C.C.: Cough for 3 days with
progressive drowsy consciousness
<Brief History
>
This 71-year-old woman was
first diagnosed as myasthesia gravis in 1993, when she
developed ptosis but without diplopia, dysphagia or limbs
weakness. Her ptosis improved after taking pyridostigmine, but
recurred after she discontinued the drug. The chest CT
revealed a thymoma and thyomectomy was suggested but she
refused. She then received pyridostigmine and prednisolone at
outpatient clinic.
She was well until Sep,
1994, when she developed proximal muscle weakness. She stopped
taking pyridostigmine and prednisolone in the morning on Nov
2, 1994. Dyspnea with cyanosis developed soon. Symptoms
improved but persisted after parenteral vagastigmine and
increasing the dose of prednisolone. She then received plasma
exchange for three times. Dyspnea subsided later.
She underwent a thymomectomy
on Nov. 20 1994 and was followed up at outpatient clinic
therefore, with oral medication of pyridostigmine and
prednisolone.
She then remained in a
disease-free status until Feb, 25, 2000, when an episode of
myesthenic crisis occurred following a flu-like episode. She
developed progressive dyspnea and was intubated due to CO2
narcosis. Plasmapheresis was performed and
she was extubated on March 2 after 4
times of plasmapheresis. Her AchR Ab titer dropped from 55.94
nmole/l (Feb 25, 2000) to 29.16 nmole/l (Mar 6, 2000). She
was discharged with oral medication of pyridostigmine and prednisolone.
Because of progressive left
hip pain for 6 months, she underwent left total hip
replacement (THR) revision on Jul. 11, 2002. She developed
mild dyspnea after operation, so the dose of prednisolone was
increased from 10 mg qod to 20 mg qd. However, dislocation of
left hip joint occurred frequently and her dyspnea persisted.
Three days prior to this admission, she sufferrede from cough
with whitish sputum. Progressive drowsy consciousness was also
noted. She was then brought to ER, and CO2 narcosis was found.
She was then intubated, and was admitted to ICU for further
care.
檢體發現 (Physical findings): Conscious: clear Vital
sign: TPR: 37/ 70/12; BP: 104/52 mmHg (On ventilator) Eye:
conjunctiva: not pale, sclera: not icteric Neck: supple,
JVE (-), LAP (-) Chest: Symmetric expansion, Breath sound:
clear Heart: regular heart beat; no murmur
Abdomen: soft and flat, Bowel sound:
normoactive
Liver/ spleen: not palpable
No tenderness, no rebound tenderness
Shifting dullness (-)
Extremity: edema (-), cyanosis (-)
住院治療經過(Course and
Management): Ampicillin/subactam was
administered at ER for suspected respiratory infection.
Plasmapheresis was started on Sep. 5 once every other days.
Prednisolone 30 mg per day was also given. Her muscle power
improved significantly after the first plasmapheresis on Sep.
5. However, there was still CO2 retention during spontaneous
breathing trials through a T-piece. The maximal inspiratory
and expiratory pressures (Pimax and Pemax), tidal volume and
vital capacity (VC) continued to improve during the course of
plasmaphoresis. There was no CO2 retention after the 5th course of plasmaphoresis on Sep.
13. She was then extubation on Sep. 14, and was transfered to
the general ward on Sep.17.
<Laboratory
data>
|
WBC |
RBC |
Hb |
Hct |
MCV |
MCHC |
PLT |
|
/ul |
M/ul |
g/dl |
% |
fL |
g/dl |
/ul |
9/5 |
11380 |
3.93 |
12.0 |
38.0 |
96.7 |
31.6 |
251K
|
|
Seg |
Eos |
Baso |
Mono |
Lym |
9/5 |
87.3 |
0.1 |
0.2 |
3.3 |
9.1 |
|
BUN |
Cre |
GOT |
Bil(T) |
Cl |
Ca |
Na |
K |
CK |
CRP |
|
Mg/dl |
Mg/dl |
U/l |
Mg/dl |
Mmole |
Mmole |
Mmole |
Mmole |
U/l |
Mg/dl |
9/5 |
23.2 |
0.63 |
21 |
0.38 |
76.0 |
1.98 |
132.2 |
2.88 |
59 |
1.82
| 9/5
AchR Ab 18.2 nmol/l (< 0.2)
Weaning parameter
|
9/5 |
9/7 |
9/9 |
9/9 |
9/11 |
9/11 |
9/13 |
9/13 |
Plasmapheresis |
1st |
2nd |
|
3rd |
4th |
|
5th |
|
Vt |
|
|
310 ml |
|
|
369 ml |
|
445 ml |
RR |
|
|
20 /min |
|
|
18 /min |
|
20 /min |
VE |
|
|
6.2 l/min |
|
|
6.6 l/min |
|
8.9 l/min |
RSBI (f/Vt) |
|
|
64.5 |
|
|
48.7 |
|
45 |
Pimax |
|
|
-40 cmH2O |
|
|
-52 cmH2O |
|
-50 cmH2O |
PeMax |
|
|
50 cmH2O |
|
|
55 cmH2O |
|
50 cmH2O |
VC |
|
|
600 ml |
|
|
550 ml |
|
750 ml |
|
|
|
|
|
|
|
|
|
Vt: tidal volume; RSBI: rapid shallow breathing index; VC:
vital capacity
本病例為一患有多年重症肌無力之女性病患。於接受胸腺切除後其症狀曾一度有較長時間的緩解。但最近幾年在接受骨科開刀或有上呼吸道感染時就會引起重症肌無力症狀惡化(crisis),導致呼吸肌肉無力加重而引發呼吸衰竭。經抗生素治療,呼吸器輔助及血漿置換術治療後,血中抗Acetyecholine受體抗體值明顯下降,臨床症狀也迅速改善,
可順利脫離呼吸器。此病例之臨床表現可說是此疾病之典型案例。內科醫師在加護病房偶而會遇到這些呼吸衰竭之病患,對其適當處理應有基本認識。
|