ABSTRACT
Background/Aims Myasthenia gravis (MG) is an antibody-mediated autoimmune disease targeting proteins at the postsynaptic membrane of the neuromuscular junction. MG is thought to occur in genetically susceptible individuals following an environmental trigger. SARS-CoV-2 infection has been associated with new-onset autoimmune disease, new-onset MG, and exacerbations of pre-existing MG, with molecular mimicry between SARS-CoV-2 epitopes and autoantigen-induced autoreactivity thought to be part of the underlying mechanism. We report a case of newonset ocular MG following first dose Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination which was referred to rheumatology as suspected mononeuritis multiplex. Methods A 53-year-old man of East Asian ethnicity presented to the emergency department (ED) with sudden onset diplopia and left lateral gaze restriction 7 days after receiving his first dose of the Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination. He had longstanding myopia and dry eyes but no other medical history, no regular medications or significant family history. He was a current smoker, with a 50-pack year history. He did not drink alcohol or use any recreational drugs. He was found to have an isolated left VI cranial nerve (CN) palsy with an otherwise normal ocular and physical examination. Blood tests were unremarkable apart from raised cholesterol, and he was discharged with a suspected self-limiting microvascular CN lesion. Three weeks later he presented to ED with worsening diplopia, increasingly restricted eye movements, headache, nausea, vomiting and blurred vision. Ophthalmology assessment noted new right sided CN III and VI palsy, persistent left CN VI palsy, and vertical diplopia in all fields of gaze. Neurological and physical examination were normal. Bloods including an autoimmune screen were unremarkable. SARS-CoV-2 Spike antibodies were positive consistent with SARS-CoV-2 vaccination but not infection. Intracranial and thoracic imaging were unremarkable. He was referred to and seen by both rheumatology and neurology as a case of suspected mononeuritis multiplex. Results A diagnosis of ocular MG was confirmed with positive serum acetylcholine receptor antibodies, and he was started on prednisolone, and pyridostigmine to good effect. Daily forced vital capacity (FVC) showed no respiratory muscle involvement, and nerve conduction studies and electromyography were normal, excluding secondary generalisation. Conclusion A review of the literature found 14 reported cases of new-onset MG all within 4 weeks following SARS-CoV-2 vaccine. Whilst these cases provide interesting insights into the pathogenesis of autoimmune conditions such as MG, they are not epidemiological studies to inform vaccine safety. Ultimately, current evidence suggests that the risks of SARS-COV-2 infection outweigh the risk of vaccine-related adverse events, therefore we suggest clinicians should be aware of potential new-onset autoimmune conditions, but support the safety of SARSCOV2 vaccination. Further, research into possible immunological mechanisms behind this phenomenon, including identifying potential epitopes inducing molecular mimicry, could help establish the likelihood of a causative link.
ABSTRACT
Background: Various neurological disorders have been reported after vaccination against coronavirus disease 2019, one of which is Guillain-Barre Syndrome (GBS). Case Presentation: We report a case of a 73-year-old woman who developed GBS and extra-GBS manifestations 19 days after the second dose of BNT162b2 mRNA vaccine. She presented lower limb predominant muscle weakness and loss of tendon reflexes. Nerve conduction study showed acute motor and sensory axonal neuropathy. In addition, she developed notable deep sensory ataxia, and showed positive pathological reflex, gaze-evoked nystagmus and altered consciousness, which suggested brainstem involvement. Conclusion(s): This is the first coronavirus disease 2019 vaccine-related GBS complicated with such central nervous system manifestations.Copyright © 2023 Japanese Society for Neuroimmunology.
ABSTRACT
Miller-Fisher syndrome (MFS), defined as a rare variant of Guillain-Barre syndrome (GBS), is characterized by the classic triad of ataxia, areflexia, and ophthalmoplegia. It is a demyelinating polyneuropathy resulting from a deregulated autoimmune response secondary to infection by viruses and bacteria. SMF and GBS have been described during the COVID-19 pandemic. There are some reports in the literature of GBS after vaccination for COVID-19. In contrast, reports of post-vaccination FMS for SARS-CoV-2 are scarce in the literature. A 75-year-old patient is presented who consults for asthenia, adynamia, and difficulty swallowing that progresses to respiratory distress. She refers to the application of the Sputnik V vaccine as an important antecedent. During the hospital stay, the diagnosis was made by electromyography and nerve conduction study of GBS variant SMF. The objective is to expose a post-vaccination SMF to SARS-CoV-2 with the biological Sputnik V and highlight the importance of this background for surveillance in clinical practice and future research.Copyright © 2022 Asociacion Colombiana de Infectologia. All rights reserved.
