Course Of Intravenous Steroids Research Articles

Hyperacute optic neuritis in a patient with COVID-19 infection and vaccination: a case report

BackgroundAs scientific knowledge continues to grow regarding coronavirus disease 2019 (COVID-19) infection, several neuro-ophthalmological manifestations have emerged, including rare reports of optic neuritis. Optic neuritis is an inflammatory demyelinating condition of the optic nerve that typically presents as subacute, unilateral vision loss and pain on eye movement. Several cases of COVID-19 infection and COVID-19 vaccination related cases of optic neuritis have been reported. We present a case of hyperacute, unilateral optic neuritis after both recent COVID-19 infection and subsequent booster vaccination.Case presentationWithin two hours after receiving her COVID-19 booster vaccination, a 58-year-old female began experiencing bilateral eye pain, worsened by eye movements. The patient had previously contracted a mild COVID-19 infection three weeks prior to receiving her booster vaccination, confirmed by a rapid antigen test. The pain persisted in her right eye for a week at which time she presented to an ophthalmology clinic. She denied any changes to her visual acuity. Neuroimaging revealed right optic nerve enhancement, and the patient was admitted to the hospital for a course of intravenous steroids, which quickly resolved her eye pain.ConclusionTo our knowledge, this is the first reported case of COVID-19 related optic neuritis following both COVID-19 infection and vaccination. High clinical suspicion is needed to make the appropriate diagnosis, as cases of COVID-19 related optic neuritis may exhibit mild presentations, as was the case with our patient.

A Dorsal Epidural Herniated Disc Fragment Initially Presenting as Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is a rare autoimmune disorder that presents with neurological symptoms that can mimic other conditions. This mimicry can hide other important neurological diagnoses. Here, we present a rare case of thoracic myelopathy secondary to a sequestered dorsal epidural herniated disc fragment that initially presented with the classic findings of GBS.A 58-year-old female presented with progressing bilateral lower extremity weakness, paresthesias, and absent bilateral lower extremity deep tendon reflexes. Lumbar magnetic resonance imaging (MRI) findings were disproportionate to presentation, and lumbar puncture fluid analysis revealed clear, colorless fluid with albuminocytological dissociation. The patient was diagnosed with GBS and treated with a short course of intravenous steroids followed by intravenous immunoglobulin. The patient later developed new-onset ulnar distribution paresthesias, lower extremity spasticity, constipation, and urinary retention that caused a decline in functional progress. Further investigation prompted evaluation with cervical and thoracic MRIs, which revealed a left dorsal epidural lesion at the T9-T10 level causing severe cord compression. The patient was definitively treated with a T9-T10 laminectomy and excision of the offending lesion. Pathology revealed collagenous tissue with fibroblastic proliferation, consistent with a sequestered fragment of the herniated intervertebral disc. The patient was further treated with both acute and subacute rehabilitation. She was eventually discharged home and was able to ambulate independently with a walker.Dependency on positive albuminocytological dissociation in cases of potential GBS can lead to errors in diagnostic accuracy and delay appropriate treatment. Clinicians should remain mindful that GBS is a diagnosis of exclusion and MRI of the entire spine should be considered when the diagnosis of GBS is uncertain.

Acute disseminated encephalomyelitis with bilateral optic neuritis following ChAdOx1 COVID-19 vaccination

BackgroundAcute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory demyelinating disease of the central nervous system. We report a case of ADEM presenting with bilateral optic neuritis temporally associated with the ChAdOx1 vaccine against SARS-COVID19 virus.Case presentationA 36-year-old female presented with bilateral optic neuritis following her first dose of the ChAdOx1 vaccine. Initial MRI Brain showed evidence of demyelination within the subcortical white matter, with no radiological involvement of the optic nerves. Visual evoked potentials were consistent with bilateral optic neuritis which was confirmed radiologically on follow up MRI. She was treated with intravenous steroids with improvement both in symptoms and radiological appearance. A pseudo-relapse occurred which was treated with a further course of intravenous steroids followed by an oral taper. The clinical, radiological and serological results were most consistent with diagnosis of ADEM.ConclusionsADEM is an exceedingly rare complication of ChAdOx1 vaccine despite millions of doses. While it is imperative clinicians remain aware of neurological complications of vaccines, the importance of vaccination to control a pandemic should not be undermined.

