A Comprehensive Review of Neurologic Manifestations of COVID-19 and Management of Pre-existing Neurologic Disorders in Children

Since the first reports of SARS-CoV-2 infection from China, multiple studies have been published regarding the epidemiologic aspects of COVID-19 including clinical manifestations and outcomes. The majority of these studies have focused on respiratory complications. However, recent findings have highlighted the systemic effects of the virus, including its potential impact on the nervous system. Similar to SARS-CoV-1, cellular entry of SARS-CoV-2 depends on the expression of ACE2, a receptor that is abundantly expressed in the nervous system. Neurologic manifestations in adults include cerebrovascular insults, encephalitis or encephalopathy, and neuromuscular disorders. However, the presence of these neurologic findings in the pediatric population is unclear. In this review, the potential neurotropism of SARS-CoV-2, known neurologic manifestations of COVID-19 in children, and management of preexisting pediatric neurologic conditions during the COVID-19 pandemic are discussed.

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Towards Central Nervous System Involvement in Adults with Hereditary Myopathies

Journal of Neuromuscular Diseases ◽

10.3233/jnd-200507 ◽

2020 ◽

Vol 7 (4) ◽

pp. 367-393

Author(s):

Jens Reimann ◽

Cornelia Kornblum

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Neuromuscular Disorders ◽

Clinical Manifestations ◽

Specific Treatment ◽

Central Nervous System Involvement ◽

Epileptic Seizures ◽

System Involvement ◽

History Of ◽

Neuroimaging Techniques

There is increasing evidence of central nervous system involvement in numerous neuromuscular disorders primarily considered diseases of skeletal muscle. Our knowledge on cerebral affection in myopathies is expanding continuously due to a better understanding of the genetic background and underlying pathophysiological mechanisms. Intriguingly, there is a remarkable overlap of brain pathology in muscular diseases with pathomechanisms involved in neurodegenerative or neurodevelopmental disorders. A rapid progress in advanced neuroimaging techniques results in further detailed insight into structural and functional cerebral abnormalities. The spectrum of clinical manifestations is broad and includes movement disorders, neurovascular complications, paroxysmal neurological symptoms like migraine and epileptic seizures, but also behavioural abnormalities and cognitive dysfunction. Cerebral involvement implies a high socio-economic and personal burden in adult patients sometimes exceeding the everyday challenges associated with muscle weakness. It is especially important to clarify the nature and natural history of brain affection against the background of upcoming specific treatment regimen in hereditary myopathies that should address the brain as a secondary target. This review aims to highlight the character and extent of central nervous system involvement in patients with hereditary myopathies manifesting in adulthood, however also includes some childhood-onset diseases with brain abnormalities that transfer into adult neurological care.

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Sedation and Analgesia in Children with Developmental Disabilities and Neurologic Disorders

International Journal of Pediatrics ◽

10.1155/2010/189142 ◽

2010 ◽

Vol 2010 ◽

pp. 1-9 ◽

Cited By ~ 6

Author(s):

Todd J. Kilbaugh ◽

Stuart H. Friess ◽

Ramesh Raghupathi ◽

Jimmy W. Huh

Keyword(s):

Traumatic Brain Injury ◽

Central Nervous System ◽

Nervous System ◽

Developmental Disabilities ◽

Obstructive Hydrocephalus ◽

Respiratory Complications ◽

Neurologic Disorders ◽

Sedation And Analgesia ◽

Ischemic Encephalopathy ◽

Children With Developmental Disabilities

Sedation and analgesia performed by the pediatrician and pediatric subspecialists are becoming increasingly common for diagnostic and therapeutic purposes in children with developmental disabilities and neurologic disorders (autism, epilepsy, stroke, obstructive hydrocephalus, traumatic brain injury, intracranial hemorrhage, and hypoxic-ischemic encephalopathy). The overall objectives of this paper are (1) to provide an overview on recent studies that highlight theincreasedrisk for respiratory complications following sedation and analgesia in children with developmental disabilities and neurologic disorders, (2) to provide a better understanding of sedatives and analgesic medications which are commonly used in children with developmental disabilities and neurologic disorders on thecentral nervous system.