ABSTRACT
Livedoid vasculopathy (LV) is a noninflammatory thrombotic disease caused by occlusion of dermal small vessels associated with systemic autoimmune disorders and coagulopathies. However, LV is often reported as being 'idiopathic', despite extensive investigation. We report a case of severe LV in an otherwise healthy 27-year-old woman, associated with parvovirus infection. The patient presented with a short history of a livedoid rash initially covering her torso, which spread to acral sites. Burning pains in the lower limb caused reduced mobility;systemically, she remained well and stable throughout. Examination revealed generalized acral skin pallor, livedoid patches of violet erythema and purpura with deep serpiginous ulcerations over extensor aspects of upper and lower limbs with a more broken/racemosa nonulcerated livedoid appearance on the trunk. On admission a transaminitisareas continued to ulcerate. Codeine was present with a creatine kinase of 1569 U L.1, but other blood test results were unremarkable including erythrocyte sedimentation rate, complement, cryoglobulins, antinuclear antibodies, antineutrophil cytoplasmic antibodies, extractable nuclear antigen, rheumatoid factor, myositis screen, antiphospholipid screen and thrombophilia screen. Parvovirus IgG and IgM were both positive and tested for, as the patient's young daughter had recently been diagnosed with 'slapped cheek disease'. Magnetic resonance imaging of the thighs showed a diffuse mild myositis;electromyography, nerve-conduction studies, barium swallow and computed tomography of the chest, abdomen and pelvis were all normal. An incisional skin biopsy was performed, which revealed a blood vessel with organizing (Solimani F, Mansour Y, Didona D et al. Development of severe pemphigus vulgaris following SARS-CoV-2 vaccination with BNT162b2. J Eur Acad Dermatol Venereol 2021;35: e649- 51) have been reported. The main proposed mechanisms for AstraZeneca vaccine-induced pemphigus could be a hyperimmune reaction in genetically predisposed individuals, with eventual formation of anti-desmoglein antibodies. An alternative hypothesis is that vaccine components could act as foreign antigens resulting in a cross-reaction with pemphigus antigens. The close association of COVID-19 vaccination with the acute onset of pemphigus in our patient, as well as exacerbations after subsequent vaccine administration, is more than coincidental. Considering the recent pandemic with COVID-19 and the widespread administration of the COVID-19 vaccine, continued observation and documentation of true adverse events is essential.
ABSTRACT
Background and Aims: In the Covid era, Continuous blood glucose monitoring(CGM) was used more frequently and it proved to be quite a helpful and accurate tool for glycemic regulation. Method(s): 75 yrs old Saudi gentleman, had Type 2 diabetes >30yrs, Hypertension, Primary Hypothyroidism, dyslipidemia, mixed polyneuropathy, Iron deficiency anemia, and benign prostatic hypertrophy. In March,2020 his BP and blood glucose readings were high at home. He had a past history of subdural hematoma with hydrocephalus(staus post-shunting). He was on Glargine, oral hypoglycemic agents, anti-hypertensives, Levothyroxine, Atorvastatin, Aspirin, iron fumarate, calcium carbonate and cholecalciferol. Fully conscious, and co-operative, of average built and height.BP 170/70 mmHg, pulse 93/m, RR 18/ m,O2sat 100%, afebrile, BMI 24.96 kg/m2. Fundoscopy normal. He had dry feet and impaired monofilament and vibration testing. Result(s): Hb% 13.1g/dl(12.6 before),MCV 93.8fl,S.Ferritin 10.5ug/l(30-400),Vit.B12 270 pmol/l(145-637),HbA1c 8%(6.4 in Feb.2020).The renal, liver and thyroid functions-intact. Albumin creatinine ratio 12.23mg/g(0-30). Nerve conduction study-mixed polyneuropathy. He continued to follow-up physically even during the Covid crisis due to the elevated SMBG and BP values. Gliclazide & antihypertensive doses were optimized and Glargine was started.On patient's follow-up in August, 2020, time in range had improved to 80%(33% in June,2020),average glucose was 147 mg/dl(200 before), glucose variability was 27.8%(28.9), hypoglycemia (54-79mg/dl) was 1%(0). On last follow-up on 27.06.2022 his HbA1c had climbed up to 8.3%(7.3 in September, 2021). He was compliant to the diabetes regime, but had stopped using the Libre sensor. Conclusion(s): The case signifies the advantage of a meticulous CGM usage during the Covid pandemic, that resulted in a reasonable glycemic control.
