Solitary pituitary sarcoidosis with normal endocrine function

2008 ◽  
Vol 108 (3) ◽  
pp. 591-594 ◽  
Author(s):  
Promod Pillai ◽  
Abhik Ray-Chaudhury ◽  
Mario Ammirati ◽  
E. Antonio Chiocca
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Biopsy Specimen ◽  
English Literature ◽  
Systemic Disease ◽  
Definitive Diagnosis ◽  
Endocrine Function ◽  
Granulomatous Disease ◽  
Cns Involvement ◽  
The Central Nervous System

✓ Sarcoidosis is a multisystemic granulomatous disease characterized by noncaseating epithelioid granulomata that affects the lung in over 90% of patients and the central nervous system (CNS) in 5–9%. Neurosarcoidosis often occurs as multifocal meningeal and parenchymal lesions, and its diagnosis is particularly difficult in the absence of concomitant systemic disease. Hypothalamic-pituitary sarcoidosis occurs in fewer than 10% of patients with neurosarcoidosis and has been previously reported in association with profound endocrinological dysfunction. The authors report the case of a patient with isolated pituitary sarcoidosis who was first evaluated for visual symptoms and showed no preoperative endocrinological dysfunction or evidence of multisystemic or other CNS involvement. To the authors' knowledge, only 1 other such presentation is previously reported in the English literature. Such presentations are diagnostically and therapeutically challenging, and definitive diagnosis requires obtaining a biopsy specimen of the lesion with histological proof of noncaseating epithelioid granuloma, as well as the exclusion of other possible entities.

scholarly journals Localized Relapse of Primary Plasma Cell Leukaemia in the Central Nervous System

2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Christian W. Eskelund ◽  
Niels Frost Andersen
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Plasma Cell ◽  
Poor Prognosis ◽  
Systemic Disease ◽  
Cell Leukaemia ◽  
Cns Involvement ◽  
Aggressive Form ◽  
Plasma Cell Leukaemia ◽  
The Central Nervous System

Primary plasma cell leukaemia (pPCL) is a rare and aggressive form of plasma cell malignancies with a very poor prognosis. Compared to other plasma cell malignancies the tendency to extramedullary spread is increased; however central nervous system (CNS) involvement is rare and only reported in few cases. We report the case of a 61-year-old man who was diagnosed with pPCL and achieved a complete remission after autologous stem cell transplantation but had a relapse in the CNS without systemic disease.

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.

scholarly journals Dying to Fall Asleep

2019 ◽  
pp. 724-742
Author(s):  
Jessica Vensel Rundo ◽  
Hillor Mehta ◽  
Reena Mehra
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Genetic Analysis ◽  
Genetic Disease ◽  
Prion Proteins ◽  
Amyloid Plaques ◽  
Definitive Diagnosis ◽  
Common Features ◽  
The Central Nervous System ◽  
Autonomic Hyperactivity

Fatal familial insomnia (FFI) is a rare autosomal dominant genetic disease characterized by progressive insomnia, autonomic hyperactivity, memory deficits, hallucinations, and myoclonus. Unlike its name, insomnia is not the most common initial presentation in patients with FFI. More common features like autonomic hyperactivity (hypertension and tachycardia) are often missed, delaying the diagnosis of FFI. Genetic analysis of FFI shows a D178N-129M mutation that results in generation of insoluble proteins (prion proteins) that aggregate to form amyloid plaques, leading to deterioration of the central nervous system, particularly in the hypothalamus. This case illustrates the difficulty in determining a definitive diagnosis in patients with FFI. Unfortunately, no treatment or cure is available for FFI. The disease is fatal in all the patients.

scholarly journals Frequency and Severity of Central Nervous System Lesions in Hemophagocytic Lymphohistiocytosis

Blood ◽  
1997 ◽  
Vol 89 (3) ◽  
pp. 794-800 ◽  
Author(s):  
Elie Haddad ◽  
Maria-Luisa Sulis ◽  
Nada Jabado ◽  
Stephane Blanche ◽  
Alain Fischer ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Disease Progression ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Systemic Disease ◽  
Neurological Symptoms ◽  
Treatment Modalities ◽  
Remission Induction ◽  
Cns Involvement ◽  
Cns Disease

