Table 1.
Reports from 1996 to 2007
| Main features | Østergaard [2] | Østergaard [9] | Ramelli [10] | Lance [11] | Weiss [12] | Carlow [13] | Carlow [13] |
|---|---|---|---|---|---|---|---|
| Age at onset | 18 mo | 7 mo | 20 mo | 9 mo | 24 mo | 18 mo | 18 mo |
| Current age | 8 yr | 19 yr | 8 yr | 16 yr | 7 yr | - | - |
| Sex | F | F | F | F | M | F | F |
| CN involved (side) | III (L) | III(L) | III (R) | III (L) | III (L) | - | - |
| Headache (side) | Yes (starting with the 2nd episode; bilateral or left sided) | Yes (starting with her 5th episode; left-sided and eye pain) | Yes (starting with the 2nd episode at the age of 6 yr and 8 mo after a fall backwards – no apparent headache at 1st episode) | Yes (behind the left eye, described as sharp and fluctuating in intensity) | Yes (L; supraorbital) | Yes | Yes |
| Associated symptoms | No | Yes | No | Yes | No | - | - |
| Photophobia | - | - | - | Yes | - | - | - |
| Phonophobia | - | - | - | Yes | - | - | - |
| Nausea | - | - | - | Yes | - | - | - |
| Vomiting | - | Yes | - | Yes (sometimes) | - | - | - |
| Irritability | - | Yes | - | - | - | - | - |
| Other findings |
Signs of varicella infection at 2nd episode Dizziness at 4th episode |
Drowsiness | - | Attacks of screaming | - | - | - |
| Ocular symptoms/signs | Yes | Yes | Yes | Yes | Yes | - | - |
| Diplopia | NS | NS | Yes | NS | Yes | - | - |
| Ophthalmoplegia | Yes (not always present) | Yes | Yes | Yes | Yes | - | - |
| Palpebral ptosis | Yes | Yes | Yes | Yes | Yes | - | - |
| Pupillary dilation | Yes (poorly reactive pupil to light) | Yes | Yes (poorly reactive to light) | Yes (reactive to light with progression to unresponsive pupil) | Yes (sluggish response to light) | No | Yes |
| MRI findings in the acute phase | MRI perfomerd 12 days later 2nd episode onset (ptosis partially resolved) | Not performed | Yes – At second episode (not performed at the 1st episode) | Yes | Yes – performed after 2 weeks of onset (several foci of white matter hyperintensity measuring 3 mm or less identified in the dorsal periventricular region) | Yes | Yes |
| Nerve thickening | Yes (from the brainstem through the prepontin cistern to the carvernous sinus) | - | Yes | No | No | Yes | Yes |
| Post-contrast enhancement | Yes | - | Yes | Yes—at the point of exit of the nerve from the midbrain, continuing along the line of the nerve | No | Yes | Yes |
| Altered CSF if lumbar puncture performed | No (2nd episode) | No | No | NS | - | - | - |
| Headache duration | 3–4 days | NS | NS | NS | NS | - | - |
| Ophthalmoplegia duration | NS | NS | NS | NS | NS | - | - |
| Interval between headache onset and ophthalmoplegia | 3–4 days | 1 day | 4 days (second episode) | NS – 3–4 days between headache and palpebral ptosis | NS | - | - |
| Time to resolution of Symptoms/Signs | 6–8 weeks | 6 mo (1st episode) | Within 2 weeks (first episode) | From few days (2–11) to 2 months | NS | - | - |
| Therapy in the acute phase | Prednisone (2 mg/kg/day) for about 10 days with apparent response | NS | NS | NS | - | - | - |
| Follow-up | Yes ( refered migraine attacks without ophthalmoplegia) | Yes (permanent partial III CN palsy) | - | Yes (with apparent decreased number of episodes) | NS | - | - |
| Prophylactic therapy | - |
Propranolol Metoclopramide Diazepam Acetaminophen |
- | Cafergot; Aspirin; Amitriptyline; Pizotifen; Flunarizine ( 10 mg/day with apparent response) | NS | - | - |
| Control MRI | Performed after 3 months of the 3rd episode onset (persistent enlargement of III CN but to a lesser degree) | MRIs at 14, 15, 16-years-of-age showing persistent enlargement (from the brainstem through the prepontin cistern to the carvernous sinus) | NS |
A repeat MRI scan showed enhancement of the oculomotor nerve still present but less intense; unenhanced MRIs of the brain at the ages of 12 and 14 years were normal |
- | - | - |
| Number of acute episodes | NS ( about four episodes) | NS | NS | NS – About 38 episodes | - | - | - |
| Interval between episodes | Range 15 mo -3 yr | 6–9 mo | NS | NS | - | - | - |
| Comorbidity | Migraine | - | Migraine without aura | - | Migraine | - | - |
| Family history of migraine | No | No | Yes (on the maternal side) | Yes (on the maternal side) | No | Yes | No |