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Multiple reversible MRI abnormalities associated with SESA syndrome
Seizure (2008) 17, 727—730
www.elsevier.com/locate/yseiz
CASE REPORT
Multiple reversible MRI abnormalities associated with SESA syndrome Jean-Marc Bugnicourt a,*, Bruno Bonnaire b, Candice Picard c, Aure ´lie Basille-Fantinato c, Olivier Godefroy d a
Department of Neurology, Amiens University Hospital, and INSERM, ERI12, University of Picardie, Amiens, France b Department of Radiology, Amiens University Hospital, France c Department of Neurology, Amiens University Hospital, France d Department of Neurology, and Laboratoire de Neurosciences Fonctionnelles et Pathologies (UMR CNRS 8160), Amiens University Hospital, France Received 17 January 2008; received in revised form 11 March 2008; accepted 21 March 2008
KEYWORDS Subacute encephalopathy with seizures in alcoholics; SESA syndrome; Complex partial status epilepticus; MRI
Summary The authors report the imaging findings in a patient with a long history of alcoholism, who presented with delirium and recurrent left hemiparesis meeting the criteria of subacute encephalopathy with seizures in alcoholics (SESA) syndrome. MRI revealed fully reversible signal intensities (T2, diffusion-weighted imaging with a decreased apparent coefficient) throughout the right hemisphere. This case suggests that the MRI characteristics of SESA syndrome resemble those of status epilepticus. # 2008 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
Introduction Subacute encephalopathy with seizures in alcoholics (SESA) syndrome is a rare epileptic entity based on typical clinical and EEG features in a setting of chronic alcoholism.1 Neuroimaging studies of these cases are rare and have not revealed any abnormalities to date. To our knowledge, this is the first report of MRI showing transient cortical abnormal* Corresponding author at: Service de Neurologie, CHU Amiens, Place Victor Pauchet, 80054 Amiens Cedex 1, France. Tel.: +33 322668240; fax: +33 322668244. E-mail address: [email protected] (J.-M. Bugnicourt).
ities identical to those associated with typical status epilepticus.
Case report A 63-year-old man presented to the Amiens University Hospital Emergency Department with a 2-day history of fluctuations of mental state and recurrent episodes of left hemiparesis. He had a history of chronic alcohol abuse (>100 g/day) and had undergone total gastrectomy in November 2006 for carcinoma, with no evidence of extragastric metastasis. He had no family history of epilepsy and no personal history of any other major systemic or infectious
1059-1311/$ — see front matter # 2008 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.seizure.2008.03.006
728
J.-M. Bugnicourt et al.
Figure 1 Brain CT scan showing an old ischaemic stroke in the territory of the right posterior cerebral artery.
diseases. On neurological examination, he was slightly confused and disoriented in time. Mild weakness of the left limbs and left hemianopia were also observed. Neurological examination did not show any signs of seizure. Laboratories studies, including complete blood count, blood urea nitrogen/creatinine, serum electrolytes and electrophoresis were within normal limits. Screening tests for drugs, syphilis, thyroid function, antinuclear antibodies, viral infection (herpes simplex virus, HIV, hepatitis B and C, cytomegalovirus and Epstein Barr virus), vitamins B1 and B12, and tumour markers were also normal. Brain computed tomography (CT) scan showed an old ischaemic stroke in
Figure 2
the territory of the right cerebral posterior artery (Fig. 1). Carotid ultrasonography showed calcified plaques in both carotid bulbs with no significant stenosis. Transthoracic and transoesophageal echocardiography were normal. Twenty-four-hour Holter monitoring did not detect any cardiac arrhythmia. On the second day of admission, the patient remained slightly confused and hemiparetic. Cerebrospinal fluid analysis was unremarkable. Delirium tremens was suspected and parenteral intravenous infusion of vitamins (B1 1 g/day; B6 500 mg/day) was started, but with no change in his neurological state despite this treatment. Brain MRI was performed 6 days after admission and showed marked gyriform cortical hyperintensities throughout the right hemisphere on diffusion-weighted imaging (DWI) (Fig. 2). These hyperintensities were extensive and did not correspond to vascular distributions. The corresponding areas of the apparent diffusion coefficient (ADC) maps were decreased in the frontal region. In the other regions, the corresponding areas on the ADC maps were too small to be analysed. Fluidattenuated inversion recovery (FLAIR) images showed cortical hyperintensity in the abnormal regions seen on DWI (Fig. 3). There was no evidence of an active central nervous system tumour. EEG showed recurrent periodic lateralised epileptiform discharges (PLEDs) involving the right hemisphere. No seizure was recorded. These findings led to the diagnosis of SESA syndrome with recurrent partial seizures of the right hemisphere, in keeping with the diagnosis of complex partial status epilepticus (CPSE). Treatment with sodium valproate (1500 mg/day by intravenous infusion) was started and led to significant improvement: 6 h later the patient was alert, and the motor deficit completely resolved 24 h later. The next day, follow-up EEG did
Diffusion-weighted MRI showing cortical hyperintensity in the right hemisphere.
