Table 1

List and definition of the clinical and paraclinical features

Memory impairmentClinical description, confirmed by cognitive tests
Dysexecutive syndromeClinical description, confirmed by cognitive tests
Temporal lobe epilepsyAt least two clinical seizures, with clinical and/or EEG evidence suggesting a mesial temporal lobe origin
Behavioural disturbancesClinical description, including confusional state
Cerebellar ataxia—permanentClinical description
Cerebellar ataxia—paroxysmalClinical description
Altered general stateAsthenia and/or weight loss and/or anorexia
Weight lossAt least 5 kg, or qualified as ‘major’ or ‘important’
Hyperkinetic movement disordersMyoclonus, dyskinesia, chorea-like movements
Insomnia—non-characterisedPatient reports difficulty to sleep, no further specifications
Major insomniaPatient reports complete or near-complete loss of sleep
Nocturnal hallucinationsClinical description
Agrypnia excitata*Polysomnography
Sleep apnoea*Polysomnography
Peripheral nerve hyperexcitability (PNH)Fasciculations, myokimia, cramps with or without ENMG correlate
Functional impairment due to PNH motor symptomsInvoluntary muscular contractions with moving of segments of limb impairing walk or basic activities
Generalised PNH motor symptomsInvolving four limbs±trunk
Severe signs of PNH on ENMGMultiplets, and/or after discharges, and/or complex repetitive discharges, and/or myokimic discharges, and/or neuromyotonic discharges
DysautonomiaClinical description
Severe dysautonomiaProfuse hypersudation (several changes per day), or orthostatism-independant blood pressure fluctuations, or postural syncopes, or intestinal pseudo-obstruction
Neuropathic painClinical description
Malignant thymoma*Histology
Myasthenia gravisENMG
DyspnoeaClinical description
Abnormal MRI*Medial temporal lobe hypersignal
Abnormal EEG*Abnormal rhythms, periodic patterns and epileptiform abnormalities
Inflammatory CSF*Pleocytosis >5 cells and/or increased proteinorachia and/or presence of OCB
  • *Variables not included in the classification analysis.

  • CSF, cerebrospinal fluid; EEG, electroencephalography; ENMG, electroneuromyography; OCB, oligoclonal bands.