Abstract We have retrospectively assessed the neurological manifestations in 34 patients with hemophagocytic lymphohistiocytosis (HLH) in a single center. Clinical, radiological, and cerebrospinal fluid (CSF ) cytology data were analyzed according to treatment modalities. Twenty-five patients (73%) had evidence of central nervous system (CNS) disease at time of diagnosis, stressing the frequency of CNS involvement early in the time course of HLH. Four additional patients who did not have initial CNS disease, who did not die early from HLH complications, and who were not transplanted, also developed a specific CNS disease. Therefore, all surviving and nontransplanted patients had CNS involvement. Initially, CNS manifestations consisted of isolated lymphocytic meningitis in 20 patients and meningitis with clinical and radiological neurological symptoms in nine patients. For these nine patients, neurological symptoms consisted of seizures, coma, brain stem symptoms, or ataxia. The outcome of patients treated by systemic and intrathecal chemotherapy and/or immunosuppression exclusively (n = 16) was poor, as all died following occurrence of multiple relapses or CNS disease progression in most cases. Bone marrow transplantation (BMT) from either an HLA identical sibling (n = 6) or haplo identical parent (n = 3) was performed in nine patients, once first remission of CNS and systemic disease was achieved. Seven are long-term survivors including three who received an HLA partially identical marrow. All seven are off treatment with normal neurological function and cognitive development. In four other patients, BMT performed following CNS relapses was unsuccessful. Given the frequency and the poor outcome of CNS disease in HLH, BMT appears, therefore, to be the only available treatment procedure that is capable of preventing HLH CNS disease progression and that can result in cure when performed early enough after remission induction.

scholarly journals Solitary tentorial sarcoid granuloma associated with Propionibacterium acnes infection: case report

2017 ◽  
Vol 127 (3) ◽  
pp. 687-690 ◽  
Author(s):  
Jiro Akimoto ◽  
Kenta Nagai ◽  
Daisuke Ogasawara ◽  
Yujiro Tanaka ◽  
Hitoshi Izawa ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Immune Response ◽  
Nervous System ◽  
Case Report ◽  
Propionibacterium Acnes ◽  
Granulomatous Disease ◽  
Tentorium Cerebelli ◽  
First Case ◽  
Sarcoid Granuloma ◽  
The Central Nervous System

Sarcoidosis is a systemic granulomatous disease with unknown cause, which very rarely occurs exclusively in the central nervous system. The authors performed biopsy sampling of a mass that developed in the left tentorium cerebelli that appeared to be a malignant tumor. The mass was diagnosed as a sarcoid granuloma, which was confirmed with the onset of antibody reaction product against Propionibacterium acnes. Findings suggesting sarcoidosis to be an immune response to P. acnes infection have recently been reported, and they give insight for diagnosis and treatment of this disease. The authors report the possible first case that was confirmed with P. acnes infection in a meningeal lesion in solitary neurosarcoidosis.

Changes in Endocrine Function after Adrenal Medullary Transplantation to the Central Nervous System*

1990 ◽  
Vol 71 (3) ◽  
pp. 773-776 ◽  
Author(s):  
NELSON B. WATTS ◽  
RICHARD V. CLARK ◽  
RAY L. WATTS ◽  
SAM D. GRAHAM ◽  
ROY A. E. BAKAY
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Endocrine Function ◽  
The Central Nervous System

scholarly journals RARE-42. INCIDENCE AND BEHAVIOR OF CENTRAL NERVOUS SYSTEM INVOLVEMENT IN PATIENTS WITH HODGKIN’S LYMPHOMA

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi230-vi230
Author(s):  
Carlos Eduardo Silva Correia ◽  
Rachna Malani ◽  
Lisa DeAngelis ◽  
Alison Moskowitz
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Median Time ◽  
Hodgkin’S Lymphoma ◽  
Systemic Disease ◽  
Hodgkin's Lymphoma ◽  
Leptomeningeal Disease ◽  
High Dose ◽  
Cns Involvement ◽  
Cns Disease

