gms | German Medical Science

We retrospectively analyzed the data of a cohort of patients suffering from idiopathic intracranial hypertension (IIH) seen in the neuro-ophthalmolgical unit Prof. Unsöld, Düsseldorf during the years 2002–17. Out of 86 patients, 66 women, 12 men and 8 children were investigated. All children had a normal weight. Mean body mass index of the adult patients ranged at 35 (range 21–64.8). 50 Females presented with a BMI of at least 30 (BMI 28-32: N = 16; 32-36: N = 16; 36-40: N = 8; >40: N = 18).

Mean observation period was 84 months/7 years (1–251 months). 59 patients exhibited a time interval between first symptoms and diagnosis of less than <1 Jahr (in 40 patients diagnosis was made within one month, in 51 within 6 months). In the remaining 27 patients the delay until final diagnosis was up to 12 years (mean: 5 years).

The initial sign enabling correct diagnosis was papilledema, which was accidentally seen in 24/86 patients during a routine ophthalmological examination; visual obscurations were reported by additional 24 patients; in contrast, headache was the presenting sign in only 21 individuals. In total, 44 patients complained about headache, 42 patients never had experienced head ache. 61 patients presented with any kind of visual complaint. It has however to be kept in mind that the distribution of signs may be biased by referral pattern: 2/3 of patients had their first investigation by an ophthalmologist, 1/3 by a neurologist. This might explain the preponderance of ophthalmological presenting signs and symptoms.

Optic disc pathology (see presentation Prof. Unsöld).

A total of 78 patients presented with papilledema. This was bilateral in 68 patients, unilateral in 10. 43 patients presented with acute, 35with chronic disc edema. In 15 patients with chronic papilledema, there was no leakage in fluorescein angiography. 8 patients exhibited normal discs despite high intracranial pressure and enlarged optic nerve sheaths. 71/86 patients exhibited a funduscopically normal optic disc, 4 patients mild bilateral and further 8 patients a mild unilateral optic atrophy. One patient presented with uni-, two with bilateral severe optic atrophy.

Radiology (see presentation PD Dr. Lutterbey).

Therapy: 23 patients (see presentation Prof. Henkes) underwent a cranial sinus stenting procedure (7 uni-, 16 bilateral); 17 of these had a favourable clinical course, 3 improved, the remaining 3 showed a dismal development.

Shunt: A total of 13/86 underwent a shuntig procedure (see presentation Prof. Scholz): 11 ventrikulo-peritoneal (VP), 2 lumbo-peritoneal (LP) shunts; 2 patients had secondary procedures (after stent thrombosis/VP shunt failure); post operatively, 2 shunt infections and one shunt insufficiency were seen.

A conservative/medical approach was chosen in 50/86 patients. Generally, it was only effective in combination with relevant weight loss . 20 of these patients were symptom free; however, only 4 patients underwent final measurement of intracranial pressure.

14 patients were lost for follow up. 7 of those had reported traumatizing experiences during lumbar puncture, 4 had evidence of concurrent central nervous damage due to the underlying disease (see presentation Prof. Arendt).

Medical therapy was unsuccessful in 16 patients, in15 of whom no weight reduction could be achieved.

Conclusion: This retrospective analysis of 86 IIH patients with a remarkably long follow up revealed the following clinically relevant aspects:

• In 24/86 patients papilledema was an incidental finding during an ophthalmological control unrelated to any visual complaints.
• There is no correlation between degree and duration of increased intracranial pressure and degree of visual field damage.
• After manifest disc swelling, the optic disc morphology is not a reliable criterion to judge the further clinical course. Only marked disc swelling, accompanied by venous engorgement may indicate repeated raise of intracranial pressure. The absence of optic disc swelling however does not rule out elevated ICP (see presentation Prof. Unsöld).
• Even after prolonged periods/years of raised ICP the optic disc/visual field may remain unaffected. The reason for a different susceptibilty remains elusive and should be investigated in prospective studies.
• A remarkably high number of patients (23/86) did not comply with follow up investigations. 21/86 pat. refused further lumbar punctures due to traumatic experiences. This is a relevant obstacle for 1) an appropriate assessment of the different therapeutic modalities and 2) may be the reason for a substantial number of therapy failures.
• The most relevant and up to now not adequately discussed complication of IIH is the occurence of mental dysfunction (20/86). In 6 of our patients it was reversible; it seems to be more frequent in long standing history of increased intracranial pressure as parraleled by an empy sella on imaging. This feature should be investigated prospectively. (see presentation Prof. Arendt).
• Medical therapy usually is only successful in combination with relevant weight loss.
• In patients in whom weight loss is not achieved, an intense and sustained psychocial and psychotherapeutic support is crucial. Additional surgical options sould be taken into consideration. In view of the central nervous damage due to the condition and the side effects of medical therapy, invasive therapeutic stragtegies most likely should be discussed early in the disease process.
• Prothrombotic factors are commonly encounterd in affected patients; thus a thorough investigation of related disorders is warranted. The administration of inhibitors of coagulation has to be discussed on an individual basis (see presentation PD Dr. Zotz.).
• In one third of patients diagnosis was delayed by many years after occurence of first symptoms. This seems to be due to an insufficient knowledge of the disorder itselfs as well as deficits in interdisciplinary cooperation. A multidisciplinary approach to this entity both in diagnosis and patient management is needed.