Motor neuropathy: |
1 | 47 | 13 | Asymmetric muscle wasting and weakness. Good response to IVIg | Normal SNAPs | Anti GM1 negative | Multifocal motor neuropathy with conduction block |
| | | | Motor conduction block | | |
| | | Max FT=1 Hz | | | |
2 | 46 | 17 | Asymmetric leg then progressive distal arm weakness and wasting. Good response to IVIg | Normal SNAPs | Anti GM1 positive | Multifocal motor neuropathy with conduction block |
| | | | EMG: asymmetric denervation | | |
| | | Max FT=2 Hz | NCV: asymmetric motor nerve slowing. | | |
3 | 66 | 16 | Progressive leg weakness and wasting. Good response to IVIg | Normal SNAPs | CSF protein 1.85 g/l | Symmetric pure motor demyelinating neuropathy |
| | | | EMG: chronic partial denervation | Anti GM1 negative | |
| | | Max FT=4 Hz | NCV: Motor slowing | | |
4 | 44 | 8 | Progressive asymmetric arm and leg weakness and wasting. Good response to IVIg | Normal SNAPs | Anti GM1 negative | Multifocal motor neuropathy with conduction block |
| | | | NCV: focal condution block | | |
| | | Max FT=4 Hz | | | |
5 | 60 | 5 | Progressive asymmetric arm and leg weakness. Good response to IVIg | Normal SNAPs | Anti GM1 negative | Multifocal motor neuropathy with conduction block |
| | | | NCV: Focal conduction block | | |
| | | Max FT=4 Hz | | | |
6 | 78 | 9 | Progressive asymmetric arm and leg weakness. Good response to IVIg | Normal SNAPs | Anti GM1 negative | Multifocal motor neuropathy with conduction block |
| | | | NCV: focal conduction block | Biopsy: axonal neuropathy | |
| | | Max FT=3 Hz | | | |
Sensory neuropathy: |
7 | 59 | 4 | Ascending sensory loss with relative presentation of pinprick sensation. Steroid responsive | SNAPs decreased or absent | Anti GQIb negative | Idiopathic pure sensory neuropathy |
| | | | EMG normal | Anti GMI negative | |
| | | | NCV: no motor slowing | | |
8 | 61 | 11 | Progressive asymmetric numbness of upper limbs. Myotonic pupils without sicca syndrome | SNAPs decreased or absent | Anti Hu negative | Sensory ganglionitis |
| | | | Motor NCV normal | | |
9 | 62 | 8 | Asymmetric ascending numbness | SNAPs absent | IgM λ peak | Idiopathic pure sensory neuropathy |
| | | | | Anti GQ1b positive | |
10 | 51 | 18 | Ascending numbness. autonomic impairment. | SNAPs absent | Anti GQ1b positive | Idiopathic pure sensory neuropathy |
| | | IVIg responsive | Motor NVC normal | | |
Inclusion body myositis: |
11 | 74 | 12 | Profound symmetric weakness finger flexors and quadriceps | NA | CK 510 IU/l | IBM |
| | | | | Muscle biopsy consistent with IBM | |
12 | 62 | 7 | Mild symmetric weakness of finger flexors and marked weakness of quadriceps | NA | CK 650 IU/L | IBM |
| | | | | Muscle biopsy consistent with IBM | |
13 | 50 | 6 | Mild asymmetric weakness of elbow and finger flexion. Moderate symmetric quadriceps weakness | NA | CK 600 IU/l | IBM |
| | | | | Muscle biopsy consistent with IBM | |
14 | 66 | 9 | Mild symmetric weakness to finger flexion. Moderate symmetric weakness to hip flexion and knee extension. Dysphagia | Normal SNAPs | CK 319 IU/l | IBM |
| | | | EMG: myopathic changes and denervation | Muscle biopsy consistent with IBM | |