scholarly journals Susac's Syndrome

2011 ◽  
Vol 38 (2) ◽  
pp. 335-337 ◽  
Author(s):  
Kevin Lian ◽  
Rekha Siripurapu ◽  
Robert Yeung ◽  
Julia Hopyan ◽  
Kenneth T. Eng ◽  
...  
Keyword(s):  
Corpus Callosum ◽  
White Matter Hyperintensities ◽  
Brain Biopsy ◽  
High Dose ◽  
Normal Brain ◽  
Left Hand ◽  
Elevated Protein ◽  
Fluid Attenuated Inversion Recovery ◽  
History Of ◽  
Previous Medical History

A 40-year-old woman with no significant previous medical history presented with a three month history of ataxia, confusion, memory difficulties, and headaches. Physical examination revealed numbness in the left hand, but was otherwise unremarkable. Magnetic resonance imaging fluid-attenuated inversion recovery (MRI FLAIR) images demonstrated multiple small white matter hyperintensities, including lesions involving the corpus callosum. There were also deep gray nuclei lesions (Figure 1). The corpus callosum lesions involved the central fibers (Figure 2). Post gadolinium T1 images demonstrated enhancement of some of the lesions as well as extensive perivascular and leptomeningeal enhancement (Figure 3). Extensive infectious serology, autoimmune panel, and paraneoplastic antibodies were negative. Lumbar puncture revealed elevated protein (1116 mg/L), but was otherwise normal. Brain biopsy indicated no apparent pathology. The patient was tentatively diagnosed with acute encephalopathy and treated with high dose steroids seven days after presentation. She was subsequently discharged and was sent for rehabilitation.

scholarly journals 079 Glial fibrillary acidic protein (GFAP) astrocytopathy associated with cerebral micro-infarction and poor therapeutic response

2019 ◽  
Vol 90 (e7) ◽  
pp. A25.2-A25 ◽  
Author(s):  
Allycia MacDonald ◽  
James Triplett ◽  
Srimathy Vijayan ◽  
Michael Bynevelt ◽  
Rahul Lakshmanan ◽  
...  
Keyword(s):  
White Matter ◽  
Glial Fibrillary Acidic Protein ◽  
Early Recognition ◽  
Oral Intake ◽  
Brain Biopsy ◽  
Acidic Protein ◽  
Gait Disturbance ◽  
High Dose ◽  
Elevated Protein ◽  
Upper Thoracic

IntroductionGlial fibrillary acidic protein (GFAP) astrocytopathy is a lesser recognised immune-mediated meningo-encephalomyelitis, which is steroid responsive in the majority of cases. Neuroimaging is unique with a distinctive symmetric white matter perivascular linear and punctate enhancement pattern. We present a case with classical phenotype but delayed clinical response, and highlight the importance of early recognition and treatment.CaseA 59-year-old Caucasian female presented with a two month history of headache, gait disturbance, insomnia, agitation, disorientation and reduced oral intake. Examination revealed a high frequency upper limb tremor, hypertonicity and pathologically brisk reflexes with impaired cognitive function. MRI brain and spinal cord demonstrated high T2 signal and striking perivascular and punctate enhancement in supratentorial white matter, cervical and upper thoracic cord. CSF examination revealed lymphocytic pleocytosis and elevated protein. Brain biopsy demonstrated reduced GFAP expression, perivascular T-lymphocytic infiltrate, and recent white matter microinfarction. CSF and serum GFAP antibodies were positive.Motor deterioration accompanied progression to a stuporous state. High dose corticosteroids were commenced, followed by intravenous immunoglobulin and mycophenolate. While there was marked improvement of perivascular contrast enhancement on imaging, the patient continued to demonstrate prominent tremor, gait disturbance and behavioural issues 9 months following symptom onset.ConclusionsThe persistence of disability in this case is likely the result of axonal loss from the initial insult, reflected by the biopsy evidence of microinfarction. Awareness of the unique pattern on MRI and the clinical phenotype will aid in early recognition and prompt treatment of this condition, thus preventing the potential long term morbidity.

scholarly journals Rheumatoid disease: an unusual cause of relapsing meningoencephalitis

