Table 2.
Reports from 2007 to 2015
| Main features | McMillan [5] | Bharucha [14] | Vecino López [15] | Vieira [16] | Riadh [17] | Ghosh [18] |
|---|---|---|---|---|---|---|
| Age at onset | 12 mo | 18 mo | Before 6 mo | 9 mo | 9 mo | 18 mo |
| Current age | 6 yr | 16 yr | 3 yr and 11 mo | 7 yr | 3 yr | NS |
| Sex | M | F | F | M | F | M |
| CN involved (side) | III (L) | III (R) | III(R) | III(R) | III(L) | III(R) |
| Headache (side) | Yes (starting with his 4th episode) | Yes (R) | - | Yes(R, frontotemporal and orbital pain) | Yes(L) | Yes(starting with 2nd episode) |
| Associated symptoms | No | No | - | Yes | Yes | No |
| Photophobia | - | - | - | Yes | - | - |
| Phonophobia | - | - | - | Yes | - | - |
| Nausea | - | - | - | Yes | Yes | - |
| Vomiting | - | - | - | Yes(occasional-ly during the first days of a episode) | Yes | - |
| Irritability | - | - | - | - | - | - |
| Other findings | - | - | - | - | Yes (abdominal pain) | - |
| Ocular symptoms/signs | Yes | Yes | Yes | Yes | Yes | Yes |
| Diplopia | - | Yes | NS | NS | - | Yes (starting with 2nd episode) |
| Ophthalmoplegia | Yes | Yes | Yes | Yes | Yes | No |
| Palpebral ptosis | Yes | Yes | Yes | Yes | Yes | Yes |
| Pupillary dilation | No—During his fourth episode, at 29-months-of-age, the authors describe a left sluggish pupil response | Yes (not reactive to light) | Yes (sluggish pupil response) | Yes | Yes (mildly dilated, reactive to light) | No |
| MRI findings in the acute phase | Yes | Yes (during last episode on the day of onset of symptoms; all previous MRI exams had yielded normal findings | Yes |
Yes (infundibular dilatation of a perforating branch of the posterior cerebral artery emerging just above the superior cerebellar artery, adjacent to the affected nerve) |
No | Yes |
| Nerve thickening | Yes – at the forth episode (29 mo of age; cisternal part of nerve root) | Yes (at nerve root origin) | Yes (cisternal part) | No | - | Yes [cisternal part – performed at 18 mo(first episode)] |
| Post-contrast enhancement | Yes – during first episode (12 mo of age; at the site of exit of nerve root) and forth episode (29 mo of age; cisternal part of nerve root) | Yes (at nerve root origin) | No | No | - | No |
| Altered CSF if lumbar puncture performed | No | No (during last episode) | NS | NS | NS | No |
| Headache duration | 2–3 days (4th episode) | NS | - | 3–7 days | NS | 6–7 days (before development of ptosis |
| Ophthalmoplegia duration | From 2–3 days (1st episode) to 2–3 weeks (4th episode) | NS | 3 mo |
2–5 days (initially) 1–4 weeks |
NS | - |
| Interval between headache onset and ophthalmoplegia | 2–3 days (4th episode) | Within 6 h of onset | NS | At onset of pain | NS | - |
| Time to resolution of Symptoms/Signs | From 2–3 days (1st episode) to 2–3 weeks (4th episode)- The authors describe periodic recurrence with each episode taking longer to recupera-te | Within 1 week of symptom onset (last episode) | 3 mo ( the authors report the use of botulinum toxin for squint) | 1–4 weeks | NS | 3 weeks (1st episode) |
| Therapy in the acute phase | Prednisone(2 mg/kg for 10 days) with tapering over the following week and apparent response | Methylprednisolone iv 25 mg/Kg for 5 days (at last episode, started immediately on the first day of onset) | Oral corticosteroids |
Oral prednisone (1 mg/kg/day) with apparent response This treatment was used twice and the pain subsided much earlier (within 24–48 h) |
3 pulses of methyl-prednisolone followed by an oral steroid therapy (1 mg/kg/day) for 1 week with gradual tapering over 6 weeks |
Methylprednisolone iv 30 mg/Kg for 3 days (1st episode); Immunoglobulin iv 2 g/kg for 2 days (2nd episode) |
| Follow-up | At the age of 6 years, periodic recurrence of complete left III CN paresis, with each episode taking longer to recuperate – episodes of migraine without aura—permanent neurological damage with relative mydriasis (reactive to light) | Yes | Yes ( not fully recovering from ophthalmople-gia) | No episodes | Normal neurologic examination | |
| Prophylactic therapy | Pizotifen (beneficial for migraine,not for ophthalmople-gia) | - | Flunarizine (decreased number of episodes) | - | - | |
| Control MRI | MRI at 15 mo of age with normal findings | Yes (at 3 and 7 months after the onset of symptoms with demonstrated reversal of abnormalities) | MRI after four mo of onset (reduced III CN enlargement) | NS | - | - |
| Number of acute episodes | NS ( the authors describes surely foru episodes at 12, 17, 23 and 29-months-of-age) | 8 | NS | NS | 4 (9 mo, 1y, 2y, 3y) | 3 ( 18mo, 3y, 5y) |
| Interval between episodes | NS | - | NS | From weeks to months | Range 3–12 mo | Range 16–24 mo |
| Comorbidity | No | No | - | No | - | - |
| Family history of migraine | No | - | - | Yes (on the maternal side) | Yes | Yes (on the maternal side) |