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Background: Post-acute sequelae of SARS-COV-2 infection (PASC) is associated with cognitive impairment (CI) with unclear pathogenesis though blood brain barrier (BBB) impairment and excitotoxic injury appear significant. Post-acute sequelae of SARS-COV-2 infection (PASC) is associated with cognitive impairment (CI) with unclear pathogenesis though blood brain barrier (BBB) impairment and excitotoxic injury appear significant. We hypothesized that PASC CI patients would have brain inflammation and BBB disruption using advanced MR imaging. Method(s): In this prospective longitudinal study, 14 patients with PASC CI (mild and non-hospitalised) were enrolled (mean age of 45;10 F and 4 M) and 10 sex and age matched healthy controls. 13 had a follow up MR at 9-12 months (mean 10 months). All participants underwent DCE perfusion (an index of BBB integrity with Ktrans as the measurement), Diffusion Tensor Imaging (DTI) and single voxel MR spectroscopy (MRS) of the frontal cortex/white matter and the brainstem in addition to brain anatomical MRI. Between group analyses were used to determine which MRI outcomes were significantly different from controls in patients with PASC CI. Result(s): The PASCI CI group showed significantly increased (ie BBB impairment) Ktrans, and increased region (Frontal white matter and Brain Stem)-specific areas in the brain (p=< 0.005), reduction in NAA (ie neuronal injury) and mild reduction of Glx (ie excitotoxicity) in the frontal white matter and brain stem (p=0.004), and reduction in white matter integrity (increased diffusivity -greater radial and mean diffusivity). Increased Ktrans was correlated with increased both radial and mean diffusivity (r=0.9) in all tested brain regions. Ktrans significantly improved in the follow up MR (p= 002596 Z=-2.794872) with no difference between subjects and controls indicating BBB normalisation (p= 0.442418, z= -0.144841). White matter integrity also improved especially in the fractional anisotropy values in the executive networks (p=< 0.00045). MRS showed significant improvement in the NAA in the frontal white matter but Glx remain high as compared to the controls (p=0.0006). Conclusion(s): PASC CI was characterised by reversible diffuse BBB impairment, neuronal/axonal and excitotoxic injury. BBB impairment was associated with white matter disruption. These are suggestive biomarkers for the presence, severity and prognosis of PASC CI. Such biomarkers could underpin appropriate trial design and timing of intervention.
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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.
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Introduction: Accumulating evidence supports that 6 months after COVID-19 almost 80% of the patients present residual neurological manifestations. Some of these symptoms may be associated with autonomic nervous system (ANS) damage. Several possible mechanisms have been proposed, including the detrimental effect of the virus-induced cytokine storm, direct viral spread into the nervous system, and an immune-mediated autoimmune mechanism. The extent of ANS involvement and exact pathological locations are unknown. Method(s): The aim of our study was to characterize the peripheral autonomic nervous system involvement in post-COVID patients. We compared the outcomes of neurological and neurophysiological examinations with the results of asymptomatic control subjects. In our study, we studied 33 patients (20 women, mean age: 39 +/- 8 years) with post-COVID, transient or persistent neurocognitive and/or autonomic nervous system symptoms and 8 (4 women, 29 +/- 5 years) control subjects. After detailed neurological examination, the ANS functional assessment was performed with Quantitative Sudomotor Axon Reflex Test (QSART) and Sudomotor Sympathetic Skin Response (SSR) using Vitalscan SudoCheck + machine. Heart rate variability (HRV) was determined using a WIWE instrument. Result(s): Focal neurological signs were not found in any of the patients. Regarding the autonomic nervous system studies, SSR measurement was the most sensitive in our population: we found abnormal SSR values in 8/33 of our post-COVID patient group (24%), while we did not find any abnormal SSR values in the control group. These 8 post-COVID patients showed moderate to severe (bioelectrical skin conductivity: 24.3-59.4 microS) ANS dysfunction. During the one-minute HRV measurement, we assessed the standard deviation of heart rate variability (SDNN) and the root mean square of the RR intervals (RMSSD). Abnormally low values were measured in 14/33 patients (42%) (SDNN mean: 18-23 ms, RMSSD mean: 13-14 ms). Conclusion(s): In our study, different neurophysiological examination modalities confirmed ANS involvement in post-COVID patients. The extent of cardiovascular autonomic involvement (42%) was higher than sudomotor dysfunction (24%). These ratios significantly exceed the results of similar measurements in the age-matched control group. We suggest that the dysautonomia profile might explain the persistent symptoms after COVID-19. Copyright © 2022
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Introduction: Following acute COVID-19 infection several persistent symptoms (sweating dysfunctions, palpitations, orthostatic intolerance) might be related to immune-mediated disruption of the autonomic nervous system. We present the results of small fiber examination in patients with post-COVID syndrome. Method(s): In our database, we enrolled 32 patients who recovered from COVID-19. After recording the demographic data and post-COVID symptoms, the participants completed the Composite Autonomic Symptom Scale - 31 (COMPASS-31) questionnaire, followed by a complete neurological examination. Sudomotor function was assessed using the Vitalscan Sudocheck machine, while the cardiac function was recorded with Wiwe device. Result(s): Dizziness, fatigue, "brain fog," palpitations, and numbness were the most common symptoms. None of the patients presented any focal signs on neurological examination. The participants were classified into four groups based on neurophysiological findings: patients in group A (n=8, 25%) showed impaired sympathetic skin response, defined as bioelectrical conductivity < 60 