Multifocal Cerebral Venous Malformations and Associated Developmental Venous Anomalies in a Case of Blue Rubber Bleb Nevus Syndrome

We report a sporadic case of probable BRBN (blue rubber bleb nevus syndrome) with multiple CNS (central nervous system) involvement. These features consisted of multiple VMs (venous malformations) and DVAs (developmental venous anomalies) in supratentorial brain, cerebellum, and diencephalon. Since its first description by Bean, there have been many cases of BRBN manifesting with gastrointestinal bleeding with or without associated hemorrhage. Cases with CNS involvement were rarely reported and many of the descriptions were confusing with different terminologies used to describe them such as capillary venous malformation, hemangiomas, and vascular malformations. The lesions illustrated are venous malformations similar to our case. The association of DVA was recognized in some cases; they are likely to be underestimated when revisiting the published case illustrations. Although our case is sporadic, the link with HHT1 is unlikely despite the involvement of the same chromosome (Ch 9).

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Developmental venous anomalies and sinus pericranii in the blue rubber-bleb nevus syndrome

Journal of Neurosurgery ◽

10.3171/jns.2003.99.2.0409 ◽

2003 ◽

Vol 99 (2) ◽

pp. 409-411 ◽

Cited By ~ 26

Author(s):

Patrik Gabikian ◽

Richard E. Clatterbuck ◽

Philippe Gailloud ◽

Danielle Rigamonti

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Cerebral Angiography ◽

Developmental Disorder ◽

Vascular Malformations ◽

Content Type ◽

Sinus Pericranii ◽

Venous Anomalies ◽

Developmental Venous Anomalies ◽

Fine Print

✓ Blue rubber-bleb nevus syndrome (BRBNS) is a developmental disorder that originally was identified by the presence of distinctive cutaneous and gastrointestinal hemangiomas. More recently it has been recognized that the number of affected organs is larger and that BRBNS includes central nervous system vascular malformations. A 52-year-old woman in whom intracranial vascular malformations had been diagnosed earlier presented for evaluation. At birth, several blue nevi had been noted on her tongue, lips, and neck. Cerebral angiography demonstrated an extensive network of developmental venous anomalies and a left anterior sinus pericranii. The literature on BRBNS and developmental venous anomalies is reviewed.

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Orbital Lymphatic-Venous Malformation Accompanied by an Intraocular Vascular Malformation: A Rare Case Study

Case Reports in Ophthalmology ◽

10.1159/000515272 ◽

2021 ◽

pp. 396-401

Author(s):

Karny Shouchane-Blum ◽

Iftach Yassur ◽

Amir Sternfeld ◽

Miriam Regev ◽

Orly Gal-Or ◽

...

Keyword(s):

Vascular Malformation ◽

Rare Case ◽

Vascular Malformations ◽

Venous Malformation ◽

Female Infant ◽

Venous Malformations ◽

Venous Anomalies ◽

Developmental Venous Anomalies ◽

The Right

Lymphatic-venous malformations (LVMs) are development defects that result in abnormal connections between the lymphatic and venous systems. The authors describe a 7-weeks-old female infant who presented with a right orbital LVM extending to the ipsilateral cheek and subconjunctiva of the right eye, intracranial developmental venous anomalies in the right cerebellum, and a significant right eye intraocular retinal vascular malformation. Since orbital LVM is usually diagnosed in infancy or childhood, pediatric ophthalmologists should actively look for intraocular vascular malformations as such findings can poorly affect a patient’s vision.

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

Chinese Neurosurgical Journal ◽

10.1186/s41016-021-00238-0 ◽

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.

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Comparison of clinical features of pulmonary cryptococcosis with and without central nervous system involvement in China

Journal of International Medical Research ◽

10.1177/0300060521991001 ◽

2021 ◽

Vol 49 (2) ◽

pp. ???

Author(s):

Xinying Xue ◽

Xuelei Zang ◽

Lifeng Wang ◽

Dongliang Lin ◽

Tianjiao Jiang ◽

...

