Clinical descriptions of patients
Patients | Age (y) | Duration of disease (y) | Clinical features | Electrophysiology | Other laboratory features | Diagnosis |
---|---|---|---|---|---|---|
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: | ||||||
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 |