Spontaneous intracranial hypotension occurs when cerebrospinal fluid leaks without prior trauma or medical intervention. Reduced CSF pressure leads to severe orthostatic headaches, which worsen when standing and improve when lying down.
Diagnosis relies on clinical symptoms and imaging studies such as MRI, which can reveal fluid leaks and brain sagging. Early recognition is crucial to prevent long-term neurological consequences.
Treatment may involve bed rest, hydration, and caffeine. In more severe cases, an epidural blood patch effectively seals the leak, restoring CSF pressure and relieving symptoms.
Patients with spontaneous intracranial hypotension often require long-term follow-up. Proper management ensures recovery and reduces the risk of recurrence.
| Medical Name | Spontaneous Intracranial Hypotension (SIH) |
| Affected Areas | Cerebrospinal fluid (CSF) and intracranial spaces (brain and spinal cord membranes) |
| Causes | Typically a small tear or leak in the dura mater (spinal membrane) in the spine; rarely due to connective tissue disorders, osteophytes, spinal cysts, or vascular anomalies. SIH develops spontaneously and independently of procedures like lumbar puncture or epidural anesthesia. |
| Symptoms | Postural headache that worsens upon standing (more severe when sitting or standing), neck stiffness, nausea, vomiting, dizziness, tinnitus, hearing loss, blurred vision, photophobia (light sensitivity), cognitive disturbances, rarely seizures or coma. |
| Diagnostic Methods | Magnetic Resonance Imaging (MRI): “Dural enhancement” and brain sagging (descensus cerebri) may be observed. CSF pressure measurement: Low CSF pressure can be detected via lumbar puncture.Computed Tomography Myelography: Used to determine the location of CSF leakage. |
| Treatment Methods | Conservative Treatment: Bed rest, fluid intake, caffeine therapy (oral or intravenous). Epidural Blood Patch: The patient’s own blood is injected into the epidural space to seal the leak in the dura mater.Surgical Treatment: Surgical repair targeting the leak area (rarely required). |
| Possible Complications | If left untreated, chronic headache, brain sagging, hydrocephalus, cognitive disturbances, permanent neurological deficits. |
| Prevention Methods | No direct preventive method is available; however, careful physical activity and early diagnosis are important for individuals with connective tissue diseases or spinal problems. |
| Recovery Time | Varies depending on treatment. Significant improvement is usually seen within a few days after an epidural blood patch. However, full recovery may take several weeks depending on the severity of the leak and the patient’s overall health. |
Prof. Dr. Özgür KILIÇKESMEZ Prof. Dr. Kılıçkesmez holds the Turkish Radiology Competency Certificate, the Turkish Interventional Radiology Competency Certificate, Stroke Treatment Certification, and the European Board of Interventional Radiology (EBIR). In his academic career, he won the Siemens Radiology First Prize in 2008.
Interventional Radiology / Interventional Neuroradiology
What Are the Symptoms of Spontaneous Intracranial Hypotension?
The symptoms of Spontaneous Intracranial Hypotension emerge as a result of reduced cerebrospinal fluid pressure. The most prominent symptom is orthostatic headache. The headache worsens when standing and decreases when lying down. In addition to headache, this disease may also manifest with various neurological and sensory symptoms:
- Neck Pain and Stiffness: A feeling of tension and pain in the neck region.
- Nausea and Vomiting: Stomach discomfort accompanying severe headaches.
- Tinnitus and Hearing Changes: Ringing in the ears or hearing impairment may be experienced.
- Visual Disturbances: Decreased visual clarity and double vision may occur.
- Cognitive and Behavioral Changes: Symptoms such as attention deficit and memory problems may appear.
- Dizziness and Vertigo: A sense of imbalance with changes in position.
- Photophobia and Phonophobia: Increased sensitivity to light and sound.
- Radicular Symptoms: Pain spreading to certain body parts when nerve roots are affected.
- Coma: A very rare condition, with loss of consciousness due to serious brainstem involvement.
What Are the Causes of Spontaneous Intracranial Hypotension?
The causes of Spontaneous Intracranial Hypotension are limited to CSF leakage and the associated drop in pressure. Fundamentally, tears in the dura mater surrounding the brain and spinal cord are mainly responsible. These tears most often result from trauma or may sometimes occur without any apparent cause.