Keyword(s):

Risk Factors ◽

Central Nervous System ◽

Nervous System ◽

Clinical Features ◽

Central Nervous System Involvement ◽

Pulmonary Cryptococcosis ◽

Cns Involvement ◽

Laboratory Findings ◽

Logistic Regression Models ◽

Presenting Symptoms

Objective This study aimed to compare the clinical features of pulmonary cryptococcosis (PC) in patients with and without central nervous system (CNS) involvement. Methods We retrospectively reviewed demographics, presenting symptoms, radiographic features, and laboratory findings of patients diagnosed with PC in 28 hospitals from 2010 to 2019. Risk factors for CNS involvement were analyzed using logistic regression models. Result A total of 440 patients were included, and 36 (8.2%) had CNS involvement. Significant differences in fever, headache, and chills occurred between the two groups (overall and with/without CNS involvement) for fever (17.8% [78/440]; 52.8% vs. 14.6% of patients, respectively), headache (4.5% [20/440]; 55.6% vs. 0% of patients, respectively), and chills (4.3% [19/440]; 13.9% vs. 3.5% of patients, respectively). The common imaging manifestation was nodules (66.4%). Multivariate analysis showed that cavitation (adjusted odds ratio [AOR] = 3.552), fever (AOR = 4.182), and headache were risk factors for CNS involvement. Routine blood tests showed no differences between the groups, whereas in cerebrospinal fluid the white blood cell count increased significantly and glucose decreased significantly. Conclusion In patients with PC, the risk of CNS involvement increases in patients with headache, fever, and cavitation; these unique clinical features may be helpful in the diagnosis.

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Central nervous system involvement in Chagas' disease: an updating

Revista do Instituto de Medicina Tropical de São Paulo ◽

10.1590/s0036-46651993000200001 ◽

1993 ◽

Vol 35 (2) ◽

pp. 111-116 ◽

Cited By ~ 42

Author(s):

José Eymard Homem Pittella

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Chagas Disease ◽

Acute Phase ◽

Central Nervous System Involvement ◽

Cns Infection ◽

Cns Involvement ◽

Anatomical Basis ◽

Immunosuppressed Patients ◽

Acute Form

A review was made of the available literature on central nervous system (CNS) involvement in Chagas' disease. Thirty-one works concerning the acute nervous form and 17 others dealing with the chronic nervous form, all presenting neuropathologic studies, were critically analysed. Based on this analysis, an attempt was made to establish the possible natural history of CNS involvement in Chagas' disease. Among others, the following facts stand out: 1) the initial, acute phase of Trypanosoma cruzi infection is usually asymptomatic and subclinical; 2) only a small percentage of cases develop encephalitis in the acute phase of Chagas' disease; 3) the symptomatic acute forms accompanied by chagasic encephalitis are grave, with death ensuing in virtually all cases as a result of the brain lesions per se or of acute chagasic myocarditis, this being usually intense and always present; 4) individuals with the asymptomatic acute form and with the mild symptomatic acute form probably have no CNS infection or, in some cases, they may have discrete encephalitis in sparse foci. In the latter case, regression of the lesions may be total, or residual inflammatory nodules of relative insignificance may persist. Thus, no anatomical basis exists that might characterize the existence of a chronic nervous form of Chagas' disease; 5) reactivation of the CNS infection in the chronic form of Chagas' disease is uncommon and occurs only in immunosuppressed patients.

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Central Nervous System Involvement in Adult Acute Lymphoblastic Leukemia (ALL) at Diagnosis and/or at First Relapse: Results from the GET-LALA Group.