Multiple reversible MRI abnormalities
Figure 3
Fluid-attenuated inversion recovery (FLAIR) MRI showing the same cortical hyperintensities.
not reveal any epileptic activity or electrographic seizures. A follow-up brain MRI performed 10 days later showed complete resolution of the previous abnormalities (Fig. 4). The patient was discharged on sodium valproate (1500 mg/day) after a normal neurological examination.
Discussion SESA syndrome is a rare entity first described in 1981 by Niedermeyer.2,3 It is characterized by a confusional state, recurrent focal or generalized motor seizures and transient focal deficits. More recently, two cases of CPSE in the setting of SESA syndrome
Figure 4
729
have been described.4,5 Although EEG abnormalities are typical with PLEDs,6 neuroimaging features in SESA syndrome are controversial. Initially, no lesion on brain imaging was reported, except for diffuse cerebral atrophy.1,7 With progress in neuroimaging, recent studies have frequently revealed the presence of old vascular lesions,5 in accordance with the higher rate of stroke in alcohol-dependent patients.8,9 These findings are consistent with the observation that alcohol precipitates PLEDs in patients with focal brain lesions.7 This is the first report of transient MRI abnormalities associated with SESA syndrome. Similar transient neuroimaging abnormalities have also been reported after generalized seizures and CPSE.10,11
Fluid-attenuated inversion recovery (FLAIR) MRI acquired 10 days later, showing reversal of the abnormalities.
730 The most frequent MRI abnormalities are focal cortical hyperintensity on T2-weighted images, FLAIR images and DWI, with reduced ADC values, increased signals of the ipsilateral middle cerebral artery on magnetic resonance angiography and leptomeningeal enhancement.11 These changes can be distinguished from other processes on MRI because they are reversible, have a gyriform pattern not restricted to a vascular distribution and lack a mass effect. The pathophysiology of these MRI changes remains unknown. Localized breakdown of the blood—brain barrier with increased permeability, hyperperfusion of the epileptic region and development of cytotoxic oedema are possible explanations. However, reduced ADC values are supposed to represent cytotoxic oedema with bound water within the cell and are believed to represent severe tissue damage progressing to necrosis.12 On the other hand, reversible reduced ADC values have also been reported after seizures and metabolic disorders, which indicates that functional disturbances can be associated with reduced ADC.10 Although these abnormalities resolve, some cerebral atrophy has been reported, indicating that diffuse brain injury may occur following status epilepticus.11 Our findings support the hypothesis that SESA syndrome may be a clinical subtype of CPSE. Indeed, CPSE is an under-recognized and heterogeneous clinical disorder, defined as a change in behaviour and/or mental processes from baseline associated with continuous epileptiform discharges on the EEG.13 CPSE has also been described in patients with cancer but with no evidence of central nervous system lesions.12 However, cancer did not play a crucial role in the present case, as there was no evidence of active cancer during the episode of altered mental state. In conclusion, SESA syndrome is a rare disorder which may be considered to be a subtype of CPSE, taking into account the characteristic clinical and EEG changes and our recent MRI findings. CPSE remains a challenging diagnosis because of its heterogeneous and subtle clinical features.
J.-M. Bugnicourt et al.
Conflict of interest The authors have no conflict of interest to declare.