Abstract INTRODUCTION Central nervous system (CNS) involvement from Hodgkin’s Lymphoma (HL) is rare, with a reported incidence of 0.07–0.5%. There is a paucity of data regarding its natural history and management. METHODS In this retrospective single-institution review, we analyzed all adult patients with HL for CNS involvement (parenchymal or meningeal), who were evaluated at Memorial Sloan Kettering Cancer Center from January 2008 until December 2018. RESULTS A total of 3478 patients with HL were identified, and CNS involvement was found in 10 patients (0.3%). All patients were symptomatic from CNS disease. Four patients had a synchronous presentation. The other 6 patients had a median time from systemic diagnosis to CNS involvement of 8 years (2.5–14). Two patients had radiographic evidence of leptomeningeal disease, however 4 had positive cerebrospinal fluid (CSF) cytology. At time of CNS involvement, 2 patients had confirmed transformation to Non-Hodgkin-Lymphoma on biopsy. 2 patients had EBV-positive HL. One patient died before treatment. Five patients received high-dose methotrexate (HD-MTX) for CNS disease. Of these patients, 1 died during treatment, 2 had partial responses, and 2 had complete response of both systemic and CNS disease; to date they are in remission. Three patients had varied responses to immunotherapy and cytotoxic chemotherapy. Removal of an immunosuppressive agent resolved disease in one EBV-positive patient. Median overall survival (OS) from diagnosis of HL was 10.6 years (1.1–21.2), and OS from time to CNS involvement was 6 years (0.2–15). CONCLUSION The median time from diagnosis to CNS involvement, and OS from time of CNS involvement are higher than previously reported, which may be related to newer therapies for systemic disease. Neuroimaging should be used in conjunction with CSF for diagnostic accuracy. HD-MTX can be used to treat CNS and refractory systemic disease, as historically used for the latter.

Involvement of the Central Nervous System in Non-Hodgkin's Lymphoma

1981 ◽  
Vol 67 (1) ◽  
pp. 39-44 ◽  
Author(s):  
Gian Luigi Cetto ◽  
Antonio Iannucci ◽  
Diego Tummarello ◽  
Nicola Rizzuto ◽  
Renata Sabbioni ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Bone Marrow ◽  
Nervous System ◽  
High Risk ◽  
Cns Lymphoma ◽  
High Grade ◽  
Cns Involvement ◽  
The Central Nervous System ◽  
Grade Malignancy ◽  
High Grade Malignancy

Fifteen of 146 (10 %) adult patients with non-Hodgkin's lymphoma showed clinical and pathologic evidence of involvement of the central nervous system (CNS); in 6 patients, the CNS lymphoma was present at the onset of disease, in 3 of them it was the only sign detected. In the remaining 9 cases, CNS involvement appeared during the course of systemic disease. In all cases symptoms related to infiltration of the CNS were associated with advanced disease (stage IV); bone marrow or bone involvement was found in 9 patients (60 %). The histologic subtypes were mostly of high-grade malignancy according to the Kiel classification: immunoblastic (3), centroblastic (3), Burkitt type (2), lymphoblastic (1), LP immunocytoma in polymorphic variant (3), unclassifiable (3). The prominent signs and symptoms of CNS lymphoma are listed: the cranial nerve palsies are the most common finding. The principal means of detecting CNS involvement are discussed: cerebrospinal fluid cytology, brain scan and CAT scan were the most useful diagnostic procedures. The reported data allow identification of patients at high risk of CNS lymphoma: this includes histologies of high-grade malignancy, advanced stage of disease, and bone marrow or bone infiltration. Therefore, either intensive systemic chemotherapy or CNS prophylaxis are recommended for patients with high risk of CNS disease.

scholarly journals Successful Bosutinib Experience in an Elderly Acute Lymphoblastic Leukemia Patient with Suspected Central Nervous System Involvement Transformed from Chronic Myeloid Leukemia

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Erden Atilla ◽  
Pinar Ataca ◽  
Elif Ozyurek ◽  
Ilhan Erden ◽  
Gunhan Gurman
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Lymphoblastic Leukemia ◽  
Central Nervous System Involvement ◽  
Limited Data ◽  
Cns Involvement ◽  
Blast Phase ◽  
The Central Nervous System

Managing the blast phase in chronic myeloid leukemia (CML) is challenging because limited data are available for elderly patients. The involvement of the central nervous system (CNS) increases the risk of a poor prognosis. Here, we present an elderly blast phase CML patient with suspected CNS involvement who was successfully treated with bosutinib.

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