We present the case of a 12-year-old boy who developed progressive lim
bic dysfunction associated with a posterior cord syndrome in conjuncti
on with complex partial seizures and intrathecal synthesis of IgG. Alt
hough the cerebral CT scan appeared normal, MR imaging showed signal a
bnormalities in both temporal lobes. As these clinical features remind
ed us of the well-known paraneoplastic encephalomyelitis (PNEM) in adu
lts, we looked for a tumour and found a pelvic neuroblastoma. The diag
nosis of PNEM was supported by the discovery of the antibody anti-HU i
n the serum of our patient.