A Series of Catastrophic Events

Case report - Introduction: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease occurring in up to 1% of antiphospholipid syndrome (APS) cases. It was first defined in 1992 and remains a difficult to treat entity with a mortality rate of 37%. We describe a patient with systemic lupus erythematosus (SLE) and CAPS presenting with simultaneous multi-organ injuries who was successfully managed with 'triple' therapy including cyclophosphamide. Case report - Case description: A 42-year-old female presented to her local hospital with chest pain and worsening vision. She had a background of SLE, triple antibody-positive APS (previous DVT, pregnancy loss and strokes), hypertension, a metallic mitral valve, a previous myocardial infarction and pre-existing visual impairment due to a prior intra-cerebral bleed related to anticoagulation. Examination revealed a faint malar rash, cortical blindness and long tract neurological signs. Her ECG showed ischaemic changes and the admission troponin was significantly raised (3773ng/L). An echocardiogram showed new left ventricular dysfunction and a subsequent cardiac MRI was in keeping with coronary artery disease. Investigations showed an acute kidney injury, newly deranged liver function tests and a raised INR (>11, with no bleeding). Complement was normal with a low dsDNA titre. Urinalysis revealed proteinuria and a protein creatinine ratio measured 176mg/mmol. MRI diffusion weighted brain imaging showed acute bilateral occipital and left fronto-parietal infarcts. She had symptoms of a lupus flare with arthralgia and a butterfly facial rash. COVID-19 PCR tests were negative and she had not been recently vaccinated. She was diagnosed with CAPS and transferred to St Thomas' hospital intensive care. On arrival, she received 1mg intravenous vitamin K followed by triple therapy for CAPS: an unfractionated heparin infusion, oral prednisolone 40mg daily, 5 days of plasma exchange and, given her background of SLE, she was treated with intravenous cyclophosphamide (according to the EUROLUPUS regimen). Intravenous methylprednisolone was avoided due to a previous hypertensive encephalopathy reaction. She responded rapidly. Her troponin fell from a peak of 5054 to 294ng/ L, her creatinine settled at a new baseline (232umol/L) and her liver function normalised. She was switched back to warfarin due to her metallic valve and started on aspirin for cardiovascular secondary prevention. She required physical and occupational therapy due to her strokes but recovered well. Case report - Discussion: According to the 2003 criteria, CAPS can be classified as definite when there is evidence of: -3 organs involved, development of manifestations simultaneously or within a week, confirmation by imaging and/or histopathology of small vessel occlusion and positive antiphospholipid antibodies. Probable CAPS is when 3 out of the 4 criteria are present. In this case, three organs were confirmed to be involved with imaging showing cerebral and cardiac ischaemia. Her creatinine rose from a base of 190 to 289umol/L coupled with a high protein creatinine ratio confirming renal involvement. A Budd-Chiari syndrome was also suspected due to deranged liver function tests and INR, though imaging performed after therapy did not confirm this. A biopsy of any of these four organs was not feasible given the severity of her presentation and coagulopathy. There are no randomised controlled trials but data from the CAPS registry guides treatment and management follows a logical approach: anticoagulation to treat thrombosis, glucocorticoids for inflammation and plasma exchange (or IVIG) to remove the circulating autoantibodies. Triple therapy was associated with a reduced mortality compared to no treatment (28.6% versus 75%, respectively). Following analyses from the CAPS registry we also chose to treat with cyclophosphamide, which is associated with improved survival in patients with SLE. This decision was based on the clinical features of an SLE flare as opposed to serological grounds. There have b en reports of rituximab and eculizumab being used successfully in CAPS, though generally as a last resort. As complement activation is seen in animal models of antiphospholipid syndrome thrombosis and rituximab is often used in refractory SLE, they may prove to be promising agents for refractory CAPS. Case report - Key learning points: 1. Prompt recognition and early treatment is vital in managing CAPS 2. Triple therapy with anticoagulation, glucocorticoids and plasma exchange / IVIG is associated with better survival in CAPS 3. Cyclophosphamide is associated with better survival in patients with CAPS and concomitant SLE.

