Clues in the Differential Diagnosis of Primary vs Secondary Cough,Exercise, and Sexual Headaches

Activity‐related headaches can be provoked by Valsalva maneuvers (“cough headache”), prolonged exercise (“exertional headache”) and sexual excitation (“sexual headache”). These entities are a challenging diagnostic problem as can be primary or secondary and the etiologies for secondary cases differ depending on the headache type. In this paper we review the clinical clues which help us in the differential diagnosis of patients consulting due to activity‐related headaches. Cough headache is the most common in terms of consultation. Primary cough headache should be suspected in patients older than 50 years, if pain does not predominate in the occipital area, if pain lasts seconds, when there are no other symptoms/signs and if indomethacin relieves the headache attacks. Almost half of cough headaches are secondary, usually to a Chiari type I malformation. Secondary cough headache should be suspected in young people, when pain is occipital and lasts longer than one minute, and especially if there are other symptoms/signs and if there is no response to indomethacin. Every patient with cough headache needs cranio‐cervical MRI. Primary exercise/sexual headaches are more common than secondary, which should be suspected in women especially with one episode, when there are other symptoms/signs, in people older than 40 and if the headache lasts longer than 24 hours. These patients must have quickly a CT and then brain MRI with MRA or an angioCT to exclude space‐occupying lesions or subarachnoid hemorrhage.     

Cough headache: a study of 83 consecutive patients

To delineate the differences in clinical characteristics and evaluate the outcome between primary and secondary cough headache, 83 consecutive patients (59M/24F, mean age 61.5 ± 17.7 years) with cough headache (1.2%) out of 7100 patients in a headache clinic were studied. All of them received brain imaging studies. Most did not have relevant brain lesions ( n  = 74, 89.2%, primary group) except for nine patients (10.8%, the secondary group). Most of the intracranial lesions were located in the posterior fossa ( n  = 6, 67%), including only two patients with Chiari malformation. The primary group had a higher response rate to indomethacin than the secondary group (72.7% vs. 37.5 %, P  = 0.046). Mild to moderate headache intensity and age onset < 50 years predicted a favourable response. At a mean follow-up of 51.4 months, 83.9% of patients with primary cough headache completely remitted. Inconsistent with the proposed International Classification of Headache Disorders, 2nd edn criteria, 10.8% of patients with primary cough headache had headache duration of > 30 min. Clinical features, neurological examinations and drug response could not safely differentiate primary from secondary cough headache. Neuroimaging studies are required in each patient.     

Cough, exertional, and other miscellaneous headaches

We have discussed several miscellaneous headache disorders not associated with structural brain disease. The first group included those headaches provoked by "exertional" triggers in various forms. These include benign cough headache, BEH, and headache associated with sexual activity. The IHS diagnostic criteria were discussed. Benign exertional headache and cough headache were discussed together because of their substantial similarities. In general, BEH is characterized by severe, short-lived pain after coughing, sneezing, lifting a burden, sexual activity, or other similar brief effort. Structural disease of the brain or skull was the most important differential diagnosis for these disorders, with posterior fossa mass lesions being identified as the most common organic etiology. Magnetic resonance imaging with special attention to the posterior fossa and foramen magnum is the preferred method for evaluating these patients. Indomethacin is the treatment of choice. The headache associated with sexual activity is dull in the early phases of sexual excitement and becomes intense at orgasm. This headache is unpredictable in occurrence. Like BEH, the headache associated with sexual activity can be a manifestation of structural disease. Subarachnoid hemorrhage must be excluded, by CT scanning and CSF examination, in patients with the sexual headache. Benign headache associated with sexual activity has been successfully treated with indomethacin and beta-blockers. The second miscellaneous group of headache disorders includes those provoked by eating something cold or food additives, and by environmental stimuli. Idiopathic stabbing headache does not have a known trigger and appears frequently in migraineurs. Its occurrence may also herald the termination of an attack of cluster headache. Indomethacin treatment provides significant relief. Three headaches triggered by substances that are eaten were reviewed: ingestion of a cold stimulus, nitrate/nitrite-induced headache, and MSG-induced headache. For the most part, avoidance of these stimuli can prevent the associated headache. Lastly, we reviewed headache provoked by high altitude and hypoxia. The headache is part of the syndrome of AMS during its early or benign stage and the later malignant stage of HACE. The pain can be exacerbated by exercise. The best treatment is prevention via slow ascent and avoidance of respiratory depressants. Acetazolamide and dexamethasone have proved useful in preventing this syndrome.     

