Case Discussion A 37-year-old man presented to a local hospital in Taiwan on February 4, 2003 with high fever, headache and generalized papulovesicular skin rash on his face, trunk, and extremities characteristic of chickenpox. His 8-year-old daughter had similar symptoms one week before his illness but recovered without significant sequelae. Unfortunately, he developed progressive four-limb weakness and shortness of breath seven days after onset of chickenpox. On the next day, he was unable to ambulate due to weakness and developed hypercapnic respiratory failure, requiring intubation for ventilatory support. He was admitted to the intensive care unit (ICU) of our institute on February 12, 2003. His past medical history had been notable for bronchial asthma since early childhood. On examination, he was conscious and well oriented. The following were his vital signs: respiratory rate 20/min, blood pressure 112/70 mm Hg, temperature 100.4°F, and heart rate 110 beats/min. He was completely dependent on the ventilator with no spontaneous inspiratory trigger. Chest auscultation revealed clear breathing sounds and without cardiac murmurs. Multiple encrusted popular lesions consistent with resolving varicella were seen on the face, trunk, and extremities. Neurological examination revealed flaccid paresis in all four extremities, but only facial expression and closure of eyelids were preserved initially. The bladder was catheterized. There was generalized areflexia, and plantar responses were flexor. Sensation to light touch and temperature were relatively preserved. However, progressive complete facial diplegia without ability to close the eyes was observed five days after intubation. Cerebrospinal fluid analysis showed no pleocytosis, and levels of protein (0.35 g/l) and sugar (5 mmol/l) were normal. The titers of varicella-zoster virus IgG and IgM antibody showed significant increases in the initial and later check-up. The nerve conduction velocity (NCV) and electromyography performed two days after hospitalization revealed absent compound muscle action potential (CMAP) amplitudes with preserved sensory action potential, suggestive of acute motor axonal neuropathy. He was treated with IVIG 400 mg/kg per day for five days, but without consequence. Two courses of plasmapheresis also failed to improve his quadriplegia and respiratory failure. Episodic bradycardia occurred frequently during the first two weeks of hospitalization. Because of prolonged intubation, tracheostomy was performed on the 20th day of hospitalization. The patient's facial expression began to recover gradually after 30 days of hospitalization, and spontaneous respiratory effort was first noted on the 36th day of hospitalization. He had a prolonged recovery period from weakness of respiratory muscles. Sequential weaning parameters are shown in Table 1. He was finally liberated from mechanical ventilation on the 145th day of hospitalization. Muscle power of the lower limbs and the routine activity of daily living did not improve until the 195th day. Table 1-- Sequential Weaning Parameters of a 37-year-old Man with Respiratory Failure Following Chickenpox-associated Guillain-Barre Syndrome   4/4/2003  5/19/2003 6/3/2003 7/3/2003 Pimax (cm H2O) -15 -20 -42 -64 Pemax (cm H2O)  -10 20 34 46 RSBI 143 93 51.8 49 MV (L/min) 3.65 3.95 8.5 13.72 f (breath/min ) 23 19 29 26 VT (ml) 156 208 405 528 Abbreviations: Pimax  = maximum inspiratory pressure; Pemax= maximum expiratory pressure; RSBI = rapid shallow breathing index; MV = minute ventilation; f = respiratory frequency; VT = tidal volume Laboratory data: 1. CBC/DC:   WBC RBC  Hb  Hct MCV PLT   K/uL M/uL G/dL %  fL  K/uL 920214 8.14 4.12 12.8 38.1 92.5 278 920302 13.57 3.64 11.3 35.4 97.3 183 920402 6.64 3.42 10.6 32.0 93.6  250 2. Coagulation:   PT PT Cont  PTT  PTT Cont  INR   Sec Sec Sec Sec   920225 15.3 12.6 47.8 37.4 1.3 920303 14  13 41.2 36.5 1.2 3. Biochemistry   BUN Cre Na K T-Bil D-Bil GOT GPT   mg/dl mg/dl mmole/l mmole/l mg/dl mg/dl U/l U/l 920214 17.1 0.62 136.9 5.15  0.45    28   920301 15.5 0.62 142.1  3.8 0.33   110   920401 10.6 0.4 139 4.0 0.4   19 15 4. ABG   pH PCO2 PO2 HCO3- BE   * mmHg mmHg mEq/l mEq/l 920223 7.5 30.6  113.9 23.8  1.7 920305 7.36 42.4 70.0 23.1 -1.7 920308  7.45 33.8 120.8 23.0 -0.4 920326 7.47  35.4 146.9 25.5 2.3 5. C-reactive protein   CRP mg/dl  920214 >12 920217 2.35 920220 2.2 920224 0.03 6. CSF study   Pandy's Test  WBC  L/N Glu TP       