- Email: [email protected]
1.244 CHOREOATHETOSIS CAUSED BY NUCLEUS CAUDATE ISCHEMIA
Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
geographical distribution, gender, age of onset and age of diagnosis as well as clinical features of all patients were summarized. Results: In total, there were 29 case reports reporting in total 39 NA cases distributed in 15 provinces of China. The prominent symptoms include movement disorder, dystonia, orofacial dyskinesias, dysarthria, dysphagia, epilepsy, and psychiatric symptoms. Diagnosis of NA was made mainly according to the clinical manifestation, percentage of acanthocytes in peripheral blood, as well as neuroimaging results and EEG. Because of limitations in diagnostic methodology in China, the diagnosis of NA can not be further classified into NA diseases ChAc, MLS or NBIA. Conclusion: There were fewer NA cases reported in China as expected considering its estimated prevalence. This might be due to limitations in the physicians’ awareness and diagnostic methodology for NA. Thus, advanced diagnostic methods for screening and classification of NA syndromes are required in China. 1.242 NEUROMUSCULAR FINDINGS IN CHOREA-ACANTHOCYTOSIS J. Liu1 , T. Arzberger2 , A. Radunovic3 , U. Pohl4 , S. Mohiddin5 , Y. Kageyama6 , R. Kiehl7 , T. Lang8 , B. Schoser9 , H.A. Kretzschmar2 , A. Danek1 , B. Bader1 . 1 Neurologische Klinik und Poliklinik, Klinikum der Universit¨ at M¨ unchen, 2 Zentrum f¨ ur Neuropathologie und Prionforschung, Ludwig-Maximilians-Universit¨ at M¨ unchen, Munich, Germany; 3 The Barts and Royal London Hospital, Queen’s Hospital, London, 4 Department of Cellular Pathology, Queen’s Hospital, Romford, 5 London Chest Hospital, Barts and the London NHS Trust, London, UK; 6 Department of Neurology, Prefectural Amagasaki Hospital, Amagasaki, Japan; 7 Department of Laboratory Medicine and Pathobiology, University of Toronto, 8 Movement Disorders Center, Toronto Western Hospital and Jack Clark Chair for Parkinson’s Disease Research, University of Toronto, Toronto, ON, Canada; 9 Friedrich-Baur-Institut, Klinikum der Universit¨ at M¨ unchen, Munich, Germany Objectives: Chorea-acanthocytosis (ChAc) is a hereditary disease due to mutations in the VPS13A gene. Besides the predominance of a hyperkinetic or hypokinetic movement disorders, neuromuscular syndromes are also present in ChAc. We did a neuropathological study on changes in ChAc patients’ muscle and nerve tissue. Methods: We collected 10 muscle samples (7 biopsies, 3 autopsies) and 2 peripheral nerve samples (autopsy tissue) of ChAc patients. Basic stainings comprise of H&E, Gomori trichrome, periodic acid Schiff. For nerve tissue, Elastica van Gieson, neurofilament and myelin basic protein staining was done. Myosin heavy chain fast and slow isotypes were stained for muscle fibre typing. Besides basic pathologic features, we assessed fibre size, internalized nuclei and calculated coefficients of variability, hypertrophy and atrophy. Results: Muscle histology is consistent with moderate myopathy. In 7 patients, the number of internalized nuclei is markedly increased in more than 5% of the fibres. Two patients show increased fibre size variation and mild endomysial fibrosis is present in one muscle. In 7 patients fibre group atrophies, fibre type grouping, and numerous angulated fibres suggest a neurogenic myopathy. In both, type 1 and 2 muscle fibres, abnormalities are found in diameter and atrophy coefficient. In peripheral nerve samples, there was no significant loss of myelinated fibres, neurofilament and no endoneural or perineural fibrosis. Conclusion: Although myopathy in ChAc seems to be neurogenic in most patients, no marked neuropathy is seen. However, since few samples of peripheral nerve are available, interpretation of peripheral nerve tissue is limited.
