed non-germinal centre B-cell-like (non-GCB)-diffuse large B-cell lymphoma (DLBCL). Prompt treatment with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab with intrathecal methotrexate chemotherapy resulted in a dramatic improvement in the patient’s condition. This vignette serves as a reminder to include aggressive lymphomas like DLBCL in the differential diagnoses of patients presenting with metabolic encephalopathy and lactic acidosis. Our patient was moribund at presentation with poor sensorium and failure to thrive. The dilemma was whether to take an aggressive stand and start chemotherapy urgently or whether to stabilise the patient first and then consider the treatment of DLBCL. We make a case for initiating therapy promptly in such patients irrespective of their performance status.

scholarly journals Encephalitis with radial perivascular emphasis

2020 ◽  
Vol 7 (2) ◽  
pp. e670 ◽  
Author(s):  
Jonathan Wickel ◽  
Ha-Yeun Chung ◽  
Klaus Kirchhof ◽  
David Boeckler ◽  
Stefan Merkelbach ◽  
...  
Keyword(s):  
Brain Tissue ◽  
Staining Pattern ◽  
Brain Mri ◽  
Brain Slices ◽  
Specific Cell ◽  
Gadolinium Enhancement ◽  
Negative Patient ◽  
Csf Analysis ◽  
Similar Disease ◽  
High Doses

ObjectiveAutoimmune steroid-responsive meningoencephalomyelitis with linear perivascular gadolinium enhancement in brain MRI is regarded as glial fibrillary acidic protein (GFAP) astrocytopathy characterized by anti-GFAP antibodies (ABs). We questioned whether anti-GFAP ABs are necessarily associated with this syndrome.MethodsTwo patients with a strikingly similar disease course suggestive of autoimmune GFAP astrocytopathy are reported. Clinical examination, MRI, laboratory, and CSF analysis were performed. Neuropathologic examination of brain tissue was obtained from one patient. Serum and CSF were additionally tested using mouse brain slices, microglia-astrocyte cocultures, and a GFAP-specific cell-based assay.ResultsBoth patients presented with subacute influenza-like symptoms and developed severe neurocognitive and neurologic deficits and impaired consciousness. MRIs of both patients revealed radial perivascular gadolinium enhancement extending from the lateral ventricles to the white matter suggestive of autoimmune GFAP astrocytopathy. Both patients responded well to high doses of methylprednisolone. Only one patient had anti-GFAP ABs with a typical staining pattern of astrocytes, whereas serum and CSF of the other patient were negative and showed neither reactivity to brain tissue nor to vital or permeabilized astrocytes. Neuropathologic examination of the anti-GFAP AB-negative patient revealed infiltration of macrophages and T cells around blood vessels and activation of microglia without obvious features of clasmatodendrosis.ConclusionsThe GFAP-AB negative patient had both a striking (para)clinical similarity and an immediate response to immunotherapy. This supports the hypothesis that the clinical spectrum of steroid-responsive meningoencephalomyelitis suggestive of autoimmune GFAP astrocytopathy may be broader and may comprise also seronegative cases.

scholarly journals Primary infratentorial diffuse large b-cell lymphoma: a challenging diagnosis in an immunocompetent patient

2019 ◽  
Vol 65 (2) ◽  
pp. 136-140 ◽  
Author(s):  
Gabriel Laverdi Beraldo ◽  
Angelo Borsarelli Carvalho Brito ◽  
Márcia Torresan Delamain ◽  
Carmino Antonio de Souza ◽  
Carmen Silvia Passos Lima ◽  
...  
Keyword(s):  
B Cell ◽  
Cerebral Blood Volume ◽  
Cell Lymphoma ◽  
Brain Mri ◽  
B Cell Lymphoma ◽  
Immunocompetent Patient ◽  
Hyperintense Lesion ◽  
Large B Cell Lymphoma ◽  
Cerebellar Peduncle ◽  
Large B Cell

SUMMARY We describe the case of a female patient, 52 years old, with dizziness and left motor incoordination for 2 weeks. Brain MRI magnetic resonance imaging) revealed a hyperintense lesion on T2-weighted images, without restricted diffusion, in the left middle cerebellar peduncle. Spectroscopy demonstrated peak of lipids and perfusion did not show any elevation in relative cerebral blood volume (rCBV). The patient underwent an open biopsy and resection, and the diagnosis of diffuse large B-cell lymphoma (DLBCL) was established. The patient received intravenous dexamethasone with symptoms remission, followed by four cycles of methotrexate plus cytarabine. After 3 months, the patient returned with decreased consciences level and a new MRI revealed a right superior frontal gyrus lesion with features suggesting a lymphomatous lesion. The patient died five days after her relapse.

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