Case Discussion Name: 林x x             Sex: M Brief history       This 28 year-old male patient was transferred from ECKH on Dec 6, 2000 He had been quite healthy until early November when he developed easy fatigability. He took some herbal drugs for almost one month. On November 22, a generalized tonic-clonic seizure occurred while he was working. The duration was 3 to 4 minutes and subsided spontaneously. Neither URI symptom nor other discomfort was noted before the episode. He sough medical advice at one hospital where EEG, brain CT were performed but no definite diagnosis was made. Unfortunately, similar episode recurred on Nov 24 and Nov 27. Intermittent fever was also noted during this period. There was no headache, nausea, vomiting or diplopia, and his consciousness remained clear and alert.       On Nov 29, the forth episode occurred and he was sent to another local hospital but the lumbar puncture did not reveal evidence of CNS infection. The CxR was clear (Fig, 1). The brain MRI increased enhancement of temporal lobe. The CBC revealed leucopenia, anemia and thrombocytopenia. Acyclovir was given since Nov 30 under the impression of aseptic meningitis. His conditions improved a little. However, fever with dyspnea developed on Dec 4, followed by generalized papular rash over the trunk. Hypoxemia developed and CxR revealed bilateral increased infiltration (Fig. 2), ARDS was suspected and he was transferred to ICU for further treatment. Spiking fever persisted despite the use of strong antibiotics, and his consciousness also worsened gradually. Thus he was transferred to NTUH on Dec 6, 2000.       On admission, his consciousness was drowsy; his blood pressure was 111/55 mm Hg, pulse rate 101 per minute, respiratory rate 19 per minute. His conjunctiva was pink and sclera was not icteric. His pupils were isocoric and light reflex was prompt. There were multiple painful lymph nodes over bilateral neck and auxiliary regions. His chest wall movement was symmetric and breathing sounds were coarse. The abdomen was soft and flat. The liver and spleen were not palpated. There was no pitting edema of his limbs. Laboratory data  1. CBC/DC Date WBC RBC Hb Hct MCV Plt   K/ul M/uL G/dL % FL K/uL 891206 2.28 3.22 10.1 29.0 93.5 26 891210 4.57 2.88 9.0 26.9 97.5 20.9 891215 1.13 3.06 9.6 28.8 94.1 68.0 891217 0.57 2.89 9.1 27.0 93.4 76.0 891221 3.95 2.7 8.4 25.5 94.4 165 2. Biochemistry Date A/G Bil(T) GOT LDH BUN Cre Na K Ca 891206   1.5 441 4450 19 1.0 139 4.0 1.87 891211 2.4/2.4 1.1 97   21 0.6 138 4.0 1.77 891218     62 653 15 0.5 130 5.0 1.88 3. Urinalysis Date Sp.Gr pH Pro Glu Ket Uro WBC RBC 891211 1.018 6.0 30 - - 0.1 0-1 0-1 891222 1.009 6.5 - - - 0.1 0-2 2-4 4. Lumbar puncture Date Pandy's Cell L/N Sugar India ink TP LDH IgG 891207 ++ 0 0/0 126 - 0.203 214   891215 +/- 0 0/0 67   0.11 93 6 5. Serology Date Item Result 891211 RA factor 1:40( - ) 891212 Anti-DNA(FA) 1:10( - ) 891212 Anti-ENA(RNP) positive 891212 Anti-ENA(SCL-70) negative 891212 Anti-ENA(SM) positive 891212 Anti-ENA(SSA) positive 891212 IgA 174 891212 IgG 737 891212 IgM < 20.0 891212 Anti-ENA(SSB) negative 891212 Anti-Nuclear antibody 1:1280 (+) speckle 891212 C3 Quantitation 90 891212 C4 Quantitation 17.6 891212 Coombs's test direct 2+ 891212 Coombs's test indirect negative 891218 Haptoglobin 38.3 6. Microbiology Date Specimen Result 891207 VDRL Non-reactive 891207 CSF No growth 891207 CSF Cryptococcal antigen: negative 891207 CSF HSV antibody 1:2(+) 891208 Urine Legionella urinary antigen: negative 891206 serum Proteus OX19 1:20 ( - ) 891206 serum Proteus OX 2 1:20 ( - ) 891206 serum Proteus OX K 1:20 ( - ) 891206 serum S. typhi H 1:20 ( - ) 7. Cytology Date Specimen Result 891207 CSF Non-made 891215 CSF Non-made 891208 BAL Negative 8. Blood pH/Gas Date pH PCO2 PO2 HCO3 BE FiO2 891208 7.46 25.4 150 17.6 -4.9 50% 891210 7.39 35.7 79.2 21.1 -2.9 40% 891212 7.38 45.8 109.4 26.3 1.4 35% 891216 7.41 52.2 98.8 32.2 7.0 30% Course and treatment  The chest CT revealed patchy alveolar consolidation over bilateral lung fields. (Fig. 3 and Fig 4.) Episodic seizure persisted despite the use of phenytoin. Lumbar puncture on Dec 7 revealed normal intracranial pressure. EEG on Dec 8 revealed diffuse cortical dysfunction. HSV encephalitis was highly suspected. In addition, ANA was strongly positive and serum complement levels were decreased. Autoimmune disease was also suspected. With supportive care hi skin rash and systemic lymphadenopathy resolved gradually. The follow-up CxR on Dec 11 also improved quickly (Fig. 5). Steroid was given on Dec 12 and the fever subsided. Acyclovir was discontinued after being used for 18 days and no recurrence of seizure was noted thereafter. However, his consciousness disturbance did not improve. Tracheostomy was performed Dec 21 and he was transferred to general ward for further care. 