A second case of Marburg’s variant of multiple sclerosis with vasculitis and extensive demyelination

2011 ◽  
Vol 17 (12) ◽  
pp. 1531-1538 ◽  
Author(s):  
Rania GA Elenein ◽  
Leroy R Sharer ◽  
Stuart D Cook ◽  
Andrew R Pachner ◽  
Jennifer Michaels ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Pathological Finding ◽  
Brain Biopsy ◽  
Original Description ◽  
Clinical Findings ◽  
Polymorphonuclear Neutrophils ◽  
Inflammatory Cell Infiltrate ◽  
Unexpected Finding ◽  
Stereotactic Brain Biopsy ◽  
True Variant

Marburg’s variant of multiple sclerosis is a rapidly progressive and malignant form of multiple sclerosis (MS) that usually leads to severe disability or death within weeks to months without remission. Few cases have been described in the literature since the original description by Marburg. The classic pathological findings usually include highly destructive zones of extensive demyelination, necrosis with dense cellular infiltrate, and giant reactive astrocytes. We report a case of a 31-year-old woman with Marburg’s variant of MS who, over a period of eight months, became totally disabled, blind, and quadriplegic, with vocal cord paralysis, requiring a tracheostomy. The patient underwent diagnostic stereotactic brain biopsy. Clinical findings, magnetic resonance imaging (MRI), serologic and cerebrospinal fluid (CSF) findings, and neuropathology are discussed. MRI showed extensive white matter involvement in the brain and spinal cord that continuously progressed over time. A diagnostic stereotactic brain biopsy revealed extensive active demyelination with unexpected finding of active vasculitis and fibrinoid necrosis with a vascular inflammatory cell infiltrate, including polymorphonuclear neutrophils and rare eosinophils. Serologic work-up for vasculitis and neuromyelitis optica was unremarkable and the CSF showed only one oligoclonal band (OCB) not present in serum. This is the second case of Marburg’s variant of MS that demonstrated both demyelination and vasculitis. In our case these features were demonstrated simultaneously, even though the demyelination was the predominant pathological finding. Since vasculitis is not a feature of classic MS, these findings pose the question as to whether Marburg’s variant of MS is a true variant or different entity altogether.

A Semi-Autonomous Stereotactic Brain Biopsy Robot With Enhanced Safety

2020 ◽  
Vol 5 (2) ◽  
pp. 1405-1412
Author(s):  
Minxin Ye ◽  
Weibing Li ◽  
Danny Tat Ming Chan ◽  
Philip Wai Yan Chiu ◽  
Zheng Li
Keyword(s):  
Brain Biopsy ◽  
Stereotactic Brain Biopsy

scholarly journals 1H-MRS-Guided Stereotactic Brain Biopsy

2012 ◽  
Vol 90 (1) ◽  
pp. 63-65 ◽  
Author(s):  
Mikhail Chernov ◽  
Yoshihiro Muragaki ◽  
Takaomi Taira ◽  
Hiroshi Iseki
Keyword(s):  
Brain Biopsy ◽  
Stereotactic Brain Biopsy

scholarly journals Disease phenotype analyses of relapsing-remitting multiple sclerosis in Canada and Saudi Arabia

2015 ◽  
Vol 42 (S1) ◽  
pp. S32-S32
Author(s):  
M Alluqmani ◽  
M Alqermli ◽  
G Blevins ◽  
B Alotibi ◽  
F Giuliani ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Saudi Arabia ◽  
Geographic Location ◽  
Clinical Findings ◽  
Cross Sectional ◽  
Relapsing Remitting ◽  
Similar Disease ◽  
The Mean ◽  
Sectional Analysis ◽  
Relapsing Remitting Multiple Sclerosis

Background: Multiple sclerosis (MS) exhibits a spectrum of clinical findings, especially in relapsing-remitting MS (RR-MS). To assess the effects of geographic location and ethnicity on RR-MS phenotype, we investigated RR-MS patients in Canada and Saudi Arabia. Methods: A retrospective cross-sectional analysis of patients receiving active care in MS Clinics was performed in Medina, Saudi Arabia and Edmonton, Alberta. Demographic and clinical data was collected for each patient. Results: 98 patients with treated RR-MS were recruited (n=51, Medina; n=47, Edmonton); 40 patients were Caucasian (Edmonton) while 46 patients were Bedouin (Medina). Although the disease duration was longer in the Edmonton (5.7+2.3 yr) compared to the Medina group (4.4+1.4 yr) (p<0.05), the mean age of RR-MS onset, relapse rate and EDSS change were similar. The female:male ratio was comparable in Edmonton (35:12) and Medina (32:19), as was the risk of optic neuritis. The likelihood of an infratentorial lesion-associated presentation differed (Edmonton, n=23; Medina; n=13) among groups (p<0.05). Spinal cord lesions on MRI were more frequent in Edmonton (n=18) compared to Medina (n=1) patients (p<0.05). Conclusions: Despite differences in location, ethnicity, and a predominance of infratentorial lesion burden the Edmonton group, the RR-MS phenotype displayed similar disease severity and trajectory in these cohorts.