2018 ◽  
pp. bcr-2017-222587 ◽  
Author(s):  
Sian K Alexander ◽  
Maria Di Cicco ◽  
Ute Pohl ◽  
Alberto Cifelli
Keyword(s):  
Brain Biopsy ◽  
Oligoclonal Bands ◽  
Rheumatoid Disease ◽  
Persistent Lymphocytosis ◽  
Meningeal Enhancement ◽  
Elevated Protein ◽  
Fluid Attenuated Inversion Recovery ◽  
Leg Weakness ◽  
Ischaemic Attack ◽  
The Brain

A 73-year-old man presented with three episodes of dysphasia and disinhibited behaviour, a single seizure and transient ischaemic attack-like events characterised by right arm and/or leg weakness. These episodes were separated by month-long asymptomatic intervals. Medical history included rheumatoid arthritis, which was clinically quiescent on leflunomide.Repeated cerebrospinal fluid examination showed a persistent lymphocytosis with mildly reduced glucose and elevated protein; oligoclonal bands and viral PCR were negative. MRI of the brain was initially normal, but 7 months after initial presentation revealed meningeal enhancement with bifrontal cortical hyperintensities on T2/fluid-attenuated inversion recovery. Brain biopsy demonstrated necrotising granulomatous meningitis with mixed T cell and B cell infiltrates and without evidence of vasculitis or infection. Serum anticyclic citrullinated peptide antibodies were strongly positive.The diagnosis of rheumatoid meningoencephalitis was made on the basis of brain biopsy findings and serological evidence of active rheumatoid disease. Steroids and rituximab therapy were started leading to clinical stabilisation.

scholarly journals PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Athira Unnikrishnan ◽  
Shila Azodi ◽  
Nadeem Ansari ◽  
Megan Brown ◽  
Joshua Kamnetz ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Granulomatosis With Polyangiitis ◽  
Brain Biopsy ◽  
High Dose ◽  
Review Of Literature ◽  
Left Hemiparesis ◽  
Cerebral Angiogram ◽  
History Of ◽  
Unilateral Proptosis ◽  
The Brain

PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis (UC) patients, the clinical implication of which is not well defined. We are reporting a case of 27-year-old Caucasian male with 14-year history of UC presenting with unilateral proptosis, conjunctival congestion, and chemosis who developed acute hemiparesis within three days of hospital admission, followed by rapid neurological deterioration correlating with brain imaging findings. Serologically he had atypical PANCA with high PR3 antibody titer with a negative infectious workup. His cerebral angiogram was normal but the brain biopsy showed necrotizing vasculitis. He was diagnosed with PR3 ANCA mediated cerebral and orbital vasculitis associated with UC. Treatment was initiated with high dose steroids, plasmapheresis, and cyclophosphamide. He improved significantly with residual left hemiparesis.

A Case of Neurosarcoid Presenting as Multiple Intraparenchymal Hemorrhages

2021 ◽  
pp. 194187442110294
Author(s):  
Amit Mehta ◽  
Fahad Khan ◽  
Chris Wagner ◽  
Taymour Hashemzadeh ◽  
Andrew Stemer ◽  
...  
Keyword(s):  
African American Male ◽  
Early Recognition ◽  
Medical Center ◽  
Delayed Diagnosis ◽  
Brain Biopsy ◽  
Neurological Deficits ◽  
High Dose ◽  
Rapid Progression ◽  
History Of ◽  
Possible Cause

This report explores the case of a 49-year-old African American male with a six-month history of multifocal neurological deficits who presented to an outside hospital after a generalized seizure. Patient was transferred to our tertiary medical center after brain imaging showed multiple bilateral supratentorial intraparenchymal hemorrhages (IPH). A brain biopsy confirmed parenchymal and perivascular non-caseating granulomas with vasculitis. The patient was definitively diagnosed with neurosarcoidosis (NS) and his condition improved with high dose corticosteroids and additional immunosuppressive therapies. Intracranial hemorrhage in the setting of NS is extremely rare, with fewer than thirty documented cases; however, this is likely an underestimation of its true prevalence. This case illustrates the difficulty in diagnosis as many other etiologies of IPH must be considered. Additionally, the clinical course and manifestations of NS is often quite variable. The uniqueness of this case lies in the rapid progression from seemingly incidental microhemorrhages to multiple large IPHs over two months. While the cause of this progression is not immediately apparent, a possible cause may be inadequate initial treatment due to delayed diagnosis. Our case demonstrates the importance of early recognition and initiation of immunosuppressive therapy, potentially leading to dramatic clinical improvement, as seen in this patient.

