A successful thrombectomy in a case of progressive cerebral sinus thrombosis

Introduction: Cerebral venous sinus thrombosIs (CVST) is a rare condition compared to other categories encountered in stroke medicine.It occurs more frequently in patients with conditions that predispose them to other venous thrombosis, such as thrombophilias, acute malignancies, nephrotic syndrome, and COVID-19. CVST was identified as one of the rare causative of stroke. The exact mechanism of the stroke is not fully understood. However, a commonly agreed pathophysiology is that a dysfunction in arachnoid granulation can lead to sinus occlusion. Subsequently, this leads to a reduction in cerebral fluid drainage, which can increase intracranial pressure, causing capillary hypertension, cerebral oedema, decrease in cerebral perfusion pressure and venous haemorrhage. The European Stroke Organisation (ESO) supports using both MRI/MR Venogram and CT venogram as modalities for diagnosis of CVST, with no particular preference of one over the other. The standard practice in the management of cerebral venous sinus thrombosis includes treating the clot and its precipitating factors and treating the sequela of the clot as in the case we are reporting. Yet, there is no clinical guideline for the more aggressive measures to break down the clot in either AHA or European Stroke Organization, but they are used in clinical practice, with promising results in certain cases. Our case is an example of a successful mechanical thrombectomy with a lifesaving outcome. Method(s): We are reporting an unusual case of a 27- year- old lady who presented to the hyperacute stroke unit with dense right- sided weakness and expressive dysphasia. After an initial CT (Computerised Tomography) scan confirming extensive cerebral venous sinus thrombosis, she went for urgent mechanical thrombectomy. The clinical assessment after the procedure showed significant recovery in power of the right limbs and speech. She was discharged 7 days later with near full recovery. Venous thrombectomy is a rarely performed procedure. However, in this case, it was potentially lifesaving and resulted in an excellent clinical outcome. Result(s): An MRI/MRV follow up in a month demonstrated that the lesion on left centrum semiovale had regressed compared to the first scan. Also, there was some evidence of recanalization of her transverse sinuses. She was assessed by the therapist two months from the event. The patient reported some word finding difficulties and clumsiness in the right hand and leg. However, no further major event since her thrombectomy, and now aiming to get back to work. Conclusion(s): Mechanical thrombectomy in cerebral venous sinus thrombosis can be an effective, life-saving, and safe procedure with an extremely rewarding outcome. It should be considered in patients with acute neurological deterioration despite anticoagulant therapy.

SEVERE IRON DEFICIENCY ANAEMIA SECONDARY TO UTERINE FIBROIDS CAUSING CEREBRAL VENOUS SINUS THROMBOSIS

Background and aims: Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke, contributing to less than 1%. We report an unusual case of severe iron deficiency anaemia (IDA) causing CVST in a young woman with menorrhagia. Methods: Case report: A 40-year-old female with underlying anaemia presented with headache, right leg numbness and expressive dysphasia. She experienced massive menstrual bleeding prior to the symptoms onset on background of uterine fibroids. There was no history of contraceptive pills consumption, massive blood transfusion, COVID-19 infection or vaccination. Systemic review was unremarkable. Results: Blood investigations revealed haemoglobin of 4.5g/dl, MCV 52.3fL, platelet 657x1////////09/L and low ferritin level. Coagulation profile, connective tissue disease, thrombophilia screening, serum homocysteine and HIV test were normal. Computed tomography (CT) of the head showed left temporoparietal lobe infarct and left dural venous sinus thrombosis. CT venography revealed CVST within the distal left transverse sinus and the vein of Labbe. Pelvic ultrasound showed multiple uterine fibroids. She was warfarinised and had iron and red cell transfusion. She agreed to take progesterone-only pill and interval hysterectomy after gynaecological review. Discussion: CVST in association with IDA is rare in adults and is more prevalent in men. In IDA, hypercoagulability and venous stasis play a vital role in thrombus formation. One study found that IDA in women caused arachidonic acid-induced platelet dysfunction causing menorrhagia which is reversible with iron repletion. Conclusion: IDA is a rare cause of CVST but should be considered in the context of relevant history and blood tests.

