Skip to main content
. Author manuscript; available in PMC: 2009 Apr 1.
Published in final edited form as: Lancet Neurol. 2008 Apr;7(4):327–340. doi: 10.1016/S1474-4422(08)70060-7

Table 4.

Recently identified antibodies in patients with cerebellar degeneration, opsoclonus-myoclonus, and limbic encephalitis

Syndrome Tumour Number of
cases
Comments
CARP VIII59 PCD Melanoma 1 CARP VIII is highly enriched in the cerebellum
Zic1, Zic 460 PCD SCLC 18 Mutations of Zic1 and Zic4 result in cerebellar malformation (Dandy-Walker)
PKCγ61 PCD Non-SCLC 1 Missense mutations of PKCγ result in autosomal dominant cerebellar ataxia
(SCA14)
Proteasome62 PCD Ovary, other 12 All patients had anti-Yo antibodies
Neuroleukin63 Opsoclonus No tumour 2 Post-streptococcal
Gliadin, IgA subtype64 Opsoclonus No tumour 1 Celiac disease; patient also had endomysial and CV2 antibodies
Adenomatous polyposis
coli65
Opsoclonus 2 SCLC, 2
idiopathic
4 Protein highly expressed in brainstem, cerebellum and hippocampus;
2/4 patients had opsoclonus, the other 2 had nystagmus, diplopia, ataxia
Zic265 Opsoclonus SCLC 1 Member of the Zic family of proteins, involved in cerebellar development (see
above)
Anti-BR serine/threonine
kinase 266
LE SCLC 1 ..
Antibodies to CRMP3-467 LE Thymoma 1 The affected patient also had GAD antibodies
Adenylate kinase 568 LE No tumour 2 Refractory to treatments
GluRε269 LE Ovarian teratoma 1 Described in many other disorders. Different from anti-NR1/NR2 heteromers of
NMDA receptor

CARP=carbonic anhydrase-related protein. PCD=paraneoplastic cerebellar degeneration. PKC=protein kinase C. SCLC=small-cell lung cancer. SCA=spinocerebellar ataxia. LE=limbic encephalitis. GAD=glutamic-acid decarboxylase. CRMP=collapsin-response mediator protein.