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Orthostatic and non-orthostatic headache in postural tachycardia syndrome.

Khurana RK, Eisenberg L.

Cephalalgia. 2010 Sep 6. [Epub ahead of print]

Union Memorial Hospital, Baltimore, USA.

Abstract

Objective: Orthostatic and non-orthostatic headache spectrum was prospectively studied in 24 consecutive patients with postural orthostatic tachycardia syndrome (POTS).Methods: Patients were interviewed about clinical aspects of headache and its precipitation during head-up tilt (HUT). Autonomic functions were assessed using a standard battery of tests. The relationship of orthostatic headache to cardiovascular variables was examined using unpaired two-tailed t-test.Results: Orthostatic headache occurred during daily activity in 14 patients (58.3%) and during HUT in 15 patients (62.5%). Age under 30 years and increasing duration of tilt were predictive for orthostatic headache. Of the 24 patients, 23 (95.8%) had non-orthostatic headache fitting the criteria of migraine or probable migraine.Conclusions: Orthostatic headache affected two-thirds of POTS patients, especially those under age 30. Patients with orthostatic headache should be clinically assessed for POTS and informed of this association to reduce short-term morbidity. Migraine afflicted almost all POTS patients. This co-morbidity should be considered in management of POTS.

PMID: 20819844

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Thanks for the article, firewatcher. I was misdiagnosed with a CSF leak and underwent a year of extreme distress... 4 myelograms, 30 plus blood patches, and 5 hospitalizations because many doctors told me that an orthostatic headache= CSF leak. They discounted all my other symptoms consistent with POTS. The verdict after 18 months: a clear case of POTS and headaches caused by cerebral hypoperfusion. There are other patients out there being treated for invisible CSF leaks that probably have dysautonomia. A top POTS researcher even told my husband at one time that orthostatic headaches were not common in POTS. I will add this article to my collection. Thanks.

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Can anyone get a full text pdf of this article? I really want to know if they found any good treatment strategies for these headaches, plus I want to give it to my neuro.

Thanks!

I'd love the full text too. My husband may be making a trip to the medical library. :) My quality of life would skyrocket if I could gain control over these headaches.

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I was able to get the article. This is a long post as I've copied and pasted the article here as I have no idea how to link to it, especially since it's behind a firewall. I also don't have the graphs here. If you want a copy please PM me.

Sara

Orthostatic and non-orthostatic headache in postural tachycardia syndrome

Ramesh K Khurana and Lindsay Eisenberg

Introduction

Post?lumbar puncture headache that develops in the upright position and improves when the patient is recumbent is a familiar entity (1). Orthostatic headaches brought on by other causes, such as tumors of the central nervous system, are considered rare (2,3). Recently, spontaneous intracranial hypotension, with or without cerebrospinal fluid (CSF) leak, has been increasingly recognized as a cause of orthostatic headache. (1). The headache occurs or worsens within 15 minutes after sitting or standing. In some patients this lag period may extend to several hours. The headache then disappears or improves within 15 to 30 minutes after lying down.

Orthostatic headache also occurs as a manifestation of autonomic dysfunction (4). Orthostatic headache in patients with orthostatic hypotension affects occipital, nape of neck and shoulder regions, coinciding with the fall in blood pressure (4). Orthostatic headache is a known clinical manifestation of autonomic dysreflexia. A spinal lesion at or above the T5 level disconnects the caudal spinal region from supraspinal control. Over the ensuing weeks or months, the visceral or somatic, noxious or innocuous stimulation below the lesion produces pulsating headache, flushing, hypertension and hyperhidrosis (5). In 2003, Mokri and Low reported four patients with orthostatic headache who failed to show a CSF leak. Three had been treated with a lumbar epidural blood patch without relief. Rather than low CSF pressure, a standard battery of autonomic function tests revealed postural orthostatic tachycardia syndrome (POTS). The authors noted that orthostatic headache may occasionally be a presenting manifestation

of POTS (6). However, the relationship between POTS and orthostatic headache has not been fully investigated.

