Table 1.
Differential diagnosis of Miller Fisher syndrome.
| Disorder | Extraocular motility abnormalities | Lid abnormalities | Pupil abnormalities | Other abnormalities | Prevalence of anti- GQ1b antibody8 |
|---|---|---|---|---|---|
| Miller Fisher syndrome | Complete bilateral external ophthalmoplegia* Isolated cranial nerve palsy (III, IV, VI) Multiple cranial nerve palsy (III, IV, VI) Appearance of gaze palsy Appearance of internuclear ophthalmoplegia |
Ptosis Lid jerk Lid nystagmus Lid retraction Lagophthalmos |
Mydriasis Anisocoria |
Ataxia* Areflexia* Facial weakness |
>90% |
| Guillain-Barré syndrome | Complete bilateral external ophthalmoplegia Isolated cranial nerve palsy (III, IV, VI) Multiple cranial nerve palsy (III, IV, VI) |
Ptosis Lagophthalmos |
Mydriasis Anisocoria |
Flaccid paralysis* Bulbar weakness* Sensory loss* Back pain |
Up to 83% (in patients with ophthalmoplegia) |
| Bickerstaff’s brainstem encephalitis | Complete bilateral external ophthalmoplegia* | Ptosis | Mydriasis Anisocoria |
Ataxia* Pyramidal weakness* Altered level of consciousness* Sensory loss |
Up to 68% |
| Acute ophthalmoparesis without ataxia | Complete bilateral external ophthalmoplegia* Isolated cranial nerve palsy (III, IV, VI) Multiple cranial nerve palsy (III, IV, VI) Appearance of gaze palsy |
Ptosis | Mydriasis Anisocoria |
100% (required for diagnosis) | |
| Ocular myasthenia gravis | Complete bilateral external ophthalmoplegia Isolated cranial nerve palsy (III, IV, VI) Multiple cranial nerve palsy (III, IV, VI) Appearance of gaze palsy Appearance of internuclear ophthalmoplegia Appearance of skew deviation Nystagmus |
Ptosis (fatigable) Cogan’s lid twitch |
None | Bulbar weakness (in generalized) Proximal limb weakness (in generalized) |
0% |
| Brainstem stroke | Complete bilateral external ophthalmoplegia Internuclear ophthalmoplegia Gaze palsy Nystagmus Multiple cranial nerve palsy (III, IV, VI) |
Ptosis | Mydriasis Anisocoria |
Pyramidal weakness Bulbar weakness Sensory loss Ataxia |
0% |
| Pituitary apoplexy | Complete bilateral external ophthalmoplegia Isolated cranial nerve palsy (III, IV, VI) Multiple cranial nerve palsy (III, IV, VI) |
Ptosis | Mydriasis Anisocoria Relative afferent pupillary defect |
Headache Vision loss Altered level of consciousness |
0% |
| Botulism | Complete bilateral external ophthalmoplegia Nystagmus |
Ptosis | Mydriasis/tonic pupil Anisocoria |
Flaccid paralysis Bulbar weakness Sphincter disturbance |
0% |
| Wernicke’s encephalopathy | Complete bilateral external ophthalmoplegia Isolated cranial nerve palsy (typically VI) Upbeat nystagmus |
Ptosis (rarely) | None | Ataxia Confusion Vision loss (rarely) |
0% |
| Anticonvulsant intoxication | Partial or complete bilateral external ophthalmoplegia Gaze-evoked and vertical nystagmus Impaired smooth pursuit |
Ptosis (rarely) | None | Ataxia Altered level of consciousness |
0% |
*
Key phenotypic feature