Primary headaches

Primary headaches include clinically heterogeneous types of headaches. Their pathogenesis remains incompletely understood, and treatment approaches have not yet been substantiated by controlled clinical trials. In most cases, the forms are primary (benign). At the same time, the symptoms of some of them may resemble clinical manifestations in secondary cephalalgias, when additional studies, including neuroimaging, are mandatory. For example, "4.6. Primary thunderclap headache" is almost always characterized by an acute onset, so patients often end up in emergency departments. For differential diagnosis with organic causes of cephalalgia, a thorough examination is necessary.

4. Other primary headaches (ICHD-2, 2004)

• 4.1 Primary stabbing headache.
• 4.2 Primary cough headache.
• 4.3. Primary headache due to physical exertion.
• 4.4 Primary headache associated with sexual activity.
  • 4.4.1. Preorgasmic headache.
  • 4.4.2. Orgasmic headache.
• 4.5. Hypnic headache.
• 4.6 Primary thunderclap headache.
• 4.7. Hemicrania continua.
• 4.8. New daily (initially) persistent headache.

[ 1 ], [ 2 ], [ 3 ]

Primary stabbing headache (4.1)

Synonyms: ice-pick headache, jab and jolt syndrome, periodic ophthalmodynia.

Description

Transient, clearly localized painful stabbing pains in the head area that occur suddenly in the absence of organic pathology of the underlying structures or cranial nerves.

Diagnostic criteria

• A. Pain that occurs as a sensation of a single prick (stab) or a series of stabs in the head area and meets the BD criteria.
• B. The pain is localized exclusively or predominantly in the innervation zone of the first branch of the trigeminal nerve (in the area of the eye, temple or crown).
• C. The stabbing pain lasts for a few seconds and recurs throughout the day with irregular frequency from one prick to several series of pricks.
• D. The pain is not accompanied by associated symptoms.
• E. Not associated with other causes (disorders).

In the only published descriptive study, 80% of the stabbing pains lasted 3 seconds or less. Rarely, patients have had multiple, recurrent stabbing pains over several days. A single episode of status primary stabbing cephalalgia has been described, lasting for 1 week. The stabbing pains may move from one area of the head to another within one half of the head or may cross to the other side. If stabbing pains occur strictly in one area of the head, structural damage to that area and to the distribution of the corresponding nerve must be excluded. Stabbing pains occur most frequently in patients with migraine (about 40%) or cluster headaches (about 30%), and, as a rule, in the areas of the head where migraine or cluster headaches are usually localized.

Treatment

Several uncontrolled studies have shown a beneficial effect of indomethacin; other studies have not confirmed its effectiveness for this form of headache.

Primary cough headache (4.2)

Synonyms

Benign cough headache, Valsalva phenomenon headache.

Description

Headache provoked by coughing or straining in the absence of intracranial pathology.

Diagnostic criteria

• A. Headache fulfilling criteria B and C.
• B. Sudden onset, pain duration from 1 sec to 30 min.
• C. Pain occurs only in connection with coughing, straining, or the Valsalva maneuver.
• D. Not associated with other causes (disorders).

Primary cough headache is usually bilateral and occurs more frequently in people over 40 years of age. Indomethacin is usually effective, but there have been a few cases of response to indomethacin in symptomatic cough headaches.

In 40% of cases, cough headache is symptomatic (secondary), and most patients have Arnold-Chiari malformation type I. Other cases of symptomatic cough pain may be due to vertebrobasilar disorders or intracranial aneurysm. Neuroimaging methods are of great importance for the differential diagnosis of symptomatic cough cephalgias and primary cough headaches.

[ 4 ], [ 5 ]

Primary exertional headache (4.3)

Description

Headache provoked by any physical exertion. Various subtypes have been noted, such as loader's headache.

Diagnostic criteria

• A. Throbbing headache fulfilling criteria B and C.
• B. Duration of pain from 5 minutes to 48 hours.
• C. Pain occurs only during or after physical exertion.
• D. Not associated with other causes (disorders).

Primary headache with physical exertion often occurs in hot weather or at altitude. Cases of relief of this pain after oral administration of ergotamine have been described. Indomethacin is also effective in most cases. At the first appearance of headaches associated with physical exertion, it is necessary to exclude subarachnoid hemorrhage or arterial dissection.

[ 6 ], [ 7 ]

Primary headache associated with sexual activity (4.4)

Synonym

Coital headache.

Description

Headache provoked by sexual activity, in the absence of an intracranial disorder. Usually begins as a dull bilateral pain, increases with sexual arousal, and reaches a maximum during orgasm.

There are two forms of coital cephalgia:

• preorgasmic (4.4.1) - dull pain in the head or neck, combined with a feeling of tension in the neck and/or chewing muscles, occurs during sexual activity and increases with sexual arousal;
• orgasmic (4.4.2) - sudden intense ("explosive") headache that occurs during orgasm.

Data on the duration of headache associated with sexual activity are inconsistent. It is believed that its duration may vary from 1 min to 3 h. The occurrence of postural headache after sexual intercourse has been described. In this case, the headache resembles pain with low cerebrospinal fluid pressure and should be assessed as "7.2.3. Headache associated with spontaneous (idiopathic) decrease in cerebrospinal fluid pressure". In approximately 50% of cases, a combination of primary headache associated with sexual activity, primary headache with physical exertion, and migraine has been described. At the first appearance of orgasmic pain, subarachnoid hemorrhage or arterial dissection should be excluded.

Treatment

In most cases, due to its short-term nature, treatment is not performed. If headache associated with sexual activity lasts more than 3 hours, the patient should be examined to determine the nature of the pain.

