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ADEMとMSの臨床的相違点

ADEMと小児多発性硬化症の臨床的に重要な相違点
出典
imgimg
1: Acute disseminated encephalomyelitis: an update.
著者: Menge T, Hemmer B, Nessler S, Wiendl H, Neuhaus O, Hartung HP, Kieseier BC, Stüve O.
雑誌名: Arch Neurol. 2005 Nov;62(11):1673-80. doi: 10.1001/archneur.62.11.1673.
Abstract/Text: Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system that typically follows a febrile infection or a vaccination. Children are predominantly affected. A plethora of viral and bacterial pathogens and a number of vaccinations have been associated with ADEM. Experimental animal studies indicate that both primary and secondary autoimmune responses contribute to central nervous system inflammation and subsequent demyelination. The clinical diagnosis of ADEM is strongly suggested by a close temporal relationship between an infectious incident or an immunization and the onset of leukoencephalopathic neurological symptoms. Paraclinical tests may support the diagnosis. Particularly helpful are acute signs of newly developed extensive, multifocal, subcortical white matter abnormalities on magnetic resonance images of the brain. The cerebrospinal fluid may disclose a mild lymphocytic pleocytosis and elevated albumin levels. Oligoclonal bands are not always present in ADEM and, if so, may be transient. The major differential diagnosis of ADEM is multiple sclerosis. Treatment options for ADEM consist of anti-inflammatory and immunosuppressive agents. In general, the disease is self-limiting and the prognostic outcome favorable. In the absence of widely accepted clinical or paraclinical diagnostic guidelines, a number of recently conducted observational case series have substantially broadened our understanding about the clinical phenotype, diagnosis, and prognosis of ADEM.
Arch Neurol. 2005 Nov;62(11):1673-80. doi: 10.1001/archneur.62.11.1673...

ADEMのMRI画像

  1. T2強調画像では皮質下白質ないし深部白質に比較的対称性に多発する異常高信号を認める。
  1. 大脳皮質や視床や大脳基底核など灰白質に病変を認めることも多い。
  1. テント下病変も多い。
  1. 多発性硬化症でみられる脳室周囲や脳梁の病変は少なく、ウイルス性脳炎で目立つ皮質病変は少ない。
  1. 浮腫の影響で病巣の境界は不明瞭である。
a:頭部MRI(T2強調像、水平断)
b:頭部MRI(T2強調像、冠状断)
c:脊髄MRI(T2強調像)
出典
img
1: Adam: Grainger & Allison's Diagnostic Radiology, 5th ed. (改変あり)

多発性硬化症のMRI画像

  1. 多発性硬化症では、T2強調画像、プロトン密度画像、FLAIR画像にて、脳室周囲白質、皮質下白質、テント下白質、脊髄白質に多発する脱髄病変を認める。
  1. ガドリニウム造影を呈する病巣は活動病変とされる。
  1. T1強調画像で黒く抜けている病巣はT1 ブラックホールといい、軸索変性を来している。
a、b:頭部MRI(FLAIR、水平断)
c:頭部MRI(FLAIR、矢状断)
d:頭部MRI(T1強調、水平断)
f、g:頭部MRI(T1強調、水平断、Gd造影)
出典
img
1: Daroff: Bradley's Neurology in Clinical Practice (改変あり)

視神経脊髄炎の画像

視神経脊髄炎/視神経脊髄炎関連疾患の脊髄灰白質に3椎体以上の連続した病変が特徴的である。
a、b:脊髄MRI(矢状断、T2強調像)
c:脊髄MRI(矢状断、T1強調像)
出典
imgimg
1: The spectrum of neuromyelitis optica.
著者: Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG.
雑誌名: Lancet Neurol. 2007 Sep;6(9):805-15. doi: 10.1016/S1474-4422(07)70216-8.
Abstract/Text: Neuromyelitis optica (also known as Devic's disease) is an idiopathic, severe, demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has a worldwide distribution, poor prognosis, and has long been thought of as a variant of multiple sclerosis; however, clinical, laboratory, immunological, and pathological characteristics that distinguish it from multiple sclerosis are now recognised. The presence of a highly specific serum autoantibody marker (NMO-IgG) further differentiates neuromyelitis optica from multiple sclerosis and has helped to define a neuromyelitis optica spectrum of disorders. NMO-IgG reacts with the water channel aquaporin 4. Data suggest that autoantibodies to aquaporin 4 derived from peripheral B cells cause the activation of complement, inflammatory demyelination, and necrosis that is seen in neuromyelitis optica. The knowledge gained from further assessment of the exact role of NMO-IgG in the pathogenesis of neuromyelitis optica will provide a foundation for rational therapeutic trials for this rapidly disabling disease.
Lancet Neurol. 2007 Sep;6(9):805-15. doi: 10.1016/S1474-4422(07)70216-...

脱髄の説明

髄鞘が主に障害を受けることを脱髄という。
a:中枢神経のしくみ
b:脱髄をおこした中枢神経

ステロイドの副作用

ステロイドの副作用の時系列、ステロイド開始前のスクリーニング、ステロイド治療中の注意点。
出典
img
1: 岸本暢将編:ステロイドの使い方こんなときどうする?日常診療で上手く使う 処方・減量・中止のコツ.レジデントノート 2011;13(9):1522.

