P14.93 Partial stiff-person syndrome, cerebral lymphoma, and HIV infection

2021 ◽  
Vol 23 (Supplement_2) ◽  
pp. ii55-ii56
Author(s):  
R Ivan ◽  
B Cîrciumaru ◽  
C M Poșircă ◽  
C A Sîrbu

Abstract BACKGROUND Stiff person syndrome (SPS) is a rare entity, characterized by rigidity or stiffness of the muscles. It is usually autoimmune, but it can also appear as a paraneoplastic (5%) or cryptogenic disorder. This study aims to establish a potential connection between what appears to be a partial SPS, an advanced HIV infection, and a primary central nervous system (CNS) lymphoma in a young adult. MATERIAL AND METHODS We present the case of a 42-year-old male, who, in the course of approximately five months developed dizziness, impairment of manual dexterity, ataxia, postural instability, hallucinations, weight loss, and fever. He was admitted to our clinic, after a progressive deterioration of his mental status, presenting global aphasia, and increased left hemibody muscle tone, with persistent left torticollis. According to the blood tests and cerebral MRIs he was diagnosed with HIV infection, thrombophilia, and a left frontal mass, of unknown etiology. RESULTS His neurocognitive deterioration was suggestive of a primary CNS lymphoma and the ipsilateral abnormal increased muscular tone raised the suspicion of a partial SPS. It appeared most likely in the context of the brain lesion, even if its nature remained unknown, due to the deteriorated status, that made biopsy impossible. The diagnosis of cerebral lymphoma was based on the IRM characteristics and the change in appearance after corticosteroid therapy. CONCLUSION Even if it represents a rare diagnosis, partial SPS can appear as a paraneoplastic entity, our patient’s evolution and clinical examination being suggestive for this pathology.

2012 ◽  
Vol 114 (9) ◽  
pp. 1217-1221 ◽  
Author(s):  
Yoshiko Okita ◽  
Yoshitaka Narita ◽  
Yasuji Miyakita ◽  
Makoto Ohno ◽  
Shintaro Fukushima ◽  
...  

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 2008-2008 ◽  
Author(s):  
Antonio Marcilio Padula Omuro ◽  
Denise Correa ◽  
Craig Moskowitz ◽  
Matthew J. Matasar ◽  
Lisa Marie DeAngelis ◽  
...  

2008 Background: In our previous study in newly diagnosed PCNSL, induction chemotherapy with MTX and cytarabine followed by consolidation HDC (carmustine, etoposide, cytarabine, melphalan [BEAM]) with ASCT without radiotherapy resulted in only 50% of pts transplanted, reflecting low efficacy of induction chemotherapy, and short intent-to-treat (ITT) median PFS (=6m). In this phase II trial, we sought to optimize this strategy by utilizing a more effective induction regimen (R-MPV) and a more aggressive HDC regimen (Soussain et al). Methods: Pts received 5-7 cycles of R-MPV (MTX: 3.5g/m2) and if a partial or complete response was achieved, HDC with thiothepa, cyclophosphamide and busulfan was given, followed by ASCT and no radiotherapy. The primary endpoint was ITT 1 year event-free survival (promising: 75%, non-promising: 50%; 90% power, significance=0.05). Follow-up included comprehensive neuropsychological evaluation. Results: Accrual has been completed (N=32 pts, median age 57 [range 23-67], median KPS=80). Following R-MPV, 17 pts achieved a CR, 13 pts a PR and two pts progressed. A total of 25 (78%) pts were transplanted; the reasons for not receiving transplant were progressive disease (N=2), poor performance status/ physician’s decision (N= 2), mobilization failure (N=1) and consent withdrawn (N= 2). One pt who withdrew consent relapsed and received HDCASCT for salvage. Two (8%) pts died from early complications of ASCT (Stevens-Johnson: one, sepsis: one) and one pt experienced a fatal late colitis of unknown etiology. In the ITT population, the median EFS and OS have not been reached after a median follow-up of 22 months. The 1 year EFS was 78% (95%CI 58-90) and the 2y OS was 76% (95% CI 54-89). No pt has developed delayed neurotoxicity. Conclusions: R-MPV induction regimen resulted in improved response rates, allowing 78% of pts to receive HDC-ASCT. Although more toxic, this regimen resulted in excellent disease control and survival in the ITT population, far exceeding the efficacy of our previous transplant study. The primary endpoint was met, warranting further investigation.


