A 22-year-old lady, an aboriginal Taiwanese, was admitted because of rapidly
deteriorated consciousness in three days with intermittent fever.
This 22-year-old kindergarten teacher, living in Taipei county, is a patient of
systemic lupus erythromatosus (SLE) diagnosed at anonther hospital three years ago,
with the initial presentation of spontaneous gum bleeding, high fever, malar rash,
and discoid rash over trunk and both thighs. Positive antinuclear antibody (ANA)
and decreased serum complement level were noted. Her presentation met the
revised American Rheumatology Association (ARA) criteria for SLE. She took
prednisolone of 15 mg/day ever since with only a few episodes of arthritis over ankle,
knee, wrist, distal, middle interphalengeal and metacarpophalengeal joints intervening.
No other events of disease flaring-up were noted, prednisone was therefore tapered to
10 mg/day.
She ever experienced an episode of bacterial meningitis in this early January.
The clinical presentation included fever, nausea/vomiting and neck stiffness.
The cerebrospinal fluid (CSF) examination revealed polymorphonuclear cells predominant
pleocytosis, decreased glucose level and elevated protein level. She was put on
ampicillin plus ceftriaxone and then shifted to vancomycin plus ciprofloxacin because
of beta-lactam related leukopenia. She was discharged later without major neurological
sequelae.
One episode of URI-like symptoms (rhinorrhea, mild cough, and sore throat) developed
about one week before this admission. Three days later, her consciousness became
drowsy and decreased physical activities were also noted. One episode of spontaneous
epistaxis took place in the next day. She was brought to a LMD, where local treatment
was applied and epistaxis stopped. However, persistent high fever with more
deteriorated consciousness occurred in the morning of 2/10 (one day before admission).
Several episodes of misnaming and decreased movement of her right limbs were noted
by her mother later. No headache, nausea or vomiting was found. She was sent to our
ER for help.
On physical examination, the temperature was 39℃, the pulse rate was 120/min and the
respiratory rate was 20/min. The blood pressure was 94/60 mmHg. The patient was drowsy
but arousable (E3M5V3) and appeared acutely ill. Plenty of erythematous, discoid
maculopapular lesion was noted over malar area. The pharyngeal wall was injected
with whitish coating over left pillar. The neck was mildly stiff (chin to
chest 3 fingerbreadth) and the carotid pulses were ++, without bruits. Grade I goiter
was palpated. There was no lymphadenopathy, ecchymosis or petechiae. The lungs were
clear and the heart sounds were normal. Abdominal examination was normal; the liver
and spleen were not palpable. There was a few erythematous discoid maculopapular lesion
of size 1×1 cm over medial side of bilateral thighs; no cyanosis, no clubbing or
peripheral edema was present. Rectal examination revealed normal sphincter tone.
On neurological exam, though the patient was arousable and could responded by murmuring,
she could not fully follow our verbal order. Her vision appeared to be preserved, with
full extraocular movements. The pupils were isocoric and reactive; there was a
questionable right ptosis. The nasolabial fold was symmetric and the tongue was not
deviated. The remaining cranial nerves were intact. Her muscle power test was impeded
by her deeply-drowsy consciousness; the deep-tendon-reflex was symmetric, though the
lower extremities showed diminished response (1+/1+), as compared with upper ones
(2+/2+).
Laboratory data at ER showed: hemogram WBC: 3680, Hb: 8.8, PLT: 129k; biochemistry:
BUN/Cre: 15/0.9, Na: 141, K: 6.0 (H2+), Ca:1.9. Brain CT scan was performed before
lumbar puncture and did not show any hemorrhage or any hypodense lesion. CSF study
later at ER disclosed (Cell count 11, L/N: 7/4, RBC (-), Sugar: <30, Pandy’s
test (+), Nonne-Apelt’s test (+)). The high fever persisted and she still
remained drowsy.
