508555 research-article2014
EEGXXX10.1177/1550059413508555Wu et alClinical EEG and Neuroscience
Article
Periodic Lateralized Epileptiform Discharges Associated With Irreversible Hyperglycemic Hemichorea–Hemiballism
Clinical EEG and Neuroscience 2014, Vol. 45(4) 315–317 © EEG and Clinical Neuroscience Society (ECNS) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1550059413508555 eeg.sagepub.com
Meng-Ni Wu1,2, Diane Ruge3, Chin-Ling Tsai4, Chung-Yao Hsu1,2, Chiou-Lian Lai1,2, and Li-Min Liou1,2,4
Abstract Periodic lateralized epileptiform discharges (PLEDs) on electroencephlography (EEG) usually indicate an acute, diffuse, and severe cerebral insult. Although hyperglycemic hemichorea–hemiballisum (HCHB) and striatal hyperintensity on T1-weighted magnetic resonance (MR) images is an accepted clinical entity, PLEDs have not previously been reported. Herein, we report a 74-year-old man with hyperglycemic HCHB, hyperintense putamen on T1-MR images and PLEDs on EEG. Aggressive sugar control with neuroleptic treatment only slightly improved the severity of HCHB. We also tried titrated oral and intravenous haloperidol, clonazepam, and propranolol sequentially and in combination; however, the effects were poor. Unlike the generally reversibility of hyperglycemic HCHB, the condition was still present 6 months later. Hyperglycemia can cause HCHB and produce subcortical type-PLEDs, which may explain the findings in our patient. In conclusion, PLEDs can be found in patients with hyperglycemic HCHB and striatal hyperintensity on T1-weighted MR images, and the appearance of PLEDs may indicate an irreversible outcome. EEG should be considered in such circumstances. Keywords hemichorea–hemiballisum, hyperglycemia, striatal hyperintensity, periodic lateralized epileptiform discharge Received June 24, 2013; accepted September 19, 2013.
Introduction HCHB with contralateral striatal hyperintensity on T1-MRI is an accepted clinical entity. However, PLEDs have not previously been reported in patients with hyperglycemic HCHB.
Case Report A 74-year-old man with prolonged, uncontrolled diabetes mellitus presented to our hospital with an acute episode of right limb HCHB for more than 1 month. Before this presentation he had been well without any disabilities. His past medical history revealed no significant diseases except for diabetes mellitus. Movements persisted throughout the day and disappeared during sleep. Cranial MRI showed hyperintensity over the left putamen on T1-MRI with no signal change on T2-MRI 2 days after admission. The initial blood glucose, osmolality level, and HbA1C were 312 mg/dL, 294 mmol/L, and 9.0%, respectively. Other laboratory findings, including thyroid profile and serum ceruloplasmin were within normal limits. Interestingly, EEG on admission showed PLEDs over the left hemisphere, which is the ipsilateral side of the hyperintense putamen on MRI and contralateral side of HCHB (Figure 1). When his
blood sugar had been controlled to around 150 to 200 mg/dL, HCHB improved slightly. We tried titrated oral and intravenous haloperidol, clonazepam, and propranolol sequentially and in combination; however, the effects were poor. The PLEDs were then replaced by sporadic theta waves, and the HCHB still existed. Six months later, when his blood sugar had been controlled to around 100 to 150 mg/dL, the HCHB was still present.
1
Department of Neurology, Kaohsiung Municipal HsiaoKang Hospital, Kaohsiung Meidcal University, Kaohsiung, Taiwan 2 Department of Neurology, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan 3 Sobell Department of Motor Neuroscience and Movement Disorders, UCLInstitute of Neurology, University College London London, UK 4 Department of Neurology, Kaohsiung Municipal Hsiaokang Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Corresponding Author: Li-Min Liou, Department of Neurology, Kaohsiung Municipal HsiaoKang Hospital, Kaohsiung Meidcal University, No. 482,Shanming Rd., Hsiaogang Dist., Kaohsiung City 812, Taiwan, ROC. Email: [email protected] Full-color figures are available online at http://eeg.sagepub.com
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Figure 1. EEG showed PLEDs over the left hemisphere, especially the frontocentral area on admission (about 1 month after onset). The PLEDs had a relatively stereotypic morphology and short duration (about 240 ms). The frequency was about 0.5 to 1.0 Hz (sensitivity, 7 µV/mm; LoFil, 0.5 Hz; HiFil, 70 Hz).
