Primary Headaches in Children Under the Age of 7 Years

Recurrent headache is increasingly recognised in young children. Migraine and tension-type headache feature commonly amongst the primary headache disorders seen at this age. Headaches at this age are more likely than in older patients to be ‘unclassifiable’, possibly a reflection of the difficulties in obtaining a detailed headache history from a young child. Together with recent epidemiological data this review highlights the more prevalent primary headache types with advice on making a focussed headache assessment and guidance on management in this age group.     

Verschiedenartige Kopfschmerzformen des Kapitels?4 der Internationalen Kopfschmerzklassifikation

Chapter 4 of the International Classification of Headaches contains a group of clinically very heterogeneous primary headache forms. Little is known about the pathogenesis of these headache types and therapy is usually based on isolated case reports and uncontrolled studies. The forms include primary stabbing headache, primary cough headache, primary exertional headache, primary headache associated with sexual activity, hypnic headache, primary thunderclap headache, hemicrania continua and the new daily persistent headache. Some of these headache forms may be of a symptomatic nature and require careful examination, imaging and further tests. Primary and secondary headache forms must be carefully distinguished.     

Juvenile myoclonic epilepsy presenting as a new daily persistent-like headache

New daily persistent headache (NDPH) is a recognized subtype of chronic daily headache with a unique presentation of a daily headache from onset typically in individuals with minimal or no prior headache history. Various secondary mimics of NDPH have now been documented but at present there has been no association made between primary epilepsy syndromes and new daily persistent-like headaches. A case patient is presented who developed a daily continuous headache from onset who 3 months after headache initiation had her first generalized tonic-clonic seizure. Further investigation into her history and her specific EEG pattern suggested a diagnosis of juvenile myoclonic epilepsy (JME). Her NDPH and seizures ceased with epilepsy treatment. Clinically relevant was that the headache was the primary persistent clinical symptom of her JME before the onset of generalized tonic-clonic seizures. The current case report adds another possible secondary cause of new daily persistent-like headaches to the medical literature and suggests another association between primary epilepsy syndromes and distinct headache syndromes.     

Persistent primary thunderclap headache responsive to gabapentin

We report the case of a woman with an apparent primary thunderclap headache which occurred frequently until she achieved a therapeutic dosage of gabapentin. Primary thunderclap headache is a rare type of headache that warrants significant testing to rule out more ominous possibilities. Whether gabapentin may help other primary thunderclap headache sufferers or not remains unclear. Further research is needed.     

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.     

Is Hemicrania Continua a Single Entity or the Association of Two Headache Forms? Considerations From a Case Report

Hemicrania continua (HC) belongs to the group of primary headaches and it is characterized by a strictly unilateral, continuous headache of moderate intensity, with superimposed exacerbations of severe intensity that are accompanied by trigeminal autonomic features. The syndrome is completely responsive to indomethacin. Here we report a case of a 49‐year‐old man with HC, which may be viewed as a combination of different types of headache, ie, chronic tension‐type headache and trigeminal autonomic cephalalgia. The analysis of this case raises interesting issues regarding the proper place of HC among the primary headache forms.     

Headaches precipitated by cough,prolonged exercise or sexual activity: a prospective etiological and clinical study

Headaches provoked by cough, prolonged physical exercise and sexual activity have not been studied prospectively, clinically and neuroradiologically. Our aim was to delimitate characteristics, etiology, response to treatment and neuroradiological diagnostic protocol of those patients who consult to a general Neurological Department because of provoked headache. Those patients who consulted due to provoked headaches between 1996 and 2006 were interviewed in depth and followed-up for at least 1 year. Neuroradiological protocol included cranio-cervical MRI for all patients with cough headache and dynamic cerebrospinal functional MRI in secondary cough headache cases. In patients with headache provoked by prolonged physical exercise or/and sexual activity cranial neuroimaging (CT and/or MRI) was performed and, in case of suspicion of subarachnoid bleeding, angioMRI and/or lumbar tap were carried out. A total of 6,412 patients consulted due to headache during the 10 years of the study. The number of patients who had consulted due to any of these headaches is 97 (1.5% of all headaches). Diagnostic distribution was as follows: 68 patients (70.1%) consulted due to cough headache, 11 (11.3%) due to exertional headache and 18 (18.6%) due to sexual headache. A total of 28 patients (41.2%) out of 68 were diagnosed of primary cough headache, while the remaining 40 (58.8%) had secondary cough headache, always due to structural lesions in the posterior fossa, which in most cases was a Chiari type I malformation. In seven patients, cough headache was precipitated by treatment with angiotensin-converting enzyme inhibitors. As compared to the primary variety, secondary cough headache began earlier (average 40 vs. 60 years old), was located posteriorly, lasted longer (5 years vs. 11 months), was associated with posterior fossa symptoms/signs and did not respond to indomethacin. All those patients showed difficulties in the cerebrospinal fluid circulation in the foramen magnum region in the dynamic MRI study and preoperative plateau waves, which disappeared after posterior fossa reconstruction. The mean age at onset for primary headaches provoked by physical exercise and sexual activity began at the same age (40 years old), shared clinical characteristics (bilateral, pulsating) and responded to beta-blockers. Contrary to cough headache, secondary cases are rare and the most frequent etiology was subarachnoid bleeding. In conclusion, these conditions account for a low proportion of headache consultations. These data show the total separation between cough headache versus headache due to physical exercise and sexual activity, confirm that these two latter headaches are clinical variants of the same entity and illustrate the clinical differences between the primary and secondary provoked headaches.     

