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1093/brain/awg233
Summary
Keywords: Bickerstaff's brainstem encephalitis; GuillainBarre syndrome; Fisher syndrome; anti-GQ1b antibody
Abbreviations: AMAN = acute motor axonal neuropathy; BBE = Bickerstaff's brainstem encephalitis;
CMAP = compound muscle action potential; ELISA = enzyme-linked immunosorbent assay; FS = Fisher syndrome;
GBS = GuillainBarre syndrome; IVIg = intravenous immunoglobulin; MRC = Medical Research Council
Introduction
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Table 1 Clinical features of cases reported by Fisher, Bickerstaff and Al-Din et al.
Authors and Antecedent illness
Case Nos
Fisher (1956)
1
Sore throat, malaise,
headache
2
3
Bickerstaff (1957)
1*
Headache, muscular
pains
2*
Malaise, headache
3*
Headache
4
Vertigo, vomiting
5
6
7
Tendon
reex
CSF
Our
albumino- diagnosis
Babinski's Ataxia cytological
sign
dissociation
Mild
Absent
Drowsiness
+
+
Absent
Absent
+
+
FS with
overlapping
GBS
BBE
FS
Drowsiness
BBE
Drowsiness
Drowsiness
Drowsiness
+
+
+
+
+
+
Drowsiness
Drowsiness
Absent
Equivocal +
+
Drowsiness
Absent
BBE
BBE
BBE with
overlapping
GBS
BBE
Brainstem
encephalitis
BBE
Drowsiness
Decreased
BBE
Drowsiness
Drowsiness
Slight
Brisk
Brisk
Absent
Normal
Absent
Absent
Drowsiness
+
+
+
+
+
+
+
+
+
+
+
+
Drowsiness
Flaccid
Brisk
Brisk
BBE
BBE
FS
BBE
FS
BBE with
overlapping
GBS
BBE with
overlapping
GBS
BBE with
overlapping
GBS
BBE
BBE
Drowsiness
8
9
10
11
12
13
14
15
16
17
18
Flu-like illness
Headache, giddiness
with vomiting
Cough, headache
Malaise
Pharyngitis, diarrhoea,
headache
Decreased
Gross accid
Drowsiness
Drowsiness
+
+
Absent
Normal
+
+
Drowsiness
Comatose
Drowsiness
+
+
+
Absent
Normal
Absent
+
+
+
Headache
Drowsiness
Malaise
Sore throat
Upper respiratory tract Drowsiness
infection
Sore throat
+
+
+
+
Absent
Absent
Absent
Absent
+
+
+
+
Mild
Mild
Absent
BBE
BBE
BBE with
overlapping
GBS
BBE
FS
FS
BBE
FS with
overlapping
GBS
BBE = Bickerstaff's brainstem encephalitis; FS = Fisher syndrome; GBS = GuillainBarre syndrome. *Three cases described by
Bickerstaff and Cloake in 1951. Blank: information not available.
Headache, malaise
Malaise, fever,
vomiting
Headache, malaise,
vomiting
Muscular pain,
malaise
Neurological signs
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Statistical analysis
Results
Case reports
Illustrative case of typical BBE
Patient 1
A 29-year-old man experienced coughing and nasal discharge, which resolved in 7 days. Ten days after resolution,
he suffered numbness of the arms and legs, diplopia,
dysarthria, drowsiness and unsteady gait (day 1). On day 7,
he was drowsy but easily aroused by stimulation.
Neurological examination revealed mild bilateral blepharoptosis, limitation of horizontal gaze and incomplete convergence. There was upbeating nystagmus on upward gaze, and
bilateral horizontal gaze-evoked nystagmus. His pupils were
dilated and light reexes were absent. Bell's phenomenon and
oculocephalic reexes were preserved. Slurred speech was
present, but there was no facial or oropharyngeal palsy. There
was no weakness. Finger-to-nose and heel-to-knee tests were
ataxic. Deep tendon reexes were diminished in the upper
limbs and absent in the lower ones. Pathological reexes were
lacking. He could hold himself erect with a wide-based
stance, but could not walk without support. Hypoaesthesiae of
the glove-and-stocking type were present, but vibration sense
was intact.
High-resolution MRI with and without gadolinium
enhancement detected no plaques in the brainstem and
brain. The serum anti-GQ1b IgG antibody titre was high
(51 200), and between days 13 and 22 the patient underwent
seven sessions of immunoadsorption with a tryptophanconjugated column (TR-350; Asahi Medical, Tokyo, Japan),
which highly adsorbs this autoantibody (Yuki, 1996). He
became fully conscious on day 14, and the next day could
walk around the room with a wide-based gait, holding onto
the furniture. There was slight movement of the eyeballs in
downward gaze. CSF protein was 149 mg/dl, with normal
cellularity. On day 20 he could walk >5 m without support,
although unsteadily. Motor and sensory nerve conduction
velocities were normal. On day 29 there was no extraocular
muscles paresis, except for limitation of abduction. Ataxic
gait disappeared, and on day 35 he could stand on one foot.
On day 60 his range of eye movements was complete, and he
was discharged. By day 73, nystagmus had disappeared.