ITI with high-dose FIX and combined immunosuppressive therapy in a patient with severe haemophilia B and inhibitor

2009 ◽  
Vol 29 (02) ◽  
pp. 155-157 ◽  
Author(s):  
H. Hauch ◽  
J. Rischewski ◽  
U. Kordes ◽  
J. Schneppenheim ◽  
R. Schneppenheim ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Immunosuppressive Agents ◽  
Tolerance Induction ◽  
Intravenous Immunoglobulins ◽  
Factor Ix ◽  
High Dose ◽  
Allergic Reactions ◽  
Success Rates ◽  
Serious Infections ◽  
History Of

SummaryInhibitor development is a rare but serious event in hemophilia B patients. Management is hampered by the frequent occurrence of allergic reactions to factor IX, low success rates of current inhibitor elimination protocols and the risk of development of nephrotic syndrome. Single cases of immune tolerance induction (ITI) including immunosuppressive agents like mycophenolat mofetil (MMF) or rituximab have been reported. We present a case of successful inhibitor elimination with a combined immune-modulating therapy and high-dose factor IX (FIX). This boy had developed a FIX inhibitor at the age of 5 years and had a history of allergic reactions to FIX and to FEIBA→. Under on-demand treatment with recombinant activated FVII the inhibitor became undetectable but the boy suffered from multiple joint and muscle bleeds. At the age of 11.5 years ITI was attempted with a combination of rituximab, MMF, dexamethasone, intravenous immunoglobulins and high-dose FIX. The inhibitor did not reappear and FIX half-life normalized. No allergic reaction, no signs of nephrotic syndrome and no serious infections were observed.

scholarly journals Sustained Opening of the Blood-Brain Barrier with Progressive Accumulation of White Matter Hyperintensities Following Ischemic Stroke

2019 ◽  
Vol 9 (1) ◽  
pp. 16 ◽  
Author(s):  
Imama Naqvi ◽  
Emi Hitomi ◽  
Richard Leigh
Keyword(s):  
Ischemic Stroke ◽  
White Matter ◽  
Acute Stroke ◽  
Blood Brain Barrier ◽  
White Matter Hyperintensities ◽  
Brain Barrier ◽  
Common Finding ◽  
Blood Brain ◽  
History Of ◽  
Iv Tpa

Objective: To report a patient in whom an acute ischemic stroke precipitated chronic blood-brain barrier (BBB) disruption and expansion of vascular white matter hyperintensities (WMH) into regions of normal appearing white matter (NAWM) during the following year. Background: WMH are a common finding in patients with vascular risk factors such as a history of stroke. The pathophysiology of WMH is not fully understood; however, there is growing evidence to suggest that the development of WMH may be preceded by the BBB disruption in the NAWM. Methods: We studied a patient enrolled in the National Institutes of Health Natural History of Stroke Study who was scanned with magnetic resonance imaging (MRI) after presenting to the emergency room with an acute stroke. After a treatment with IV tPA, she underwent further MRI scanning at 2 h, 24 h, 5 days, 30 days, 90 days, 6 months, and 1-year post stroke. BBB permeability images were generated from the perfusion weighted imaging (PWI) source images. MRIs from each time point were co-registered to track changes in BBB disruption and WMH over time. Results: An 84-year-old woman presented after acute onset right hemiparesis, right-sided numbness and aphasia with an initial NIHSS of 13. MRI showed diffusion restriction in the left frontal lobe and decreased blood flow on perfusion imaging. Fluid attenuated inversion recovery (FLAIR) imaging showed bilateral confluent WMH involving the deep white matter and periventricular regions. She was treated with IV tPA without complication and her NIHSS improved initially to 3 and ultimately to 0. Permeability maps identified multiple regions of chronic BBB disruption remote from the acute stroke, predominantly spanning the junction of WMH and NAWM. The severity of BBB disruption was greatest at 24 h after the stroke but persisted on subsequent MRI scans. Progression of WMH into NAWM over the year of observation was detected bilaterally but was most dramatic in the regions adjacent to the initial stroke. Conclusions: WMH-associated BBB disruption may be exacerbated by an acute stroke, even in the contralateral hemisphere, and can persist for months after the initial event. Transformation of NAWM to WMH may be evident in areas of BBB disruption within a year after the stroke. Further studies are needed to investigate the relationship between chronic BBB disruption and progressive WMH in patients with a history of cerebrovascular disease and the potential for acute stroke to trigger or exacerbate the process leading to the development of WMH.

