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The association between Guillain-Barré Syndrome (GBS) and its variants including Miller Fisher syndrome (MFS) has been reported and debated in the literature. Herein, we are reporting a 59-year-old male patient who had flu-like symptoms for 10 days prior to presentation with rapidly progressive weakness, dysphagia, and dysarthria. He tested positive for COVID-19 and further workup showed positive anti-GQ1b and GQ1d antibodies. The diagnosis of MFS was presumed and prompted the commencement of intravenous immunoglobulin (IVIG). Respiratory deterioration prompted intubation and failure of extubation necessitated plasmapheresis. This treatment culminated in successful extubation and discharge to a long-term care facility. This case adds to the currently limited body of cases that report the association of a rare GBS variant with COVID-19 infection. Only a few of the reported cases of COVID-19-related MFS cases had positive anti-GQ1b antibodies. This may well be the first reported case of COVID-19-related MFS with positive anti-GQ1b and anti-GQ1d antibodies.
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Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder with a largely unknown etiology. In this case, we are presenting an 84-year-old male patient who was admitted for acute hypoxemic respiratory failure secondary to coronavirus disease 2019 (COVID-19) infection. He was neurologically intact. His infection improved and oxygen requirement was gradually weaned off allowing for discharge. However, he was admitted again a month later with progressive dysphagia and aspiration that were confirmed on videofluoroscopic study. He was also found to have mild dysarthria, bulbar muscle weakness, bilateral lower motor neuron facial nerve palsy, diffuse hyporeflexia on four extremities with intact sensory function. Diagnosis of ALS was suspected after extensive workup was pursued and ruled out nutritional, structural, autoimmune, infectious and inflammatory disorders. This case is only the third reported case in medical literature to suggest COVID-19 infection as a triggering/accelerating factor of ALS progression.
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Background: Sydenham chorea, or rheumatic chorea, is a movement disorder that is more prevalent among young people, with a mean age at symptom onset between 8 and 9 years. The condition is more common in females. Sydenham chorea is associated with rheumatic fever and is considered the most common cause of acute chorea in children. We believe that the present case is worth reporting since the occurrence of Sydenham chorea as a post-COVID-19 sequela has not been described in Brazil. Case Presentation: We report here the case of a 14-year-old girl with symptoms of acute chorea that emerged 15 days after treatment resolution of COVID-19 (SARS-CoV-2 or severe acute respiratory syndrome coronavirus 2). Brain computed tomography (CT) and magnetic resonance imaging scans showed no changes, and the laboratory tests revealed no signs of an active infectious process. In contrast, neurological positron-emission tomography/CT showed mild glycolytic hypometabolism in the bilateral mesial frontal region. Administration of an oral anticonvulsant resulted in a marked improvement in her symptoms. Conclusion(s): Despite major efforts of the scientific community for discovering treatments, preventive methods, mechanisms of action, and possible sequelae of SARS-CoV-2, there is still a long way to go to better understand this devastating pathological agent that has affected the global population.Copyright © 2023 Camargo and Morcillo.
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Background: Coronavirus disease-2019 (COVID-19) has caused a pandemic that has recently affected every aspect of life. Fortunately, many vaccines with high safety and efficacy profiles were developed timely to face this pandemic. In a very short time, billions of people were vaccinated. In the meantime, a wide range of neurological syndromes are being reported. Guillain-Barre syndrome (GBS) which is a rare immune-mediated post-infectious peripheral neuropathy was reported after both the COVID-19 infection itself and many types of its vaccines. Method(s): We are reporting a case of post-AstraZeneca vaccine GBS and reviewing the literature of all reported post-COVID-19 vaccines GBS till July 2021. Result(s): 29 adult patients were reported. Of them 58.6% were males. Their mean age is 58.2 years. The median time to clinical onset after vaccine administration was 13.2 days. 86.2% of patients had their symptoms following immunization with the 1st dose of AstraZeneca vector-based covid vaccine. Facial palsy was the most predominant single symptom in 75.8% of patients. Conclusion(s): Guillain-Barre syndrome is a well-recognized but still rare adverse event following vaccination against COVID-19. Although preliminary data incriminates viral vector-based vaccines more than the other types, active post-vaccination surveillance and more powerful statistics are mandatory to reach a solid conclusion regarding the presence of a causal relation.Copyright © 2022
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Arterial dissection is an uncommon complication of reversible cerebral vasocon-striction syndrome (RCVS). We describe the case of a 35-year-old woman with a migraine history who presented with recurrent thunderclap headache and focal neurological signs, including right hemiataxia. She had been diagnosed with COVID-19 disease two weeks earlier. Neuroimaging revealed multifocal stenosis of the posterior circulation arteries and dissection of the right superior cerebellar artery. She improved significantly throughout her one-week hospitalization and maintained only mild ataxia. The interplay between COVID-19 disease, RCVS, and arterial dissection requires further investigation.Copyright © Author(s) (or their employer(s)) and Sinapse 2022.
