Idiopathic intracranial hypertension
Idiopathic intracranial hypertension (IIH, literally " over-pressure in the skull with no known cause " ) is the medical term for increased intracranial pressure without explanatory cause. The older name pseudotumor cerebri (PTC, literally "apparent swelling of the brain " ) results ( medical history ) from the fact that a tumor ( swelling) can cause increased intracranial pressure. It was coined by the German neurologist Max Nonne in 1904 under the notion that despite Intracranial pressure no tumor is detectable. Since this disease is no cell proliferation can be found, it is also called Benign intracranial hypertension (BIH, literally " benign pressure in the skull ").
Only 1 out of 100,000 are affected, the disease is thus one of the rare diseases.
Clinical manifestations
As symptom occurs headache or sustained pressure in the head without any real pain. The headaches reinforce itself in a horizontal position, when sneezing and / or stooping. Also tension in the neck and thoracic vertebrae area can be felt, the noticeable increase particularly during rotation of the head. Another feature, optical sensations such as long-lasting " lightning " in the eyes by sudden physical exertion or discomfort geometric surfaces such as tile floors occur (soil appears curved or wavy ). This is triggered by the increased cerebrospinal fluid pressure on the optic nerve and can also cause dizziness and nausea. It also comes in the course by the increased pressure on optic nerve damage, rare for eye muscle paralysis with diplopia after lesion of the nervus abducens. Chance also failures of other cranial nerves may cause dizziness or tinnitus. Here, a tingling sensation on the skin and / or noise in the ear with throbbing in time with the heart rate. In particular, the term benign disease in the name is disputed, as the optic nerve can die permanently, and in about 2% of it comes to - mostly one-sided - blindness.
Risk Factors
The disease is most common in young women. Obesity is the strongest risk factor. Other risk factors include hormonal disorders, iron deficiency and chronic obstructive pulmonary disease. In addition, drugs such as tetracyclines, hypervitaminosis A, cortisone therapy and retinoids are to lead as risk factors. To prevent intracranial pressure, it is true, for example, in the treatment of acne vulgaris to avoid a simultaneous retinoid and Tetracyclingabe. Single cases have been reported after ingestion of ibuprofen.
In very rare cases, in men on this disease. For this purpose, there is the classic neurology no really tangible explanation. Overall, the cause of the disease to date is unclear.
Methods of investigation
The diagnosis is made by measuring the Liquorentnahme with Liquoröffnungsdrucks, imaging ( MRI) and corroborated reflection of the fundus ( evidence of papilledema ). In addition, a field determination ( perimetry) is necessary because in many cases an enlargement of the blind spot and therefore a disturbance of vision ( blurred vision ) occurs.
Treatment
For therapy Liquorentnahmen or diuretic medications ( acetazolamide, furosemide) and consistent weight loss are used. The frequency of lumbar punctures depends individually on the severity of the disease and the measured pressure level.
In contrast, antibiotics, cortisone, or vitamin A should be avoided, they increase the intracranial pressure. In isolated cases, a shunt is surgically created to Hindruckentlastung. There are various shunt systems ( ventrikuloperitoneal, ventrikuloatrial, lumboperitoneal ). Furthermore, also bring a fenestration of the dura mater ( dura mater) to the optic nerve, a pressure relief, because the optic nerve is a cranial portion.
In addition to this, symptomatic therapy with analgesics, anti-emetics and anti-depressants. The use of antidepressants to prevent the formation of chronic headaches.
Cure views
In a Liquorentnahme it is possible that previously perceived pain, stiffness, are near a vertebral body or on the sciatic nerve directly greatly weakened after the procedure or completely decayed. These sensations can but recur with increasing CSF pressure. Affected may be restricted considerably in their quality of life and ability to work according to the severity of the disease. In some cases a spontaneous decline of the disease are reported. The disease is considered chronic. For mortality are no reliable figures.