Serum negative neuromyelitis optica spectrum disorder after vaxzevria vaccination: A case report

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)

It Takes Two to Make a Thing Go Wrong

Seventy-three-year-old diabetic male was a high-risk transfer from Alaska for respiratory decompensation in the setting of progressive bulbar and proximal weakness. He was diagnosed with COVID-19 two months prior and viral mononucleosis 1 month prior to presentation. While the patient had a fall 3 months prior to presentation, and decreased mobility at home, there was abrupt onset of progressive upper/lower extremity weakness, dysphagia, and difficulties managing secretions 2 weeks prior to presentation. Initial exam was notable for MRC 3-4/5 proximal upper/lower extremity weakness, areflexia, and negative inspiratory force of 224 to 230 cm H20. A subtle periorbital heliotrope rash was documented. Lumbar puncture demonstrated albumino-cytologic dissociation (protein 142 mg/dL, 6 WBCs) and CK remained elevated (1930 U/L) despite intravenous hydration. Outside electrodiagnostic testing demonstrated a sensorimotor axonal neuropathy with questionable myopathic features on needle electromyography. Given concern for an inflammatory neuropathy and concomitant inflammatory myopathy, intravenous immunoglobulin 2G/kg and IV methylprednisolone 1G/day over 5 days was started. He was transferred for further diagnostic workup and supportive care 6 days after presentation and required intubation within 24 hours of admission. Exam showed progressive proximal and distal weakness of the extremities and general areflexia/hyporeflexia. Repeat electromyography confirmed a severe sensorimotor axonal polyneuropathy without acquired demyelinating features and normal repetitive nerve stimulation. While the patient could no longer activate muscles voluntarily, proximal muscles had increased spontaneous activity with predominant myotonia. Neuroaxis imaging was notable only for enhancement of the lumbar nerve roots. Combined vastus lateralis muscle biopsy and serologic testing confirmed a second pathologic process contributing to the patient's weakness. This case highlights the cooccurrence of 2 distinct neuropathological entities, with potential relation to a prior viral infection, and the importance of ancillary testing to guide treatment for acute causes of neuromuscular respiratory failure.

A paralysis poser: a growing COVID concern

Purpose of Study: Report a rare case of onset of seronegative, juvenile dermatomyositis likely potentiated by Covid-19 infection Methods Used: Case analysis and literature research Summary of Results: A 7 year-old previously healthy male presented with 3 weeks of progressive, bilateral upper and lower extremity weakness, difficulty swallowing, voice changes, periorbital edema, and rash. Recent history was notable for diagnoses of COVID-19 one month prior to presentation and streptococcal pharyngitis 2 months prior to presentation. Notably, there is a family history of systemic lupus erythematosus. On examination, the patient demonstrated bilateral periorbital swelling with purple discoloration of the upper eyelids, a violaceous, pruritic, macular rash on his upper extremities and on his abdomen. Musculoskeletal exam was significant for severe axial (strength 2/5) and proximal (strength 3/5) muscleweakness with notable inability to sit unsupported or maintain head control. His neurologic exam was nonfocal;however, diffuse hyporeflexia in both upper and lower extremities were elicited. Initial screening labs were notable for mild transaminitis;positive ANA (1:80 in speckled pattern), negative ANCA, negative dsDNA/Anti- Sm, elevated aldolase of 10.3, CK 464, and LDH 665;normal thyroid studies and normal inflammatory markers. MRI with and without contrast of the spine indicated diffuse myositis of all muscle groups. Due to concern for autoimmune mediated myositis, Rheumatology was involved early in the patient's course. Empiric treatment was initiated early in the patient's presentation with IVIG, steroids, methotrexate, and plaquenil leading to gradual improvement in symptoms. Subsequent muscle biopsy was consistent with juvenile dermatomyositis (JDM). Conclusion(s): JDM is rare, occurring in 1 to 15 per million children. It classically presents with proximal myopathy and dermatologic findings of Gattron's papules, a heliotrope and malar rash. Its pathophysiology is not yet well defined but is thought to be a humoral mediated autoimmune disease. Muscle biopsies characteristically show perifascicular and perivascular infiltration. Early diagnosis and treatment with steroids, immune modulators, and physical therapy is critical to limit muscle atrophy. Viral infections are known triggers of rheumatologic diseases broadly;however, the more pronounced type 1 interferon response associated with COVID-19, which is known to be a driving pathway of JDM, may be a risk factor for severe, recalcitrant disease. Future research is needed to better identify involved pathophysiology and target future treatment efforts. Additionally, more education and case reports could focus on dermatologic presentations of individuals with pigmented skin. Copyright © 2023 Southern Society for Clinical Investigation.

