Table 2

Clinical data for the 14 patients with a short delay between the onset of the neuropathy and the discovery of the tumour who had no anti-onconeural antibodies (group IIA). Delay indicates the interval between the onset of neurological symptoms and cancer diagnosis and is expressed in months (m) or weeks (w). In every case, except case 13, the neuropathy preceded the discovery of the tumour. The revised Rankin score is used to estimate the maximal deficit reached by the patients

NoSex/ageDelayClinical manifestationsCourseRankinElectro-
CSFTumourPathological studyTreatments (neuopathy)
8M/653 mProximal and distal sensory motor deficit, pain in four limbs, diffuse areflexia, amyothrophia. Respiratory deficiency. Left Babinski Subacute5Neuronal1.12 g/l 11 lymphoLung (CT)Inflammatory ganglionitis and myelitis, preservation of motor neurons, inflammatory lesions in the nerves, necrotising myopathyNo improvement steroids PE and IgIV
9F/7126 mPain, paresthesia, proximal > distal motor deficit in four limbs. Lower limbs areflexia. Bilateral Babinksi. Reticular livedo Progressive4Axonal0.3 g/l 1 lymphoPancreas adenocarcinomaMild inflammatory ganglionitis and axonal neuropathy, vessel thickening, inflammatory myositisNo improvement steroids
10M/7326 mSevere sensory > motor deficit in four limbs lower limbs areflexia, transient diplopia bilateral Babinski Progressive4Axonal1.20 g/l 35 lymphoSCLC lungNerve biopsy: fibre loss, axonal degeneration, slight inflammatory reaction, normal muscleNo improvement steroids PE azat
11M/744 mSensory motor proximal and distal deficit in four limbs, amyotrophia, normal tendon reflexes, bilateral Babinski Progressive4Neuronal2.02 g/l 28 lymphoUrinary (CT)Nerve biopsy: multifocal axonal lesions, endoneurial inflammatory reaction vasculitis. Muscle: neurogenic atrophyNo improvement steroids
12F/726 mMononeuropathy multiplex. Peroneal and tibial nerves hypereosinophylia, raised ESR, sinusitisAcute3Axonal0.32 g/l 1 lymphoColon adenocarcinoma (recidive)Nerve biopsy: axonal degeneration, vasculitis in a nasal polypoid formation. Muscle: neurogenic atrophyImprovement steroids (Rankin 3 to 1)
13M/652 wMultifocal sensory motor deficit in right arm and lower limbs. Leg areflexia. Orthostatic hypotension Relapsing2Axonal0.50 g/l 1 lymphoTongue epidermoidNerve biopsy: fibre loss, degenerating fibres, epineural vasculitisSpontaneous improvement
14M/847 mDistal sensory painful asymmetric in four limbs, lower limb areflexiaSubacute3Axonal0.49 g/l 1 lymphoLung undiffentiated adenocarcinomaNDNo improvement steroids
15M/859 mDistal pain, sensory loss and areflexia in lower limbsProgressive2Axonal0.53 g/l 1 lymphoLung (CT scan)NDND
16M/611 mMotor>sensory asymmetric deficit in four limbs, fasciculations, absent or reduced tendon reflexes Acute3Neuronal0.30 g/l 1 lymphoGastric adenocarcinomaNeurogenic atrophy in muscleNo improvement steroids
17M/480 mSensory motor Guillain-Barré syndrome, areflexia in four limbs, facial nerve palsyAcute4Demyelinating0.66 g/l 1 lymphoTongue epidermoid (recidive)Nerve biopsy: ongoing macrophage induced demyelinationImprovement IgIV (Rankin 4 to 2)
18M/733 mCIDP. Sensory motor mainly proximal deficit and areflexia in four limbs Progressive3Demyelinating1.27 g/l 1 lymphoPancreas adenocarcinomaNDImprovement IgIV (Rankin 3 to 2)
19M/736 mCIDP. Sensory motor mainly proximal mainly upper limbs deficit and areflexia in four limbsProgressive2Demyelinating0.90 g/l 1 lymphoColon adenocarcinomaNerve biopsy: fiber loss, remyelinated fibers, onion bulb formations, slight inflammatory changesImprovement after surgery (Rankin 2 to 1)
20M/619 mCIDP. Mainly motor proximal>distal deficit and areflexia in four limbsProgressive4Demyelinating1.60 g/l 1 lymphoLiver adenocarcinomaNerve biopsy: almost normal fibers endoneurial lymphocytesImprovement steroid IgIV azat (Rankin 4 to 1)
21M/78+ 2 mSensory motor proximal and distal deficit in four limbs. Depressed or abolished tendon reflexesSubacute5Demyelinating + axonal1.60 g/l 1 lymphoProstate adenocarcinomaDemyelinated fibers, slight onion bulbs degenerated fibers and regenerating clusters endoneurial macrophagesImprovement steroids (Rankin 5 to 4)
  • Course corresponds to the onset of neurological symptoms: acute, <1 month; subacute 1-2 months; progressive, >2 months.. CIDP=chronic inflammatory demyelinating polyneuropathy; TD=temporal dispersion; CB= conduction block. PE=plasma exchanges. IgIV=intravenous immunoglobulins. azat=azathioprine. When improvement occurred after immunotherapy, the Rankin score before and after treatment is given in parentheses. Other abbreviations are the same as in table 1