microS. Decreased quantitative sudomotor axon reflex test, established as low sweat activity reflex was noted in 5 (16%) patients (group B). Group C (n=14, 44%) consisted of cases with impaired heart rate variability, established as a reduction in any of the time domain indices (SDNN, PNN50, and RMSSD). Group D included 7 (22%) post-COVID patients, who did not present any functional abnormality on the above-mentioned neurophysiological modalities. Despite the small sample size, group B showed the highest COMPASS-31 total score (12.5/100) compared to the other groups (group A: 9/100, group B:10/100). Among all the cases, group B patients presented the highest scores in COMPASS-31 pupillomotor subdomain assessment (4/12). Group A presented lower SDNN and RMSSD indices compared to patients without sudomotor dysfunction (only the RMSSD data showed a statistically significant difference;p< 0,05). Despite our expectations, the scores of the COMPASS-31 secretomotor subdomain in Group A did not show any significant difference compared to the other groups. Examining the COMPASS-31 subdomains in neurophysiologically intact cases in group D, we found that the highest scores pertain to the gastrointestinal subdomain. Conclusion(s): Dysautonomia is becoming more widely evident as a chronic consequence of COVID infection. The COMPASS-31 questionnaire showed a sensitivity of 100% and a positive predictive value of 80%, but we noticed several inconsistencies. The discrepancy between the sub-scores of the small fiber questionnaire and the results of the neuropsychological examination address the need for further extended autonomic studies. Copyright © 2022
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Introduction: This study was aimed to assess the clinical features and electrophysiological subtypes of patients with Guillain-Barre syndrome (GBS) in Istanbul, as well as to analyze the probably different characteristics of COVID-associated GBS. Method(s): From the patients who were admitted to the major hospitals in Istanbul between April 2019 and November 2021, those aged over 18 years and diagnosed as having GBS within the 21 days after the initial symptoms were selected for the study. Electrophysiologic examinations were performed twice within the first 6 weeks along with close clinical observation throughout the acute illness. The patients were divided into groups as those admitted in the pre-pandemic and pandemic periods. The characteristics of the patients who developed GBS after COVID infection (C-GBS) were also evaluated separately. Axonal and demyelinating subtypes were determined according to the previously described electrophysiologic criteria. Result(s): From 12 centers, 134 patients were included in the study. The number of patients diagnosed in the pre-pandemic and pandemic periods were 61 and 73, respectively. Eighteen patients developed C-GBS in the pandemic era. According to Uncini's criteria, 33.6% of the patients were classified as axonal GBS (29/45 patients had reversible conduction failure) and electrophysiological distinction could not be made in 8.2% of the patients. In the second electrophysiological examination performed in 116 patients, the subtype diagnosis was changed in 29 who had been classified according to the Hadden's criteria in the first examination and in 17 who had been categorized by using Rajabally's criteria. Sensory symptoms were found in all C-GBS patients and in only 67.9% of all patients diagnosed during the pandemic (p = 0.006). The frequency of demyelinating subtype was 83.3% in the C-GBS group, and this rate was 47.8% in the patients without recent COVID infection (p = 0.026). While MRC sum score, Hughes score, and modified Erasmus outcome score were not found to be different in the pre-pandemic and post-pandemic groups, C-GBS had lower Hughes score (<3) than the other patients diagnosed during the pandemic (p = 0.040). Conclusion(s): The frequency of axonal GBS in Istanbul, a large metropole inhabiting people from every region of Turkey, seems to be placed in between the values reported from high and low incidence countries. The second electrophysiologic examination is important for precise determination of the subtypes. C-GBS seems to cause more frequent sensory symptoms, demyelinating electrophysiologic characteristics, and moderate clinical features. Copyright © 2022
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Introduction: Since the coronavirus disease 2019 (COVID-19) emerged in Wuhan, neurological complications affecting both the central and peripheral nervous systems have been reported. Multiple etiological mechanisms as immuno-mediation, direct nerve infection, prolonged intensive care units (ICU) hospitalization and prolonged positioning have been proposed as a cause of peripheral lesion. The aim of this study is to report an observational description of peripheral nervous system complications in patients with severe COVID-19. Method(s): We include patients with COVID-19 infections with weakness or sensory deficit, with one or more EMG tests carried out between April 2020 and December 2021. Standard neurophysiological techniques with motor and sensory nerve conductions, F responses and needle EMG exam in representative upper and lower limb muscles were performed. Result(s): A total of 89 patients were included, 66 males (74%) and 23 females (26%), with an average age of 55.7 years old (range from 11 to 90). Most of them (74%) were studied during hospitalization (16 of them during ICU admission). Nearly all patients (90%) had a prolonged ICU hospitalization (between 8 and 120 days). The reason for consultation was diffuse or focal weakness, difficulty in weaning, facial palsy or sensory deficits. The results of EMG tests showed myopathic findings in 61% of patients, focal peripheral nerve lesions in 64%, Guillain-Barre syndrome (GBS) in 5 (6%), and other types of peripheral polyneuropathy in 24%. From peripheral nerve injuries, peroneal neuropathy was the most frequent (58%), brachial plexopathy was found in 26%, median neuropathy in 25%, ulnar in 11%, lateral femoral cutaneous in 9%, axillary and spinal in 5%, radial and hypoglossal in 4% and musculocutaneous in 2%. Tapia's syndrome was diagnosed in two patients. Peripheral nerve injuries correlated with longer admissions in ICU and prone positioning. The follow-up studies showed a good recovery from myopathy but persistent motor sequelae in axonal GBS patients and in most peroneal nerve injuries. Neurophysiological findings are described. Conclusion(s): Peripheral nerve complications are frequent in patients affected by severe COVID-19 and prolonged hospitalization, mainly focal nerve injuries (61%), critical illness myopathy (64%) and peripheral polyneuropathy (30%) including GBS (5 patients). Prone and prolonged positioning in ICU may be associated with peripheral nerve injuries although other mechanisms cannot be excluded. Copyright © 2022
