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