The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Migraine subtypes
Surat Tanprawate, M.D., MSc (Lond.), FRCP(T)
Director of Neurology Unit, The Northern Neuroscience Centre,
Faculty of Medicine, Chiang Mai University

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Why migraine has many subtypes?

Migraine has aura, and aura has many forms

Migraine has many phases, and each phase can

occur without independently

Migraine involve neuronal hyperexcitability, and

vascular event

Migraine has change over time, and over age group

How ICHD-III Beta classify migraine subtypes?

Migraine
Migraine
without aura

Typical aura

Migraine
with typical
aura

Migraine
with aura

Other aura

Migraine with
brainstem
aura
Hemiplegic
migraine

Typical aura
without
headache

Chronic
migraine

Complication
of migraine

Status
migrainosus
Persistent
aura without
infarct

Migraineous
infarction

Migraine auratriggered seizure

Retinal
migraine

Episodic migraine
that may be
associated with
migraine

Cyclic
vomiting
syndrome
Abdominal
migraine
Benign
paroxysmal
vertigo
Benign
paroxysmal
torticollis

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Aura in migraine

Jann AE Headache 2012 Apr;52(4):687-8.

Somatosensory aura: Cheiro-oral numbness

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Cheiro-oral numbness (CON) due to structural lesion

CON in pontine infarct

CON in parasaggital Hemangioma
Lin HS, Lui CC et al. Neurology India 2005; 53(3)

The Northern Neuroscience Centre

Chiang Mai University

Cheiro-oral-pedal
numbness syndrome
Lin HS, Lui CC et al.
Neurology India 2005; 53(3)

NNC CMU

10 articles
evaluated
aura

Aura more than 1 hour:12-37 %

- Visual aura: 6-10 %
- Sensory aura: 14-27 %
- Aphasic aura: 17-60%

In ICHD-III Beta, they

dont have prolong
aura in migraine
category

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Persistent aura without infarction (PMA)

PMA is a condition in which auras last longer than 1

week, in absence of radiological evidence of infarction.

2 types of PMA

PMA with typical aura (PMA-TA): persistent typical

migraine aura with oscillation, scotoma, fortification in
one hemifield

Persistent primary visual disturbance (PMA-PPVS):

visual snow or television static in the whole visual
field of both eyes in addition to intermittent scooter or
oscillating light
Thissen S and Vos IG et al. Headache 2014

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Pathophysiology

Report of SPECT in patient with persistent aura without infarction

A decreased left fronto-parieto-occipital and right occipital blood perfusion
Relja G, Granato A et al. Cephalalgia 2004, 25, 5659

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Persistent aura without infarction (PMA)

Treatment

Conventional preventive medication have been

tried

Most effective drug: Lamotrigine 50-150 mg

Thissen S and Vos IG et al. Headache 2014

Migraine with brainstem aura

Hemiplegic migraine

(previous term: Basilar migraine)

In some patients, motor

weakness may last weeks

ICHD-III Beta 2013

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Migraine with brainstem aura

Previous terms: basilar migraine, basilar artery

migraine, Bickerstaffs syndrome)

Most common in adolescent females

Rarely headache is absent

MRI/MRA are considered for exclude other possible

causes

Typical form of migraine may appear later

The Northern Neuroscience Centre

Chiang Mai University

Basilar migraine: SPECT

showed hypo perfusion
during an attack
Spina La, Vignati A. Headache 1997;37:4347

NNC CMU

Hemiplegic Migraine - Familial and Sporadic types

Functional roles of the proteins coded by known FHM genes within a glutamatergic synapse
Lancet Neurol 2007; 6: 52132

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Sex distribution, mean age at onset, and attack

frequency of FHM, and SHM

Russell MB, and Ducros A Lancet Neurol 2011;10:457-470

The Northern Neuroscience Centre

Chiang Mai University

Longitudinal f/u 18 pts with SHM

18 pts

NNC CMU

12/18 unchanged

2/18 no longer attack with hemiplegia

4/18 evolved to FHM

Stam AH, Louter MA et al. Cephalalgia 2011; 31(2): 199205

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Motor in FHM and SHM
Overall

Unilateral

Face/tongue

Hand/arm

Foot/leg

100

75

50

25

0
FHM

Mean duration

5 hrs 36 mins

SHM

7 hrs 5 mins

Body

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Accompanying aura in FHM, SHM

Visual aura
Basilar-type aura

Sensory aura

Aphasic aura

100

75

50

25

0
FHM

SHM

Migraine with aura

Thomsen LL, Eriksen MK Brain 2002; 125: 137991
Russell MB, Olesen J. A Brain 1996; 119: 35561
Thomsen LL,Ostergaard E, Neurology 2003; 60: 595601

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Other paroxysmal features

Severe attacks with impaired consciousness

Migraine without aura and typical migraine with

aura (15-34% in FHM)

Epilepsy (60 families has been reported)

Elicited repetitive daily blindness

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Treatment - may aim to reduce weakness

Acute attack

Avoid vasoconstrictor that may trigger aura

Intranasal Ketamine, Triptan (debate)