2020 ◽  
Vol 27 ◽  
pp. 00128
Author(s):  
Eugene Marin ◽  
Valery Ermolaev ◽  
Oksana Marina ◽  
Elena Rezaeva

The article presents the results of treatment of aseptic meningoencephalitis in dogs of dwarf breeds. Diagnostics included a thorough neurological examination: assessment of mental status, ability to perform diagnostic tests, proprioceptive and postural reaction, the consistency and symmetry of cranial reflexes, including of pupil response to light and response to the threat, the presence of involuntary movements and forced poses, the intensity of the muscle tone and tendon reflexes. If an animal is suspected of having a brain lesion, other possible causes of encephalopathy were excluded. For this purpose, a General blood test was performed (to detect signs of inflammation, anemia); a detailed biochemical blood test (excluding renal and hepatic encephalopathy, indirect signs of the presence of a portosystemic shunt); if a portosystemic shunt was suspected, a blood test for bile acids was performed; when excluding all other causes of damage to the central nervous system. Magnetic resonance imaging of the brain was performed with contrast with the use of the drug OmniScan. During the magnetic resonance imaging, multiple foci were detected in all parts of the brain, including in the thalamus and trunk; including in some animals, foci that have a tendency to decay and form a necrotic cavity. It was established that before the start of treatment, animals had a wobbly gait, convulsive signs, a comatose state in some dogs, and weak visual reflexes. As a result of studies of morphological and biochemical parameters of blood in dogs, it was found that all the data obtained were within the reference values and did not have significant significance in the diagnosis of aseptic meningoencephalitis. Against the background of etiotropic and symptomatic therapy, positive dynamics was observed in patients with aseptic meningoencephalitis.


Author(s):  
Roberto Jose Diaz ◽  
Gregory W. Basil ◽  
Ricardo J. Komotar

Central nervous system (CNS) lymphoma must be considered in the differential diagnosis of any immunocompromised patient with a solid brain lesion. In such patients, diagnosis can be made via a careful review of important signs, symptoms, and classic radiologic findings. While there is no single physical exam finding classic for lymphoma, the clinician must carefully evaluate patients for the presence or absence of findings that may suggest an alternative diagnosis. Such findings include the stigmata of endocarditis, symptoms suggestive of pneumonia, or additional non-CNS mass lesions. Additionally, several imaging modalities including magnetic resonance imaging, diffusion-weighted magnetic resonance imaging, susceptibility weighted imaging, and dynamic contrast-enhanced imaging can be useful in identifying this condition. While steroids can be helpful in reducing the disease burden and decreasing edema, they may also hinder diagnosis. Surgery may be indicated for either diagnostic or decompressive purposes; however, the mainstay of treatment is chemotherapeutic and immunotherapeutic agents with radiation reserved for refractory cases.


2017 ◽  
Vol 35 (21) ◽  
pp. 2410-2418 ◽  
Author(s):  
Christian Grommes ◽  
Lisa M. DeAngelis

Primary CNS lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma that is typically confined to the brain, eyes, and cerebrospinal fluid without evidence of systemic spread. The prognosis of patients with PCNSL has improved during the last decades with the introduction of high-dose methotrexate. However, despite recent progress, results after treatment are durable in half of patients, and therapy can be associated with late neurotoxicity. PCNSL is an uncommon tumor, and only four randomized trials and one phase III trial have been completed so far, all in the first-line setting. To our knowledge, no randomized trial has been conducted for recurrent/refractory disease, leaving many questions unanswered about optimal first-line and salvage treatments. This review will give an overview of the presentation, evaluation, and treatment of immunocompetent patients with PCNSL.


Neurosurgery ◽  
1987 ◽  
Vol 21 (6) ◽  
pp. 920-923 ◽  
Author(s):  
Albert H. Capanna ◽  
Joseph LaMancusa ◽  
Franco Erculei ◽  
Kazem Fathie ◽  
Peter Christenson ◽  
...  

Abstract Two consortial partners presented within 3 weeks of one another with signs and symptoms of neurological dysfunction. Both were found to have acquired immune deficiency syndrome (AIDS) and primary cerebral lymphoma. We found 17 case reports of primary central nervous system (CNS) lymphoma in AIDS patients. Ten of these cases were described sufficiently to enable analysis. Our 2 cases are unique in that this is the first time primary CNS lymphoma has been reported in sexual partners with AIDS. One case includes the use of magnetic resonance imaging. Both patients underwent craniotomy and received radiation therapy.


2022 ◽  
Vol 71 (6) ◽  
pp. 2245-46
Author(s):  
Hassan Mumtaz ◽  
Shahzaib Ahmad ◽  
Fatima Yasin ◽  
Muhammad Ahsan Shafiq

Primary central nervous system lymphoma (PCNSL) is a rare variety of extra nodal non-Hodgkin lymphoma that reportedly involves leptomeninges, the brain, spinal cord, eyes, or may involve other organs systemically. We present a case of 46-yearold woman with complaints of headache and fever for three weeks, associated with right-sided weakness & altered state of consciousness for one week. The most common presentation of primary central nervous lymphoma is diffuse or multifocal supratentorial masses causing cognitive deterioration and involvement of vitreous, retina, and optic nerve. Most cases ofPCNSL are left undiagnosed due to uncommon


Author(s):  
D. V. Sashin ◽  
M. B. Dolgushin ◽  
E. A. Kobyakova ◽  
E. A. Nechipay ◽  
T. G. Gasparyan

Primary lymphomas of the central nervous system (PCLCS) are relatively rare tumors, usually having a multifocal manifestation in the brain and rapid progression. It is not always possible to make a correct diagnosis for MRI, since similar radiological manifestations (markers) of this disease, when using routine protocols, MRI can occur, for example, in malignant gliomas. This article presents a clinical case of a refinement diagnosis — PLCNS when using MRI in sequence (SWI), which was confirmed by the data of histological examination of surgical material.


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