発症11年経過後に実施したステロイドパルス療法が有効であった，成人発症型Rasmussen脳炎の1例
症例は48歳男性．37歳，全身けいれん，失語で発症し，左側大脳半球のT2高信号域を認め，carbamazepine, sodium valproateを開始した．42歳，右半身不全麻痺が出現し，48歳，二次性全般化発作が頻回となり，失語，歩行障害が進行した．頭部MRI上，左側大脳半球が高度に萎縮し，脳波で左側前頭極に焦点のある鋭波を認めた．血清，髄液で抗GluN2B抗体が陽性であった．一側性大脳半球萎縮，焦点性てんかん発作があり，知的退行，運動障害が進行性であるため，Rasmussen脳炎と診断し，ステロイドパルス療法を施行した．治療後，二次性全般化発作の頻度が減り，自立歩行も可能となった．R...
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| Published in | 神経治療学 Vol. 34; no. 5; pp. 543 - 546 |
|---|---|
| Main Authors | , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経治療学会
2018
Japanese Society of Neurological Therapeutics |
| Subjects | |
| Online Access | Get full text |
| ISSN | 0916-8443 2189-7824 |
| DOI | 10.15082/jsnt.34.5_543 |
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| Abstract | 症例は48歳男性．37歳，全身けいれん，失語で発症し，左側大脳半球のT2高信号域を認め，carbamazepine, sodium valproateを開始した．42歳，右半身不全麻痺が出現し，48歳，二次性全般化発作が頻回となり，失語，歩行障害が進行した．頭部MRI上，左側大脳半球が高度に萎縮し，脳波で左側前頭極に焦点のある鋭波を認めた．血清，髄液で抗GluN2B抗体が陽性であった．一側性大脳半球萎縮，焦点性てんかん発作があり，知的退行，運動障害が進行性であるため，Rasmussen脳炎と診断し，ステロイドパルス療法を施行した．治療後，二次性全般化発作の頻度が減り，自立歩行も可能となった．Rasmussen脳炎は，主に小児期に発症する自己免疫性てんかんで，早期治療介入が予後を改善する．本症例は，長期経過後の導入にもかかわらずステロイドパルス療法が有効であった．成人発症の緩徐進行性難治性てんかんでは，同疾患の可能性も疑い，長期経過例でも免疫療法を検討する必要があると思われる． |
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| AbstractList | Refractory epilepsy, right hemiplegia, and impaired fluency of expression had progressed over a period of 10 years in a 48–year–old male who had initially been diagnosed with secondary generalized seizures and aphasia. He was also unable to walk without support and levels of anti–N–methyl–D–aspartate type glutamate receptor subunit antibody were elevated in serum and cerebrospinal fluid. T2–weighted and fluid attenuated inversion recovery brain imaging showed hyperintensity on paraventricular white matter and EEG revealed a sharp wave with a front pole focus in the left cerebral cortex, where atrophy was more prominent. The brain MRI findings were worse at ten years than at two years after onset. He was diagnosed with Rasmussen's encephalitis based on the Bien diagnostic criteria and underwent high–dose intravenous methylprednisolone pulse (IVMP) therapy. Thereafter, he was able to walk without support for over 100 meters and the secondary generalized seizures and aphasia were improved. Rasmussen's encephalitis tends to progress more slowly in cases of adult onset than in childhood onset. The frequency of epilepsy increases and motor and cognitive decline progresses within months without immediate immunomodulation therapy. However, the frequency of epilepsy, gait disturbance, and aphasia was improved in our patient despite IVMP therapy being implemented eleven years after onset. Slowly progressive, refractory epilepsy of adult onset should be carefully assessed because immunomodulation therapy could be effective even if Rasmussen's encephalitis is diagnosed late.