Prophylaxis

Verapamil, Acetazolamide, Lamotrigine

Treatment disturbing aura in

migraine with Lamotrigine

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Pascual J, Caminero AB et al. Headache 2004;44:1024-1028

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Migraine aura without headache

benign, but a diagnosis of exclusion

Migraine aura without headache - recurrent

episodes of transient symptoms that similar to
aura in migraine

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Diagnostic criteria for typical aura without headache

A. At least two attacks fulfilling criteria B-D
B. Aura consisting of at least one of the following, with or without speech disturbance but no motor weakness
1. Fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or

negative features (ie, loss of vision)

2. Fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative
features (ie, numbness)

C. At least two of the following

1. Homonymous visual symptoms and/or unilateral sensory symptoms
2. At least one aura symptom develops gradually over 5 min and/or different aura symptoms occur in
succession over 5 min
3. Each symptom lasts 5 min and 60 min
D. Headache does not occur during aura nor follow aura within 60 min
E. Not attributed to another disorder

Age distribution of patients with typical aura without headache.

Prevalence of typical migraine

aura without headache

range from 0.2-6.5%

age distribution : late life can
occur
Aiba S, et al. Cephalalgia 2010;30(8):962-967

Age distribution of patients with migraine and aura.

Late life migraine accompaniments

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Diagnostic criteria for Late-life migraine accompaniments

Scintillations or other visual display in the spell. Next in order: paresthesias, aphasia, dysarthria
and paralysis.
Build-up of scintillations. This does not occur in cerebrovascular disease.
March of paresthesias. This does not occur in cerebrovascular disease.
Progression from one accompaniment to another, often with delay.
The occurrence of two or more spells. This helps to exclude embolism.
Headache (present in 50%)
Episodes last 15-25 minutes
Characteristic mid-life flurry of migrainous accompaniments
A general benign course
Normal angiography
Exclusion of cerebral thrombosis, embolism, and dissection, epilepsy, thrombocytopenia,
polycythemia, and TTP

Purdy A. Geriatric and Aging 2003: 6(6): 38-40

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Age-related cerebrovascular change after in

course of migraine
measuring Local Cerebral Blood Flow (LCBF) for cerebral vasodilator
capacity after introduce Acetazolamide (Diamox)
Migraineurs: Severe headache

Migraineurs: Mild or absent headache

or Late-life migraine accompaniments

Meyer JS et al. Cephalalgia 1998; 18: 202-208

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Premonitory symptoms without headache

A middle age woman with recurrent pitting leg

edema, especially during menstrual period

The laboratory test for kidney function, thyroid

function, cardiac function, test for autoimmune
disease were normal

Neither headache nor neurologic symptoms

Typical migraine with aura developed later on

Migraine aura triggered seizure

Migraineous infarction

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Headache and Epilepsy

Migraine aura-triggered seizure (Migralepsy)

Headache attributed to epileptic seizure

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Migraine aura-triggered seizure (Migralepsy)

Diagnostic criteria:

A seizure fulfilling diagnostic criteria for one type of

epileptic attack, and criterion B below

Occurring in a patient with 1.2 Migraine with aura, and

during, or within 1 hour after, an attack of migraine with
aura

Not better accounted for by another diagnosis.

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Headache attributed to epileptic seizure

Description:

Headache caused by an epileptic seizure, occurring during and/or after

the seizure and remitting spontaneously within hours or up to 3 days.

7.6.1. Hemicrania epileptica

Headache occurring during a partial epileptic seizure, ipsilateral to the epileptic
discharge, and remitting immediately or soon after the seizure has terminated

7.6.2. Post-ictal headache

Headache caused by and occurring within 3 hours after an epileptic seizure, and
remitting spontaneously within 72 hours after seizure termination.

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Migraineous infarct

Description:

One or more migrainous aura symptoms associated with an ischaemic

brain lesion in appropriate territory demonstrated by neuroimaging

Diagnostic criteria:
A The present attack in a patient with 1.2 Migraine with aura is typical
of previous attacks except that one or more aura symptoms persists for
>60 minutes
B

Neuroimaging demonstrates ischaemic infarction in a relevant area

Not attributed to another disorder

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Problem in diagnosis

Migraine subtypes

acephalgic migraine

aura without headache

episodic syndrome: cyclic vomiting syndrome,

abdominal migraine, benign paroxysmal
vertigo

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Prevalence of migraine headache in

children with related syndrome
Population prevalence

Recurrent limb pains

Paroxysmal vertigo

Cyclic vomiting

Abdominal migraine
0

10

20

30

40

Olsen J, et al. The Headache 3rd ed.

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Abdominal migraine

Description

An An idiopathic disorder seen mainly in children

as recurrent attacks of moderate to severe
midline abdominal pain, associated with
vasomotor symptoms, nausea and vomiting,
lasting 272 hours and with normality between
episodes.

Headache does not occur during these

episodes.

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Cyclic Vomiting Syndrome (CVS)

Description:

Recurrent episodic attacks of intense nausea and

vomiting, usually stereotypical in the individual and
with predictable timing of episodes.

