scholarly journals Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib

2021 ◽  
Vol 9 ◽  
pp. 232470962110212
Author(s):  
Hassaan Jafri ◽  
Isna Khan ◽  
Adarsh Sidda ◽  
Noman Ahmed Jang Khan ◽  
Murad Kheetan ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Kidney Function ◽  
Central Nervous System Involvement ◽  
Kidney Injury ◽  
Immunoglobulin M ◽  
Dramatic Improvement ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
The Central Nervous System

Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma associated with a monoclonal immunoglobulin M protein. Extranodal involvement in WM is not very common. In this article, we present a rare case of WM with kidney and central nervous system involvement. Bing-Neel syndrome is a distinct complication of WM where lymphoplasmacytic cells involve the central nervous system (CNS). Our patient was initially treated with dialysis and steroids with improvement in his kidney function. He was then started on systemic treatment with rituximab, cyclophosphamide, and dexamethasone with stable kidney function but persistent CNS symptoms. Due to rarity of cases, there is no standard treatment for Bing-Neel syndrome. His treatment was switched to ibrutinib with dramatic improvement in his CNS symptoms as well as radiological findings on magnetic resonance imaging.

scholarly journals Central Nervous System Involvement by Waldenstrom Macroglobulinemia: A Case Report of the Bing–Neel Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Ananth Arjunan ◽  
Hema Rai
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Standard Dose ◽  
Rare Complication ◽  
Central Nervous System Involvement ◽  
Serum Markers ◽  
High Dose ◽  
Neurologic Symptoms ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia

Bing–Neel syndrome (BNS) is a rare complication of Waldenstrom macroglobulinemia (WM) defined by a lymphoplasmacytic infiltration of the central nervous system (CNS). Patients present with a range of neurologic symptoms of variable severity. Diagnosis requires a low threshold of suspicion and is considered in WM patients with unexplained neurologic symptoms. It can occur in the presence of quiescent serum markers of WM. Direct CNS tissue biopsy should be pursued if feasible and remains the gold standard for diagnosis. No standard of care treatment exists, but expert guidelines suggest intravenous chemotherapy in standard dose or high-dose regimens or use of oral ibrutinib. Consideration is also made for intravenous rituximab, intrathecal therapies, and autologous stem cell transplantation. Patient factors and tolerability should drive decisions regarding treatment choice in this arena, given a lack of data for standard frontline therapy.

scholarly journals Treatment of secondary central nervous system involvement in systemic aggressive B cell lymphoma using R-MIADD chemotherapy: a single-center study

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yuchen Wu ◽  
Xuefei Sun ◽  
Xueyan Bai ◽  
Jun Qian ◽  
Hong Zhu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Central Nervous System Lymphoma ◽  
Progression Free Survival ◽  
B Cell Lymphoma ◽  
High Dose ◽  
Cns Involvement ◽  
The Central Nervous System ◽  
Secondary Central Nervous System

Abstract Background Secondary central nervous system lymphoma (SCNSL) is defined as lymphoma involvement within the central nervous system (CNS) that originated elsewhere, or a CNS relapse of systemic lymphoma. Prognosis of SCNSL is poor and the most appropriate treatment is still undetermined. Methods We conducted a retrospective study to assess the feasibility of an R-MIADD (rituximab, high-dose methotrexate, ifosfamide, cytarabine, liposomal formulation of doxorubicin, and dexamethasone) regimen for SCNSL patients. Results Nineteen patients with newly diagnosed CNS lesions were selected, with a median age of 58 (range 20 to 72) years. Out of 19 patients, 11 (57.9%) achieved complete remission (CR) and 2 (10.5%) achieved partial remission (PR); the overall response rate was 68.4%. The median progression-free survival after CNS involvement was 28.0 months (95% confidence interval 11.0–44.9), and the median overall survival after CNS involvement was 34.5 months. Treatment-related death occurred in one patient (5.3%). Conclusions These single-centered data underscore the feasibility of an R-MIADD regimen as the induction therapy of SCNSL, further investigation is warranted.