POTS affects an estimated 500,000 Americans between the ages of 14 and 45 years, with a 5 : 1 ratio of females to males. It can be a disabling disorder (7?9). This autonomic disorder is characterized by orthostatic dizziness and other symptoms mimicking panic disorder including palpitations, presyncope, breathing difficulty, fatigue, chest pain and tremulousness.An orthostatic heart rate (HR) increase exceeding 30 beats per minute (bpm) over the supine baseline or over 120 bpm without orthostatic hypotension is a diagnostic criterion for this disorder. Patients may demonstrate

an orthostatic rise in blood pressure and exaggerated increase in norepinephrine levels. It is commonly associated with orthostatic purplish acral discoloration.

A retrospective review of 50 POTS patients revealed a history of orthostatic headache during daily activities in 22% and during head-up tilt (HUT) in 18% (10). The objectives of the current study were to prospectively study the spectrum of headache in POTS patients and to examine the relationship of orthostatic headache to several cardiovascular variables that could contribute to orthostatic headache.

Patients and methods

Institutional review board approval was obtained. Twenty-four consecutive patients with orthostatic symptoms of dizziness, palpitations, nausea, excessive or reduced sweating and difficulty breathing were prospectively studied. All 24 patients referred to the senior author (RKK) had a detailed clinical evaluation, including elaboration of the chief complaint and various somatic neurologic symptoms affecting cranial nerves, the motor system, the sensory system, as well as cerebellar and higher cortical functions. A detailed questionnaire was used to assess autonomic symptoms in several domains.

Informed consent was obtained. A standard battery of autonomic tests was done to document the diagnosis of POTS (11). Patients were monitored for HR by a beat-to-beat HR monitor and for blood pressure by a plethysmographic device fastned around the left middle finger (Finapres, Ohmeda 2300). The HR response to deep breathing (HRDB) and Valsalva ratio (VR) were used to assess cardiovagal function. Blood pressure responses to the Valsalva maneuver and head-up tilt HUT were used to evaluate sympathetic adrenergic function. Thermoregulatory sweat test was employed to assess sympathetic cholinergic function. The patients were off all medications for five days. They were studied in headache-free phase. They were instructed to avoid

alcohol, caffeine and nicotine overnight and to arrive at the laboratory after a light breakfast.

Procedures

Heart rate response to deep breathing

All patients were verbally instructed to breathe evenly and deeply at a rate of six breaths per minute, five seconds in and five seconds out. The difference between the maximum and minimum HR during each respiratory cycle was determined. The mean of differences during six consecutive breathing cycles provided HRDB. The normal value in our laboratory is 25.632.3 standard error (SE) (age 23?49 years) (11).

Valsalva maneuver

The patient, in a semi-recumbent position, started forced expiration from the normal inspiratory position and maintained expiratory pressure of 40mmHg for 10 seconds. A mouthpiece attached by connective tubing to a mercury manometer quantified expiratory pressure. Care was taken to ensure that pressure rose sharply at the onset and fell abruptly at the termination of expiratory strain. The VR, an index of cardiovagal baroreflex activity, was calculated from the ratio of maximal to minimal HR. The test was repeated three times, and the highest VR was selected. The normal

value in our laboratory is 1.750.03 (12).

Head-up tilt test

The patient was strapped across the knees, thighs and chest. Baseline blood pressure and HR were recorded for five minutes. The patient was tilted to 90 for 10 minutes. The patient was asked to enumerate symptoms as they occurred. Blood pressure and HR were recorded continuously during 10 minutes of tilt and for three minutes after the table was returned to the horizontal position. The normal value for HR increase under these conditions in our laboratory is 18.82.3 bpm (13).

Thermoregulatory sweat test

The patient, in supine position and unclothed, was exposed to a heated environment (45?50C) at 35%?40% humidity in a sweat chamber. Iodine and starch combination was employed as a color indicator. The test was continued until generalized sweating occurred or a rise in oral temperature of >1C above baseline was observed (11).

