Episodes of Loss of Consciousness in a Patient with a Background Of

Clinical commentary with video sequences Epileptic Disord 2013; 15 (2): 175-80 Episodes of loss of consciousness in a patient with a background of cerebral venous thrombosis María Eugenia García García 1, Irene García Morales 1, José María Serratosa Fernández 2, Beatriz González Giráldez 2, Daniela Dicapua Sacoto 1, Paloma Balugo Bengoechea 3 1 Neurology Department, Hospital Clínico San Carlos 2 Neurology Department, Hospital Fundación Jiménez Díaz 3 Neurophysiology Department, Hospital Clínico San Carlos, Madrid, Spain Received June 10, 2012; Accepted April 15, 2013 ABSTRACT – Episodes of loss of consciousness are common, even in young, healthy people, and can sometimes represent a diagnostic challenge. The main diagnoses to consider are syncope and epileptic seizures, both of which may have similar symptomatology such as dizziness, loss of consciousness, falls, or “convulsive” phenomena. We present the case of a young male patient with a background of two venous thrombosis episodes (superior vena cava thrombosis and cerebral venous thrombosis), attributed to protein C and S deficiency and complicated by high intracranial pressure. A lumboperitoneal shunt was performed and anticoagulant therapy was initiated. He did not experience any medical problems until several years later, when he suddenly began to develop frequent, repetitive, transient episodes of dizziness, followed by loss of consciousness. Simulta- neous video-EEG and ECG performed during these events showed a typical pattern normally observed during syncope. Due to the absence of changes in heart rate or blood pressure, and taking into account his medical history, intracranial hypertension was considered as a possible cause of cerebral hypoperfusion. Cerebral arteriography demonstrated chronic thrombosis of all the cerebral sinuses, and the lumbar puncture an intracranial pressure of 47 mm Hg. The lumboperitoneal shunt was replaced and the patient has since not presented with any episodes. The use of simultaneous video-EEG and ECG is a reliable and efficient approach to differentiate between syncope and seizure and in this case, was the key to finding the cause of these episodes. [Published with video sequences] Key words: syncope, intracranial hypertension, venous cerebral thrombosis, loss of consciousness, seizure, video-EEG Correspondence: María Eugenia García García Neurology Department, Syncope is an abrupt, transient caused by sudden and global cere- Hospital Clínico San Carlos, C/ Profesor Martin Lagos s/n, loss of consciousness and postu- bral hypoperfusion (Kapoor, 2000). 28040 Madrid, Spain ral tone characterised by complete The pressure of blood flowing to <[email protected]> doi:10.1684/epd.2013.0572 and spontaneous recovery, which is the brain (the cerebral perfusion Epileptic Disord, Vol. 15, No. 2, June 2013 175 M.E.G. García, et al. pressure [CPP]) depends on two parameters: the a discrete tonic flexion of his superior limbs. During median arterial pressure (MAP) and the intracranial some of the episodes, he yawned, snored, perspired pressure (ICP), which is the pressure inside the skull profusely, and had bilateral mydriasis and facial red- and thus in the brain tissue and cerebrospinal fluid ness. These episodes would last several minutes and (CSF). afterwards the patient would recover consciousness Many disorders can cause syncope. The most common with short or non-existent postictal confusion. These are classified as neurally-mediated syncope (vaso- events would occur at any time of the day, whether vagal syncope), cardiac arrhythmias, and orthostatic sitting or standing, and the patient did not identify any hypotension (Brignole et al., 2004; Miller and Kruse, triggers. At first, the episodes could be aborted at onset 2005). Uncommon aetiologies, such as metabolic or by lying down, but later they became unresponsive to neurological disorders, should be considered when postural change (see video sequence). other causes have been ruled out (Jhanjee et al., 2006), The patient’s neurological and physical examinations including episodes of transient increase of intracranial were normal except for a chronic bilateral papil- pressure. loedema which was not resolved, despite the shunt. We present a case of a patient with recurrent loss of The results of various blood tests, cerebral MRI, interic- consciousness of unknown aetiology in which video- tal EEG, electrocardiography (ECG), echocardiography, electroencephalogram (VEEG) monitoring indicated a and Holter-electrocardiography did not reveal any diagnosis of syncope with uncommon aetiology. anomaly. During the tilt-test, and with the adminis- tration of nitro-glycerine, the patient experienced a loss of consciousness but reported that “this time it Case report was different because I did not experience the usual symptoms”. A 37-year-old, right-handed man was referred to the Different possible aetiologies were considered. Cere- Epilepsy Unit for the evaluation of episodes of uncon- bral MRI venography (MRV) revealed a chronic