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Introduction: Guillain-Barre syndrome (GBS) is an acute inflammatory neuropathic illness with striking clinical manifestations and significant morbidity. Rare sporadic cases of GBS have been described following vaccination. This case in particular was temporally related to the Sinovac-CoronaVac (COVID-19) vaccine. A causal link with the vaccine is not proven but is possible and warrants further investigation. Hence, the objective of this study is to describe a case of GBS after COVID-19 vaccination. Method(s): Case report Results: 55-year-old Filipino woman presented with progressive symmetric ascending weakness of bilateral upper and lower extremities which began four weeks after receiving vaccination with the Sinovac-CoronaVac (COVID-19) vaccine. She also described concurrent facial weakness, dysphagia, and paresthesia of both feet. Fortunately, there were no clinical findings of dysautonomia. On neurological examination, she had facial diplegia, quadriparesis with lower extremity predominance, manual muscle testing revealed 4-/5 on bilateral upper limbs and 2/5 on bilateral lower limbs. The deep tendon reflexes were absent generally. The cerebrospinal fluid analysis showed protein-cytological dissociation and the nerve conduction study (NCS) revealed generalized motor axonopathy. Thus, the patient was managed as GBS, specifically the Acute Motor Axonal Polyneuropathy (AMAN) variant. She underwent plasma exchange in 5 sessions over 10 days with significant clinical improvement at a 4-week follow up visit. Conclusion(s): Only a few cases of GBS after COVID-19 vaccination have been reported. Among these, AMAN is uncommonly described. In patients with GBS, several viral and bacterial pathogens have been found in several studies but the factors that induce the immune-mediated destruction of the nerve tissues need more rigorous research. Copyright © 2022
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Introduction: Neurological complications of SARS-CoV-2 disease have received growing attention, but only few studies have described to date clinical and neurophysiological findings in COVID patients during their stay in intensive care units (ICUs). Here, we assessed the presence of either critical illness neuropathy (CIP) or myopathy (CIM) in ICU patients. Method(s): Patients underwent a neurophysiological assessment, including bilateral examination of the median, ulnar, deep peroneal and tibial motor nerves and of the median, ulnar, radial, and sural sensory nerves. Needle electromyography (EMG) was performed for both distal and proximal muscles of the lower and upper limbs. The technique of Direct Muscle Stimulation (DMS) was applied either to the deltoid or tibialis anterior muscles. Peak to peak amplitudes and onset latencies of the responses evoked by DMS (DMSamp, DMSlat) or by motor nerve stimulation (MNSamp, MNSlat) were compared. The ratio MNSamp to DMSamp (NMR) and the MNSlat to DMSlat difference (NMD: MNSlat-DMSlat) were also evaluated. Result(s): Nerve conduction studies showed a sensory-motor polyneuropathy with axonal neurogenic pattern, as confirmed by needle EMG. Both MNSamp and NMR were significantly reduced when compared to controls (p < 0.0001), whereas MNSlat and NMD were markedly increased (p = 0.0049). Conclusion(s): We have described COVID patients in the ICU with critical illness neuropathy (CIP). The predominance of CIP as compared to critical illness myopathy (CIM) has implications for the functional recovery and rehabilitation strategies in severe COVID-19. Copyright © 2022
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Introduction: Cladribine is a CNS penetrant disease-modifying treatment, which - in an oral preparation (Mavenclad) - was licensed for people with highly active relapsing MS in August 2017. Our experience with cladribine dates back to 2014 when we started using subcutaneously injected cladribine as a compassionate immunotherapy in people with MS (pwMS) off-label. This programme enabled us to embed CLADRIPLAS, a mechanistic study of the effect on intrathecal B cell and plasma cell function and axonal damage focussing on progressive MS (PMS) (IRAS # 240360). Objective(s): To study the effect of cladribine on peripheral and intrathecal B and plasma cells. Aim(s): To study the effect of cladribine on oligo-clonal bands (OCB) and the level of neurofilament light (NfL) chain. Method(s): Observational study involving two lumbar punctures and phlebotomies, 6-12 months apart, to collect B cell subsets, and intrathecal plasma cell as well as neurofilament light chain (NfL) level in pwMS eligible and not eligible for cladribine treatment based on cerebro-spinal fluid (CSF) NfL, clinical and/or MRI evidence of inflammatory disease activity. Here, we report baseline cohort characteristics. Result(s): Thirty-eight pwMS were recruited (19 women, 19 men) and had their first sample collections. Eight pwMS were eligible for cladribine treatment (7 based on elevated NfL, 1 due to MRI activity). Follow-up samples have been collected in 21. Mean age at baseline was 55 years (40-76). Fourteen had primary, 24 secondary PMS. Median EDSS=6.5 (3.5-8). Twenty-one pwMS had been treated with DMT before consideration of cladribine, 17 were immunotherapy-naive. Mean CSF-NfL level was 552 (176- 2072) pg/ml. Conclusion(s): Despite restrictions due to COVID-19, 38 of 40 planned pwMS were enrolled. 7/8 were eligible based on CSFNfL level indicating the importance of using biomarkers other than MRI to establish disease activity in PMS. We expect our cohort to enable meaningful comparison between groups. CLADRIPLAS will finish in early 2023.