A Case of Cortical Blindness Secondary to Pres

Blindness brought on by an intact anterior visual pathway but due to bilateral occipital lobe involvement is known as cortical blindness. Compared to partial blindness, it is less frequent. The posterior reversible encephalopathy disease has a well-documented history of reversible cortical visual blindness (PRES). The neurological condition PRES is characterised by reversible subcortical vasogenic brain oedema and sudden onset neurological symptoms. This illness can be reversed with prompt diagnosis and treatment. It frequently occurs in conjunction with disorders like eclampsia, cancer, kidney disease, hypertension, and hypertension. This case involves a male teenager who was infected with COVID and later developed PRES. The presence of Anton's blindness complicated PRES. Utilizing radiological investigation, an early diagnosis was obtained, and once therapy was started, all symptoms disappeared. Copyright © 2023 Ubiquity Press. All rights reserved.

Herpes Simplex/Epstein Barr Virus Reactivation in a Patient with Guillain-Barre Syndrome Secondary to COVID-19 Pneumonia

Case Diagnosis: Guillain-Barre Syndrome secondary to COVID-19 pneumonia complicated by reactivation of Herpes Simplex and Epstein-Barr virus causing ventriculitis/ encephalitis Case Description or Program Description: 64 y/o male admitted to acute care hospital (ACH) with progressive bilateral lower extremity weakness associated with recent "flu-like" symptoms and positive COVID-19 test. ACH course was complicated by flaccid tetraplegia, hypoxemic respiratory failure and altered mental status. Initial CSF studies revealed elevated protein with albuminocytologic dissociation, negative cultures and negative viral panel, consistent with Guillain Barre Syndrome (GBS) in the setting of recent COVID-19 pneumonia. Patient was treated with plasmapheresis and IV corticosteroids with no neurological improvement. Setting(s): Inpatient rehab Assessment/Results: Patient then developed visual loss and was diagnosed with cortical blindness that could not be explained by his underlying GBS. MRI brain revealed findings consistent with ventriculitis/ encephalitis. Repeat CSF studies revealed positivity for Herpes Simplex Virus (HSV-2) as well as Epstein-Barr virus, both of which were previously undetected on admission. Patient was transferred to acute inpatient rehabilitation facility (IRF) with flaccid tetraplegia, cortical blindness, pressure ulcers, malnutrition at a total assist level of function. During IRF stay majority of rehab efforts were focused on training family to care for his various needs;tube feeds, medications, avoiding pressure ulcers. Discussion (relevance): The initial negative CSF studies and sequential development of this patient's symptoms are evidence to support that the COVID-19 virus can likely cause reactivation of other viruses. One study, "Investigation of Long COVID Prevalence and Its Relationship to Epstein-Barr Virus Reactivation", reported that up to 66.7% of COVID-19 patients were found to be positive for EBV reactivation. In our patient, the reactivation of HSV-2 and EBV causing ventriculitis/ encephalitis with cortical blindness had major implications on his rehabilitation outcomes. Conclusion(s): To date, there has been minimal published research on the potential of COVID-19 to cause reactivation of other viruses. This case report emphasizes the remarkable ability of the COVID-19 virus to reactivate other viruses and also provides an example of the clinical implications of this within acute rehabilitation.

Bilateral Cortical Blindness Following Hypoxic Ischemic Brain Injury Related to COVID-19: A Case Report

