Preferred Label : Paraneoplastic opsoclonus myoclonus;
ICD-11 definition : Paraneoplastic opsoclonus myoclonus (OM) results from a targeted attack on the brainstem
as a consequence of a potentially effective tumor immune response initiated by onco-neural
antigens derived from a systemic cancer. In children the cancer is almost always neuroblastoma,
while in adults a variety of cancers may be detected but small-cell lung cancers and
breast cancers are most common. In adults paraneoplastic OM it is often associated
with the neural autoantibody anti-neuronal nuclear antibody type 2 [ANNA-2 or anti-Ri],
but other neural autoantibodies may also accompany OM. Associated autoantibodies:
ANNA-2(anti-Ri) (antineuronal nuclear autoantibody type 2); NMDA-R (N-methyl-D-aspartate
receptor autoantibodies).;
ICD-11 synonym : paraneoplastic Kinsbourne syndrome; paraneoplastic opsoclonus-myoclonus-ataxia syndrome;
Origin ID : 2088074552;
Automatic exact mappings (from CISMeF team)
Paraneoplastic opsoclonus myoclonus (OM) results from a targeted attack on the brainstem
as a consequence of a potentially effective tumor immune response initiated by onco-neural
antigens derived from a systemic cancer. In children the cancer is almost always neuroblastoma,
while in adults a variety of cancers may be detected but small-cell lung cancers and
breast cancers are most common. In adults paraneoplastic OM it is often associated
with the neural autoantibody anti-neuronal nuclear antibody type 2 [ANNA-2 or anti-Ri],
but other neural autoantibodies may also accompany OM. Associated autoantibodies:
ANNA-2(anti-Ri) (antineuronal nuclear autoantibody type 2); NMDA-R (N-methyl-D-aspartate
receptor autoantibodies).
