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1.
Multiple Sclerosis and Related Disorders ; 59, 2022.
Article in English | EMBASE | ID: covidwho-2004360

ABSTRACT

Background: CNS involvement in CLL is rare and it usually occurs in late-stage CLL disease. There is usual delay in the diagnosis due to its variable manifestations, challenging diagnosis process and possible misdiagnosis with a mimicker condition. I am sharing our relative successful experience with this challenging case that had satisfied outcome after going through comprehensive investigations and treatment journey treating his symptoms until arriving the final diagnosis and getting the best treatment option. Material(s) and Method(s): A 42 years old male, with recent COVID-19 infection, presented with multiple progressive neurologic symptoms over one month;started as numbness around the mouth, reduced facial sensation and a feeling of band like sensation below the costal margins. On exam, he had left abduction restriction, diplopia on left gaze and upbeat nystagmus, reduced facial sensation and hyperesthesia. The reflexes were 1+ in the upper limbs, 3+ in the lower limbs, up going planters, tingling from the feet up to T6 level and postural tremor bilaterally. His CSF showed high protein level. MRI brain/ spine revealed left frontal juxtacortical white matter and bilateral middle cerebral peduncles lesions with post-contrast enhancement and long segment spinal cord demyelinating plaques. He was initially treated as a case of Acute disseminated encephalomyelitis (ADEM) post viral infection in a background of CLL. The delayed diagnosis was due to temporal relation of neurological manifestation to viral infection, similar MRI lesions to ADEM and multiple negative CSF results of cytology and flow cytometry. He had persistent disabling symptoms and enhancing lesions in MRI despite being treated with IVMP, IVIG and PLEX. He was managed for ADEM based on responsiveness to the recommended therapy step by step. Firstly, he received a high-dose corticosteroids, secondly IV immunoglobulin but he was still progressing and considered as steroid-unresponsive ADEM. lastly, plasma exchange was done when he exhibited progressive symptoms with fair improvement. Interestingly, the patient showed significant improvement in the clinical and radiological parameters after starting him with a new anti-leukemia medication (Acalabrutinib) for his concurrent active condition. He run out of his medication for around 1 week and he experienced recurrent of the neurological manifestation and the previous lesions in the images. A repeated flow cytometry for the third time came positive for CLL cells and the final diagnosis of CNS involvement by CLL was established. The diagnosis was made after the exclusion of other etiologies. Result(s): The patient received Ibrutinib at a standard dose and as a monotherapy. It is an efficient chemotherapy that crosses the blood brain barrier and has showed a favorable clinical, biological and radiological outcome. The patient is back to his work and his daily activities have improved. Conclusion(s): In case of inconclusive work up, CSF analysis should be repeated testing for cytology and flow cytometry\immunophenotypes as the false negative results are common. Our patient had an active CLL proved in his investigations, and the fact that the patient responded very well to the new chemotherapy should alert the diagnosis of CNS involvement by CLL and directs towards repeating investigations and introducing aggressive treatment strategy to target both hematological and neurological complications of the condition.

2.
Cureus ; 14(6): e26258, 2022 Jun.
Article in English | MEDLINE | ID: covidwho-1969744

ABSTRACT

Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disease of the central nervous system, commonly triggered by viral infections or after immunization. ADEM occurrences in adults are rare. Full spectrum of complications is unknown for novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mRNA vaccines. A previously healthy 44-year-old female presented to the emergency room (ER) with acute onset of tingling, numbness, and weakness of both lower extremities, urinary retention, blurred vision in right eye, and midline lower back pain. Physical examination revealed bilateral lower extremity weakness 1/5, absent deep tendon reflexes, and decreased sensation. She received the first dose of SARS-CoV-2 vaccine six days prior to presentation to ER. Imaging of her lumbar spine and head were consistent with an active demyelinating plaque consistent with demyelinating disease either multiple sclerosis (MS) or ADEM. The patient was started on SoluMedrol 500 mg IV twice daily for five days. Serological workup and CSF analysis were nonsignificant except for Mycoplasma pneumonia IgM, elevated myelin basic protein, and positive IgG, IgA, and IgM. Patient gradually improved and was transferred to rehabilitation. Repeat MRI brain and spine showed improvement in previous lesions. However, she had worsening left eye symptoms that prompted her transfer to another facility for plasmapheresis. Plasma exchange was done for five treatments for ADEM. Patient started noticing improvement in vision and was discharged on steroid taper. We report a case of a possible association between ADEM and SARS-CoV-2 mRNA vaccine. It should be considered in the differential diagnosis in any case suggestive of acute demyelination after COVID-19 vaccination.