Frameless image-guided stereotactic brain biopsy procedure: diagnostic yield, surgical morbidity, and comparison with the frame-based technique

2006 ◽  
Vol 104 (2) ◽  
pp. 233-237 ◽  
Author(s):  
Graeme F. Woodworth ◽  
Matthew J. McGirt ◽  
Amer Samdani ◽  
Ira Garonzik ◽  
Alessandro Olivi ◽  
...  
Keyword(s):  
Stereotactic Biopsy ◽  
Diagnostic Yield ◽  
Brain Biopsy ◽  
Morbidity Rate ◽  
Frameless Stereotaxy ◽  
Cortical Lesions ◽  
Stereotactic Brain Biopsy ◽  
Biopsy Procedure ◽  
Image Guided ◽  
Permanent Morbidity

Object The gold standard for stereotactic brain biopsy target localization has been frame-based stereotaxy. Recently, frameless stereotactic techniques have become increasingly utilized. Few authors have evaluated this procedure, analyzed preoperative predictors of diagnostic yield, or explored the differences in diagnostic yield and morbidity rate between the frameless and frame-based techniques. Methods A consecutive series of 110 frameless and 160 frame-based image-guided stereotactic biopsy procedures was reviewed. Associated variables for both techniques were reviewed and compared. All stereotactic biopsy procedures were included in a risk factor analysis of nondiagnostic biopsy sampling. Frameless stereotaxy led to a diagnostic yield of 89%, with a total permanent morbidity rate of 6% and a mortality rate of 1%. Larger lesions were fivefold more likely to yield diagnostic tissues. Deep-seated lesions were 2.7-fold less likely to yield diagnostic tissues compared with cortical lesions. Frameless compared with frame-based stereotactic biopsy procedures showed no significant differences in diagnostic yield or transient or permanent morbidity. For cortical lesions, more than one needle trajectory was required more frequently to obtain diagnostic tissues with frame-based as opposed to frameless stereotaxy, although this factor was not associated with morbidity. Conclusions With regard to diagnostic yield and complication rate, the frameless stereotactic biopsy procedure was found to be comparable to or better than the frame-based method. Smaller and deep-seated lesions together were risk factors for a nondiagnostic tissue yield. Frameless stereotaxy may represent a more efficient means of obtaining biopsy specimens of cortical lesions but is otherwise similar to the frame-based technique.

Screening for MOG-IgG and 27 other anti-glial and anti-neuronal autoantibodies in ‘pattern II multiple sclerosis’ and brain biopsy findings in a MOG-IgG-positive case

2016 ◽  
Vol 22 (12) ◽  
pp. 1541-1549 ◽  
Author(s):  
Sven Jarius ◽  
Imke Metz ◽  
Fatima Barbara König ◽  
Klemens Ruprecht ◽  
Markus Reindl ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Glutamate Receptors ◽  
Glutamate Receptor ◽  
Metabotropic Glutamate Receptor ◽  
Brain Biopsy ◽  
Acid Decarboxylase ◽  
Gamma Aminobutyric Acid ◽  
Serum Samples ◽  
Metabotropic Glutamate ◽  
Histopathological Studies

Background: Histopathological studies have revealed four different immunopathological patterns of lesion pathology in early multiple sclerosis (MS). Pattern II MS is characterised by immunoglobulin and complement deposition in addition to T-cell and macrophage infiltration and is more likely to respond to plasma exchange therapy, suggesting a contribution of autoantibodies. Objective: To assess the frequency of anti-myelin oligodendrocyte glycoprotein (MOG), anti-M1-aquaporin-4 (AQP4), anti-M23-AQP4, anti-N-methyl-d-aspartate-type glutamate receptors (NMDAR) and 25 other anti-neural antibodies in pattern II MS. Methods: Thirty-nine serum samples from patients with MS who had undergone brain biopsy ( n = 24; including 13 from patients with pattern II MS) and from histopathologically non-classified MS patients ( n = 15) were tested for anti-MOG, anti-M1-AQP4, anti-M23-AQP4, anti-NMDAR, anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor-type glutamate receptors (AMPAR), anti-gamma-aminobutyric acid receptors (GABABR), anti-leucine-rich, glioma-activated protein 1 (LGI1), anti-contactin-associated protein 2 (CASPR2), anti-dipeptidyl-peptidase-like protein-6 (DPPX), anti-Tr/Delta/notch-like epidermal growth factor–related receptor (DNER), anti-Hu, anti-Yo, anti-Ri, anti-Ma1/Ma2, anti-CV2/collapsin response mediator protein 5 (CRMP5), anti-glutamic acid decarboxylase (GAD), anti-amphiphysin, anti-Ca/RhoGTPase-activating protein 26 (ARHGAP26), anti-Sj/inositol-1,4,5-trisphosphate receptor 1 (ITPR1), anti-Homer3, anti-carbonic anhydrase–related protein (CARPVIII), anti-protein kinase gamma (PKCgamma), anti-glutamate receptor delta 2 (GluRdelta2), anti-metabotropic glutamate receptor 1 (mGluR1) and anti-mGluR5, as well as for anti-glial nuclei antibodies (AGNA) and Purkinje cell antibody 2 (PCA2). Results: Antibodies to MOG belonging to the complement-activating immunoglobulin G1 (IgG1) subclass were detected in a patient with pattern II MS. Detailed brain biopsy findings are shown. Conclusion: This is the largest study on established anti-neural antibodies performed in MS so far. MOG-IgG may play a role in a small percentage of patients diagnosed with pattern II MS.