Classification of headache disorders

In 2003 the International Headache Society (IHS) published the second edition of the International Classification of Headache Disorders. Diagnostic criteria for no less than 206 separate headache diagnoses are presented in the parts (I) primary headaches, (II) secondary headaches and (III) cranial neuralgia, central and primary facial pain. The headaches are classified according to the etiology in case of the secondary headaches and according to the phenomenology in case of the primary headaches. It is the task of the headache specialist to identify the correct headache diagnose with the smallest effort possible. Both, the differentiation between secondary and primary headaches and the differentiation between the various primary headaches are of equal importance.     

Klassifikation von Kopfschmerzen

In 2003 the International Headache Society (IHS) published the second edition of the International Classification of Headache Disorders. Diagnostic criteria for no less than 206 separate headache diagnoses are presented in the parts (I) primary headaches, (II) secondary headaches and (III) cranial neuralgia, central and primary facial pain. The headaches are classified according to the etiology in case of the secondary headaches and according to the phenomenology in case of the primary headaches. It is the task of the headache specialist to identify the correct headache diagnose with the smallest effort possible. Both, the differentiation between secondary and primary headaches and the differentiation between the various primary headaches are of equal importance.     

Unusual Headaches in the Elderly

Prevalence of headache lowers with age, and headaches of elderly adults tend to be different than those of the younger population. Secondary headaches, such as headaches associated with vascular disease, head trauma, and neoplasm, are more common. Also, certain headache types tend to be geriatric disorders, such as primary cough headache, hypnic headache, typical aura without headache, exploding head syndrome, and giant cell arteritis. This review provides an overview of some of the major and unusual geriatric headaches, both primary and secondary.     

Kopf- und Gesichtsschmerz im Alter

Whilst headache disorders belong to the most common health problems of the younger population, the occurrence diminishes with advancing age. However, in individual cases headaches may be especially severe in old age significantly reducing the quality of life. Typical causes of headache in the elderly are giant cell arteritis (arteritis temporalis), cranial neuralgia and hypnic headache. The incidence of intracranial mass lesions also increases with age. In addition to these secondary forms of headache, the typical primary headache disorders migraine, tension headache and cluster headache may also persist in the elderly. In drug treatment of headaches in the elderly, an impairment of renal and/or hepatic function has to be taken in account, as should be the potential multimorbidity of elderly patients.     

Cluster-like headache and idiopathic intracranial hypertension: a case report

Cluster headache (CH) is a well-defined primary headache syndrome, but cases of symptomatic headache with clinical features of CH have been previously reported. Idiopathic Intracranial Hypertension (IIH) is a secondary headache disorder characterized by headache and visual symptoms, without clinical, radiological or laboratory evidence of intracranial pathology. Both papilloedema and IIH-related headache are typically bilateral, however asymmetrical or even unilateral localizations are described in literature. We report the case of a previously headache-free woman who presented cluster-like headache and asymmetrical papilloedema related to IIH. In our opinion the asymmetrical presentation supports, in this case, the hypothesis of cavernous sinus involvement in the IIH-related cluster-like headache pathogenesis.     

Headache and facial pain in the elderly

Whilst headache disorders belong to the most common health problems of the younger population, the occurrence diminishes with advancing age. However, in individual cases headaches may be especially severe in old age significantly reducing the quality of life. Typical causes of headache in the elderly are giant cell arteritis (arteritis temporalis), cranial neuralgia and hypnic headache. The incidence of intracranial mass lesions also increases with age. In addition to these secondary forms of headache, the typical primary headache disorders migraine, tension headache and cluster headache may also persist in the elderly. In drug treatment of headaches in the elderly, an impairment of renal and/or hepatic function has to be taken in account, as should be the potential multimorbidity of elderly patients.     