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Case Diagnosis: Post COVID-19 infection multisystem inflammatory syndrome in adults presenting in a 28-year-old African American female with pain, ascending weakness, paresthesias, and chest pain. Case Description or Program Description: Patient with documented COVID-19 infection 5 weeks prior to arrival and presented with pain, paresthesias, and weakness in the bilateral lower extremities. Symptoms began shortly after patient recovered from COVID-19 infection, however, patient developed ascending weakness extending into the hands with left sided chest pain, prompting patient to present for evaluation. Setting(s): Major academic and referral center with level 1 adult trauma. Assessment/Results: Lumbar puncture and cerebrospinal fluid studies were not suggestive of Guillain- Barre syndrome. Imaging of the entire neuraxis was unremarkable. Echocardiogram revealed new onset heart failure with reduced ejection fraction of 35% consistent with non-ischemic cardiomyopathy and cardiac imaging was not suggestive of amyloidosis. EMG was consistent with primarily axonal greater than motor peripheral polyneuropathy. Further inflammatory workup revealed elevated erythrocyte sedimentation rate and C reactive protein. Paraneoplastic workup was unremarkable. Patient was started on intravenous immunoglobulin (IVIG) for suspected Multisystem Inflammatory Syndrome in Adults (MIS-A), however, patient developed infusion reaction shortly after infusion began and IVIG was discontinued pending hemodynamic stability. Discussion (relevance): Post COVID-19 multisystem inflammatory syndrome is seen more commonly in children than in adults per literature review. Clinicians must be mindful of potential MIS-A in adult patients with symptoms mimicking Guillain-Barre syndrome with negative workup and imaging, especially with concomitant cardiovascular compromise and elevated inflammatory markers. This case demonstrates one of the various presentations documented of MIS-A and is important for accurate diagnosis of this syndrome in the future. Conclusion(s): Post COVID-19 patients presenting with symptoms similar to Guillain-Barre syndrome with negative workup should be evaluated for MIS-A as this syndrome can affect multiple organ systems simultaneously, such as the nervous system and cardiovascular system as seen in this patient.
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Case Diagnosis: Critical Illness Polyneuropathy following COVID-19 Infection Case Description or Program Description: A 58-yearold female presented to PM&R clinic following a prolonged hospitalization for COVID-19, after which she developed bilateral wrist and foot drop. Prior to her hospitalization, she ambulated independently. At the time of evaluation, she was wheelchair bound. Physical exam demonstrated bilateral wrist and foot drop, with bilateral foot numbness. EMG demonstrated bilateral common peroneal incomplete axonal neuropathy, with bilateral incomplete axonal brachial plexopathy affecting mainly the lower trunk on the left side and the middle cord on the right side. Setting(s): Tertiary-care teaching hospital Assessment/Results: Outpatient physical and occupational therapy were initiated and she was prescribed bilateral ankle foot orthoses. She was referred to neurology, with diagnosis of multifocal axonal neuropathy, with critical illness polyneuropathy, post viral (COVID) immune axonopathy with mononeuritis multiplex. Repeat EMG obtained 8 months later demonstrated mild improvement on the needle study with decreased denervation potentials of the muscles tested however, NCS remained unchanged. Over a 1-year span of therapy, her strength, function and numbness improved. She progressed to ambulate independently without an assistive device. Discussion (relevance): Critical illness polyneuropathy (CIP) is a neurologic manifestation of systemic inflammatory response syndrome, causing axonal injury by unclear mechanism. It is suspected that distal nerve microcirculation causes ischemia and axonal degeneration. There are increasing reports of polyneuropathy following COVID-19 infection. Diagnosis involves EMG, which demonstrates axonal loss without demyelinating features, with NCS showing decreased amplitude of SNAPs. CIP treatment includes reduction of dose and duration of steroids and neuromuscular blocking agents, rehabilitation programs, and careful extremity positioning. Conclusion(s): Our patient experienced functional improvement with conservative management, including outpatient physical and occupational therapy with bracing. As the COVID-19 pandemic continues, CIP must be considered in patients with weakness and a history of COVID-19 infection, particularly in those with severe infection and ICU stay.
