Delayed subdural hematoma and cerebral venous thrombosis in a patient with spontaneous intracranial hypotension

Abstract

Spontaneous low cerebrospinal fluid (CSF) pressure syndrome, first described by Schaltenbrand in 1938 [1], is an uncommon but increasingly recognized disease. This syndrome is defined by CSF pressures of 70 mmH2O or less in the lateral position and no history of injuries. Severe complications of this syndrome such as subdural hemorrhage (SDH) and cerebral venous thromboses (CVT) have been rarely reported. In this letter, we discuss a case of this syndrome accompanied by both SDH and CVT. A 34-year-old man presented with diffuse holocranial headache and no history of injuries on 27 September 2007. The pain resolved on recumbency and occurred while sitting and standing, and was relieved in 1 day. The patient again developed orthostatic headache 6 days later which was more severe than the first headache. A computed tomography (CT) scan indicated abnormal superior sagittal sinus (SSS) and straight sinus (Fig. 1a). The patient’s neurological examination showed only neck stiffness. The opening pressure of CSF in the lateral position was 0 mm H2O. Complete blood count (CBC) and hemoglobin electrophoresis were normal. Antiphospholipid, anticardiolipin antibodies, antinuclear antibody, anti-DNA antibodies, and neutrophil cytoplasmic antibodies (ANCA) were negative. There were no laboratory evidence found for protein S and C deficiencies, antithrombin III deficiency and Leiden factor V mutation. Homocystine was also in normal range. Magnetic resonance imaging (MRI) of the brain showed bilateral SDH (Fig. 2a) and diffuse pachymeningeal enhancement on enhanced-gadolinium. T1-weighted images (Fig. 2b). In addition, the results of magnetic resonance venography (MRV) of the brain implied thrombosis in the inferior sagittal sinus (ISS) and straight sinus (Fig. 3a, b). The MR venography 2D-TOF angiographic technique was used. Anticoagulant therapy was considered. When the patient became drowsy 1 week later, an urgent CT scan was obtained, and it showed greater SDH (Fig. 1b). Hematomas were surgically evacuated immediately. After being treated conservatively with analgesics, anticoagulants, hydration, and bed rest, the patient showed an obvious increase in responsiveness, and his headache significantly improved 3 days later. Pain was completely resolved and the neck stiffness disappeared after 3 weeks. At that time, CT and MRV both indicated the recanalization of the cerebral venae (Figs. 1c, 3c, d). The patient was followed regularly for 3 years without incident, and he was able to work and live normally. Because of the absence of any history of trauma or lumbar puncture, spontaneous low CSF pressure syndrome in this patient can be confirmed on the basis of orthostatic headache, low CSF pressure, and diffuse meningeal enhancement observed on MR images. Low CSF pressure, also known as intracranial hypotension (IH), is closely associated with CSF leaks [2]. Although radioisotope cisternography is a valuable technique for localizing any leakages, it may worsen the IH [3], and therefore, we treated him conservatively instead of further examination. In this patient, the occurrence and recovery of IH preceded the SDH and CVT. We believe that there may be a causal relationship between them. It has been reported that 10% of patients with IH had SDH [4]. The dura is relatively fragile and weak because the cell layer bordering the dura lacks collagen fibers. The Y.-T. Mao Q. Dong J.-H. Fu (&) Department of Neurology Huanshan Hospital, Fudan University, Shanghai 200040, China e-mail: neverbad999@yahoo.com.cn