Table 4.
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.