3.
Radiol Case Rep ; 17(8): 2824-2825, 2022 Aug.
Article in English | MEDLINE | ID: covidwho-1946366
4.
Mult Scler ; : 13524585221109397, 2022 Jul 13.
Article in English | MEDLINE | ID: covidwho-1928025

ABSTRACT

BACKGROUND: Limited information is available on associations between COVID-19 vaccines and central nervous system (CNS) demyelinating diseases. OBJECTIVES: We investigated potential safety signals for CNS demyelinating diseases related to COVID-19 vaccines using the World Health Organization pharmacovigilance database. METHODS: Disproportionality analyses of CNS demyelinating disease following COVID-19 vaccination were performed by calculating the information component (IC) or the reporting odds ratio (ROR) compared with those for the entire database and for all other viral vaccines. RESULTS: We identified 715 cases of optic neuritis, 515 of myelitis, 220 of acute disseminated encephalomyelitis (ADEM), and 2840 total CNS demyelinating events adverse drug reactions from July 2020 through February 2022. For mRNA-based and ChAdOx1 nCoV-19 vaccines, there were no potential safety signals of disproportionality for optic neuritis (IC025 = -0.93, ROR025 = 0.38; IC025 = -1.76, ROR025 = 0.26), myelitis (IC025 = -0.69, ROR025 = 0.50; IC025 = -0.63, ROR025 = 0.53), ADEM (IC025 = -1.05, ROR025 = 0.33; IC025 = -1.76, ROR025 = 0.20), or overall CNS demyelinating disease events (IC025 = -0.66, ROR025 = 0.52; IC025 = -1.31, ROR025 = 0.34) compared with other viral vaccines. CONCLUSION: As with other viral vaccines, our disproportionality analyses indicate that the risk of COVID-19 vaccine-associated CNS demyelinating disease was low.

5.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927834

ABSTRACT

A 27- year-old previously healthy man presented to the emergency department with a 3-day history of confusion, urinary retention, cough, slurred speech, intermittent vertigo, and unsteady gait after being started on outpatient abx for diagnosis of community acquired pneumonia at an outside facility. On presentation, his vital signs were significant for a Temp. of 102.4 F, a HR of 118 bpm, a RR of 22 bpm, a BP of 135/75 mmHg, O2 sats down to the 80s% with a new oxygen requirement of 3 L/min. His neurological exam was remarkable for dysarthria, a wide based and unsteady gait, forward drift on standing, bilateral dysmetria and was otherwise non-focal. Bilateral basilar rales were noted on lung exam. Initial workup was significant for a sodium level of 129, potassium 3.1, AST 178, ALT 31. His urine legionella antigen test was positive and COVID-19 PCR test was negative. CT of the chest demonstrated multifocal pneumonia. The predominant neurological picture on presentation warranted a lumbar puncture as well as an MRI of the brain, both of which came back negative. HIV test was negative as well. The spectrum of neurological manifestations of legionnaire's disease is quite wide ranging from simple headaches to acute disseminated encephalomyelitis (ADEM). While up to 40% of patients may present with headaches and some confusion, the specific cerebellar presentation is quite rare and has been described in case reports. According to Shelburne(1), there has been instances where the neurological manifestations have lasted up to 3 years after resolution of the pulmonary disease. In a review article detailing 29 cases of cerebellar dysfunction in Legionnaire's disease, a lumbar puncture performed in 16 of the cases showed that the majority had no abnormalities in CSF studies. The propensity for Legionella to specifically manifest as cerebellar Sx remains to be studied. Interestingly, another case report of two patients with neurological symptoms, showed hypoperfusion of the cerebellar and frontal lobes on single photon emission while CT and MRI imaging of the brain were normal. We add to the literature another 'cerebellar' presentation of Legionnaire's disease.

