Little is known about the diseases classified as rare primary headache syndromes. This article summarizes the clinical presentation, pathophysiology, epidemiology, and treatment of rare primary headache syndromes. Increasing public awareness of the different types of rare primary headache syndromes will allow for better research as well as clinical management. The International Headache Society has also recently updated its classification criteria for headache, adding some disease entities and classifying others under other disease classifications. This article concludes that neuroimaging should be performed in all patients suspected of having rare primary headaches to rule out secondary headache etiologies. Indomethacin treatment may be effective for some disorders, but larger randomized controlled studies are needed.
INTRODUCTION: Headache disorders are considered rare headaches when their prevalence in the general population is less than 1%. In Europe, rare headache is defined as having no more than 10,000 patients. For most rare headache disorders, the true prevalence is still unknown or can only be determined by literature case reports from tertiary headache centers or small cohort studies.
International classification of headache disorders.
1. primary cough headache.
2. primary motor headache.
3. primary headache associated with sexual activity.
4. primary thunderclap-like headache.
5. cold irritation headache.
6. external pressure headache.
7. primary stabbing headache.
8. coin-shaped headache.
9. Sleep headache.
10. new-onset daily persistent headache.
11, Cranial surface inverse linear radioactive headache A new beta version of ICHD-3 was published in 2013 and introduced for clinical testing.
It is planned to be included in the International Classification of Diseases (ICD-11) in the future. Some of these diseases are classified as primary headache or have been reclassified. For example, coin-shaped headache, from the Appendix section of the original ICHD-2, was moved up to the classification of primary headache, as more and more evidence suggests that it should be treated as such. Cold irritation headache and external pressure headache were separated from Chapter 13, and continuous hemithoracic pain was eventually classified as trigeminal spontaneous headache (TAC).
Most headaches may also be secondary headaches and need to be considered in both routine outpatient and emergency care. In most cases, neuroimaging must be performed and secondary/symptomatic headache symptoms must be excluded before a definitive diagnosis and classification of primary headache can be made. Primary cough headache Primary cough headache is characterized by an attack of headache caused by or associated with coughing, straining, and/or Valsalva maneuvers. The headache usually comes on suddenly and lasts 1-2 hours. The new classification extends the possible duration from 30 minutes to 2 hours. It is mainly people over 40 years of age who are more commonly affected. As many as 40% of patients with cough headache are symptomatic. Therefore, neuroimaging should be performed in all patients to rule out intracranial lesions or abnormalities, such as Arnold-Chiari malformation type I, tumors of the middle or posterior cranial fossa. One study attempted to differentiate between primary and secondary cough headache using a modified Valsava test (exhalation into a standard aneroid sphygmomanometer with a connecting tube). The authors studied 16 patients with cough headache not associated with other neurological signs and symptoms. Eleven of these patients presented with pathologic findings on the Valsava test, and all but one of them had confirmed posterior cranial fossa disease that resolved with surgical treatment. These results suggest that a modified Valsava test may be useful in distinguishing primary from secondary cough headaches. Despite this, neuroimaging is mandatory for patients with cough headache. Indomethacin therapy may be an effective treatment with the possibility of symptom relief and the need for intermittent treatment pauses. Primary Exercise Headache Primary exercise headache (PEH) is a headache that occurs after or simultaneously with intense physical activity and usually lasts up to 48 hours. Prevalence rates of up to 30% have been reported in the literature. Indomethacin may be an effective prophylactic medication and, in addition, excessive exercise leading to headache attacks needs to be avoided. Primary headache associated with sexual activity is, as the word implies, a headache that occurs during sexual activity. The level of headache may increase with increased sexual arousal. It may also occur as an intense explosive headache that occurs before or simultaneously with orgasm. Severe headaches can last from 1 minute to 24 hours, with mild headaches or can last longer.
The ICHD-2 also distinguishes between two subtypes, pre-orgasmic headache and orgasmic headache. However, clinical studies were unable to distinguish between the two subtypes, so the ICHD-3 beta version combined them together again. The underlying pathophysiological mechanisms of this type of headache are still unknown. One study showed that patients with primary headache associated with sexual activity were more likely to have abnormalities of cerebral venous circulation. 12 of 19 patients had venous stenosis, compared with normal in controls. Similar results were observed in patients with primary cough headache and primary exertional headache, suggesting that a common pathway may exist in these disorders leading to the onset of headache after strenuous activity. Propranolol and indomethacin are effective prophylactic medications that can be administered prior to sexual activity with a tretinoin. Primary lightning-like headache Primary lightning-like headache (PTH) is characterized by a very intense headache that peaks within seconds and can resemble the presentation of a ruptured cerebral aneurysm. Therefore, secondary causes (imaging including cerebrovascular imaging and cerebrospinal fluid examination) must be excluded. The headache peaks within 1 minute and the duration of the headache must exceed 5 minutes in duration. The new criteria extend the ICHD-2 criteria, as the old criteria specified headaches lasting between 1 hour and 10 days.
