Features of double-positive patients reported in the literature
| Demographics | |
| Age (mean, range) | 46.7, 2–86 |
| Male gender, % | 28 M, 76% (n=37) |
| Clinical presentation | n (%) with feature* |
| Morvan’s syndrome | 20/37 (54) |
| Limbic encephalitis | 3/38 (8) |
| Tumour | 16/35 (46) |
| Thymoma | 12/34 (35) |
| Myasthenia gravis | 7/25 (28) |
| Death | 3/21 (14) |
| Peripheral nerve features | |
| NMT/PNH | 27/37 (73) |
| Pain/paraesthesia | 21/34 (62) |
| Peripheral neuropathy | 12/31 (39) |
| Autonomic features | |
| Any dysautonomia | 27/32 (84) |
| Hyperhidrosis | 22/30 (73) |
| Tachycardia/arrhythmia | 9/16 (56) |
| BP abnormalities | 7/23 (30) |
| Sexual dysfunction | 1/15 (7) |
| Gastrointestinal | 7/28 (25) |
| Urinary | 5/25 (20) |
| Sleep | |
| Insomnia | 20/28 (71) |
| Neuropsychiatric | |
| Any | 20/23 (87) |
| Disorientation/confusion/CI | 17/38 (45) |
| Amnesia | 7/19 (37) |
| Hallucinations | 12/22 (55) |
| Agitation/anxiety/depression | 10/28 (36) |
| Delusions | 3/18 (17) |
| Seizures | |
| Any | 8/39 (21) |
| GTCS | 5/31 (16) |
| FBDS/myoclonus | 3/31 (10) |
| Focal/partial | 1/31 (3) |
| Paroxysmal dizziness | 1/24 (4) |
| Systemic | |
| Weight loss | 7/17 (41) |
| Skin lesions/itch/flush | 4/16 (25) |
| Respiratory failure | 3/19 (16) |
| Investigations | |
| Normal brain MRI | 16/17 (94) |
| Normal CSF | 6/15 (40) |
| Serum hyponatraemia | 8/29 (28) |
Demographic and clinical features of 46 double-positive patients described in the literature from 13 papers. *From the published reports, not all features could be evaluated in all patients, so the denominator varies between features. Additional information, not reported in the publication, was included for the 15 patients with double-positive Morvan’s syndrome in Irani et al 13 and may weight this analysis towards features of Morvan’s syndrome.
BP, blood pressure; CSF, cerebrospinal fluid; CI, cognitive impairment; FBDS, faciobrachial dystonic seizures; GTCS, generalised tonic clonic seizure; NMT, neuromyotonia; PNH, peripheral nerve hyperexcitability.