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Primary Stabbing, Cough, Exertional, and Thunderclap Headaches

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Primary Stabbing, Cough, Exertional, and Thunderclap Headaches P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-99 Olesen- 2057G GRBT050-Olesen-v6.cls August 18, 2005 1:7 ••OTHER PRIMARY HEADACHES ••Chapter 99 ◗ Primary Stabbing, Cough, Exertional, and Thunderclap Headaches David Dodick and Julio Pascual PRIMARY STABBING HEADACHE The mean age of onset of primary stabbing headache is 47 years (range 12 to 70) (52). The female:male ratio has International Headache Society (IHS) ICHD-II Code: varied from 1:49 to 6.6. 4.1 Primary stabbing headache World Health Organization (WHO) ICD-10NA Code: G44.800 Idiopathic stabbing headache CLINICAL FEATURES Short description: Transient and localized stabs of pain in the head that occur spontaneously in the absence of or- Diagnostic Criteria for Primary Stabbing Headache (28): ganic disease of underlying structures or of the cranial A. Pain occurring as a single stab or a series of stabs con- nerves fined to the head and exclusively or predominantly felt Previously used terms: Ice-pick pains; jabs and jolts; oph- in the distribution of the first division of the trigeminal thalmodynia periodica nerve (orbit, temple, and parietal areas). B. Stabs last for up to a few seconds and recur with EPIDEMIOLOGY irregular frequency ranging from one to many per day. Prevalence of the ultrashort paroxysms of head pain that C. No accompanying symptoms. characterize primary stabbing headache has been difficult D. Not attributed to another disorder. to estimate and results have varied considerably. In two population-based studies, idiopathic stabbing headache The painful attacks of primary stabbing headache are ul- was found to be less than 1 to 2% (48,65). However, in trashort, lasting only 1 second in more than two thirds a large-scale, cross-sectional study of headache in a parish of cases (52). In some patients, the pain can last up to in the mountainous region of V˚ag˚a,Norway, primary stab- 10 seconds. The pain is frequently unifocal, often in the or- bing headache was verified in 35.2% of the 1,838 adult bital region, but multifocal patterns have been described parishioners (18 to 65 years of age) who were examined with the pain quickly and erratically changing location. (79). The pain has also been described in the facial, occipital, Prevalence estimates vary depending on the studied retroauricular, temporal, and even parietal regions. The population. For example, primary stabbing headache attacks are often sudden, and moderate to high intensity, is more prevalent in migraineurs and individuals with with a pricking or stabbing character. The frequency of at- tension-type headache. Not surprisingly, given the preva- tacks vary widely from 1 attack per year to 50 attacks daily. lence of these primary headache disorders in the popula- In one study, chronic attacks (>80% of days) occurred in tion, one would expect a prevalence of at least one third 14% of 38 patients followed over a 1-year period. Most in a general unselected population. Indeed, the prevalence attacks are distributed throughout the day. The paroxysms of primary stabbing headache in one study of migraine generally occur spontaneously without provocation. There sufferers was 42% compared to only 3% in controls (64). are seldom associated features such as lacrimation, rhi- The high prevalence of primary stabbing headache among norrhea, conjunctival injection, nausea, or photophobia. migraineurs has been confirmed in subsequent studies Attack-related conjunctival hemorrhage and monocular vi- (20). sual loss have been described (52,94). Occasionally, bright 831 P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-99 Olesen- 2057G GRBT050-Olesen-v6.cls August 18, 2005 1:7 832 Tension-Type Headaches, Cluster Headaches, and Other Primary Headaches light, emotional stress, and postural changes are reported Previously used terms: Benign cough headache, valsalva to trigger attacks. The ultrashort duration and lack of maneuver headache cranial autonomic features distinguish this disorder from short-lasting unilateral neuralgiform pain with conjunc- tival injection and tearing (SUNCT) syndrome, where at- EPIDEMIOLOGY AND ETIOLOGY tacks last from 15 to 180 seconds. The presence of trig- ger points, duration of a few seconds, and occurrence of Cough headache is considered a rare entity. Rasmussen pain in the second and third trigeminal distribution are and Olesen (66) have shown that the lifetime prevalence of characteristic of trigeminal neuralgia, with which primary cough headache is one percent (95% confidence interval stabbing headache can be confused. In addition to a high [CI] 0 to 2%). Over 15 years, of the 3,498 patients attend- prevalence in patients with migraine, primary stabbing ing a neurology department