Coxsackievirus Group B Antibodies in the Ventricular Fluid of Infants with Severe Anatomic Defects in the Central Nervous System

PEDIATRICS ◽  
1985 ◽  
Vol 76 (1) ◽  
pp. 64-68
Author(s):  
Charles J. Gauntt ◽  
Richard J. Gudvangen ◽  
Yves W. Brans ◽  
Arthur E. Marlin
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Newborn Infants ◽  
Neutralizing Antibody ◽  
Detectable Level ◽  
Immunoglobulin M ◽  
Congenital Infections ◽  
The Central Nervous System ◽  
Group B

Ventricular fluids from four of 28 newborn infants who were initially seen with severe congenital anatomic defects in the central nervous system contained neutralizing antibody to at least one serotype of coxsackieviruses group B. Two of the four infants with anticoxsackieviruses group B antibody in the ventricular fluid did not have a detectable level of the same antibody(ies) in their serum. The ventricular fluid of one of the infants had immunoglobulin M neutralizing antibody directed against coxsackievirus B6. Of 11 mother-infant pairs that had neutralizing antibody to coxsackieviruses group B in both sera, nearly half had antibodies directed against more than one serotype. These data suggest the possibility of an association between congenital infections with coxsackieviruses group B and rare severe CNS defects.

CHRONIC LEAD ENCEPHALOPATHY

PEDIATRICS ◽  
1960 ◽  
Vol 25 (2) ◽  
pp. 309-315
Author(s):  
Harry H. White ◽  
Fred D. Fowler
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Pediatric Patients ◽  
Early Recognition ◽  
Central Nervous System Involvement ◽  
Fast Screening ◽  
The Central Nervous System ◽  
Lead Encephalopathy ◽  
Urinary Coproporphyrin

Chronic lead encephalopathy must be considered in the differential diagnosis of pediatric patients who present with manifestations of schizophrenia, behavior disorders or degenerative diseases of the central nervous system. Determination of urinary coproporphyrin is a simple, fast screening procedure applicable to office practice. The prognosis for normal mental development following encephalopathy is poor. It is hoped that early recognition of the more subtle signs of central nervous system involvement will allow treatment to be instituted soon enough to prevent the crippling mental deterioration which is so often a sequela of lead poisoning.

Use of rituximab in lymphomatoid granulomatosis with isolated central nervous system involvement

2020 ◽  
Vol 13 (9) ◽  
pp. e235412
Author(s):  
Jesse Mooneyham ◽  
Cesar Gentille ◽  
Andrea Barbieri ◽  
Shilpan Shah
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Single Agent ◽  
Central Nervous System Involvement ◽  
Brain Lesions ◽  
The Body ◽  
Lymphomatoid Granulomatosis ◽  
Vasogenic Oedema ◽  
The Central Nervous System

A 33-year-old woman presented to the emergency room with severe headaches. A CT scan of the head revealed two brain lesions with associated vasogenic oedema. Diagnostic resection of one of the lesions followed by pathological analysis revealed grade III lymphomatoid granulomatosis (LYG). Staging investigations elsewhere in the body were negative, isolating this case of LYG to the central nervous system, an atypical presentation. After the resection, she was treated with single-agent rituximab 375 mg/m2. The follow-up MRI demonstrated the resolution of brain lesions and no progression of the disease.

