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Botulism (CLINICAL MANIFESTATIONS (Bilateral cranial neuropathy (Blurred…

- - - - fixed pupillary dilation
      - palsies of CN3,4,6
  - - - Smooth muscle paralysis
    - - 原因
        
        accessory breathing muscles paralysis
        
        pharyngeal collapse
        
        cranial nerve paralysis
        
        diaphragmatic paralysis
      - 需intubation和mechanical ventilation
      - 40%
      - 導致tidal volume不正常
  - - - 1週～12月
      - 嚴重度與細菌對宿主免疫力有關
      - constipation
      - weakness
        
        weak cry
        
        floppy neck
      - feeding difficulties
        
        reduced ability to suck and swallow
      - descending or global hypotonia
      - drooling
      - anorexia
      - irritability
      - Ptosis
    - - 吃下毒素後12~36小時發作
      - Prodromal symptoms
        
        nausea
        
        vomiting
        
        abdominal pain
        
        diarrhea
        
        Autonomic symptoms
        
        sore throat
        
        dry mouth
        
        dizziness
      - botulinum toxin type E
        
        home-salted fish
      - incubation period:數小時～2週
    - - 沒有prodromal GI symptoms
      - incubation period長達10天
      - 唯一一型造成fever,leukocytosis
        
        concurrent bacterial infection
      - injection drug use病史
  - - - incremental increase in M wave amplitude
- - - - 但會falsely positive
  - - - salivation and erectile function
- - - - Heptavalent botulinum antitoxin (HBAT)
        
        Equine
        
        A~ G
        
        children older than one year of age and adults
      - Botulinum immune globulin
        
        BIG-IV or BabyBIG
        
        infants less than one year of age
    - - Because botulinum antitoxin cannot reverse paralysis
- - - - 8種 antigenically distinct botulinum toxins (A to H)
        
        A, B, E, F, G, H與人的疾病有關
      - 其他strains
        
        無法容易察覺
      - A和B
        
        產生proteolytic enzymes
        
        使食物毀壞
    - - marine sediment
      - soil
      - seafood
      - fruits
      - vegetables
      - home-canned foods
      - Honey
        
        Infant botulism
    - - Spore
        
        germinate and grow into toxin-producing bacilli
        
        temperature
        
        有些strains可在4°C生長
        
        25 to 37°C for ideal growt
        
        Low acidity (pH >4.6) water
        
        Restricted oxygen exposure
        
        anaerobic or semi-anaerobic environment
        
        heat resistant
        
        100°C存活5小時
    - - heat-labile polypeptide
        
        80°C
        
        light and heavy chain
- - - - A, B, E, and rarely F, G, and H造成人類疾病
        
        F:Clostridium barati
        
        E:Clostridium butyricum
    - - 被bacterial proteases切成fully active neurotoxin
        
        接在presynaptic nerve terminal的receptor上
        
        internalization進神經細胞
        
        translocation across the endosomal membrane
        
        intracellular endopeptidase activity
        
        against proteins necessary for neurotransmitter release
    - - motor neurons
      - sensory neurons
      - cholinergic neuromuscular innervation
        
        striated muscle
        
        smooth muscles
        
        tear, salivary, and sweat glands
      - excitatory synapses
        
        主要
      - inhibitory synapses
      - 神經傳導物質
        
        dopamine, serotonin, somatostatin, noradrenaline, and gamma aminobutyric acid.
    - - 但可影響CNS
        
        systemic spread
        
        axonal retrograde or anterograde
    - - 被胃，小腸，大腸吸收
        
        耐胃酸