From Low to High: Late - onset intracranial hypertension after treatment of spontaneous intracranial hypotension

Abstract

Sirs: An epidural blood patch (EBP) is widely accepted as a standard treatment of spontaneous intracranial hypotension (SIH). The procedure itself is safe and effective [1, 3]. However, the possibility of a rebound increase of intracranial pressure after EBP with sudden or slow onset and a maximum duration of several months [2, 4] is known. We report a patient who developed an intracranial hypertension in the third year after an EBP as treatment of SIH. In October 2002 a 35-year-old man presented with persistent headache worsening upon standing. Neurological examination was normal. He had a history of colitis ulcerosa and HLA-B 27 positive arthritis. There was no history of antecedent trauma. Cranial MRI (before lumbar puncture) showed typical signs of intracranial hypotension (Figure 1). Radioisotope cisternography revealed a bilateral cervicothoracal leak and absence of tracer over the convexities. There was a pleocytosis of 34/ll and protein elevation of 66 mg/dL in otherwise normal CSF including opening pressure. After diagnosis of SIH a lumbar EBP with 30 mL autologous blood was administered in December 2002. The orthostatic headache resolved completely. Cranial MRI returned to normal in March 2003. In April 2005 the patient experienced blurred vision on both eyes and a dull, persistent pain in his neck. Visual acuity was reduced to 0,8 on the left side, there was bilateral papilloedema (Figure 2). Cranial MRI and MR-venography were normal. Lumbar puncture opening pressure was 28 cm H2O, there were no pathological findings in CSF. After withdrawal of a relatively small volume of CSF (15 – 20 ml) and treatment with acetazolamide (250 mg twice daily) opening pressure returned to 15 cm H2O in 7/05. Papilloedema completely resolved by August 2005. Acetazolamide was stopped in November 2005. In June 2006 visual acuity and funduscopy were normal. Our patient’s orthostatic headache was due to a marked SIH and was effectively treated with an EBP. After a long period of more than two years without orthostatic headache he developed intracranial hypertension with bilateral papilloedema and reduced visual acuity. Intracranial venous thrombosis was excluded. The patient’s arthritis and colitis ulcerosa were stable in the postpatch years, he received no Cox2 inhibitors or other drugs potentially related to intracranial hypertension. He was not obese with a slightly elevated BMI of 27. Because of the absence of other definitive causes for intracranial hypertension there might be a causal link between the episode with SIH treated with an EPB and the intracranial hypertension. Our patient differs from previous reports of ‘‘rebound’’intracranial hypertension [2, 4] because of an extremely long latency after EBP. The mechanism of this lateonset rise in intracranial pressure is not clear. Mokri [4] explains the ‘‘rebound’’intracranial hypertension with an onset several weeks after an EBP and a duration of a few months by different theories. One theory is an induction of increase in CSF production in response to prolonged CSF volume depletion. Another explanation according to Mokri could be a J. Philipps (&) AE O. Busse Klinikum Minden Dept. of Neurology Friedrichstrasse 17 32427 Minden, Germany E-Mail: joerg.philipps@klinikum-minden.de Fig. 1 Sagittal T1 weighted cranial MRI (in 2002) showing massive downward displacement of the brain with descent of cerebellar tonsils resembling a chiari-1-malformation