<Case History>
This 60 year-old
woman,a case of hypertension and asthma for years, suffered
from myocardial infarction 10 years ago. She had a fall into a pool
2 weeks before admission and began to have dry cough,
general malaise, muscle pain and poor appetite since then.
The symptoms persisted under medication from a local doctor.
She suffered from headache, nausea and weakness on April 26
and was admitted to local hospital under the impression
of pneumonia on April 27. She became sickner and even was too weak to
walk. On April 28, she was noted be have tonic seizure with
loss of consciousness. Similar episodes were noted about six
times within 8 hours. Her consciousness became drowsy and she
was intubated. She was transferred to our hospital for further
care on April 29.
She denied
other systemic disease, smoking, alcohol
drinking or allergy history. She did not travel
recently. The family history is unremarkable.
On arrival at
our ER, her blood pressure was 146 / 94 mmHg, temperature was
39 ℃, and pulse rate was 134 /min. She was
intubated and consciousness was drowsy. The pupils were isocoric with
prompt light reflex. The conjunctiva was not pale and the sclera
was anicteric. The neck was supple and there was
neither goiter nor lymphadenopathy. The jugular vein was not
engorged. The breath sounds were coarse without wheezes and
the heartbeats were regular without audible murmur. The
abdomen was soft without tenderness or rebounding pain. Liver
and spleen were impalpable. The extremities were not edematous
and there was no skin lesion.
Hemogram revealed markedly
leukocytosis with left shift (WBC=18,730 /uL, neutrophil
80.6%) and thrombocytopenia (platelet=37,000/uL). PT was
13.8s/12.6s and PTT was 74.5s/37.5s. Peripheral blood smear
showed marked thrombocytopenia, no giant platelets, no
fragmented RBC, no RBC anisocytosis. Blood chemistry studies
showed hyperglycemia, abnormal renal function, abnormal liver
function, rhabdomyolysis, slight hyponatremia, and elevated
C-reactive protein (shown in the data list). Arterial blood
gas disclosed metabolic acidosis (PH 7.31, PCO2 24.1 mmHg,
HCO3- 11.8 mEq/L) and lactic acid was 5.2 mM. Chest X ray was
clear. Under the impression of severe sepsis probably due to
meningoencephalitis, intravenous ceftriaxone and acyclovir
were given. Brain CT was arranged and she was admitted to ICU
immediately.
Brain CT did not
reveal significant finding. CSF study disclosed high opening
pressure (open pressure=270 mmH2O, close pressure=120 mmH2O)
and high protein level (160 mg/dL), no pleocytosis (WBC=2;
lymphocyte=1 and neutrophil =1), no hypoglycorrhachia
(sugar=164 md/dL) but RBC=
230/uL. Seizure did not recurred after the use
of dilantin. EEG revealed mild diffuse cortical dysfunction. Brain
MRI disclosed bilateral temporal lobe swelling compatible with encephalitis.
Infection specialist suggested further work-up of atypical pathogen due to
multiple organ involvement. Doxycycline and penicillin-G were added. Thrombocytopenia, coagulopathy,
renal function, liver function, metabolic acidosis and consciousness
improved gradually and she was extubated on the third
day of admission. She was transferred
to general ward. Fever subsided gradually. She was
discharged on 5/10 with stable condition.
Blood cultures of bacteria and
fungus were negative. The CSF cultures of bacteria, fungus,
virus and TB were negative. Cryptococcal antigen of CSF and
blood were negative. Autoimmune profiles were negative. HSV
PCR of CSF was negative. Serologies for mycoplasma pneumoniae,
leptospirosis, Japanese encephalitis virus were negative.
Paired serum of rickettsia was positive for Orientia
tsutsugamushi.
