The International Classification of Headache Disorders 2nd Edition



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14. Other Headache, cranial neuralgia, central or primary facial pain


14.1 Headache not elsewhere classified

14.2 Headache unspecified



Introduction


In order to make this classification exhaustive there is, after the entries for many disorders, a subcategory for conditions that fulfil all but one criterion for that disorder. Still there may be headaches that cannot fit into any of the existing chapters because they are being described for the first time, or because there simply is not enough information available. This chapter is intended for these types or subtypes of headaches.

14.1 Headache not elsewhere classified

Previously used term:

Headache not classifiable
Diagnostic criteria:

A. Headache with characteristic features suggesting that it is a unique diagnostic entity

B. Does not fulfil criteria for any of the headache disorders described above



Comment:

Several new headache entities have been described in the time between the first edition of The International Classification of Headache Disorders and this second edition. It is anticipated that there are more entities still to be described. Such headaches, until classified, can be coded as 14.1 Headache not elsewhere classified.

14.2 Headache unspecified

Previously used term:

Headache not classifiable
Diagnostic criteria:

A. Headache is or has been present

B. Not enough information is available to classify the headache at any level of this classification



Comment:

It is also apparent that a diagnosis must be made in a large number of patients where very little information is available, allowing only to state that they have headache but not which type of headache. Such patients are coded as 14.2 Headache unspecified. This code, however, must never be used as an excuse for not gathering detailed information about a headache when such information is available. It should be used only in situations where information cannot be obtained because the patient is dead, unable to communicate or unavailable.

APPENDIX



Introduction


In the first edition of The International Classification of Headache Disorders there was no appendix. This time an appendix is added which, we hope, will be used in several ways.

The primary purpose of the appendix is to present research criteria for a number of novel entities that have not been sufficiently validated by research studies. However, the experience of the experts in the Headache Classification Subcommittee and publications of variable quality suggest that there are a number of diagnostic entitities that are believed to be real but for which further scientific evidence must be presented before they can be formally accepted. Therefore it is anticipated that a number of the disorders now in the appendix will move into the main body of the classification next time the classification is revised.

In a few places we present an alternative set of diagnostic criteria to those in the main body of the classification. This is again because clinical experience and a certain amount of published evidence suggest that this may be a good idea, but the subcommittee still does not feel that the evidence is sufficient to change the main classification. This is, for example, the case for the accompanying symptoms of migraine without aura. The alternative diagnostic criterion D in the appendix is easier both to understand and to apply, but not yet sufficiently validated.

Finally, the appendix is used as a first step in eliminating disorders included as diagnostic entities in the first edition because of tradition but for which sufficient evidence has still not been published.


A1. Migraine

A1.1 Migraine without aura

Alternative diagnostic criteria:

A. At least 5 attacks fulfilling criteria B-D

B. Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated) and occurring on 15 days/month

C. Headache has at least two of the following characteristics:

1. unilateral location

2. pulsating quality

3. moderate or severe pain intensity

4. aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)


  1. During headache at least two of the following:

1. nausea

2. vomiting

3. photophobia

4. phonophobia

5. osmophobia

E. Not attributed to another disorder



Comment:

Only criterion D is different from those in the main body of the classification. Whilst this alternative appears easier both to understand and to apply, it is not yet sufficiently validated.

A1.1.1 Pure menstrual migraine without aura

Diagnostic criteria:

  1. Attacks, in a menstruating woman, fulfilling criteria for 1.1 Migraine without aura

  2. Attacks occur exclusively on day 1±2 (ie, days –2 to +3)1 of menstruation2 in at least two out of three menstrual cycles and at no other times of the cycle



Notes:

1. The first day of menstruation is day 1 and the preceding day is day –1; there is no day 0.

2. For the purposes of this classification, menstruation is considered to be endometrial bleeding resulting from either the normal menstrual cycle or from the withdrawal of exogenous progestogens, as in the case of combined oral contraceptives and cyclical hormone replacement therapy.


A1.1.2 Menstrually-related migraine without aura

Diagnostic criteria:

  1. Attacks, in a menstruating woman, fulfilling criteria for 1.1 Migraine without aura

  2. Attacks occur on day 1±2 (ie, days –2 to +3)1 of menstruation2 in at least two out of three menstrual cycles and additionally at other times of the cycle



Notes:

1. The first day of menstruation is day 1 and the preceding day is day –1; there is no day 0.

2. For the purposes of this classification, menstruation is considered to be endometrial bleeding resulting from either the normal menstrual cycle or from the withdrawal of exogenous progestogens, as in the case of combined oral contraceptives and cyclical hormone replacement therapy.