Blood ◽

10.1182/blood.v110.11.4326.4326 ◽

2007 ◽

Vol 110 (11) ◽

pp. 4326-4326

Author(s):

Oumedaly Reman ◽

Arnaud Pigneux ◽

Francoise Huguet ◽

Norbert Vey ◽

Andre Delannoy ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Lymphoblastic Leukemia ◽

Central Nervous System Involvement ◽

Unrelated Donor ◽

Cns Involvement ◽

Cns Disease ◽

Cns Relapse ◽

First Relapse ◽

B Lineage

Abstract Outcome of adult ALL with central nervous system (CNS) involvement is not clearly defined. We studied 104 patients presenting with CNS involvement at diagnosis among 1493 patients (7%) included into the LALA-87 or LALA-94 trials, and 109 patients (9% of first remitters) presenting CNS disease at the time of first relapse among the 709 relapsing patients (15%) included initially in these trials. Treatment of patients presenting CNS involvement at diagnosis consisted in initial chemotherapy completed by 18 double or triple intrathecal injections associated with 15 to 20 Gy cranial irradiation, followed when possible by intensification by allogeneic or autologous stem cell transplantation (SCT). At diagnosis, 43 patients (41%) presenting with CNS involvement had T-lineage ALL, 53 (51%) had B-lineage ALL (of whom 9 were diagnosed as Philadelphia (Ph) chromosome positive ALL), 8 had undifferentiated ALL or unknown immunophenotype. Eighty-seven of 104 (84%) patients with CNS involvement at diagnosis achieved complete remission (CR). Fifty-three patients underwent SCT (25 allogeneic SCT from matched related or unrelated donor, 28 autologous SCT). Overall survival at 7 years was 34% in those with CNS involvement at diagnosis versus 29% (p = NS) for those without. DFS at 7 years was 35% versus 28% (p = NS). There were no significant differences between patients with CNS involvement and those without CNS involvement regarding T lineage ALL, B lineage ALL (including or not Ph ALL). There were also no significant differences regarding patients who underwent transplantation as consolidation intensification, while in patients receiving only chemotherapy patients without initial CNS involvement had a better outcome (p = 0.01). Among the 709 patients with primary relapse, 66 patients (61%) presented a CNS relapse combined with bone marrow relapse, whereas 17 relapses (15%) and 26 relapses (24%) were CNS relapses combined with another extramedullary relapse or isolated CNS relapses respectively. Median time to relapse was 6.7 months (range, 1–62) in patients with CNS relapse versus 11.2 months (1.7–111) in relapsing patients without CNS involvement. Eleven patients (10%) with CNS relapse had CNS involvement at diagnosis, while 98 patients were diagnosed with CNS disease only at the time of first relapse. Overall, 38 out of 109 patients with CNS relapse (35%) achieved CR. The median OS was 6.3 months. Outcome was similar in terms of CR proportion and OS in relapsing patients without CNS involvement. The 2-year OS rates did not show any difference among patients with CNS relapse who had CNS involvement at diagnosis and those with CNS disease only diagnosed at the time of first relapse.Overall, CNS leukemia in adult ALL is uncommon at diagnosis. Patients have a similar outcome than those who did not present with CNS involvement. However, patients benefit from intensification therapy by autologous or allogeneic SCT. CNS leukemia at first relapse are also uncommon but probably underestimated. Outcome is particularly poor as this of all adult ALL in first relapse.

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Central Nervous System Involvement in Henoch-Schonlein Purpura in Children and Adolescents

Case Reports in Pediatrics ◽

10.1155/2017/5483543 ◽

2017 ◽

Vol 2017 ◽

pp. 1-6 ◽

Cited By ~ 2

Author(s):

Iliyana H. Pacheva ◽

Ivan S. Ivanov ◽

Krastina Stefanova ◽

Elena Chepisheva ◽

Lyubov Chochkova ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Arterial Hypertension ◽

Rare Complication ◽

Central Nervous System Involvement ◽

Henoch Schonlein Purpura ◽

Cns Involvement ◽

Hyperintense Lesion ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009–2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9%) had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES), which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.