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Background Management of patients with multiple sclerosis (MS) and evidence of disease activity during treatment with cladribine tablets represents a challenging point. Objectives To report a patient with highly active multiple sclerosis (HAMS) who has been early switched from cladribine to alemtuzumab owing to tumultuous clinical and radiological activity Methods A single retrospective case report. Results. Treatment with alemtuzumab has led to a complete suppression of disease activity without any evidence of infections or acquired autoimmune diseases. Conclusion Our report suggests that an early switch from cladribine to alemtuzumab, may be safe and efficacious in selected HAMS cases.Copyright © 2022 The Authors
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SARS-CoV-2 causes Coronavirus Disease 2019 (COVID-19), an infectious condition that can present none or one or more of these symptoms: fever, cough, headache, sore throat, loss of taste and smell, aches, fatigue and musculoskeletal pain. For the prevention of COVID-19, there are vaccines available including those developed by Pfizer, Moderna, Sinovac, Janssen, and AstraZeneca. Recent evidence has shown that some COVID-19-vaccinated individuals can occasionally develop as a potential side effect Guillain-Barre syndrome (GBS), a severe neurological autoimmune condition in which the immune response against the peripheral nerve system (PNS) can result in significant morbidity. GBS had been linked previously to several viral or bacterial infections, and the finding of GBS after vaccination with certain COVID-19, while rare, should alert medical practitioners for an early diagnosis and targeted treatment. Here we review five cases of GBS that developed in different countries after COVID-19 vaccination.Copyright © 2021
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Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease, targeting the central nervous system, rarely associated with vaccination. Case report: We report a case of a 47 year-old healthy woman who presented, ten days after the first dose of SARS-CoV-2 Vaccine AstraZeneca (Vaxzevria), with back pain, tetraplegia, urinary retention, dysarthria and dysphagia. The patient was diagnosed NMOSD. She underwent intravenous-corticosteroids, vein-Immunoglobulin and plasma-exchange without significant improvement . Conclusion(s): The absence of any possible related conditions, the temporal relation with anti-SARS-CoV-2 vaccination, suggest that, in our case, NMOSD may be due to the cross reaction from Vaxzevria.Copyright © 2021 The Author(s)
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Objective: To describe ischemic stroke due to floating thrombus of ascending aorta occurring as acute and subacute complication of SARS-CoV-2 infection. Material(s) and Method(s): consecutive identification in clinical practice of ischemic strokes secondary to aortic arch thrombosis and history of acute or recent Covid-19 infection. Result(s): two patients had ischemic stroke with evidence of aortic arch thrombosis. The first case had concomitant acute Covid-19 infection, the second had recent Covid-19 infection. Both patients underwent intravenous thrombolysis, and subsequent anticoagulation. One patient died due to cerebral hemorrhage. Discussion and Conclusion(s): aortic arch thrombosis can be an incidental finding in acute ischemic stroke in patients with concomitant and recent COVID-19 disease. However, the infection may lead to thrombosis in non-atherosclerotic vessels and to cerebral embolism. Our findings support active radiological search for aortic thrombosis during acute stroke in patients with acute or recent COVID-19 disease.Copyright © 2022
