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Clinical Pediatrics Jan 2024
Topics: Humans; Child; Ocular Motility Disorders; COVID-19
PubMed: 37991173
DOI: 10.1177/00099228231212770 -
Neurologia Mar 2023Opsoclonus-myoclonus-ataxia syndrome is a rare neuroinflammatory disorder with onset during childhood; aetiology may be paraneoplastic, para-infectious, or idiopathic....
INTRODUCTION
Opsoclonus-myoclonus-ataxia syndrome is a rare neuroinflammatory disorder with onset during childhood; aetiology may be paraneoplastic, para-infectious, or idiopathic. No biomarkers have yet been identified, and diagnosis is clinical. Better cognitive prognosis appears to be related to early onset of immunomodulatory therapy.
METHODS
We describe the epidemiological, clinical, therapeutic, and long-term prognostic characteristics of a cohort of 20 Spanish patients.
RESULTS
The mean age of onset was 21 months (range, 2-59). Ataxia and opsoclonus were the most frequent symptoms both at disease onset and throughout disease progression. The mean time from onset to diagnosis was 1.1 months. Neuroblast lineage tumours were detected in 45% of patients; these were treated with surgical resection in 7 cases and chemotherapy in 2. Cerebrospinal fluid analysis revealed pleocytosis in 4 cases (25%) and neither antineuronal antibodies nor oligoclonal bands were detected in any patient. Immunomodulatory drugs were used in all cases. Nine patients started combined immunomodulatory treatment at the time of diagnosis, and 5 patients after a mean of 2.2 months. In the long term, 6 of the 10 patients followed up for more than 5 years presented mild or moderate cognitive sequelae. Four patients presented relapses, generally coinciding with the decrease of corticosteroid doses.
CONCLUSIONS
Early initiation of immunotherapy, as well as triple combination therapy, where needed, was associated with a lower frequency of cognitive impairment 2 years after onset.
Topics: Humans; Child; Infant; Child, Preschool; Opsoclonus-Myoclonus Syndrome; Prognosis; Neoplasm Recurrence, Local; Disease Progression; Ataxia; Ocular Motility Disorders
PubMed: 36396095
DOI: 10.1016/j.nrleng.2020.04.030 -
Neurology Sep 2005
Topics: Brain Stem; Diagnosis, Differential; Humans; Ischemic Attack, Transient; Male; Middle Aged; Neural Inhibition; Neurologic Examination; Ocular Motility Disorders; Raphe Nuclei; Saccades; Video Recording; Volition
PubMed: 16157893
DOI: 10.1212/01.wnl.0000180351.66206.a6 -
The Journal of Pediatrics May 1995
Topics: Abdominal Neoplasms; Child, Preschool; Female; Head and Neck Neoplasms; Humans; Infant; Myoclonus; Neoplasm Recurrence, Local; Neoplasms, Second Primary; Neuroblastoma; Ocular Motility Disorders; Recurrence
PubMed: 7752022
DOI: 10.1016/s0022-3476(95)70436-1 -
The Journal of the Association of... Feb 1993Opsoclonus is a rare and dramatic ocular sign. A case of opsoclonus is reported here with an unusually located CNS lesion.
Opsoclonus is a rare and dramatic ocular sign. A case of opsoclonus is reported here with an unusually located CNS lesion.
Topics: Adult; Brain; Demyelinating Diseases; Eye Movements; Female; Humans; Magnetic Resonance Imaging; Ocular Motility Disorders
PubMed: 8335604
DOI: No ID Found -
Neurology May 2019
Topics: Dipeptidyl-Peptidases and Tripeptidyl-Peptidases; Encephalitis; Humans; Male; Middle Aged; Nerve Tissue Proteins; Ocular Motility Disorders; Potassium Channels
PubMed: 31061220
DOI: 10.1212/WNL.0000000000007463 -
Progress in Brain Research 2019Opsoclonus consists of bursts of involuntary, multidirectional, back-to-back saccades without an intersaccadic interval. We report a 60-year-old man with post-concussive...
Opsoclonus consists of bursts of involuntary, multidirectional, back-to-back saccades without an intersaccadic interval. We report a 60-year-old man with post-concussive headaches and disequilibrium who had small amplitude opsoclonus in left gaze, along with larger amplitude flutter during convergence. Examination was otherwise normal and brain MRI was unremarkable. Video-oculography demonstrated opsoclonus predominantly in left gaze and during pursuit in the left hemifield, which improved as post-concussive symptoms improved. Existing theories of opsoclonus mechanisms do not account for this eye position-dependence. We discuss theoretical mechanisms of this behavior, including possible dysfunction of frontal eye field and/or cerebellar vermis neurons; review ocular oscillations in traumatic brain injury; and consider the potential relationship between the larger amplitude flutter upon convergence and post-traumatic ocular oscillations.
Topics: Brain Concussion; Eye Movement Measurements; Fixation, Ocular; Humans; Male; Middle Aged; Ocular Motility Disorders; Saccades
PubMed: 31325998
DOI: 10.1016/bs.pbr.2019.04.016 -
Pediatrics International : Official... Jan 2020
Topics: Cataract; Cataract Extraction; Female; Humans; Infant; Mutation; Ocular Motility Disorders; Treatment Outcome; beta-Crystallin A Chain
PubMed: 31957102
DOI: 10.1111/ped.14039 -
Neuropediatrics Jun 1993Antibodies to ACTH1-24 detected by radioimmunoassay were present in the serum of a child with opsoclonus-myoclonus for at least 24 weeks after discontinuation of chronic... (Review)
Review
Antibodies to ACTH1-24 detected by radioimmunoassay were present in the serum of a child with opsoclonus-myoclonus for at least 24 weeks after discontinuation of chronic ACTH treatment. The antibody-bound ACTH did not interfere with cortisol secretion. Six other children with opsoclonus-myoclonus and 16 control sera, including patients with chronically elevated endogenous ACTH, did not exhibit autoantibodies to ACTH. Antibodies to ACTH should be sought in patients who develop tolerance to ACTH treatment. The indirect but not direct ACTH assay method is sensitive to the presence of ACTH antibodies.
Topics: Adolescent; Adrenocorticotropic Hormone; Antibodies, Monoclonal; Autoantibodies; Child; Child, Preschool; Drug Tolerance; Female; Humans; Immunoglobulin G; Male; Myoclonus; Ocular Motility Disorders; Radioimmunoassay
PubMed: 8395029
DOI: 10.1055/s-2008-1071529 -
Internal Medicine (Tokyo, Japan) Mar 2023A 72-year-old woman with opsoclonus visited our hospital and was diagnosed with small-cell lung cancer. Blood tests revealed anti-SOX1 antibodies, so the patient was...
A 72-year-old woman with opsoclonus visited our hospital and was diagnosed with small-cell lung cancer. Blood tests revealed anti-SOX1 antibodies, so the patient was diagnosed with paraneoplastic opsoclonus-myoclonus syndrome. After steroid pulse therapy was started, chemotherapy of treatment, the opsoclonus showed an improving trend. Anti-Ri and anti-Hu antibodies have been reported as autoantibodies associated with neoplastic opsoclonus-myoclonus syndrome; however, there are no such reports concerning anti-SOX1 antibody. Therefore, this is a valuable case.
Topics: Female; Humans; Aged; Opsoclonus-Myoclonus Syndrome; Small Cell Lung Carcinoma; Ocular Motility Disorders; Autoantibodies; Lung Neoplasms
PubMed: 35989271
DOI: 10.2169/internalmedicine.0168-22