Chiari-associated exertional,cough, and sneeze headache responsive to medical therapy

Benign exertional headache is coded as a separate entity within the International Headache Society's classification system, but the pathophysiological mechanisms underlying this clinical headache subtype are unknown and possibly are similar to those generating migraine. Coexistence of migraine and benign exertional headache in the same patient is not unusual, and antimigraine pharmacologic treatments are often effective in both headache types. Regardless, optimal management mandates that the clinician exclude any intracranial or systemic disease that could mimic "primary" exertional headache. The same holds for primary headaches induced by coughing or sneezing; congenital malformations or neoplasms, particularly within the posterior fossa, are not rare in these patients. The neurologic examination may not be sufficiently sensitive to detect the offending lesion. We describe a patient with migraine without aura and exertional secondary headache due to Chiari malformation type I whose headaches responded to treatment with propranolol and indomethacin.     

Primary cough headache

Primary cough headache is defined as head pain brought on by coughing or other Valsalva maneuvers, but not by prolonged physical exercise, in the absence of any intracranial disorder. Primary cough headache is considered to be a rare condition, accounting for 0.4% of all headaches consulting our Neurology Department. Its pathophysiology remains a mystery. Primary cough headache is a sudden-onset headache that usually lasts from 1 second to 30 minutes, tends to be bilateral and posterior, does not begin earlier than the fifth decade of life, is more frequent in men, is not accompanied by other neurologic manifestations, and responds to indomethacin. These clinical characteristics allow its differential diagnosis from other entities, even though a craniocervical magnetic resonance imaging study is mandatory to rule out posterior fossa lesions, especially Chiari type-I malformation.     

Headache in Behçet's disease

The aim of this study was to investigate the characteristics of headache in patients with Beh?et's disease (BD) seeking neurological consultation. Consecutive patients with BD seen within 1 year at the neuro-Beh?et's out-patient clinic were studied using an electronic database according to the classification criteria of the International Headache Society. During a 12-month period 118 BD patients were admitted, and 98 had headaches. Migraine was the most common type of primary headache diagnosed in 45 patients (46.4%) in the whole headache group, followed by tension-type headache (TTH) in 26 cases (26.8%). Thirty-seven patients had secondary headaches mainly due to cerebral venous thrombosis or parenchymal neurological involvement of BD. Of these patients, 15 had both primary and secondary headaches. The primary headaches of 13 patients were exacerbated with systemic BD flare-ups, and four patients had migraine attacks triggered only by systemic BD activation which showed a good response to the treatment of systemic inflammation. The majority of the headaches of patients with BD do not indicate any neurological involvement; they are usually due to migraine or TTH. In a minority of patients, migraine associated with systemic inflammation of BD is notable.     

The “Other” Headaches: Primary Cough,Exertion, Sex,and Primary Stabbing Headaches

Primary cough headache, primary exertional headache, primary sexual headache, and idiopathic stabbing headache are included in “Other Primary Headaches” (Group 4) in the International Classification of Headache Disorders, 2nd edition (ICHD-II). Headaches provoked by cough, exertion, and sex have different age distributions, but they do share some clinical and pathogenic characteristics. The triggering activities frequently involve Valsalva-like maneuvers, which may explain part of the pathogenesis. Primary stabbing headache is common and characterized by ultra-short stabbing headaches. All these headache disorders respond well to indomethacin, and they are commonly comorbid with migraine except for primary cough headache. Of note, some patients with sexual headache had reversible cerebral vasoconstriction syndromes. Recent large-scaled studies have revealed that the ICHD-II criteria of these four headache disorders cannot be completely fulfilled. Further revisions for the ICHD-II criteria are required based on these results of the evidence-based studies.     