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SARS-CoV-2 infection has been associated with multiple neurological manifestations. One such manifestation, which has been described since the early stages of the COVID-19 pandemic and is relevant for current neurological practice, is Guillain-Barre syndrome (GBS). The literature describes neurotoxic mechanisms of the virus itself and the possible pathways by which it may affect the peripheral nerves in experimental studies;however, we still lack information on the mechanisms causing the immune response that gives rise to GBS in the context of SARS-CoV-2 infection. Colombia is one of the Latin American countries worst affected by the pandemic, with the third-highest number of cases in the region;thus, it is essential to recognise GBS, as this potential postinfectious complication may severely compromise the patient's functional status in the absence of timely diagnosis and treatment. We present a series of 12 cases of GBS associated with SARS-CoV-2 infection from hospitals in 4 different Colombian cities and describe the clinical presentation, laboratory and electrophysiological study findings, and treatment.Copyright © 2022 Sociedad Espanola de Neurologia
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Background: Cases of SARS-COV-2 triggering or exacerbating autoimmune responses has been described in the literature, and it has shown that use of steroids in non-severe COVID-19 may potentially increase mortality. Case presentation: A 22 year-old African-American man presented with headache, weight, loss, and oral/scrotal ulcerations. Case report: Neurological exam revealed somnolence and right hemiplegia. MRI was remarkable multiple enhancing lesions involving the brainstem and left hemisphere. He was found to have a positive SARS-CoV-2 test. Work-up was unrevealing, and he was diagnosed with Neuro-Behcet's disease (NBD) based on the International Criteria for Behcet's Disease (ICBD)ackspaceD)BackspaceBackspacep. The patient was treated with systemic steroids, which resulted in both clinical and radiological improvement of his disease without exacerbation of his SAR-CoV-2 infection. Conclusion(s): This case presentation suggests that IV steroids may be safe in the treatment of NBD in adult patients presenting with SARS-CoV-2 infection.Copyright © 2021
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INTRODUCTION: Acute MG exacerbations have been attributable to infections and medications. This has surged during the COVID-19 pandemic especially with medications like hydroxychloroquine and azithromycin initially used to treat acute infections. We present a case of new onset MG secondary to COVID-19 vaccination. DESCRIPTION: A 46 year old with a family history of multiple sclerosis presented with 4 months of progressive lower extremity weakness, starting five days after her initial BNT162b2 COVID-19 vaccine. She had difficulty rising from a seated position and raising her arms above her head associated with blurry vision. Exam showed bilateral ptosis (improvement with the ice pack test), dysarthria, weakness worst in proximal bilateral lower extremities and symmetric hyperreflexia. Workup was unremarkable. Pyridostigmine was initiated with improvement. Two months later, she was admitted to the ICU for acute progressive fatigability and shortness of breath concerning for myasthenic crisis. Electromyography/repetitive nerve stimulation of the ulnar nerve showed 60-70% electrical decrement. ABG was stable, but NIF was -36 mmHg. She underwent therapeutic plasma exchange (PLEX). She approached her baseline strength and was discharged on prednisone and mycophenolate mofetil. Two weeks later, she was again admitted with myasthenic crisis with daily NIF at home averaging mid -30s mmHg. She underwent PLEX with significant improvement and was initiated on biweekly PLEX as an outpatient. DISCUSSION: While vaccine-induced flares or onset of autoimmune disease have been described in the literature, new diagnosis of MG following vaccination is rare, limited to 1 to 3 case reports. No case presented after the first dose like our patient. One study on COVID-19 vaccination in patients with existing MG showed that the symptoms of MG did worsen after recombinant subunit vaccination. We query if the COVID vaccine induced a similar mechanism like cytokine storm which hyperstimulated the immune system to a point that broke immunologic self-tolerance. Our patient may have had low titers of pre-existing self-antigen to the AChR that were released upon administration of the BNT162b2 COVID-19 vaccine. However, this vaccine effect may represent a correlation;further studies are needed to infer causality.