Adult aqueductal stenosis presenting as a thunderclap headache: a case report

Thunderclap headache is well known to be a presenting feature of a variety of causal events. Indeed, a primary form is considered in the International Classification of Headache Disorders-II, but such diagnosis must be made only after exclusion of a possible secondary cause. We report a case of late-onset idiopathic aqueductal stenosis presenting with thunderclap headache, in the absence of abnormal neurological findings or indirect signs of raised intracranial pressure. The patient recovered completely after endoscopic third ventriculostomy. This case indicates primary aqueduct stenosis as a possible, never previously reported, cause of thunderclap headache.     

Medication overuse headache: clinical features predicting treatment outcome at 1-year follow-up

We present a prospective study of 240 patients with medication overuse headache (MOH) treated with drug withdrawal and prophylactic medications. At 1-year follow-up, 137 (57.1%) patients were without chronic headache and without medication overuse, eight (3.3%) patients did not improve after withdrawal and 95 (39.6%) relapsed developing recurrent overuse. Age at time of MOH diagnosis, regular use of benzodiazepines, frequency and Migraine Disability Assessment (MIDAS) score of chronic headache, age at onset of primary headache, frequency and MIDAS score of primary headache, ergotamine compound overuse and daily drug intake were significantly different between successfully and unsuccessfully treated patients. Multivariate analysis determined the frequency of primary headache disorder, ergotamine overuse and disability of chronic headache estimated by MIDAS as independent predictors of treatment efficacy at 1-year follow-up.     

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.     

Nummular headache: a series with symptomatic and primary cases

Nummular headache (NH) is a primary headache adopting the form of local pain in a circumscribed area of < 7 cm in diameter in the tuber parietale, albeit it may also be located in other areas of the head. Although it is chronic, it is commonly associated with exacerbations and short periods of remission. Here we report four cases. Two of them could not be considered primary: in one the pain was related to an underlying, pointed and benign lesion disclosed only by magnetic resonance imaging (case 1); the second one had persistent NH days after trans-sphenoidal surgery for a pituitary adenoma, similar to a postcraniotomy headache (case 2). The two final patients suffered from typical forms of primary NH, one associated with migraine without aura, the other with chronic tension-type headache. The response to pain-related treatments and to preventive drugs was poor in the symptomatic as well as in the primary cases. The mechanisms are not clear, and peripheral (case 1) and also central pathways (case 2) could be involved. In the end, secondary forms of NH might coexist with classical primary NH. Particular attention should be paid to tiny skull lesions and to key events preceding the pain.     

Hypnic Headache

Hypnic headache is a rare short-lasting headache occurring exclusively during sleep in the elderly population. First described in 1988 by Raskin, this headache syndrome was adopted by the International Classification of Headache Disorders in 2004 within the “other primary headache” group (code 4.5). Since then, additional case reports and case series were published and provided data to best understand hypnic headache. This review highlights new findings to discuss the relevance of actual diagnostic criteria and the treatment approach of this nocturnal headache syndrome. We also consider hypnic headache pathophysiology that remains speculative.     

Headache in the elderly

Chronic headache is still a frequent problem in old age, affecting about 10% of all women and 5% of all men older than 70 years. The incidence of primary headache decreases with advancing age, while that of secondary headache increases. The clinical characteristics of migraine can also change with age; for example, vegetative symptoms are less prominent, and less intense migrainous pain localized predominantly in the neck is frequently reported. Migraine aura can also be experienced more frequently in isolation, without a headache. Hypnic headache is a rare primary headache syndrome that occurs almost exclusively in the elderly. Most of the secondary headache syndromes that occur more frequently in old age present clinically as tension-type headache. Examples of rather common reasons for secondary headache syndromes in the elderly are intracranial space-occupying lesions, ophthalmological problems and autoimmune diseases such as giant cell arteritis. Elderly patients are especially likely to have a number of illnesses at any one time for which they take various medications each day, so that headaches can also quite often be caused by their medication or by withdrawal of these. As a result of such multimorbidity the homeostasis is disturbed in such patients, leading to various conditions that can entail concomitant headaches (sleep apnoea syndrome, dialysis headache, headache attributed to arterial hypertension or hypothyroidism). Familiar facial neuralgias, such as trigeminal neuralgia or postherpetic neuralgia following manifest herpes zoster affecting the face, become markedly more frequent with age. In general, in the treatment of headaches in the elderly it is essential to pay careful attention to potential interactions with the multiple drugs needed because of other diseases; in addition, the comorbidities themselves have to be taken into account, especially depression, anxiety and cognitive impairment, necessitating multimodal, interdisciplinary therapy plans.