Headache in acute stroke. A prospective study in the first 8 days

We aimed to describe and classify headaches associated with acute stroke, by interviewing patients consecutively admitted to a stroke unit using a validated headache questionnaire and the International Classification of Headache Disorders of the International Headache Society (IHS). One hundred and twenty-four patients (61% ischaemic and 39% haemorrhagic stroke) reported headache. Headaches started mostly on the day of stroke, were more often continuous, pressure-type, bilateral and located in the anterior region, were increased by movement and by cough and lasted for a mean of 3.8 days. Tension-type was the most frequent type of headache. Eleven per cent of headaches could not be classified using the criteria of the IHS. Previous primary headache was documented in 71 patients. The presence of nausea/vomiting due to acute stroke can confound headache classification using the IHS criteria. In up to half of the patients, headache seems to be a reactivation of previous primary headache.     

Headache in the elderly: a clinical study

Abstract Although the prevalence of headache in the elderly is relevant, until now few studies have been conducted in patients over the age of 65 years. We analyzed the clinical charts of 4,417 consecutive patients referred to our Headache Centre from 1995 to 2002. There were 282 patients over 65 years of age at the first visit, corresponding to 6.4% of the study population. Primary headaches were diagnosed in 81.6% of the cases, while secondary headaches and non-classifiable headaches represented, respectively, 14.9% and 3.5% of the cases. Among primary headaches, the prevalence was almost the same for migraine without aura (27.8%), transformed migraine (26.1%) and chronic tension- type headache (25.7%). The most frequent secondary headaches were trigeminal neuralgia and headache associated with cervical spine disorder.     

Chronic daily headache in a paediatric headache centre

The prevalence and the clinical features of chronic daily headache (CDH) were studied in 968 children and adolescents observed during a period of one year in the Headache Centre of the Anna Meyer Paediatric Hospital of Florence. Nine hundred and fortyfour patients (97.52%) had primary headache according to ICHD-II, 24 subjects had secondary headache and 56 patients had CDH (5.93% of primary headaches). The mean age of subjects with CDH was higher than general (13.5 vs. 11.5 years), with a female preponderance (69.6% vs. 30.4%). According to the ICHD-II, headaches were classified as chronic migraine in 10 patients (1.5.2 ICHD-II), chronic tension-type headache in 36 (2.3 ICHD-II), new daily persistent headache in 8 (4.8 ICHD-II) and 2 patients reported mixed pattern (chronic migraine+chronic tension type headache). Medication overuse was not implicated in our patients.     

Evaluation of acute headaches in adults

Classifying headaches as primary (migraine, tension-type or cluster) or secondary can facilitate evaluation and management A detailed headache history helps to distinguish among the primary headache disorders. "Red flags" for secondary disorders include sudden onset of headache, onset of headache after 50 years of age, increased frequency or severity of headache, new onset of headache with an underlying medical condition, headache with concomitant systemic illness, focal neurologic signs or symptoms, papilledema and headache subsequent to head trauma. A thorough neurologic examination should be performed, with abnormal findings warranting neuroimaging to rule out intracranial pathology. The preferred imaging modality to rule out hemorrhage is noncontrast computed tomographic (CT) scanning followed by lumbar puncture if the CT scan is normal. Magnetic resonance imaging (MRI) is more expensive than CT scanning and less widely available; however, MRI reveals more detail and is necessary for imaging the posterior fossa. Cerebrospinal fluid (CSF) analysis can help to confirm or rule out hemorrhage, infection, tumor and disorders related to CSF hypertension or hypotension. Referral is appropriate for patients with headaches that are difficult to diagnose, or that worsen or fail to respond to management     

Cluster headache as a manifestation of intracranial inflammatory myofibroblastic tumour: a case report with pathophysiological considerations

We report a patient with a secondary form of chronic cluster headache, caused by an intracranial presentation of inflammatory myofibroblastic pseudotumour located in the posterior fossa, with total remission of the pain after resection. The headaches were resistant to many of the usual treatments for cluster headache. The patient had two normal computed tomography scans and one normal magnetic resonance imaging of the head before the additional diagnosis of brain tumour was made. This is an unusual cause of cluster headache with intracranial mass, with an unexpected clinical presentation, a rare triggering manoeuvre, unusual pathology and successful treatment. This patient probably had the hypothalamic biological predisposition to cluster headache and, when a small mass disturbed pain-sensitive structures in the posterior fossa, it excited the trigeminovascular system via posterior fossa trigeminal and upper cervical afferents, and triggered the pathophysiological processes that resulted in a secondary form of chronic cluster headache.     