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Case Diagnosis: Asymmetric acute inflammatory demyelinating polyneuropathy as presenting symptom of COVID-19 infection. Case Description or Program Description: A 54-year-old male with history of alcohol use with fiveday history of left leg weakness with progression to numbness and weakness to the left side of his face. He then developed right sided weakness, more severe than his left side, primarily distally, as well as numbness and tingling into his bilateral hands. Imaging was negative for acute cause of his symptoms. He did test positive for COVID-19 but was asymptomatic except for occasional dry cough one week prior. His symptoms were initially thought to be from peripheral neuropathy secondary to alcohol use, however he then developed new dysarthria and dysphagia. Setting(s): Acute Inpatient Rehabilitation Hospital Assessment/Results: MRI brain, CTA head and neck, CT cervical spine were negative for acute causes of weakness. Negative HIV and paraneoplastic panel. Lumbar puncture showed albuminocytologic dissociation consistent with Guillain-Barre Syndrome (GBS) . Electrodiagnostic testing is scheduled and pending at this time. Discussion (relevance): GBS generally presents as a symmetric, ascending weakness secondary to a potential viral attack on myelin and Schwann cells. GBS can rapidly ascend and cause autonomic and respiratory failure, so early and accurate diagnosis and treatment are essential. This case demonstrates GBS related to COVID-19 with minimal other symptoms. It also demonstrates the importance of thorough investigation of the potential differentials for neuropathy, as this patient's diagnosis was initially thought to be from alcohol use, which can lead to inappropriate treatment with worse outcomes. In this case, there was a lower suspicion for GBS given its unusual presentation and non-definitive association with COVID-19 infection. Conclusion(s): There is growing research of a positive correlation between COVID-19 infection and GBS, which is further supported by this case with unusual presentation of asymmetric GBS. Continued research is needed to determine the risk of GBS with COVID-19 infection.
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Case Diagnosis: A 65-year-old woman who developed multiple system atrophy as a sequelae of COVID-19 infection Case Description or Program Description: The patient developed progressive dizziness, blurriness, and unsteady gait immediately following hospitalization for COVID-19 infection. Formal evaluation noted rightward nystagmus, mild resting tremor of the right hand, slowed finger tapping test bilaterally, overshooting on right finger to nose, and shuffling gait. MRI of the brain revealed moderate cerebellar and pontine volume loss with crossed hyperintensity of the pons, or "hot cross bun sign", raising suspicion for degenerative disease. Lumbar puncture analysis was normal. The patient was diagnosed with multiple system atrophy with parkinsonism features and started on a trial of amantadine and carbidopa/levodopa. Videonystagmography confirmed cerebellar etiology of her symptoms. Vestibular rehabilitation and meclizine was initiated with subsequent improvement in dizziness and functionality. Setting(s): Acute inpatient rehabilitation facility. Assessment/Results: After 20 days of acute inpatient rehabilitation including vestibular therapy, amantadine, carbidopa/levodopa, and meclizine, there was improvement of dizziness, dysarthria, tremor, weakness, and gait. The therapy team noted good progress since admission regarding performance of self-care tasks as well as transfers and mobility. The patient was discharged home with outpatient vestibular therapy. Discussion (relevance): This is a case of newly diagnosed multiple system atrophy associated with "hot cross buns sign" on MRI with COVID19 infection as the implicated etiology. To our knowledge, this is the first case of central cerebellopontine degeneration associated with COVID19. Conclusion(s): New onset multiple system atrophy may be a sequelae of COVID19 infection.