6.
Revista Científica de la Sociedad Española de Enfermería Neurológica ; 2022.
Article in English | ScienceDirect | ID: covidwho-1926898

ABSTRACT

The new coronavirus disease 2019 (COVID-19) outbreak has rapidly spread throughout the world, becoming a serious public health problem. COVID-19, in addition to respiratory symptoms, has neurotropic characteristics that can impact the nervous system. We present a 22-year-old female who developed diplopia and numbness of her limbs eight days after affecting by COVID-19 and she showed a left-sided outward gaze palsy without any history of previous a specific illness or medication. Supratentorial, infratentorial, and cervical cord enhancing plaques confirmed an acute demyelinating process in favor of Acute disseminated encephalomyelitis (ADEM). Her oligoclonal bands (OCB) were positive as well. As a result, a provisional diagnosis of ADEM was made. Her condition improved dramatically after starting corticosteroids and rituximab. Resumen El nuevo brote de coronavirus de 2019 (COVID-19) se ha propagado rápidamente a nivel mundial, convirtiéndose en un serio problema de salud pública. La COVID-19, además de síntomas respiratorios, tiene características neurotrópicas que pueden repercutir en el sistema nervioso. Presentamos el caso de una mujer de 22 años, que desarrolló diplopía y entumecimiento de las extremidades inferiores ocho días después de verse afectada por la COVID-19, mostrando parálisis de la mirada excéntrica en el lado izquierdo sin antecedentes de enfermedades o medicaciones específicas previas. La intensidad de las placas supratentoriales, infratentoriales y de la columna cervical confirmó un proceso desmielinizante agudo que favorecía encefalomielitis diseminada aguda (ADEM). Asimismo, sus bandas oligoclonales (OCB) fueron positivas. En consecuencia, se diagnosticó provisionalmente ADEM. Su situación mejoró considerablemente tras iniciar tratamiento con corticosteroides y rituximab.

7.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925550

ABSTRACT

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

8.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925485

ABSTRACT

Objective: To explore various post covid immune mediatedneurological manifestations in children Background: The neurological manifestation following a SARS-CoV2 infection is varied and till now there are only few studies reported regarding the same. Our study aimed to identify the varied spectrum of neurological manifestation following SARS-CoV2 infection. Design/Methods: Retrospective data were collected from May 2021 to September 2021, including all children aged from 1month to 18 years of age who presented to our pediatric emergency or OPD (a tertiary care center from western Rajasthan, India) with the neurological manifestation with history of COVID-19 infection or exposure and positive SARS-CoV-2 serology. Those who are RT PCR positive were excluded. The neurological manifestations were further categorized in a pre-designed proforma. Results: Case records of the 18 children who fulfilled the criteria were included in the study, among them 7 (38.8%) were male and 11 (61.1%) were female. Predominant presentation in our study group was seizures (6/18) and Gullian Barre Syndrome (5/18). Other manifestations included stroke (2/18), ADEM (1/18), MS (1/18), LETM (1/18), Autoimmune encephalitis[NMDAR](2/18). In our study group, 13/18 (72.2%) required immunomodulatory therapy either IVIG or high dose methylprednisolone pulse therapy. Steroids were used upfront in patients with elevated inflammatory markers. Cerebrovascular complications in children were less common compared to adults. Most of the children had favourable outcomes except for one mortality in our cohort. Conclusions: Delayed complications following SARS-CoV2 infection with varied manifestations are seen in children. A temporal correlation between the COVID 19 infection and the increasing number of neurological cases after the second wave was noted. Steroids are beneficial while treating such patients. Testing for SARS-CoV2 serology during the pandemic can give a clue to the underlying etiology. However, further studies are required to understand the CNS effects of SARS-CoV2 infection in children.