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Neurology of Infancy

PEDIATRICS ◽

10.1542/peds.25.3.489 ◽

1960 ◽

Vol 25 (3) ◽

pp. 489-489

Author(s):

JAMES F. HAMMILL

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Clinical Manifestations ◽

Illustrated Text ◽

Degenerative Disorders ◽

Neurologic Disorders ◽

The Central Nervous System ◽

Mature Patient

Dr. Dekaban states that his purpose in writing this excellently printed and illustrated text is to provide those concerned with anatomic and developmental landmarks of the maturation of the central nervous system during the first 2 years of life, and to outline the principles and clinical manifestations of neurologic disorders occurring during infancy, stressing the differences between the mature patient and infants in these regards. The text fulfills the author's aims in scope encompassing the neurologic spectrum from metabolic to neoplastic and degenerative disorders.

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361. Aseptic Meningitis Associated to SARS Cov2 Infection and MIS-C: Pediatric Presentation of Covid-19

Open Forum Infectious Diseases ◽

10.1093/ofid/ofab466.562 ◽

2021 ◽

Vol 8 (Supplement_1) ◽

pp. S283-S284

Author(s):

Mónica J Olguín Quintero ◽

Sergio RenÉ Bonilla Pellegrini ◽

Rodolfo N JimÉnez JuÁrez ◽

María Citlalli Casillas Casillas

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Viral Load ◽

Aseptic Meningitis ◽

Pediatric Population ◽

Clinical Manifestations ◽

Maculopapular Rash ◽

Oral Steroid ◽

Laboratory Findings ◽

Pediatric Presentation

Abstract Background Novel SARS CoV2 may target the central nervous system and several neurological symptoms have been reported in patients with Coronavirus disease (COVID-19). Mucocutaneous and inflammatory symptoms are important in pediatric population associated to immune dysregulation. There are few reports of clinical manifestations in children and less frequently the isolation and affection of Central Nervous System. Methods A previously healthy four months female infant with familiar contact to SARS-CoV2 four weeks ago. Start with fever of 104°F, vomiting, maculopapular rash on the anterior thorax and upper extremities involving the palms and soles associated with edema. On physical examination, irritable, bulging anterior fontanelle, non-purulent bilateral conjunctival injection, cheilitis and rash was confirmed. Results Laboratory findings: thrombocytopenia, elevated D-Dimer, fibrinogen, PCT, CRP, ferritin and ESR with hypoalbuminemia. MIS-C is integrated with cutaneous, gastrointestinal and neurological affections. Empirically ceftriaxone, vancomycin and acyclovir are started due to suspicion of meningoencephalitis. RT-PCR for SARS-CoV-2 positive. CSF: transparent appearance, slightly xanthochromic color, coagulation and negative film. Proteins 105 mg / dl, glucose 45 mg / dl, leukocytes 121 mm3, erythrocytes 66 mm3, PMN 8% and MNN 92%. Negative culture,PCR Herpes Virus negative,Viral load for SARS CoV2 in CSF 3,400 cop / ml and plasma 118,900 cop / ml, Aseptic meningitis is confirmed by SARS-CoV-2. Antiviral and antibiotics are discontinued and Gamma globulin and methylprednisolone are administered. Evolving favorably and egress at 6th day to complete oral steroid treatment for 3 more day. Conclusion The mechanism by which SARS-CoV2 affects the CNS is still unknown.This findings suggests direct infection can be possible. Although it is also described vascular affection has been found that the Spike protein of the virus binds to ACE-2 receptor present in the cerebral vascular endothelium. Neurological manifestations have been described even without respiratory symptoms. A novel pediatric case with viral load for SARS-CoV-2 in CSF is demonstrated. Importance of detecting SARS-CoV-2 in children with encephalitis, which can progress satisfactorily. Disclosures All Authors: No reported disclosures

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Lesions of the nervous system associated with relapsing polychondritis

Clinical Medicine (Russian Journal) ◽

10.18821/0023-2149-2016-94-2-108-113 ◽

2016 ◽

Vol 94 (2) ◽

pp. 108-113

Author(s):

