Other Less Common Primary Headache Syndromes

Primary Exercise Headache1,82

Description: headache precipitated by any form of exercise in the absence of any intracranial disorder.

ICHD-3 diagnostic criteria:

  • • At least two headache episodes fulfilling criteria В and C
  • • Brought on by and occurring only during or after strenuous physical exercise
  • • Lasting <48 hours
  • • Not better accounted for by another ICHD-3 diagnosis.
  • *On first occurrence of this headache type it is mandatory to exclude subarachnoid hemorrhage, arterial dissection, and reversible cerebral vasoconstriction syndrome (RCVS).

Key clinical points: primary exercise headache is more common in females. The location is typically bilateral but can be one-sided. The quality can be throbbing, pulsatile, and should be nonexplosive. Duration is normally 5 minutes to 48 hours.

Triggers include prolonged physical exertion, and the pain typically starts at the peak of exertion. Associated symptoms outside of head pain are typically lacking although migrainous symptoms can occur in those with a history of migraine. The key is that the age of onset is normally below age 50 years. It can occur in athletes and non-athletes.


Acute: Abortives are not very useful as the induced pain normally alleviates on its own or becomes low level when exercise is stopped. Additionally, patients normally want to prevent pain rather than treat it when it happens.


• Non-Medicinal

=> Avoiding activity is a preventive maneuver of choice but may not be acceptable for the patient

° Improve warm-up routine prior to exertion

• Medicinal

Pretreat Activity

• Indomethacin short-acting formulation: dosing 25 mg to 250 mg has been published—no controlled trials

Suggested strategy: pretreat event by 30-60 minutes with 25-50 mg

• Tripans: pretreat event by 60 minutes—although has not been studied

Primary Couch Headache1,82

ICHD-3 diagnostic criteria:

  • • At least two headache episodes fulfilling criteria B-D
  • • Brought on by and occurring only in association with coughing, straining, and/or other Valsalva maneuver
  • • Sudden onset
  • • Lasting between 1 second and 2 hours

Clinical features: the headache arises immediate after cough, reaches peak almost instantaneously, then subsides over several seconds to a few minutes; sometimes peak pain can last several seconds. Most patients are pain-free in between attacks, but some have pain that lingers for hours after triggered attack. Migrainous-associated symptoms are rare. Attacks are typically bilateral but can be one-sided. For age of onset primary cough headache normally starts after age 50 years versus primary exercise headache which normally begins younger than age 50. If a patient presents with these headaches but in the wrong age range then evaluation for secondary causes is mandatory although in reality primary cough headache needs at least a brain MRI to rule out secondary disorders.


  • • Acute: abortive therapy not utilized secondary to short duration of attacks.
  • • Preventive

=> Indomethacin—most frequent choice. Would suggest extended-release formulation 75 mg to 150 mg per day.

° Acetazolamide (125-500 mg per day).

° Lumbar puncture: typically remove CSF volume until cough headache ceases. This can be a very effective treatment if medicines are not helpful or tolerated.

Primary Headache Associated with Sexual Activity1,82

ICHD-3 diagnostic criteria:

  • • At least two episodes of pain in the head and/or neck fulfilling criteria B-D
  • • Brought on by and occurring only during sexual activity
  • • Either or both of the following:

=> Increasing in intensity with increasing sexual excitement => Abrupt explosive intensity just before or with orgasm

  • • Lasting from 1 minute to 24 hours with severe intensity and/or up to 72 hours with mild intensity
  • *On the first onset of headache with sexual activity, it is mandatory to exclude subarachnoid hemorrhage, intra- and extracranial arterial dissection, and reversible cerebral vasoconstriction syndrome (RCVS). Multiple explosive headaches during sexual activities should be considered as RCVS until proven otherwise.

Clinical presentation: typical headache duration is minutes to a few hours to a day. The intense pain normally occurs in the first 5-15 minutes, and then subsides, although duration may be longer if pain comes at orgasm versus before orgasm. The headache is bilateral in two-thirds and unilateral in one-third of cases and is diffuse or occipitally located.15 Associated symptoms are rare. The course of these headaches over time can be unpredictable, and thus can be regular for periods of time occurring with every coital event and then become sporadic or alleviate all together.