Primary headaches in HIV-infected patients

Headache in patients with human immunodeficiency virus (HIV) infection may indicate life-threatening illnesses such as opportunistic infections or neoplasms. Alternatively, such patients may develop benign self-limiting headaches. Hence, defining the various types of headache in these patients is essential for proper management. This study describes the clinical characteristics of primary headaches occurring in a group of HIV-infected patients. Of 115 patients seen from 1990 to 1996, 44 (38%) had headaches. Primary headaches were present in 29 (66%) patients and secondary causes were identified in 15 (34%). Among those with primary headaches, migraine occurred in 22 (76%), tension-type headache in 4 (14%), and cluster headache in 3 (10%) patients. Half of those with migraine (n=ll), 1 patient with tension-type headache, and 1 patient with cluster headache developed chronic daily headaches which were severe and refractory to conventional headache or antiretroviral therapy. We conclude that primary headaches in patients with HIV infection are: (1) the commonest type of headache; (2) may present for the first time in individuals with severe immunosuppression; (3) usually bear no relationship to antiretroviral drug therapy; (4) polypharmacy, depression, anxiety, and insomnia are commonly associated comorbidities; (5) frequently do not respond to conventional management and carry a poor prognosis; and (6) do not require neuroradiological and/or cerebrospinal fluid evaluations.     

Etiology and distribution of headaches in two Brazilian primary care units

OBJECTIVES: To determine (a) which patients seek primary care services with a complaint of headache, (b) the percentages of the various types of headache in this population, and (c) the impact of the care provided to these patients on the basic health care network. BACKGROUND: Headache is one of the most frequent symptoms reported in medical practice, resulting in significant medical services costs and loss of patient productivity, as well as reduced quality of life. METHODS: A prospective study was conducted in two towns (Ribeir?o Preto and S?o Carlos) in the State of S?o Paulo, Brazil. The participants in the study consisted of 6006 patients (52.4% women) with highly varied acute symptoms. The patients ranged in age from 14 to 98 years. RESULTS: Headache as the main complaint was reported by 561 (9.3%) of the patients considered, with 312 (55.6%) of those patients presenting with primary headache, 221 (39.4%) with headaches secondary to systemic disorders, and 28 (5.0%) with headaches secondary to neurological disorders. Migraine, the most prevalent primary headache, accounted for 45.1% of patients reporting headache as the single symptom. The most frequent etiologies of headaches secondary to systemic disorders were fever, acute hypertension, and sinusitis. The most frequent headaches secondary to neurological disorders were posttraumatic headaches, headaches secondary to cervical disease, and expansive intracranial processes. Of the 26 cases of drug abuse, 20 were secondary to alcohol (hangover). Headaches secondary to systemic disorders were more frequent in the extreme age ranges. CONCLUSIONS: Headache is a very frequent symptom among patients seen at primary health care units and should be considered a public health problem. The dissemination of the diagnostic criteria of the International Headache Society among primary health care physicians is urgently needed in order to avoid the repeated return of patients or their referral to more differentiated emergency units, which overburden an already insufficient health care network.     

Prevalence and diagnosis of migraine in patients consulting their physician with a complaint of headache: data from the Landmark Study