References 1. Mani J, Sitajayalaksmi S, Borgohain E, Mohandas S. Subacute encephalopathy with seizures in alcoholics. Seizure 2003;12:126—9. 2. Niedermeyer E, Freund G, Krumholz A. Subacute encephalopathy with seizures in alcoholics: a clinico-electroencephalographic study. Clin Electroencephalogr 1981;12:113—29. 3. Freund G, Niedermeyer E. Subacute encephalopathy with seizures in alcoholics. Electroencephalogr Clin Neurophysiol 1981;51:53P—4P. 4. Fernandez-Torre JL, Agirre Z, Martinez-Martinez M, Rodriguez E. Subacute encephalopathy with seizures (SESA syndrome) in alcoholics: report of an unusual case. Clin EEG Neurosci 2006;37:215—8. 5. Fernandez-Torre JL, Hernandez-Hernandez JL, JimenezBonilla J, Gonzalez-Mandly A, Garcia-Regata O. Complex partial status epilepticus is an unrecognised feature in SESA syndrome: new insights into its pathophysiology. Epileptic Disord 2007;9:134—9. 6. Rothmeier J, Friese M, Willemsen F, Froescher W. Subacute encephalopathy with seizures in chronic alcoholism (SESA syndrome). Clin Electroencephalogr 2001;32:186—90. 7. Chu NS. Periodic lateralized epileptiform discharges with preexisting focal brain lesions. Role of alcohol withdrawal and anoxic encephalopathy. Arch Neurol 1980;37:551—4. 8. Klatsky AL, Armstrong MA, Sidney S, Friedman GD. Alcohol drinking and the risk of hemorrhagic stroke. Am J Cardiol 2001;88:703—6. 9. Mazzaglia G, Britton AR, Altman DR, Chenet L. Exploring the relationship between alcohol consumption and non-fatal and fatal stroke: a systematic review. Addiction 2001;93:1743— 56. 10. Aykut-Bingol C, Tekin S, Ince D, Aktan S. Reversible MRI lesions after seizures. Seizure 1997;6:237—9. 11. Lansberg MG, O’Brien MW, Norbash AM, Moseley ME, Morrell M, Albers GW. MRI abnormalities associated with partial status epilepticus. Neurology 1999;52:1021—7. 12. Hormigo A, Liberato B, Lis E, DeAngelis LM. Nonconvulsive status epilepticus in patients with cancer. Arch Neurol 2004;61:362—5. 13. Meierkord H, Holtkamp M. Non-convulsive status epilepticus in adults: clinical forms and treatment. Lancet Neurol 2007;6:329—39.
www.elsevier.com/locate/yseiz
CASE REPORT
Multiple reversible MRI abnormalities associated with SESA syndrome Jean-Marc Bugnicourt a,*, Bruno Bonnaire b, Candice Picard c, Aure ´lie Basille-Fantinato c, Olivier Godefroy d a
Department of Neurology, Amiens University Hospital, and INSERM, ERI12, University of Picardie, Amiens, France b Department of Radiology, Amiens University Hospital, France c Department of Neurology, Amiens University Hospital, France d Department of Neurology, and Laboratoire de Neurosciences Fonctionnelles et Pathologies (UMR CNRS 8160), Amiens University Hospital, France Received 17 January 2008; received in revised form 11 March 2008; accepted 21 March 2008
KEYWORDS Subacute encephalopathy with seizures in alcoholics; SESA syndrome; Complex partial status epilepticus; MRI
Summary The authors report the imaging findings in a patient with a long history of alcoholism, who presented with delirium and recurrent left hemiparesis meeting the criteria of subacute encephalopathy with seizures in alcoholics (SESA) syndrome. MRI revealed fully reversible signal intensities (T2, diffusion-weighted imaging with a decreased apparent coefficient) throughout the right hemisphere. This case suggests that the MRI characteristics of SESA syndrome resemble those of status epilepticus. # 2008 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
Introduction Subacute encephalopathy with seizures in alcoholics (SESA) syndrome is a rare epileptic entity based on typical clinical and EEG features in a setting of chronic alcoholism.1 Neuroimaging studies of these cases are rare and have not revealed any abnormalities to date. To our knowledge, this is the first report of MRI showing transient cortical abnormal* Corresponding author at: Service de Neurologie, CHU Amiens, Place Victor Pauchet, 80054 Amiens Cedex 1, France. Tel.: +33 322668240; fax: +33 322668244. E-mail address: [email protected] (J.-M. Bugnicourt).
ities identical to those associated with typical status epilepticus.
