| Orthostatic headache | |
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| Location of cerebral spinal fluid within the brain |
Orthostatic headache is a medical condition in which a person develops a headache while vertical and the headache is relieved when horizontal. [1] [2] 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. [3]
The most common cause of orthostatic headache is low cerebrospinal fluid pressure, due to a spontaneous cerebrospinal fluid leak, a traumatic cerebrospinal fluid leak, or a post-dural-puncture leak. [4] It is also occasionally the most prominent symptom of postural orthostatic tachycardia syndrome (POTS). [3] Distinguishing POTS from a cerebrospinal fluid leak can be difficult, because the defining symptom of POTS, positional tachycardia, also occurs in some people with cerebrospinal fluid leaks. [3] Furthermore, both POTS and cerebrospinal fluid leaks are sometimes present in the same person, especially in people with Ehlers–Danlos syndrome. [3]
Other causes include colloid cysts and possibly connective tissue disorders. [5] It may occur as a complication of decompressive surgery for Chiari malformation or decompressive craniectomies for cerebral edema. [5]
Cerebrospinal Fluid (CSF) Leak causes loss of CSF 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 pain is what results in a headache, 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. [3]
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 saggital views, [6] pituitary hyperemia, and sagging of the brain. [3]
Another cause of orthostatic headaches is postural orthostatic tachycardia syndrome (POTS), a form of dysautonomia, 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. [3]
Cerebrospinal Fluid leaks can be managed short term with bed rest and plentiful hydration. They can then be treated with an epidural blood patch (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. [3]
POTS patients manage their symptoms with medication, diet, and preventative behaviors. Drugs such as midodrine, fludrocortisone, droxidopa, and pyridostigmine 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. [7]
Most orthostatic headaches have a favorable outcome. [3] POTS patients who manage their symptoms can recognize when their symptoms are occurring and prevent them from affecting their everyday life. [7] CSF leaks are usually repaired successfully with only about 10% of patients experiencing recurrence. [3]
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. [3]
Cerebrospinal fluid (CSF) is a clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord of all vertebrates.
Orthostatic hypotension, also known as postural hypotension, is a medical condition wherein a person's blood pressure drops when standing up or sitting down. The drop in blood pressure may be sudden, within 3 minutes or gradual. It is defined as a fall in systolic blood pressure of at least 20 mmHg or diastolic blood pressure of at least 10 mmHg when a person assumes a standing position. It occurs predominantly by delayed constriction of the lower body blood vessels, which is normally required to maintain adequate blood pressure when changing the position to standing. As a result, blood pools in the blood vessels of the legs for a longer period, and less is returned to the heart, thereby leading to a reduced cardiac output and inadequate blood flow to the brain.
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. CMs can cause headaches, difficulty swallowing, vomiting, dizziness, neck pain, unsteady gait, poor hand coordination, numbness and tingling of the hands and feet, and speech problems. Less often, people may experience ringing or buzzing in the ears, weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis.
Lumbar puncture (LP), also known as a spinal tap, is a medical procedure in which a needle is inserted into the spinal canal, most commonly to collect cerebrospinal fluid (CSF) for diagnostic testing. The main reason for a lumbar puncture is to help diagnose diseases of the central nervous system, including the brain and spine. Examples of these conditions include meningitis and subarachnoid hemorrhage. It may also be used therapeutically in some conditions. Increased intracranial pressure is a contraindication, due to risk of brain matter being compressed and pushed toward the spine. Sometimes, lumbar puncture cannot be performed safely. It is regarded as a safe procedure, but post-dural-puncture headache is a common side effect if a small atraumatic needle is not used.
Dysautonomia or autonomic dysfunction is a condition in which the autonomic nervous system (ANS) does not work properly. This may affect the functioning of the heart, bladder, intestines, sweat glands, pupils, and blood vessels. Dysautonomia has many causes, not all of which may be classified as neuropathic. A number of conditions can feature dysautonomia, such as Parkinson's disease, multiple system atrophy, dementia with Lewy bodies, Ehlers-Danlos syndromes, autoimmune autonomic ganglionopathy and autonomic neuropathy, HIV/AIDS, autonomic failure, and postural orthostatic tachycardia syndrome.
Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury (mmHg) and at rest, is normally 7–15 mmHg for a supine adult. The body has various mechanisms by which it keeps the ICP stable, with CSF pressures varying by about 1 mmHg in normal adults through shifts in production and absorption of CSF.
Dura mater is a thick membrane made of dense irregular connective tissue that surrounds the brain and spinal cord. It is the outermost of the three layers of membrane called the meninges that protect the central nervous system. The other two meningeal layers are the arachnoid mater and the pia mater. The dura surrounds the brain and the spinal cord. It envelops the arachnoid mater, which is responsible for keeping in the cerebrospinal fluid. It is derived primarily from the neural crest cell population, with postnatal contributions of the paraxial mesoderm.
A subdural hematoma (SDH) is a type of bleeding in which a collection of blood—usually associated with a traumatic brain injury—gathers between the inner layer of the dura mater and the arachnoid mater of the meninges surrounding the brain. It usually results from tears in bridging veins that cross the subdural space.