Acute Motor Axonal Neuropathy presenting as Person-in-a-Barrel syndrome

Background and aims: Person-in-a-barrel syndrome (PIBS) is clinically characterized by brachial diparesis, with preserved cranial and crural muscle strength. It is a rare neurological syndrome most commonly caused by bilateral and symmetric injury of the motor neurons that control the upper limb movements, including bilateral injury to the brain hemispheres, brainstem, cervical spinal cord, brachial plexus, or peripheral nerves. Methods: N/A Results: A 70-year-old male patient with a history of hypertension, dyslipidaemia and hyperuricemia was admitted with an acute and rapidly progressive (10 days of evolution) bilateral upper limb weakness. The patient denied respiratory or gastrointestinal symptoms, fever or recent cervical trauma/pain. Two weeks earlier he was given the first dose of the Pfizer-BioNTech™ vaccine for COVID- 19. The neurological examination revealed severe brachial diparesis and generalized hyporeflexia. Ancillary testing revealed positive serum anti-GM1 and GD1b antibodies. The PCR for SARS-CoV-2 was negative (with positive serum T-Spike antibodies). CSF analysis and Brain/Spinal MRI were normal. The electromyography and nerve conduction studies disclosed diffuse motor conduction blocks in the upper extremities, ultimately fulfilling criteria for Acute Motor Axonal Neuropathy (AMAN) with reversible conduction failure. Intravenous human immunoglobulin (0.4g/kg/day, 5 days) and rehabilitation were started, with subsequent motor improvement. Conclusion: To our knowledge this is the first case of AMAN presenting as PIBS. We intend to highlight that AMAN should be added to the list of causes of this syndrome. The role of the COVID-19 vaccine in this case remains uncertain, and it is not possible, at this moment, to infer causality.

Combined central and peripheral demyelination syndrome after COVID-19 vaccination with brisk response to plasma exchange and steroids

Objective: - Report a case of combined central and peripheral combined demyelination (CCPD) syndrome after COVID19 vaccine with brick improvement to steroids and PLEX - Provide a brief literature review of CCPD etiology and management Background: Combined central and peripheral demyelination (CCPD) is a rare, immunemediated disorder that presents with concurrent demyelination in the central and peripheral nervous system. Known clinical course and radiologic/electrodiagnostic features stem only from a limited number of case reports and small case series. The disease course can be monophasic or chronic. Response to immunomodulatory treatment is variable. Design/Methods: N/A Results: 59-year-old female presented with progressive lower extremity pain, weakness and urinary incontinence four weeks after receiving her second COVID-19 vaccine. Exam was notable for mild somnolence, restricted lateral gaze, right eye red desaturation without APD, ocular ataxia, left lower facial weakness, and lower extremity paresis with decreased reflexes. MRI of the brain and spinal cord showed multifocal supratentorial, intratentorial, cervical and thoracic cord white matter signal abnormalities with trace enhancement in combination with marked cauda equina enhancement. CSF showed albuminocytologic dissociation (protein of 184 and 2 nucleated cells). NMO, Anti-MOG and neurofascin-155 antibodies were negative. Electromyelogram and nerve conduction studies were consistent with a demyelinating polyneuropathy. The patient was treated with a 1 gram IV methylprednisolone daily and five treatments of plasma exchange. At six months, the patient had nearly returned to her previous baseline. Conclusions: CCPD is a rare inflammatory neurologic condition of peripheral and central demyelination. The etiology remains unclear, though viral infections and immunizations have been reported to proceed CCPD in some patients. Limited data is available to guide treatment but PLEX, IVIG and steroids are the most common. Outcomes are heterogeneous and methods to predict long term course remains uncertain. To our knowledge, this is the first reported case of CCPD after the COVID 19 vaccine.

Atypical Pharyngo-Cervico-Brachial Variant of GBS Associated with Covid-19 Antibodies