Pseudo-Parkinson Disease Secondary to Ritonavir–Buspirone Interaction

2003 ◽  
Vol 37 (2) ◽  
pp. 202-205 ◽  
Author(s):  
Patrick G Clay ◽  
Molly M Adams
Keyword(s):  
Drug Interaction ◽  
Inhibitory Effect ◽  
Hiv Positive ◽  
High Dose ◽  
Resting Tremor ◽  
Case Summary ◽  
History Of ◽  
Drug Drug Interaction ◽  
To Receive

OBJECTIVE: To report a case of Parkinson-like symptoms appearing in a patient after introduction of ritonavir to buspirone therapy. CASE SUMMARY: A 54-year-old HIV-positive white man presented to the clinic with a 2-week history of ataxia, shuffling gait, cogwheel rigidity, resting tremor, and sad affect with masked features. This patient had been receiving high-dose buspirone (40 mg every morning and 30 mg every evening) for 2 years prior to the introduction of ritonavir/indinavir combination therapy (400 mg/400 mg twice daily) 6 weeks prior to initiation of the above symptoms. Buspirone was decreased to 15 mg 3 times daily, ritonavir/indinavir was discontinued, and amprenavir 1200 mg twice daily was added. The patient's symptoms began to subside after 1 week, with complete resolution after about 2 weeks. The patient continued to receive buspirone for an additional 12 months without recurrence of symptoms. DISCUSSION: This is the first reported interaction of buspirone and antiretrovirals. Buspirone, extensively metabolized by CYP3A4, was likely at supratherapeutic levels due to the inhibitory effect of ritonavir and, secondarily, indinavir. The Parkinson-like symptoms developed rapidly and severely, impacted this patient's quality of life, and necessitated significant clinic expenditures to identify this drug–drug interaction. CONCLUSIONS: This case demonstrates a severe drug–drug interaction between buspirone and ritonavir and further demonstrates the need for awareness of the metabolic profile for all agents an HIV-infected patient is receiving.

scholarly journals Topographic patterns of white matter hyperintensities are associated with multimodal neuroimaging biomarkers of Alzheimer’s disease

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Malo Gaubert ◽  
Catharina Lange ◽  
Antoine Garnier-Crussard ◽  
Theresa Köbe ◽  
Salma Bougacha ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
White Matter ◽  
Glucose Metabolism ◽  
Corpus Callosum ◽  
Gray Matter ◽  
Fdg Pet ◽  
White Matter Hyperintensities ◽  
Gray Matter Volume ◽  
Matter Volume

Abstract Background White matter hyperintensities (WMH) are frequently found in Alzheimer’s disease (AD). Commonly considered as a marker of cerebrovascular disease, regional WMH may be related to pathological hallmarks of AD, including beta-amyloid (Aβ) plaques and neurodegeneration. The aim of this study was to examine the regional distribution of WMH associated with Aβ burden, glucose hypometabolism, and gray matter volume reduction. Methods In a total of 155 participants (IMAP+ cohort) across the cognitive continuum from normal cognition to AD dementia, FLAIR MRI, AV45-PET, FDG-PET, and T1 MRI were acquired. WMH were automatically segmented from FLAIR images. Mean levels of neocortical Aβ deposition (AV45-PET), temporo-parietal glucose metabolism (FDG-PET), and medial-temporal gray matter volume (GMV) were extracted from processed images using established AD meta-signature templates. Associations between AD brain biomarkers and WMH, as assessed in region-of-interest and voxel-wise, were examined, adjusting for age, sex, education, and systolic blood pressure. Results There were no significant associations between global Aβ burden and region-specific WMH. Voxel-wise WMH in the splenium of the corpus callosum correlated with greater Aβ deposition at a more liberal threshold. Region- and voxel-based WMH in the posterior corpus callosum, along with parietal, occipital, and frontal areas, were associated with lower temporo-parietal glucose metabolism. Similarly, lower medial-temporal GMV correlated with WMH in the posterior corpus callosum in addition to parietal, occipital, and fontal areas. Conclusions This study demonstrates that local white matter damage is correlated with multimodal brain biomarkers of AD. Our results highlight modality-specific topographic patterns of WMH, which converged in the posterior white matter. Overall, these cross-sectional findings corroborate associations of regional WMH with AD-typical Aß deposition and neurodegeneration.

