Table 2. Prevalence of recorded neuromuscular disease in the UK on 1/1/2019.
| Classification | n | All (95% CI) | Females (95%CI) | Males (95%CI) |
|---|---|---|---|---|
| Motor Neuron Disorders | ||||
| • Motor neurone disease (MND) | 1,586 | 12.6 (11.9–13.2) | 10.1 (9.3–10.9) | 15.1 (14.1–16.0) |
| • Post polio syndrome | 468 | 3.7 (3.4–4.0) | 3.7 (3.2–4.2) | 3.7 (3.3–4.2) |
| • Spinal muscular atrophy | 629 | 5.0 (4.6–5.4) | 4.2 (3.7–4.7) | 5.8 (5.2–6.4) |
| Muscle Disease | ||||
| Acquired myopathies | ||||
| • Endocrine myopathy | 59 | 0.5 (0.3–0.6) | 0.5 (0.3–0.7) | 0.4 (0.3–0.6) |
| • Infectious myopathy | 438 | 3.5 (3.1–3.8) | 2.9 (2.5–3.3) | 4.1 (3.6–4.6) |
| • Inflammatory myopathies (IIM) | 3,152 | 25.0 (24.1–25.8) | 31.3 (30.0–32.7) | 18.6 (17.5–19.6) |
| • Toxic or drug-induced myopathy | 324 | 2.6 (2.3–2.8) | 2.0 (1.7–2.4) | 3.1 (2.7–3.5) |
| Hereditary myopathies | ||||
| • Congenital myopathy | 387 | 3.1 (2.8–3.4) | 3.0 (2.5–3.4) | 3.2 (2.7–3.6) |
| • Metabolic myopathies | 709 | 5.6 (5.2–6.0) | 4.9 (4.3–5.4) | 6.4 (5.7–7.0) |
| • Muscular dystrophy (MD) | 3,723 | 29.5 (28.5–30.4) | 23.3 (22.1–24.5) | 35.7 (34.2–37.1) |
| Other Muscle Disease | ||||
| • Mitochondrial disease | 563 | 4.5 (4.1–4.8) | 4.8 (4.3–5.3) | 4.1 (3.6–4.6) |
| • Myotonic disorders (non-dystrophic) | 334 | 2.6 (2.4–2.9) | 2.2 (1.8–2.6) | 3.1 (2.7–3.5) |
| • Myotonic disorders (unspecified) | 407 | 3.2 (2.9–3.5) | 3.4 (3.0–3.9) | 3.0 (2.6–3.4) |
| • Periodic paralysis | 159 | 1.3 (1.1–1.5) | 0.8 (0.6–1.1) | 1.7 (1.4–2.0) |
| Neuropathies | ||||
| Hereditary neuropathy | ||||
| • Charcot-Marie Tooth (CMT) | 3,724 | 29.5 (28.5–30.4) | 26.8 (25.5–28.0) | 32.2 (30.8–33.6) |
| • Other | 83 | 0.7 (0.5–0.8) | 0.5 (0.4–0.7) | 0.8 (0.6–1.0) |
| Inflammatory & autoimmune neuropathies | ||||
| • Guillain-Barré syndrome (GBS) | 5,064 | 40.1 (39.0–41.2) | 36.3 (34.8–37.8) | 43.9 (42.3–45.6) |
| • GBS recorded in last 5 years only | 1,325 | 10.5 (9.9–11.1) | 8.8 (8.1–9.6) | 12.2 (11.3–13.0) |
| • Other | 1,656 | 13.1 (12.5–13.7) | 9.5 (8.7–10.2) | 16.8 (15.8–17.8) |
| Neuromuscular Junction Disorders | ||||
| • Eaton-Lambert syndrome | 44 | 0.3 (0.2–0.5) | 0.4 (0.2–0.5) | 0.3 (0.2–0.5) |
| • Myasthenia gravis (MG) | 4,250 | 33.7 (32.7–34.7) | 33.6 (32.2–35.1) | 33.7 (32.3–35.1) |
| • Other | 401 | 3.2 (2.9–3.5) | 2.9 (2.5–3.4) | 3.4 (3.0–3.9) |
| Muscular or neuromuscular disease unspecified | 930 | 7.4 (6.9–7.8) | 7.0 (6.3–7.7) | 7.7 (7.0–8.4) |
| All Neuromuscular Disease | ||||
| • Any | 28,230 | 223.6 (221.0–226.2) | 208.3 (204.8–211.9) | 239.0 (235.1–242.8) |
| • Any, standardised to 2019 mid-year UK population estimates | 220.3 (217.7–222.9) | 206.2 (202.7–209.7) | 234.8 (231.1–238.6) | |
| All Neuromuscular Disease, only with GBS in last 5 years | ||||
| • Any | 24,565 | 194.6 (192.1–197.0) | 181.4 (178.1–184.7) | 207.8 (204.2–211.4) |
| • Any, standardised to 2019 mid-year UK population estimates | 191.9 (189.5–194.3) | 179.6 (176.3–182.9) | 204.5 (201.0–208.0) |
Note: Rates are per 100,000 persons. All neuromuscular disease is additionally age-sex standardised to ONS 2019 mid-year population estimates for the whole of the UK. All rates represent lifetime prevalence, except for GBS which is additionally presented including only codes recorded in last 5 years. Patients can belong to multiple categories except for “unspecified”, where they are only classified if no other appropriate classification was available. Denominators used were: 12,625,287 (All), 6,328,836 (Females) and 6,296,451 (Males).