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
No | Sex/age | Delay | Clinical manifestations | Course | Rankin | Electro- physiology | CSF | Tumour | Pathological study | Treatments (neuopathy) |
---|---|---|---|---|---|---|---|---|---|---|
8 | M/65 | 3 m | Proximal and distal sensory motor deficit, pain in four limbs, diffuse areflexia, amyothrophia. Respiratory deficiency. Left Babinski | Subacute | 5 | Neuronal | 1.12 g/l 11 lympho | Lung (CT) | Inflammatory ganglionitis and myelitis, preservation of motor neurons, inflammatory lesions in the nerves, necrotising myopathy | No improvement steroids PE and IgIV |
9 | F/71 | 26 m | Pain, paresthesia, proximal > distal motor deficit in four limbs. Lower limbs areflexia. Bilateral Babinksi. Reticular livedo | Progressive | 4 | Axonal | 0.3 g/l 1 lympho | Pancreas adenocarcinoma | Mild inflammatory ganglionitis and axonal neuropathy, vessel thickening, inflammatory myositis | No improvement steroids |
10 | M/73 | 26 m | Severe sensory > motor deficit in four limbs lower limbs areflexia, transient diplopia bilateral Babinski | Progressive | 4 | Axonal | 1.20 g/l 35 lympho | SCLC lung | Nerve biopsy: fibre loss, axonal degeneration, slight inflammatory reaction, normal muscle | No improvement steroids PE azat |
11 | M/74 | 4 m | Sensory motor proximal and distal deficit in four limbs, amyotrophia, normal tendon reflexes, bilateral Babinski | Progressive | 4 | Neuronal | 2.02 g/l 28 lympho | Urinary (CT) | Nerve biopsy: multifocal axonal lesions, endoneurial inflammatory reaction vasculitis. Muscle: neurogenic atrophy | No improvement steroids |
12 | F/72 | 6 m | Mononeuropathy multiplex. Peroneal and tibial nerves hypereosinophylia, raised ESR, sinusitis | Acute | 3 | Axonal | 0.32 g/l 1 lympho | Colon adenocarcinoma (recidive) | Nerve biopsy: axonal degeneration, vasculitis in a nasal polypoid formation. Muscle: neurogenic atrophy | Improvement steroids (Rankin 3 to 1) |
13 | M/65 | 2 w | Multifocal sensory motor deficit in right arm and lower limbs. Leg areflexia. Orthostatic hypotension | Relapsing | 2 | Axonal | 0.50 g/l 1 lympho | Tongue epidermoid | Nerve biopsy: fibre loss, degenerating fibres, epineural vasculitis | Spontaneous improvement |
14 | M/84 | 7 m | Distal sensory painful asymmetric in four limbs, lower limb areflexia | Subacute | 3 | Axonal | 0.49 g/l 1 lympho | Lung undiffentiated adenocarcinoma | ND | No improvement steroids |
15 | M/85 | 9 m | Distal pain, sensory loss and areflexia in lower limbs | Progressive | 2 | Axonal | 0.53 g/l 1 lympho | Lung (CT scan) | ND | ND |
16 | M/61 | 1 m | Motor>sensory asymmetric deficit in four limbs, fasciculations, absent or reduced tendon reflexes | Acute | 3 | Neuronal | 0.30 g/l 1 lympho | Gastric adenocarcinoma | Neurogenic atrophy in muscle | No improvement steroids |
17 | M/48 | 0 m | Sensory motor Guillain-Barré syndrome, areflexia in four limbs, facial nerve palsy | Acute | 4 | Demyelinating | 0.66 g/l 1 lympho | Tongue epidermoid (recidive) | Nerve biopsy: ongoing macrophage induced demyelination | Improvement IgIV (Rankin 4 to 2) |
18 | M/73 | 3 m | CIDP. Sensory motor mainly proximal deficit and areflexia in four limbs | Progressive | 3 | Demyelinating | 1.27 g/l 1 lympho | Pancreas adenocarcinoma | ND | Improvement IgIV (Rankin 3 to 2) |
19 | M/73 | 6 m | CIDP. Sensory motor mainly proximal mainly upper limbs deficit and areflexia in four limbs | Progressive | 2 | Demyelinating | 0.90 g/l 1 lympho | Colon adenocarcinoma | Nerve biopsy: fiber loss, remyelinated fibers, onion bulb formations, slight inflammatory changes | Improvement after surgery (Rankin 2 to 1) |
20 | M/61 | 9 m | CIDP. Mainly motor proximal>distal deficit and areflexia in four limbs | Progressive | 4 | Demyelinating | 1.60 g/l 1 lympho | Liver adenocarcinoma | Nerve biopsy: almost normal fibers endoneurial lymphocytes | Improvement steroid IgIV azat (Rankin 4 to 1) |
21 | M/78 | + 2 m | Sensory motor proximal and distal deficit in four limbs. Depressed or abolished tendon reflexes | Subacute | 5 | Demyelinating + axonal | 1.60 g/l 1 lympho | Prostate adenocarcinoma | Demyelinated fibers, slight onion bulbs degenerated fibers and regenerating clusters endoneurial macrophages | Improvement 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