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

Case Reports in Hematology ◽

10.1155/2015/689423 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5 ◽

Cited By ~ 1

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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Extremely High Levels of Central Nervous System Involvement in Miliary Tuberculosis

10.21203/rs.3.rs-696092/v1 ◽

2021 ◽

Author(s):

Guirong Wang ◽

Ruixia Liang ◽

Qing Sun ◽

Xinlei Liao ◽

Chenqian Wang ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Miliary Tuberculosis ◽

Central Nervous System Involvement ◽

Cns Involvement ◽

Molecular Tests ◽

Cerebral Computed Tomography ◽

Magnetic Resonance Imaging Mri ◽

Almost All ◽

Csf Examination

Abstract BackgroundMiliary tuberculosis (TB) is one of the severest manifestations of TB that can be lethal when concomitant with the central nervous system (CNS) involvement. Bacteriological, biochemical and radiological methods for find CNS comorbidity in miliary TB was evaluated in this study.MethodsConsecutive miliary TB adults were retrospectively enrolled from two designated TB hospitals in China. The capacities of examinations of cerebrospinal fluid (CSF), cerebral computed tomography (CT) and magnetic resonance imaging (MRI) for diagnosis of CNS involvement were assessed.ResultsAssessment of CNS involvement with a lumbar puncture and/or neuroimaging was undertaken in 282 out of 392 of acute miliary TB. Of these 282 patients, 87.59% (247/282) had CNS involvement. Cerebral contrast-enhanced MRI (96.05%, 170/177) and MRI (93.15%, 204/219) yielded significantly higher sensitivities over CSF examination (71.92%, 146/203, P<0.001) and CT (34.69%, 17/49, P<0.001). The sensitivity of CSF examination was superior to CT scan (P<0.001). Although 59.65% (134/225) miliary TB patients acquired bacteriological evidence with sputum examination, the positivity was only 8.82% (21/238) for CSF examination by conventional and molecular tests.ConclusionAlmost all miliary TB had CNS involvement and MRI demonstrated outstanding potential over other methods. Therefore, a routinely screening of CNS TB should be strongly suggested in miliary TB and MRI could be used as the initial approach in resources rich settings.

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Central Nervous System Involvement under Intensive Immunosuppressive Treatment in a Patient Diagnosed with Granulomatosis Polyangiitis: A Case Report

BANTAO Journal ◽

10.1515/bj-2015-0011 ◽

2015 ◽

Vol 13 (1) ◽

pp. 48-50

Author(s):

Zeynep Kendi Celebi ◽

Orhan Kucuksahin ◽

Elif Peker ◽

Sim Kutlay ◽

Gokhan Nergizoglu ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Leukocytoclastic Vasculitis ◽

Immunosuppressive Treatment ◽

Central Nervous System Involvement ◽

Respiratory Arrest ◽

Upper Respiratory Tract ◽

Cns Involvement ◽

System Involvement ◽

Pulse Steroid

Abstract Granulomatosis polyangiitis (Wegener's granulomatosis) is an ANCA-associated necrotising vasculitis. The disease involves upper respiratory tract, the lungs and kidneys but central nervous system (CNS) involvement is 1-5%. A 40-year-old male patient was admitted to the hospital with joint pain, rash, aphthous lesions. The skin biopsy from the lesion showed leukocytoclastic vasculitis. The patient had c-ANCA positive and was diagnosed granulomatosis polyangiitis. He was treated with a pulse steroid and cyclophosphamide. Before the 5th session of therapy, the patient developed hemoptysis and hematuria. Thorax CT (computarized tomography) showed a diffuse alveolar hemorrhage and hence plasmapheresis and IVIG (intravenous immunoglobulin) were added to the treatment. Two days after IVIG, the patient developed globe vesical, headache and respiratory arrest. MR (magnetic resonance) showed CNS involvement. The patient was treated with a pulse steroid, but did not respond to therapy and died after 5 months since establishing the diagnosis. More studies are needed to identify effective treatment and course of disease for patients with central nervous system involvement.

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