Central nervous system involvement in goats undergoing primary infections with Trypanosoma brucei and relapse infections after chemotherapy

Parasitology ◽  
1985 ◽  
Vol 90 (2) ◽  
pp. 255-268 ◽  
Author(s):  
D. D. Whitelaw ◽  
J. E. Moulton ◽  
W. I. Morrison ◽  
M. Murray
Keyword(s):  
Central Nervous System ◽  
Drug Resistance ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Drug Treatment ◽  
Trypanosoma Brucei ◽  
Central Nervous System Involvement ◽  
Severe Illness ◽  
Cellular Infiltration ◽  
The Central Nervous System

Relapse of infection after trypanocidal drug treatment of trypanosome infections is normally attributed to drug resistance on the part of the parasite, under-dosage of the drug, or reinfection of the host. We have demonstrated relapse infections in goats arising from none of these. Fourteen goats infected with Trypanosoma brucei suffered severe illness and 3 died within 45 days. Despite treatment with the trypanocidal drug Berenil, a 4th goat died 2 days later. Recovery of the remainder followed chemotherapy, and in 2 goats, necropsiecl 45 days after treatment, no trypanosomes or abnormalities were detected. However 2–3 months after Berenil chemotherapy, despite trypanosomes being undetectable in the blood during the intervening period, infections in 4 of the remaining 8 animals relapsed. At all stages of the primary and relapse infections, trypanosomes isolated from the blood of the goats were completely susceptible to Berenil when tested in mice, as were parasites isolated from cerebrospinal fluid and brain tissue at necropsy. At the time of treatment, only minimal cellular infiltration was found in the central nervous system (CNS), but death from the relapse infection was associated with a very severe meningoencephalitis. We conclude that the relapse infections were caused by the re-emergence of trypanosornes from the CNS, where sequestered parasites were inaccessible to the trypanocidal effects of the drug.

scholarly journals Signs and Symptoms of Central Nervous System Involvement and Their Pathogenesis in COVID-19 According to The Clinical Data (Review)

2021 ◽  
Vol 17 (3) ◽  
pp. 65-77
Author(s):  
N. V. Tsygan ◽  
A. P. Trashkov ◽  
A. V. Ryabtsev ◽  
V. A. Yakovleva ◽  
A. L. Konevega ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Data ◽  
Nervous Tissue ◽  
Central Nervous System Involvement ◽  
Signs And Symptoms ◽  
Neurological Manifestations ◽  
Altered Level ◽  
System Involvement ◽  
The Central Nervous System

Detailed clinical assessment of the central nervous system involvement in SARS-CoV-2 infection is relevant due to the low specificity of neurological manifestations, the complexity of evaluation of patient complaints, reduced awareness of the existing spectrum of neurological manifestations of COVID-19, as well as low yield of the neurological imaging.The aim. To reveal the patterns of central nervous system involvement in COVID-19 and its pathogenesis based on clinical data.Among more than 200 primary literature sources from various databases (Scopus, Web of Science, RSCI, etc.), 80 sources were selected for evaluation, of them 72 were published in the recent years (2016-2020). The criteria for exclusion of sources were low relevance and outdated information.The clinical manifestations of central nervous system involvement in COVID-19 include smell (5-98% of cases) and taste disorders (6-89%), dysphonia (28%), dysphagia (19%), consciousness disorders (3-53%), headache (0-70%), dizziness (0-20%), and, in less than 3% of cases, visual impairment, hearing impairment, ataxia, seizures, stroke. Analysis of the literature data revealed the following significant mechanisms of the effects of highly contagious coronaviruses (including SARS-CoV-2) on the central nervous system: neurodegeneration (including cytokine- induced); cerebral thrombosis and thromboembolism; damage to the neurovascular unit; immune-mediated damage of nervous tissue, resulting in infection and allergy-induced demyelination.The neurological signs and symptoms seen in COVID-19 such as headache, dizziness, impaired smell and taste, altered level of consciousness, bulbar disorders (dysphagia, dysphonia) have been examined. Accordingly, we discussed the possible routes of SARS-CoV-2 entry into the central nervous system and the mechanisms of nervous tissue damage.Based on the literature analysis, a high frequency and variability of central nervous system manifestations of COVID-19 were revealed, and an important role of vascular brain damage and neurodegeneration in the pathogenesis of COVID-19 was highlighted.

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