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CNS Pathology (I (Infection (種類 (Others: Prion Disease (常見相關 (Creutzfeldt…

- - - - Global
        
        Border zone (watershed) infarct
        
        通常發生在低血壓之後，形成sickle-shaped band of necrosis
        
        病理變化
        
        Early changes (前12到24小時): 急性缺血變化 Red neurons PMN infiltration
        
        Subacute changes (24hr-2weeks): Necrosis, Macrophage phage influx, Vascular proliferation, Reactive gliosis**
        
        Repair (2 weeks之後):Pseudolaminar necrosis
      - Focal
        
        分類
        
        Non-hemorrhagic
        
        Thrombosis
        
        Pale, bland, anemic
        
        Hemorrhagic
        
        Red infarction
        
        常見原因
        
        Thrombosis (最多)
        
        常見因atherosclerosis
        
        Carotid bifurcation
        
        MCA origin
        
        Basilar Artery either end
        
        常伴隨高血壓、糖尿病
        
        僅 thrombosis對thrombocytes therapy有效
        
        Embolization (次多)
        
        種類
        
        Cardiac mural thrombi
        
        心肌梗塞
        
        瓣膜疾病
        
        心房顫動
        
        Arterial thromboemboli
        
        於carotid arteries的動脈斑塊
        
        Paradoxical emboli
        
        先天性心臟異常造成
        
        發生在血流量大之處，最常見MCA，通常發生時已經存在有管腔狹窄
        
        Vasculitides血管炎 (第三多)
        
        Infectious vasculitis
        
        Polyarteritis nodosa
      - Ischemic changes
        
        Hours: Red neuron, edema, myelin disintegration (還有救)
        
        Days: PMNs infiltration (沒救了)
        
        Week(s): Microglial phagocytosis, gitter cells (腦中 phagocytes), necrosis, liquefaction
        
        Weeks to months: Repair, reactive astrocytic response (gliosis。要記得不是像其他細胞的fibroelastic reaction)
    - - Epidural
        
        Trauma 急性撕裂 middle meningial artery，急診殺手—清明死
      - Subdural
        
        Trauma 撕裂 bridging veins
      - Subarachnoid
        
        Aneurysm
      - Parenchymal
        
        HTN
        
        產生
        
        Lacunae infarct : common in lenticular nuclei, thalamus
        
        Hemorrhage
        
        最常見 primary brain parenchymal hemorrhage 原因
        
        15%高血壓患者的死因
        
        種類
        
        Slit hemorrhage
        
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        Basal ganglion hemorrhagic
        
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        Hypertensive encephalopathy
        
        Vasculitis
- - - - Focal
      - Multifocal
      - Disseminated
    - - Bacteria
      - Viral
        
        Viral encephalitis
        
        腦部的parenchymal infection
        
        Meningoencephalitis
        
        伴隨腦膜發炎
        
        Encephalomyelitis
        
        脊髓同時受影響
        
        感染源
        
        HSV-1 encephalitis
        
        最常見於小孩與年輕人
        
        影響
        
        Inf. & Med. temporal lobe
        
        Orbital Geri of frontal lobe
        
        特性
        
        CNS sign: 行為、心情、記憶改變
        
        Necrotizing hemorrhagic inflammation
        
        Crowdry type A intranuclear inclusion
        
        CMV
        
        主要影響 paraventricular subependymal region
        
        Periventricular calcification
        
        常見於免疫缺陷、嬰兒（畸形症）
        
        Poliomyelitis
        
        主要影響：脊髓 ventral horn
        
        常由 arboviruses (from insect) 病毒感染
        
        常見三種造成因素
        
        Latency潛伏期 with neural tropism
        
        因各病毒而異
        
        Immune-mediated Disease 造成的 Systemic viral infections
        
        急性、直接攻擊、最常見
        
        因一次病毒感染而造成慢性的退化疾病
        
        攻擊myelin
        
        CSF 檢查
        
        無色
        
        壓力輕微上升
        
        Lymphocyte 增加
        
        Glucose正常
        
        病理
        
        主要為perivascular accumulation of lymphocytes
        
        Neuronophagia
        
        Single-cell neuronal necrosis & phagocytosis of the debris
        
        Microglial nodules
        
        聚集在壞死區域周圍
        
        Progressive Multifocal leukoencephalopathy (PML)
        
        JC papovavirus 造成
        
        伺機性感染免疫不全病患(AIDS)
        