Discussion PLEDs are believed to be associated with acute, large, and destructive cortical lesions.1 However, subcortical lesions and metabolic disorders should be emphasized in patients with PLEDs. One study reported that most patients with PLEDs are associated with both cortical and adjacent subcortical lesions (64.7%), while cortical and subcortical lesions only are found in 11.3% and 4.2% respectively.2 Furthermore, metabolic disorders are concurrently found in patients with PLEDs3 accounting for 15% of the primary etiology.4 Basal ganglia are very sensitive to metabolic changes. Generalized periodic EEG patterns of severe metabolic diseases may originate in the basal ganglia, with a wide cortical representation achieved via a similar mechanism to subcortical PLEDs.5 The periodic complex morphology in our case was relatively stereotyped and of short duration, like that of subcortical PLEDs.5 Hyperglycemia can cause transient metabolic impairment within the basal ganglia, and this has been considered to be a possible cause of HCHB6 and proved to be associated with PLEDs.3 This may explain why PLEDs were found in our patient with hyperglycemic HCHB. Although up to 80% of patients with PLEDs may develop seizures,7 the “HCHB” presentation in our case was more like a movement disorder than a seizure disorder, as it disappeared when asleep and appeared immediately on awakening. When PLEDs disappeared, HCHB still persisted during hyperglycemia. PLEDs predict a poor prognosis with death and dependence in 24.14% and 33.10% of patients, respectively.7 Unlike the general reversibility of HCHB, the hyperglycemic HCHB in our case was irreversible after normalization of hyperglycemia, even six months later. Cerebral events caused by hyperglycemia can be irreversible during a prolonged and untreated clinical course, and may cause further injury to the basal
ganglia8 and the appearance of PLEDs. We hypothesize that the reversibility of HCHB is based on the appearance of PLEDs, which reflects the degree of damage to the basal ganglia.
Conclusion PLEDs can be found in patients with hyperglycemic HCHB and striatal hyperintensity on T1-weighted MRI, and this may indicate an irreversible outcome. EEG should be considered in these circumstances. Declaration of Conflicting Interests The author(s) declared no conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Raroque HG Jr, Purdy P. Lesion localization in periodic lateralized epileptiform discharges: gray or white matter. Epilepsia. 1995;36:58-62. 2. Gurer G, Yemisci M, Saygi S, Ciger A. Structural lesions in periodic lateralized epileptiform discharges (PLEDs). Clin EEG Neurosci. 2004;35:88-93. 3. Neufeld MY, Vishnevskaya S, Treves TA, et al. Periodic lateralized epileptiform discharges (PLEDs) following stroke are associated with metabolic abnormalities. Electroencephalogr Clin Neurophysiol. 1997;102:295-298. 4. Snodgrass SM, Tsuburaya K, Ajmone-Marsan C. Clinical significance of periodic lateralized epileptiform discharges: relationship with status epilepticus. J Clin Neurophysiol. 1989;6:159-172.
Wu et al 5. Kalamangalam GP, Diehl B, Burgess RC. Neuroimaging and neurophysiology of periodic lateralized epileptiform discharges: observations and hypotheses. Epilepsia. 2007;48: 1396-1405. 6. Pisani A, Diomedi M, Rum A, et al. Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism. J Neurol Neurosurg Psychiatry. 2005;76: 1717-1719.
317 7. García-Morales I, García MT, Galán-Dávila L, et al. Periodic lateralized epileptiform discharges: etiology, clinical aspects, seizures, and evolution in 130 patients. J Clin Neurophysiol. 2002;19:172-177. 8. Tung CS, Guo YC, Lai CL, Liou LM. Irreversible striatal neuroimaging abnormalities secondary to prolonged, uncontrolled diabetes mellitus in the setting of progressive focal neurological symptoms. Neurol Sci. 2010;31:57-60.