ABSTRACT
Background: Severe acute respiratory syndrome coronavirus 2 (SARS-COV2) infection has been associated with the development and persistence of various neurological symptoms in some patients. There have been few prior case reports of small fiber neuropathy (SFN) associated with this infection. However, literature is limited, and the etiology of these symptoms is unclear. Some reports suggested neuroinflammation. Objective(s): To present case series of 3 patients who developed small fiber neuropathy after SARS-COV2 infection. Method(s): We identified patients with SFN after SARSCOV2 infection from our neuromuscular database. We performed chart review and obtained clinical, demographic and, outcomes information of these patients. Result(s): The age of patients was 46, 56 and 71 years. Two female and one male. Time to neuropathy symptom evaluation in clinic from positive COVID19 test was 4 months, 6 months, and 10 months, respectively. None of the patients were hospitalized or given additional medications for COVID treatment. Patients 1& 3 had mild respiratory symptoms. Patient 2 had no symptoms, just a routine pre-op test was positive. All patients had numbness, tingling and painful paresthesias as the main symptoms. The second patient reported autonomic symptoms of heart racing and temperature dysregulation. Nerve conduction studies did not show large fiber peripheral neuropathy in any of the patients. Skin biopsy was performed at 7 months, 8 months and 13 months respectively post COVID infection and was positive in all 3 patients. There was no other etiology identified for the neuropathy Treatment included gabapentin for the first patient, the second patient received narcotic medication for a surgery and continued this for her neuropathic symptomsand the third patient is not on any medications for SFN. Conclusion(s): SFN can be associated with prior SARSCOV infection. There is need for further research to determine possible underlying neuropathological mechanisms and find effective treatments in COVID-related SFN.
ABSTRACT
Introduction: Sarcoidosis is a rare granulomatosis. The absence of well-defined criteria for definition and the existence of differential diagnosis makes the positive diagnosis difficult. Method(s): We report a case of sarcoidosis that illustrates the difficulty of this diagnosis in the presence of atypical clinical manifestations and a strong suspicion of tuberculosis. Ultimately, renal histology allowed the positive diagnosis and the response to corticosteroids confirmed it retrospectively. Result(s): Our patient was a 66 years-old female with a history of hypertension who presented with a sensory and motor polyneuropathy a couple of months after a mild COVID-19 pneumonia, hospitalized for exploration of a worsening renal function due to a tubulointerstitial neuropathy (creatinine upon admission at 250 micromol/l, eGFR = 16 ml/min/1,73m2 -MDRD). Kidney biopsy revealed an interstitial infiltrate of monocytes and fibrosis alongside non-necrotic and giant-cell epithelioid interstitial granulomas. Extra-renal signs consisted of the above-mentioned neuropathy, bilateral mediastinal adenopathies with no signs of a pulmonary disease at the bodyscan, a hepatomegaly, splenomegaly, a pleural and pericardial effusion of low abundance, and a peritoneal thickening. Bronchoscopy and bronchoalveolar washing found no evidence for malignancies and screening for mycobacterial infections by polymerase chain reaction was negative. No granulomas were found at the hepatic biopsy. Digestive tract endoscopy and biopsies showed no abnormalities. During hospitalization, the patient presented an episode of acute polyradiculonevritis confirmed by cerebral-spine fluid study and nerve conduction study results. Our patient received intraveinous immunoglobulins (IgIV) with a favorable outcome but relapsed one month later, showing signs of respiratory failure. Upon the second relapse of the chronic polyradiculonevritis and based on the absence of bacteriological and histological evidence for a mycobacterial infection and the results or the renal biopsy, the patient received high-dose corticosteroids alongside a second course of IgIV. The neuropathy regressed totally within a month with a decrease of creatinine level to 140 