Table 2 Reports from 2007 to 2015

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)