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1.243 CASE SERIES: ANTI-NMDAR ENCEPHALITIS – A SYNAPTIC AUTOIMMUNE DISORDER C.H. Chai1 , B.H. Ong1 , S.D. Puvanarajah1 , N.R. Mohd Zain2 , M. Arip3 , M.H. Rafia1 . 1 Dept. of Neurology, 2 Dept. of Diagnostic Imaging, Kuala Lumpur Hospital, 3 Allergy and Immunology Research Center, Institute for Medical Research, Kuala Lumpur, Malaysia The N-methyl-D-aspartate (NMDA) subtype of glutamate receptor (NMDAR) in the central nervous system (CNS) is essential for synaptic transmission and plasticity. Both hypo- and hyperactivity of NMDA receptors are proposed underlying mechanisms in various neurological disorders. Four patients were diagnosed with antiNMDAR encephalitis at Kuala Lumpur Hospital (HKL) between 2009 and 2011. Findings: The patients’ ages ranged from 14 to 33 years with equal number of males and females – two Malays, one Chinese and one Iban descent. Three patients reported viral prodrome. Generalized seizures were observed in three patients and all four patients developed psychiatric symptoms. Involuntary oro-facial and limb dyskinesias were seen in all. Both male patients demonstrated predominantly cardiovascular dysautonomia. Electroencephalography (EEG) in all patients showed diffuse delta wave activity. All four patients were positive for antiNMDAR antibody. Neuroimaging was abnormal in two patients – diffuse cerebral atrophy and hyperintense T2/FLAIR signals at the medial temporal lobes bilaterally. Only the female patients had tumours – primary ovarian. All four patients received parenteral methylprednisolone and three of them, additionally had intravenous immunoglobulin (IVIG) infusion. One patient underwent plasmapheresis following poor response to IVIG. All our patients survived with mild to moderate cognitive disability. Discussion: Our case series reports a higher incidence of male patients (50%), but similar incidence of viral prodrome, psychiatric symptoms, seizures and abnormal movements in comparison with other larger studies. However, dysautonomia appears less common (50%). We concluded that all four NMDAR encephalitis patients responded well to a combination of immunosuppressants, immunoglobulin and plasmapheresis. 1.244 CHOREOATHETOSIS CAUSED BY NUCLEUS CAUDATE ISCHEMIA ¨ M.G. Senol, ¸ E. Cengiz, F. Ozda˘ g, M. Saraco˘ ¸ glu. GATA Haydarpasa ¸ E˘gitim Hastanesi, Neurology Department, Istanbul, Turkey Background: Stroke is one of the rare signs of choreoathetosis. We have detected cases of choreoathetosis which is reason of caudate ischemia. Case: 40-year-old male patient was admitted to our outpatient clinic with the right body half involuntary movements. His symptoms started suddenly, without resorting to a month ago. The patient had no history of drug abuse, alcohol and cigarettes. In neurological examination showed choreiform movements in right body. Movements were evaluated as choreoathetosis. In Cranial MRI FLAIR, T2-sequence hyperintense signal changes in the localization was found matching the left caudate nucleus. Cranial MR angiography, approximately 50% lumen narrowing was confirmed at M1 segment of the left middle cerebral artery distal, a short segment. Caudate lesions were evaluated as an ischemia. There was no cardiac pathology. The EEG examination was normal. Carbamazepin (400 mg) and ASA (300 mg) were added treatment and all symptoms disappeared. Discussion: Cerebrovascular events in the etiology of Chorea; infarction or hemorrhage in the basal ganglia, arteriovenous malformation, polycythemia vera, migraine, listed as transient ischemic attack. A usually typical cause is cerebral infarction. The anatomical basis of chorea is unknown, or at least there are inconsistencies. Huntington’s chorea is lesions in the caudate nucleus and prominent putamen. Often vascular lesions in the
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same areas can be monitored without chorea. At nucleus caudatus ischemia the expected clinical symptoms are cognitive dysfunction and minimal motor speech disorder. As in our patient, nucleus caudate ischemia cause contralateral choreoathetosis and these are rare reasons. 1.245 HEMICHOREA-HEMIBALLISM: NON KETOTIC HYPERGLYCEMIA SYMPTOM
medical therapy consisted of one dose of cyclophosphamide and four rounds of rituximab. Pharmacological agents utilized in her management included steroids, immunoglobulins, bromocriptine, opioids, gabapentin, quetiapine. By the time of her discharge, eleven weeks from admission, she was interactive and ambulating with assistance. Conclusion: This case underscores the importance of a high index of suspicion for NMDA encephalitis despite an atypical presentation.