9.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925405

ABSTRACT

Objective: Vaccination remains core-stone of protection against viral infections like COVID-19. Various side effects of vaccines have been reported including anaphylaxis;myocarditis, pericarditis;however Neurological involvement is rare. We are reporting a case of optic neuropathy post COVID vaccination. The exact mechanism remains unclear. Case: A 42-year-old male, post Pfizer vaccine;presented with 3-day history of acute onset of painful blurry vision in left eye. Neurological exam revealed decreased visual acuity, afferent pupillary defect in left eye. He was treated with high dose steroids for 3 days followed by oral steroid taper. Follow up visit showed significant improvement in his vision. Design/Methods: Information regarding patient obtained via retrospective review of electronic medical records Results: MRI orbits showed T2 abnormal hyperintensity and enhancement of intracanalicular portion of left optic nerve;thus, diagnosed as retro-orbital optic neuritis. Lumbar puncture showed normal glucose and protein levels;MOG and NMO/AQP4 test were negative. Conclusions: Vaccine administration has been associated with a wide variety of inflammatory mediated CNS demyelinating syndromes that usually appear few days following the immunization such as optic neuritis with encephalomyelitis such as MOGAD, ADEM, and NMOSD. Post-SARS-CoV-2 vaccination Optic Neuritis (ON) is an extremely rare adverse event with the incidence of post-vaccination ON being much lower than for ON alone. Most cases were reported post AstraZeneca vaccine, Pfizer-BioNTech and Sinovac. Autoantibodies against MOG were mostly detected. The condition is treated spontaneously and both steroid responsive and plasma exchange. The underlying mechanism in post vaccination ON is systemic autoimmune process in contrast to regular ON cases, which are predominantly Multiple Sclerosis linked. Majority of patients had no prior history of neuro-inflammation and the risk is higher with increasing age, women and with a history of autoimmune disease.

10.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925270

ABSTRACT

Objective: To report a case of a patient who developed acute disseminated encephalomyelitis in the setting of recent COVID-19 vaccination. Background: NA Design/Methods: Data was obtained through the medical records system of Westchester Medical Center in Valhalla, New York. Results: We report on a 53-year-old female patient with a past medical history of obstructive sleep apnea who presented in the emergency room (ER) with left-sided numbness and weakness, ataxia, vertigo, and slurred speech in the context of a three month long history of headaches that started one day after she received her first dose of COVID-19 vaccine. Brain imaging studies showed findings most suggestive of acute disseminated encephalomyelitis (ADEM). Initial pretreatment cerebrospinal fluid (CSF) analysis showed markedly elevated protein with normal glucose level. Extensive workup was negative for infectious, oncologic, autoimmune, and psychiatric etiologies. Over the course of hospitalization, the patient was found to have isolated elevated liver function tests (LFTs) with transaminitis and hepatomegaly on ultrasound. The initial treatment of a five-day methylprednisolone course did not result in significant clinical improvement. The patient was then started on plasma exchange therapy (PLEX) and successfully recovered by the end of the last session. Conclusions: Our case report exemplifies a rare but severe neurologic condition observed in the setting of recent COVID-19 vaccination in a previously healthy woman.