CONTEXT: Headache experts have suggested that to improve the recognition of migraine, patients with a stable pattern of episodic, disabling headache and a normal physical exam should be considered to have migraine in the absence of contradictory evidence. The premise upon which this approach is based-that is, that episodic, recurrent primary headache in the clinic is usually migraine-has not been evaluated in prospective clinical studies. OBJECTIVES: To (1) evaluate the diagnoses of patients consulting their physician with primary episodic headache and (2) compare clinic diagnoses and patient self-diagnoses with International Headache Society (IHS) headache diagnoses assigned on the basis of longitudinal data from patient diaries. DESIGN: Prospective, open-label study. During the screening visit, patients self-reported a headache diagnosis and then were assigned a headache diagnosis by their physician following his or her customary practice. Patients with a new physician diagnosis of migraine or nonmigraine primary headache were given diaries to record headache symptoms for up to 3 months or 6 attacks. Members of an expert panel, unaware of the clinic diagnosis, used diary data to assign a headache diagnosis to each attack and to each patient. SETTING: One hundred twenty-eight (128) practices in 15 countries including the United States. PATIENTS: A total of 1203 male and female patients between 18 and 65 years of age who consulted their physician with headache as a primary or secondary complaint. RESULTS: Overall, 94% of patients with a physician diagnosis of nonmigraine primary headache or a new clinic diagnosis of migraine had IHS-defined migraine (76%) or probable migraine (migrainous) (18%) headache on the basis of longitudinal diary data. A new clinic diagnosis of migraine was almost always correct: 98% of patients with a clinic diagnosis of migraine had IHS-defined migraine (87% of patients) or probable migraine (11% of patients) headache on the basis of longitudinal diary data. On the other hand, review of diaries of patients with a clinic diagnosis of nonmigraine revealed that 82% of these patients had IHS-defined migraine (48%) or probable migraine (34%) headache. Altogether, one in four patients (25%) with IHS-defined migraine according to longitudinal diary data did not receive a clinic diagnosis of migraine. CONCLUSIONS: These findings support the diagnostic approach of considering episodic, disabling primary headaches with an otherwise normal physical exam to be migraine in the absence of contradictory evidence. If in doubt of diagnosis or when assigning a nonmigraine diagnosis, strong consideration should be given to the use of a diary to confirm primary headache diagnosis.     

Primary cough headache is associated with posterior fossa crowdedness: a morphometric MRI study

The aetiology of primary cough headache (PCH) is obscure. The aim of this study was to investigate the magnetic resonance (MR) morphometric characteristics of the posterior cranial fossa (PCF) in patients with PCH. Eighteen consecutive patients with PCH (14M/4F, mean age 75.1 +/- 6.0 years) and 18 sex- and age-matched control subjects were recruited for study. Based on the midline sagittal MR images, parameters indicating posterior fossa crowdedness were measured. Compared with controls, patients with PCH had a similar size of hindbrain tissue area but a significantly smaller PCF area, resulting in a higher mean hindbrain/PCF ratio (0.78 +/- 0.04 vs. 0.73 +/- 0.06, P = 0.005). In addition, these patients also had a lower position of the cerebellar tonsillar tip, a shorter clivus length and shorter distances from the clivus to the mid-pons and from the basion to the medulla than the control group. Patients with PCH were associated with a more crowded PCF, which might be a contributing factor for the pathogenesis of this headache syndrome.     

Indicators of a major depressive episode in primary care patients with a chief complaint of headache

OBJECTIVE: To identify the indicators of major depressive episode (MDE) in primary care patients with a chief complaint of headache. BACKGROUND: MDE is very frequent among headache patients in primary care. However, primary care physicians often fail to recognize the coexistence of MDE. METHODS: A total of 177 consecutive new adult patients who visited 19 primary care clinics from January 2002 to December 2002 with a chief complaint of headache were enrolled in the study. All subjects completed a self-report questionnaire that included questions regarding the duration and severity of their headaches, changes in headache severity, and other symptoms. The questionnaire also identified distressed high utilizers (patients who consulted different doctors for the same episode of an illness, without being referred). MDE was diagnosed using a module of the mini international neuropsychiatric interview (MINI). To identify potential indicators of depression, both univariate analysis and multiple logistic regression analysis were performed. RESULTS: Forty-five of 177 patients (25.4%) fulfilled the diagnostic criteria for MDE. Univariate analysis revealed that severe headache, longer duration of headaches, multiple somatic symptoms, and being a distressed high utilizer were associated with MDE. Multiple logistic regression analysis revealed that patients with headaches lasting 6 months or longer and those with multiple somatic symptoms were more likely to be suffering from MDE (adjusted odds ratios: 3.1, 95% CI: 1.7-10.6; and 3.9, 95% CI: 1.2-8.1, respectively). CONCLUSIONS: MDE is highly prevalent in headache patients visiting a primary care setting. Multiple somatic symptoms and longer duration (> or =6 months) of headaches are particularly useful indicators of MDE.     