Short-term or mini Prevention—thus treating just prior to activity:

  • • Indomethacin: 25-50 mg dosed 1-2 hours prior to coitus Acute treatment at time of headache:
  • • Indomethacin 25-50 mg and or a triptan but only after ruling out secondary vascular issues for triptan use

New Daily Persistent Headache

ICHD-3 diagnostic criteria:

  • • Persistent headache fulfilling criteria В and C
  • • Distinct and clearly remembered onset, with pain becoming continuous and unremitting within 24 hours
  • • Present for >3 months

This is a unique form of chronic daily headache that is hallmarked by a daily headache from onset (typically in individuals who do not have a prior headache history), and most individuals can name the date or at least the month their headache began.83 There is a remitting form in which the headache can go away up to 2 years after onset and a refractory form in which the headache will continue for years unabated. It is recognized as one of the most treatment-refractory of all headache subtypes. Known triggers include post-infection, post-stressful life event, and post-surgical procedure, although 50% plus cannot recognize a triggering event. Clinical characteristics include female predominance noted in almost all studies with a younger age of onset in women. Typical age of onset is mid- to late teens to early twenties in most, but this also depends on triggering event (for example post-surgical is an older age class, 50-60s). Location is bilateral in most and pain intensity is typically moderate to severe. Pain is constant. Migrainous-associated symptoms are common in NDPH, but this is not migraine. Pathogenesis is unknown, but it appears NDPH is not one entity but multiple disparate conditions that can present as a daily headache from onset.84 Possible etiologies include changes in CSF pressure high or low, hyperimmune response to infectious agents, typically viruses, and possible cerebral artery vasospasm if the first headache is a thunderclap headache (pain reaches maximum intensity without latency). Finally the majority of these patients have an underlying hypermobility disorder and thus this may play a role in headache pathogenesis; the main theory is that cervical hypermobility leads to upper cervical facet irritation and chronic daily headache. At present there are no known specific treatments for this syndrome. Possibly effective treatments include doxycycline, mexiletine, high-dose IV corticosteroids, gabapentin, and topiramate.83

Secondary Disorders Mimicking NDPH

All patients with a daily headache from onset must be ruled out for secondary underlying conditions. The most frequent mimics are cerebral vein thrombosis and a spinal fluid leak. Other possible causes include elevated CSF pressure, nasal contact syndrome, sphenoid sinusitis, and neoplasm. If NDPH is one-sided/side-fixed, the differential diagnosis includes cerebral vein thrombosis or vein occlusive syndrome, sphenoid sinus lesion (sinusitis, fungal, mass), spinal fluid leak, cervicogenic- based headache, nasal contact syndrome, cavernous sinus lesion, aneurysm, carotid dissection, and giant cell arteritis. Primary headache syndromes must be considered including hemicrania continua and trochlear headache.

Key Points in Regard to NDPH

  • • Always rule out a secondary cause of the daily headache.
  • • Try to determine a triggering event for NDPH if possible as that may help to establish an underlying pathogenesis theory and treatment (post-infectious, post-surgical, post-stressful life event).
  • • Always ask about the first ever headache and temporal profile of that headache onset. There is a distinct form of NDPH that starts with a thunderclap headache, and in that setting the evaluation is different (include arterial studies with imaging), and one may consider nimodipine as a preventive choice if no secondary cause is noted.
  • • Utilize the Trendelenburg test to help with diagnosis. If a patient improves in head down tilt position, consider evaluation for a CSF leak, or if they rapidly worsen then consider utilizing a medication that lowers CSF pressure/volume.
  • • Evaluate for cervical hypermobility as, if there is no triggering event and significant cervical irritation on exam, the predisposing cause of the headaches is probably related to hypermobility issues.
  • • Be aggressive with therapy up front, especially if you meet an individual within 1 year of headache onset. Treating with infusion therapy or inpatient therapy with intravenous medications (even with standard migraine protocols) may help break the cycle. This is less effective years into the syndrome.
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