C. Hernando. Neurology, Povisa hospital, Vigo, Spain Object of the study: Hemichorea-hemiballism is a movemente disorder caused by multiple etiologies, vascular, tumors, infections and metabolic disorders like non ketotic hyperglycemia. We present two clinical cases of secondary hemichore3a non ketotic hyperglycemia. Clinical cases: We present two clinical cases of secondary hemiballism hemichorea – non ketotic hyperglycemia in a young woman with type 2 diabetes mellitus and an older man, in which the clinical symptom hemichorea was his initial type 2 diabetes. The first is a 24 year old woman with a history of diabetes mellitus type 2 and heterozygous carrier of the mutation of MTHFR and prothrombin gene admitted for 4 days of development of involuntary movements in left side of the body. The second is a 77 year old male was admitted for 12-day history of involuntary movements in the right arm in relation to diabetic debut. Discussion: The pathogenesis by which hyperglycemia produces lesions in the basal ganglia is unclear. Several mechanisms have been postullated: vascular, alterations in GABA metabolism or immune-mediated inflammatory process, among others. What we must not forget is that a patient with a movement disorder of sudden onset was to study glucose metabolism, since in some cases is totally reversible and most improve with good metabolic control. 1.246 PROFUSE SIALORRHEA AND HEMIBALLISM IN A CASE OF ANTI N-METHYL-D-ASPARTATE RECEPTOR (NMDAR) ENCEPHALITIS R. Salazar Montero1 , E. James1 , M. Elsayed1 , P. Varelas2 , J. Bartscher2 , J. Corry2 , T. Abdelhak2 . 1 Neurology, 2 Neuro Intensive Care, Henry Ford Hospital, Detroit, MI, USA Introduction: Anti-NMDAR encephalitis is an under recognized immune-mediated syndrome, analogous to a paraneoplastic phenomenon. This syndrome is predominantly described in young females with an occult ovarian teratoma who present with a constellation of symptoms, including personality changes, autonomic instability, dyskinesias and neurologic decompensation. Profuse sialorrhea or hemiballism are not typical clinical manifestations in this type of encephalitits. Objective: To highlight the clinical course of Anti-NMDAR encephalitis with a rapidly progressive encephalopathy associated with profuse sialorrhea, autonomic instability and marked dyskinesias including hemiballism. Design: Retrospective chart review. Results: A 29-year-old female presented with gradually worsening depression and hallucinations. She subsequently developed severe encephalopathy, respiratory failure, autonomic dysfunction followed by profuse sialorrhea, choreoathetotic movements, hemiballismus, jaw opening/upper extremities dystonia and orofacial dyskinesia (see video). The amount of sialorrhea was up to 2 liters per day that required volume replacement. She received Onabotulinum toxin injections in her salivary glands, masticatory muscles and arms with minimal improvement. Multiple brain MRI studies were unremarkable. EEG revealed marked slowing without epileptiform discharge. CSF revealed pleocytosis with lymphocytic predominance and positive oligoclonal bands. Her serum contained NMDAR antibodies. An ultrasound of the pelvis revealed an ovarian teratoma that was subsequently excised. The
1.247 HEMICHOREA IN NON-KETOTIC HYPERGLYCEMIA WITH BILATERAL HYPERINTENSITY IN BASAL GANGLIA ON T1-WEIGHTED BRAIN MRI C.K. Ha1 , H.-K. Park2 . 1 Department of Neurology, 2 Neurology, Inha University Hospital, Incheon, Republic of Korea Introduction: Hemichorea in non-ketotic hyperglycemia (HCNKH) is well recognized hyperkinetic condition in patient with type 2 diabetes. It is associated with contralateral striatal hyperintensity on T1-weighted brain MRI (T1WI). However, HCNKH with bilateral hyperintensity on T1WI is very rare. Case report: A 90 year old woman presented with sudden onset of choreiform movement in the left arm and leg from 5 days ago. She had 5 year history of hypertension and taken an ACE inhibitor, but didn’t know she had diabetes. Choreiform movement began in the left hand and spread to the left arm and leg. Neurological examination was otherwise normal. The blood glucose level was 380 mg/dl, HbA1C was 12.8%, and the serum