A1.1.3 Non-menstrual migraine without aura

Diagnostic criteria:

A. Attacks, in a menstruating woman, fulfilling criteria for 1.1 Migraine without aura

  1. Attacks have no menstrual relationship1



Note:

1. That is, they do not fulfil criterion B for A1.1.1 Pure menstrual migraine without aura or A1.1.2 Menstrually-related migraine without aura.
Comments:

This subclassification of 1.1 Migraine without aura is applicable only to menstruating women.

The importance of distinguishing between A1.1.1 Pure menstrual migraine and A1.1.2 Menstrually-related migraine is that hormone prophylaxis is more likely to be effective for pure menstrual migraine. Documented prospectively-recorded evidence, kept for a minimum of three cycles, is necessary to confirm the diagnosis as many women over-report an association between attacks and menstruation.

Menstrual attacks are mostly migraine without aura. In a woman who has migraine both with and without aura, migraine with aura does not appear to be associated with menstruation.

The mechanism(s) of migraine may be different with endometrial bleeding resulting from the normal menstrual cycle and bleeding due to the withdrawal of exogenous progestogens (as occurs with combined oral contraception and cyclical hormone replacement therapy). For example, the endogenous menstrual cycle results from complex hormonal changes in the hypothalamic-pituitary-ovarian axis resulting in ovulation, which is suppressed by use of combined oral contraceptives. Therefore research should separate these subpopulations. Management strategies may also differ for these distinct subpopulations.

There is some evidence that menstrual attacks, at least in some women, result from oestrogen withdrawal, although other hormonal and biochemical changes at this time of the cycle may also be relevant. If pure menstrual migraine or menstrually-related migraine is considered to be associated with exogenous oestrogen withdrawal, both codes A1.1.1 Pure menstrual migraine without aura or A1.1.2 Menstrually-related migraine without aura and 8.4.3 Oestrogen-withdrawal headache should be used.

A1.2.7 Migraine aura status

Diagnostic criteria:

A. Migraine aura fulfilling aura criteria for 1.2 Migraine with aura or one of its subtypes

B. At least 2 auras per day for ≥5 consecutive days


A1.3.4 Alternating hemiplegia of childhood

Description:

Infantile attacks of hemiplegia involving each side alternately, associated with a progressive encephalopathy, other paroxysmal phenomena and mental impairment.
Diagnostic criteria:

A. Recurrent attacks of hemiplegia alternating between the two sides of the body

B. Onset before the age of 18 months

C. At least one other paroxysmal phenomenon is associated with the bouts of hemiplegia or occurs independently, such as tonic spells, dystonic posturing, choreoathetoid movements, nystagmus or other ocular motor abnormalities, autonomic disturbances

D. Evidence of mental and/or neurological deficit(s)

E. Not attributed to another disorder

Comment:

This is a heterogeneous condition that includes neurodegenerative disorders. A relationship with migraine is suggested on clinical grounds. The possibility that it is an unusual form of epilepsy cannot be ruled out.

A1.3.5 Benign paroxysmal torticollis

Description:

Recurrent episodes of head tilt to one side, perhaps with slight rotation, which remit spontaneously. The condition occurs in infants and small children with onset in the first year. It may evolve into 1.3.3 Benign paroxysmal vertigo of childhood or 1.2 Migraine with aura, or cease without further symptoms.
Diagnostic criteria:

A. Episodic attacks, in a young child, with all of the following characteristics and fulfilling criterion B:

1. tilt of the head to one side (not always the same side), with or without slight rotation

2. lasting minutes to days

3. remitting spontaneously and tending to recur monthly

B. During attacks, symptoms and/or signs of one or more of the following:

1. pallor

2. irritability

3. malaise

4. vomiting

5. ataxia1



  1. Normal neurological examination between attacks

  2. Not attributed to another disorder



Note:

1. Ataxia is more likely in older children within the affected age group.
Comments:

The child’s head can be returned to the neutral position during attacks: some resistance may be encountered but can be overcome.

A1.3.5 Benign paroxysmal torticollis may evolve to 1.3.3 Benign paroxysmal vertigo of childhood or 1.2 Migraine with aura (particularly 1.2.6 Basilar-type migraine).

These observations need further validation by patient diaries, structured interviews and longitudinal data collection. The differential diagnosis includes gastro-oesophageal reflux, idiopathic torsional dystonia and complex partial seizure, but particular attention must be paid to the posterior fossa and craniocervical junction where congenital or acquired lesions may produce torticollis.

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