Review Article

Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?

Table 2

Diagnostic criteria of paraneoplastic limbic encephalitis.

Criteria by Gultekin et al. [13]

Pathological demonstration of limbic encephalitis, or all 4 of the following.
(1) Symptoms of short-term memory loss, seizures, or psychiatric symptoms suggesting involvement of the limbic system
(2) <4 yr between the onset of neurological symptoms and the cancer diagnosis
(3) Exclusion of metastasis, infection, metabolic and nutritional deficits, stroke, and side-effects of therapy that may cause limbic encephalopathy
(4) At least one of the following:
 (a) CSF with inflammatory findings
 (b) MRI FLAIR or T2 unilateral or bilateral temporal lobe hyperintensities
 (c) EEG with epileptic or slow activity focally involving the temporal lobes

Criteria by the Paraneoplastic Neurological Syndrome Euronetwork [14]

All 4 of the following items are met.
(i) Subacute onset (days or up to 12 wk) of seizures, short-term memory loss, confusion, and psychiatric symptoms
(ii) Neuropathologic or radiologic evidence (MRI, SPECT, PET) of involvement of the limbic system
(iii) Exclusion of other possible etiologies of limbic dysfunction
(iv) Demonstration of a cancer within 5 yr of the diagnosis of neurologic symptoms or the development of classic symptoms of limbic dysfunction in association with a well-characterized paraneoplastic antibody (Hu, Ma2, CRMP5, amphiphysin, Ri)