- Dura Mater Tears: The most common causes include surgical interventions and medical procedures such as spinal or epidural anesthesia. Spontaneous tears are linked to genetic factors or weakness of the dura mater.
In the pathophysiology of the disease, a CSF leak leads to decreased intracranial pressure. This affects the position of the brain, causing it to descend. Brain sagging causes various structures to stretch and deform. These structures include the meninges, cranial nerves, and blood vessels. The list below explains the stretched structures and their consequences:
- Meninges: Stretching of the brain membranes directly causes headache.
- Cranial Nerves: Stretched nerves may lead to various neurological symptoms.
- Blood Vessels: Stretched vessels can cause venous congestion and thus intensify headache.
As a result, the main cause of spontaneous intracranial hypotension is abnormal tears in the dura mater. These tears directly cause CSF leaks and decreased intracranial pressure. These processes trigger brain sagging, which is the primary cause of headache. *We recommend filling out all fields so we can respond in the best possible way.
What Are the Diagnostic Methods for Spontaneous Intracranial Hypotension?
Diagnostic methods for Spontaneous Intracranial Hypotension are varied and are critical for confirming the disease. These methods are used to determine the underlying causes of orthostatic headache, which worsens when standing and improves when lying down. The diagnostic process includes various imaging techniques:
Magnetic Resonance Imaging (MRI):
- Brain MRI detects structural changes in the brain.
- Spinal MRI shows CSF leaks and related structures.
Myelography:
- CT Myelography is preferred especially for dynamic leaks.
- Digital Subtraction Myelography is effective in identifying difficult and small leaks.
Radioisotope Cisternography: Shows CSF leakage by tracking the distribution after injection of a radioactive substance.
These imaging methods play a leading role in identifying the underlying causes of symptoms and making an accurate diagnosis. In some cases, CSF pressure may also be directly measured by lumbar puncture to support the diagnosis.
How Is Spontaneous Intracranial Hypotension Treated?
Treatment of Spontaneous Intracranial Hypotension is performed through various methods. The first step is generally conservative approaches. Bed rest and increased fluid intake are recommended for most patients. These methods help relieve symptoms. Caffeine therapy can also be applied to reduce headache. Analgesics and antiemetics may be prescribed as symptomatic treatment, especially to control discomfort such as pain and nausea.
Initial Conservative Treatment:
- Bed Rest and Hydration
- Symptomatic Treatment
If these simple measures are not sufficient, more advanced treatment methods are used. Epidural Blood Patch is an invasive procedure used to seal the leak. During this procedure, the patient’s own blood is injected into the epidural space to create a clot over the leak. For patients who do not respond to initial treatment or when a clear leak site is identified, targeted Epidural Blood Patch can be applied.
Epidural Blood Patch:
- Non-targeted Epidural Blood Patch
- Targeted Epidural Blood Patch
During the treatment process, advanced imaging techniques are used to identify the exact location of the leak. This information is critical for treatment planning. If conservative methods and Epidural Blood Patch are insufficient, surgical intervention may be required. Surgery aims to directly seal the leak and is usually performed to correct a specific structural defect.
How Is a Blood Patch Applied?
Under sterile conditions, 20 cc of venous blood is drawn from the patient’s arm vein. Under angiographic guidance, it is slowly injected into the epidural space. The procedure is performed under local anesthesia while the patient is awake. It takes about 15 minutes, and after a few hours of rest, the patient is discharged home; one week of rest is recommended for full recovery.
What Are the Complications of Spontaneous Intracranial Hypotension?
The complications caused by spontaneous intracranial hypotension are quite varied and each can cause serious health problems. Displacement of the brain stretches and tears bridging veins, leading to subdural hematoma formation. This is characterized by blood accumulation between the brain membranes and can cause significant neurological deficits and require surgical intervention. Additionally, a decrease in CSF changes venous outflow from the brain, causing cerebral venous stasis and thrombosis. This sets the stage for life-threatening cerebral venous thrombosis.
Chronic CSF leaks can result in hemosiderin accumulation on the surfaces of the brain and spinal cord. This leads to superficial siderosis, which causes progressive neurological impairment such as hearing loss, ataxia, and myelopathy. Due to brain sagging, muscle atrophy in the upper limbs can also occur. This rare bibracial amyotrophy is a result of chronic mechanical stress.