437 - COGNITIVE, PATHOLOGICAL, GENETIC AND NEURO-RADIOLOGICAL CORRELATES OF CEREBRAL AMYLOID ANGIOPATHY

Background:Cerebral Amyloid Angiopathy related inflammatory process (CAA-ri), a rare condition caused by an inflammatory reaction occurring within essential cerebral blood vessels against beta-amyloid deposits, leads to subclinical cognitive decline. Often misdiagnosed as dementia, this process can be treated through aggressive immunosuppression, thereby reversing much of the cognitive impairment.Case Report:We report a 69 year old female who came to the clinic for a second opinion and had received a previous diagnosis of Alzheimer’s Dementia (AD) from an outside hospital two years prior. She presented with her husband who provided some key aspects of the history. The husband reported two years of worsening of memory, while the patient denied her symptoms. Per husband and patient, she was able to perform activities of daily living (ADLs), including bathing, dressing and toileting, but had difficulties with many instrumental ADLs (IADLs). The clinical course was somewhat fluctuating with progressive cognitive symptoms and significant word-finding difficulties. Patient had been started on Donepezil 5 mg daily by her primary provider.Results:On examination, the patient did exhibit significant word-finding difficulties and scored 12/30 on the Montreal Cognitive Assessment (MoCA), indicating moderate cognitive impairment. The Patient was as well confused and disoriented to time and place. Neurological examination was otherwise unremarkable. Magnetic Resonance Imaging (MRI) studies were ordered and showed patchy and diffuse T2/FLAIR hyper intensities and particularly concentrated in the posterior cerebral artery and inferior division of the middle cerebral artery. These findings were consistent with cerebral amyloid angiopathy related inflammation (CAA-ri). Besides susceptibility weighted image (SWI) was showing multiple widely distributed microhemorrhages typical for CAA.To address the acute inflammatory reaction the patient was hospitalized and received high dose, 3 day course of intravenous steroids, followed by an oral steroid taper. The treatment had to be monitored due to an unrelated hypertensive emergency and WPW syndrome (both newly diagnosed and treated emergently) thus the Patient was hospitalized for a 3 days and discharged on oral steroids tapper in improved condition.Additionally, imaging showed that the patient’s hippocampal volumes were within normal range so this particular imaging biomarker didn’t support the diagnosis of AD. CSF biomarkers analysis didn’t support the diagnosis of AD either since had p-Tau levels were found to be within normal limits. Patient was found to be homozygous for the APOE e4 gene. Follow-up evaluation (including a repeat MRI study) was performed 2 months later showed clinical recovery and near complete resolution of diffuse hyperintensities, suggesting inflammation had resolved. Both the patient and the husband reported significant improvement in orientation and other aspects of cognition including working memory. The Patient scored 26/30 on MoCA.Discussion:Cerebral amyloid angiopathy (CAA) has been commonly associated with brain hemorrhages in the elderly, but the inflammatory subtype CAA-I occur much less frequently and may be often misdiagnosed as a cancerous process (Ronsin et al. 2016). In a recent systematic review by Caldas A et al. 2015, of the 155 patients with documented CAA-I, almost half displayed some form of cognitive impairment and 86% received corticosteroids. Nearly half of the cases improved following treatment.Conclusion:We present a case of a patient previously diagnosed with AD, upon further investigation, likely CAA-I, treated aggressively with intravenous steroids to good effect. Although rare, CAA-I is a reversible disorder that may be masked by a dementia or/and delirious process and should be considered in patients showing relatively rapid and fluctuating cognitive decline.

