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Table 1 Main clinical features of family members with ATP1A2 mutation

From: Psychotic aura symptoms in familial hemiplegic migraine type 2 (ATP1A2)

Subject/gender/age (years)

Onset, remission, (peak) (years)

Lifetime occurrence (no. episodes)

Aura

Headache

Accompanying symptoms

Precipitating factors

Duration

Type

Location

Laterality

Duration (h)

Character

Laterality

Intensity

III:6/F/(73)

15 (20–50)

>100

15 min–1 h

Scintillating scotoma; sensitive; motor; aphasic

F, T, UL, LL, Tr

Side-shifting

72 h

Pressing/tightening

Side-shifting

Moderate

N, V

Menstruation.

Pregnancy.

IV:3/M/51

10, 44 (10–16)

>40

15 min

Zigzag lines; sensitive; aphasic.

UL

Side-shifting (90 % right)

6 h

Throbbing

Bilateral

Moderate

N, Pt, Pn

Stress.

IV:4/M/48

16, 45 (26–30)

>20

30–60 min

Scintillating scotoma; sensitive; motor; aphasic; illusion of time-travel.

F, T, UL, LL, Tr

Bilateral (50 %) or Side-shifting (70 % right)

8 h

Throbbing

Side-shifting (70 % right)

Severe

N, V, Pt, Pn Sneezing

Chocolate.

Heading.

IV:6/F/38

11 (36–38)

>50

1 h–5 days

Scintillating scotoma; sensitive; motor; aphasic; illusion of time-travel.

F, T, UL, LL, Tr

Side-shifting (80 % left)

24–72 h

Throbbing

Side-shifting (80 % left)

Severe

N, V, Pt, Pn, Dizziness

Menstruation.

IV:7/F/35

9 (33–35)

>30

15 min-16 days

Scintillating scotoma; sensitive; motor; aphasic.

F, T, UL, LL

Side-shifting (80 % left)

30 min

Pressing/tightening

Side-shifting

Moderate

N, V, Pt, Pn,

Menstruation,

chocolate, coffee.

Hitting a goal post with her head.

Immediate puerperium.

V:1/F/19

13, 15

2

15 min

Sensitive; motor; aphasic.

F, T, UL

Side-shifting

30 min

Pressing/tightening

Bilateral

Moderate

N, Pt, Pn

Facial trauma.

  1. F face, T tongue, UL upper limb, LL lower limb, Tr trunk, N nausea, V vomiting, Pt photophobia, Pn phonophobia, S somnolence