症例は48歳男性．37歳，全身けいれん，失語で発症し，左側大脳半球のT2高信号域を認め，carbamazepine, sodium valproateを開始した．42歳，右半身不全麻痺が出現し，48歳，二次性全般化発作が頻回となり，失語，歩行障害が進行した．頭部MRI上，左側大脳半球が高度に萎縮し，脳波で左側前頭極に焦点のある鋭波を認めた．血清，髄液で抗GluN2B抗体が陽性であった．一側性大脳半球萎縮，焦点性てんかん発作があり，知的退行，運動障害が進行性であるため，Rasmussen脳炎と診断し，ステロイドパルス療法を施行した．治療後，二次性全般化発作の頻度が減り，自立歩行も可能となった．Rasmussen脳炎は，主に小児期に発症する自己免疫性てんかんで，早期治療介入が予後を改善する．本症例は，長期経過後の導入にもかかわらずステロイドパルス療法が有効であった．成人発症の緩徐進行性難治性てんかんでは，同疾患の可能性も疑い，長期経過例でも免疫療法を検討する必要があると思われる． 症例は48歳男性．37歳，全身けいれん，失語で発症し，左側大脳半球のT2高信号域を認め，carbamazepine, sodium valproateを開始した．42歳，右半身不全麻痺が出現し，48歳，二次性全般化発作が頻回となり，失語，歩行障害が進行した．頭部MRI上，左側大脳半球が高度に萎縮し，脳波で左側前頭極に焦点のある鋭波を認めた．血清，髄液で抗GluN2B抗体が陽性であった．一側性大脳半球萎縮，焦点性てんかん発作があり，知的退行，運動障害が進行性であるため，Rasmussen脳炎と診断し，ステロイドパルス療法を施行した．治療後，二次性全般化発作の頻度が減り，自立歩行も可能となった．Rasmussen脳炎は，主に小児期に発症する自己免疫性てんかんで，早期治療介入が予後を改善する．本症例は，長期経過後の導入にもかかわらずステロイドパルス療法が有効であった．成人発症の緩徐進行性難治性てんかんでは，同疾患の可能性も疑い，長期経過例でも免疫療法を検討する必要があると思われる． |
| Author | 黒羽, 泰子 小池, 亮子 谷, 卓 長谷川, 有香 高橋, 幸利 松原, 奈絵 長谷川, 直哉 高橋, 哲哉 |
| Author_FL | Takahashi Yukitoshi Tani Takashi Hasegawa Naoya Takahashi Tetsuya Hasegawa Arika Kuroha Yasuko Matsubara Nae Koike Ryoko |
| Author_FL_xml | – sequence: 1 fullname: Kuroha Yasuko – sequence: 2 fullname: Hasegawa Naoya – sequence: 3 fullname: Hasegawa Arika – sequence: 4 fullname: Tani Takashi – sequence: 5 fullname: Takahashi Tetsuya – sequence: 6 fullname: Matsubara Nae – sequence: 7 fullname: Takahashi Yukitoshi – sequence: 8 fullname: Koike Ryoko |
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| References | 3) Bien CG, Granata T, Antozzi C : Pathogenesis, diagnosis and treatment of Rasmussen encephalitis : a European consensus statement. Brain 128 : 454–471, 2005 6) McLachlan RS, Girvin JP, Blume WT : Rasmussen's chronic encephalitis in adults. Arch Neurol 50 : 269–274, 1993 4) Bien CG, Widman G, Urbach H : The natural history of Rasmussen's encephalitis. Brain 125 : 1751–1759, 2002 2) Fukuyama T, Takahashi Y, Kubota Y et al : Semi–quantitative analyses of antibodies to N–methyl–d–aspartate type glutamate receptor subunits (GluN2B & GluN1) in the clinical course of Rasmussen syndrome. Epilepsy Res 113 : 34–43, 2015 8) Villani F, Spreafico R, Farina L : Positive response to immunomodulatory therapy in an adult patient with Rasmussen's encephalitis. Neurology 56 : 248–250, 2001 10) Villani F, Pincherle A, Antozzi C et al : Adult–onset Rasmussen's encephalitis : anatomical–electrographic–clinical features of 7 Italian cases. Epilepsia 47(Suppl 5) : 41–46, 2006 7) Vadlamudi L, Galton CJ, Jeavons SJ : Rasmussen's syndrome in a 54 year old female : more support for an adult variant. J Clin Neurosci 7 : 154–156, 2000 9) Frucht S : Dystonia, athetosis, and epilepsia partialis continua in a patient with late–onset Rasmussen's encephalitis. Mov Disord 17 : 609–612, 2002 5) Gray F, Serdaru M, Baron H : Chronic localised encephalitis (Rasmussen's) in an adult with epilepsia partialis continua. J Neurol Neurosurg Psychiatry 50 : 747–751, 1987 1) Rasmussen T, Olszewski J, Lloydsmith D : Focal seizures due to chronic localized encephalitis. Neurology 8 : 435–445, 1958 11) Deleo F, Matricardi S, Didato G et al : The dilemma of adult–onset Rasmussen encephalitis clinical assessment : Proposal for a new bedside tool to evaluate disease progression. Epilepsy Behav 46 : 249–251, 2015 |