osmolality was 324 mOsm/l. Urinalysis was negative for ketone bodies. Brain MRI showed high intensity on T1WI in bilateral basal ganglia symmetrically with normal T2 signal. The hemichorea improved slowly over 3 days with control of the blood glucose level with insulin therapy. Conclusion: We report a case of hemichorea as presenting symptom in non-ketotic hyperglycemia with bilateral basal ganglia hyperintensity on T1WI. Non-ketotic hyperglycemia is a systemic condition, but it is unclear why HCNKH presents almost always unilaterally with contralateral basal ganglia lesion on T1WI. Moreover, it is peculiar why bilateral lesions on T1WI produce unilateral hemichorea. 1.248 A CASE OF HEMICHOREA AS A RARE MANIFESTATION OF POLYCYTHEMIA VERA H.I. Ma, Y.J. Jang, Y.K. Jang, S.Y. Kang, Y.J. Kim. Neurology, Hallym University College of Medicine, Anyang, Republic of Korea Background and objective: Polycythemic chorea occurs predominantly in female and starts off with face and brachial area symmetrically then it proceeds to generalized form. We report a patient who was diagnosed with polycythemia vera presented with hemichorea showing late response to phlebotomy and dopamine antagonist. Case: A 76-year-old female patient was admitted to our hospital due to sudden involuntary movement of the left hand and foot. Abnormal movement of the left foot was presented seven days prior to admission. After one day, involuntary movements involved the left hand then progress to left face at admission. She has had polycythemia vera since ten years ago and gotten treated regularly. The patient had no familial history of chorea and denied the use of chorea-inducing drugs. A CBC revealed an elevated red cell count (5.59 million/ml), hemoglobin concentration (16.7 g/dl) and hematocrit (53.4%). The routine biochemistry, including serum glucose, calcium and thyroid function tests were within normal limits. Serologic tests for syphilis, FANA, anti-cardiolipin antibodies and HIV were negative. Brain MRI was normal. The FDG-PET showed relatively decreased uptake in the left basal ganglia. For treatment, she took haloperidol 3.9 mg
geographical distribution, gender, age of onset and age of diagnosis as well as clinical features of all patients were summarized. Results: In total, there were 29 case reports reporting in total 39 NA cases distributed in 15 provinces of China. The prominent symptoms include movement disorder, dystonia, orofacial dyskinesias, dysarthria, dysphagia, epilepsy, and psychiatric symptoms. Diagnosis of NA was made mainly according to the clinical manifestation, percentage of acanthocytes in peripheral blood, as well as neuroimaging results and EEG. Because of limitations in diagnostic methodology in China, the diagnosis of NA can not be further classified into NA diseases ChAc, MLS or NBIA. Conclusion: There were fewer NA cases reported in China as expected considering its estimated prevalence. This might be due to limitations in the physicians’ awareness and diagnostic methodology for NA. Thus, advanced diagnostic methods for screening and classification of NA syndromes are required in China. 1.242 NEUROMUSCULAR FINDINGS IN CHOREA-ACANTHOCYTOSIS J. Liu1 , T. Arzberger2 , A. Radunovic3 , U. Pohl4 , S. Mohiddin5 , Y. Kageyama6 , R. Kiehl7 , T. Lang8 , B. Schoser9 , H.A. Kretzschmar2 , A. Danek1 , B. Bader1 . 1 Neurologische Klinik und Poliklinik, Klinikum der Universit¨ at M¨ unchen, 2 Zentrum f¨ ur Neuropathologie und Prionforschung, Ludwig-Maximilians-Universit¨ at M¨ unchen, Munich, Germany; 3 The Barts and Royal London Hospital, Queen’s Hospital, London, 4 Department of Cellular Pathology, Queen’s Hospital, Romford, 5 London Chest Hospital, Barts and the London NHS Trust, London, UK; 6 Department of Neurology, Prefectural Amagasaki Hospital, Amagasaki, Japan; 7 Department of Laboratory Medicine and Pathobiology, University of Toronto, 8 Movement Disorders Center, Toronto Western Hospital and Jack Clark Chair for Parkinson’s Disease Research, University of Toronto, Toronto, ON, Canada; 9 Friedrich-Baur-Institut, Klinikum der Universit¨ at M¨ unchen, Munich, Germany Objectives: Chorea-acanthocytosis (ChAc) is a hereditary disease due to mutations in the