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Introduction: With the increase in number of the people receiving COVID-19 vaccination, different adverse effects associated with vaccine are being described. While vocal fold paresis (VFP) after both COVID-19 disease and COVID-19 vaccination has been rarely reported, data on this entity in dialysis population is still lacking. We present a case of VFP in a hemodialysis patient following the administration of Pfizer-BioNTech COVID-19 vaccine. Case Description: 45-year-old West Indian female with DM, HTN and End Stage Kidney Disease 2/2 Focal Segmental Glomerulosclerosis s/p kidney transplant that failed after 16 years (on low dose tacrolimus) requiring to start hemodialysis presented to the ED with complaints of voice hoarseness with dysarthria and throat itching that started ~30- 45 minutes after having received the first dose of Pfizer-BioNTech COVID-19 vaccine. She underwent Fiberoptic Indirect Laryngoscopy that showed widely patent airway with mobile vocal cords bilaterally. Symptoms were thought to be secondary to a reaction to the vaccine vs mild GERD. She received steroids and was discharged home within 24 hours after symptomatic improvement on steroid therapy. Her voice normalized within a week. Six months later, she received the second dose of Pfizer-BioNTech vaccine, ~30 minutes after which again developed dysphonia and dysarthria. This time, she was found to have bilateral VFP with incomplete closure. Steroid therapy was reinitiated and is slowly being tapered. Her dysarthria has improved;however, she continues to have hoarseness of voice even after 9 months of having received 2nd dose of vaccine. She has not received the booster dose of vaccine. Discussion(s): Current guidelines recommend booster doses of COVID-19 vaccine for immunocompromised individuals including those on dialysis. The benefits of vaccination markedly outweigh the risk of very rarely reported development of VFP after vaccination. Further research is needed to determine the prevalence of this complication in dialysis patients and to elucidate the underlying mechanisms leading to it.
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Myasthenia gravis (MG) is an acquired autoimmune disorder of the neuromuscular junction, caused by antibodies that target the post-synaptic membrane. These antibodies most commonly bind to the nicotinic acetylcholine receptor (AChR), but in a smaller proportion of cases, antibodies to muscle specific tyrosine kinase (MuSK)(1-10%) or to lipoprotein receptor-related protein 4 (Lrp-4)(1-3%) can be present instead. These antibodies act on the receptors, prevent neuromuscular transmission and induce weakness of skeletal muscles. In 10-15% of MG patients, no antibodies are detected and these patients are designated as seronegative. Weakness can be generalized or localized, is usually more proximal than distal, and nearly always includes eye muscles, causing diplopia and/or ptosis. The pattern of involvement is usually symmetric, except for the eye involvement, which is mostly markedly asymmetric. The muscle weakness typically increases with exercise and repetitive muscle use (fatigue) and varies over the course of a day and from day to day. Patients commonly present first with ocular manifestations, however, the majority develop generalized muscle weakness, involving the facial and bulbar muscles (dysarthria, dysphagia), the limbs, the neck and axial muscles (dropped head, bent spine), and in severe cases the diaphragmatic and intercostal muscles. MuSK-MG predominantly appears in women, who show weakness in mostly cranial and bulbar muscles, commonly with an acute onset and a tendency to rapid progression in comparison to AchRMG. Myasthenic crisis (MC), the severe end of the disease spectrum, can occur at any age and is potentially life-threatening. This is a clinical emergency that requires management in an intensive care setting. MC is mostly provoked by infections or inadequate treatment. In 15-40% of the reported patients with COVID-19 infection a MC occurred. MC appears in around 15-20% of MG patients in the first 2 years after diagnosis. MC can be the first manifestation of MG. Up to a half of MuSK-MG patients develop a MC in their disease course and it is also common in patients with thymoma-associated disease, or AChR-positive late-onset disease;after surgery (including thymectomy);during or after childbirth;in patients taking a contraindicated medication;at the start of corticosteroid treatment or during the tapering of immunosuppression. In approximately 20% the cause of an exacerbation remains unknown. Characteristic symptoms for the impending MC include rapidly progressive muscle weakness, 'inverse aspiration', dysphagia with choking, and dyspnoea associated with orthopnoea and/or tachypnoea which can result in respiratory insufficiency. The clinical management of MC with mechanical ventilation, extended intensive care management and intravenous immunoglobulins (IVIg) or plasmapheresis (PLEX) or in case of persistent MC escalation with rituximab has led to a significant decline in mortality from around 40% in the early 1960s to 5-22% in recent studies with negative prognostic factors including older age at onset, prolonged intubation, and associated comorbidities. At present IVIg and PLEX are considered the gold standard treating MC. However, it may be conceiv- able that newly developed monoclonal antibody therapy (eculizumab, efgartigimod), could be used as rescue therapy to achieve a significant and rapid clinical improvement.