Case Diagnosis: Bilateral Cortical Blindness. Case Description or Program Description: A 44-year-old male chef suffered cardiopulmonary arrest following COVID-19 pneumonia. His airway was not secured for about ten minutes. Consequent complex acute care course involved a prolonged period of nonresponsiveness. Complications included hypoxicischemic brain injury, with bilateral occipital infarcts and edema, which led to significant encephalomalacia. Setting(s): He arrived at the rehabilitation unit about two months later. Assessment/Results: As his mental status improved, he reported inability to see. Exam findings included intact light perception and pupillary reaction, inability to see hand motion or differentiate colors, no discernible blink reflex and negative optokinetic nystagmus drum response. Management involved a multidisciplinary team. Along with his speech, cognition, and motoric status, his vision slowly improved to 20/20 after correction and a central visual field of less than 20 degrees. He could reliably detect colors, large print, and objects. This improvement allowed him to participate in activities he liked, including cooking. Discussion (relevance): Cortical blindness (CB) occurs following damage to the primary visual pathway (V1). Often, the extra-striate pathway (V2) is also affected. Damage to V1 or its afferents could occur in 27-57% of new strokes and is characterized by contralateral homonymous hemianopia with macular sparing. Bilateral destruction of V1 is rare. Some improvement with vision is anticipated within the first few months. However, significant residual deficits usually persist. There is sparse evidence for CB rehab modalities. Approaches to consider include restitution therapies (using computer-based training programs to recover visual field deficits), compensation therapies (using saccadic eye movements to capture blind fields), substitution therapies (using prisms to redirect stimuli to intact visual fields), and pharmacology to optimize neuroplasticity (fluoxetine appears to have shown some benefit for visual pathway in rodents). Conclusion(s): Bilateral CB is a rare complication following brain injury. Although evidence-based treatment modalities are needed, support in a multidisciplinary framework was beneficial for optimizing outcomes in this unique case.

Hypertension Secondary to COVID-19 Leading to Posterior Reversible Encephalopathy Syndrome in a 13-year-old Male

Posterior Reversible Encephalopathy Syndrome (PRES) is a clinico-radiological condition defined by white matter vasogenic oedema predominantly affecting the posterior occipital and parietal lobes. A 13-year-old male presented with complaints of fever for 4 days.Upon evaluation, he turned out positive for COVID-19 with a Computed Tomography (CT) severity score of 5/25. Three days post admission (day 7 of illness), patient developed sudden onset of painless, diminution of vision in both eyes followed by two episodes of generalised tonic clonic seizures. Examination revealed a blood pressure of 180/110 mmHg. Characteristic Magnetic Resonance Imaging (MRI) findings led to a diagnosis of PRES. Patient was treated with antiepileptics, antihypertensives and intravenous mannitol and made a complete recovery. Early identification, treatment of symptomatology and correction of the underlying cause are all key aspects of management.

Progressive Multifocal Leukoencephalopathy Presenting as Expressive Aphasia in a Lung Transplant Recipient

Introduction: Progressive multifocal leukoencephalopathy (PML) is a demyelinating brain disease caused by reactivation of JC virus in immunocompromised patients. PML typically manifests with subacute neurologic deficits including altered mental status, motor deficits, limb ataxia, gait ataxia, and visual symptoms. We report an atypical presentation of PML in a lung transplant recipient (LTR). Case Report: A 71-year-old LTR received rituximab induction followed by a combination of mycophenolate mofetil, tacrolimus, and prednisone. He had a single episode of minimal acute cellular rejection early after LT, but never required significantly augmented immunosuppression. Notably, he had mild leukopenia throughout his post-LT course (WBC count 3-4 thousand/µL), and he had no response to 4 doses of the Pfizer SARS-CoV-2 vaccine, suggesting advanced immunosuppression. At 14 months after LT, the patient reported progressive anomia and aphasia, but no other neurologic deficits. MRI showed an abnormal increased T2/FLAIR signal in the left posterior parieto-occipital subcortical white matter, involving subcortical U fibers, characteristic of PML (Figure A, C). Serum JC virus PCR showed low-level viremia, but CSF from 2 lumbar punctures was PCR negative. Thus, he was diagnosed with possible PML, and immunosuppression was narrowed to a combination of tacrolimus (goal trough 4-6) and prednisone, 5 mg daily. Despite reduced immunosuppression, a repeat MRI 2 months after the initial diagnosis showed worsening PML (Figure B, D) and his symptoms progressed to severe anomia, aphasia, and cortical blindness, but still no motor deficits. PML is a rare, albeit well described, complication of LT, but presentation with only anomia and aphasia is unusual. Without CSF viral isolation, a definitive diagnosis requires brain tissue;however, MRI changes involving subcortical U fibers in the parieto-occipital area are highly characteristic. Early recognition allows for expeditious management.