Table 2

Clinical manifestations and paraclinical investigations in patients with anti-FGFR3 Abs and sensory neuropathy

Number	Sex/age	Associated context	Before neuropathy	Neuropathy onset	Clinical presentation	SNN criteria	Non-length dependent	CSF Protein/cell	Electrophysiology	Other findings
1	M/42	Psoriasic rheumatism, SSA and SSB antibodies	Yes	Progressive	Ataxia and lower limb areflexia	NA	NA	ND	ND
2	F/33	Systemic lupus erythematosus	Yes	Subacute	Burning and constricting pain in chest and limbs	No	Yes	ND	ND
3	F/67	Systemic lupus erythematosus	Yes	Progressive	Pain in hands and legs. Lower limb areflexia and vibration sensory loss in lower limbs	No	No	ND	ND
4	F/69	Systemic lupus erythematosus	Yes	Progressive	Ataxia, paraesthesia, pain and vibration loss in all four limbs. Lower limb areflexia	NA	Yes	ND	ND
5	F/54	Rheumatic polyarthritis, primary biliary cirrhosis, Sjögren's syndrome	Yes	Subacute	Right facial pain with allodynia and left facial paraesthesia	No	Yes	ND	ND
6	F/37	None		Progressive	Burning pain in feet. Reduced pain sensation in lower limbs	No	No	ND	ENMG normal. Abolished potentials in both feet and both hands on LEP	Reduced fibre density on skin biopsy
7	F/73	None		Progressive	Burning pain in feet and hands. Reduced pain sensation in lower limbs. Lower limb ataxia	No	Yes	ND	Reduced SAP in both feet and both hands. Normal MCV
8	M/44	Melanoma in remission 8 years earlier	Yes	Progressive	Paraesthesia and pain and vibration sensory loss in the four limbs. Left face numbness. Asymmetrical distribution. Diffuse areflexia	Yes	Yes	ND	Abolished SAP in both feet and both hands Normal MCV	Nerve biopsy: moderate MF loss. No regenerating fibres
9	F/44	HIV infection	Yes	Acute	Pain, dysesthesia, ataxia and pain and vibration sensory loss in all four limbs. Areflexia	Yes	Yes	1.1 g/L polyclonal 27 WCC/mm³	Reduced or abolished SAP in both feet and both hands. Normal MCV
10	F/59	Thyroid cancer in remission 5 years earlier	Yes	Subacute	Paraesthesia and pain and vibration sensory loss in all four limbs and face. Asymmetrical distribution. Diffuse areflexia	Yes	Yes	Normal	Reduced or abolished SAP in both feet and both hands. Normal MCV	Nerve biopsy: severe MF loss. No regenerating fibres
11	M/38	None		Subacute	Loss of pain and thermal sensation over the whole body. Lower limb areflexia. Depressed vibration sensation	Yes	Yes	Normal	Reduced or abolished SAP in both feet and both hands. Normal MCV	Reduced fibre density on skin and nerve biopsy. No regenerating fibres
12	M/18	Sjögren's syndrome	No	Progressive	Severe sensory loss predominately in large fibres with ataxia in all four limbs. Areflexia. Abnormal pupils and sudomotor disturbances	Yes	Yes	0.26 g/L oligoclonal 1 WCC/mm³	Abolished SAP in both feet and both hands. Normal MCV	Nerve biopsy: MF loss. No regenerating fibres
13	M/58	Sjögren's syndrome	Yes	Progressive	Severe ataxia. Sensory loss in all four limbs and left face. Areflexia	Yes	Yes	0.80 g/L polyclonal 3 WCC/mm³	Reduced or abolished SAP in both feet and both hands. Normal MCV
14	F/51	ANA and anticitrullin antibodies	No	Progressive	Pain in all four limbs. Dysesthesia and pain and vibration sensory loss in the left leg. Areflexia	Yes	Yes	0.52 g/L polyclonal 1 WCC/mm³	Reduced SAP in both feet and both hands. Normal MCV	Nerve biopsy: MF loss. No regenerating fibres
15	M/50	Ulcerative rectohaemorrhagic colitis, ankylosing spondylitis	No	Acute	Severe paraesthesia and ataxia in all four limbs with pain and vibration sensory loss. Areflexia	Yes	Yes	0.73 g/L polyclonal 1 WCC/mm³	Abolished SAP in both feet and both hands. Normal MCV	Nerve fibre loss on skin biopsy
16	F/34	None		Progressive	Electric pain and paraesthesia in the upper limbs and left leg. Pain and vibration sensory loss. Areflexia	Yes	Yes	Normal	Reduced SAP in both feet and both hands. Normal MCV	Nerve biopsy: fibre loss. Axonal degeneration. T-cells infiltration

‘Before neuropathy’ indicates whether or not the autoimmune disease preceded the onset of neuropathy.
CSF, cerebrospinal fluid; ENMG, electroneuromyography; F, female; FGFR3, fibroblast growth factor receptor 3; LEP, laser evoked potentials; M, male; MCV, motor conduction velocities; MF, myelinated fibre; NA, not available; ND, not done; SAP, sensory action potentials; SNN, sensory neuronopathy; WCC, white cell count.