VPS13A gene. Besides the predominance of a hyperkinetic or hypokinetic movement disorders, neuromuscular syndromes are also present in ChAc. We did a neuropathological study on changes in ChAc patients’ muscle and nerve tissue. Methods: We collected 10 muscle samples (7 biopsies, 3 autopsies) and 2 peripheral nerve samples (autopsy tissue) of ChAc patients. Basic stainings comprise of H&E, Gomori trichrome, periodic acid Schiff. For nerve tissue, Elastica van Gieson, neurofilament and myelin basic protein staining was done. Myosin heavy chain fast and slow isotypes were stained for muscle fibre typing. Besides basic pathologic features, we assessed fibre size, internalized nuclei and calculated coefficients of variability, hypertrophy and atrophy. Results: Muscle histology is consistent with moderate myopathy. In 7 patients, the number of internalized nuclei is markedly increased in more than 5% of the fibres. Two patients show increased fibre size variation and mild endomysial fibrosis is present in one muscle. In 7 patients fibre group atrophies, fibre type grouping, and numerous angulated fibres suggest a neurogenic myopathy. In both, type 1 and 2 muscle fibres, abnormalities are found in diameter and atrophy coefficient. In peripheral nerve samples, there was no significant loss of myelinated fibres, neurofilament and no endoneural or perineural fibrosis. Conclusion: Although myopathy in ChAc seems to be neurogenic in most patients, no marked neuropathy is seen. However, since few samples of peripheral nerve are available, interpretation of peripheral nerve tissue is limited.
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1.243 CASE SERIES: ANTI-NMDAR ENCEPHALITIS – A SYNAPTIC AUTOIMMUNE DISORDER C.H. Chai1 , B.H. Ong1 , S.D. Puvanarajah1 , N.R. Mohd Zain2 , M. Arip3 , M.H. Rafia1 . 1 Dept. of Neurology, 2 Dept. of Diagnostic Imaging, Kuala Lumpur Hospital, 3 Allergy and Immunology Research Center, Institute for Medical Research, Kuala Lumpur, Malaysia The N-methyl-D-aspartate (NMDA) subtype of glutamate receptor (NMDAR) in the central nervous system (CNS) is essential for synaptic transmission and plasticity. Both hypo- and hyperactivity of NMDA receptors are proposed underlying mechanisms in various neurological disorders. Four patients were diagnosed with antiNMDAR encephalitis at Kuala Lumpur Hospital (HKL) between 2009 and 2011. Findings: The patients’ ages ranged from 14 to 33 years with equal number of males and females – two Malays, one Chinese and one Iban descent. Three patients reported viral prodrome. Generalized seizures were observed in three patients and all four patients developed psychiatric symptoms. Involuntary oro-facial and limb dyskinesias were seen in all. Both male patients demonstrated predominantly cardiovascular dysautonomia. Electroencephalography (EEG) in all patients showed diffuse delta wave activity. All four patients were positive for antiNMDAR antibody. Neuroimaging was abnormal in two patients – diffuse cerebral atrophy and hyperintense T2/FLAIR signals at the medial temporal lobes bilaterally. Only the female patients had tumours – primary ovarian. All four patients received parenteral methylprednisolone and three of them, additionally had intravenous immunoglobulin (IVIG) infusion. One patient underwent plasmapheresis following poor response to IVIG. All our patients survived with mild to moderate cognitive disability. Discussion: Our case series reports a higher incidence of male patients (50%), but similar incidence of viral prodrome, psychiatric symptoms, seizures and abnormal movements in comparison with other larger studies. However, dysautonomia appears less common (50%). We concluded that all four NMDAR encephalitis patients responded well to a combination of immunosuppressants, immunoglobulin and plasmapheresis. 