Table 2.
Differential diagnosis of ROPPVAL syndrome
| ROPPVAL | REEC | CLUSTER HEADACHE | SUNA/SUNCT | CLASSICAL MIGRAINE | TYPICAL OPTIC NEURITIS | ANTERIOR UVEITIS | |
|---|---|---|---|---|---|---|---|
| Age (years) | < 45 (mean 25) | Any | 20–40 | >45 | <40 at onset | Young | Any |
| Sex | F > M | Any | M > F | M > F | F > M | F > M | F = M |
| Laterality | Unilateral | Often unilateral | unilateral | Unilateral | Unilateral | Unilateral | Uni- or bilateral |
| Onset | Acute | Acute | Acute | Acute | Variable | Acute | Acute |
| History of corneal trauma | No | Yes (often) | No | No | No | No | No |
| Visual Loss | Yes (variable) | Possible | No | No | No | Yes | Variable |
| Retro-Ocular Pain | Yes | No | yes | In SUNCT | Yes | Possible | |
| Retro-Ocular Pain exacerbated by eye movement | No | Possible (related to cornea) | No | No | No | Yes | No |
| Eye pain relieved by local anesthetics | No | Yes | Yes | No | No | No | No |
| RAPD | No | No | no | Possible miosis | No | Yes | No |
| Headache | Possible | Rare | Yes | Yes | Yes | Possible | Possible |
| Systemic Symptoms | Possible | No | Possible | In SUNA | Yes | No (except for symptoms of MS) | Rare (e.g. if IOP is markedly elevated etc.) |
| Photophobia | Yes (unilateral and ipsilateral) | Yes | Possible | Possible | Possible (bilateral) | No | Yes |
| Tearing | No | Yes | Yes (possible) | In SUNCT | No | No | No |
| Visual Aura | No | No | Rare | No | Yes | No | No |
| Dischromatopsia | Yes (variable) | Rare | No | No | No | Yes | Rare |
| Nasal Discharge | No | Yes | Possible | Possible | No | No | No |
| SLE | Normal | Abnormal | Normal (except tearing) | Tearing and conjunctival injection | Normal | Normal | Abnormal |
| Fundus Findings | Normal | Normal | Normsal | Normal | Normal | Normal | Normal |
| Neuro-Imaging | Normal | Normal | Normal | Normal | Normal | Abnormal | Normal |
| Known/proposed mechanism and pathophysiology | Unknown (possible migraine like syndrome) | Abnormal corneal epithelial growth (often after trauma) | Hypotalamus related (sleep role in the disease?) | Part of trigeminal autonomic cephalalgias | Primarily a neurogenic process with secondary changes in cerebral perfusion | Demyelinating (MS most frequent) |
Variable (infectious/inflammatory, traumatic, lens related, etc) |
| Treatment | Systemic (as per migraine) ± cycloplegics | Local | Systemic | Systemic | Systemic | Systemic (IV steroids) | Local/systemic |
| Prognosis | Good | Usually good | Long-standing disease | Long-standing disease | Long-standing disease | Good – prognosis specific to MS | good |
F = Female; IOP = intraocular pressure; IV = intravenous; M = Male; MS = Multiple sclerosis; RAPD = Relative afferent pupil defect; REEC = Recurrent epithelial erosion of the cornea; SUNA = Short-lasting unilateral neuralgiform headache with autonomic symptoms; SUNCT = Short-lasting neuralgiform headache attacks with conjunctival injection and tearing.