Characterisation of pain in people with hereditary neuropathy with liability to pressure palsy

Abstract

Hereditary neuropathy with liability to pressure palsy (HNPP) has historically been considered a pain-free condition, though some people with HNPP also complain of pain. This study characterised persistent pain in people with HNPP. Participants provided cross-sectional demographic data, information on the presence of neurological and persistent pain symptoms, and the degree to which these interfered with daily life. The painDETECT and Central Sensitization Inventory questionnaires were used to indicate potential neuropathic, central sensitisation and musculoskeletal (nociceptive) pain mechanisms. Additionally, participants were asked if they thought that pain was related to/part of HNPP. 32/43 (74%) subjects with HNPP had persistent pain and experience this pain in the last week. Of those with pain, 24 (75%) were likely to have neuropathic pain and 27 (84%) were likely to have central sensitisation. All 32 participants felt that their pain could be related to/part of their HNPP. Significant negative impact of the pain was common. Pain characterisation identified neuropathic pain and/or central sensitisation as common, potential underlying processes. Pain may plausibly be directly related to the underlying pathophysiology of HNPP. Further consideration of including pain as a primary symptom of HNPP is warranted.

A large-scale population-based epidemiological study on the prevalence of central sensitization syndromes in Japan

“…A 40-point cut-off score of CSI part A was recommended to classify the presence of CSS in patients with chronic pain 6 , 8 . Many clinical studies have since reported the rates of a CSI score ≥ 40 in patients with CSS-related diseases: 11.0% in 290 orthopedic patients with musculoskeletal pain, including the neck, shoulders, hips, knees, or ankles 9 , 13% in 238 patients with low back pain 10 , 34.9% in 66 patients with pelvic floor muscle tenderness 11 , 48.4% in 91 patients with osteoarthritis 12 , 68.2% in 763 patients with chronic spinal pain disorder 13 , and 84% in 32 patients with hereditary neuropathy 14 . These findings demonstrated that the prevalence of CSS varied in a CSS-related disease-dependent manner; however, data on the general population is lacking.…”

Comparison of clinical and electrophysiological features of patients with hereditary neuropathy with liability to pressure palsies with or without pain

“…In our study, half of the patients with HNPP suffered from neuropathic pain (52%), mainly burning in the lower limbs. In other cohorts of similar size, the pain rate varied between 16.6% and 74% of patients (13,14). There were no electrophysiological differences based on the presence/absence of pain in these series.…”

“…Pain in HNPP is an emerging concept, in both short and long-term. Regarding recent cohorts, 15 to 75% of HNPP patients suffer from chronic neuropathic pain (12)(13)(14). These patients may be erroneously considered as suffering from fibromyalgia.…”

A large-scale population-based epidemiological study on the prevalence of central sensitization syndromes in Japan

“…The 40-point cut-off score of CSI part A was recommended to classify the presence of CSS in patients with chronic pain 6,8 . Many clinical studies have since reported the rates of a CSI score ≥40 in patients with CSS-related diseases: 11.0% in 290 orthopedic patients with musculoskeletal pain, including the neck, shoulders, hips, knees, or ankles 9 , 13% in 238 patients with low back pain 10 , 34.9% in 66 patients with pelvic floor muscle tenderness 11 , 48.4% in 91 patients with osteoarthritis 12 , 68.2% in 763 patients with chronic spinal pain disorder 13 , and 84% in 32 patients with hereditary neuropathy 14 . These findings demonstrated that the prevalence of CSS varied in a CSS-related disease-dependent manner; however, data on the general population is lacking.…”