The utility of proton MR spectroscopy in diagnosing B cell lymphoma.

e13536 Background: Diffuse Large B Cell Lymphoma (DLBCL) is the most common and increasingly prevalent subtype of Non-Hodgkin lymphoma in both immunocompromised and immunocompetent patients. Its pathogenesis is thought to represent rearrangements, translocations, and transformations of both the immunoglobulin heavy and light chain genes along with somatic mutations of the variable regions of these same genes. A 66-year old Caucasian male with a past medical history of smoking presented to the hospital due to concerns of mental status change, flat affect, and fronto-orbital headache for one-week duration. The patient denied any drug or substance abuse. Physical exam was diffusely normal with the exception of flat affect. Methods: Magnetic resonance spectroscopy is a noninvasive, non-ionizing diagnostic analytical technique that has been used to measure metabolic changes in brain lesions. Results: Lhermitte’s sign was negative and NIH stroke scale was calculated to be 0. CT demonstrated a 17mm frontal lobe lesion. Follow up with MRI showed three bilateral enhancing brain lesions, but no metastasis or primary tumor was seen via CT of chest, abdomen, and pelvis. Initial differential diagnosis included: Tumefactive Multiple Sclerosis, Acute Demyelinating Encephalomyelitis or CNS lymphoma. EEG did not highlight any focal or epileptiform abnormalities and lumbar puncture was negative for Creutzfeld Jacob Disease and Multiple Sclerosis. The patient was discharged with a course of intravenous steroids only to present a few weeks later with no improvement of symptoms. The patient then had proton magnetic resonance spectroscopy (MRS). MRS pattern spikes and chemical profile suggested lymphoma, which was confirmed by a brain biopsy. The patient was subsequently transferred to the University of Pennsylvania for treatment and further management. Conclusions: This case illustrates the beneficial use of MRS, as data patterns it provides compliments the clinician’s knowledge to proceed with appropriate treatment.

Calcific tendonitis of the longus colli muscle: a noninfectious cause of retropharyngeal fluid collection.

Calcific tendonitis of the longus colli (CTLC) muscle is an underrecognized cause of spontaneous acute or subacute neck pain, dysphagia, or odynophagia. Imaging may reveal a retropharyngeal fluid collection leading to the presumed diagnosis of retropharyngeal abscess. Recognition of this uncommon presentation is important to prevent unnecessary surgical incision and drainage. A 44-year-old otherwise healthy male presented with a 2-week history of progressive neck pain, stiffness, and odynophagia. A noncontrast CT scan of the cervical spine revealed a retropharyngeal fluid collection with a small area of calcification anterior to C2. There was a presumed diagnosis of retropharyngeal abscess. The patient was afebrile with normal vital signs. Flexible nasolaryngoscopy was unremarkable. C-reactive protein was elevated but all other bloodwork was normal with no evidence of an infective process. A CT scan was repeated with IV contrast showing no enhancement around the fluid collection. A diagnosis of CTLC was made. The patient was successfully managed with a short course of intravenous steroids and oral NSAIDs with complete resolution of symptoms. Clinically CTLC can mimic more serious disease processes. Identifying pathognomonic imaging findings often confirms the diagnosis. Awareness of this condition by the otolaryngologist will ensure proper patient management and avoidance of unnecessary procedures.