EEGXXX10.1177/1550059413508555Wu et alClinical EEG and Neuroscience
Article
Periodic Lateralized Epileptiform Discharges Associated With Irreversible Hyperglycemic Hemichorea–Hemiballism
Clinical EEG and Neuroscience 2014, Vol. 45(4) 315–317 © EEG and Clinical Neuroscience Society (ECNS) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1550059413508555 eeg.sagepub.com
Meng-Ni Wu1,2, Diane Ruge3, Chin-Ling Tsai4, Chung-Yao Hsu1,2, Chiou-Lian Lai1,2, and Li-Min Liou1,2,4
Abstract Periodic lateralized epileptiform discharges (PLEDs) on electroencephlography (EEG) usually indicate an acute, diffuse, and severe cerebral insult. Although hyperglycemic hemichorea–hemiballisum (HCHB) and striatal hyperintensity on T1-weighted magnetic resonance (MR) images is an accepted clinical entity, PLEDs have not previously been reported. Herein, we report a 74-year-old man with hyperglycemic HCHB, hyperintense putamen on T1-MR images and PLEDs on EEG. Aggressive sugar control with neuroleptic treatment only slightly improved the severity of HCHB. We also tried titrated oral and intravenous haloperidol, clonazepam, and propranolol sequentially and in combination; however, the effects were poor. Unlike the generally reversibility of hyperglycemic HCHB, the condition was still present 6 months later. Hyperglycemia can cause HCHB and produce subcortical type-PLEDs, which may explain the findings in our patient. In conclusion, PLEDs can be found in patients with hyperglycemic HCHB and striatal hyperintensity on T1-weighted MR images, and the appearance of PLEDs may indicate an irreversible outcome. EEG should be considered in such circumstances. Keywords hemichorea–hemiballisum, hyperglycemia, striatal hyperintensity, periodic lateralized epileptiform discharge Received June 24, 2013; accepted September 19, 2013.
Introduction HCHB with contralateral striatal hyperintensity on T1-MRI is an accepted clinical entity. However, PLEDs have not previously been reported in patients with hyperglycemic HCHB.
Case Report A 74-year-old man with prolonged, uncontrolled diabetes mellitus presented to our hospital with an acute episode of right limb HCHB for more than 1 month. Before this presentation he had been well without any disabilities. His past medical history revealed no significant diseases except for diabetes mellitus. Movements persisted throughout the day and disappeared during sleep. Cranial MRI showed hyperintensity over the left putamen on T1-MRI with no signal change on T2-MRI 2 days after admission. The initial blood glucose, osmolality level, and HbA1C were 312 mg/dL, 294 mmol/L, and 9.0%, respectively. Other laboratory findings, including thyroid profile and serum ceruloplasmin were within normal limits. Interestingly, EEG on admission showed PLEDs over the left hemisphere, which is the ipsilateral side of the hyperintense putamen on MRI and contralateral side of HCHB (Figure 1). When his
blood sugar had been controlled to around 150 to 200 mg/dL, HCHB improved slightly. We tried titrated oral and intravenous haloperidol, clonazepam, and propranolol sequentially and in combination; however, the effects were poor. The PLEDs were then replaced by sporadic theta waves, and the HCHB still existed. Six months later, when his blood sugar had been controlled to around 100 to 150 mg/dL, the HCHB was still present.
1
Department of Neurology, Kaohsiung Municipal HsiaoKang Hospital, Kaohsiung Meidcal University, Kaohsiung, Taiwan 2 Department of Neurology, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan 3 Sobell Department of Motor Neuroscience and Movement Disorders, UCLInstitute of Neurology, University College London London, UK 4 Department of Neurology, Kaohsiung Municipal Hsiaokang Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Corresponding Author: Li-Min Liou, Department of Neurology, Kaohsiung Municipal HsiaoKang Hospital, Kaohsiung Meidcal University, No. 482,Shanming Rd., Hsiaogang Dist., Kaohsiung City 812, Taiwan, ROC. Email: [email protected] Full-color figures are available online at http://eeg.sagepub.com
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Clinical EEG and Neuroscience 45(4)
Figure 1. EEG showed PLEDs over the left hemisphere, especially the frontocentral area on admission (about 1 month after onset). The PLEDs had a relatively stereotypic morphology and short duration (about 240 ms). The frequency was about 0.5 to 1.0 Hz (sensitivity, 7 µV/mm; LoFil, 0.5 Hz; HiFil, 70 Hz).