A spectrum of exertional headaches

Headaches that have an explosive onset with exercise, including sexual activity, generally are benign in origin. A subarachnoid hemorrhage, a mass lesion in the brain, or an anomaly of the posterior fossa must be considered, however. The mechanisms that produce sexually induced or cough headaches of abrupt onset are unknown. It is known, however, that a rapid increase in intrathoracic pressure suddenly reduces right atrial pressure and presumably decreases venous sinus drainage from the brain. This situation results in a transient increase in intracranial pressure. Jaw pain that occurs with chewing often is considered to be TMJ dysfunction when arthritic in quality and if subluxations of the jaw can be shown on the physical examination. Giant cell arteritis and common or external carotid artery occlusive disease should be considered when the pain is ischemic in quality. An anginal equivalent is another possibility. Headaches that worsen with vigorous exercise are commonly migrainous. When their onset is apoplectic with exertion (particularly exertion against a closed glottis), the most likely diagnoses are increased intracranial pressure, a posterior fossa abnormality, or benign exertional headaches. Most cardiac induced headaches, but not all, are of a more gradual onset. If there are significant risk factors for coronary artery disease, an exercise stress test is appropriate. A therapeutic trial of nitroglycerin may help to establish a diagnosis if it improves the headache. Using antimigraine drugs as a diagnostic test is inappropriate because triptans and ergots are contraindicated in the presence of coronary artery disease, and a positive response is not diagnostic of migraine.     

Management of primary chronic headache in the general population: the Akershus study of chronic headache

Primary chronic headaches cause more disability and necessitate high utilisation of health care. Our knowledge is based on selected populations, while information from the general population is largely lacking. An age and gender-stratified cross-sectional epidemiological survey included 30,000 persons aged 30–44 years. Respondents with self-reported chronic headache were interviewed by physicians. The International Classification of Headache Disorders was used. Of all primary chronic headache sufferers, 80% had consulted their general practitioner (GP), of these 19% had also consulted a neurologist and 4% had been hospitalised. Co-occurrence of migraine increased the probability of contact with a physician. A high Severity of Dependence Scale score increased the probability for contact with a physician. Complementary and alternative medicine (CAM) was used by 62%, most often physiotherapy, acupuncture and chiropractic. Contact with a physician increased the probability of use of CAM. Acute headache medications were taken by 87%, while only 3% used prophylactic medication. GPs manage the majority of those with primary chronic headache, 1/5 never consults a physician for their headache, while approximately 1/5 is referred to a neurologist or hospitalised. Acute headache medication was frequently overused, while prophylactic medication was rarely used. Thus, avoidance of acute headache medication overuse and increased use of prophylactic medication may improve the management of primary chronic headaches in the future.     

Incidence and influence on referral of primary stabbing headache in an outpatient headache clinic

Primary stabbing headache (PSH) is a pain, as brief, sharp, jabbing stabs, predominantly felt in the first division of trigeminal nerve. Population studies have shown that PSH is a common headache. However, most people suffer attacks of low frequency or intensity and seldom seek for medical assistance. There are few clinic-based studies of PSH, and its real influence as a primary cause for referral to neurology outpatient offices is to be determined. We aim to investigate the burden of PSH as main complaint in an outpatient headache clinic. We reviewed all patients with PSH (ICHD-II criteria), attended in an outpatient headache clinic in a tertiary hospital during a 2.5-year period (January 2008–June 2010). We considered demographic and nosological characteristics and if PSH was main cause of submission. 36 patients (26 females, 10 males) out of 725 (5%) were diagnosed of PSH. Mean age at onset 34.1 ± 2.9 years (range 10–72). Mean time from onset to diagnosis 68.8 ± 18.3 months. Twenty-four patients fulfilled ICHD-II criteria for other headaches (14 migraine, 6 tension-type headache, 2 hemicrania continua, 1 primary cough headache and 1 primary exertional headache). 77.7% of patients were submitted from primary care. In 14 patients (39%), PSH was main reason for submission, its intensity or frequency in 5 (35.7%) and fear of malignancy in 9 (74.3%). Only two patients of those who associated other headaches were submitted due to PSH. In conclusion, PSH is not an uncommon diagnosis in an outpatient headache office. However, and according to our data, it is not usually the main cause of submission to a headache clinic.