Tolerance of intravenous methylprednisolone for relapse treatment in demyelinating CNS disease

In Switzerland, the first course of intravenous steroids for treatment of episodes of demyelinating CNS disease is usually administered in an inpatient setting. We prospectively evaluated short term tolerance of treatment with special emphasis on sleep quality. Patients with a first event of presumed demyelinating disease (CIS), multiple sclerosis relapses (MS) or sub-acute disease progression were treated with a 5-day regimen of intravenous methylprednisolone (IVMP) in our inpatient clinic. Patients' experience was documented by self-report questionnaires including a standardised depression scale (ADSL). Laboratory tests were performed on a routine basis. Fasting glucose, blood pressure and pulse were measured before every infusion. Activity and sleep patterns were analysed by wrist actigraphs during the 5 day infusion period and at follow-up after 1-2 months. A total of 66 patients participated in the study. Of these, 55 were steroid treatment naïve, and 11 patients, who had received intravenous steroid relapse treatment before, were admitted because of disabling symptoms. Mood disturbances were reported before steroid treatment, however significantly less often at the end of the steroid pulse and during follow-up. Sleep efficiency as measured by wrist actimetry was high before, during and after steroid treatment. Therapy was well tolerated without severe side effects in CIS and MS patients. Sleep efficiency was not disturbed. In conclusion there are no obstacles to change from an inpatient to an outpatient setting for the steroid treatment of relapses in MS and CIS, but rare psychotic reactions to steroid treatment are not predictable.

Inflammatory pseudotumor of nerve: clinicopathological characteristics and a potential therapy

We sought to determine the clinical, electrophysiological, neuroimaging, and pathological features of inflammatory pseudotumor of nerve. Five patients were identified. All cases presented with a gradually progressive mononeuropathy with symptoms of weakness, sensory loss, and prominent neuropathic pain. The median duration of symptoms was 7 months (range 3-36 months). Electrophysiological results were in keeping with chronic axonal mononeuropathies with variable findings of active denervation and reinnervation. MRI demonstrated irregular, large masses involving and surrounding nerve with heterogenous signal characteristics on T1- and T2-weighted and post-contrast sequences. Histopathological features of the nerve slightly varied but shared commonalities including chronic inflammatory infiltrates, increased collagen, and increased numbers of microvessels. Axonal degeneration and decreased density of myelinated fibers were also noted. Three patients were treated with weekly courses of intravenous steroids for 3 months. All reported improvement in pain and weakness. Inflammatory pseudotumor of nerve is not a neoplasm and has reactive features of inflammation, increased vascularity, and marked fibrosis. It presents as a progressive axonal mononeuropathy with weakness, sensory loss, and pain that may be episodic. The primary pathophysiology is unknown but the inflammation and response to treatment suggests that there may be an immune component.

Rapidly Progressive Lethal Infection With Novel Influenza A (H1N1) 2009 Virus in a Kidney Recipient: A Cautionary Tale

Rapidly Progressive Lethal Infection With Novel Influenza A (H1N1) 2009 Virus in a Kidney Recipient: A Cautionary Tale

Natural History of Severe Ulcerative Colitis in a Community-Based Health Plan

We sought to evaluate long-term outcomes of patients with severe ulcerative colitis (UC) after their first hospitalization for the disease. A cohort of 656 patients hospitalized for UC during 1996 to 2004 was followed up for 9 years through 2004. Time-to-event was estimated using actuarial methods, and the proportions of those under follow-up evaluation who experienced outcomes at month 3, year 1, and year 5 were determined. Outcome measures studied were time to subsequent colectomy, rehospitalization for inflammatory bowel disease, and restarting steroid medication. We also used survival analysis to evaluate whether patient characteristics predicted the risk of rehospitalization and colectomy. Among 656 patients initially hospitalized for severe UC, 20% (N = 129) underwent colectomy during their initial hospitalization. Of the remaining 527, a total of 95% (N = 498) were discharged on a steroid taper. At 1 year after discharge, 29% of those remaining under observation were rehospitalized for UC, and an additional 10% required colectomy. At 1 and 5 years after discharge, 34% and 26% received at least a 90-day supply of steroid in the preceding 9 months. Risk of rehospitalization and colectomy were unrelated to the patient's age, sex, or race/ethnicity. The risk for colectomy in patients hospitalized for the first time with severe UC is 20%. In the 3 months after hospitalization the risk for colectomy is 6%. After that, risks appear to decrease proportionate to the time since initial hospitalization.