However, it is now considered that headaches of longer or shorter duration may also be PTH. ICHD-2 also excludes patients with recurrent thunderclap-like headaches. The new classification criteria put this limitation aside. However, it remains controversial whether it is a form of PTH or a condition for which PTH is an excluded diagnosis. Therefore, ICHD-3 beta suggests that it should not be used as a diagnosis of likely PTH. In most patients, lightning-like headaches are self-limiting and therefore do not require additional treatment. Cold irritation headache The ICHD-3 beta distinguishes between two subtypes of cold irritation headache.
The first subtype is headache due to external cold stimulation (HEACS). The headache occurs only after or simultaneously with external cold stimulation of the head. The pain is relieved within 30 minutes after removal of the cold stimulus. The second subtype is headache due to ingestion or inhalation of a cold stimulus (HICS), formerly also called ice cream headache. The headache attack occurs after or simultaneously with cold stimulation of the palate or posterior pharyngeal wall when cold food is ingested or cold air is inhaled. Pain relief occurs within 10 minutes after removal of the cold stimulus. An observational study investigated the response to experimental cold stimulation in 414 volunteers, using the ICHD-2 for HICS and the diagnostic criteria for ice cream headache. During cold stimulation of the palate, 37% of subjects reported headaches, only half of which met the ICHD-2 diagnostic criteria. Most subjects complained of frontal or temporal throbbing headaches. Migraineurs were more likely to have ice cream headaches than those without a history of headaches. Primary stabbing headache Primary stabbing headache (PSH) is characterized by a spontaneous single stabbing pain or a continuous stabbing attack lasting several seconds. The frequency of attacks is irregular, ranging from one attack to several attacks per day. The pain is located mainly in the distribution of the first branch of the trigeminal nerve, namely the orbital, temporal or parietal lobes.
PSH is a rare headache syndrome, the prevalence of which is unknown.Ramon et al. analyzed 100 headache patients with strictly unilateral attacks in a tertiary headache center, and only one of the 100 patients had PSH.The underlying pathophysiological mechanisms of this type of headache are also unknown. A study investigated the possible correlation between cerebral venous return disorders and PSH. The prevalence of dural venous sinus stenosis was higher in patients diagnosed with other types of headache, such as migraine, tension-type headache, cranial hypertension without optic papilledema (ss-IHWOP), exertional, cough and sexual activity-related headache. A retrospective analysis of eight patients with symptomatic PSH showed that in all patients, MRV examination revealed venous sinus stenosis, and the authors hypothesized that there may be a correlation between PSH and venous sinus stenosis and that there may be undiagnosed ss-IHWOP involved in the development of PSH. Another study confirmed the results of the previous study, with venous stenosis present in 5 of 7 patients, but no stenosis was demonstrated in controls. However, these results need to be confirmed in a larger sample size study. In addition, further confirmation of the causal correlation between the two is needed. Clinical treatment with indomethacin is more effective. Coin-shaped headache Coin-shaped headache is characterized by continuous or intermittent headaches within a single scalp region. A new review has reported more than 250 cases of this type of headache. The pain is mainly located in the parietal lobe and is mild to moderate in intensity. A worsening of the pain is usually observed. Additional sensory symptoms, such as abnormal sensation or tenderness to touch, are also present in the affected area. Unifocal, bifocal or multifocal coin-shaped headaches have been described in reviews. Because it is a relatively rare disorder, there are no controlled studies to date. Treatment with gabapentin, tricyclic antidepressants and botulinum toxin may be effective. Improvement in pain in a single patient treated with neurotoxin has also been reported. Neuroimaging is required in all patients presenting with coin-shaped headaches in order to be able to rule out secondary causes especially pituitary lesions. A case report describes a case of a hematoma with scalp calcification presenting as a secondary coin-shaped headache. The pain often resolved after surgical intervention. However, in contrast, coin-shaped headaches may also occur postoperatively. In a patient with a pituitary prolactinoma who underwent pterygoid sinusectomy, intermittent coin-shaped headaches developed and improved after treatment with gabapentin. Sleep headache Sleep headache is characterized by headache attacks strictly associated with sleep. Because of its low prevalence, diagnostic criteria