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.

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 Cerebral aspergillosis due to Aspergillus fumigatus in AIDS patient: first culture - proven case reported in Brazil

2005 ◽  
Vol 47 (3) ◽  
pp. 161-165 ◽  
Author(s):  
José E. Vidal ◽  
Rafi F. Dauar ◽  
Marcia S.C. Melhem ◽  
Walderez Szeszs ◽  
Sandra R.B.S. Pukinskas ◽  
...  
Keyword(s):  
Aspergillus Fumigatus ◽  
Amphotericin B ◽  
Brain Abscess ◽  
Brain Biopsy ◽  
Mass Effect ◽  
Aids Patients ◽  
History Of ◽  
Proven Case ◽  
Mycological Examination ◽  
The Brain

Cerebral aspergillosis is a rare cause of brain expansive lesion in AIDS patients. We report the first culture-proven case of brain abscess due to Aspergillus fumigatus in a Brazilian AIDS patient. The patient, a 26 year-old male with human immunodeficiency virus (HIV) infection and history of pulmonary tuberculosis and cerebral toxoplasmosis, had fever, cough, dyspnea, and two episodes of seizures. The brain computerized tomography (CT) showed a bi-parietal and parasagittal hypodense lesion with peripheral enhancement, and significant mass effect. There was started anti-Toxoplasma treatment. Three weeks later, the patient presented mental confusion, and a new brain CT evidenced increase in the lesion. He underwent brain biopsy, draining 10 mL of purulent material. The direct mycological examination revealed septated and hyaline hyphae. There was started amphotericin B deoxycholate. The culture of the material demonstrated presence of the Aspergillus fumigatus. The following two months, the patient was submitted to three surgeries, with insertion of drainage catheter and administration of amphotericin B intralesional. Three months after hospital admission, his neurological condition suffered discrete changes. However, he died due to intrahospital pneumonia. Brain abscess caused by Aspergillus fumigatus must be considered in the differential diagnosis of the brain expansive lesions in AIDS patients in Brazil.

scholarly journals A Case of Cerebral Vasculitis Associated with Ulcerative Colitis

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Naveen Raj ◽  
Matthew Arkebauer ◽  
Barry Waters ◽  
Brucha Dickinson
Keyword(s):  
Ulcerative Colitis ◽  
Central Nervous System Disease ◽  
Altered Mental Status ◽  
Inflammatory Disorder ◽  
Cerebral Lesions ◽  
System Disease ◽  
Organ Systems ◽  
History Of ◽  
Inflammatory Bowel ◽  
The Brain

Ulcerative colitis (UC) is a chronic, debilitating condition characterized by inflammation of the colonic mucosa. It is regarded as a systemic inflammatory disorder that can affect a number of organ systems. Central nervous system disease associated with UC is a rare sequela of inflammatory bowel disease, occurring in less than 5% of cases. These manifestations include arterial and venous thrombosis, leukoencephalitis, seizures, and vasculitis. We present a case of a 61-year-old female with a two-year history of well-controlled ulcerative colitis, who developed altered mental status and weakness. On brain imaging, she was found to have cerebral lesions which were biopsied. Histopathology subsequently revealed coagulative necrosis and inflammation characteristic of vasculitis. Rheumatology serologies were negative, and the patient was started on steroids that dramatically improved her neurological function, with no residual deficits, and led to resolution of the brain lesions.

scholarly journals Granulomatosis with Polyangiitis Presenting as Pyrexia of Unknown Origin, Leukocytosis, and Microangiopathic Haemolytic Anemia

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Sima Terebelo ◽  
Iona Chen
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Kidney Injury ◽  
Unknown Origin ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
High Dose ◽  
Normal Range ◽  
Pyrexia Of Unknown Origin ◽  
History Of ◽  
Haemolytic Anemia

A 66-year-old woman presented to the Emergency Department with a florid sepsis-like picture, a two-week history of fever, relative hypotension with end organ ischemia (unexplained liver enzyme and troponin elevations), and nonspecific constitutional symptoms. She was initially found to have a urinary tract infection but, despite appropriate treatment, her fever persisted and her white blood cell count continued to rise. During her hospitalization the patient manifested leukocytosis to 47,000 WBC/μL, ESR 67 mm/hr (normal range 0–42 mm/hr), CRP 17.5 mg/dL (normal range 0.02–1.20 mg/dL), and microangiopathic haemolytic anemia, with declining haemoglobin and haematocrit. An infectious aetiology was not found despite extensive bacteriologic studies and radiographic imaging. The patient progressed to acute kidney injury with “active” urinary sediment and proteinuria. Kidney biopsy results and serological titres of myeloperoxidase positive perinuclear-antineutrophil cytoplasmic antibodies (MPO+ p-ANCA) led to a diagnosis of granulomatosis with polyangiitis. Immunosuppressive treatment with high dose methylprednisolone and rituximab led to resolution of the leukocytosis and return of the haemoglobin and haematocrit values toward normal without further signs of hemolysis.