Thalidomide for acute treatment of neurosarcoidosis

Case report. Johns Hopkins University School of Medicine, Baltimore, MD, USA. Sarcoidosis is a multi-system granulomatous disease of unknown etiology with worldwide distribution. The involvement of the nervous system is common-neurosarcoidosis. Immune responses play an important role in the inflammatory process and granuloma formation. We report a case of neurosarcoidosis that was refractory to two courses of intravenous steroids. Upon initiation of oral thalidomide, the patient showed dramatic improvement clinically and on magnetic resonance imaging. Thalidomide is an immunomodulatory agent that acts to inhibit production of tumor-necrosis factor-alpha (TNF-alpha), an important mediator in CNS inflammation, by enhancing TNF-alpha mRNA degradation. Corticosteroids have been the mainstay of treatment of neurosarcoidosis with success at halting progression of the immune process in 50% cases. Thalidomide offers unique opportunities at managing CNS inflammation due to neurosarcoidosis. None.

Serum sickness following rabbit antithymocyte-globulin induction in a liver transplant recipient: Case report and literature review

Thymoglobulin (Genzyme, Cambridge, MA) is an antithymocyte globulin preparation used for induction immunosuppression therapy in solid organ transplantation. It is being utilized with increasing frequency in orthotopic liver transplantation (OLT) in an effort to minimize or delay the use of calcineurin inhibitors due to their inherent nephrotoxicity. Experience with thymoglobulin in OLT remains limited. We report a case of serum sickness in a patient who received thymoglobulin following OLT. The patient experienced intermittent fevers, polyarthralgia, and acute renal failure 9 days after completion of thymoglobulin administration. The patient's symptoms resolved rapidly and completely with a course of intravenous steroids. We review a set of diagnostic criteria for serum sickness and emphasize the importance of early recognition of the process. Early treatment of serum sickness with steroids or plasmapheresis is highly effective and can reduce unnecessary morbidity from this unusual sequela of induction immunosuppression with antithymocyte globulin.

Pediatric Liver Transplantation: Preliminary Results at Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione

Between July 2003 and November 2004 14 pediatric liver transplantations (LTx) have been performed in 12 children using cadaveric donors. The primary diseases were as follows biliary atresia in 9 cases, whereas the other 3 children were affected by cystic fibrosis, Langherans cells histiocytosis, and hepatoblastoma, respectively. Median patient waiting time was 103 days (range, 2–158); no patient died while on the waiting list. Patients who underwent transplantation included 7 boys and 5 girls, ranging in age from 6 months to 14 years (median age, 5 years). Recipient median weight was 16 kg (range, 6–38). Donor median age was 19 years (range, 3–47), whereas donor median weight was 74 kg (range, 15–90). All children who underwent primary LTx were United Network for Organ Sharing (UNOS) status 2B. Of the 12 transplanted patients, 9 received a left lateral segment (LLS) from an in situ split liver, whereas 3 received a whole graft. Two children developed an episode of acute cellular rejection on the seventh postoperative day, which was treated successfully with a course of intravenous steroids for 3 days. After a median follow-up of 245 days, 10 children are alive but 2 children died due to primary nonfunction (PNF) on the second postoperative day and septic shock on the fifth postoperative day after retransplantation for acute hepatic artery thrombosis, respectively. One child who underwent retransplantation for hepatic artery thrombosis on the 31st postoperative day after primary LTx is currently alive. Evaluation of our initial data suggests that the split liver technique has the potential to meet the needs of pediatric LTx allowing grafting early in the course of the original disease and reducing waiting time.

Cerebral volume changes in multiple sclerosis patients treated with high-dose intravenous methylprednisolone.