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Case Diagnosis: Femoral neuropathy secondary to iliacus/iliopsoas hematoma Case Description or Program Description: A 66-year-old Hispanic male presented to PM and R clinic with left leg weakness and left anterior thigh numbness. His symptoms began during a recent hospitalization for COVID-19 pneumonia, during which he developed left thigh pain, swelling, and weakness while on warfarin for a DVT. CT revealed a 6.5 cm by 3.0 cm by 7.4 cm intramuscular hematoma of the left iliacus/iliopsoas. PM and R and physical therapy evaluated him while inpatient. While he was previously independent in ambulation, he was discharged home with a walker. Setting(s): Tertiary-care teaching hospital Assessment/Results: Upon follow up in PM and R clinic, he ambulated with a rolling walker with frequent falls, noting difficulty with activities of daily living. Physical exam demonstrated 1/5 left hip flexion and knee extension strength, without left thigh light touch sensation. EMG demonstrated acute denervation of the left quadriceps muscle with no MUAP recruitment on activation, suggestive of an axonal femoral neuropathy. He began outpatient physical therapy, with improvement in ambulation and ADLs. Five months post hematoma, he continues to ambulate with a rolling walker, however with no further falls. Discussion (relevance): The femoral nerve originates from the posterior division of L2-L4 and runs between the psoas tendon and iliacus muscle under the inguinal ligament. Femoral nerve compression occurs along the iliopsoas gutter, where it is at highest ischemia risk due to poor vascular supply. Iliacus hematoma occurs most commonly in patients with hemophilia and those receiving anticoagulation. Treatment is typically conservative;however, embolization may be indicated with active bleeding. In cases of femoral neuropathy, early iliacus muscle fasciotomy with or without hematoma evacuation may be considered. Conclusion(s): Our patient's femoral neuropathy was managed conservatively with physical therapy, and he experienced improvements in strength, ambulation, and function. Femoral neuropathy should be considered in patients with weakness following thigh intramuscular hematoma.
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Case Diagnosis: Severe global axonal neuropathy Case Description or Program Description: A 36-year-old healthy male presented with fever and body aches, found to be COVID positive. During hospitalization, he became hypoxic and obtunded, requiring emergent intubation. Physical exam notable for flaccid paralysis of extremities with diffuse atrophy. Patient opened his eyes without following commands. He was treated with a prolonged course of steroids, Remdesivir and antibiotics for COVID-19 pneumonia. Due to diffuse weakness, Nerve Conduction Studies (NCS)/ Electromyography (EMG) performed showed acute motor and sensory axonal neuropathy (AMSAN), a severe subtype of acute inflammatory demyelinating polyneuropathy (AIDP). Subsequently, the patient received 5 days of IVIG and 7 sessions of plasmapheresis but failed extubation. Setting(s): University hospital Assessment/Results: On NCS, the patient had absent sensory and motor responses to direct stimulation in the extremities. The sensory study included radial, ulnar, median and sural nerves. The motor studies included musculocutaneous, median, ulnar, peroneal and tibial nerves. EMG of selected extremities revealed fibrillations in all major muscle groups. Muscle recruitment was not assessed due to mental status. Neurological work-up including CTH and MRI spine was negative for a central process. LP was inconclusive. Additional studies notable for anti-ganglioside GQD1 antibody positive. Discussion (relevance): There continues to be neuromuscular compromise precipitated by COVID-19. The patient presented with global weakness of the entire musculature including the muscles of respiration. As evidenced by anti-GQ D1 IgG antibody and NCS/EMG, the patient presented with acute motor and sensory axonal neuropathy (AMSAN), which is the most severe form of AIDP. Conclusion(s): Within the literature, anti-ganglioside antibodies occur within 25% of AIDP and 50% of patients with the axonal variant of AIDP. There has been previous investigation regarding the relationship of Anti-ganglioside GM antibodies in GBS and the relationship with Campylobacter Jejuni infection. It is important to identify that this same relationship may be present with COVID-19, necessitating further research.
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Objective: After the outbreak of the global pandemic caused by SARS-CoV-2 infection at the end of the year 2019, it took one year to start vaccinatioagainst this infection with products from various manufacturers. As of November 2021, more than 8 billion vaccine doses against COVID-19 havbeen administered, which is essentially linked to a spike in adverse events reports following these vaccinations, including a number of neurologicaadverse events. Case Report: We report a case of a 71-year-old patient with lethal fulminant onset of Guillain-Barre syndrome after the second dose of mRNA vaccintozinameran. This is, to our best knowledge, the first case report of this adverse event supported by autopsy and histological examination. Thpatient presented with progressive ascending weakness and paresthesia, with typical cytoalbuminologic dissociation in cerebrospinal fluid ansevere motoric and sensitive axonal-demyelinating polyneuropathy on electromyography. The patient's history and complex diagnostic workup dinot reveal any other possible causative factors. The patient did not respond to the treatment with intravenous immunoglobulins and died 10 daylater due to aspiration bronchopneumonia as a complication of respiratory muscles paralysis. Conclusion(s): Most of the reported adverse reactions following COVID-19 vaccination include mild or moderate events noticed in the post-vaccination periodhowever, reports of possible lethal outcomes are no exception. Still, the overall incidence of GBS after vaccination does not significantly exceed itincidence in the general population. Each such report should be carefully examined by a team of specialists to prevent overestimation of lethaadverse events linked to vaccinations, especially in fatalities that happen in the post-vaccination period. Copyright © 2022 Mosna et al.