Case report A 63-year-old man presented to the Amiens University Hospital Emergency Department with a 2-day history of fluctuations of mental state and recurrent episodes of left hemiparesis. He had a history of chronic alcohol abuse (>100 g/day) and had undergone total gastrectomy in November 2006 for carcinoma, with no evidence of extragastric metastasis. He had no family history of epilepsy and no personal history of any other major systemic or infectious
1059-1311/$ — see front matter # 2008 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.seizure.2008.03.006
728
J.-M. Bugnicourt et al.
Figure 1 Brain CT scan showing an old ischaemic stroke in the territory of the right posterior cerebral artery.
diseases. On neurological examination, he was slightly confused and disoriented in time. Mild weakness of the left limbs and left hemianopia were also observed. Neurological examination did not show any signs of seizure. Laboratories studies, including complete blood count, blood urea nitrogen/creatinine, serum electrolytes and electrophoresis were within normal limits. Screening tests for drugs, syphilis, thyroid function, antinuclear antibodies, viral infection (herpes simplex virus, HIV, hepatitis B and C, cytomegalovirus and Epstein Barr virus), vitamins B1 and B12, and tumour markers were also normal. Brain computed tomography (CT) scan showed an old ischaemic stroke in
Figure 2
the territory of the right cerebral posterior artery (Fig. 1). Carotid ultrasonography showed calcified plaques in both carotid bulbs with no significant stenosis. Transthoracic and transoesophageal echocardiography were normal. Twenty-four-hour Holter monitoring did not detect any cardiac arrhythmia. On the second day of admission, the patient remained slightly confused and hemiparetic. Cerebrospinal fluid analysis was unremarkable. Delirium tremens was suspected and parenteral intravenous infusion of vitamins (B1 1 g/day; B6 500 mg/day) was started, but with no change in his neurological state despite this treatment. Brain MRI was performed 6 days after admission and showed marked gyriform cortical hyperintensities throughout the right hemisphere on diffusion-weighted imaging (DWI) (Fig. 2). These hyperintensities were extensive and did not correspond to vascular distributions. The corresponding areas of the apparent diffusion coefficient (ADC) maps were decreased in the frontal region. In the other regions, the corresponding areas on the ADC maps were too small to be analysed. Fluidattenuated inversion recovery (FLAIR) images showed cortical hyperintensity in the abnormal regions seen on DWI (Fig. 3). There was no evidence of an active central nervous system tumour. EEG showed recurrent periodic lateralised epileptiform discharges (PLEDs) involving the right hemisphere. No seizure was recorded. These findings led to the diagnosis of SESA syndrome with recurrent partial seizures of the right hemisphere, in keeping with the diagnosis of complex partial status epilepticus (CPSE). Treatment with sodium valproate (1500 mg/day by intravenous infusion) was started and led to significant improvement: 6 h later the patient was alert, and the motor deficit completely resolved 24 h later. The next day, follow-up EEG did
Diffusion-weighted MRI showing cortical hyperintensity in the right hemisphere.
Multiple reversible MRI abnormalities
Figure 3
Fluid-attenuated inversion recovery (FLAIR) MRI showing the same cortical hyperintensities.
not reveal any epileptic activity or electrographic seizures. A follow-up brain MRI performed 10 days later showed complete resolution of the previous abnormalities (Fig. 4). The patient was discharged on sodium valproate (1500 mg/day) after a normal neurological examination.
Discussion SESA syndrome is a rare entity first described in 1981 by Niedermeyer.2,3 It is characterized by a confusional state, recurrent focal or generalized motor seizures and transient focal deficits. More recently, two cases of CPSE in the setting of SESA syndrome
Figure 4
729
have been described.4,5 Although EEG abnormalities are typical with PLEDs,6 neuroimaging features in SESA syndrome are controversial. Initially, no lesion on brain imaging was reported, except for diffuse cerebral atrophy.1,7 With progress in neuroimaging, recent studies have frequently revealed the presence of old vascular lesions,5 in accordance with the higher rate of stroke in alcohol-dependent patients.8,9 These findings are consistent with the observation that alcohol precipitates PLEDs in patients with focal brain lesions.7 This is the first report of transient MRI abnormalities associated with SESA syndrome. Similar transient neuroimaging abnormalities have also been reported after generalized seizures and CPSE.10,11
Fluid-attenuated inversion recovery (FLAIR) MRI acquired 10 days later, showing reversal of the abnormalities.
