Table 2

Clinical and paraclinical data of anti-FGFR3-positive patients at onset and at full development of the neuropathy (n=65)

ParametersAnti-FGFR3-positive patients
Neurological diagnosis—N SNN/N SFN/ N OSN (% SNN)42/11/12 (65)
Clinical characteristics at the onset of neuropathy
 Age—median year (25th–75th percentile)52.6 (39.7–64.0)
 Sex—F/M (% of F)40/25 (62)
 Course—N (%)
 Acute9/64 (14)
 Subacute13/64 (20)
 Progressive42/64 (66)
 Symptoms reported by patient—N (%)
 Paresthesia/dysesthesia52/65 (80)
 Pain35/65 (54)
 Ataxia34/65 (52)
 Topography of reported symptoms—N (%)
 Purely LL27/65 (42)
 Including UL33/65 (51)
 Asymmetry23/60 (38)
Clinical characteristics at full development of the neuropathy
 Delay—median year (25th–75th percentile)2.9 (1.0–6.0)
 SNN score—median (25th–75th percentile)9.9 (5.1–11.0)
 Symptoms reported by patient—N (%)
 Paresthesia51/65 (79)
 Dysesthesia34/65 (52)
 Pain36/65 (55)
 Topography of reported symptoms—N (%)
 Purely LL18/65 (28)
 Including UL46/65 (71)
 Asymmetry25/65 (39)
 Clinical signs at clinical exam—N (%)
 Purely PTS altered9/63 (14)
 Purely DSS altered14/63 (22)
 PTS + DSS altered38/63 (60)
 Face included8/61 (13)
 Trunk included9/62 (15)
 Ataxia UL25/65 (39)
 Ataxia LL39/65 (60)
 Global areflexia18/65 (28)
 Dysautonomia11/65 (17)
 M-Rankin—median (25th–75th percentile)2.0 (1.0–2.0)
Associated autoimmune diseases—N (%)22/65 (34)
 SS10/64 (16)
 SLE3/64 (5)
 AIH2/64 (3)
 Other7/64 (11)
Other associated diseases—N (%)
 Cancer3/64 (5)
Electrophysiological data
 Delay—median year (25th–75th percentile)3.7 (1.8–8.2)
 Sensory nerve conduction—mean n±SD (mean %)
 UL
 N tested nerves5.0±1.4
 N abnormal SNAPs3.9±2.2 (77)
 N abolished SNAPs1.5±1.9 (30)
 LL
 N tested nerves3.4±0.9
 N abnormal SNAPs2.6±1.5 (77)
 N abolished SNAPs1.6±1.6 (49)
Motor nerve conduction (all limbs)—mean n±SD (mean %)
 N tested nerves5.8±2.2
 N nerves with normal pattern4.7±2.0 (75)
 N nerves with axonal pattern0.6±1.0 (8)
 N nerve with primary demyelinating pattern0.2±0.9 (4)
 N nerve with mixed axonal and demyelinating pattern0.9±1.3 (14)
  • AIH, autoimmune hepatitis; DSS, deep and superficial sensation (myelinated sensory fibres); F, female;FGFR3, fibroblast growth factor receptor 3; LL, lower limbs; LLN, lower limit of the normal; M, male;M-Rankin, modified-Rankin; N, number;OSN, other SN; PTS, pain and temperature sensation (unmyelinated sensory fibres);SFN, small fibre neuropathy; SLE, systemic lupus erythematous;SNAPs, sensory nerve action potentials; SNN, sensory neuronopathy; SS, Sjögren syndrome; UL, upper limbs.