owing to headache, 20 (0.6%) headache may also be seen in association with tension- consulted because of cough headache (54). type headache, cluster headache, cervicogenic headache, Cough headache can be either a primary benign condi- and during painful exacerbations in up to two thirds of tion or secondary to structural cranial disease. From older patients with hemicrania continua. case series (prior to computed tomography [CT] and mag- Although invariably benign, primary stabbing headache netic resonance imaging [MRI]), it was concluded that may be associated with intracranial structural lesions such about 20% of patients with cough headache had struc- as meningioma and pituitary tumors (38,44). Primary tural lesions, most of them a Chiari type I deformity stabbing headache has also been associated with onset (49,54,68,70,84). However, with modern neuroradiologic of cerebrovascular diseases, cranial and ocular trauma, techniques, it is clear that almost half of cough headache and herpes zoster (52). An association may also exist be- patients have symptomatic cough headache owing to ton- tween new-onset primary stabbing headache and intraoc- sillar descent or, more rarely, to other space-occupying le- ular pressure elevation. Therefore, when clinically sus- sions in the posterior fossa/foramen magnum area (55). pect, patients with new-onset primary stabbing headache Around 30% of patients with Chiari type I malformation should undergo a diagnostic evaluation to exclude organic experience headache aggravated by Valsalva maneuvers, pathology. mainly cough (56) (Fig. 99-1). In summary, it can be con- cluded that about 50% of the patients with cough headache show no demonstrable etiology, and the other half are sec- MANAGEMENT ondary to structural lesions, mostly at the foramen mag- num level (57). Acute treatment of primary stabbing headache is not fea- sible given its ultrashort duration and repetitive nature. When attacks occur with a frequency that warrants pro- PATHOPHYSIOLOGY phylactic therapy, indomethacin is usually the treatment of choice (grade C recommendation). Indomethacin pro- Secondary cough headache seems to be caused by a tem- vides complete or partial improvement in two thirds of porary impaction of the cerebellar tonsils below the fora- patients (16,46,52). The usual effective dose range from 25 men magnum (50,71,90,91). In two patients with cough to 150 mg per day. Recently, melatonin was described as headache and tonsillar herniation, Williams demonstrated completely effective in three patients at doses ranging from a pressure difference between the ventricle and the lum- 3to9mgatbedtime (69). A patient with primary stabbing bar subarachnoid space during coughing (91). This cra- headache and exploding head syndrome was reported to niospinal pressure dissociation displaces the cerebellar have responded to nifedipine (31). In three patients who tonsils into the foramen magnum. Williams also ob- began to experience primary stabbing headache after is- served that the headache disappeared after decompres- chemic stroke, complete response was obtained with the sive craniectomy. Subsequently, Nightingale and Williams use of celecoxib 100 mg twice daily (60). described four more patients who had headache from episodic impaction of the cerebellar tonsils in the foramen magnum after abrupt Valsalva maneuvers (50). In our se- PRIMARY COUGH HEADACHE ries, not only was it demonstrated that tonsilar descent is the actual cause of cough headache, but it was also shown International Headache Society (IHS) ICHD-II Code: that the presence of cough headache in Chiari type I pa- 4.2 Primary cough headache tients only correlated with the degree of tonsilar descent World Health Organization (WHO) ICD-10NA Code: (55,56). Pujol et al. (61), using cine phase-contrast MRI, G44. 803 Benign cough headache were able to detect this abnormal pulsatile motion of the Short description: Headache precipitated by coughing or cerebellar tonsils in Chiari type I patients, but not in con- straining in the absence of any intracranial disorder trols. This movement produced a selective obstruction of P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-99 Olesen- 2057G GRBT050-Olesen-v6.cls August 18, 2005 1:7 Primary Stabbing, Cough, Exertional, and Thunderclap Headaches 833 FIGURE 99-1. Preoperative (A) and post- operative (B) T2-weighted sagittal MR images from a 36-year old woman with secondary cough headache. (A) Note the presence of tonsillar descent (arrow) and flattening of posterior fossa (asterisks) as well as the absence of cisterna magna. (B) After posterior fossa reconstruction, note the appearance of cisterna magna with restitution of cerebrospinal fluid transit (asterisks) with upward migration of the tonsils. Reproduced with permission from Pascual J. Activity-related headache. In: Gilmar S, ed. MedLink Neurol.
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