1.244 CHOREOATHETOSIS CAUSED BY NUCLEUS CAUDATE ISCHEMIA ¨ M.G. Senol, ¸ E. Cengiz, F. Ozda˘ g, M. Saraco˘ ¸ glu. GATA Haydarpasa ¸ E˘gitim Hastanesi, Neurology Department, Istanbul, Turkey Background: Stroke is one of the rare signs of choreoathetosis. We have detected cases of choreoathetosis which is reason of caudate ischemia. Case: 40-year-old male patient was admitted to our outpatient clinic with the right body half involuntary movements. His symptoms started suddenly, without resorting to a month ago. The patient had no history of drug abuse, alcohol and cigarettes. In neurological examination showed choreiform movements in right body. Movements were evaluated as choreoathetosis. In Cranial MRI FLAIR, T2-sequence hyperintense signal changes in the localization was found matching the left caudate nucleus. Cranial MR angiography, approximately 50% lumen narrowing was confirmed at M1 segment of the left middle cerebral artery distal, a short segment. Caudate lesions were evaluated as an ischemia. There was no cardiac pathology. The EEG examination was normal. Carbamazepin (400 mg) and ASA (300 mg) were added treatment and all symptoms disappeared. Discussion: Cerebrovascular events in the etiology of Chorea; infarction or hemorrhage in the basal ganglia, arteriovenous malformation, polycythemia vera, migraine, listed as transient ischemic attack. A usually typical cause is cerebral infarction. The anatomical basis of chorea is unknown, or at least there are inconsistencies. Huntington’s chorea is lesions in the caudate nucleus and prominent putamen. Often vascular lesions in the
S58
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same areas can be monitored without chorea. At nucleus caudatus ischemia the expected clinical symptoms are cognitive dysfunction and minimal motor speech disorder. As in our patient, nucleus caudate ischemia cause contralateral choreoathetosis and these are rare reasons. 1.245 HEMICHOREA-HEMIBALLISM: NON KETOTIC HYPERGLYCEMIA SYMPTOM
medical therapy consisted of one dose of cyclophosphamide and four rounds of rituximab. Pharmacological agents utilized in her management included steroids, immunoglobulins, bromocriptine, opioids, gabapentin, quetiapine. By the time of her discharge, eleven weeks from admission, she was interactive and ambulating with assistance. Conclusion: This case underscores the importance of a high index of suspicion for NMDA encephalitis despite an atypical presentation.
C. Hernando. Neurology, Povisa hospital, Vigo, Spain Object of the study: Hemichorea-hemiballism is a movemente disorder caused by multiple etiologies, vascular, tumors, infections and metabolic disorders like non ketotic hyperglycemia. We present two clinical cases of secondary hemichore3a non ketotic hyperglycemia. Clinical cases: We present two clinical cases of secondary hemiballism hemichorea – non ketotic hyperglycemia in a young woman with type 2 diabetes mellitus and an older man, in which the clinical symptom hemichorea was his initial type 2 diabetes. The first is a 24 year old woman with a history of diabetes mellitus type 2 and heterozygous carrier of the mutation of MTHFR and prothrombin gene admitted for 4 days of development of involuntary movements in left side of the body. The second is a 77 year old male was admitted for 12-day history of involuntary movements in the right arm in relation to diabetic debut. Discussion: The pathogenesis by which hyperglycemia produces lesions in the basal ganglia is unclear. Several mechanisms have been postullated: vascular, alterations in GABA metabolism or immune-mediated inflammatory process, among others. What we must not forget is that a patient with a movement disorder of sudden onset was to study glucose metabolism, since in some cases is totally reversible and most improve with good metabolic control. 1.246 PROFUSE SIALORRHEA AND HEMIBALLISM IN A CASE OF ANTI N-METHYL-D-ASPARTATE RECEPTOR (NMDAR) ENCEPHALITIS R. Salazar Montero1 , E. James1 , M. Elsayed1 , P. Varelas2 , J. Bartscher2 , J. Corry2 , T. Abdelhak2 . 