Cerebrospinal Fluid Disorders
Idiopathic Intracranial Hypertension
Idiopathic intracranial hypertension (IIH) occurs when the pressure inside your skull increases. In severe cases, this can cause headaches and vision loss. This condition is more common in patients who are overweight, female and of childbearing age. The cause of IIH is unknown.
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The most common symptoms are headaches and impaired vision. Other symptoms include nausea and vomiting, temporary episodes of blindness, double vision, and associated neck and shoulder pain. The symptoms generally are persistent but can resolve spontaneously and recur later.
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Diagnosis of IIH includes brain imaging with an MRI. This test excludes other causes of increased pressure and can demonstrate other associated conditions such as narrowing of the venous channels in the skull. A confirmed diagnosis of IIH also requires a lumbar puncture or spinal tap to confirm the diagnosis and assessment of the eyes and vision by an ophthalmologist to identify swelling of the optic nerve.
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Treatment may be necessary in cases of worsening vision or debilitating headaches. Medical treatment is first line using diuretics such as acetazolamide that reduces the production of cerebrospinal fluid and may improve symptoms. If the condition is severe or progressive surgical procedures to reduce the pressure may be required such as insertion of a shunt in the fluid spaces in the brain to divert excess fluid to the abdominal cavity or stenting of narrowed venous channels in the skull to facilitate blood flow out of the skull cavity. In cases of isolated deteriorating vision a procedure called an optic nerve sheath fenestration may be required where an ophthalmologist cuts an opening into the covering of the optic nerve to help drain excess cerebrospinal fluid.
Spontaneous Intracranial Hypotension
Spontaneous intracranial hypotension (SIH) is a rare condition characterised by low cerebrospinal fluid (CSF) pressure usually without a clear causative event. This condition typically occurs because of a CSF leak which leads to a reduction in CSF volume and pressure. Other rare causes of this condition include an abnormal connection between the CSF and a spinal vein called a cerebrospinal fluid venous fistula.
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Most commonly people report a headache that is worse on being upright and improving when lying down. Other common symptoms include neck pain and stiffness, dizziness and vertigo, ringing in the ears (tinnitus), nausea, light sensitivity, difficulty concentrating and thinking. In rare cases there may be signs of increased intracranial pressure that may be associated.
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Initial diagnosis of SIH is patient on patient history and description of the headache. MRI of the brain and spine demonstrates changes related to brain sagging from the low pressure and in some circumstances can identify the potential leak site or a collection of CSF. A myelogram may be necessary to detect the leak or CSF-venous fistula and this is performed by inserting a spinal needle and injecting contrast into the CSF fluid space. A spinal tap or lumbar puncture may also be necessary to identify a low opening pressure and support the clinical diagnosis.
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Initial treatment is conservative and includes bed rest, hydration and increased caffeine intake to increase the CSF pressure. Epidural blood patching may be needed where the patient’s own blood is injected around the spinal membrane sac prompting a clotting reaction and potentially sealing the leak. In cases of leaks that are not responsive to patching there may be a need for an open surgical repair to close the leak site. In rare cases of CSF-venous fistulae endovascular embolization of the abnormal vein or surgical clipping may be required to close the abnormal connection.
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