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Patient 3
Normal limit
Median
nerve
Ulnar
nerve
Tibial
nerve
Median
nerve
Ulnar
nerve
Tibial
nerve
Median
nerve
Ulnar
nerve
Tibial
nerve
4.1
60.2
4.3
4.2
2.3
60.8
5.3
5.1
4.6
44.1
4.0
3.9
4.2
52.2
2.1
2.4
2.5
50.7
1.6
1.2
6.0
37.9
1.9
0.9
<4.5
>48
>4.4
>4.2
<3.5
>48
>4.6
>4.2
<5.6
>38
>5.8
>5.8
Patient 3
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Table 3 Clinical proles of patients with BBE and BBE with limb weakness
Number of patients
Sex (male/female)
Age (years): median (range)
62
37/25
39 (391)
25
17/8
48 (391)
37
20/17
37 (1380)
45 (73)
7 (11)
9 (15)
16 (64)
3 (12)
5 (20)
29 (78)
4 (11)
4 (11)
32
22
12
8
7
7
3
2
(52)
(35)
(19)
(13)
(11)
(11)
(5)
(3)
13
7
2
6
1
0
2
1
(52)
(28)
(8)
(24)
(4)
19
15
10
2
6
7
1
1
(51)
(41)
(27)
(5)
(16)
(19)
(3)
(3)
28
18
18
62
21
17
28
21
37
(45)
(29)
(29)
(100)
(34)
(27)
(45)
(34)
(60)
12
4
8
25
7
11
7
5
0
(48)
(16)
(32)
(100)
(28)
(44)
(28)
(20)
16
14
10
37
14
6
21
16
25
(43)
(38)
(27)
(100)
(38)
(16)
(57)
(43)
(68)*, 12 (32)**
21
5
36
25
62
10
19
(34)
(8)
(58)
(40)
(100)
(16)
(31)
10
4
11
7
25
2
3
(40)
(16)
(44)
(28)
(100)
(8)
(12)
11
1
25
18
37
8
16
(30)
(3)
(67)
(49)
(100)
(22)
(43)
(8)
(4)
Total
Table 4 Abnormal MRI and neurophysiological study ndings for patients with BBE and BBE with limb weakness
Abnormal ndings of
MRI
EEG
Motor nerve conduction study
Axonal degeneration
Demyelination
Needle electromyography
Available
data
n (%)
Available
data
n (%)
Available
data
n (%)
54
33
34
16 (30)
24 (73)
15 (44)
13 (38)
2 (6)
3 (50)
23
13
10
9 (39)
10 (77)
1 (10)
1 (10)
0
0
31
20
24
7 (23)
14 (70)
14 (58)
12 (50)
2 (8)
3 (75)
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Table 5 CSF ndings during the rst, second, and third and fourth weeks for patients with BBE and BBE with limb
weakness
54 (87)
6 (126)
20 (80)
6 (119)
34 (92)
5 (126)
1st
week
2nd
week
3rd and
1st
4th weeks week
2nd
week
3rd and
1st
4th weeks week
2nd
week
3rd and
4th weeks
14 (23)
14 (23)
2 (0142) 1 (021)
15 (60) 7 (28)
4 (060) 1 (063)
3 (12)
1 (05)
27 (73) 7 (19)
11 (30)
3 (071) 2 (1142) 1 (021)
6 (43)
89
(24295)
11 (79)
6 (43)
6 (40)
42
(1081)
7 (47)
4 (27)
1 (33)
46
(24149)
2 (67)
2 (67)
9 (33)
34
(16292)
9 (33)
4 (15)
3 (21)
89
(24678)
9 (64)
8 (57)
3 (43)
81
(24121)
6 (86)
4 (57)
3 (43)
106
(25295)
5 (71)
2 (29)
2 (18)
89
(25678)
8 (73)
6 (55)
IgG antibody to
GM2
GM1
GD1a
GalNAc-GD1a
GD1b
GT1b
GQ1b
GM1, GD1a or GalNAc-GD1a
BBE, total
(n = 62)
BBE without
limb weakness
(n = 25)
2
6
8
1
11
3
41
11
0
2
1
0
2
0
15
2
2
4
7
1
9
3
26
9
(3)
(10)
(13)
(2)
(18)
(5)
(66)
(18)
(8)
(4)
(8)
(60)
(8)
(5)
(11)
(19)
(3)
(24)
(8)
(70)
(24)
Table 6 Anti-ganglioside IgG antibodies for patients with BBE and BBE with limb weakness
Discussion
Nosological entity of BBE
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Acknowledgements
and
Bickerstaff
ER,
Cloake
PCP.
Mesencephalitis
rhombencephalitis. Br Med J 1951; 2: 7781.
Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Grifn JW, et al.
GuillainBarre syndrome in northern China: relationship to
Campylobacter jejuni infection and anti-glycolipid antibodies.
Brain 1995; 118: 597605.
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Yaqub BA, al-Deeb SM, Daif AK, Sharif HS, Shamena AR,
al-Jaberi M, et al. Bickerstaff brainstem encephalitis: a grave