Paraneoplastic Neurologic Disorders in Children

Abstract

Paraneoplastic neurologic syndromes are rare disorders that have potentially devastating effects on the developing brain. Recently, there has been increased interest in possible immunotherapy for these disorders. Recognition of paraneoplastic syndromes in children may lead to early detection and treatment of the pediatric cancer and may diminish the neurologic damage that is the major source of morbidity in children with successfully treated tumors. This article reviews the presenting symptoms, immunology, long-term sequelae, and management options for paraneoplastic neurologic syndromes, focusing on those most commonly reported in children: opsoclonus-myoclonus ataxia, limbic encephalitis, and anti-NMDAR encephalitis. The child neurologist plays an important role in recognizing these disorders, initiating a tumor search, and directing ongoing treatment and management of neurologic symptoms after oncologic treatment is complete. Given the rarity of these conditions, multisite collaborative efforts are needed to develop standardized approaches to characterization and treatment.

Demyelinating Neuropathy and Autoimmune Hemolytic Anemia in a Patient with Pancreatic Cancer

“…The representative forms of paraneoplastic neurological syndrome include encephalomyelitis, limbic encephalitis, subacute cerebellar degeneration and sensory neuronopathy (2)(3)(4). Although several types of paraneoplastic neurological syndromes may occur simultaneously or at different time points in a single patient (2,(4)(5)(6)(7), the combination of involvement of the nervous system and extraneural organs in a paraneoplastic setting has not been well documented.…”

Acute‐onset chorea, dystonia, and cardiac fibroelastoma in a child: A paraneoplastic association?

“…Our observation highlights the link between acute-onset choreodystonia and paraneoplastic origin and will hopefully aid in recognition of this unusual syndrome. Maryline Carneiro, MD, 1 Carla Fernandez, MD, 3 Didier Garbi, MD, 4 Loïc Mace, MD, PhD, 5 Mathieu Milh, MD, PhD, 6 Sophie Guillaumont, MD, 2 Bernard Echenne, MD, 1 J erô me Honnorat, MD, PhD, 7,8,9 Agathe Roubertie, MD, PhD* Centre de R ef erence Maladie Rare ''Syndromes Neurologiques Paran eoplasiques,'' Bron, France; 10 INSERM U1051, Institut des Neurosciences de Montpellier, Montpellier, France though he had presented with frequent falls, subtle behavioral changes, and general fatigue since his late 20s. His family history was unremarkable, except for the death of his twin brother soon after birth from unknown causes.…”

“…1 Although the onset of the clinical symptoms usually occurs in adulthood, 2 distinctive neuroimaging features indicating iron deposition are thought to appear decades before symptomatic presentation. 3 We studied a 42-year-old Italian man who first sought neurological advice at age 32 because of hand tremor, even Relevant conflicts of interest/financial disclosures: Nothing to report. Full financial disclosures and author roles may be found in the online version of this article.…”

Recognition and Treatment of Anti-N-Methyl-d-Aspartate Receptor Encephalitis

“…19 Opsoclonus-myoclonus syndrome is marked by opsoclonus (rapid, arrhythmic, conjugate, and multidirectional saccades), myoclonic jerks, ataxia, and behavioral and/or sleep disturbances. 20 Opsoclonus can go unnoticed or be preceded by behavioral/sleep disturbances and ataxia.…”