Vera I. Chernyak ◽

A. I. Savel’ev ◽

I. V. Men’shikova ◽

A. P. Pogromov

Keyword(s):

Nervous System ◽

Clinical Manifestations ◽

Relapsing Polychondritis ◽

Cranial Neuropathy ◽

Nerve Lesion ◽

Neurologic Manifestations ◽

The Third ◽

Optic Nerve Lesion ◽

High Doses ◽

Lymphocytic Meningitis

Three clinical cases are described including two of relapsing polychondritis with lesions in the central and peripheral nervous system (one of long-standing aseptic lymphocytic meningitis and one of cranial neuropathy of 2, 5, 7, and 8 pairs) and the third case of the optic nerve lesion with amblyopia. The two former cases were successfully treated with high doses of corticosteroids, the third one with moderate doses of the same medications. The data from the current literature concerning variants of clinical manifestations, methods for diagnostics and treatment of neurologic manifestations of relapsing polychondritis are discussed.

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Clinical syndromes associated with Coenzyme Q10 deficiency

Essays in Biochemistry ◽

10.1042/ebc20170107 ◽

2018 ◽

Vol 62 (3) ◽

pp. 377-398 ◽

Cited By ~ 29

Author(s):

María Alcázar-Fabra ◽

Eva Trevisson ◽

Gloria Brea-Calvo

Keyword(s):

Skeletal Muscle ◽

Nervous System ◽

Biosynthetic Pathway ◽

Coenzyme Q ◽

Clinical Manifestations ◽

Comprehensive Review ◽

Pathogenic Variants ◽

Clinical Syndromes ◽

Number Of Patients ◽

Clinical Pictures

Primary Coenzyme Q deficiencies represent a group of rare conditions caused by mutations in one of the genes required in its biosynthetic pathway at the enzymatic or regulatory level. The associated clinical manifestations are highly heterogeneous and mainly affect central and peripheral nervous system, kidney, skeletal muscle and heart. Genotype–phenotype correlations are difficult to establish, mainly because of the reduced number of patients and the large variety of symptoms. In addition, mutations in the same COQ gene can cause different clinical pictures. Here, we present an updated and comprehensive review of the clinical manifestations associated with each of the pathogenic variants causing primary CoQ deficiencies.

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DIAGNOSTIC VALUE OF SPECIFIC ANTIBODY SYNTHESIS IN BRAIN OF PATIENTS WITH NEUROINFECTIONS

Wiadomości Lekarskie ◽

10.36740/wlek201908103 ◽

2019 ◽

Vol 72 (8) ◽

pp. 1437-1441

Author(s):

Pavel Dyachenko ◽

Igor Filchakov ◽

Anatoly Dyachenko ◽

Victoria Kurhanskaya

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Clinical Manifestations ◽

Diagnostic Value ◽

Diagnostic Challenge ◽

Specific Antibodies ◽

Intrathecal Synthesis ◽

Varicella Zoster ◽

Mrz Reaction ◽

Wide Range

Introduction: Viral encephalitis accounts for 40-70% of all cases worldwide, central nervous system infections pose a diagnostic challenge because clinical manifestations are not typically pathognomonic for specific pathogens, and a wide range of agents can be causative. The aim: To assess the diagnostic value of intrathecal synthesis of specific antibodies in patients with inflammatory lesions of the central nervous system. Materials and methods: Within the framework of the study, two groups of 90 people in each were formed from the patients with neuroinfections admitted to our Center. Intrathecal synthesis (ITS) of total (unspecific) IgG in members of one of group (group of compare) was determined. Brain synthesis of specific antibodies (Ab) to some neurotropic pathogens (herpes simplex virus 1/2, cytomegalovirus, Epstein-Barr virus, varicella zoster virus, rubella virus, Borrelies) was studied in the second group of patients (group of interest). There were no statistically significant differences between groups by gender and age. Encephalitis and encephalomyelitis prevailed among patients of both groups Results: ITS of total IgG was established in 30 (33.3 ± 6.1 %) patients of the first group with IgG index more than 0.6 indicating on inflammatory process in CNS and no marked changes of CSF. ITS of specific Ab was determined in 23 of 90 (25.6 ± 4.6 %) patients included into group of interest. In more than half of cases Ab to several infectious agents were detected simultaneously. ITS of various specificity, in particular, to measles and rubella viruses, and VZV, known as MRZ-reaction, is characteristic of some autoimmune lesions of CNS, multiple sclerosis first of all. In fact, further research of 5 patients with MRZ-reaction confirmed their autoimmune failure of CNS. Detection of ITS in the CSF samples didn’t depend on concentration of specific Ab in serum and CSF and wasn’t followed by HEB dysfunctions which were observed with the same frequency in patients with or without ITS (13.0 % and 13.6 % respectively). Conclusion: Specific Ab synthesis to several neurotropic pathogens in the CSF of significant part of examined patients was established. Thus, diagnostic value of ITS of specific immunoglobulins seems to be limited to cases in which autoimmune damage of the CNS is suspected.