<Laboratory
Data>
|
GLU |
UN |
CRE |
Na |
K |
Cl |
T-BIL |
AST |
CK |
CK-MB |
|
mg/dl |
mg/dl |
mg/dl |
mM |
mM |
mM |
mg/dl |
U/l |
U/l |
U/l |
910427 |
|
24 |
1.3 |
|
|
|
|
132 |
|
|
910429 |
261 |
53 |
2.79 |
128.8 |
4.14 |
100.0 |
0.65 |
84 |
571 |
31 |
910508 |
|
13 |
0.6 |
145.0 |
3.5 |
|
|
30 |
|
|
|
Albumin |
globulin |
LDH |
ALP |
lactic acid |
CRP |
|
g/dL |
g/dL |
U/L |
U/L |
mM |
mg/dL |
910429 |
1.97 |
2.55 |
995 |
179 |
5.32 |
9.62 |
|
WBC |
RBC |
HB |
HCT |
MCV |
MCH |
MCHC |
PLT |
|
K/μL |
M/μL |
g/dL |
% |
fL |
pg |
g/dL |
K/μL |
910427 |
|
|
14.2 |
|
|
|
|
118 |
910429 |
18.73 |
6.8 |
14.6 |
43.7 |
64.3 |
21.5 |
33.4 |
37 |
910429 |
17.14 |
5.32 |
11.4 |
33.4 |
62.8 |
21.4 |
34.1 |
12 |
910508 |
6.33 |
4.13 |
9.4 |
29.5 |
71.4 |
22.8 |
31.9 |
239
|
|
Seg |
Eos. |
Baso. |
Mono |
Lym. |
910429 |
80.6 |
0.1 |
0.2 |
6.9 |
12.2 |
|
PT |
PT Cont |
PTT |
PTT Cont |
|
sec |
sec |
sec |
sec |
910429 |
13.8 |
12.6 |
74.5 |
37.5 |
910501 |
11.2 |
12.5 |
38.2 |
35.8 |
|
PH |
PCO2 |
PO2 |
HCO3 |
BaseExcess |
|
* |
mmHg |
mmHg |
mEq/l |
mEq/l |
910429 |
7.31 |
24.1 |
119.1 |
11.8 |
-12.7 |
910504 |
7.44 |
40.3 |
77.3 |
27.0 |
3.1 |
項 目 |
檢驗值 |
參考值 (單位) |
Fibrinogen |
215 |
214 ~ 474 (mg/dl) |
3P |
1+ |
Negative |
FDP |
5-10 |
<10 (μg/ml) |
D-Dimer |
2.96 |
<0.5 (μg/ml) |
SPINAL FLUID
|
Pandy's Test |
None-Apelt |
Cell Count L/N |
India Ink |
910429 |
Negative |
Negative |
WBC2; (RBC:230/uL) L:N=1:1 |
Negative |
病例分析
此60歲女士一開始以非特異性(non-specific)症狀表現,疲倦,全身沒力,肌肉酸痛,胃口變差,然後才出現頭痛、噁心、發燒、白血球增高、最後發生痙攣及神智不清,整個過程會讓人想到encephalitis。但是病患並無明顯的neck
stiffness,且出現許多器官機能受損(肝臟GOT
GPT升高,腎功能急速惡化,rhabdomyolysis),所以可能的診斷是encephalitis with
multiple organs
involvement,非典型的致病菌(例如:leptospirosis, rickettesia等)必須列入考慮,在empirical
antibiotis的使用下,病情改善,而能很快的出院。最後在疾病管制局配對血清學(paired serum)檢驗下,才能確定是scrub
typhus。
此病患在高度懷疑有encephalitis with
multiple organs involvement的情況下,仍有其他systemic
diseases需要考慮,例如autoimmune
diseases(如SLE等全身性vasculitis),thrombotic thrombocytopenic
purpura(TTP)等。而血液抹片並沒有破碎的RBC,RBC形狀正常可排除TTP的可能。在抗生素治療下,病情改善迅速,而autoimmune
profile皆negative,比較不像autoimmune diseases。
在懷疑encephalitis的情況下,不管是細菌或病毒培養,或血清免疫學的檢查,皆須有幾日或幾星期的檢查。必須根據臨床的判斷儘速給予適當的抗生素治療,第三代cephalosporin對blood-brain
barrier通透性佳,且對大部份革蘭性陰性菌有效,penicillin-G對listeriosis,
leptospirosis有效,minocycline對rickettsia及chlamydia有效,acyclovior可治療herpes
simplex encephalitis。
恙蟲病又稱斑疹傷寒(scrub
typhus),台灣地區是屬於恙蟲病的分佈區域,人遭蟲叮咬而感染,叮咬處會結痂形成eschar,且可能會有淋巴腫大及皮膚紅疹,但並不是所有的病患皆有這些表現。Orientia
tsutsugamushi是obligate intracellular
bacterium,進入人體後侵犯endothelial cells,而造成廣泛的vasculitis
(可包括capillaries, arterioles,及small
arteries,而產生各個器官的症狀,嚴重的病患可產生pneumonitis, meningitis,
encephalitis以及DIC,沒有適當的治療可能造成死亡。
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