病情解說         本病例為一年輕男性產生高燒, 抽筋及意識障礙。由CSF的檢查及MRI，EEG的結果大概可推測此病患有一嚴重之HSV腦部感染。雖經Acyclovir治療仍留下許多後遺證。此病例有興趣之發現在於其肺部病變。雖經痰及支氣管鏡所得細菌學檢查仍無確切有肺炎之證據。此病例兩側肺野病變在意識不清及抽筋後產生，且於數天之內迅速消失，且PaO2也迅速改善， 神經性肺水腫 (Neurogenic pulmonary edema)應為合理之診斷。此病患之淋巴節及血液學上之結果也顯示其可能有一已存在之自體免疫疾病，(如SLE)。         神經性肺水最常出現於頭部外傷合併腦出血之病患，單純因seizure之後產生者只占2-5%左右。診斷必須排除其他肺水腫或感染之可能性。       繼續教育考題 1. (C) Which of the following conditions is less likely for a patient who recovered rapidly from a respiratory failure with bilateral dense alveolar infiltrates ? A Cardiogenic lung edema B Neurogenic pulmonary edema C Pneumonia with ARDS D Pulmonary hemorrhage 2. (D) Neurogenic pulmonary edema can occur after which of the following conditions ? A Head injury B Subarachnoid hemorrhage C Seizure D All of the above 3. (C) Which description below is WRONG for postictal pulmonary edema ? A May develop immediately after a seizure or may be delayed for several hours B It occurs most frequently in young patients with idiopathic seizure and in those associated with expanding CNS lesions C Is the most common form of neurogenic pulmonary edema D May resolve within several days after surgical relieve of IICP 4. (D) Which description below is true for the theories about the pathogenesis of  neurogenic pulmonary edema ? A May be related to the transient release of large amount of catecholamines B May be associated with hydrostatic as well as permeability changes in the pulmonary circulation C Elevation of the levels of some neuropeptides have been found in the bronchoalveolar lavage in neurogenic pulmonary edema D All of the above 5. (D) Which statements below is WRONG for the typical CxR finding of neurogenic pulmonary edema ? A Usually there is no cardiomegaly or Kerly's B lines B May manifests as bilateral, homogeneous airspace consolidations C Commonly involves the upper and apical portions of bilateral lungs D Only involves bilateral lower lung fields 6. (D) Which statement about the management of neurogenic pulmonary edema is true? A Mechanical ventilatory support may be used for severe cases B High levels of PEEP may be used to improve arterial oxygenation, but special concern should be paid to its effects on IICP C Diuretics and osmotic agents may be used to control IICP and blood volume D All of the above 7. (C) Which of the following description about HSV encephalitis is WRONG ? A Is the most common cause of sporadic focal encephalitis B Inferior frontal and anterior temporal lobes are the most common sites of involvement C There is low risk of severe residual neurologic deficit D The mortality rate is about 10-30% 8. (B) Which of the following description about the diagnosis of HSV encephalitis is WRONG ? A PCR analysis of CSF may provide rapid diagnosis of this disease B CSF culture is useful in the diagnosis C EEG may reveal periodic sharp wave activity temporally or with background of focal or diffuse slowing D Brain biopsy is the gold standard for diagnosis 9. (A) Which of the following description about the management of HSV encephalitis is WRONG ? A Acyclovir should be given at 10-12.5 mg/kg IV push every 8 hours B Acyclovie should be start early to maximize best possible outcome C The duration of acyclovir therapy is 10-14 days D None of the above 10. (D) Which of the following findings is compatible with the suspicion of systemic lupus erythematosus (SLE) in this case ? A Systemic lymphadenopathy B ANA titer strongly positive C Pancytopenia and hemolytic anemia D All of the above

答案解說 答案解說: (C) Pulmonary edema or hemorrhage should be suspected in the setting o rapid resolution of bilateral CxR opacification (D) Neurogenic pulmonary edema (NPE) is a recognized, serious, relatively uncommon, acute complication of cerebral insults of various types. (C) Subarachoiund hemorrhage is the most common cause of form of neurogenic pulmonary edema. Postictal pulmonary edema only account for 2-5% of al cases of neurogenic pulmonary edema. (D) Despite numerous animal experimental investigations and case reports, the pathological physiology is not yet elucidated. An acute cerebral insult with raised intracranial pressure is accompanied by violent activation of the sympathetic system and increase in the concentrations of circulating catecholamines (D) CxR: may show bilateral, homogeneous airspace consolidations that predominate in the upper and apical portions of both lungs in about 50%n of cases (D) All descriptions are correct。 (C) HSV encephalitis: high risk of severe residual neurologic deficit (B) CSF culture : seful of no use in the diagnosis (A) IV acyclovir should be given slowly (over 60 minutes) (D) All findings listed are compatible with the suspicion of SLE in this case Top of Page