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1707. Clinical Profile of Human T-Lymphotropic Virus Type I Infection in Pediatric Population in a Referral Hospital in Colombia

Open Forum Infectious Diseases ◽

10.1093/ofid/ofaa439.1885 ◽

2020 ◽

Vol 7 (Supplement_1) ◽

pp. S836-S837

Author(s):

Juliana Mejia- Mertel ◽

Juan P Rojas -Hernandez

Keyword(s):

Virus Type ◽

Low Income ◽

Pacific Coast ◽

Vision Loss ◽

Pediatric Population ◽

Clinical Manifestations ◽

Low Income Countries ◽

Nutritional Deficiencies ◽

Type I ◽

T Lymphotropic Virus

Abstract Background The Human T-lymphotropic virus type 1 (HTLV-1), affects around ten to twenty million people worldwide, predominantly in intertropical regions (Africa, Japan, Melanesia, Australia, and South America Pacific Coast). The most common disorders associated are T-cell leukemia/lymphoma (ALT) and HTLV-1-associated myelopathy (HAM). Studies have reported other clinical manifestations in HTLV-1, still studies are needed in pediatric population to improve diagnosis and treatment of infected patients. Methods Descriptive, retrospective cohort study, conducted in our referral pediatric hospital in Cali, Colombia. Included pediatric patients (1 to 18 years of age) diagnosed with HTLV-1 infection, between January 2017 to March 2020. Results Twelve patients were included, seven males and five females. Eleven patients were from and resided in the Colombian Pacific coast. Ten patients showed nutritional deficiencies. None showed clinical or laboratory signs of ALT, neither neurological symptoms or physical exam suggesting HAM. In terms of associated diseases and opportunistic infections, none had a positive HIV ELISA test, and stool tests were all negative for Strongiloydes. Four presented infective dermatitis, and two showed lesions suggesting scabies. Eight patients presented respiratory symptoms with chest CT scans showing signs of chronic inflammation, bronchiectasis, and subpleural bullae as the major findings. Additional tests were carried out in bronchoalveolar fluid, four had positive galactomannan test,suggesting pulmonary aspergillosis, two exhibited positive gene PCR testing for Mycobacterium tuberculosis. Regarding inflammatory diseases, one patient presented with symptoms of Inflammatory Bowl Disease, with biopsy confirming Crohn’s disease. Another patient presente abrupt vision loss, diagnosed with Vogt Koyanagi Hadara Syndrome after ophthalmological evaluation. Summary features HTLV-1 patients Ground-glass opacity diffusely distributed in both lungs with multiple bronchiectasis involving predominantly lung bases. Cystic images diffusely distributed in both lungs, some subpleural and other centrilobular. Conclusion It is important to consider alternative manifestations of HTLV-1 infection in the pediatric population, including pulmonary disease, opportunistic co-infections, and inflammatory disorders. It is crucial to diagnose this disease in childhood to reach a better control of this neglected infection that affects predominantly vulnerable population in low-income countries. Disclosures All Authors: No reported disclosures

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Bladder and bowel responses to lumbosacral epidural stimulation in uninjured and transected anesthetized rats

Scientific Reports ◽

10.1038/s41598-021-81822-3 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Robert F. Hoey ◽

Daniel Medina-Aguiñaga ◽

Fahmi Khalifa ◽

Beatrice Ugiliweneza ◽

Sharon Zdunowski ◽

...