<Lab data>
|
WBC |
Hb |
PLT |
Seg |
Band |
Meta |
My |
CRP |
2/10(ER) |
3680 |
8.8 |
129k |
88.4% |
|
|
|
|
2/14 |
3430 |
8.9 |
103k |
85.8% |
|
|
|
|
2/16 |
5310 |
11.0 |
162k |
77% |
|
|
|
5.03 |
2/19 |
7350 |
8.5 |
150k |
64% |
1% |
6% |
4% |
|
2/24 |
9190 |
9.9 |
205k |
67% |
3% |
4% |
3% |
<0.01 |
|
Pressure (mmH2O) |
Character |
Cell (L/N) |
Pandy |
N-Apelt |
Sugar (serum) |
Protein (mg) |
Culture |
2/10 |
150/110 |
Clear, |
11(7/4) |
+ |
++ |
<30( only routine) |
nil |
Nil |
2/14 |
84/80 |
Clear |
0 |
+ |
+ |
66(125) |
144 |
No growth |
2/24 |
54/10 |
Clear, |
2(2/0) |
+ |
+ |
74(130) |
67 |
No growth |
|
AST/ALT |
rGT/ALP |
Bil (T/D) |
BUN |
creatinine |
Na |
K |
Cl |
Ca |
2/10 |
|
|
|
15 |
0.9 |
141 |
6.0 (H2+) |
- |
1.9 |
2/12 |
|
|
|
13 |
1.0 |
141 |
4.1 |
107 |
1.44 |
2/14 |
105/49 |
438/233 |
0.4/0.2 |
9.3 |
0.8 |
141 |
4.0 |
111 |
1.77 |
2/16 |
46/36 |
544/252 |
|
11.4 |
0.8 |
140 |
4.3 |
111 |
1.95 |
2/19 |
45/37 |
536/311 |
0.4/0.2 |
15.5 |
0.6 |
133 |
4.5 |
-- |
1.97 |
2/24 |
72/72 |
1108/688 |
0.5/0.2 |
20.8 |
0.7 |
136 |
4.4 |
-- |
2.19 |
2/29 |
29/46 |
800/482 |
0.5/0.2 |
25.6 |
0.6 |
135 |
4.0 |
-- |
2.29 |
3/4 |
20/37 |
408/284 |
0.4/0.2 |
16.5 |
0.6 |
137 |
3.9 |
-- |
-- |
ECG: normal sinus rhythm. No ST-T changes
Coagulation profile
PT: 13.8/12.3
PTT: 49.6/39.4
FDP: 10-20
D-D dimmer: 2.01
3P: negative
dRVVT: negative,
antiphospholipid antibody: negative
anticardiolipin antibody: negative
|
C3 |
C4 |
Anti-dsDNA |
2/12 |
28.7 |
<6 |
492 |
2/25 |
38 |
<6 |
|
3/4 |
61.4 |
6.8 |
419 |
EEG: Diffuse slow wave.
Thyroid function: free T4: 0.59; TSH: 0.705
Hepatitis profile: Anti-HBs Ab: +;
HBeAg: negative;
Anti-HCV Ab: negative
Viral isolation of Herpes simplex and enterovirus: negative
Enterovirus antibody type 70 (paired serum): negative
HTLV-1 antibody (paired serum): negative
| She was put on intravenous Amoxicillin/clavulanate (Augmentin) and fluconazole
(Diflucan) at first for suspected tosillopharyngitis. But the clinical condition did
not improve. The CSF examination revealed lymphocyte predominant pleocytosis and
decreased glucose level. The antibiotics was therefore shifted to ciprofloxacin
and vancomycin. Solu-medrol, 80 mg/day, was also used at the same time. The clinical
condition did not improved. Repeated lumbar puncture disclosed cell-protein
dissociation. The dosage of solu-medrol was increased to 160 mg/day under the
impression of high activity of SLE. Brain MRI (T2WI) disclosed abnormally high
signal along periventricular system and multiple lesions at brain stem and pons.
But there was no meningeal enhancement. Viral encephalitis was suspected by
neurologist and neuroradiologist. Acyclovir was added therefore. However, her
consciousness had improved 2 days before the use of acyclovir.
As the consciousness became more clear on the 5th day, asymmetric paraparesis
(right lower extremity: 2/5, left lower extremity: 3/5) and bilateral positive
Babinski’s sign developed. Areflexia over bilateral lower limbs was also noted.
Severe parasthesia (numbness and severe pain with light touch) over right leg and
foot was complained. Severe wasting of bilateral calf muscle (within 10 days) was
also noted. No sphincter dysfunction developed. Neurophysiologic studies including
nerve conduction velocity (NCV), F-wave and H-reflex documented polyradiculoneuropathy
with severe axonal degeneration. The patient became quite clear in about 7 days.
All antimicrobial agents were discontinued, as the CSF showed no evidence of infection,
except high protein content. MRI follow-up on the 14th day disclosed complete
resolution of previously high-signal area, but the patient’s paraparesis improved
slowly. She was finally diagnosed to have neuropsychiatric lupus with the involvement
of peripheral nervous system (axonal, motor type). But the contribution of other
unknown viral infection could not be totally excluded.
請參考腦部MRI圖
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