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In the midst of the global pandemic of COVID-19 and its significant morbidity and mortality reported across the world due to severe acute respiratory distress syndrome (SARS), it has always been posing a new set of complications each passing day. As we are still in the process of understanding about the complications related to COVID-19, we are encoun-tered with complications related to immunization for COVID-19. We are reporting a case of facial onset Guillain-Barré syndrome (GBS) in the patient who received first dose of COVISHIELD vaccine a couple of weeks prior to the onset of his illness. © 2021, Editura Medicala. All rights reserved.
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Intracranial cystic lesions are common findings in cerebral imaging and might represent a broad spectrum of conditions. These entities can be divided into nonneoplastic lesions, comprising Rathke cleft cyst, arachnoid cyst, and colloid cyst, as well as neoplastic lesions, including benign and malignant components of neoplasms such as pilocytic astrocytoma, hemangioblastoma, and ganglioglioma. Surgical resection and histological evaluation are currently the most effective methods to classify cysts of the central nervous system. The authors report two uncommon cases presenting as cystic lesions of the encephalic parenchyma-a enterogenous cyst and a glioblastoma-and discuss typical histological findings and differential diagnosis.
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CASE: A 64-year-old woman was brought in by husband for inability to care for patient. Previously active, she developed gait instability, slurred speech, and memory lapse to the point of selective mutism and being bed-bound within three months. Her medical history was notable for hypertension and Covid four months prior. She had had mild upper respiratory symptoms and recovered in ten days. Examination revealed general encephalopathy, dysarthria, limited ability to follow commands. She had decreased strength but increased tone and rigidity in all extremities. She had rhythmic jaw movement and bradykinesia with scatter myoclonic movements. Cerebellar exam was notable for ataxia, but she had normal cranial nerve and sensory exams and normal reflexes. MRI of the brain revealed restricted diffusion and T2/Flair signal abnormality involving bilateral basal ganglia, ventral medial thalami, hippocampi, and cerebral cortices. Toxic metabolic workup was unrevealing. CSF was positive for 14-3-3 protein and elevated total tau protein, confirming Creutzfeldt-Jakob disease. IMPACT/DISCUSSION: Creutzfeldt-Jakob Disease (CJD) is a prion disease with one in a million prevalence. Patients present with rapidly progressing dementia, myoclonus, and signs of cerebellar, corticospinal and extrapyramidal involvement including nystagmus, ataxia, hyperreflexia, spasticity, hypokinesia, bradykinesia, dystonia, and rigidity. CJD is fatal within months to two years. Patients with end stage disease may have akinetic mutism. Magnetic resonance imaging (MRI), electroencephalogram (EEG), and cerebrospinal fluid (CSF) analysis are important for evaluation of CJD. Most sensitive in early stages, MRI Brain commonly shows hyperintense signal involving the cerebral cortex, corpus striatum, caudate, and putamen. EEG may capture pattern of periodic bi-or triphasic period sharp wave complexes. CSF might detect 14-3-3 protein with elevation of tau protein but real-time quaking-induced conversion (RT-QuIC) has the highest specificity for diagnosis for CJD. Though brain biopsy is the sole method of definitive diagnosis, results of MRI, EEG, and CSF analysis along with presenting signs and symptoms are sufficient for clinical diagnosis of CJD. Our patient's dementia, myoclonus, ataxia, hypokinesia, bradykinesia, dystonia, and rigidity all progressing to akinetic mutism within three months are classic presentation of CJD. EEG was normal, but MRI with hyperintensity of basal ganglia and cerebral cortices and CSF analysis with positive 14-3-3 and elevated tau proteins are all lead to diagnosis of CJD. CONCLUSION: This case illustrates a classic case of a Creutzfeldt-Jakob Disease, a rare prion disease marked by rapidly progressive dementia with neuropsychiatric features.