初回の中枢性脱髄疾患の診断のアプローチ

初回中枢性脱髄病変より、NMO(Neuromyelitis optica spectrum disorder)、ADEM、CIS(Clinically Isolated syndrome)を鑑別する。
出典
imgimg
1: Pediatric multiple sclerosis.
著者: Chabas D, Strober J, Waubant E.
雑誌名: Curr Neurol Neurosci Rep. 2008 Sep;8(5):434-41. doi: 10.1007/s11910-008-0067-1.
Abstract/Text: Diagnosing multiple sclerosis (MS) in a child is challenging because of the limited diagnostic criteria and their overlap with acute disseminated encephalomyelitis. Pediatric-onset MS patients are more likely to be male, have seizures, and have brainstem and cerebellar symptoms than adults, and are less likely to have spinal cord symptoms than adults. They mostly experience a relapsing-remitting course. Their initial brain MRI shows more frequent involvement of the posterior fossa, less well-defined ovoid lesions, and more confluent lesions that decrease over time in patients with prepubertal onset, making early diagnosis even more difficult. Although disability progression is slower than in adults, pediatric onset MS leads to significant disability at a younger age and may be worse in non-white patients (up to 50% in North America). The rareness of pediatric-onset MS has precluded enrollment in clinical trials. Thus, children are receiving off-label adult therapies without clear evidence of their effectiveness and limited knowledge of their tolerability.
Curr Neurol Neurosci Rep. 2008 Sep;8(5):434-41. doi: 10.1007/s11910-00...

ADEM、再発性ADEM、多相性ADEMの定義

通常ADEMは単相性であるが、再発性ADEMないし多相性ADEMも定義されている。
出典
imgimg
1: Consensus definitions proposed for pediatric multiple sclerosis and related disorders.
著者: Krupp LB, Banwell B, Tenembaum S; International Pediatric MS Study Group.
雑誌名: Neurology. 2007 Apr 17;68(16 Suppl 2):S7-12. doi: 10.1212/01.wnl.0000259422.44235.a8.
Abstract/Text: BACKGROUND: The CNS inflammatory demyelinating disorders of childhood include both self-limited and lifelong conditions, which can be indistinguishable at the time of initial presentation. Clinical, biologic, and radiographic delineation of the various monophasic and chronic childhood demyelinating disorders requires an operational classification system to facilitate prospective research studies.
METHODS: The National Multiple Sclerosis Society (NMSS) organized an International Pediatric MS Study Group (Study Group) composed of adult and pediatric neurologists and experts in genetics, epidemiology, neuropsychology, nursing, and immunology. The group met several times to develop consensus definitions regarding the major CNS inflammatory demyelinating disorders of children and adolescents.
RESULTS: Clinical definitions are proposed for pediatric multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), recurrent ADEM, multiphasic ADEM, neuromyelitis optica, and clinically isolated syndrome. These definitions are considered operational and need to be tested in future research and modified accordingly.
CONCLUSION: CNS inflammatory demyelinating disorders presenting in children and adolescents can be defined and distinguished. However, prospective research is necessary to determine the validity and utility of the proposed diagnostic categories.
Neurology. 2007 Apr 17;68(16 Suppl 2):S7-12. doi: 10.1212/01.wnl.00002...

ADEMとMSの臨床的相違点

ADEMと小児多発性硬化症の臨床的に重要な相違点
出典
imgimg
1: Acute disseminated encephalomyelitis: an update.
著者: Menge T, Hemmer B, Nessler S, Wiendl H, Neuhaus O, Hartung HP, Kieseier BC, Stüve O.
雑誌名: Arch Neurol. 2005 Nov;62(11):1673-80. doi: 10.1001/archneur.62.11.1673.
Abstract/Text: Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system that typically follows a febrile infection or a vaccination. Children are predominantly affected. A plethora of viral and bacterial pathogens and a number of vaccinations have been associated with ADEM. Experimental animal studies indicate that both primary and secondary autoimmune responses contribute to central nervous system inflammation and subsequent demyelination. The clinical diagnosis of ADEM is strongly suggested by a close temporal relationship between an infectious incident or an immunization and the onset of leukoencephalopathic neurological symptoms. Paraclinical tests may support the diagnosis. Particularly helpful are acute signs of newly developed extensive, multifocal, subcortical white matter abnormalities on magnetic resonance images of the brain. The cerebrospinal fluid may disclose a mild lymphocytic pleocytosis and elevated albumin levels. Oligoclonal bands are not always present in ADEM and, if so, may be transient. The major differential diagnosis of ADEM is multiple sclerosis. Treatment options for ADEM consist of anti-inflammatory and immunosuppressive agents. In general, the disease is self-limiting and the prognostic outcome favorable. In the absence of widely accepted clinical or paraclinical diagnostic guidelines, a number of recently conducted observational case series have substantially broadened our understanding about the clinical phenotype, diagnosis, and prognosis of ADEM.
Arch Neurol. 2005 Nov;62(11):1673-80. doi: 10.1001/archneur.62.11.1673...

ADEMのMRI画像

  1. T2強調画像では皮質下白質ないし深部白質に比較的対称性に多発する異常高信号を認める。
  1. 大脳皮質や視床や大脳基底核など灰白質に病変を認めることも多い。
  1. テント下病変も多い。
  1. 多発性硬化症でみられる脳室周囲や脳梁の病変は少なく、ウイルス性脳炎で目立つ皮質病変は少ない。
  1. 浮腫の影響で病巣の境界は不明瞭である。
a:頭部MRI(T2強調像、水平断)
b:頭部MRI(T2強調像、冠状断)
c:脊髄MRI(T2強調像)
出典
img
1: Adam: Grainger & Allison's Diagnostic Radiology, 5th ed. (改変あり)