11.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925234

ABSTRACT

Objective: NA Background: Acute disseminated encephalomyelitis (ADEM) is an inflammatory disease of the central nervous system thought to be caused by environmental factors to genetic susceptible individuals, where there is autoimmunity towards myelin components. Given the wide variety of symptoms, etiology and mimickers, ADEM is a diagnosis of exclusion, making difficult a prompt diagnosis. The pathogenesis associated between COVID-19 and ADEM is unknown, however, it could be secondary to immune-mediated mechanisms or molecular mimicry, creating a neuro-inflammatory response. Design/Methods: A previously healthy 22-year-old Puerto Rican male presented to the ED with altered mental status, incoherent speech, imbalance, and dizziness of 2 months progression. Neurological examination was remarkable for slow mentation, positive right Hoffman, left leg weakness and bilateral sustained clonus. Brain MRI showed innumerable foci of increased T2/FLAIR signal intensity throughout supra and infratentorial gray and white matter, none showing contrast enhancement. CSF with evidence of high protein levels, WBC of 7 (100% mononuclear) and normal glucose levels. Extensive workup for evaluation of infectious, demyelinating, inflammatory and vascular etiologies came back negative. Patient with recent history of gastrointestinal symptoms, reason why COVID-19 IgG/IgM Rapid Test was performed with positive IgG results 2 months prior to our evaluation. Patient was treated with intravenous steroids and intravenous immunoglobulin, with marked clinical improvement. Results: NA Conclusions: Here we present a case of atypical coronavirus disease 2019 (COVID-19) manifestation of a Puerto Rican male patient that 2 weeks after detecting SARS-COV-2 on his blood neurological symptoms started to develop. This is the first case of a young Puerto Rican patient without any other comorbidities where an association of COVID-19 infection and ADEM was found. Since ADEM if a diagnosis exclusion, it is vital to being able to distinguish this syndrome when it comes to a patient with history of COVID-19 infection and vaccine for prompt management.

12.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925157

ABSTRACT

Objective: To present a case of a longitudinal myelitis (LM) in the setting of SARS-CoV-2 infection. Background: While there is an established link between viral infections and new-onset demyelinating diseases [1, 2], data on the association between SARS-CoV-2 and myelitidies is lacking. Design/Methods: Consent obtained from patient. Results: A 53-year-old man presented with epigastric pain and urinary retention following ten days of fever, chills, headache, and cough. On presentation, he tested positive for SARS-CoV-2, prompting admission, then subsequently developed bilateral lower extremity weakness and numbness. MRI revealed T2 cord hyperintensity from C7 caudally to the conus. CSF revealed elevated total protein (67), WBC (16), IgG (11.1), and myelin-based protein (9). Testing for other demyelinating, infectious, and inflammatory conditions was negative. He had modest improvement in sensation following intravenous methylprednisolone and plasma exchange although remains paraplegic. Conclusions: This unique case of a longitudinal myelitis in the setting of SARS-CoV-2 infection adds to previously reported cases of acute disseminated encephalomyelitis, meningoencephalitis, and optic neuritis as neurological manifestations of SARS-CoV-2 infections [2].

13.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925151

ABSTRACT

Objective: 5-year-old boy with refractory acute disseminated encephalomyelitis (ADEM) found to have CNS-isolated Hemophagocytic Lymphohistiocytosis (HLH) with PRF1 mutation, c.4422G>A variant, not previously known to be pathogenic. Background: HLH is a hyperinflammatory condition that presents with fever, hepatosplenomegaly and characteristic laboratory findings. HLH can be familial (fHLH) or secondary (sHLH). Mutations in the perforin gene PRF1 have been identified as pathogenic for fHLH and they can also cause isolated CNS-HLH in the absence of systemic HLH. HLH can be triggered by malignancy, infections, or autoimmunity. Viruses are a common trigger and there have been cases of SARS-CoV-2 triggering HLH. Design/Methods: Clinical course, evaluation, management. Results: A 5-year-old boy presented to the emergency department with 3 weeks of headaches, right blurry vision, and emesis. He underwent an extensive evaluation and was diagnosed with ADEM that was thought to be triggered by SARS-CoV-2 after a positive nasal swab. Myelin oligodendrocyte glycoprotein (MOG) antibodies were negative. Patient completed a 5-day course of IV pulse steroids and plasma exchange. In the subsequent months, he was admitted twice due to worsening neuroinflammation and after several courses of IV pulse steroids, PLEX, and IVIG, the CNS inflammation stabilized with rituximab and monthly IVIG. A few months later, his younger brother presented to the emergency department with a similar syndrome. It was found that his parents were cousins, leading to concern for a genetic disorder. Genetic testing revealed a homozygous mutation for PRF1 in both siblings (variant c.4422G>A). Conclusions: This is the first presentation of CNS-isolated HLH triggered by SARS-CoV-2 in the pediatric population. Furthermore, thisis the first report of PRF1 mutation, the variant c.4422G>A, shown to be pathogenic. It highlights the relevance of genetics in pediatric neuroinflammatory disorders. It is possible that as our knowledge in neurogenetics develops, certain genes will be identified as predisposing factors to presentations such as ADEM.