partial sciousness that had begun two months earlier. He was superior longitudinal sinus thrombosis, and several born after an uneventful pregnancy, at term, to non- EEGs did not show epileptiform activity. Nevertheless, consanguineous parents, and his development was epilepsy was considered probable and thus antiepilep- normal. There was no family history of epilepsy. He tic treatment was introduced (levetiracetam, up to had suffered two episodes of venous thrombosis at the 2000 mg a day). The patient experienced a reduction ages of 20 and 22, respectively. In the first episode, he in the number of episodes, which did not completely experienced discomfort, dyspnoea, and facial redness; disappear. VEEG monitoring recorded several events, he was diagnosed with superior vena cava thrombosis identified by the family and the patient as “his usual and was prescribed anticoagulants for six months. Two epileptic seizures”. Arterial blood pressure, heart rate, years later, he started to have headaches, diplopia, and and glycaemia were normal during the episodes. The paresis of the sixth cranial nerve. Cerebral MRI showed patient recovered without confusion from each event, a superior longitudinal sinus cerebral thrombosis and although he did not remember the episode. Simul- therefore anticoagulant treatment was reintroduced. taneous EEG showed a generalised, theta activity, Testing apparently revealed protein C and S deficiency followed by hypersynchronous, diffused, delta activity and anticoagulant therapy was therefore continued of high voltage with frontal predominance. When the indefinitely. During the following year, he remained patient recovered, this activity disappeared following a asymptomatic, however, during a routine consulta- reverse sequence (each episode lasted for 5-8 minutes) tion, a bilateral papilloedema was discovered and a (figure 1). In some of the episodes, a slow-flat-slow lumbar puncture demonstrated high intracranial pres- pattern was observed. The interictal EEG showed sure. Due to these findings, a lumboperitoneal shunt general and discrete slowing-down without any was inserted and during the subsequent 14 years, the epileptiform abnormalities. patient did not experience any medical problems. These EEG alterations are similar to the pattern At the age of 36, the patient presented with paroxys- observed during syncope but neither blood pressure tic, transient episodes of loss of consciousness. On nor heart rate changed, therefore cardiac arrhyth- presentation, these events were sporadic but they mias, vasovagal episodes and orthostatic hypotension increased in severity and frequency, such that by the were ruled out. This led us to consider other less time he came to our attention, the episodes were frequent causes of cerebral hypoperfusion, such as occurring almost daily. During these episodes, he typi- an increase in ICP. We performed a lumbar puncture cally experienced malaise and dizziness, accompanied which demonstrated an ICP of 47 mm Hg. Lum- by generalised weakness. After several seconds, he lost bar cisternography revealed that the lumboperitoneal consciousness but his eyes would remain open and the shunt was not working properly. Cerebral arterio- family reported that he stayed motionless, except for graphy showed partial and chronic thrombosis of all 176 Epileptic Disord, Vol. 15, No. 2, June 2013 Venous cerebral thrombosis and syncope A *Fp1-F3 F3-C3 C3-P3 P3-O1 *Fp2-F4 F4-C4 C4-P4 P4-O2 *Fp1-F7 F7-T3 T3-T5 T5-O1 *Fp2-F8 F8-T4 T4-T6 T6-O2 Fz-Cz Cz-Pz T1-T2 *EKG1-EKG2 B Fp1-F3 F3-C3 C3-P3 P3-O1 Fp2-F4 F4-C4 C4-P4 P4-O2 Fp1-F7 F7-T3 T3-T5 T5-O1 Fp2-F8 F8-T4 T4-T6 T6-O2 Fz-Cz Cz-Pz T1-T2 *EKG1-EKG2 Figure 1. EEG recording in an average montage during an epileptic seizure. (A) EEG showing the generalised theta activity at the beginning of the episode. (B and C) After a few seconds, this activity was followed by hypersynchronous, diffused, delta activity of high voltage with frontal predominance. (D) When the patient recovered, the activity disappeared and the EEG normalised, following a reverse sequence. There were no alterations on the ECG. Epileptic Disord, Vol. 15, No. 2, June 2013 177 M.E.G. García, et al. C Fp1-F3 F3-C3 C3-P3 P3-O1 Fp2-F4 F4-C4 C4-P4 P4-O2 Fp1-F7 F7-T3 T3-T5 T5-O1 Fp2-F8 F8-T4 T4-T6 T6-O2 Fz-Cz Cz-Pz T1-T2 *EKG1-EKG2 D Fp1-F3 F3-C3 C3-P3 P3-O1 Fp2-F4 F4-C4 C4-P4 P4-O2 Fp1-F7 F7-T3 T3-T5 T5-O1 Fp2-F8 F8-T4 T4-T6 T6-O2 Fz-Cz Cz-Pz T1-T2 *EKG1-EKG2 Figure 1. (Continued) the cerebral sinuses and of both of the internal jugular endovascular treatment was rejected. The lumbo- veins. Venous return occurred through the vertebral peritoneal shunt was replaced and since then, plexus and external facial veins (figure 2). The superior the patient has been free of episodes. A 24-hour cava vein, the brachiocephalic trunk, and the sub- period of VEEG monitoring performed one month clavian vein were also thrombosed, as seen on chest later was completely normal. Antiepileptic treat- computed tomography. ment was slowly withdrawn.

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