Orthostatic intolerance (OI) is the development of symptoms when standing upright that are relieved when reclining. There are many types of orthostatic intolerance. OI can be a subcategory of dysautonomia, a disorder of the autonomic nervous system occurring when an individual stands up. Some animal species with orthostatic blood pressure have evolved to cope with orthostatic disturbances.
Postural orthostatic tachycardia syndrome (POTS) is a condition in which a change from lying to standing causes an abnormally large increase in heart beat rate. This occurs with symptoms that may include lightheadedness, trouble thinking, blurred vision, or weakness. Other commonly associated conditions include Ehlers–Danlos syndrome, mast cell activation syndrome, irritable bowel syndrome, insomnia, chronic headaches, chronic fatigue syndrome, and fibromyalgia. It can be treated with lifestyle changes such as increasing fluid and salt intake, compression stockings, rising slowly after lying down, avoiding prolonged bedrest, and medication.
A thunderclap headache is a headache that is severe and has a sudden onset. It is defined as a severe headache that takes seconds to minutes to reach maximum intensity. Although approximately 75% are attributed to "primary" headaches—headache disorder, non-specific headache, idiopathic thunderclap headache, or uncertain headache disorder—the remainder are secondary to other causes, which can include some extremely dangerous acute conditions, as well as infections and other conditions. Usually, further investigations are performed to identify the underlying cause.
A tilt table test (TTT), occasionally called upright tilt testing (UTT), is a medical procedure often used to diagnose dysautonomia or syncope. Patients with symptoms of dizziness or lightheadedness, with or without a loss of consciousness (fainting), suspected to be associated with a drop in blood pressure or positional tachycardia are good candidates for this test.
A subdural hygroma (SDG) is a collection of cerebrospinal fluid (CSF), without blood, located under the dural membrane of the brain. Most subdural hygromas are believed to be derived from chronic subdural hematomas. They are commonly seen in elderly people after minor trauma but can also be seen in children following infection or trauma. One of the common causes of subdural hygroma is a sudden decrease in pressure as a result of placing a ventricular shunt. This can lead to leakage of CSF into the subdural space especially in cases with moderate to severe brain atrophy. In these cases the symptoms such as mild fever, headache, drowsiness and confusion can be seen, which are relieved by draining this subdural fluid.
Dural ectasia is widening or ballooning of the dural sac surrounding the spinal cord. This usually occurs in the lumbosacral region, as this is where the cerebrospinal fluid pressure is greatest, but the spinal canal can be affected in any plane.
Tolosa–Hunt syndrome is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.
Dopamine beta (β)-hydroxylase deficiency is a condition involving inadequate dopamine beta-hydroxylase. It is characterized by increased amounts of serum dopamine and the absence of norepinephrine (NE) and epinephrine. Dopamine is released, as a false neurotransmitter, in place of norepinephrine. Other names for norepinephrine include noradrenaline (NA) and noradrenalin. This condition is also sometimes referred to as "norepinephrine deficiency". Researchers of disorders such as schizophrenia are interested in studying this disorder, as patients with these specific diseases can have an increase in the amount of dopamine in their system and yet do not show other symptoms of DβH deficiency.
A cerebrospinal fluid leak is a medical condition where the cerebrospinal fluid (CSF) surrounding the brain or spinal cord leaks out of one or more holes or tears in the dura mater. A cerebrospinal fluid leak can be either cranial or spinal, and these are two different disorders. A spinal CSF leak can be caused by one or more meningeal diverticula or CSF-venous fistulas not associated with an epidural leak.
Tarlov cysts, are type II innervated meningeal cysts, cerebrospinal-fluid-filled (CSF) sacs most frequently located in the spinal canal of the sacral region of the spinal cord (S1–S5) and much less often in the cervical, thoracic or lumbar spine. They can be distinguished from other meningeal cysts by their nerve-fiber-filled walls. Tarlov cysts are defined as cysts formed within the nerve-root sheath at the dorsal root ganglion. The etiology of these cysts is not well understood; some current theories explaining this phenomenon have not yet been tested or challenged but include increased pressure in CSF, filling of congenital cysts with one-way valves, inflammation in response to trauma and disease. They are named for American neurosurgeon Isadore Tarlov, who described them in 1938.
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing, is a rare headache disorder that belongs to the group of headaches called trigeminal autonomic cephalalgia (TACs). Symptoms include excruciating burning, stabbing, or electrical headaches mainly near the eye and typically these sensations are only on one side of the body. The headache attacks are typically accompanied by cranial autonomic signs that are unique to SUNCT. Each attack can last from five seconds to six minutes and may occur up to 200 times daily.
Lymphocytic pleocytosis is an abnormal increase in the amount of lymphocytes in the cerebrospinal fluid (CSF). It is usually considered to be a sign of infection or inflammation within the nervous system, and is encountered in a number of neurological diseases, such as pseudomigraine, Susac's syndrome, and encephalitis. While lymphocytes make up roughly a quarter of all white blood cells (WBC) in the body, they are generally rare in the CSF. Under normal conditions, there are usually less than 5 white blood cells per µL of CSF. In a pleocytic setting, the number of lymphocytes can jump to more than 1,000 cells per µL. Increases in lymphocyte count are often accompanied by an increase in cerebrospinal protein concentrations in addition to pleocytosis of other types of white blood cells.