A Case of an 83 Year Old Male with Acute Disseminated Encephalomyelitis-like Presentation Post COVID-19 Vaccination

Objective: NA Background: A variety of neurologic disorders have been described in patients after receiving the COVID-19 vaccines. Acute disseminated encephalomyelitis (ADEM) have been reported especially in the younger population following any vaccination, including the Covid-19 vaccines. Reports of ADEM in the elderly patients are scarce. Design/Methods: An 83 year old male with history of hypertension, presented with suddenonset of progressive multifocal neurological deficits including blurry vision, upper extremity weakness, numbness and clumsiness with imbalance resulting in multiple falls. A few days later, he reported dysphagia, intermittent expressive aphasia and confusion. Thirteen days prior, he received his second dose of Moderna vaccine. Examination showed mild bilateral upper motor neuron and cerebellar signs. Laboratory tests were unremarkable except for elevated ESR (72), low Vitamins-B12 (311 pg/mL), and D (14.9 ng/mL) levels, and iron deficiency anemia. MRI brain with gadolinium revealed non-enhancing multifocal and confluent supra/infratentorial T2/FLAIR hyperintensity lesions. Cerebrospinal fluid (CSF) analysis showed pleocytosis (whitecell count 13 with 60% lymphocytes), elevated protein (54), and glucose (80), suggestive of underlying inflammation. CSF cytology, meningoencephalitis panel, VDRL, JC-virus PCR, India-ink, acid-fast, bacterial and fungus cultures were negative. HIV antibody was negative. Intravenous Ceftriaxone was initiated until CSF cultures returned negative. Serum anti-MOG and anti-NMO were negative. Repeat imaging within a week showed decreased confluent T2 hyperintensities, but also demonstrated new areas of patchy involvement. The patient received intravenous methylprednisolone 1000 mg daily for 5 days. In the following weeks, his symptoms improved remarkably. Results: NA Conclusions: This 83 year old patient presented with multiple neurologic symptoms, confluent T2-Flair white matter hyperintensities on imaging studies, 13 days post Covid-19 vaccination. Workup for other inflammatory and infectious etiologies was unrevealing. Symptoms improved after intravenous corticosteroids treatment. ADEM is a consideration. Theoretical and actual concerns of vaccine-related neurologic diseases exist, timely recognition and treatment can alter the course and disease progression.

Pediatric Cerebral Infarction associated with recent SARS-CoV-2 mild infection treated with intravenous thrombolysis and endovascular thrombectomy

Objective: N/A Background: Acute ischemic stroke is a major cause of disability worldwide in adults and children. It is a common disease after middle age but uncommon in the pediatric population. Disabling arterial ischemic strokes due to acute intracranial large vessel occlusion within 3-4 weeks of SARS-CoV-2 (COVID-19) infection have been described. Design/Methods: N/A Results: A 15-year-old boy presented with sudden onset right-sided weakness and expressive aphasia witnessed by mother. He presented within 50 minutes of symptom onset to the regional ER facility. Around 4 weeks ago, patient had mild SARS-Cov-2 infection with flu-like symptoms and mild chest pain that worsened with exertion lasting 3-4 days. Neurological examination revealed diminished fluency, anomia, and right upper extremity drift. Initial non contrast computed tomography (CT) demonstrated hyperdense left middle cerebral artery (MCA) sign with subtle loss of gray/white matter differentiation in the left anterior insula. Aphasia and right-sided weakness worsened as he was coming back from CT 2 hours after symptom onset. Intravenous Tenecteplase was administered. CT angiography of head/neck confirmed left proximal M2 occlusion with no arterial dissection. Patient underwent successful mechanical thrombectomy. Three days later his deficits completely resolved. Transthoracic echocardiography with contrast bubble study was unremarkable. Laboratory workup demonstrated mildly low ATIII, positive Factor V Leiden screen with negative genetic testing, positive SARS coronavirus-2 IgG, mildly low PTT. Remaining coagulopathy workup was unremarkable. Conclusions: To our knowledge this is the first case of large vessel occlusion in a pediatric patient treated successfully with both intravenous thrombolysis and mechanical thrombectomy associated with recent SARS-Cov-2 infection. The AIS etiology in our case remains uncertain as abnormal laboratory findings do not explain this presentation. There is high clinical suspicion of an embolic event as possible explanation, possibly related to SARS-CoV-2 postinfectious stage.