14.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925140

ABSTRACT

Objective: To characterize the incidence and spectrum of neurological adverse events (AE) after COVID-19 vaccination. Background: The devastating COVID-19 pandemic has led to 230 million people diagnosed and greater than 4.8 million deaths worldwide. Widespread vaccination efforts have resulted in administration of over 6 million vaccine doses to curb the significant health and socioeconomic impacts of the disease. While there are numerous reports of adverse events following COVID-19 vaccine, there is limited characterization of the spectrum of neurological AEs post-vaccination. Design/Methods: Data was gathered from the publicly available Vaccine Adverse Event Reporting System (VAERS), a passive reporting system not implying causality. Among individuals who received the J&J, Moderna, and Pfizer vaccines from 1/1/2021-6/14/2021, 314,610 adverse events were reported and these were reviewed by Neurology trained clinicians to determine the presence of various neurological AEs (40 conditions coded). Results: 306,473,169 COVID vaccine dose were administered in the USA during the study period with 314,610 total AEs (0.10%) and 105,930 neurological AEs (0.03%) reported. J&J vaccine was associated with the most AEs (17,670, 0.15%), followed by Moderna (42656, 0.03%) and Pfizer (42267, 0.03%). On average more events were reported in women (71%) and a majority occurred after the first dose (54%). < 1 events were reported per million vaccine doses for serious neurological conditions such as Bell's palsy (0.0007%), Guillain-Barre syndrome (0.00009%), cerebral venous thrombosis (0.00005%), transverse myelitis (0.00003%), and acute disseminated encephalomyelitis (0.00006%). Overall neurological complications following vaccine were drastically lower than complications post-COVID infection (14-80%). Conclusions: Adverse neurological events following COVID-19 vaccination are extremely rare and significantly less common than adverse neurological effects following SARS-CoV-2 infection. Current evidence suggests that along with being up to 100,000 times more likely to experience a major complication from COVID infection vs. vaccine, the risk of neurological complication is up to 5000 times more likely from infection itself.

15.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925126

ABSTRACT

Objective: To present a single-health system retrospective analysis of post-mRNA-based COVID-19 vaccination CNS autoimmunity conducted in the greater New York City area. Background: There have been rare reports associating mRNA-based COVID-19 vaccines with central nervous system (CNS) inflammation. We report a case series of five patients with newonset neurological disorders of immunological origin temporally associated with these vaccines. Design/Methods: Case-series. Results: Five cases of post-vaccination CNS disorders of immune origin were observed within two weeks of inoculation with either the first or second dose of mRNA-based COVID-19 vaccines (Moderna = 3, Pfizer = 2). This includes: Fatal ADEM (n = 1), new-onset NMO (n = 2), new-onset fulminant MS (n = 1), and meningoencephalitis (n = 1). The age of our patients ranged from 27 to 81, and three were female. None of the patients had pre-existing neurological illnesses and one had a pre-existing autoimmune condition (immune thrombocytopenia purpura). New-onset focal neurological symptoms were present in all five patients, including quadriparesis, numbness, diplopia, and encephalopathy. CSF pleocytosis was present in all patients, and three had elevated protein. All but one patient (meningoencephalitis) had contrastenhancing lesions involving either the cerebrum or spinal cord. Both NMO patients had longitudinally extensive transverse lesions involving the central thoracic cord. Aquaporin-4 serum antibody was present in one NMO patients and aquaporin-4 CSF antibody present in the other. All but one patient (fatal ADEM) clinically improved with pulse steroids or plasmapheresis. Conclusions: These are among the emerging cases of CNS immunological events temporally associated with mRNA-based COVID-19 vaccines. These findings should be interpreted with great caution as they neither prove a link nor imply a potential long-term increased risk in postvaccination CNS autoimmunity. Larger prospective studies are needed. The mRNA-based SARS-CoV-2 vaccines should continue to be strongly encouraged given their high efficacy in overcoming this pandemic.

