Table 2

Neuronal surface antibody associated syndromes

SyndromeAntibodiesParticular clinical featuresPossible tumoursImmunotherapy responseIn vitro evidence of Ab pathogenicityFrequency or No of cases reported
NMDAR-Ab encephalitisNMDARDyskinetic movements, decreased consciousness, psychiatric presentation in young women. Epilepsy and abnormal movements more frequent at onset in childrenOvarian teratoma. Rare in children. Up to 50% after age 18 yearsYesIn vitro and in vivo reduction of NMDA receptors
  • Common syndrome.

  • More than 500 cases reported, mainly in USA

LE
  • LGI1

  • CASPR2 (<10%)

Male predominance, hyponatraemia, faciobrachial dystonic seizures, myoclonusRare with LGI1-Ab. Thymoma in some with CASPR2-AbYesIn vitro production of epileptogenic activity in brain slices
  • Common syndrome

  • More than 600 cases reported, mainly in UK

AMPARPossible isolated psychiatric symptoms70% (lung, breast, thymus)Yes, frequent relapsesDownregulation of AMPA receptors14
GABABRProminent seizures60% (SCLC)YesNone25
mGluR5Ophelia syndromeHodgkin lymphomaUnknownNone2
Morvan's syndromeCASPR2Encephalopathy, peripheral nerve hyperexcitability, dysautonomiaThymomaYesNot tested9
PERMGlyREncephalomyelitis with myoclonus, rigidity and brainstem signsThymomaYesNot tested6
Cerebellar ataxiaVGCCPossible coexistence of LEMSSCLCPoorNot tested16
mGluR1Remote history of Hodgkin lymphomaHodgkin lymphomaYesIn vivo3
  • The frequencies given depend on reported cases. Many cases are being diagnosed but are not reported.

  • Ab, antibody; AMPA, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; AMPAR, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; CASPR2, contactin associated protein 2; GABABR, gamma-aminobutyric acid B receptor; GlyR, glycine receptor; LE, limbic encephalitis; LEMS, Lambert-Eaton myasthenic syndrome; LGI1-Ab, leucine rich glioma inactivated 1 protein antibody; mGluR, metabotropic glutamate receptor; NMDA, N-methyl-D-aspartate; NMDAR, N-methyl-D-aspartate receptor; PERM, progressive encephalomyelitis with rigidity and myoclonus; SCLC, small cell lung cancer; VGCC, voltage gated calcium channel.