Background And Objectives: Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged between 6 and 36 months. It is characterized by opsoclonus (rapid, irregular, horizontal and vertical eye movements) and myoclonus that may affect trunk, limbs or face, and cerebellar ataxia. It may be considered a paraneoplastic syndrome by association with neuroblastomas, hepatoblastomas and, rarely, ganglioneuromas. The aim of this paper was to present the most relevant aspects of Kinsbourne syndrome, as well as the technique used for resection of mediastinal tumor in a child with this syndrome.
Case Report: Child, 1 year and 5 months, with a diagnosis of posterior mediastinal tumor and Kinsbourne syndrome. Premedicated with oral midazolam. Anesthesia induced with sevoflurane, nitrous oxide, fentanyl, and rocuronium. Maintenance of anesthesia with sevoflurane, nitrous oxide, fentanyl, and rocuronium. Neuromuscular block reversal with neostigmine combined with atropine. Postoperative analgesia with the use of dipyrone, morphine, and ketoprofen. Taken to the intensive care unit extubated, with stable hemodynamic and respiratory parameters. ICU discharge four days after surgery and hospital discharged on the seventh postoperative day without complications. Anatomopathological examination revealed ganglioneuroblastoma.
Conclusions: Kinsbourne syndrome is a rare neurological disorder. The drugs used in our patient proved safe and allowed an uneventful anesthesia. Drugs that trigger or aggravate opsoclonus and myoclonus, such as ketamine and etomidate, should be avoided in these patients.
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http://dx.doi.org/10.1016/S0034-7094(13)70232-0 | DOI Listing |
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Kemerovo State Medical University, Kemerovo, Russia.
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder characterized by a combination of main symptoms: opsoclonus, myoclonus, ataxia, psychoemotional and behavioral disturbances. OMS can develop in children as a result of immunopathological processes against the background of infectious or oncological pathology and lead to persistent neurological deficit. A case of ten-year observation of paraneoplastic OMS associated with neuroblastoma in a child is presented.
View Article and Find Full Text PDFCureus
November 2024
Neuropediatrics, Centre for Child Development, Hospital Pediátrico, Unidade Local de Saúde de Coimbra, Coimbra, PRT.
Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare neurological disorder, affecting approximately 0.18 per million individuals annually. It presents with a triad of opsoclonus, myoclonus, and ataxia, often including cognitive dysfunction and behavioral disturbances.
View Article and Find Full Text PDFActa Med Acad
August 2024
Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Background: Paraneoplastic Neurological Syndromes (PNS) constitute a heterogeneous cluster of disease manifestations related to various cancers. Small Cell Lung Cancer (SCLC) is strongly related to PNS. This narrative review conducted a survey in the available PubMed literature to highlight the appearance of PNSs in SCLC cases and discuss published research highlights on the subject so that general practitioners can be acquainted with the medical phenomenon present in SCLC patients.
View Article and Find Full Text PDFCureus
October 2024
Neurology, Hospital San Juan de Dios, San José, CRI.
Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare immunological central nervous system disorder that mostly affects children, and it is extremely uncommon in adults. It usually presents idiopathically, as a parainfectious condition, or as a paraneoplastic syndrome. We present a case of a patient who developed adult-onset opsoclonus-myoclonus-ataxia syndrome (OMAS) without any associated infectious or neoplastic disease, a condition that is considered very rare in Central America.
View Article and Find Full Text PDFEur J Paediatr Neurol
November 2024
Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Department Women and Children's Health, School of Life Course Sciences (SoLCS), King's College, London, United Kingdom. Electronic address:
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