Table 1

Clinical descriptions of patients

PatientsAge (y)Duration of disease (y)Clinical featuresElectrophysiologyOther laboratory featuresDiagnosis
Max FT, maximum finger-thumb opposition rate; IVIg, intravenous immunoglobulin; SNAP, sensory nerve action potential; EMG, electromyography; NCV, nerve conduction velocity; CSF, cerebrospinal fluid; ND, not done; NA, not available (another hospital).
Motor neuropathy:
    14713Asymmetric muscle wasting and weakness. Good response to IVIgNormal SNAPsAnti GM1 negativeMultifocal motor neuropathy with conduction block
Motor conduction block
Max FT=1 Hz
    24617Asymmetric leg then progressive distal arm weakness and wasting. Good response to IVIgNormal SNAPsAnti GM1 positiveMultifocal motor neuropathy with conduction block
EMG: asymmetric denervation
Max FT=2 HzNCV: asymmetric motor nerve slowing.
    36616Progressive leg weakness and wasting. Good response to IVIgNormal SNAPsCSF protein 1.85 g/lSymmetric pure motor demyelinating neuropathy
EMG: chronic partial denervationAnti GM1 negative
Max FT=4 HzNCV: Motor slowing
    4448Progressive asymmetric arm and leg weakness and wasting. Good response to IVIgNormal SNAPsAnti GM1 negativeMultifocal motor neuropathy with conduction block
NCV: focal condution block
Max FT=4 Hz
    5605Progressive asymmetric arm and leg weakness. Good response to IVIgNormal SNAPsAnti GM1 negativeMultifocal motor neuropathy with conduction block
NCV: Focal conduction block
Max FT=4 Hz
    6789Progressive asymmetric arm and leg weakness. Good response to IVIgNormal SNAPsAnti GM1 negativeMultifocal motor neuropathy with conduction block
NCV: focal conduction blockBiopsy: axonal neuropathy
Max FT=3 Hz
Sensory neuropathy:
    7594Ascending sensory loss with relative presentation of pinprick sensation. Steroid responsiveSNAPs decreased or absentAnti GQIb negativeIdiopathic pure sensory neuropathy
EMG normalAnti GMI negative
NCV: no motor slowing
    86111Progressive asymmetric numbness of upper limbs. Myotonic pupils without sicca syndromeSNAPs decreased or absentAnti Hu negativeSensory ganglionitis
Motor NCV normal
    9628Asymmetric ascending numbnessSNAPs absentIgM λ peakIdiopathic pure sensory neuropathy
Anti GQ1b positive
    105118Ascending numbness. autonomic impairment.SNAPs absentAnti GQ1b positiveIdiopathic pure sensory neuropathy
IVIg responsiveMotor NVC normal
Inclusion body myositis:
    117412Profound symmetric weakness finger flexors and quadricepsNACK 510 IU/lIBM
Muscle biopsy consistent with IBM
    12627Mild symmetric weakness of finger flexors and marked weakness of quadricepsNACK 650 IU/LIBM
Muscle biopsy consistent with IBM
    13506Mild asymmetric weakness of elbow and finger flexion. Moderate symmetric quadriceps weaknessNACK 600 IU/lIBM
Muscle biopsy consistent with IBM
    14669Mild symmetric weakness to finger flexion. Moderate symmetric weakness to hip flexion and knee extension. DysphagiaNormal SNAPsCK 319 IU/lIBM
EMG: myopathic changes and denervationMuscle biopsy consistent with IBM