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

ABSTRACT: 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.

SUBMITTER: Ghosh A

PROVIDER: S-EPMC8619734 | biostudies-literature |

REPOSITORIES: biostudies-literature

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A rare case of orthostatic headache due to spontaneous intracranial hypotension.

Project description:Headache is one of the most common clinical entities, and has a long list of differential diagnoses; however, one of the more uncommon causes of postural headache is spontaneous intracranial hypotension. It is important that clinicians be aware of this condition, as it is often overlooked, leading to invasive and unnecessary diagnostic testing. A good history and physical exam, paired with an MRI of the brain is sufficient to make the diagnosis of SIH, relieve the symptomology of the patient in a quick and efficient manner, and avoid costly invasive procedures.

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Spontaneous intracranial hypotension complicated by cerebral venous thrombosis.

Project description:Spontaneous intracranial hypotension (SIH) is a well-known cause of orthostatic headache. Although subdural fluid collection is a usual complication of SIH, SIH as a risk factor for cerebral venous thrombosis (CVT) is not well-known. There are several mechanisms that could contribute to the development of CVT in SIH. Herein, we report a case of a 33-year-old woman with SIH complicated by CVT. She was treated with anticoagulation but did not receive a blood patch for the SIH, because there was resolution of orthostatic headache with bed rest and sufficient hydration. Follow-up magnetic resonance imaging showed resolution of the findings of SIH and CVT. Patients with SIH should be closely observed for any change in the headache pattern, which might suggest the development of CVT.

| S-EPMC6020096 | biostudies-literature

Widespread cardiovascular autonomic dysfunction in primary amyloidosis: does spontaneous hyperventilation have a compensatory role against postural hypotension?

Project description:OBJECTIVE:To investigate the possible causes of abnormal blood pressure control in light chain related (primary, AL) amyloidosis. DESIGN:Cardiovascular, autonomic, and respiratory response to passive tilting were investigated in 51 patients with primary amyloidosis (mean (SEM) age 56 (2) years) and in 20 age matched controls. Spontaneous fluctuations in RR interval, respiration, end tidal carbon dioxide, blood pressure, and skin microcirculation were recorded during supine rest and with tilting. The values were subjected to spectral analysis to assess baroreflex sensitivity and the autonomic modulation of cardiac and vascular responses. SETTING:Tertiary referral centre. RESULTS:Autonomic modulation of the heart and blood pressure was nearly absent in the patients with amyloidosis: thus baroreflex sensitivity and the low frequency (0.1 Hz) fluctuations in all cardiovascular signals were severely reduced (p < 0.01 or more), as were respiratory fluctuations in the RR interval, and no change was observed upon tilting. Despite reduced autonomic modulation, blood pressure remained relatively stable in the amyloid group from supine to tilting. End tidal carbon dioxide was reduced in the amyloid patients (p < 0.001) indicating persistent hyperventilation; the breathing rate correlated inversely with the fall in blood pressure on tilting (p < 0.05). CONCLUSIONS:In primary amyloidosis, pronounced abnormalities in arterial baroreflexes and cardiovascular autonomic modulation to the heart and the vessels may be partly compensated for by hyperventilation at a slow breathing rate.

| S-EPMC1767452 | biostudies-literature

Management of spontaneous intracranial hypotension - Transorbital ultrasound as discriminator.