Progressive Multifocal Leukoencephalopathy in immunocompetent a case report and review of literature

Objective: NA Background: Progressive multifocal leukoencephalopathy, (PML) a demyelinating disease of the brain, caused by the John Cunningham (JC Virus) is usually seen in patients who are immunocompromised. Here, we describe a case of an immunocompetent patient diagnosed with PML and a comprehensive literature review. Case Description: A 64-year-old Caucasian male presented with acute worsening of progressive neurological decline with difficulty in vision and reading. The patient was diagnosed with Coronavirus disease 2019 (COVID-19). Following COVID-19 infection he started to have difficulty in maintaining balance, poor attention span and expressive aphasia. Based on history, examination, CSF markers, histopathology, and T2/FLAIR MRI Brain at the time of presentation the patient was diagnosed with PML in a setting of no immunosuppression disorder. Results: In our literature review, it was seen that the average age of symptom presentation was 57.5 with predominance in males. Most of the patients presented with progressive neurological deficits with symptomology ranging from mild confusion, aphasia, anxiety to sensory disturbances with numbness, hemiparesis and hemianopsia. Out of the 21 cases, patients responded to mirtazapine and intravenous pulse methylprednisolone (IVMP). The mortality rate was close to 50% with 11 fatal cases and 10 non-fatal cases. None of the cases had any immunosuppressive conditions or underlying HIV, malignancy, solids organ or stem cell transplants and were not on immunosuppressive medications. Conclusions: Our case and literature review demonstrate the possibility that PML may very rarely occur in patients that are immunocompetent. Furthermore, our review showed that patients responded well to mirtazapine and IVMP. In our case, the patient was treated with Pelfilgrastim with encouraging results and could be explored as a possible treatment option. We also want to highlight that mortality rate was lower in this review and was only compared to mortality in PML associated with immunocompromised status.

Atypical Presentation of Lance Adams Syndrome in the setting of Clonazepam overdose and SARS-CoV-2 pneumonia

Objective: To report an atypical presentation of Lance-Adams Syndrome presenting from severe respiratory depression rather than cardiac arrest and to highlight the importance of distinguishing it from post hypoxic myoclonic variants. Background: Clinicians often face difficulty distinguishing Lance-Adams Syndrome (LAS) from Myoclonic Status Epilepticus (MSE). Similarities between the two conditions frequently result in confusion when diagnosing, managing and prognosticating for post-hypoxic myoclonus patients. Design/Methods: A 23-year-old male with a history pertinent for Hemophilia B, depression, opiate and alcohol abuse and chronic pain was found down in his home next to an empty bottle of clonazepam. He was hypoxic with oxygen saturation in the 40s and intubated in the field. Upon arrival to the Emergency room, neurological examination revealed intact corneal reflexes but no gag reflex, cough, or purposeful movements of the extremities. The patient exhibited stimulus induced myoclonic jerking which lasted >30 minutes despite being loaded on valproic acid and levetiracetam. Jerking subsequently ceased with propofol drip. Chest X-ray confirmed interstitial opacities and tested positive for SARS-CoV-2. On attempting to wean sedation, patient exhibited full-body myoclonus including face and palate with inability to follow commands and lack of spontaneous movements. As the EEG showed BIPEDS greater than 2.5 HZ, we decided to burst suppress him and treat with targeted temperature management. After 10 days, the patient was successfully weaned from sedation and extubated, but remained on multiple anti-seizure medications. Results: Patient responded well despite his diffuse cerebral anoxic injury. He regained the ability to follow commands upon discharge but had residual moderate expressive aphasia and post-hypoxic action-induced myoclonus, consistent with LAS. Conclusions: The atypical presentation of this case emphasizes the importance of distinguishing LAS from MSE to guide neurologists to aggressively treat LAS to improve outcome, particularly since MSE historically results with a 90-100% mortality rate.

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.