- Spinal CSF Leaks: May be recurrent or persistent.
- Persistent Headaches and Orthostatic Symptoms: Some patients may continue to experience these symptoms despite treatment.
- Cognitive and Psychological Disorders: Memory and concentration difficulties, depression, and anxiety may occur.
Frequently Asked Questions
What specific genetic factors or hereditary connective tissue diseases play a role in the development of spontaneous intracranial hypotension?
Although a single “SIH gene” has not been identified, certain hereditary connective tissue diseases may increase the risk of spontaneous intracranial hypotension (SIH). Especially Marfan syndrome, some types of Ehlers-Danlos syndrome (especially hypermobility and vascular types), and Loeys-Dietz syndrome are associated with genetic defects in the production or structure of collagen or other proteins that make up the body’s connective tissues, leading to a weaker dura mater (the brain-spinal cord membrane). This structural weakness makes the membrane more susceptible to CSF leaks, increasing the risk of SIH in individuals with these syndromes compared to the general population.
What is the recurrence risk of spontaneous intracranial hypotension after treatment and are there any special precautions to prevent relapse?
The recurrence rate of spontaneous intracranial hypotension after treatment varies depending on whether the underlying cause has been fully resolved and the individual’s structural characteristics. Some studies report this rate as 10% to 30%. Recurrences typically occur due to incomplete healing of the initial leak site, formation of a new leak, or persistent dural weakness. While there is no way to completely prevent recurrence, patients are advised to avoid heavy lifting, excessive straining (such as in constipation), high-impact sports, and activities that put sudden stress on the spine. Additionally, if there is an underlying connective tissue disease, appropriate management and regular medical follow-up are important.
What should individuals diagnosed with or treated for spontaneous intracranial hypotension pay attention to in daily life, and what activities should be avoided?
During the recovery period and afterwards, patients with spontaneous intracranial hypotension (SIH) should take certain precautions in daily life. Activities that can suddenly alter CSF pressure should be avoided, such as lifting heavy objects, severe coughing or sneezing, situations requiring straining (such as constipation), and sports involving sudden and strong movements (such as weight training, certain yoga poses, jumping). Standing or sitting for long periods may increase symptoms, so regular position changes and resting when needed are beneficial. Ensuring adequate fluid intake and following the doctor’s activity restrictions help control symptoms and prevent possible recurrences.
What other medical conditions can be confused with spontaneous intracranial hypotension, and what should be considered for differential diagnosis?
The most typical symptom of spontaneous intracranial hypotension (SIH)—orthostatic headache worsening with standing—can be confused with many other conditions. Migraine, tension-type headaches, and meningitis are often considered in differential diagnosis. Additionally, Chiari malformation, sinus-related headaches, non-aneurysmal subarachnoid hemorrhage, cervical disc diseases, and even psychogenic headaches may produce similar symptoms. For accurate diagnosis, a detailed patient history, presence of typical postural headache, findings such as pachymeningeal thickening, brain sagging, and subdural fluid collections on brain and spinal MRI, and sometimes demonstration of low CSF pressure are important.
What is the long-term prognosis for patients with spontaneous intracranial hypotension and how is their quality of life affected?
The long-term prognosis for patients with spontaneous intracranial hypotension (SIH) is generally good, especially when diagnosed early and treated appropriately, as most patients achieve complete or significant recovery. However, symptoms may become chronic or be resistant to treatment in some cases. Quality of life may be adversely affected, particularly due to ongoing symptoms such as chronic headache, nausea, fatigue, and cognitive difficulties. While most individuals can return to normal activities after successful treatment, a small percentage may require lifestyle restrictions due to persistent neurological deficits or recurrent leaks. A multidisciplinary approach and individualized follow-up are important to improve long-term quality of life.
Interventional Radiology and Neuroradiology Speaclist Prof. Dr. Özgür Kılıçkesmez graduated from Cerrahpaşa Medical Faculty in 1997. He completed his specialization at Istanbul Education and Research Hospital. He received training in interventional radiology and oncology in London. He founded the interventional radiology department at Istanbul Çam and Sakura City Hospital and became a professor in 2020. He holds many international awards and certificates, has over 150 scientific publications, and has been cited more than 1500 times. He is currently working at Medicana Ataköy Hospital.
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