based on small case studies or case reports remain controversial, but have also been increasingly reported recently. Therefore new diagnostic criteria also attempt to reflect these new data. Headaches that occur only during sleep, with episodes lasting for 3 months, more than 10 days per month, and each episode lasting at least 15 minutes to 4 hours are diagnosed as sleep headaches. The headache must not be accompanied by autonomic symptoms or fidgeting. In addition, the age of the patient is no longer required in the new diagnostic criteria. However, the majority of patients are older than 50 years of age at onset. The frequency of headache was reduced from at least 15 times per month to 10 times per month. More importantly, under the new classification criteria, sleep headache allows for the presence of some of the features that accompany migraine, such as nausea, photophobia, and fear of sound. A recent literature review that reviewed all reported cases of sleep headache suggests that there may be clinical features that do not meet the new diagnostic criteria. Some patients report headache episodes that can be longer, up to 10 h. Almost all patients have some sort of motor symptoms accompanying the headache episode. Recommended diagnostic tests include cranial MRI and 24-hour blood pressure monitoring to rule out symptomatic sleep headaches. In terms of efficacy and side effects, caffeine is probably the best treatment and prevention.
New daily persistent headache The diagnostic criteria for new daily persistent headache (NDPH) have been significantly broadened in ICHD-3 beta. The ICHD-2 criteria are more restrictive in that migraine-like features, such as nausea and vomiting, and exacerbation from daily activities are not allowed. Usually, NDPH occurs in patients without any prior history of headache, but it can also be seen in patients with migraine or tension-type headache. The underlying pathophysiologic mechanisms of NDPH remain unclear. Standard recommendations suggest that NDPH may not in fact be a single or consistent headache and can be divided into several subtypes. rozen et al. reported a case of NDPH in a patient with a 13-month-old or thunderclap-like headache. The headache was associated with persistent computational deficits and neuroimaging ruled out secondary headache as the cause. Interestingly, the headache and neurological symptoms disappeared after treatment with nimodipine. The authors suggest that this may be a subtype of NDPH, which is due to cerebral artery spasm caused by a rapid increase in tumor necrosis factor-alpha (TNF-a) levels in the cerebrospinal fluid. There are no controlled studies of NDPH to date. Overdoses of headache-causing drugs should be avoided. Craniofacial retrograde linear radioactive headache was first described in the literature in 2008 and is characterized by brief paroxysmal attacks of moderate to severe pain, usually starting in the posterior scalp and rapidly radiating to the forehead, eyes and nose, following a linear or Z-shaped trajectory. In some patients, the symptoms start in the forehead. The starting and ending areas are in different innervation zones. Most of the pain is unilateral, but there may be a lateral shift. The frequency of attacks varies, from a few times per year to several times per day. The duration of the attack is usually 1-15 seconds. Seizures may be triggered by touching an affected area, while the affected area is normal during the interictal period. Other triggers include neck or eye movements, coughing, tension, Valsalva movements, and emotional stress. A total of 66 such patients have been described in the literature to date. Females appear to be more common (2:1) and the age of onset ranges from 23-84 years. Attempts at treatment with gabapentin (900 -1200 mg/d), pregabalin (50 – 150 mg/d), lamotrigine (100 mg/d), levetiracetam (500 mg/d) and indomethacin (75 mg/d) appear to be effective. Invasive treatments include greater occipital nerve (GON) closure (2 cm3 5% bupivacaine alone, or concomitantly with trimethoprim) and GON and supraorbital nerve (SON) closure. Summary Rare primary headaches are a heterogeneous group of headache disease syndromes. The underlying pathophysiology of all types of headache is poorly understood. Recommended treatments are based on case reports or small series, and there is a lack of controlled studies due to low patient disease. All patients with headache should have appropriate neuroimaging to rule out secondary causes, and in some cases lumbar puncture is indicated. Learning points All patients with clinical presentation of rare primary headache require neuroimaging to rule out a symptomatic headache etiology. There is a lack of controlled studies for all rare primary headaches, and treatment recommendations are mainly genetic with single case reports and small series. Rare primary headache should be diagnosed using the new ICHD-3 beta diagnostic criteria to further validate or modify the new criteria.