Autonomic laboratory data obtained from each patient was compared with historic controls from our laboratory. If patients met the defined criteria of POTS, laboratory tests were performed to evaluate its possible etiology. All patients were asked about orthostatic and nonorthostatic headache. The headache-related questions were based on the classification criteria of the International Headache Society (14). Questions were asked about frequency, duration, severity, location, character, aggravating factors, relieving factors and associated symptoms. The patients were asked whether

headache began before or after the onset of POTS symptoms. Occurrence of orthostatic headache prompted questions about the duration of upright position needed to precipitate headache, the influence on daily activities and the effect of recumbency. The patients were specifically asked about the occurrence of headache at one, three, five, seven and 10 minutes during HUT. If the patient developed headache during the HUT test, details of headache symptoms were recorded during and after tilt, including severity, character, location and accompanying symptoms. The patients were asked if their headache was relieved when the table was returned to the horizontal position. At the follow-up visit, the patients were asked to supplement

information about orthostatic and non-orthostatic headache, if necessary.

Autonomic and headache data were tabulated and analyzed by descriptive statistics. An unpaired twotailed t-test was used to compare patients with

HUT-induced headache (HUTHA?) to those without HUT-induced headache (HUTHA) for average age and several cardiovascular variables, including baseline

HR, changes in HR, pulse pressure, baseline systolic blood pressure, baseline diastolic blood pressure, maximum diastolic blood pressure during HUT, HRDB and VR. The number of HUTHA? patients under and above the age of 30 years were compared using the Chi-square test.

Results

The patients included 19 women and 5 men, age range 17?47 (33.42.08 meanSE) years. Their presenting complaints in decreasing order of frequency were orthostatic dizziness, fatigue, palpitations, headache, nausea, abnormal sweating, shortness of breath and blurred vision. The duration of symptoms varied from three months to 29 years, with an average of 7.9 years, exceeding six months in all but one patient. All 24 patients had a normal somatic neurologic examination.

The data on MRI were available on 16 patients; 15 were normal, and one had a small non-secreting pituitary adenoma. Laboratory tests including complete blood count, sedimentation rate, antinuclear antibody, serum electrolytes, blood glucose, rapid plasma reagin, urine porphyrin and tests of thyroid and adrenal gland function revealed no significant abnormalities.

Serum protein immunoelectrophoresis, heavy metal screen and paraneoplastic profile, including ganglionic acetylcholine receptor antibodies, were also normal. The results of various autonomic function tests confirmed the diagnosis of POTS (Table 1). The HUT test was abnormal in all patients, as expected. All 24 patients had orthostatic HR increase exceeding 30 bpm above the supine baseline or over 120 bpm along with symptoms of orthostatic intolerance. HRDB and VR, both indices of cardiovagal activity, did not show evidence of vagal neuropathy. Systolic blood pressure overshoot during the fourth phase of the Valsalva maneuver was exaggerated, indicating sympathetic overactivity. The thermoregulatory test was performed in 21 patients. Six patients demonstrated distal anhidrosis suggesting neuropathic pattern. One patient had hyperhidrosis (8).

Three patients had orthostatic headache as the chief presenting complaint. One of them, a chef, could not perform her professional activities. Of the 24 patients, 14 (58.3%) reported orthostatic headache during daily activities (Figure 1). Orthostatic headache occurred usually within the initial 10 minutes of being upright, although two patients reported headache occurrence after being upright for up to two hours. Headache was mostly frontal or holocranial in location, pressure or throbbing in character and moderate to severe in intensity. Frequent accompaniments were photophobia,

phonophobia and nausea. In 10 patients, headache subsided within 2 to 15 minutes of being recumbent. In four patients, relief from headache did not occur until 24 hours after onset. Clinical features were consistent with migraine without aura in four patients. Shorter duration, absence of accompanying symptoms or both excluded other ten patients from current IHS criteria for migraine.

Orthostatic headache induced by HUT in the laboratory was reported by 15 (62.5%) of the 24 patients (Figure 1). Orthostatic dizziness was reported by 75% of patients, followed by paresthesias (50%), palpitations (37.5%) and difficulty breathing (33.3%). Twelve of the 15 patients were younger than 30 years of age. Three of three patients younger than 20 years and eight of nine patients aged 20 to 29 years developed headache during HUT. Only four of 12 patients age30 years had HUT headache (Figure 2).