730 The most frequent MRI abnormalities are focal cortical hyperintensity on T2-weighted images, FLAIR images and DWI, with reduced ADC values, increased signals of the ipsilateral middle cerebral artery on magnetic resonance angiography and leptomeningeal enhancement.11 These changes can be distinguished from other processes on MRI because they are reversible, have a gyriform pattern not restricted to a vascular distribution and lack a mass effect. The pathophysiology of these MRI changes remains unknown. Localized breakdown of the blood—brain barrier with increased permeability, hyperperfusion of the epileptic region and development of cytotoxic oedema are possible explanations. However, reduced ADC values are supposed to represent cytotoxic oedema with bound water within the cell and are believed to represent severe tissue damage progressing to necrosis.12 On the other hand, reversible reduced ADC values have also been reported after seizures and metabolic disorders, which indicates that functional disturbances can be associated with reduced ADC.10 Although these abnormalities resolve, some cerebral atrophy has been reported, indicating that diffuse brain injury may occur following status epilepticus.11 Our findings support the hypothesis that SESA syndrome may be a clinical subtype of CPSE. Indeed, CPSE is an under-recognized and heterogeneous clinical disorder, defined as a change in behaviour and/or mental processes from baseline associated with continuous epileptiform discharges on the EEG.13 CPSE has also been described in patients with cancer but with no evidence of central nervous system lesions.12 However, cancer did not play a crucial role in the present case, as there was no evidence of active cancer during the episode of altered mental state. In conclusion, SESA syndrome is a rare disorder which may be considered to be a subtype of CPSE, taking into account the characteristic clinical and EEG changes and our recent MRI findings. CPSE remains a challenging diagnosis because of its heterogeneous and subtle clinical features.
J.-M. Bugnicourt et al.
Conflict of interest The authors have no conflict of interest to declare.
References 1. Mani J, Sitajayalaksmi S, Borgohain E, Mohandas S. Subacute encephalopathy with seizures in alcoholics. Seizure 2003;12:126—9. 2. Niedermeyer E, Freund G, Krumholz A. Subacute encephalopathy with seizures in alcoholics: a clinico-electroencephalographic study. Clin Electroencephalogr 1981;12:113—29. 3. Freund G, Niedermeyer E. Subacute encephalopathy with seizures in alcoholics. Electroencephalogr Clin Neurophysiol 1981;51:53P—4P. 4. Fernandez-Torre JL, Agirre Z, Martinez-Martinez M, Rodriguez E. Subacute encephalopathy with seizures (SESA syndrome) in alcoholics: report of an unusual case. Clin EEG Neurosci 2006;37:215—8. 5. Fernandez-Torre JL, Hernandez-Hernandez JL, JimenezBonilla J, Gonzalez-Mandly A, Garcia-Regata O. Complex partial status epilepticus is an unrecognised feature in SESA syndrome: new insights into its pathophysiology. Epileptic Disord 2007;9:134—9. 6. Rothmeier J, Friese M, Willemsen F, Froescher W. Subacute encephalopathy with seizures in chronic alcoholism (SESA syndrome). Clin Electroencephalogr 2001;32:186—90. 7. Chu NS. Periodic lateralized epileptiform discharges with preexisting focal brain lesions. Role of alcohol withdrawal and anoxic encephalopathy. Arch Neurol 1980;37:551—4. 8. Klatsky AL, Armstrong MA, Sidney S, Friedman GD. Alcohol drinking and the risk of hemorrhagic stroke. Am J Cardiol 2001;88:703—6. 9. Mazzaglia G, Britton AR, Altman DR, Chenet L. Exploring the relationship between alcohol consumption and non-fatal and fatal stroke: a systematic review. Addiction 2001;93:1743— 56. 10. Aykut-Bingol C, Tekin S, Ince D, Aktan S. Reversible MRI lesions after seizures. Seizure 1997;6:237—9. 11. Lansberg MG, O’Brien MW, Norbash AM, Moseley ME, Morrell M, Albers GW. MRI abnormalities associated with partial status epilepticus. Neurology 1999;52:1021—7. 12. Hormigo A, Liberato B, Lis E, DeAngelis LM. Nonconvulsive status epilepticus in patients with cancer. Arch Neurol 2004;61:362—5. 13. Meierkord H, Holtkamp M. Non-convulsive status epilepticus in adults: clinical forms and treatment. Lancet Neurol 2007;6:329—39.