Keyword(s):

Spinal Cord ◽

Nervous System ◽

Autonomic Nervous System ◽

Surface Area ◽

Electrode Array ◽

Frequency Dependent ◽

Epidural Stimulation ◽

Sphincter Electromyography ◽

Neurologic Disorders ◽

Movement Threshold

AbstractSpinal cord epidural stimulation (scES) mapping at L5-S1 was performed to identify parameters for bladder and bowel inhibition and/or contraction. Using spinally intact and chronic transected rats of both sexes in acute urethane-anesthetized terminal preparations, scES was systematically applied using a modified Specify 5–6–5 (Medtronic) electrode during bladder filling/emptying cycles while recording bladder and colorectal pressures and external urethral and anal sphincter electromyography activity. The results indicate frequency-dependent effects on void volume, micturition, bowel peristalsis, and sphincter activity just above visualized movement threshold intensities that differed depending upon neurological intactness, with some sex-dependent differences. Thereafter, a custom-designed miniature 15-electrode array designed for greater selectivity was tested and exhibited the same frequency-dependent urinary effects over a much smaller surface area without any concurrent movements. Thus, select activation of autonomic nervous system circuitries with scES is a promising neuromodulation approach for expedient translation to individuals with SCI and potentially other neurologic disorders.

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EPID-26. CLINICAL AND HISTOLOGICAL CHARACTERIZATION OF PRIMARY CENTRAL NERVOUS SYSTEM SARCOMA IN PEDIATRICS. A SINGLE INSTITUTION EXPERIENCE IN A MIDDLE-INCOME COUNTRY

Neuro-Oncology ◽

10.1093/neuonc/noaa215.344 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii84-ii84

Author(s):

Edgar Cabrera ◽

Nelson Aponte ◽

Johnny Garcia ◽

Fredy Salazar ◽

Eric Bouffet ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Pediatric Population ◽

Progression Free Survival ◽

Molecular Data ◽

Middle Income ◽

Patient Demographics ◽

Time Period ◽

Cell Architecture ◽

Intracranial Lesions

Abstract INTRODUCTION Primary central nervous system (CNS) sarcomas are rare mesenchymal non-meningothelial tumors accounting for less than 0.2% of intracranial lesions. Diagnosis and management are challenging due to the current lack of substantive clinical, histological and molecular data. METHODS We retrospectively identified all patients with diagnosis of primary CNS sarcoma at the Hospital Fundación Pediatrico la Misericordia (HOMI) in Bogota, Colombia. We collected patient demographics, disease characteristics, and outcomes for analysis. RESULTS Between 2008 and 2020, twenty-four consecutive patients were diagnosed at the HOMI representing 6% of all CNS tumors diagnosed over the same time period. The median age at presentation was 9.48 years (range:1.6–13.4). The median time of symptoms prior to diagnosis was 2 weeks (0.1–24). The most common presentation was headache (21/24- 89%) and vomiting (19/24- 79%). The frontal lobe was involved in 63% of patients (15/24) and only one patient presented with a cerebellar lesion. Histologically, these tumors were characterized by a pleomorphic spindle cell architecture and high mitotic activity. All samples lacked immunoreactivity to GFAP, CD34, EMA, and S100 and all samples had strong nuclear immunopositivity for TLE-1; BCL-2 was reactive in eighteen cases. Gross total resection was attained in fifteen patients, most patients received focal radiation therapy and ICE chemotherapy. Progression-free survival at 12 and 24 months was 57% and 31% respectively. Overall survival was 77% at 12 months and 39% at 24 months. Thirteen patients relapsed, 11 presented with local failures, and 2 with intracranial recurrences outside of the radiation field. CONCLUSION Our study identifies TLE-1 as a diagnostic marker of primary CNS sarcoma, a highly malignant supratentorial tumor of childhood. Further molecular studies are urgently needed to elucidate the biology of this disease and the unusually high incidence observed in the Colombian pediatric population.

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