929 Not Another Abscess - A Case of Streptococcal Myositis Misdiagnosed as a Right Axillary Abscess

2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
O Oyende ◽  
J Jackman
Keyword(s):  
White Cell Count ◽  
General Surgeon ◽  
High Dose ◽  
General Anaesthetic ◽  
Microbiological Analysis ◽  
Reactive Protein ◽  
Affected Tissue ◽  
Life Threatening ◽  
History Of ◽  
High Degree

Abstract Introduction Streptococcal myositis is a rare form of infectious myositis caused by Lansfield A beta-haemolytic streptococci. It is characterised by rapidly spreading inflammation that can result in severe systemic toxicity and necrosis of the affected tissue if not diagnosed and aggressively treated. Presentation We report a case of a 42-year-old male who presented with a one-week history of worsening right axillary swelling that progressed to painful swelling of his arm. Inflammatory markers were significantly elevated with a white cell count of 17 ×109/L and C-reactive protein of 212 mg/L. On examination, a fluctuant axillary swelling was appreciated, and a decision was made for incision and drainage under general anaesthetic. Intraoperative aspiration of his arm revealed copious purulent fluid prompting intraoperative orthopaedic consult and exploration of the anterior compartment in which there was extensive involvement of the biceps muscle. The microbiological analysis revealed gram-positive cocci in chains, and microbiology advice sought for tailoring of antibiotic regimen. He has recovered well. Discussion Though uncommon, the emergency general surgeon should have a high degree of suspicion when evaluating soft tissue infections to avert potentially disastrous outcomes. Conclusion Early diagnosis, aggressive management with high-dose intravenous antibiotics, and surgical debridement are principles to treat this rare, life-threatening infection.

A Rare Case of Acute Interstitial Pneumonia Diagnosed at Autopsy

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S153-S154
Author(s):  
E Conner ◽  
D Troxclair ◽  
H Khokhar ◽  
W Beversdorf
Keyword(s):  
Respiratory Failure ◽  
Interstitial Pneumonia ◽  
Diffuse Alveolar Damage ◽  
Ventilatory Support ◽  
Respiratory Illness ◽  
High Dose ◽  
Atypical Pneumonia ◽  
Acute Interstitial Pneumonia ◽  
Diagnostic Role ◽  
History Of

Abstract Introduction/Objective Acute interstitial pneumonia (AIP) is a rare disease clinically characterized by rapidly progressing respiratory failure in individuals with no history of respiratory illness or other inciting factors. While most often diagnosed in middle-aged adults, it may present in any age group. Initial presentation is described as influenza- like, and respiratory failure requiring ventilatory support often progresses within weeks to months. Prognosis is poor, with an estimated mortality rate approaching 80% without treatment. Methods We present the case of a 44-year-old male nonsmoker with no significant medical history, who presented in 2018 with 1.5 months of dyspnea and headache initially diagnosed as atypical pneumonia. Chest imaging revealed bilateral opacities; however, microbial workup revealed no evidence of infectious etiology. Autoimmune serology studies were likewise unrevealing. Despite aggressive supportive and medical management, he deteriorated to respiratory failure and succumbed. Results At autopsy, the lungs were symmetrically congested and edematous (combined weight 2,340 g) but free of evident consolidation or discrete lesions. Microscopic examination revealed diffuse alveolar damage with extensive hyaline membrane formation, interstitial edema, and fibroblastic proliferation. The vasculature was severely congested, and the alveoli contained hemorrhage and scattered macrophages. No fungal or mycobacterial elements were identified by staining. Based on the histologic features and clinical context, the diagnosis of AIP was made. Conclusion AIP is a rare, aggressive, and diagnostically challenging disease that includes a broad range of both clinical and histologic differentials. Timely recognition and intervention with aggressive respiratory support and high- dose glucocorticoids are the mainstays of clinical management. The diagnostic role of histology is significant, but hinges on early clinical consideration of AIP as disease progression may later preclude the biopsy procedure. We share this case to raise awareness of this rapidly progressive and diagnostically troubling interstitial lung disease while emphasizing the importance of clinicopathologic correlation.

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