scholarly journals New Treatment Options for Pituitary Granulomatosis With Polyangiitis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A594-A594
Author(s):  
Hessa Boharoon ◽  
Majid AlAmeri ◽  
Abdulla Mohamed Alnuaimi ◽  
Nigel Mendoza ◽  
Stephen McAdoo ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Treatment Guidelines ◽  
Dynamic Assessment ◽  
Treatment Options ◽  
Case Series ◽  
Rare Condition ◽  
High Dose ◽  
Pituitary Mass ◽  
Remission Maintenance ◽  
History Of

Abstract Background: Granulomatosis with polyangiitis (GPA) rarely involves the pituitary gland and has been reported in 1% of all cases of GPA. Most frequently, it presents with pituitary mass effect and diabetes insipidus (DI). To date, there are no treatment guidelines for this rare condition. Case Presentation: Case 1: A 55 year old female with a history of ANCA-positive pulmonary GPA, previously treated with glucocorticoids and immunosuppressants, presented two years later with cranial DI and bitemporal hemianopia. MRI showed a large sellar mass with suprasellar extension. High dose glucocorticoids resulted in good clinical and radiological response. Further treatment consisted in a combination of Cyclophosphamide and Rituximab (RTX). Further doses of RTX are planned aiming for a period of B-cell depletion. Case 2: A 38 year old female, presented with polyuria, recurrent nosebleeds, headaches and a left visual field defect. Pituitary profile revealed ACTH deficiency and MRI showed a heterogenous cystic lesion with peripheral enhancement and stalk thickening. Steroid replacement led to immediate improvement in her symptoms. Cranial DI was confirmed and raised Proteinase 3 (PR3) antibody suggested GPA. A combination of prednisolone and Methotrexate led to significant improvement of MRI appearances and declining PR3 antibody levels. For remission maintenance, two cycles of RTX were given with further radiological and biochemical improvement, and, following dynamic assessment of her HPA axis, she could be fully weaned off steroids. Case 3 is a 47 year old female with a history of childhood asthma. She was found to have cavitating lung lesions. ANCA positivity confirmed GPA and she was commenced on high-dose steroids. During follow-up, she developed headache, polyuria and polydipsia. MRI pituitary showed a suprasellar lesion and pituitary biopsy revealed inflammatory hypophysitis. Cranial DI was confirmed by water deprivation testing. Previous allergic reactions to both RTX and Ofatumumab precluded anti-CD40 monoclonal antibodies and she was commenced on Azathioprine. A further recurrence of pituitary GPA necessitated escalation of the steroid dose and switch of azathioprine to mycophenolate mofetil. She remains in remission and her steroids reduced to a maintenance dose. Conclusion: GPA pituitary has been observed to occur at variable time after diagnosis often in the absence of any other systemic features. A combination of glucocorticoids and RTX has been approved for severe relapsing pulmonary GPA, however, limited data is available for pituitary GPA. In this case series, the response to high dose steroids and RTX for remission maintenance has been encouraging. Experience with ‘conventional’ immunosuppresants remains limited and therapeutic responses remain variable. Further clinical studies are required to establish effective treatment for pituitary GPA.

Artificial human hibernation: from hypothermia to pharmacological hypobiosis. Part I. History of hypothermia

2020 ◽  
pp. 57-62
Author(s):  
Dmitriy Cheboksarov ◽  
Oleg Artyukov ◽  
Marina Muntyan
Keyword(s):  
Critical Conditions ◽  
Human Metabolism ◽  
Review Of Literature ◽  
Historical Aspects ◽  
History Of ◽  
The Brain

This article is review of literature on the development of human hibernation, it highlights historical aspects of the formation of hypothermia as a method for controlling human metabolism and neuroprotection of the brain in critical conditions.

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.