16.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925124

ABSTRACT

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.

17.
Cureus ; 14(5): e24961, 2022 May.
Article in English | MEDLINE | ID: covidwho-1924630

ABSTRACT

Acute disseminated encephalomyelitis (ADEM) is a rare illness. Generally characterized by encephalopathy and non-specific, heterogeneous neurological deficits depending on the location of the demyelinated lesions, ADEM is considered a clinical diagnosis with radiological findings that may or may not have supportive features based on the temporal relationship of an inciting factor and symptom onset. Even rarer, hyperacute or malignant ADEM can be defined by rapid symptom onset followed by catastrophic brain edema and its sequelae. We present a case of a patient who presented with an acute stroke with activation of a rapid sequence care pathway (stroke alert protocol) to mobilize resources that could expedite his care to determine eligibility for thrombolysis. ADEM was the definitive diagnosis with a subsequent rapid and treatment-refractory decline.

18.
Curr Neurol Neurosci Rep ; 22(8): 499-513, 2022 Aug.
Article in English | MEDLINE | ID: covidwho-1889030

ABSTRACT

PURPOSE OF REVIEW: To discuss the neurological complications and pathophysiology of organ damage following malaria infection. RECENT FINDINGS: The principal advancement made in malaria research has been a better understanding of the pathogenesis of cerebral malaria (CM), the most dreaded neurological complication generally caused by Plasmodium falciparum infection. However, no definitive treatment has yet been evolved other than the use of antimalarial drugs and supportive care. The development of severe cerebral edema in CM results from two distinct pathophysiologic mechanisms. First, the development of "sticky" red blood cells (RBCs) leads to cytoadherence, where red blood cells (RBCs) get stuck to the endothelial walls and between themselves, resulting in clogging of the brain microvasculature with resultant hypoxemia and cerebral edema. In addition, the P. falciparum-infected erythrocyte membrane protein 1 (PfEMP1) molecules protrude from the raised knob structures on the RBCs walls and are in themselves made of a combination of human and parasite proteins in a tight complex. Antibodies to surfins, rifins, and stevors from the parasite are also located in the RBC membrane. On the human microvascular side, a range of molecules involved in host-parasite interactions, including CD36 and intracellular adhesion molecule 1, is activated during interaction with other molecules such as endothelial protein C receptor and thrombospondin. As a result, an inflammatory response occurs with the dysregulated release of cytokines (TNF, interleukins 1 and 10) which damage the blood-brain barrier (BBB), causing plasma leakage and brain edema. This second mechanism of CNS injury often involves multiple organs in adult patients in endemic areas but remains localized only to the central nervous system (CNS) among African children. Neurological sequelae may follow both P. falciparum and P. vivax infections. The major brain pathology of CM is brain edema with diffuse brain swelling resulting from the combined effects of reduced perfusion and hypoxemia of cerebral neurons due to blockage of the microvasculature by parasitized RBCs as well as the neurotoxic effect of released cytokines from a hyper-acute immune host reaction. A plethora of additional neurological manifestations have been associated with malaria, including posterior reversible encephalopathy syndrome (PRES), reversible cerebral vasoconstriction syndrome (RCVS), malarial retinopathy, post-malarial neurological syndrome (PMNS), acute disseminated encephalomyelitis (ADEM), Guillain-Barré syndrome (GBS), and cerebellar ataxia. Lastly, the impact of the COVID-19 pandemic on worldwide malaria control programs and the possible threat from co-infections is briefly discussed.


