Spontaneous Intracranial Hypotension Associated with Postural Tachycardia Syndrome

Kato, Yukio; Hayashi, Takeshi; Arai, Noriko; Tanahashi, Norio; Takahashi, Koichi; Takao, Masaki

doi:10.2169/internalmedicine.2587-18

Cited by 15 publications

(14 citation statements)

References 10 publications

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“…Postural tachycardia syndrome has been described as a final common-end pathway for multiple different disorders. 30 It is seen with not only the aforementioned co-morbidities, such as concussion, autoimmune disease, and joint hypermobility, as well as being seen more in females and in Caucasians, but it is also associated with such diverse entities as mast cell activation syndrome, 31 median arcuate ligament syndrome, 32 Chiari malformation, 33 cervical instability, 34 cerebrospinal fluid leak, 35 and syringomyelia. 36 Once this disorder is better defined and established, we would expect to offer new hypotheses or better explanations as to why these family members experience increased associated medical complications.…”

Section: Discussionmentioning

confidence: 99%

Family history of associated disorders in patients with postural tachycardia syndrome

Boris¹,

Huang

Shuey³

et al. 2020

Cardiol Young

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Introduction:Postural tachycardia syndrome is more frequently being recognised in adolescents and adults. However, its pathophysiology remains undefined. We evaluated our database for patterns in family history of clinical symptoms and associated disorders in these patients.Materials and methods:Patients with postural tachycardia syndrome diagnosed in our clinic between 2014 and 2018 and who were less than 19 years at diagnosis were included. The history was reviewed for family members with postural tachycardia syndrome, dizziness and/or syncope, joint hypermobility with or without hypermobile Ehlers–Danlos syndrome, and autoimmune disorders. Statistical analysis assessed the entire cohort plus differences in gender, presence or absence of joint hypermobility, and presence or absence of familial autoimmune disease.Results:A total of 579 patients met inclusion criteria. We found that 14.2% of patients had a family member with postural tachycardia syndrome, with male patients more likely to have an affected family member (20% versus 12.7%, p = 0.04). If the patient also had joint hypermobility, male patients were more likely to have a family member with postural tachycardia syndrome (25% versus 12.6%, p = 0.017), more than one affected family member (7.1% versus 0.74%, p = 0.001), and a family member with joint hypermobility (37.5% versus 23.7%, p = 0.032). Autoimmune disease was seen in 45.1% of family members, but more likely in female patients with concurrent hypermobility (21.1% versus 8.9%, p = 0.035).Discussion:This in-depth analysis of associated familial disorders in patients with postural tachycardia syndrome offers further insight into the pathophysiology of the disorder, and informs further screening of family members in these patients.

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Section: Discussionmentioning

confidence: 99%

Family history of associated disorders in patients with postural tachycardia syndrome

Boris¹,

Huang

Shuey³

et al. 2020

Cardiol Young

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“…5 In this study, all 9 patients with SIH had an increase in HR during HUTT and showed features of orthostatic intolerance. Kato Y et al 7 described an adult female having SIH associated with orthostatic intolerance. Orthostatic headaches in orthostatic intolerance is postulated to be due to relative CSF hypovolemia.…”

Section: Discussionmentioning

confidence: 99%

“…5 International Classification of Headache Disorders (ICHD) third edition recommends treating patients with typical orthostatic headaches with no apparent cause and after exclusion of POTS with autologous lumbar epidural blood patch (EBP). 6 SIH and orthostatic intolerance may co-exist in adults 5,7 but have not been described in young patients. We report a series of seven young patients presenting with orthostatic headaches with an initial diagnosis of SIH and subsequently shown to have orthostatic intolerance on evaluating persistence of symptoms.…”

Section: Introductionmentioning

confidence: 99%

Orthostatic Headaches Associated With Spontaneous Intracranial Hypotension and Autonomic Dysfunction—A Case Series in Young Patients

Ghosh

Tran

Grant

et al. 2021

Child Neurology Open

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Background: Orthostatic headaches can be noted in spontaneous intracranial hypotension and orthostatic intolerance. We present a case series of young patients diagnosed with spontaneous intracranial hypotension and were treated for the same but subsequently developed orthostatic intolerance. Methods: We retrospectively reviewed charts for seven young patients with orthostatic headaches related to spontaneous intracranial hypotension and orthostatic intolerance. Results: Patients were diagnosed with spontaneous intracranial hypotension. Diagnosis was confirmed by identifying epidural contrast leakage and three of seven patients were noted to have early renal contrast excretion on computerized tomography myelography. Patients were treated with epidural blood patches. All patients showed persistent symptoms of autonomic dysfunction after treatment of spontaneous intracranial hypotension and orthostatic intolerance was confirmed with head-up tilt table test. Conclusions: Patients with spontaneous intracranial hypotension failing to improve following epidural blood patching should be evaluated for orthostatic intolerance.

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“…In a case series of 48 patients with POTS and 9 patients with SIH, all patients with SIH fulfilled the clinical criteria of POTS leading the authors to conclude that patients with POTS and orthostatic headache should be screened with further diagnostic tests for SIH [ 44 ]. Kato et al reported a patient with POTS and SIH whose SIH and POTS resolved after treatment with an epidural blood patch, suggesting that POTS may be secondary to SIH [ 45 ]. Using dynamic ultrasound of the optic nerve may differentiate between SIH and POTS based on the decreasing optic nerve sheath diameter upon standing in SIH, but not POTS [ 46 ].…”

Section: Pots and Intracranial Hypotensionmentioning

confidence: 99%

Is postural orthostatic tachycardia syndrome (POTS) a central nervous system disorder?

Blitshteyn

2021

J Neurol

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Postural orthostatic tachycardia syndrome (POTS), a disorder of the autonomic nervous system characterized by a rise in heart rate of at least 30 bpm from supine to standing position, has been traditionally viewed as a dysfunction of the peripheral nervous system. However, recent studies and evidence from overlapping conditions suggest that in addition to being considered a disorder of the peripheral nervous system, POTS should be viewed also as a central nervous system (CNS) disorder given (1) significant CNS symptom burden in patients with POTS; (2) structural and functional differences found on neuroimaging in patients with POTS and other forms of orthostatic intolerance; (3) evidence of cerebral hypoperfusion and possible alteration in cerebrospinal fluid volume, and (4) positive response to medications targeting the CNS and non-pharmacologic CNS therapies. This review outlines existing evidence of POTS as a CNS disorder and proposes a hypothetical model combining key mechanisms in the pathophysiology of POTS. Redefining POTS as a CNS disorder can lead to new possibilities in pharmacotherapy and non-pharmacologic therapeutic interventions in patents affected by this disabling syndrome.

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Spontaneous Intracranial Hypotension Associated with Postural Tachycardia Syndrome

Cited by 15 publications

References 10 publications

Family history of associated disorders in patients with postural tachycardia syndrome

Family history of associated disorders in patients with postural tachycardia syndrome

Orthostatic Headaches Associated With Spontaneous Intracranial Hypotension and Autonomic Dysfunction—A Case Series in Young Patients

Is postural orthostatic tachycardia syndrome (POTS) a central nervous system disorder?

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