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Specific clinical, electrophysiological and serological features are used to recognise a phenotype fitting the atypical chronic inflammatory demyelinating (CIDP) variant spectrum. We report a 28-year-old male patient, without any significant history apart from a recent asymptomatic COVID-19 infection, presenting at first with bilateral facial nerve palsy, subsequently -three months later- developing an subacute onset symmetric sensory ataxia and arefl exia, and thirdly experiencing diffuse rapidly progressive motor deficits. Additional investigations suggested an autoimmune polyneuropathy: Liquor analysis showed cytoalbuminologic dissociation. Cerebrospinal fluid protein elevation was remarkable: 631 mg/dL. Nerve conduction studies showed prominent distal latencies prolongation and dispersion of the potentials, meeting the electrodiagnostic criteria of the European Federation of Neurological Societies/Peripheral Nerve Society for CIDP (2021). Full spine magnetic resonance imaging depicted pathological thickening and enhancement of the roots of the cauda equina as seen in radiculitis. There was no or poor response to conventional treatment, i.e. immunoglobulins (IVIG), corticosteroids and even plasmapheresis. Muscle weakness deteriorated. Presence of serum IgG4 anti- contactin-1 (CNTN1) antibodies was found by ELISA identifi- cation and titration, and the patient improved substantially after rituximab treatment. While contributing to the expanding confidence in nodal and paranodal antibodies as valuable biomarkers in clinical practice, our case entails several peculiarities: 1/ SARS-CoV2 positivity as a possible trigger of this auto-immune polyneuropathy 2/ A considerably younger age of onset than in the patients already described (range 33-76 years). 3/ The clinical course progressed in an atypical manner even for atypical CIDP: Initial presentation with bilateral asymmetric facial palsy, followed by sensory ataxia, which prompted the initial diagnosis of Miller-Fisher syndrome, and later development of severe motor impairment. 4/ Proteinorachy was so pronounced that we considered neuroborreliosis as a potential associated disorder. Borrelia seroconversion occurred after the first IVIG-treatment, and could be false positive. However, the patient was treated with intravenous ceftriaxone, which had no effect on the clinic. 5/ Antibodies against CNTN1 were undetectable after 2 months of rituximab. Emphasising the both diagnostic and therapeutic importance of recognising a phenotype compatible with atypical CIDP, an underrecognized and consequently undertreated disease where early diagnosis and prevention of axonal damage is crucial in.
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COVID-19-related neuropathy in Colombia: The experience during the first 23 months of pandemic Introduction: The SARS-CoV-2 virus has a high neuroinvasive capacity due to the increased expression of angiotensin-converting enzyme receptor 2 (ACE-2) in neurons (1) and it is believed that the mechanism by which it can cause injury to the nervous system peripheral nervous system is immunemediated, although a direct cytotoxic effect of the virus cannot be ruled out (2). Multiple types of neuropathy associated with SARS-CoV-2 infection have been described, the most frequent being Guillain- Barré syndrome, pre-existing diabetes, compression neuropathies and drugs used to treat symptoms of COVID-19 (3). Objetives: To characterize the patients who were referred to the electromyography laboratory at the Fundacion Santa Fé de Bogotá, Colombia due to suspected COVID-19-related neuropathy Methods: Descriptive observational study, case series type. The electrodiagnostic studies carried out between January 2020 and December 2022 in the electromyography laboratory at the Fundacion Santa Fé de Bogotá, Colombia with suspected COVID- 19-related neuropathy were reviewed. Results: 94 patients were evaluated in the electromyography laboratory with suspected COVID 19-related neuropathy between January 2020 and December 2021, of which 53% (50/94) were men. The average age was 54.8 years. 32% (30/94) had severe COVID and 31% (29/94) were hospitalized in the ICU. Most of the studies were normal: 35% (33/94). of the abnormal findings, it was found in order of frequency: Symmetric motor and sensory axonal polyneuropathy in 21.2%, and of this group of patients, 55% were in the ICU, 35% had no data and 20% were hospitalized-not ICU. 18% presented compression neuropathy of the median nerve in the carpal tunnel, 6.3% asymmetric motor and sensory axonal neuropathy, 6.3% suggestive findings of cervical and/or lumbosacral root involvement, 4.2% Guillain Barré syndrome, 4.2% compression neuropathy of the peroneal nerve , 2.1% brachial plexus axonal injury, 2.1% peroneal nerve axonal injury, 2.1% radial axonal injury, 2.1% myopathic changes, 1% hypoglossal nerve axonal injury, 1% symmetric axonal and demyelinating polyneuropathy, 1% hereditary neuropathy, 1% asymmetric demyelinating neuropathy, 1% axonal injury of the sciatic nerve, 1% axonal injury of the median nerve in the forearm, 1% axonal injury of the lumbosacral plexus, 1% compression neuropathy of the ulnar nerve in the elbow and 1% axonal injury from a sensory branch of the median nerve. Conclusions: The most frequent abnormality in the study was symmetric motor and sensory axonal polyneuropathy, which can be explained by the prolonged ICU stay, which increases the risk of Critical illnes Neuropathy.
