Table 6

Comparison of clinical features between patients with CCPD with simultaneous or temporarily separated onset of CNS and PNS involvement*

Temporarily separated onset groupSimultaneous onset groupp Value†
 Sex ratio (male/female)7:23 (1:3.3)2:6 (1:3)NS
 Age at onset (years, mean±SD)29.4±13.235.0±14.9NS
 Age at examination (years, mean±SD)35.5±14.836.0±14.1NS
 Follow-up period (months, mean±SD)‡112.0±97.744.6±45.00.0316
 Disease duration (months, mean±SD)‡169.3±128.556.9±58.20.0055
Mode of onsetn/N (%)n/N (%)
 Acute4/22 (18.2)2/8 (25.0)NS
 Subacute9/22 (40.9)4/8 (50.0)NS
 Chronic9/22 (40.9)2/8 (25.0)NS
Clinical coursen/N (%)n/N (%)
 Monophasic3/29 (10.3)6/8 (75.0)0.0008
 Relapsing–remitting19/29 (65.5)1/8 (12.5)0.0140
 Chronic progressive7/29 (24.1)1/8 (12.5)NS
Fulfilment of MS or CIDP criterian/N (%)n/N (%)
 McDonald criteria for MS22/30 (73.3)4/8 (50.0)NS
 EFNS/PNS definite criteria for CIDP26/30 (86.7)7/8 (87.5)NS
The number of patients who had already been diagnosed as MS when PNS demyelination developed9/15 (60.0)
The number of patients who had already been diagnosed as CIDP when CNS demyelination developed7/15 (46.7)
Hughes functional scale scoreN=30N=8
 At the peak of illness2.73±1.143.75±1.390.0457
 In remission1.43±1.281.50±1.60NS
 Score changes after treatment1.30±0.992.25±1.160.0427
Symptoms and signsn/N (%)n/N (%)
 Visual disturbance19/30 (63.3)0/8 (0.0)0.0015
 Cranial nerve involvement (other than optic nerves)12/29 (41.4)5/8 (62.5)NS
 Motor weakness29/30 (96.7)7/8 (87.5)NS
 Muscle atrophy9/30 (30.0)2/8 (25.0)NS
 Respiratory disturbance0/30 (0.0)3/8 (37.5)0.0066
 Gait disturbance22/29 (75.9)7/8 (87.5)NS
 Cerebellar ataxia8/30 (26.7)2/6 (33.3)NS
 Sensory disturbance30/30 (100.0)5/7 (71.4)0.0315
 Pathological reflexes13/30 (43.3)5/8 (62.5)NS
 Sphincter disturbance14/29 (48.3)3/7 (42.9)NS
Bloodn/N (%)n/N (%)
 Antineurofascin 155 Ab3/8 (37.5)2/3 (66.7)NS
 Amounts of protein85.3±64.9126.5±88.3NS
 Cell counts4.61±6.0626.0±52.3NS
n/N (%)n/N (%)
 Amounts of protein >40 mg/dL24/30 (80.0)7/8 (87.5)NS
 Cell counts >5/µL7/30 (23.3)3/8 (37.5)NS
 OB2/21 (9.5)0/5 (0.0)NS
 Increased IgG index level4/20 (20.0)1/6 (16.7)NS
MRIn/N (%)n/N (%)
 Brain lesions23/30 (76.7)8/8 (100.0)NS
  Cerebral lesions21/30 (70.0)8/8 (100.0)NS
   Lesions more than 3 cm5/30 (16.7)5/8 (62.5)0.0186
  Cerebellar lesions6/30 (20.0)0/8 (0.0)NS
  Brainstem lesions10/30 (33.3)2/8 (25.0)NS
  Optic nerve lesions6/30 (20.0)1/8 (12.5)NS
  Spinal cord lesions24/30 (80.0)4/8 (50.0)NS
   LESCLs0/30 (0.0)3/8 (37.5)0.0066
VEPsn/N (%)n/N (%)
 Abnormal VEP findings14/17 (82.4)1/4 (25.0)0.0526§
Affected CNS sitesn/N (%)n/N (%)
 Brain only1/30 (3.3)3/8 (37.5)0.0237
 Optic nerves only1/30 (3.3)0/8 (0.0)NS
 Spinal cord only6/30 (20.0)0/8 (0.0)NS
 Brain+optic nerves4/30 (13.3)1/8 (12.5)NS
 Brain+spinal cord9/30 (30.0)3/8 (37.5)NS
 Optic nerves+spinal cord2/30 (6.7)0/8 (0.0)NS
 Brain+optic nerves+spinal cord7/30 (23.3)1/8 (12.5)NS
Treatment efficacyn/N (%)n/N (%)
 Corticosteroid pulse therapy25/27 (92.6)6/8 (75.0)NS
 Oral corticosteroids17/20 (85.0)4/6 (66.7)NS
 IVIg13/20 (65.0)4/5 (80.0)NS
 Plasmapheresis5/6 (83.3)2/2 (100.0)NS
  • *Two patients were excluded because their patterns of onset were undetermined.

  • †A p value<0.05 is regarded as significant. Qualitative variables were analysed by Fisher exact test. Continuous variables that follow a parametric distribution were analysed by Student's t test, while non-parametric variables were analysed by Mann-Whitney U test.

  • ‡Two patients’ data in the temporarily separated onset group were missing.

  • §Indicates a trend (ie, p<0.1).

  • Ab, antibodies; CCPD, combined central and peripheral demyelination; CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CNS, central nervous system; CSF, cerebrospinal fluid; IFN-β, interferon β; IVIg, intravenous immunoglobulin; LESCLs, longitudinally extensive spinal cord lesions; MS, multiple sclerosis; N, number of cases collated; n, number of involved cases; NS, not significant; OB, oligoclonal IgG bands; PNS, peripheral nervous system; VEPs, visual-evoked potentials.