micromol/l (eGFR = 35ml/min/1,73m2) alongside the polyserositis and organomegaly. The final diagnosis was that of a sarcoidosis with pulmonary and renal involvement. Although the neuropathy could be considered a manifestation of sarcoidosis, its origin was intricated as post-viral original could not be formally excluded. Conclusion(s): The etiological diagnosis for granulomatous interstitial nephropathies can be challenging due to similar clinical presentations and the need to start specific treatments especially in the presence of life-threatening situations and the absence of clear criteria defining sarcoidosis further enhances the level of difficulty. No conflict of interestCopyright © 2023
ABSTRACT
Introduction: European Medicines Agency (EMA) approved SARS-Cov-2 vaccines that are administered in Andalucia, south of Spain, have a very good clinical efficacy against Covid-19 and safety profile. Secondary effects (SE) associated with these vaccines are mainly mild (arthralgias myalgias), being SE related with the nervous system infrequent (>1/1000 to <1/100) tremor, paraesthesia, dizziness;rare (>=1/10.000 to < 1/1000) peripheral facial palsy, or very rare (<1/10.000) Guillain Barre syndrome (GBS). 85% of the Andalusian population have been already fully vaccinated, so our environment constitutes an ideal observatory for the real-life analysis of possible neuromuscular SE related to this vaccine. Result(s): Multicentric retrospective observational study of postvaccination against SARS-Cov-2 neuromuscular SE. We actively searched in 10 Andalusian hospitals for objective SE referred for electroneurography (ENG) and/or electromyography (EMG) after SARS-Cov-2 vaccination. We have registered 21 patients (12 males/9 females): 4 acute demyelinating polyneuropathies GBS;2 brachial plexopathies (Parsonage Turner type);1 ipsilateral of vaccine injection and one contralateral;1 inferior limb proximal myopathy in the context of a myocarditis;and 1 presented an acute neuromuscular postsynaptic defect Miastenia Gravis;the rest of the patients had distal paraesthesia with normal ENG-EMG. Conclusion(s): Neurophysiological studies in patients with peripheral neurological symptoms after SARS-Cov-2 vaccination are generally normal, but we should keep alert for possible serious and treatable complications that can be diagnosed with ENG-EMG tests. It would be advisable to extend this multicentric study the get a real idea of the performance of SARS-Cov-2 postvaccine ENG-EMG tests. Copyright © 2022
ABSTRACT
Various neurological manifestations are observed in about 36.4% of patients infected with SARS-CoV-2 and post-COVID neuropathy is one of them. There is lack of studies describing neurophysiological abnormalities in peripheral nerves in case of patients who had SARS-CoV-2 infection. The aim of this study was to analyze the changes in peripheral nervous system in case of COVID-19 survivors. In the presented study, 45 COVID-19 survivors who had nerve conduction study (NCS) were involved. Results were compared with control group consisting of healthy patients who had nerve conduction study before the COVID-19 pandemic. In our study group, neurophysiological abnormalities were present in the case of both sensory and motor nerve fibers. The most significant reduction of NCS parameters was observed in the case of sensory action potential amplitude of sural nerve. Moreover, that correlation was the most significant in the case of amplitude and conduction velocity in sensory and motor neuron fibers both in arms and legs. Those abnormalities were observed even 6 mo after COVID-19. Further investigation needs to be done regarding the polyneuropathies associated with human coronaviruses, and we should answer the question whether the virus directly damages peripheral nerves or factors mediating inflammatory response are responsible for the neural damage.NEW & NOTEWORTHY Various neurological manifestations are observed in about 36.4% of patients infected with SARS-CoV-2 and post-COVID neuropathy is one of them. There is lack of studies describing neurophysiological abnormalities in peripheral nerves in case of patients who had SARS-CoV-2 infection. The aim of this study was to analyze changes in peripheral nervous system in case of COVID-19 survivors.