Discussion PLEDs are believed to be associated with acute, large, and destructive cortical lesions.1 However, subcortical lesions and metabolic disorders should be emphasized in patients with PLEDs. One study reported that most patients with PLEDs are associated with both cortical and adjacent subcortical lesions (64.7%), while cortical and subcortical lesions only are found in 11.3% and 4.2% respectively.2 Furthermore, metabolic disorders are concurrently found in patients with PLEDs3 accounting for 15% of the primary etiology.4 Basal ganglia are very sensitive to metabolic changes. Generalized periodic EEG patterns of severe metabolic diseases may originate in the basal ganglia, with a wide cortical representation achieved via a similar mechanism to subcortical PLEDs.5 The periodic complex morphology in our case was relatively stereotyped and of short duration, like that of subcortical PLEDs.5 Hyperglycemia can cause transient metabolic impairment within the basal ganglia, and this has been considered to be a possible cause of HCHB6 and proved to be associated with PLEDs.3 This may explain why PLEDs were found in our patient with hyperglycemic HCHB. Although up to 80% of patients with PLEDs may develop seizures,7 the “HCHB” presentation in our case was more like a movement disorder than a seizure disorder, as it disappeared when asleep and appeared immediately on awakening. When PLEDs disappeared, HCHB still persisted during hyperglycemia. PLEDs predict a poor prognosis with death and dependence in 24.14% and 33.10% of patients, respectively.7 Unlike the general reversibility of HCHB, the hyperglycemic HCHB in our case was irreversible after normalization of hyperglycemia, even six months later. Cerebral events caused by hyperglycemia can be irreversible during a prolonged and untreated clinical course, and may cause further injury to the basal
ganglia8 and the appearance of PLEDs. We hypothesize that the reversibility of HCHB is based on the appearance of PLEDs, which reflects the degree of damage to the basal ganglia.
Conclusion PLEDs can be found in patients with hyperglycemic HCHB and striatal hyperintensity on T1-weighted MRI, and this may indicate an irreversible outcome. EEG should be considered in these circumstances. Declaration of Conflicting Interests The author(s) declared no conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Raroque HG Jr, Purdy P. Lesion localization in periodic lateralized epileptiform discharges: gray or white matter. Epilepsia. 1995;36:58-62. 2. Gurer G, Yemisci M, Saygi S, Ciger A. Structural lesions in periodic lateralized epileptiform discharges (PLEDs). Clin EEG Neurosci. 2004;35:88-93. 3. Neufeld MY, Vishnevskaya S, Treves TA, et al. Periodic lateralized epileptiform discharges (PLEDs) following stroke are associated with metabolic abnormalities. Electroencephalogr Clin Neurophysiol. 1997;102:295-298. 4. Snodgrass SM, Tsuburaya K, Ajmone-Marsan C. Clinical significance of periodic lateralized epileptiform discharges: relationship with status epilepticus. J Clin Neurophysiol. 1989;6:159-172.
Wu et al 5. Kalamangalam GP, Diehl B, Burgess RC. Neuroimaging and neurophysiology of periodic lateralized epileptiform discharges: observations and hypotheses. Epilepsia. 2007;48: 1396-1405. 6. Pisani A, Diomedi M, Rum A, et al. Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism. J Neurol Neurosurg Psychiatry. 2005;76: 1717-1719.
317 7. García-Morales I, García MT, Galán-Dávila L, et al. Periodic lateralized epileptiform discharges: etiology, clinical aspects, seizures, and evolution in 130 patients. J Clin Neurophysiol. 2002;19:172-177. 8. Tung CS, Guo YC, Lai CL, Liou LM. Irreversible striatal neuroimaging abnormalities secondary to prolonged, uncontrolled diabetes mellitus in the setting of progressive focal neurological symptoms. Neurol Sci. 2010;31:57-60.
