Table 2

Demographic, immunological, clinical and therapeutic data of anti-neurofascin-155-IgM-positive patients (in the order of antibody titres)

Patient 1Patient 2Patient 3Patient 4Patient 5
Age at onset (years), gender37, male76, male40, male42, male22, female
Duration of disease5 months6 years36 years1.5 weeks1 year
DiagnosisCIDPSuspected CIDP (not fulfilling the INCAT/EFNS criteria)CIDPGBSSuspected CIDP (not fulfilling the INCAT/EFNS criteria)
ELISA titre1:4001:2001:2001:1001:100
Western blotPositivePositivePositivePositiveNegative
Clinical symptomsDistal >proximal tetraparesisDistal >proximal tetraparesis muscle atrophy, fasciculationsDistal >proximal tetraparesis, muscle atrophy, fasciculations hypesthesia 20 years after onsetSevere tetraparesisDistal >proximal tetraparesis
Distal hypesthesia, neuropathic painDistal hypesthesia, neuropathic painDistal hypesthesia 20 years after onsetHypesthesia, neuropathic painDistal hypesthesia, neuropathic pain
Facial and oculomotor palsy disabling sensory ataxiaSensory ataxiaAutonomic dysfunction, respiratory insufficiencyUrinary incontinence
Antecedent infectionsNoneNoneNoneRespiratory infection 2 weeks prior to onsetExacerbation during infections
Course of diseaseAcute onset, relapsing-remittingSlowly progressiveSlowly progressiveAcute onset, monophasicAcute onset, relapsing-remitting
TremorAction and postural tremorAction tremorDisabling postural tremorRest and postural tremor during recovery phaseNone
CSF (normal values: protein <50 mg/dL, cells<5/µl)Protein 834 mg/dL, 7 cells/µlProtein 103.9 mg/dL, 2 cells/µl58 mg/dL, 1 cell/µlProtein 37 mg/dL, 3 cells/µl31 mg/dL, 2 cells/µl
Nerve biopsyAxonal neuropathy, vasculitic componentDiscrete axonal neuropathyMixed neuropathy, vasculitic infiltratesNot doneAxonal neuropathy
Nodal/paranodal architectureNormalNot doneNot doneNormalNormal
IENFD (distal leg)4.9/mm (slightly reduced)Not doneNot done5.6/mm (slightly reduced)8.9/mm (normal)
NCS, EMGSpontaneous activityNo spontaneous activitySpontaneous activitySpontaneous activitySpontaneous activity
↑↑DML, ↑F wave latency, ↓F wave persistence, ↓CMAP and SNAP, ↓NCV↓CMAP and SNAP, normal DML and F waves, normal NCV
Treatment responsePoor response to IVIG, good response to plasmapheresis and rituximabResponse to IVIGResponse to IVIG and plasma exchangeResponse to IVIG and plasma exchangeNo response to steroids, moderate response to IVIG
  • CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CMAP, compound muscle action potential; CSF, cerebrospinal fluid; DML, distal motor latency; EFNS, European Federation of Neurological Societies; GBS, Guillain-Barré syndrome; IENFD, intraepidermal nerve fibre density; INCAT, Inflammatory Neuropathy Cause And Treatment Group; IVIG, intravenous immunoglobulins; NCS, nerve conduction studies; NCV, nerve conduction velocity; PE, plasma exchange; SNAP, sensory nerve action potential.