Project description:ObjectiveSpontaneous intracranial hypotension (SIH) is most commonly caused by cerebrospinal fluid (CSF) leakage. Therefore, we hypothesised that patients with orthostatic headache (OH) would show decreased optic nerve sheath diameter (ONSD) during changes from supine to upright position.MethodsTransorbital B-mode ultrasound was performed employing a high-frequency transducer for ONSD measurements in the supine and upright positions. Absolute values and changes of ONSD from supine to upright were assessed. Ultrasound was performed in 39 SIH patients, 18 with OH and 21 without OH, and in 39 age-matched control subjects. The control group comprised 20 patients admitted for back surgery without headache or any orthostatic symptoms, and 19 healthy controls.ResultsIn supine position, mean ONSD (±SD) was similar in patients with (5.38±0.91?mm) or without OH (5.48±0.89?mm; p=0.921). However, in upright position, mean ONSD was different between patients with (4.84±0.99?mm) and without OH (5.53±0.99?mm; p=0.044). Furthermore, the change in ONSD from supine to upright position was significantly greater in SIH patients with OH (-0.53±0.34?mm) than in SIH patients without OH (0.05±0.41?mm; p?0.001) or in control subjects (0.01±0.38?mm; p?0.001; area under the curve: 0.874 in receiver operating characteristics analysis).ConclusionsSymptomatic patients with SIH showed a significant decrease of ONSD, as assessed by ultrasound, when changing from the supine to the upright position. Ultrasound assessment of the ONSD in two positions may be a novel, non-invasive tool for the diagnosis and follow-up of SIH and for elucidating the pathophysiology of SIH.

| S-EPMC4893146 | biostudies-literature

Efficacy of atomoxetine versus midodrine for the treatment of orthostatic hypotension in autonomic failure.

Project description:The clinical presentation of autonomic failure is orthostatic hypotension. Severely affected patients require pharmacological treatment to prevent presyncopal symptoms or frank syncope. We previously reported in a proof of concept study that pediatric doses of the norepinephrine transporter blockade, atomoxetine, increases blood pressure in autonomic failure patients with residual sympathetic activity compared with placebo. Given that the sympathetic nervous system is maximally activated in the upright position, we hypothesized that atomoxetine would be superior to midodrine, a direct vasoconstrictor, in improving upright blood pressure and orthostatic hypotension-related symptoms. To test this hypothesis, we compared the effect of acute atomoxetine versus midodrine on upright systolic blood pressure and orthostatic symptom scores in 65 patients with severe autonomic failure. There were no differences in seated systolic blood pressure (means difference=0.3 mm Hg; 95% confidence [CI], -7.3 to 7.9; P=0.94). In contrast, atomoxetine produced a greater pressor response in upright systolic blood pressure (means difference=7.5 mm Hg; 95% CI, 0.6 to 15; P=0.03) compared with midodrine. Furthermore, atomoxetine (means difference=0.4; 95% CI, 0.1 to 0.8; P=0.02), but not midodrine (means difference=0.5; 95% CI, -0.1 to 1.0; P=0.08), improved orthostatic hypotension-related symptoms as compared with placebo. The results of our study suggest that atomoxetine could be a superior therapeutic option than midodrine for the treatment of orthostatic hypotension in autonomic failure.

| S-EPMC4231172 | biostudies-literature

Postural changes in optic nerve and optic nerve sheath diameters in postural orthostatic tachycardia syndrome and spontaneous intracranial hypotension: A cohort study.

Project description:BackgroundPostural orthostatic tachycardia syndrome is a disorder of the autonomic nervous system. Approximately 30% of patients experience orthostatic headaches. Orthostatic headaches also are a hallmark symptom in spontaneous intracranial hypotension. While the cause of orthostatic headaches in spontaneous intracranial hypotension can be linked to the cerebrospinal fluid loss at the spinal level and consecutively reduced intracranial pressure in the upright position, the cause of orthostatic headaches in postural orthostatic tachycardia syndrome still remains unknown. The present study examined orthostatic changes of intracranial pressure using dynamic ultrasound of the optic nerve and optic nerve sheath diameter in postural orthostatic tachycardia syndrome, spontaneous intracranial hypotension and healthy subjects.MethodsData was obtained from postural orthostatic tachycardia syndrome patients with (n = 7) and without orthostatic headaches (n = 7), spontaneous intracranial hypotension patients (n = 5) and healthy subjects (n = 8). All participants underwent high-resolution transorbital ultrasound in the supine and upright position to assess optic nerve and optic nerve sheath diameter.ResultsGroup differences were found in percentage deviations when changing position of optic nerve sheath diameter (p < 0.01), but not regarding the optic nerve diameter. Pairwise comparisons indicated differences in optic nerve sheath diameter only between spontaneous intracranial hypotension and the other groups. No differences were found between postural orthostatic tachycardia syndrome patients with and without orthostatic headaches.ConclusionThis study shows that the size of the optic nerve sheath diameter dynamically decreases during orthostatic stress in spontaneous intracranial hypotension, but not in postural orthostatic tachycardia syndrome with or without orthostatic headaches, which indicates different underlying causes.

