Table 1 Reports from 1996 to 2007

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