Hypnic headache (4.5)

Synonyms

"Alarm clock" headache.

Description

Attacks of dull headache, always awakening the patient from sleep.

Diagnostic criteria

• A. Dull headache meeting BD criteria.
• B. The pain develops only during sleep and awakens the patient.
• C. At least two of the following characteristics:
  • occurs >15 times per month;
  • continues for >15 min after awakening;
  • first appears after 50 years.
• D. Not accompanied by vegetative symptoms, one of the following symptoms may be present: nausea, photo- or phonophobia.
• E. Not associated with other causes (disorders).

The pain in hypnic cephalgia is often bilateral, usually mild or moderate in intensity. Severe pain is noted in 20% of patients. Attacks last for 15-180 minutes, sometimes a little longer. At the first appearance of hypnic cephalgia, it is necessary to exclude intracranial pathology, as well as to conduct differential diagnostics with trigeminal vegetative cephalgia.

Treatment

Caffeine and lithium have been effective in a few patients.

Primary thunderclap headache (4.6)

Description

Intense, acute headache, reminiscent of the pain of a ruptured aneurysm.

Diagnostic criteria

• A. Severe headache fulfilling criteria B and C.
• B. Both of the following:
  • sudden onset with peak intensity reached in less than 1 min;
  • pain duration from 1 hour to 10 days.
• C. Does not recur regularly over subsequent weeks or months.
• D. Not associated with other causes (disorders).

There is still insufficient evidence to suggest that thunderclap headache is a primary disorder. The diagnosis of primary thunderclap headache can be made when all diagnostic criteria are met and neuroimaging and lumbar puncture are normal. Therefore, a thorough examination to exclude other causes of pain is absolutely necessary. Thunderclap headache is often associated with intracranial vascular disorders, in particular with subarachnoid hemorrhage. Therefore, additional examination should be aimed primarily at excluding subarachnoid hemorrhage, as well as such disorders as cerebral venous thrombosis, unruptured vascular malformation (usually aneurysm), arterial dissection (intra- and extracranial), CNS angiitis, reversible benign CNS angiopathy, and pituitary apoplexy. Other organic causes of thunderclap headache include colloid cyst of the third ventricle, decreased cerebrospinal fluid pressure, and acute sinusitis (particularly with barotraumatic injuries). Thunderclap headache symptoms may also be a manifestation of other primary forms: primary cough headache, primary cephalgia with physical exertion, and primary headache associated with sexual activity. The coding "4.6. Primary thunderclap headache" can only be used after all organic causes of pain have been excluded.

Treatment

There is evidence of the effectiveness of gabapentin in the primary form of thunderclap headache.

Hemicrania continua (4.7)

Description

Persistent, strictly unilateral headache, relieved by indomethacin.

Diagnostic criteria

• A. Headache lasting more than 3 months, meeting BD criteria.
• B. All of the following:
  • one-sided pain without changing sides;
  • daily continuous pain without clear intervals;
  • moderate intensity with episodes of increased pain.
• C. During an exacerbation (intensification) of pain on her side, at least one of the following vegetative symptoms occurs:
  • conjunctival injection and/or lacrimation;
  • nasal congestion and/or rhinorrhea;
  • ptosis and/or miosis.
• D. Efficacy of therapeutic doses of indomethacin.
• E. Not associated with other causes (disorders).

Hemicrania continua usually occurs without remissions, but rare cases with a remitting course have been described. Differential diagnostics must be carried out with chronic tension headache, chronic migraine and chronic cluster headache. A distinctive feature is the effectiveness of indomethacin.

Treatment

Indomethacin has a beneficial effect in most cases.

New daily (initially) persistent headache (4.8)

Description

Daily headache, without remissions from the very beginning (chronization occurs no later than 3 days after the onset of pain). The pain is usually bilateral, pressing or squeezing in nature, mild or moderate in intensity. Photo-, phonophobia or mild nausea are possible.

Diagnostic criteria

• A. Headache lasting more than 3 months, meeting criteria B and B.
• B. Occurs daily, proceeds without remissions from the very beginning, or becomes chronic no later than 3 days after the onset of pain.
• C. At least two of the following pain characteristics:
  • bilateral localization;
  • pressing/squeezing (non-pulsating) character;
  • light to moderate intensity;
  • does not worsen with normal physical activity (eg, walking, climbing stairs).
• D. Both of the following symptoms:
  • no more than one of the following symptoms: photophobia, phonophobia, or mild nausea;
  • absence of moderate or severe nausea and vomiting.
• E. Not associated with other causes (disorders).

New daily persistent headache may be without remissions from the very beginning or very quickly (within a maximum of 3 days) become continuous. This onset of pain is well remembered, and patients usually describe it clearly. The patient's ability to accurately recall how the pain began and its initially chronic nature are the most important criteria for diagnosing new daily persistent headache. If the patient has difficulty describing the initial period of pain, the diagnosis of chronic tension headache should be established. Unlike new daily persistent headache, which occurs in individuals who have not previously complained of cephalgia, chronic tension headache has a history of typical attacks of episodic tension headache.

The symptoms of a new daily persistent headache may also resemble some secondary forms of cephalgia, such as headache with decreased cerebrospinal fluid pressure, post-traumatic cephalgia, and headache caused by infectious lesions (in particular, viral infections). Additional studies are needed to exclude such secondary forms.

Treatment

A new daily persistent headache can have two outcomes. In the first case, the headache can spontaneously end after several weeks without treatment, in the second case (refractory type of course) even intensive treatment (traditional for chronic tension headache and chronic migraine) can be useless and the pain remains chronic for a long time.