1. Quantitative Analysis of CSF Total Protein and Albumin 醫學四 B9402036 陳佑丞 指導老師：張健宏醫師

2. Quantitative Analysis of CSF Total Protein and Albumin Blood-CSF barrier : Physical barrier for the diffusion and filtration of macromolecules from blood to CSF. The integrity of the barrier and the CSF bulk flow determine the protein content of the CSF. CSF to serum albumin concentration quotient (Q alb ): used to evaluate blood-CSF barrier integrity. - Q alb ↑  integrity↓ - Q alb ↓  integrity↑

3. Quantitative Analysis of CSF Total Protein and Albumin The Q alb is influenced by body weight, sex, degenerative lower back disease, hypothyroidism, alcohol consumption, and smoking. Elevated CSF protein concentration can be found in the majority of patients of bacterial, cryptococcal, tuberculosis meningitis and neuroborreliosis. In viral neuroinfections CSF protein concentrations are raised to a lesser degree.

4. Albuminocytological dissociation

5. 定義 : Elevated total protein concentration with normal CSF cell count. 解釋 : Dissociation 意指「分離，不相稱」。即 CSF 裡的蛋白質 ( 主要是 Albumin) 升高時 CSF 裡的細胞 ( 主要是 mononuclear cell) 沒 有跟著升高的現象。

6. Albuminocytological dissociation 臨床指標 : Acute and chronic inflammatory demyelinating polyradiculoneuropathy. (Guillain–Barré syndrome, GBS) *Protein levels may be normal during the first week. Our case: Total Protrin (TP): 63.4 mg/dL WBC: 0

7. Guillain–Barré syndrome (GBS)

8. 大綱 Introduction Epidemiology Clinical Features Laboratory Features Pathogenesis

9. Introduction 1895, Landry described a neuropathy characterized by acute ascending paralysis. Guillain, Barré, and Strohl noted the areflexia and albuminocytological dissociation in the CSF associated with this neuropathy. The contribution of Landry and Strohl have been neglected and the neuropathy has been most commonly referred to as Guillain–Barré syndrome.

10. Introduction The earliest feature: edema of the proximal nerves, followed by degeneration of the myelin sheaths within the first week of the illness. They did not appreciate inflammatory cells infiltrate until the later in the course of the illness. Prominent perivascular inflammation in the dorsal root ganglion, cranial nerve, and randomly along the whole length of peripheral nerves along with segmental demyelination adjacent to the areas of inflammation. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

11. Epidemiology AIDP is the most common cause of acute generalized weakness. Incidence: 1~4 / 100,000 可發生在任何年齡, 最常見在 38~40 歲, 男性居多

12. Epidemiology 60-70% of patients with AIDP have a history of a recent infection a few weeks prior to the onset of the neuropathy -Campylobacter jejuni (32%) -Cytomegalovirus (CMV) (13%) -Epstein-Barr virus (EBV) (10%) -Mycoplasma pneumoniae (5%)

13. Clinical Features AIDP usually presents with numbness and tingling in the feet that gradually progresses up the legs and then into the arms. Numbness and paresthesia can also involve the face. Severe, aching, prickly, or burning neuritic pain sensations in the back and limbs are present in at least half of the patients. Large fiber modalities (touch, vibration, position sense) are more severely affected than small fiber functions (pain and temperature perception).

14. Clinical Features Progressive weakness typically accompanies the sensory disturbance. The severity can range from mild distal weakness to complete quadriplegia and need for mechanical ventilation. Weakness is usually first noted in the legs, and ascended to the arms, trunk, head, and neck. Ophthalmoparesis and ptosis develop in 5~15% of the patient.

15. Clinical Features The bowel and bladder are usually spared, although they may become involved in particularly severe disease state. Muscle stretch reflexes progressively diminished or becomes unobtainable. 病程 : -AIPD (acute) : 2-4 weeks -SIPD (subacute) : 4-8 weeks -CIPD (chronic) : >8 weeks

16. Laboratory Features Albuminocytological dissociation In approximately one-third patients, CSF protein levels are normal within the first week. Enhancement of the nerve roots may be appreciated on magnetic resonance imaging (MRI) of the spine. Antiganglioside antibodies, particularly anti-GM1 IgG antibodies

17. Pathogenesis T-cell mediated process molecular mimicry *glycolipids  gangliosides

18. Reference F. Deisenhammer, A. Bartos, R. Egg, N. E. Gilhus, G. Giovannoni, S. Rauer and F. Sellebjerg, Guidelines on routine cerebrospinal fluid analysis. Report from an EFNS task force. Amato AA, Guillain-Barré syndromeand related disorders, Rev Mex Neuroci 2005; 6(5): 455-469.

19. Thank You