Endovascular biopsy of a sigmoid sinus lesion using a stent retriever and aspiration catheter

2021 ◽  
pp. neurintsurg-2021-018121
Author(s):  
Kasra Khatibi ◽  
Hamidreza Saber ◽  
Ramin Javahery ◽  
Naoki Kaneko ◽  
Lucido Luciano Ponce Mejia ◽  
...  
Keyword(s):  
Sigmoid Sinus ◽  
Stent Retriever ◽  
Myeloid Sarcoma ◽  
Pathologic Finding ◽  
Cerebral Angiogram ◽  
History Of ◽  
Blurry Vision ◽  
The Right ◽  
Oncologic Treatment ◽  
The Brain

A teenager with a history of acute myeloid leukemia presented with headache, nausea and blurry vision over a 2 week period. The MRI of the brain was concerning for the presence of a myeloid sarcoma within the right sigmoid sinus. For evaluation of venous obstruction and the underlying lesion the patient underwent a cerebral angiogram and transvenous biopsy of the sigmoid sinus lesion using a stent retriever and aspiration catheter. The tissue extracted was consistent with myeloid sarcoma. This pathologic finding was consistent with the recurrence of leukemia and guided the targeted oncologic treatment.

scholarly journals Cladophialophora bantianaCerebral Phaeohyphomycosis Complicated by Pulmonary Nocardiosis: A Tale of Two Infections

2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Muhammad Farhan Khaliq ◽  
Rayan E. Ihle ◽  
Christopher P. Schirtzinger
Keyword(s):  
Multidisciplinary Approach ◽  
Brain Mri ◽  
Brain Biopsy ◽  
Term Treatment ◽  
Chest Ct ◽  
Long Term Treatment ◽  
History Of ◽  
The Brain ◽  
Neurosurgical Intervention

Cladophialophora bantiana, a melanized neurotropic fungus, is the most commonly reported agent of cerebral phaeohyphomycosis. We present a case of cerebral phaeohyphomycosis due toC. bantianawith a concomitantNocardiainfection in the lung. The patient was a 64-year-old male who presented with one-week history of productive cough, confusion, and staggering gait. Brain MRI showed multiple enhancing masses, and chest CT demonstrated multifocal consolidation. To confirm diagnosis, brain biopsy was performed that showedCladophialophora bantiana. Bronchoscopic lung biopsy confirmed infection withNocardia araoensis. The patient was treated with trimethoprim-sulfamethoxazole, meropenem, voriconazole, and liposomal amphotericin in addition to partial resection of the brain mass. After several weeks in the hospital and deteriorating status with poor prognosis, medical care was withdrawn.Cladophialophora bantianainfection is rare and requires multidisciplinary approach for accurate diagnostic confirmation. Aggressive and long-term treatment with voriconazole along with early neurosurgical intervention may offer an improved chance of survival in these patients.

scholarly journals Delayed Intracerebral Hemorrhage after Placement of a Ventriculoperitoneal Shunt in a Case of Hydrocephalus: A Rare Case Report and Review of Literature

2019 ◽  
Vol 10 (03) ◽  
pp. 533-536
Author(s):  
Siddartha Reddy Musali ◽  
Srikrishnaditya Manne ◽  
Hemant K. Beniwal ◽  
Nagarjuna Butkuri ◽  
Prakash Rao Gollapudi ◽  
...  
Keyword(s):  
Intracerebral Hemorrhage ◽  
Intracerebral Hematoma ◽  
Review Of Literature ◽  
Vp Shunt ◽  
Sudden Loss ◽  
Rare Case Report ◽  
History Of ◽  
Csf Diversion ◽  
Gcs Score ◽  
The Brain

AbstractVentriculoperitoneal (VP) shunt is a frequently performed operation to achieve cerebrospinal fluid (CSF) diversion but is associated with many complications. Postoperative delayed intracerebral hemorrhage is a kind of rare but catastrophic complication of a VP shunt which questions the survival of a seemingly recovered patient. Here, we present one such case where the patient presented to casualty in altered sensorium and with a history of vomiting. On examination, the Glasgow Coma Scale (GCS) score was E2V1M3; pupils were middilated but reactive. Computerized tomography of the brain showed ventriculomegaly and hydrocephalus, and an emergency VP shunt was done. There was an initial phase of good recovery followed by sudden loss consciousness, vomiting, and a fall in the GCS scores on postoperative day 7. Computed tomography showed an intracerebral hematoma along the shunt track and an intraventricular bleed. The presumed cause for this occurrence is a deranged coagulation profile.

Sign in / Sign up

Export Citation Format

Share Document