x11/9/μl    mg/dl mg/dl 920212 Negative 0 0/0 89 35.5 920213 Negative 0 0/0 107 31.4 7. CSF 920213    Varicella-Zoster Virus IgM Antibody: Negative 920213    Varicella-Zoster Virus Antibody: 1:2+ Virus isolation: no virus isolated Blood 920213    Varicella-Zoster Virus IgM Antibody: positive 920213    Varicella-Zoster Virus Antibody: 1:256+ 920313    Varicella-Zoster Virus IgM Antibody: equivocal 920313    Varicella-Zoster Virus Antibody: 1:2- 本病例為一曾有支氣管氣喘病史的男性，於四肢肌肉無力及呼吸衰竭前一個禮拜有水痘病毒感染的情形，在排除了其他可能性，例如血管炎，有機磷和鉛中毒，臘腸毒素中毒，白喉，紫質沉著病，及局部脊髓或馬尾症候群等，由臨床、實驗室及神經電生理的檢查判斷為Guillain-Barre症候群(GBS)，於是給予IVIG達到2g/kg的劑量後，再給予兩個療程的血漿減除術，期間病人曾出現心搏過緩的情形，推斷為GBS之自主神經功能失常造成，病人的肌肉無力及呼吸衰竭現象，有緩慢改善中，但仍須仰賴呼吸器的輔助。住院期間併有院內呼吸道感染及泌尿道黴菌感染的情形，所幸在抗生素的治療下皆得到控制，最後病人於施行氣管切開術後轉至普通病房繼續照顧，並在住院後第145天脫離呼吸器，第195天下肢肌力及生活自理才開始恢復。 繼續教育考題 1. (D) Which description about Guilain-Barre syndrome (GBS) is wrong? A Autoimmune in nature B Males and females are equally at risk C In western countries adults are more frequently affected than hildren D Fever is a common initial presentation. 2. (C) Which description about the clinical manifestations of GBS is wrong? A A rapidly evolving areflexic motor paralysis B with or without sensory disturbance C The usual pattern is an descending paralysis D The legs are usually more affected than the arms 3. (D) Which description about GBS is true? A The lower cranial nerves are also frequently involved B About 30% require ventilatory assistance at some time during the illness. C Autonomic involvement is common. D All of the above 4. (D) Which has been identified as antecedent infections of GBS? A Campylobacter jejuni. B human herpes virus infection (often CMV or Epstein-Barr virus) C HIV D All of the above 5. (D) Which is the least common antecedent events of GBS? A Compylobacter jejuni B Mycoplasma pneumoniae C Cytomegalovirus D Varicella-zoster virus 6. (D) Which description about the CSF findings in GBS is true ? A An elevated CSF protein level is common B Pleocytosis is unusual. C CSF is often normal when symptoms have been present for <= 48 h D All of the above 7. (B) Which therapy is currently considered not effective for GBS? A IVIG B steroid C plasmapheresis D IVIG following plasmapheresis 8. (E) Which description about the treatment of GBS is true?  A Treatment should be initiated as soon after diagnosis as possible B High-dose IVIG or plasmapheresis are equally effective C Frequent turning and skin care are important D Daily range-of-motion exercise is essential to avoid joint contractures. E All of the above 9. (A) Which is the major cause of death in severe GBS? A  pulmonary complication B massive bleeding C intra-abdominal sepsis D catheter-related bacteremia E IICP 10. (D) Which is necessary for patients in the worsening phase of GBS? A Admission to a critical care setting B Close monitoring of vital capacity and cardiovascular status C Chest physiotherapy in patients with ventilatory support D All of the above 答案解說 (D) Fever and constitutional symptoms are absent at the onset, and, if present, cast doubt on the diagnosis. (C) The usual pattern is an ascending paralysis (D) All descriptions are correct. The lower cranial nerves are also frequently involved, causing bulbar weakness and difficulty with handling secretions and maintaining an airway. (D) Seventy-five percent of cases of GBS are preceded by an acute infectious process, usually respiratory or gastrointestinal. 20 to 30% of all cases occurring in North America, Europe, and Australia are preceded by infection or reinfection with Campylobacter jejuni. A similar proportion is preceded by a human herpes virus infection, often cytomegalovirus or Epstein-Barr virus. Other viruses and also Mycoplasma pneumoniae have been identified as agents involved in antecedent infection (D) Please see the answer to question 4 (B) Typical findings include an elevated CSF protein level (100 to 1000 mg/dL) without accompanying pleocytosis. The CSF is often normal when symptoms have been present for 48 h; by the end of the first week the level of protein is usually elevated.  (B) Glucocorticoids have not been found to be effective in GBS. (E) All descriptions are correct (A) Pulmonary complications are the major causes of death in severe GBS (D) Most patients in the worsening phase of GBS require monitoring in a critical care setting, with particular attention to vital capacity, cardiovascular status, and chest physiotherapy. 1. Chest physiotherapy in patients with