Idiopathic intracranial hypertension
| Classification according to ICD-10 | |
|---|---|
| G93.2 | Benign intracranial hypertension (pseudotumor cerebri) |
| ICD-10 online (WHO version 2019) | |
Idiopathic intracranial hypertension (IIH, literally "overpressure in the skull without a known cause") is the medical name for increased intracranial pressure without an explanatory cause. The older name pseudotumor cerebri (PTC, literally “apparent swelling of the brain”) results (in medical history) from the fact that a tumor (swelling) can also cause increased pressure inside the skull. It was coined by the German neurologist Max Nonne in 1904 under the idea that despite an increase in intracranial pressure, no tumor could be detected. Since no cell growth can be found in this clinical picture, it is also called benign intracranial hypertension (BIH, literally “benign overpressure in the skull”).
Only 1 in 100,000 is affected, making the disease a rare disease.
Clinical manifestations
The main symptom is headache or a persistent feeling of pressure in the head without any real pain sensation. The headache intensifies in a horizontal position, when sneezing and / or bending over. Tensions in the cervical and thoracic vertebrae can also be felt, which increase noticeably when the head is turned. Optical perceptions such as long-lasting “flashing” in the eyes after sudden physical exertion or abnormal sensations of geometric surfaces such as B. Tiled floors occur (floor appears curved or wavy). This is triggered by the increased nerve water pressure on the optic nerve and can also lead to dizziness and nausea . In addition, the increased pressure leads to damage to the optic nerve and, less often, to eye muscle paralysis with double vision when the abducens nerve is damaged . Occasionally, failures of other cranial nerves can lead to dizziness or tinnitus . Here, a tingling sensation on the skin and / or noise in the ear with throbbing in rhythm with the heart rate can occur. Above all, the term benign in the name of the disease is controversial, as the optic nerve can permanently die off and around 2% of them lead to - mostly one-sided - blindness.
Risk factors
The clinical picture often occurs in young women. Overweight is the strongest risk factor . Other risk factors are hormonal imbalances , iron deficiency and chronic obstructive pulmonary disease . In addition, drugs such as tetracyclines , hypervitaminosis A, cortisone therapies and retinoids are to be cited as risk factors. To prevent an increase in intracranial pressure, z. B. to avoid simultaneous administration of retinoids and tetracycline in the therapy of acne vulgaris . However, a retrospective study of 6 million patients shows that fluoroquinolone antibiotics are associated with a higher risk of developing intracranial hypertension than tetracyclines. Individual cases have also been described after taking ibuprofen .
In very rare cases, this disease also occurs in men. There is currently no really tangible explanation for this from classical neurology. Overall, the cause of the disease is still unclear.
Investigation methods
The diagnosis is confirmed by the removal of the CSF with measurement of the CSF opening pressure, imaging ( MRI ) and reflection of the fundus (evidence of a congestive papilla ). In addition, a visual field determination ( perimetry ) is necessary, since in many cases an enlargement of the blind spot and thus a visual disturbance (blurred vision) occurs.
treatment
For therapy , liquor withdrawals or diuretic drugs ( acetazolamide , furosemide ) and consistent weight reduction are used. The frequency of the lumbar punctures depends individually on the severity of the disease and the measured pressure level.
On the other hand, antibiotics , cortisone or vitamin A should be avoided; they increase intracranial pressure. In isolated cases, a shunt is surgically applied to relieve pressure. There are different shunt systems (ventriculoperitoneal, ventriculoatrial, lumboperitoneal). Furthermore, fenestration of the hard meninges (dura mater) around the optic nerve can also relieve pressure, since the optic nerve is part of the brain.
In addition, there is symptomatic therapy with painkillers, antiemetics and also antidepressants. The use of antidepressants is supposed to prevent the development of chronic headaches.
Prospect of healing
When removing cerebrospinal fluid, it can happen that previously felt tension, pain near a vertebral body or on the sciatic nerve is greatly weakened or has completely subsided immediately after the procedure. However, these sensations can return when the CSF pressure rises. Depending on the severity of the disease, those affected can be considerably restricted in their quality of life and ability to work. In some cases, spontaneous resolution of the disease has been reported. The disease is considered chronic. No reliable figures are available on mortality.
Web links
- S1- Guideline Idiopathic Intracranial Hypertension (IIH) of the German Society for Neurology. In: AWMF online (as of 2008)
Individual evidence
- ^ Medicines Commission of the German Medical Association: UAW-News - International: Intracranial pressure increase due to tetracyclines. (PDF, 49 kB) In: Dtsch Arztebl 100 (30). German Medical Association, July 25, 2003, p. A-2033 , accessed on June 17, 2010 .
- ↑ Mohit Sodhi, Claire A. Sheldon, Bruce Carleton, Mahyar Etminan: Oral fluoroquinolones and risk of secondary pseudotumor cerebri syndrome: Nested case-control study . In: Neurology . tape 89 , no. 8 , 22 August 2017, p. 792-795 , doi : 10.1212 / WNL.0000000000004247 , PMID 28754842 .
- ^ AINS - (Anaesthesiology - Intensive Care Medicine - Emergency Medicine - Pain Therapy), Vol. 4: Pain Therapy. Beck, Helge et al, 2002, p. 111 , accessed on June 18, 2010 (1st, edition): “After taking ibuprofen, some cases of pseudotumor cerebri, ... were registered” ISBN 3-13-114881-0