Multiple sderosis (MS) patients develop varying degrees of cerebral atrophy, which may already begin at disease onset The purpose of this study is to examine the effect of steroid treatment on cerebral volume in MS patients. Thirty-five MS patients participating in a clinical trial of oral interferon beta, which induded monthly MRI, were included in this study. They suffered from an acute relapse and were treated with intravenous methylprednisolone (IV-MP); 13 of the patients were treated with oral prednisolone tapering after IV-MP. The last MRI scan before and the first (and second for oral tapering patients) scan after IV-MP treatment were used for measuring parenchymal fraction (PF) and ventricular fraction (VF). Changes in PF and VF were analysed using Student's t test. For the total population no significant changes in PF or VF were found. However, the subgroup of patients receiving oral tapering after IV-MP showed changes, compatible with atrophy in both PF and VF, that were significant immediately after IV-MP treatment and still persisted (though not statistically significant anymore) after a mean interval of 30 days. The magnitude of these changes was about the same as the annual change in cerebral volume as reported in natural history studies. Our data indicate that short courses of intravenous steroids (restricted to three or five days) have no major impact, whereas prolonged treatment with oral tapering does significantly affect brain volume. These findings are important for longitudinal studies and clinical trials in which brain volume is used as an outcome measure.

Angiotensin-converting enzyme inhibitor-induced angioedema: a multicenter review and an algorithm for airway management.

Angioedema is a nonpitting edema of which the presentation ranges from benign facial swelling to airway obstruction managed by intubation or tracheotomy. The presentation of this disease is reviewed, and a treatment algorithm based on initial signs and symptoms is proposed for proper airway management. We performed a retrospective review of 108 patients treated in 2 tertiary care centers in the Washington, DC, area over a 5-year period. Ninety-eight patients (90.7%) were African-American, and 81 (75%) were female. Seventy-four patients (68.5%) were taking angiotensin-converting enzyme inhibitors (ACEIs). A classification system was developed based on the location of the edema at initial presentation: 1) isolated facial swelling and oral cavity edema, excluding the floor of the mouth; 2) floor of mouth and/or oropharyngeal edema, and 3) oropharyngeal edema with glottic and/or supraglottic involvement. Fourteen patients (13%) needed airway intervention, 2 of whom underwent a cricothyrotomy after a failed intubation attempt. Eleven (78.6%) were taking ACEIs. The indication for each intubation was massive tongue and floor of mouth edema. The patients were extubated 48 to 72 hours later. No patient demonstrated symptom progression after medical treatment was initiated. Therapy included discontinuation of the ACEI or other inciting agent, a high-humidity face tent, an initial dose of intravenous antihistamines, and a continued course of intravenous steroids. Within 48 hours, most patients had a resolution of their edema. Only cases of significant tongue and oropharyngeal edema took longer than 48 hours to resolve. The ACEIs are a common cause of angioedema. Left untreated, angioedema may progress to involve the oropharynx and supraglottis, resulting in a life-threatening airway compromise. Marked floor of mouth and tongue edema are the indications for airway intervention. An algorithm based on the initial presentation is essential for proper airway and patient management. Once treatment has begun, angioedema is nonprogressive and often resolves within 24 to 48 hours.

Early invasive CNS aspergillosis

A 46-year-old woman with non-Hodgkin's lymphoma presented with a new onset of seizures. A cranial CT was interpreted as normal. Eight months later, she presented with changed mental status and leg weakness, and a repeat CT scan showed multiple ring-enhancing lesions close to the left frontal sinus, with mass effect. A review of the previous CT scan showed a very small area of sinusitis as well as a small ring-enhancing lesion contiguous to it. A short course of intravenous steroids markedly relieved her symptoms, and a stereotactic biopsy confirmed Aspergillus fumigatus to be the cause of the infection. She was successfully treated with Amphotericin B. Central nervous system (CNS) aspergillosis is a potentially fatal disease. The therapeutic success in this case was related to a high index of suspicion at her second presentation. As the early signs of infection might be subtle and easily missed, patients at high risk for opportunistic infection should be studied carefully when new onset of CNS symptoms occur. Early initiation of therapy should improve the prognosis in such cases.