Biomaterial Database

Paraneoplastic neurologic disorders in children. 2011

Elizabeth M Wells, and Josep Dalmau

Department of Neurology, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC, 20010, USA. ewells@cnmc.org

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.

UI	MeSH Term	Description	Entries
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D016194	Receptors, N-Methyl-D-Aspartate	A class of ionotropic glutamate receptors characterized by affinity for N-methyl-D-aspartate. NMDA receptors have an allosteric binding site for glycine which must be occupied for the channel to open efficiently and a site within the channel itself to which magnesium ions bind in a voltage-dependent manner. The positive voltage dependence of channel conductance and the high permeability of the conducting channel to calcium ions (as well as to monovalent cations) are important in excitotoxicity and neuronal plasticity.	N-Methyl-D-Aspartate Receptor,N-Methyl-D-Aspartate Receptors,NMDA Receptor,NMDA Receptor-Ionophore Complex,NMDA Receptors,Receptors, NMDA,N-Methylaspartate Receptors,Receptors, N-Methylaspartate,N Methyl D Aspartate Receptor,N Methyl D Aspartate Receptors,N Methylaspartate Receptors,NMDA Receptor Ionophore Complex,Receptor, N-Methyl-D-Aspartate,Receptor, NMDA,Receptors, N Methyl D Aspartate,Receptors, N Methylaspartate
D053578	Opsoclonus-Myoclonus Syndrome	A neurological condition that is characterized by uncontrolled rapid irregular movements of the eye (OPSOCLONUS) and the muscle (MYOCLONUS) causing unsteady, trembling gait. It is also known as dancing eyes-dancing feet syndrome and is often associated with neoplasms, viral infections, or autoimmune disorders involving the nervous system.	Paraneoplastic Opsoclonus-Myoclonus Ataxia,Dancing Eyes, Dancing Feet Syndrome,Dancing Eyes-Dancing Feet Syndrome,Kinsbourne Syndrome,Myoclonic Encephalopathy of Infants,Opsoclonus Myoclonus,Opsoclonus Myoclonus Ataxia,Dancing Eyes Dancing Feet Syndrome,Infants Myoclonic Encephalopathies,Infants Myoclonic Encephalopathy,Myoclonus, Opsoclonus,Opsoclonus Myoclonus Syndrome,Paraneoplastic Opsoclonus Myoclonus Ataxia
D020361	Paraneoplastic Syndromes, Nervous System	Degenerative or inflammatory conditions affecting the central or peripheral nervous system that develop in association with a systemic neoplasm without direct invasion by tumor. They may be associated with circulating antibodies that react with the affected neural tissue. (Intern Med 1996 Dec;35(12):925-9)	Paraneoplastic Autonomic Dysfunction,Paraneoplastic Encephalomyelitis,Autonomic Dysfunction, Paraneoplastic,Nervous System Paraneoplastic Syndromes,Paraneoplastic Neurologic Syndromes,Autonomic Dysfunctions, Paraneoplastic,Encephalomyelitis, Paraneoplastic,Neurologic Syndrome, Paraneoplastic,Neurologic Syndromes, Paraneoplastic,Paraneoplastic Autonomic Dysfunctions,Paraneoplastic Encephalomyelitides,Paraneoplastic Neurologic Syndrome,Syndrome, Paraneoplastic Neurologic,Syndromes, Paraneoplastic Neurologic
D020363	Limbic Encephalitis	A paraneoplastic syndrome marked by degeneration of neurons in the LIMBIC SYSTEM. Clinical features include HALLUCINATIONS, loss of EPISODIC MEMORY; ANOSMIA; AGEUSIA; TEMPORAL LOBE EPILEPSY; DEMENTIA; and affective disturbance (depression). Circulating anti-neuronal antibodies (e.g., anti-Hu; anti-Yo; anti-Ri; and anti-Ma2) and small cell lung carcinomas or testicular carcinoma are frequently associated with this syndrome.	Paraneoplastic Limbic Encephalitis,Encephalitis, Limbic,Encephalitides, Paraneoplastic Limbic,Encephalitis, Paraneoplastic Limbic,Limbic Encephalitis, Paraneoplastic