| References_xml | – reference: 4) Bien CG, Widman G, Urbach H : The natural history of Rasmussen's encephalitis. Brain 125 : 1751–1759, 2002 – reference: 3) Bien CG, Granata T, Antozzi C : Pathogenesis, diagnosis and treatment of Rasmussen encephalitis : a European consensus statement. Brain 128 : 454–471, 2005 – reference: 6) McLachlan RS, Girvin JP, Blume WT : Rasmussen's chronic encephalitis in adults. Arch Neurol 50 : 269–274, 1993 – reference: 10) Villani F, Pincherle A, Antozzi C et al : Adult–onset Rasmussen's encephalitis : anatomical–electrographic–clinical features of 7 Italian cases. Epilepsia 47(Suppl 5) : 41–46, 2006 – reference: 5) Gray F, Serdaru M, Baron H : Chronic localised encephalitis (Rasmussen's) in an adult with epilepsia partialis continua. J Neurol Neurosurg Psychiatry 50 : 747–751, 1987 – reference: 7) Vadlamudi L, Galton CJ, Jeavons SJ : Rasmussen's syndrome in a 54 year old female : more support for an adult variant. J Clin Neurosci 7 : 154–156, 2000 – reference: 9) Frucht S : Dystonia, athetosis, and epilepsia partialis continua in a patient with late–onset Rasmussen's encephalitis. Mov Disord 17 : 609–612, 2002 – reference: 2) Fukuyama T, Takahashi Y, Kubota Y et al : Semi–quantitative analyses of antibodies to N–methyl–d–aspartate type glutamate receptor subunits (GluN2B & GluN1) in the clinical course of Rasmussen syndrome. Epilepsy Res 113 : 34–43, 2015 – reference: 8) Villani F, Spreafico R, Farina L : Positive response to immunomodulatory therapy in an adult patient with Rasmussen's encephalitis. Neurology 56 : 248–250, 2001 – reference: 1) Rasmussen T, Olszewski J, Lloydsmith D : Focal seizures due to chronic localized encephalitis. Neurology 8 : 435–445, 1958 – reference: 11) Deleo F, Matricardi S, Didato G et al : The dilemma of adult–onset Rasmussen encephalitis clinical assessment : Proposal for a new bedside tool to evaluate disease progression. Epilepsy Behav 46 : 249–251, 2015 |
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| Snippet | 症例は48歳男性．37歳，全身けいれん，失語で発症し，左側大脳半球のT2高信号域を認め，carbamazepine, sodium valproateを開始した．42歳，右半身不全麻痺が出現し，48... Refractory epilepsy, right hemiplegia, and impaired fluency of expression had progressed over a period of 10 years in a 48–year–old male who had initially been... |
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| SubjectTerms | adult–onset Rasmussen's encephalitis anti N–methyl–D–aspartate type glutamate receptor subunits antibody focal progressive cerebral atrophy slowly progressive refractory epilepsy steroid pulse therapy |
| Title | 発症11年経過後に実施したステロイドパルス療法が有効であった，成人発症型Rasmussen脳炎の1例 |
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