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Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. This is the first systematic clinical guideline, developed by an international task force using formal GRADE methodology. The diagnostic criteria remain primarily clinical, based on history and examination findings of acute progressive limb weakness and areflexia. Variants of GBS may include motor GBS, Miller Fisher Syndrome, and regional variants with weakness predominantly in lower limbs, face, or pharynx/neck/ arms. The differential diagnosis is wide. When uncertain, diagnosis may be assisted by nerve conduction tests, raised cerebrospinal fluid protein, and less often by MRI spine with contrast, or serum antibodies to gangliosides (especially for variants) or nodalparanodal antibodies (especially if not improving). Axonal versus demyelinating subtyping does not affect clinical management. A history of recent gastrointestinal or respiratory infection or of surgery may support the diagnosis. The risk of GBS is only very slightly increased after Covid-19 infection and after the adenovirus-vector vaccines to SARS-CoV2 (AstraZeneca or Johnson & Johnson) but not mRNA vaccines. Immune treatment is recommended with intravenous immunoglobulin or plasma exchange, for most patients except those mildly affected or after four weeks from onset. A repeat course is reasonable after a treatment-related fluctuation. Corticosteroids are not recommended. There is no evidence of benefi t from any other disease-modifying treatment. Respiratory function should be monitored by forced vital capacity and single breath count to assess the risk of needing mechanical ventilation, guided by the mEGRIS scale. Pain is very common. It may be musculoskeletal or neuropathic, and treated with gabapentin, tricyclic antidepressants or carbamazepine. Patients who fail to improve should be reassessed for the correct diagnosis and for axonal degeneration. Around 5% of patients with GBS may later develop CIDP but no test can reliably indicate this within the first eight weeks. Nodal-paranodal antibodies should be tested if CIDP is suspected or if the patient is not recovering well. The long-term outcome is less good in patients of older age, with preceding diarrhoea, or more severe weakness, as quantified by the mEGOS scale, and also in patients with smaller motor potential amplitudes or raised serum neurofilament light chain level.
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Introduction: During Covid-19 pandemic periods, various studies have been revealed the coexistence of these two diseases, raising the question of whether SARS-CoV-2 has a role in triggering GBS or it's just co-incidentally. So far, 255 cases of this concurrence have been reported. In this study, we publish 45 patients' demographic, clinical, electro diagnostic study, response to treatment and prognostic features association of Covid- 19 and GBS during the 5 corona's epidemiologic peaks in Isfahan province. Methods: This cross-sectional, multi-central study was performed during covid-19 pandemic since 2020 February until 2021 October. In this period 5 epidemiologic peaks of corona virus occurred in Isfahan (one of providence of Islamic republic of Iran) and total of 417166 people became infected. 45 patient with definitive Covid-19 (based on positive nasopharynx Reverse transcription polymerase chain reaction (RT-PCR) or highly suggestion of Highresolution computed tomography (HRCT) for covid- 19) were referred to one of the 2 referral hospitals (Alzahra and Kashani hospital). All patients whom suspected of peripheral nerve symptoms referred to the neuromuscular fellowship for further examination and performing EDx. Demographic, clinical, therapeutic and prognostic features were collected according to Hospital records. Results & discussion: 45 patients (60% male, 40% female) were surveyed. The mean age was 54.66±10.021 (max: 84, min:14, range:80). The most EDx pattern was AIDP (57.8%, n=26).42.2%(n=19) of patients had axonal pattern. 8 of them were Acute motor axonal neuropathy(AMAN) and 11 patients were Acute motor-sensory axonal neuropathy(AMSAN). The most (91%) GBS phenotype was classic pattern which defined as acute-sub acute flaccid length dependent paralysis. 2 patients had pure para paretic pattern and 2 had miller-fisher pattern. The most common symptom of covid-19 was fever (89.7%), Other symptoms included dyspnea (48.7%), cough (46.2%), myalgia (28.2%), headache (28.2%), diarrhea (28.2%) and the less common was anosmia (12.8%). No significant difference was found between any of the covid-19 symptoms with EDx patterns. 7 patients had a history of GBS which were more than 1 year before the onset of new symptoms. 13.6% of patients had no any symptoms of covid-19 on the day of the onset of neurological symptoms, either the symptoms of covid-19 developed after the neurological symptoms or covid-19 was discovered accidentally. Mean distance between onset of covid-19 and neurological symptoms was 18.05±8.88 which was significantly lower in the axonal injury groups (12.00±800 pvalue: 0.013). Also There was also signifi cant difference between frequency of para/post infectious patient in axonal and demyelinating subtypes (p value: 0.045). So that Para infection was more associated with axonal injuries. Among other prognostic findings, include respiratory equipment (33% no equipment, 44% none-invasive and 22.2% mechanical ventilation), required to ICU admission (46.7%), length of ICU admission (16.66 ±12.03), length of intubation (12.10±6.24) , length of hospitalization( 23.66±14.13) and mortality(8.9%) no Significant differences were detected among each subgroups of EDx patterns and also between axonal/ demyelinating injuries. There was also significant difference among erythrocyte sedimentation rate and C-reactive protein among axonal patterns that means axonal patterns (AMAN and AMSAN) had more level of ESR and CRP at the first neurological symptom's day.