        病理
        
        Oligodendrocytes demyelination
        
        含有病毒抗原的 enlarged dark pink “ground glass” nuclei
        
        Nuclear viral inclusion + Alzheimer’s type I glia (國考)
      - Granulomatous
      - Abscess
        
        Acute focal suppurations infection
        
        常見因
        
        Direct extension from sinusitis: 顱底為主
        
        Acute bacterial endocarditis: multiple sites
        
        病理：central liquefaction necrosis
        
        Granulation tissue
        
        Collagenous / fibrous capsule ：因為血管也受影響 , trichromatic stain for collagen
        
        Reactive gliosis
        
        Gemistocytes swelling
      - Others: Prion Disease
        
        Spongiform encephalopathy (些微發炎) + Progressive dementia
        
        Prion protein (host genome coding 發生 Autocatalytic propagation，造成 gliosis & neuronal loss 但不會有明顯atrophy
        
        常見相關
        
        Scrapie (goats) 搔癢症
        
        Creutzfeldt-Jakob dz
        
        污染引起
        發展迅速
  - - - 中樞(CNS) oligodendroglial injury
        
        Axon degeneration: 白質去髓鞘化，被gliosis取代，造成 sclerosis
        
        好發於 periventricular areas
        
        發生在年輕成年人的自體免疫疾病
        
        Episodically , 會復發
        
        常見症狀
        
        Sensory disturbance
        
        Retrobulbar neuritis
        
        和溫度(因為自體抗體活性和溫度有關)、種族有關
        
        Variants
        
        Marburg MS
        
        Rapidly progressing
        
        Devic’s disease
        
        Large lesion, paralysis
- - - - 體染色體顯性
        
        TSC 1 (ch9)
        TSC 2 (ch16)
      - Tubers: various hamartomas
        
        Giant cell astrocytomas (腦室內壁像蠟炬般鈣化，gliosis 造成)
        
        Cardiac rhabdomyomas
        
        Renal ( melanocytes 相關)
        
        在皮膚會長青春痘的地方 adenoma sebaceous
      - Seizures
      - Ash-leaf spots
      - Sclerosis: 皮質缺乏正常的stratification，灰白質分界不明顯
- - - - Glial
        
        Astrocytoma
        
        Subtype
        
        Juvenile pilocytic astrocytoma
        
        Grade I
        
        發生在小孩
        
        位在Post. Fossa 的良性腦瘤
        
        Biphasic pattern:
        
        Pilocystic: Bipolar cells + Rosenthal fibers
        
        Multipolar cells + microcysts + 有時 granular bodies
        (3. Hyaline vessels + Endothelial proliferation)
        
        MAPK mutation
        
        若發生在 optic nerve 可能為 NF1 mutation
        
        Circumscribed, cystic, slow-growing
        
        Pleomorphic xanthoastrocytoma
        
        Infiltrating astrocytomas
        
        80% of adult primary brain tumors
        
        由大腦半球開始擴散到小腦、腦幹或脊髓
        
        Seizures, headache, focal neurological deficits
        
        依以下程度區別惡性程度：
        
        Nuclear pleomorphism
        
        Mitosis
        
        Necrosis (pseudopalisadingj
        
        Microvascular proliferation (腦瘤獨有)
        
        Grade II
        
        Diffuse astrocytoma
        
        Gemistocystic astrocytoma
        
        Grade III
        
        Anaplastic astrocytoma
        
        Gliomatosis cerebri
        
        Grade IV
        
        Glioblastoma
        
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        Oligodendroglioma
        
        Diffusely infiltrating, slow-growing
        
        長在大腦半球
        
        會區域性復發且有惡性可能
        
        IDH1/2 mutation
        
        Codeletion chromosomal arm 1p & 19q
        
        5C tumor
        
        HoneyComb
        Cyst
        Calcification
        Clear halos
        Delicate capillaries
        
        Ependymoma
        
        Ventricle-related
        
        Molecularly heterogeneous——variable clinical outcomes
        
        Children + adult
        
        Posterior fossa (children)
        
        Supratentorial
        
        Spinal cord (adult, Most common adult spinal tumor)
        
        Uniform cells in fibrillary matrix
        2. Perivascular pseudorosettes
        3. Ependymal rosettes & canals
      - Neuonal
      - Meningeal
        
