ADEMに関しては友人が意識障害の原因がわからず、他院に送ったところ後日ADEMの診断になったとのエピソードのみです。
自分は見たことないです。鑑別にあげていないだけかも?!
問題解いていても見たことない疾患が多くて困ります。
MGは5人ぐらいみました。
・Acute disseminated (postinfectious) encephalomyelitis (ADEM) is multifocal periventricular inflammation and demyelination after infection (eg, chickenpox, measles) or certain vaccinations (eg, rabies, smallpox).
ワクチンはちゃんと打ったほうがいいですからね。
ADEM produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter, presenting like multiple sclerosis because of its demyelinating process.
ADEM does not have an increased incidence in patients with HIV infection or AIDS.
白質に病変をきたし、脱髄所見からMSっぽい。
AIDSの人に多いわけではない。
・This patient presents with difficulty maintaining posture and head support, diminished deep tendon reflexes, and weak cry, all of which suggest a lower motor neuron (LMN) lesion.
起立保持困難、腱反射低下、下位運動ニューロン障害
↓
・Spinal muscular atrophy(SMA) type 1 (Werdnig-Hoffmann disease) is an autosomal recessive disorder(mutation in the SMN1 gene (survival motor neuron): thought to play a role in mRNA synthesis in motor neurons)
→It manifests with diffuse muscle atrophy, hypotonia, fasciculations, and decreased deep tendon reflexes.
This condition affects only the LMN system, which includes the anterior horn cells of the spinal cord, ventral nerve root, peripheral nerve, neuromuscular junction, and skeletal muscle.
Poliomyelitis infection could also cause isolated LMN disease.
前角細胞の障害の劣性遺伝の疾患。SMN1遺伝子はmRNA合成関与。
下位運動ニューロンの障害でびまん性に筋委縮。
ポリオ鑑別！？
・Upper motor neurons (UMNs) are the neurons found in the brain and spinal cord that synapse with the lower motor neurons at the anterior horn of the spinal cord (ie, UMNs are proximal to the anterior horn). 上位運動ニューロンは脳脊髄に見られる。
They are also found in the precentral gyrus (ie, primary motor cortex) and decussate (ie, cross) at the medulla oblongata to course down the lateral spinal cord via the lateral corticospinal tract.
Knowing when the tracts cross is useful in localizing which side a lesion is based off of the symptoms; UMN lesions are typically contralateral to the side affected. Lower motor neurons (LMNs) are found distal to the anterior horn and directly innervate skeletal muscle; symptoms are typically ipsilateral to the lesion.
上位運動ニューロンの障害は反対側に影響する。
下位運動ニューロンは直接筋骨格系に働き、同側に影響する。
・Neostigmine and pyridostigmineの機序
→inhibit AchE and can be used in symptomatic management of myasthenia gravis.
ネオスチグミンとピリドスチグミンはMGの治療薬です。