Subject(s)
Brain Edema , COVID-19 , Malaria, Cerebral , Malaria, Falciparum , Posterior Leukoencephalopathy Syndrome , Adult , Child , Cytokines , Humans , Hypoxia , Malaria, Cerebral/complications , Malaria, Cerebral/parasitology , Malaria, Falciparum/complications , Malaria, Falciparum/parasitology , Pandemics , Plasmodium falciparum/physiology
19.
J Cent Nerv Syst Dis ; 14: 11795735221102747, 2022.
Article in English | MEDLINE | ID: covidwho-1868969

ABSTRACT

As the world embarks on mass vaccination against SARS-CoV2 to alleviate the spread of this highly contagious novel coronavirus, there are growing anecdotal reports on immune-related neurological complications following immunisation. Similarly, we encountered 2 cases of central nervous system demyelination at our centre with Comirnaty (BNT162b2), a mRNA-based COVID-19 vaccine. Our first patient had typical clinical-radiological manifestations of acute disseminated encephalomyelitis (ADEM) after his COVID-19 vaccination. This was the sixth reported case to date. Our second patient presented with an unusual complaint of trigeminal neuralgia, with an identifiable demyelinating lesion observed in the pons on neuroimaging. Both cases responded well to immunotherapy. However, larger prospective controlled studies and formal registries are much needed to ascertain a possible relationship between COVID-19 vaccines and acute central nervous system demyelination.

20.
Egyptian Journal of Radiology and Nuclear Medicine ; 53(1), 2022.
Article in English | EMBASE | ID: covidwho-1869114

ABSTRACT

Background: Coronavirus disease COVID-19 is a viral illness, currently affecting millions of people worldwide. Pneumonia is the most common extreme presentation of COVID-19 infection, manifesting by fever, dry cough, difficulty of breathing or shortness of breath and mainly ground-glass infiltrates in radiological images. Chest computed tomography (CT) has a potential role in the diagnosis, detection of complications and prognostication of coronavirus disease COVID-19. In addition to severe respiratory manifestations, there are a wide range of neurological manifestations ranging from nonspecific symptoms to necrotizing encephalopathies and stroke. Our study aimed to review lung and neurological manifestations in recent and post-COVID-19 Egyptian patients and to be familiar with magnetic resonance imaging (MRI) findings of Neuro-COVID patients. Results: The present study included eighty COVID-19 patients with age ranged from 28 to 78 years (mean age 57.84 + 12.58 years) who were 54 males (mean age 56.64 + 12.50) and 26 females (mean age 48.65 + 14.24). All our patients were with recent or previous history of COVID-19 infection and subjected to careful history taking, thorough clinical examination, routine laboratory investigations and CT examination. The reported lung manifestations included normal lung shadows, ground-glass opacifications (GGOs), consolidations, reticulation, reticulation and GGOs (crazy paving) and fibrotic-like changes. Out of eighty COVID-19 patients, twenty showed neurological manifestations ranging from nonspecific symptoms to necrotizing encephalopathies and stroke. Patients with neurological manifestation were in addition to CT submitted to magnetic resonance imaging (MRI) as appropriate. MRI done to neuro-COVID patients showed that 8/20 (40%) had no abnormalities and 12/20 (60%) had abnormalities. The most common abnormalities are infarction, major or lacunar infarction, followed by acute disseminated encephalomyelitis (ADEM), posterior reversible encephalopathy syndrome (PRES) and meningoencephalitis. Conclusion: Old age patients, especially males, were more affected than females. Lung manifestations are common in COVID-19 patients than neurological manifestations. The presence of fibrotic changes in the lung could predict severe COVID-19 affection and bad prognosis. There might be an association between appearance of neurological manifestations and poor outcome in COVID-19 patients.

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