Objective: Neurological manifestations are common with Covid-19 illness. Many unusual neurological manifestations have been described and we herewith report one such case. Background: COVID-19 is predominantly a respiratory pathogen but can cause multi system involvement. Many studies have shown significant neurological manifestations associated with Covid-19 infection. Some of these neurological manifestations are quite specific like GuillainBarre syndrome. But, many uncommon manifestations have been described like the following case. Design/Methods: A 35-year old woman with no prior history of fever or any other illness presented with insidious onset, gradually progressive weakness of bulbar and bilateral facial weakness along with asymmetrical weakness of both upper limbs of one and half months duration. She was evaluated and investigated accordingly. Results: On neurological examination, the patient had dysphonia, dysphagia, bilateral lower motor neuron facial palsy. Along with weakness of neck flexors and grossly asymmetrical weakness of upper limbs. The motor power on right is MRC 2/5 and MRC 4/5 in left upper limb with diffuse hypotonia. Motor power was normal in lower limbs. There was diffuse hyporeflexia in all the four limbs. Nerve conduction studies showed absent SNAPS with decreased motor nerve conduction velocities and increased CMAP latencies in both upper and lower limbs. CSF examination showed albumin-cytological dissociation. MRI Brain and Cervical Spine were normal. Serum ANA and Serum Ganglioside antibodies were negative. She was tested for total Covid-19 antibodies which was significantly positive with 55.25 COI. Patient was treated initially with IV Methylprednisolone with no significant response. So, followed with intravenous immunoglobulins and showed some improvement. Conclusions: Atypical Pharyngo-cervico-brachial variant of GB Syndrome with gross asymmetrical upper limb weakness and progressed over six weeks associated with positive SARS Cov-2 antibodies. During the pandemic, unusual neurological manifestations should be evaluated for possibility of SARS-CoV-2 associated antibodies as neuropathogenesis has shown both vascular and post infectious demyelinating disorders.

A Case of ChronicInflammatory Demyelinating Polyneuropathy after Pfizer COVID-19 Vaccination

Objective: NA Background: Chronic inflammatory demyelinating polyneuropathy is an immune-mediated polyneuropathy characterized by peripheral demyelination, resulting in symmetrical sensory loss and distal and proximal muscle weakness. While CIDP has been reported after influenza, tetanus, and other common vaccinations, this is the first reported case of CIDP after Pfizer COVID-19 vaccination to our knowledge. Design/Methods: NA Case Presentation: A 34-year-old right-handed male with an unremarkable past medical history presented with bilateral distal paresthesias, proximal and distal muscle weakness, and fine motor difficulties. Symptoms initially manifested with toe numbness, approximately two weeks after receiving the first dose of the Pfizer COVID-19 vaccine. Paresthesias gradually progressed from lower extremities to upper extremities. Two months after the initial COVID-19 vaccine, symptoms worsened with decreased muscle strength, difficulties with fine motor activities, difficulties climbing stairs, and lifting objects above his head. Neurologic evaluation revealed 4/5 strength in upper and lower extremities, generalized hyporeflexia, decreased vibration, and proprioception. MRI of the brain and spine revealed no abnormalities. Nerve conduction studies were consistent with demyelination and cerebral spinal fluid analysis revealed albuminocytologic dissociation. The patient was diagnosed with CIDP and began steroids after poor response to a four-day treatment course of IVIG 2g/kg which resulted in partial improvement of strength. The patient continues to follow up with long-term prednisone therapy. Conclusions: Demyelinating polyneuropathies are a rare complication of vaccination. While the benefits outweigh the risks of immunization, we aim to inform of this potential complication.

A case of a 14 year old male with MOGAD (Myelin Oligocyte Glycoprotein Antibody Disease) post-COVID-19 infection responsive to high dose corticosteroids and intravenous immunoglobulin (IVIG) therapy

Objective: NA Background: The etiology of MOGAD post-COVID-19 infection is not well understood and there are limited publications describing cases in pediatric patients. Here we report a case of a 14-year-old male with MOG antibody positive ADEM (Acute Disseminated Encephalomyelitis) and positive COVID-19 PCR. Design/Methods: NA Results: The patient presented to our hospital in December of 2020 with acute onset of ataxia and lower extremity weakness. His exam was pertinent for mild and symmetric weakness in bilateral hip flexors, dysmetria with ataxic gait, as well as bilateral patellar and ankle hyporeflexia. MRI brain showed symmetric areas of T2 signal hyperintensity, predominantly adjacent to the fourth ventricle as well as the peri-insular and frontal regions. MRI of the lumbosacral spine demonstrated T2 signal hyperintensity within the conus medullaris without enhancement. CSF studies revealed an increased white blood cell count of 74 (90% lymphocyte), elevated protein of 51, elevated kappa free light chain (0.12) and positive oligoclonal bands (3). He was also found to be serum anti-MOG antibody positive (1:100) and COVID-19 positive (PCR). He received 1000 mg of intravenous methylprednisolone daily for 5 days and 2 g/kg IVIG. He was subsequently placed on a 6 week taper of oral prednisone. 2 months after his initial presentation, his neurologic symptoms have completely resolved, and he has been asymptomatic since. Repeat MRI brain 4 months later showed improvement in his multifocal supratentorial FLAIR signal abnormalities. Conclusions: Here we describe a case of a 14-year-old male with MOGAD post-COVID-19, with complete resolution of his symptoms after high dose corticosteroid and IVIG treatment.