Incidence of silent hemorrhage and delayed deterioration after stereotactic brain biopsy

1998 ◽  
Vol 89 (1) ◽  
pp. 31-35 ◽  
Author(s):  
Abhaya V. Kulkarni ◽  
Abhijit Guha ◽  
Andres Lozano ◽  
Mark Bernstein
Keyword(s):  
Ct Scan ◽  
Neurological Deficit ◽  
Stereotactic Biopsy ◽  
Brain Biopsy ◽  
Ct Scanning ◽  
Ct Scans ◽  
Stereotactic Brain Biopsy ◽  
Content Type ◽  
Fine Print

Object. Many neurosurgeons routinely obtain computerized tomography (CT) scans to rule out hemorrhage in patients after stereotactic procedures. In the present prospective study, the authors investigated the rate of silent hemorrhage and delayed deterioration after stereotactic biopsy sampling and the role of postbiopsy CT scanning. Methods. A subset of patients (the last 102 of approximately 800 patients) who underwent stereotactic brain biopsies at the Toronto Hospital prospectively underwent routine postoperative CT scanning within hours of the biopsy procedure. Their medical charts and CT scans were then reviewed. A postoperative CT scan was obtained in 102 patients (aged 17–87 years) who underwent stereotactic biopsy between June 1994 and September 1996. Sixty-one patients (59.8%) exhibited hemorrhages, mostly intracerebral (54.9%), on the immediate postoperative scan. Only six of these patients were clinically suspected to have suffered a hemorrhage based on immediate postoperative neurological deficit; in the remaining 55 (53.9%) of 102 patients, the hemorrhage was clinically silent and unsuspected. Among the clinically silent intracerebral hemorrhages, 22 measured less than 5 mm, 20 between 5 and 10 mm, five between 10 and 30 mm, and four between 30 and 40 mm. Of the 55 patients with clinically silent hemorrhages, only three demonstrated a delayed neurological deficit (one case of seizure and two cases of progressive loss of consciousness) and these all occurred within the first 2 postoperative days. Of the neurologically well patients in whom no hemorrhage was demonstrated on initial postoperative CT scan, none experienced delayed deterioration. Conclusions. Clinically silent hemorrhage after stereotactic biopsy is very common. However, the authors did not find that knowledge of its existence ultimately affected individual patient management or outcome. The authors, therefore, suggest that the most important role of postoperative CT scanning is to screen for those neurologically well patients with no hemorrhage. These patients could safely be discharged on the same day they underwent biopsy.

519 Restless Legs and Periodic Limb Movements in 86 patients with Multiple Sclerosis

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A205-A205
Author(s):  
Davide Sparasci ◽  
Raffaele Ferri ◽  
Anna Castelnovo ◽  
Claudio Gobbi ◽  
Chiara Zecca ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Diagnostic Criteria ◽  
Large Population ◽  
Female Gender ◽  
Clinical Findings ◽  
Periodic Limb Movements ◽  
Limb Movements ◽  
Cross Sectional ◽  
Restless Legs ◽  
Maintenance Of Wakefulness Test