        Meningioma
        
        大部分良性、發展慢
        
        來源： arachnoid layer 的 meningiothelial cells
        
        分級（三級）
        
        Grade I : 良性復發率低
        
        Meningothelial meningioma (Most common)
        
        Syncytial, whorled
        
        Fibrous (Fibroblastic) meningioma
        
        Spindle, collagen
        
        Grade II: 惡性、復發機會高
        
        Atypical meningioma
        
        Grade III: Pure malignancy
        
        Anaplastic (malignant) meningioma
        
        Chromosomes 22上的 NF2 gene 突變
        
        Psammoma bodies：其實正常腦膜就有這個東東
      - Germ cell
    - - Medulloblastoma
        
        Embryonal 所以好發於小時候
        
        Cerebellum/ Dorsal brain stem
        
        Undifferentiated cells (Grade IV, aggressive)
        
        Most common CNS embryonal tumor
        
        Most common malignant tumor of childhood
        
        惡性但是 radiosensitive
        
        主要為 WNT & SHH 基因突變
        
        病理變化
        
        小藍圓細胞（高度惡性表現）
        
        Homer- Wright pseudorosettes
        
        Pale islands
        
        Infiltration borders with CSF dissemination & drop metastases
    - - Hemagioblastoma
        
        組織特性
        
        Neoplastic stromal cells + Abundant small vessels
        
        70% Sporadic
        
        30% Familial 顯性 (Early onset): von Hippel-Linda’s (VHL) disease，通常為多發 (Renal cell carcinoma、Phaeochromocytoma)
        
        位置：腦幹、小腦、神經根
        
        良性、好發成人
    - - Craniopharyngioma
        
        Area: Suprasellar (Remnants of Rathke’s pouch)
        
        Behavior: childhood borderline，可手術切除。但體積越大越不好
        
        Clinical features, 視野障礙(偏盲)、內分泌缺陷、顱內壓上升(第三腦室受壓迫)
        
        Diagnosis: 影像學 + 病理(tooth germ cells + cholesterol-rich cystic spaces + spongy stellate reticulum wet keratin + calcification)
    - - 小孩：20%癌症為腦瘤，其中有70%在posterior fossa。補充小孩腫瘤排名
        
        白血病
        
        腦瘤
        
        一歲以下 Neuroblastoma /一歲以上 Nephroblastoma (Wilm’s tumor)
      - 特性
        
        一般惡性居多
        
        神經學功能異常
        
        會有spread, seeding pattern，但很少轉移
        
        Progression 不治療的話會越變越壞
- - - - Cortical
        
        Alzheimer’s Disease
        
        最常見造成失智症原因
        
        大部分為sporadic
        
        造成 cortical dysfunctions
        
        病理
        
        cerebral atrophy ：主要在 Frontal & parietal lobe
        
        Widened sulci
        
        Narrowed gyri
        
        Hydrocephalus ex vacuo代償性腦室變大
        
        Neurofibrillary tangle
        
        Tau protein (microtubule-associated)
        
        Hippocampus & cerebral cortex的 pyramidal cells
        
        量正比於退化程度
        
        Senile plaques
        
        Beta-amyloid
        
        Granulovacuolar degeneration
        
        Hirano body
        
        Familial form (Early onset)
        
        Down syndrome (chromosome 21)
        
        PS1 (chr14) PS2 (chr1): gain of function in r-secretase! A-beta42 變多
        
        FTLD
        
        Subtype: Variant Pick’s diseased
        
        Frontal lobe atrophy: knife-like gyri
        
        FTD-TDP-43 和ALS有關
        
        Pick’s body
      - Basal ganglion
        
        Parkinsonism
        
        Lewy bodies
        
        Parkinson’s disease: substantia nigra
        
        Dementia: cortex
        
        免疫染色：ubiquitin (+)
        
        Progressive supranuclear palsy
        
        Multiple systems atrophy
        
        Huntington’s
        
        體染色體顯性遺傳
        
        中年發病，平均存活15年
        
        Choreiform movements, dementia
        
        Chromosome 4 HD gene Huntington protein — 增加 trinucleotide CAG (Glutamine) repeat sequences* (Poly Q*)
        
        Astrocytomas: Neuronal loss in caudate & putamen
        
        Loss of GABA, Ach, substance P
      - Spinocerebellar
        
        Ataxia
      - Spinal cord
        
        Amyotrophic lateral sclerosis
        
        Spinal cord ventral horn cells + Betz cells loss
        
        SOD1, FUS, TDP-43 mutation