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Background: We present a case of a 36 year-old female who developed Acute Immune-mediated Demyelinating Polyneuropathy (AIDP) after receiving the second dose of Pfzer COVID-19 vaccine. Objectives: To report a rare auto-immune complication of COIVD-19 vaccination. To educate and inform physicians about the approach to diagnosing AIDP and narrowing down its etiology. Methods: Case report and literature review Results: A 36 year-old female with no signifcant past medical history presented to the hospital with progressive bilateral paresthesia. She started to experience numbness and tingling sensation in her extremities 1 week after receiving the second dose of Pfzer COVID-19 vaccine. Following 5 days of symptoms onset, she was no longer able to hold onto objects and experienced difficulty ambulating without assistance. Physical exam was notable for decreased distal sensation to touch and pain in all 4 limbs, otherwise, the rest of her neurological and musculoskeletal evaluation was normal. MRI-head showed small scattered foci of increased FLAIR signal in the white matter, suggesting an underlying infammatory process. Electromyography (EMG) was performed and showed evidence of acute diffuse sensorimotor neuropathy with mixed axonal and demyelinating features. These results along with the clinical features allowed us to diagnose our patient with Acute Immune-mediated Demyelinating Polyneuropathy (AIDP). Extensive autoimmune workup, including anti-GM1, GD1b, Gq1b, ANA, DS-DNA, RF, CCP, and C/P ANCA, were unremarkable. She had positive anti-Ro atb but did not have any clinical or physical features that would suggest Sjogren's Syndrome. Vitamin levels (B12, folate, thiamine) were found to be normal. Infectious workup of serum and CSF which included hepatitis serologies, Campylobacter jejuni serology, Lyme atb, CMV atb, EBV atb were all negative. The possible etiology of her disease was attributed to Pfzer COVID-19 vaccine given the temporal correlation. She was subsequently treated with 6 cycles of IVIG which resulted in moderate symptomatic improvement. Conclusion: AIDP is an autoimmune-guided infammatory neuropathy which result in axonal degeneration of myelinated nerves [1]. In some extremely rare cases, molecular mimicry following vaccination may lead to this disease [1]. There have been reports of AIDP linked to Johnson & Johnson and AstraZeneca COVID-19 vaccines [2]. Recently, a few cases have also been observed with Pfzer COVID-19 vaccine [2-3]. Interestingly, the majority of these cases occurred after the frst dose of the vaccine, making our case even more peculiar [2]. We report this case as physicians should be made aware that AIDP is a potential complication of COVID-19 vaccination. Given the extreme rarity of these cases, it is also important to note that more common infectious and autoimmune etiology of AIDP should be investigated before attributing any potential causal relationship to COVID-19 vaccines.
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Objectives: To assess and compare the admission rates of medical complications (MC) after Bariatric and metabolic surgery (BMS) over a period of 6 years prior to and during the pandemic. Bariatric and metabolic surgery could be associated with MC, including malnutrition and neuromuscular complications (NC). Methods: Retrospective study of all patients admitted to Hamad General Hospital, Qatar, with post-BMS MC before (n=12, January 2014-December 2019) and during the pandemic (n=36, January 2020-31 May 2021). We assessed 17 nutrients, nerve conduction/electromyography diagnosed NC, and we explored whether patients had clustering of gastrointestinal symptoms, barium meal findings, excess weight loss percentage (EWL%), or non-compliance with post-BMS clinic visits and multivitamin supplements. Results: The sample comprised 95.8% sleeve gastrectomies, mean age was 26.62 years, and 54.2% were women. Admissions increased from pre-pandemic 0.29 per 100 BMS to 11.04 during the pandemic (p<0.0001), despite no significant differences in patients' demographic/surgical profiles, nutrient deficiencies, or MC characteristics. Across the sample, the most frequent neuropathies were mixed sensory/motor/axonal;albumin and total protein deficiencies were observed in 54.2% and 29.2% of patients, respectively (no pre-pandemic/ pandemic differences). Most frequent micronutrient and trace element deficiencies were potassium, vitamin D, and zinc (no pre-pandemic/pandemic differences). Admitted patients had high non-compliance with multivitamins supplementation (87.5%), high post-BMS nausea/vomiting (66.7%, 62.6%, respectively), high EWL% (mean=74.19±27.84%), no post-BMS outpatient follow up (75% during pre-pandemic, 88.9% during pandemic) (no pre-pandemic/pandemic differences for all), and gastroesophageal reflux (higher during the pandemic, p=0.016). Conclusion: Despite the reduced number of BMS during the pandemic, hospital admissions of MC significantly increased.