Abstract Introduction The aim of this study was to assess the prevalence of restless legs syndrome (RLS), periodic limb movements during sleep (PLMS) and their overlap in a large population of patients with multiple sclerosis (MS), and to compare clinical and paraclinical findings between patients with and without RLS/PLMS. Methods In this cross–sectional, observational, instrumental study, eighty-six patients (M/F: 27/59; mean age 48.0 ± 10.8 years) with a diagnosis of MS underwent a structured telephone interview assessing the five standard diagnostic criteria for RLS. Seventy-six participants underwent Video-polysomnography and Maintenance of Wakefulness Test (MWT). Instrumental and clinical findings were subsequently statistically compared to investigate their association with RLS and PLMS index (PLMSI). Results RLS and PLMS (PLMSI ≥15/h) prevalence in patients with MS was of 31.4% and 31.6% respectively. Among patients with RLS, 37.5% had a PLMSI ≥15/h. In the group with PLMS, 37.5% met all diagnostic criteria for RLS. No differences were found between patients with and without RLS (F = 0.99, p = 0.45), and between patients with and without a PLMSI ≥15/hour (F = 0.32 p = 0.94) on the pool of clinical and instrumental variables. Conclusion RLS is highly prevalent and severe in patients with MS. The prevalence of PLMS is comparable to the general population. The low percentage of patients with RLS having a high PLMSI, together with the absence of correlation between RLS and female gender and older age, support the existence of a distinct symptomatic form of RLS in MS. Support (if any):

Stereotactic Brain Biopsy in Eloquent Areas Assisted by Navigated Transcranial Magnetic Stimulation: a Technical Case Report

2018 ◽  
Vol 17 (3) ◽  
pp. E124-E129 ◽  
Author(s):  
Jiri Bartek ◽  
Gerald Cooray ◽  
Mominul Islam ◽  
Margret Jensdottir
Keyword(s):  
Transcranial Magnetic Stimulation ◽  
Magnetic Stimulation ◽  
Stereotactic Biopsy ◽  
Brain Biopsy ◽  
Awake Surgery ◽  
Histopathological Diagnosis ◽  
Navigated Transcranial Magnetic Stimulation ◽  
Stereotactic Brain Biopsy ◽  
Eloquent Areas ◽  
The Brain

Abstract BACKGROUND AND IMPORTANCE Stereotactic brain biopsy (SB) is an important part of the neurosurgical armamentarium, with the possibility of achieving histopathological diagnosis in otherwise inaccessible lesions of the brain. Nevertheless, the procedure is not without the risk of morbidity, which is especially true for lesions in eloquent parts of the brain, where even a minor adverse event can result in significant deficits. Navigated transcranial magnetic stimulation (nTMS) is widely used to chart lesions in eloquent areas, successfully guiding maximal safe resection, while its potential role in aiding with the planning of a stereotactic biopsy is so far unexplored. CLINICAL PRESENTATION Magnetic resonance imaging of a 67-yr-old woman presenting with dysphasia revealed a noncontrast enhancing left-sided lesion in the frontal and parietal pars opercularis. Due to the location of the lesion, nTMS was used to chart both primary motor and language cortex, utilizing this information to plan a safe SB trajectory and sampling area according to the initial work-up recommendations from the multidisciplinary neuro-oncology board. The SB was uneventful, with histology revealing a ganglioglioma, WHO I. The patient was discharged the following day, having declined to proceed with tumor resection (awake surgery) due to the non-negligible risk of morbidity. Upon 1- and 3-mo follow-up, she showed no signs of any procedure-related deficits. CONCLUSION nTMS can be implemented to aid with the planning of a stereotactic biopsy procedure in eloquent areas of the brain, and should be considered part of the neurosurgical armamentarium.

Stereotactic Brain Biopsy

1990 ◽  
Vol 14 (10) ◽  
pp. 990
Author(s):  
Paul E. McKeever ◽  
Donald A. Ross
Keyword(s):  
Brain Biopsy ◽  
Stereotactic Brain Biopsy

Discrepancies in the interpretation of clinical symptoms and signs in the diagnosis of multiple sclerosis. A proposal for standardization

2005 ◽  
Vol 11 (2) ◽  
pp. 227-231 ◽  
Author(s):  
Bernard MJ Uitdehaag ◽  
Ludwig Kappos ◽  
Lars Bauer ◽  
Mark S Freedman ◽  
David Miller ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Demyelinating Disease ◽  
Clinical Symptoms ◽  
Clinical Findings ◽  
Large Trial ◽  
Mri Findings ◽  
Mcdonald Criteria ◽  
Magnetic Resonance Imaging Mri ◽  
Eligibility Assessment

The new McDonald diagnostic criteria for multiple sclerosis (MS) incorporate detailed criteria for the interpretation and classification of magnetic resonance imaging (MRI) findings, but, in contrast, provide no instructions for the interpretation of clinical findings. Because MS according to the McDonald criteria is one of the primary endpoints in a large trial enrolling patients after the first manifestation suggestive for a demyelinating disease (BENEFIT study), it was decided to organize a centralized eligibility assessment for this trial. During this eligibility assessment it was observed that there were marked inconsistencies in the decisions of participating neurologists with respect to the classification of clinical symptoms as being caused by one or more lesions provoking discussions in about one in every five patients. This paper describes these inconsistencies and their sources, and recommends a systematic approach that attempts to reduce the variability in interpreting clinical findings.

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