1 Neurology, 2 Neuro Intensive Care, Henry Ford Hospital, Detroit, MI, USA Introduction: Anti-NMDAR encephalitis is an under recognized immune-mediated syndrome, analogous to a paraneoplastic phenomenon. This syndrome is predominantly described in young females with an occult ovarian teratoma who present with a constellation of symptoms, including personality changes, autonomic instability, dyskinesias and neurologic decompensation. Profuse sialorrhea or hemiballism are not typical clinical manifestations in this type of encephalitits. Objective: To highlight the clinical course of Anti-NMDAR encephalitis with a rapidly progressive encephalopathy associated with profuse sialorrhea, autonomic instability and marked dyskinesias including hemiballism. Design: Retrospective chart review. Results: A 29-year-old female presented with gradually worsening depression and hallucinations. She subsequently developed severe encephalopathy, respiratory failure, autonomic dysfunction followed by profuse sialorrhea, choreoathetotic movements, hemiballismus, jaw opening/upper extremities dystonia and orofacial dyskinesia (see video). The amount of sialorrhea was up to 2 liters per day that required volume replacement. She received Onabotulinum toxin injections in her salivary glands, masticatory muscles and arms with minimal improvement. Multiple brain MRI studies were unremarkable. EEG revealed marked slowing without epileptiform discharge. CSF revealed pleocytosis with lymphocytic predominance and positive oligoclonal bands. Her serum contained NMDAR antibodies. An ultrasound of the pelvis revealed an ovarian teratoma that was subsequently excised. The
1.247 HEMICHOREA IN NON-KETOTIC HYPERGLYCEMIA WITH BILATERAL HYPERINTENSITY IN BASAL GANGLIA ON T1-WEIGHTED BRAIN MRI C.K. Ha1 , H.-K. Park2 . 1 Department of Neurology, 2 Neurology, Inha University Hospital, Incheon, Republic of Korea Introduction: Hemichorea in non-ketotic hyperglycemia (HCNKH) is well recognized hyperkinetic condition in patient with type 2 diabetes. It is associated with contralateral striatal hyperintensity on T1-weighted brain MRI (T1WI). However, HCNKH with bilateral hyperintensity on T1WI is very rare. Case report: A 90 year old woman presented with sudden onset of choreiform movement in the left arm and leg from 5 days ago. She had 5 year history of hypertension and taken an ACE inhibitor, but didn’t know she had diabetes. Choreiform movement began in the left hand and spread to the left arm and leg. Neurological examination was otherwise normal. The blood glucose level was 380 mg/dl, HbA1C was 12.8%, and the serum osmolality was 324 mOsm/l. Urinalysis was negative for ketone bodies. Brain MRI showed high intensity on T1WI in bilateral basal ganglia symmetrically with normal T2 signal. The hemichorea improved slowly over 3 days with control of the blood glucose level with insulin therapy. Conclusion: We report a case of hemichorea as presenting symptom in non-ketotic hyperglycemia with bilateral basal ganglia hyperintensity on T1WI. Non-ketotic hyperglycemia is a systemic condition, but it is unclear why HCNKH presents almost always unilaterally with contralateral basal ganglia lesion on T1WI. Moreover, it is peculiar why bilateral lesions on T1WI produce unilateral hemichorea. 1.248 A CASE OF HEMICHOREA AS A RARE MANIFESTATION OF POLYCYTHEMIA VERA H.I. Ma, Y.J. Jang, Y.K. Jang, S.Y. Kang, Y.J. Kim. Neurology, Hallym University College of Medicine, Anyang, Republic of Korea Background and objective: Polycythemic chorea occurs predominantly in female and starts off with face and brachial area symmetrically then it proceeds to generalized form. We report a patient who was diagnosed with polycythemia vera presented with hemichorea showing late response to phlebotomy and dopamine antagonist. Case: A 76-year-old female patient was admitted to our hospital due to sudden involuntary movement of the left hand and foot. Abnormal movement of the left foot was presented seven days prior to admission. After one day, involuntary movements involved the left hand then progress to left face at admission. She has had polycythemia vera since ten years ago and gotten treated regularly. The patient had no familial history of chorea and denied the use of chorea-inducing drugs. A CBC revealed an elevated red cell count (5.59 million/ml), hemoglobin concentration (16.7 g/dl) and hematocrit (53.4%). The routine biochemistry, including serum glucose, calcium and thyroid function tests were within normal limits. Serologic tests for syphilis, FANA, anti-cardiolipin antibodies and HIV were negative. Brain MRI was normal. The FDG-PET showed relatively decreased uptake in the left basal ganglia. For treatment, she took haloperidol 3.9 mg