Onset and course of chronic inflammatory demyelinating polyneuropathy

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is clinically heterogeneous. Our purpose was to determine whether initial progression time, clinical features, and distribution of nerve conduction slowing at presentation correlate with clinical course and prognosis. We examined how findings at presentation related to clinical course during an average follow-up time of 4.0 (range 1.0-9.0) years in 44 patients with CIDP. We calculated terminal latency index (TLI), a measure of differential slowing in distal relative to more proximal nerve segments. Patients with acute or subacute onset (progression over less than 8 weeks) had a higher remission rate (P = 0.012) than patients with chronic onset (progression over more than 8 weeks). Patients with proximal weakness had a higher remission rate than patients with the distal phenotype (P < 0.001). All 5 patients with a relapsing course had subacute onset. They had lower TLIs, suggesting a more distal pattern of demyelination, than patients with a monophasic or chronic course. In conclusion, subacute onset and presence of proximal weakness are good prognostic signs that correlate with a high rate of recovery to normal in CIDP. Distal accentuation of conduction slowing at presentation correlates with subacute onset and a relapsing course.

Length‐dependent weakness and electrophysiological signs of secondary axonal loss in chronic inflammatory demyelinating polyradiculoneuropathy

“…There is no evidence in the present study for the suggestion that the conduction is more affected distally than in the intermediate nerve segment in CIDP, and post‐hoc estimation of the terminal latency index (TLI)26 (TLI; distal conduction distance [mm]/forearm conduction velocity [m/s]/distal latency [ms]), which compares conduction in distal and intermediate nerve segments, showed a mean TLI of 0.44 ± 0.07 in CIDP compared to 0.41 ± 0.04 ( P = 0.06) in controls. These numbers are in line with previous observations 26, 31. Regression analysis of the TLI and strength or clinical assessment scores did not show any significant relationships, most likely reflecting the interrelationship between the distal and intermediate conduction velocity.…”

Length‐dependent weakness and electrophysiological signs of secondary axonal loss in chronic inflammatory demyelinating polyradiculoneuropathy

“…There is no evidence in the present study for the suggestion that the conduction is more affected distally than in the intermediate nerve segment in CIDP, and post‐hoc estimation of the terminal latency index (TLI)26 (TLI; distal conduction distance [mm]/forearm conduction velocity [m/s]/distal latency [ms]), which compares conduction in distal and intermediate nerve segments, showed a mean TLI of 0.44 ± 0.07 in CIDP compared to 0.41 ± 0.04 ( P = 0.06) in controls. These numbers are in line with previous observations 26, 31. Regression analysis of the TLI and strength or clinical assessment scores did not show any significant relationships, most likely reflecting the interrelationship between the distal and intermediate conduction velocity.…”

Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy

“…Because of the retrospective design of the study, we could not compare the various therapies performed in our population of patients (intravenous immune globulin, plasma exchange or corticosteroids). In other studies, a worse course of CIDP was related to a chronic onset (progression over more than 8 weeks)22 and to asymmetrical symptoms and demyelination in the intermediate nerve segments 21…”

Outcome in chronic inflammatory demyelinating polyneuropathy: A systematic review and meta‐analysis

“…A total of 952 patients in 18 reports were included in the meta‐analysis for case fatality rate 4–6,8,10,11,19,35–45 . The study characteristics are outlined in the Table 1.…”

“…Eleven studies reported that most of the patients had typical CIDP, 5,6,8,10,11,19,20,35–38 with the pooled fraction being 62%. Forty‐eight percent were currently receiving immunotherapy 4,6,11,19,37–39 …”

Length‐dependent weakness and electrophysiological signs of secondary axonal loss in chronic inflammatory demyelinating polyradiculoneuropathy

“…There is no evidence in the present study for the suggestion that the conduction is more affected distally than in the intermediate nerve segment in CIDP, and post‐hoc estimation of the terminal latency index (TLI)26 (TLI; distal conduction distance [mm]/forearm conduction velocity [m/s]/distal latency [ms]), which compares conduction in distal and intermediate nerve segments, showed a mean TLI of 0.44 ± 0.07 in CIDP compared to 0.41 ± 0.04 ( P = 0.06) in controls. These numbers are in line with previous observations 26, 31. Regression analysis of the TLI and strength or clinical assessment scores did not show any significant relationships, most likely reflecting the interrelationship between the distal and intermediate conduction velocity.…”

Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy

“…Because of the retrospective design of the study, we could not compare the various therapies performed in our population of patients (intravenous immune globulin, plasma exchange or corticosteroids). In other studies, a worse course of CIDP was related to a chronic onset (progression over more than 8 weeks)22 and to asymmetrical symptoms and demyelination in the intermediate nerve segments 21…”

Outcome in chronic inflammatory demyelinating polyneuropathy: A systematic review and meta‐analysis

“…A total of 952 patients in 18 reports were included in the meta‐analysis for case fatality rate 4–6,8,10,11,19,35–45 . The study characteristics are outlined in the Table 1.…”

“…Eleven studies reported that most of the patients had typical CIDP, 5,6,8,10,11,19,20,35–38 with the pooled fraction being 62%. Forty‐eight percent were currently receiving immunotherapy 4,6,11,19,37–39 …”

Length‐dependent weakness and electrophysiological signs of secondary axonal loss in chronic inflammatory demyelinating polyradiculoneuropathy

“…There is no evidence in the present study for the suggestion that the conduction is more affected distally than in the intermediate nerve segment in CIDP, and post‐hoc estimation of the terminal latency index (TLI)26 (TLI; distal conduction distance [mm]/forearm conduction velocity [m/s]/distal latency [ms]), which compares conduction in distal and intermediate nerve segments, showed a mean TLI of 0.44 ± 0.07 in CIDP compared to 0.41 ± 0.04 ( P = 0.06) in controls. These numbers are in line with previous observations 26, 31. Regression analysis of the TLI and strength or clinical assessment scores did not show any significant relationships, most likely reflecting the interrelationship between the distal and intermediate conduction velocity.…”

Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy

“…Because of the retrospective design of the study, we could not compare the various therapies performed in our population of patients (intravenous immune globulin, plasma exchange or corticosteroids). In other studies, a worse course of CIDP was related to a chronic onset (progression over more than 8 weeks)22 and to asymmetrical symptoms and demyelination in the intermediate nerve segments 21…”

Outcome in chronic inflammatory demyelinating polyneuropathy: A systematic review and meta‐analysis

“…A total of 952 patients in 18 reports were included in the meta‐analysis for case fatality rate 4–6,8,10,11,19,35–45 . The study characteristics are outlined in the Table 1.…”

“…Eleven studies reported that most of the patients had typical CIDP, 5,6,8,10,11,19,20,35–38 with the pooled fraction being 62%. Forty‐eight percent were currently receiving immunotherapy 4,6,11,19,37–39 …”