Orthostatic headache is a medical condition in which a person develops a

headache A headache, also known as cephalalgia, is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of Depression (mood), depression in those with severe ...

while vertical and the headache is relieved when horizontal. Previously it was often misdiagnosed as different primary headache disorders such as migraine or tension headaches. Increasing awareness of the symptom and its causes has prevented delayed or missed diagnosis.

Causes

The most common cause of orthostatic headache is low

cerebrospinal fluid Cerebrospinal fluid (CSF) is a clear, colorless Extracellular fluid#Transcellular fluid, transcellular body fluid found within the meninges, meningeal tissue that surrounds the vertebrate brain and spinal cord, and in the ventricular system, ven ...

pressure, due to a

cerebrospinal fluid leak A cerebrospinal fluid leak (CSF leak or CSFL) is a medical condition where the cerebrospinal fluid (CSF) that surrounds the brain and spinal cord leaks out of one or more holes or tears in the dura mater. A CSF leak is classed as either spontane ...

, or a post-dural-puncture leak. It is also occasionally the most prominent symptom of

postural orthostatic tachycardia syndrome Postural orthostatic tachycardia syndrome (POTS) is a condition characterized by an abnormally large increase in heart rate upon sitting up or standing. POTS is a disorder of the autonomic nervous system that can lead to a variety of symptoms, ...

(POTS). Distinguishing POTS from a cerebrospinal fluid leak can be difficult, because the defining symptom of POTS, positional

tachycardia Tachycardia, also called tachyarrhythmia, is a heart rate that exceeds the normal resting rate. In general, a resting heart rate over 100 beats per minute is accepted as tachycardia in adults. Heart rates above the resting rate may be normal ...

, also occurs in some people with cerebrospinal fluid leaks. Furthermore, both POTS and cerebrospinal fluid leaks are sometimes present in the same person, especially in people with

Ehlers–Danlos syndrome Ehlers–Danlos syndromes (EDS) is a group of 14 genetic connective-tissue disorders. Symptoms often include loose joints, joint pain, stretchy velvety skin, and abnormal scar formation. These may be noticed at birth or in early childhood. Co ...

. Other causes include

colloid cyst A colloid cyst is a non-malignant tumor in the brain. It consists of a gelatinous material contained within a membrane of epithelial tissue. It is almost always found just posterior to the foramen of Monro in the anterior aspect of the third v ...

s and possibly

connective tissue disorder Connective tissue diseases (also termed connective tissue disorders, or collagen vascular diseases), are medical conditions that affect connective tissue. Connective tissues protect, support, and provide structure for the body's other tissues a ...

s. It may occur as a complication of decompressive surgery for

Chiari malformation In neurology, the Chiari malformation ( ; CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull). CMs can cau ...

or decompressive craniectomies for

cerebral edema Cerebral edema is excess accumulation of fluid ( edema) in the intracellular or extracellular spaces of the brain. This typically causes impaired nerve function, increased pressure within the skull, and can eventually lead to direct compres ...

Mechanism

causes loss of

volume around the brain. This causes the brain to lose its buoyancy, which results in pressure on pain-sensitive areas like the dura and blood vessels. The resulting pain is a

, and because the brain is more reliant on its buoyancy in an upright position the headache can be relieved by switching to a horizontal position.

Diagnosis

Cerebrospinal fluid leaks are diagnosed by performing different tests. A diagnostic dural puncture is commonly used because its results show the presence of a leak easily. Other types of tests that could be used are cranial CT, cranial MRI, spinal MRI, and CT myelography. A cranial MRI can be diagnostic by showing one of the five main findings, which are subdural fluid collections, enhancement of venous structures, dural enhancement on MRI sagittal views,Citation: Gaillard, F., Baba, Y. Intracranial hypotension. Reference article, Radiopaedia.org. (accessed on 21 Oct 2021) https://doi.org/10.53347/rID-1519 DOI:https://doi.org/10.53347/rID-1519 pituitary hyperemia, and sagging of the brain. Another cause of orthostatic headaches is

(POTS), a form of

dysautonomia Dysautonomia, autonomic failure, or autonomic dysfunction is a condition in which the autonomic nervous system (ANS) does not work properly. This condition may affect the functioning of the heart, bladder, intestines, sweat glands, pupils, and ...

, which is diagnosed with autonomic testing instead of the imaging tests that are used to determine a CSF leak. It can be difficult to distinguish if a patient is solely affected by POTS because patients with CSF leaks have similar symptoms and may even develop secondary POTS.

Management

Cerebrospinal fluid leaks can be managed short term with bed rest and plentiful hydration. They can then be treated with an

epidural blood patch An epidural blood patch (EBP) is a surgical procedure that uses autologous blood, meaning the patient's own blood, in order to close one or many holes in the dura mater of the spinal cord, which occurred as a complication of a lumbar puncture ...

(EBP) with autologous blood, which is the standard initial procedure. If an EBP is ineffective, surgery is also an option for treatment. A surgical procedure would be customized to the patient depending on the location and size of the leak. POTS patients manage their symptoms with medication, diet, and preventative behaviors. Drugs such as

midodrine Midodrine, sold under the brand names ProAmatine and Orvaten among others, is an antihypotensive medication used to treat orthostatic hypotension (low blood pressure when standing) and urinary incontinence. It is taken oral administration, by m ...

fludrocortisone Fludrocortisone, sold under the brand name Florinef among others, is a corticosteroid used to treat congenital adrenal hyperplasia, postural hypotension, and adrenal insufficiency. In adrenal insufficiency, it is generally taken together wit ...

, droxidopa, and

pyridostigmine Pyridostigmine is a medication used to treat myasthenia gravis and underactive bladder. It is also used together with atropine to end the effects of neuromuscular blocking medication of the non-depolarizing type. It is also used off-label to t ...

are sometimes prescribed to help stabilize blood pressure. Patients can work with their diet to ensure they are getting the crucial amount of fluid intake and increasing their intake of salt. Both dietary changes can help prevent symptoms from occurring. Water-bolus treatment can help in times of increased orthostatic stress. Patients drink two 8 ounce glasses of cold water rapidly, which will increase standing blood pressure for a short amount of time.

Outlook

Most orthostatic headaches have a favorable outcome. POTS patients who manage their symptoms can recognize when their symptoms are occurring and prevent them from affecting their everyday life. CSF leaks are usually repaired successfully with only about 10% of patients experiencing recurrence.

Epidemiology

All different types of patients have presented orthostatic headaches as symptoms. It is slightly more prevalent in females, with a female-to-male ratio of 1.5:1. The average age of symptom presentation is around 40 years old; however, there have been cases with patients from all ages. CSF leaks are more common in patients that have connective tissue diseases such as Ehlers-Danlos syndrome.

References

{{reflist Headaches