| S-EPMC6785073 | biostudies-literature

Spontaneous Intracranial Hypotension and its Association with Movement Disorders?

Project description:In Response To: Salazar R. Spontaneous intracranial hypotension associated with kinetic tremor and ataxia. Tremor Other Hyperkinet Mov. 2016; 6. doi: 10.7916/D8HQ3ZN5.

| S-EPMC5121194 | biostudies-other

Management of Orthostatic Hypotension.

Project description:PURPOSE OF REVIEW:This article reviews the management of orthostatic hypotension with emphasis on neurogenic orthostatic hypotension. RECENT FINDINGS:Establishing whether the cause of orthostatic hypotension is a pathologic lesion in sympathetic neurons (ie, neurogenic orthostatic hypotension) or secondary to other medical causes (ie, non-neurogenic orthostatic hypotension) can be achieved by measuring blood pressure and heart rate at the bedside. Whereas fludrocortisone has been extensively used as first-line treatment in the past, it is associated with adverse events including renal and cardiac failure and increased risk of all-cause hospitalization. Distinguishing whether neurogenic orthostatic hypotension is caused by central or peripheral dysfunction has therapeutic implications. Patients with peripheral sympathetic denervation respond better to norepinephrine agonists/precursors such as droxidopa, whereas patients with central autonomic dysfunction respond better to norepinephrine reuptake inhibitors. SUMMARY:Management of orthostatic hypotension is aimed at improving quality of life and reducing symptoms rather than at normalizing blood pressure. Nonpharmacologic measures are the key to success. Pharmacologic options include volume expansion with fludrocortisone and sympathetic enhancement with midodrine, droxidopa, and norepinephrine reuptake inhibitors. Neurogenic supine hypertension complicates management of orthostatic hypotension and is primarily ameliorated by avoiding the supine position and sleeping with the head of the bed elevated.

| S-EPMC7339914 | biostudies-literature

Reply to: Spontaneous Intracranial Hypotension and its Association with Movement Disorders?

Project description:In Response To: Onder H. Spontaneous intracranial hypotension and its association with movement disorders? Tremor Other Hyperkinet Mov. 2016; 6. doi: 10.7916/D84B31NS Original Article: Salazar R. Spontaneous intracranial hypotension associated with kinetic tremor and ataxia. Tremor Other Hyperkinet Mov. 2016; 6. doi: 10.7916/D8HQ3ZN5.

| S-EPMC5121195 | biostudies-other

Orthostatic Hypotension in Parkinson Disease.

Project description:Orthostatic hypotension (OH) is a sustained fall in blood pressure on standing that can cause symptoms of organ hypoperfusion. OH is associated with increased morbidity and mortality and leads to a significant number of hospital admissions. OH can be caused by volume depletion, blood loss, cardiac pump failure, large varicose veins, medications, or defective activation of sympathetic nerves and reduced norepinephrine release upon standing. Neurogenic OH is a frequent and disabling problem in patients with synucleinopathies such as Parkinson disease, multiple system atrophy, and pure autonomic failure, and it is commonly associated with supine hypertension. Several therapeutic options are available.

| S-EPMC7029426 | biostudies-literature

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