Headache was mostly frontal or fronto-temporal in location and pressure type in character. Photophobia accompanied headache in five patients, and three complained of nausea. Eight had no accompanying symptoms. Five patients had mild headache, and 10 patients had moderate to severe headache. Only two patients developed headache within two minutes of HUT, but the number of patients developing headache increased gradually to 15 of 24 as the tilt duration increased (Figure 3). The duration of tilt was limited to 10 minutes per the protocol. Upon return to the horizontal position, four patients reported headache improvement within 10 to 15 minutes, nine improved in one to four hours, and two patients took up to two days to

improve. There was no correlation between severity and duration of headache. Based on current IHS criteria, only two of 15 patients developed migraine without aura. Nine patients developed migrainous symptoms but did not fit the criteria for migraine or probable migraine due either to shorter duration or absence of accompanying symptoms. Four patients reported mild frontal or occipital headache lasting a few minutes. None of the 24 patients developed aura in response to orthostatic challenge. HUT failed to precipitate headache in two patients who complained of orthostatic

headache, and a few patients with HUT-induced headache did not complain of orthostatic headache during daily activities.

Of 24 patients, 23 (95.8%) had non-orthostatic headache (Figure 1). Headache preceded the onset of POTS in 10 patients, and only four had been diagnosed with migraine. Imitrex improved headache in one patient, had no benefit in two patients and worsened headache in one patient. Seven patients developed headache concomitantly with onset of POTS. In six patients, headache began after the onset of POTS. Precipitating factors of headache included change in barometric pressure, fatigue, odors, sounds and menstruation. One patient reported visual aura. The headache was of variable frequency and of moderate to severe intensity.

Unilateral and retro-orbital locations were common but temporal, occipital and holocranial locations were also reported. Nausea (65%) and photophobia (60%) were frequent accompaniments, followed by sonophobia and vomiting. Headache was aggravated by standing in 56% of patients. For example, six of 10 patients in whom headache preceded POTS reported aggravation of headache upon standing and marked improvement when supine. One had migraine with aura, 19 had migraine without aura and three had probable migraine (14,15). One patient did not have a history of nonorthostatic headache.

Statistical analysis (Table 2) revealed no significant differences between HUTHA- and HUTHA? patients in baseline HR, change in HR, pulse pressure, baseline systolic blood pressure, baseline diastolic blood pressure, maximum diastolic blood pressure, VR and HRDB. The average age of the HUTHA group was 37.0 ?/ 2.5 years (mean ?/ SE), significantly higher than in the HUTHA? group (26.4 ?/ 2.5 years, p?.01). Of the HUTHA? patients, 73% were under 30 years of age, compared with 11% of HUTHA patients (p?.003).

Discussion

Orthostatic headache was reported by two-thirds of the 24 patients in the current study, and three patients had it as a presenting complaint. Younger age (under 30 years) and increasing duration of upright posture were predictive of increased incidence of orthostatic headache. Orthostatic headache had a ??migrainous?? character, but duration was usually short. In 1999, Jacob and colleagues observed orthostatic headache in 45% of POTS patients (16). Our findings indicate that orthostatic headache is frequently associated with POTS and that recording of HR and blood pressure in the supine and standing positions may help avoid unnecessary invasive studies to exclude low CSF pressure headache. Awareness of association of orthostatic

headache with duration of upright posture may reduce morbidity by limiting the duration of orthostatic stress.

Non-orthostatic headache, migraine without aura, occurred in almost all patients. Migraine prevalence in the U.S. population is 17.6% for women and

6% for men (17,18). A cross-sectional population based survey of 21,177 Norwegians showed lifetime prevalence of migraine of 18.5% in men and 34.1%

in women (19). The present data show that incidence of migraine in POTS patients far exceeds that found in the general population and is higher than the 27.6% incidence reported previously in POTS patients (20).

POTS and migraine are strongly associated. Both occur predominantly in women, with higher incidence during the productive years of life (9,21). Migraine patients display frequent autonomic manifestations including nausea, vomiting and flushing during an attack (22). Cyclical vomiting, a variant of migraine, may be accompanied by postural tachycardia syndrome (23). These findings suggest that migraine is co-morbid with POTS, and like other conditions that are comorbid with POTS, such as chronic fatigue syndrome, it adds to the disability (24,25). However, this co-morbidity,

if appreciated, also provides a therapeutic opportunity in that drugs such as beta-blockers may benefit both disorders.

