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。 |