Background
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 |
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) |