Can orthostatic headache be differentiated from migraine aggravated by physical activity? Aggravation of migraine by physical activity, a specific diagnostic criterion of the International Headache Society classification, may occur in 90% of patients (26) and incidence may decline with advancing age (21). POTS patients also report aggravation of non-orthostatic headache with physical activity, but they may be free from headache when supine and develop headache with orthostatic challenge. The current finding of increased likelihood of orthostatic headache with longer upright posture suggests that orthostatic stimulus must reach a threshold to precipitate headache. In contrast, in migraine patients, any physical activity, including

upright posture, immediately worsens the headache.

The cause of orthostatic headache in POTS is not known. Impaired cerebrovascular autoregulation may explain orthostatic headache and orthostatic intolerance. However, the data on cerebral hypoperfusion in POTS are conflicting (27,28). In the present study, orthostatic headache was never preceded by aura even in a patient who had migraine with aura and orthostatic headache did not correlate with various cardiovascular

variables. Migraine and POTS co-exist in a majority of patients. POTS is a heterogeneous disorder with several possible pathophysiological mechanisms,

including hyperadrenergic state and beta-receptor supersensitivity (8,27). The perturbations in the dorsal raphae nucleus and locus coeruleus initiate events that generate migraine without aura through the trigeminovascular system along with altered monoaminergic modulation of pain (28). Allodynia, cephalic and extracephalic, is now a recognized manifestation of migraine with clinical and neurophysiological correlates (29,30).

Intracranial hypersensitivity from migraine may predispose POTS patients to orthostatic headache. There are extensive interconnections between the nociceptive and visceral inputs at all levels of the neuraxis for the modulation of pain and autonomic output. The nucleus tractus solitarius, the locus coeruleus and the periaqueductal gray regions have been shown to be important both in pain processing and cardiovascular control.

The lateral periaqueductal gray region, which receives cutaneous nociceptive inputs from the spinal and trigeminal dorsal horn, initiates ??fight or flight?? responses characterized by sympathetic activation with hypertension and tachycardia (31). Additionally, vagus nerve stimulation in awake rats has been shown to significantly reduce formalin-induced trigeminal nociception (32). It can be postulated that migraine induces central

somatic and visceral sensitization in POTS patients.

Convergence of inputs from the nucleus tractus solitarius, altered central monoaminergic balance and withdrawal of vagal tone with reduction of antinociceptive effect upon orthostatic challenge activates the trigeminovascular system to produce orthostatic headache.

In our study, the incidence of orthostatic headache was substantially higher than previously reported (16), due possibly to specific inquiry about the symptom. The current data were collected from a small subspecialty referral sample, a fact that may have resulted in overestimation of incidence of headache in POTS. However, evidence for co-morbidity of orthostatic headache and migraine with POTS was clearly observed.

In conclusion, orthostatic headache affected two thirds of POTS patients, especially those under age 30. Patients with orthostatic headache should be clinically assessed for POTS and informed of this association to reduce short-term morbidity. Migraine afflicted almost all POTS patients. This co-morbidity should be considered in management of POTS.

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I have the headaches every day and every time I try to sit or stand up, unless I am exercising. I can not even sit up without being in a rocker or recliner due to the headaches. I am grateful that researchers are delving into this issue. Maybe it will save other patients from over aggressive CSF leak work-ups. I think migraines and POTS have been well established, but the orthostatic headaches and coat hanger pain are not always mentioned in the literature. Thank you for posting the full text, Dragon.

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I'm with you all. I was really hoping this would shed some light on ideas for treating the headaches. I think mine are more the migraine without aura type but they (along with the accompanying nausea) have been the most debilitating symptom I've been having the past 6 months. They are definitely worse with activity.

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I'm so glad I didn't agree to EMG's in relationship to my headaches. I thought they were POTS related and the doc that wanted to do the testing didn't believe in POTS. She gave me all kinds of grief.

Thanks for posting this article.

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Headaches are definitely a part of my POTS. I used to get them all the time when I first got sick. Now when I have a certain kind of headache I can also almost be positive that my HR is too fast and/or my BP is too low. Usually I can stop the headache with advil and atenolol. In general, even when I was young and didn't have POTS I was always prone to headaches and would get them upon stress, overexertion and during PMS. Luckily the headaches aren't as bad now as they were in my teens.

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