Attacks may be associated with pallor and lethargy.

There is complete resolution of symptoms between
attacks.

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Symptoms associated with attacks of migraine and

abdominal migraine; results are number (%)

Ishaq Abu-Arafeh, Gerorge Russel Arch of Dis in Child 1995; 72:413-417

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

The range of autonomic co-morbidities in cyclic vomiting

syndrome, migraine and healthy control groups.

Chelimsky G et al. Gastro Res and Prac 2009

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Treatment

Abdominal migraine

pizotifen, propranolol, cyproheptadine

F/U 7-10 years; no current abdominal pain = 61% (migraine

headache 46%)

Cyclic vomiting syndrome

Cyproheptadine, Amitriptyline , Anticonvulsants (topiramate,

zonisamide, and levetiracetam), Propranolol, Phenobarbital,
Erythromycin

F/U 7-10 years; no current CVS = 69% (migraine headache 46%)

Dignan F, and Abu Arafeh Arch Dis Child 2001;84:415418

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Migraine subtypes appear in the appendix

Menstrual migraine: pure menstrual migraine

without aura, Non-menstrual migraine without aura

Vestibular migraine

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Menstrual migraine

A1.1.1 Pure menstrual migraine without aura

A. Attack fulfilling criteria for 1.1 Migraine without aura
B. Recorded evidence over at least 3 consecutive cycles has confirmed
that attacks occur exclusively on day 1 +/- 2 (i.e. days -2 to + 3) of
menstruation in at least two out of three menstrual cycles and at no other
times of the cycle.

A1.1.2 Menstrually related migraine without aura

A. Attack fulfilling criteria for 1.1 Migraine without aura
B. Recorded evidence over at least three consecutive cycles has confirmed
that attacks occur on day 1 +/- 2 (i.e. days -2 to +3) of menstruation in at
least two out of three menstrual cycles, and additionally at other times of
the cycle.
ICHD-III beta

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

Menstrual migraine treatment

decision tree
Tepper SJ. Headache 2006;46 [Suppl 2]:S61-S68)

NSAIDs and Triptans

Magnesium, naproxen, triptans

d -(2 or 3) to +(3-5)

OC therapy, other
migraine preventive med

New diagnostic criteria of Vestibular migraine

(A1.1.6) : ICHD-III Beta version 2013
A. At least five episodes fulfilling criteria C and D

B. A current or past history of 1.1 Migraine without aura or 1.2 Migraine with aura

C. Vestibular symptoms of moderate or severe intensity, lasting between 5

minutes and 72 hours
D. At least 50% of episodes are associated with at least one of the following three
migrainous features:

1. headache with at least two of the following four characteristics: unilateral location,
pulsating quality, moderate or severe intensity, aggravation by routine physical activity

2. Photophobia/phonophobia

3. Visual aura

E. Not better accounted for by another ICHD-III diagnosis or by another vestibular

disorder
ICHD-III Beta 2013

The Northern Neuroscience Centre

Chiang Mai University

Migraine with brainstem aura

Vestibular migraine

NNC CMU

Vestibular migraine pathway

peripheral

inner ear -> TVS innervation

endolymp homeostasis
change

Vestibular migraine pathway

central
shared vestibular system
and migraine generator

posterior insular cortex

anterior insular
orbitofrontal cortex
posterior and anterior
cingulate gyri

Vestibular migraine
treatment

Few studies

acute; zolmitriptan

anti-vertigo agent: promethazine, dimemhydrinate,

meclozine

prophylactic;

nortriptylline, verapamil, metoprolol, topiramate,

flunarizine, valproic acid, lamotrigine

CAI: acetazolamine

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
Simplify the complexity
Axis I
Classify migraine headache
(migraine subtype based on
ICHD criteria)

Classification

Axis II
Classify migraine patients
(simple vs complicate)

Migraine patient classification

1) Uncomplicated migraine
(one of the following)
A. History of IHS migraine currently or
in the past
B. Two of the following
i. strong family history of migraine
ii. sensitivity to light not at time of
headache
iii. sensitivity to sound not at time of
headache
iv. sensitivity to other sensory input
not at time of headache
v. intermittent vertigo not at time of
headache

Kelman L. Headache 2005; 45(8):1088-9

2) Complicated migraine
(one of the following)
A. Comorbidity (anxiety, depression,
bipolar disorder, fibromyalgia, IBS,
sleep disorder, others)
i. easily controlled and not evidently
affecting the headache disorder
ii. complicated (not easily controlled
and possibly affecting the headache
disorder)
B. Medication overuse
i. causing CDH
ii. associated with, but not causing
CDH
iii. medication resistant
i.acute
ii.preventive
iii.acute and preventive

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU

The Northern Neuroscience Centre

Chiang Mai University

NNC CMU
ICHD-III Beta

The Northern Neuroscience Centre

Chiang Mai University

Migraine has many faces

NNC CMU
Conclusion

Event these is under the spectrum of migraine,

but disability, prognosis, complication and
treatment response are different

Diagnostic work-up may need among these

patients