Yonago Acta medica 2008;51:83–88

Urinary Retention as an Initial Symptom of Acute MeningoEncephalo-Radiculitis in Epstein-Barr Virus Infection
Yasumasa Asai, Ryosuke Fujita, Hiroyuki Nakayasu, Kaori Suzuki, Taizo Kaneto*
and Kenji Nakashima†
Clinic of Neurology, Tottori Prefectural Central Hospital, Tottori 680-0901, *Clinic of Neurology, Tottori Medical Center, Tottori 689-0203 and †Department of Neurology, Institute of Neurological Sciences, Tottori University Faculty of Medicine, Yonago 683-8503 Japan

A 48-year-old man presented with urinary retention followed by disturbance of consciousness, areflexia, ophthalmoplegia, muscle weakness, and atrophy. Epstein-Barrvirus DNA by PCR was positive in his cerebrospinal fluid. The cerebrospinal fluid revealed elevated myelin basic protein and an oligoclonal band. Magnetic resonance imaging showed high signal intensity in the pons, basal ganglia, and cerebral white matter on
T2-weighted imaging and fluid-attenuated inversion recovery imaging. His consciousness, ophthalmoplegia, and muscle weakness almost full recovered. In this patient, theinflammation is thought to have begun as sacral radiculitis, and then extended to encephalitis, and brachial and lumbar radiculoneuritis.
Key words: acute disseminated encephalitis; Guillain-Barré syndrome; meningo-encephaloradiculitis; urinary retention

Acute disseminated encephalomyelitis (ADEM) is
a monophasic inflammatory demyelinating disorder of the central nervous system, typically occurring after infection or vaccination. The disease ischaracterized by multifocal white matter lesions
on neuroimaging. The polysymptomatic presentation reflects the demyelinating lesions and consistsof various combinations of motor, sensory, visual,and cognitive symptoms.
Guillain-Barré syndrome (GBS), which is anacute immune-mediated polyneuropathy with amonophasic course, is classified as acute inflammatory demyelinating polyneuropathy and acutemotor axonal neuropathy. The cardinal clinicalfeatures of GBS are weakness, paresthesia, andhyporeflexia. Albuminocytological dissociationis one of the diagnostic findings in GBS. Central
nervous system involvement in GBS has beenreported (Baker, 1943; Morley and Reynolds,
1966; Blennow et al., 1968; Gamstorp, 1974; Amit
et al., 1986; Laarman et al., 1987; Amit et al.,
1992; Okumura et al., 2002; Pfausler et al., 2002;
Tsugawa et al., 2004; Chikakiyo et al., 2005).
Blennow et al. introduced the term “encephalomyelo-radiculo-neuropathy” to describe thiscombined involvement. However, there are a few
reports of both GBS and ADEM occurring simultaneously (Amit et al., 1986, 1992). In thesereports, the term “acute severe central and peripheral nervous system combined demyelination”
was used to replace the combination of GBS and
ADEM. Guillain (1936) has refused to recognize
this syndrome as belonging to any condition witha spinal fluid pleocytosis (Pfausler et al., 2002).
However, Baker (1943) proposed that GBS might
involve any part of the peripheral or the central

Abbreviations: ADEM, acute disseminated encephalomyelitis; CSF, cerebrospinal fluid; GBS, Guillain-Barré syndrome; Ig, immunoglobulin; MER, meningo-encephalo-radiculitis; MRI, magnetic resonance imaging

83

Y. Asai et al.

Fig. 1. Brain MRI
showed high signal
intensity in thepons, basal ganglia,
cerebral white matter on T2 weighted
imaging (a) andfluid attenuatedimaging (b).

abOn physical examination, his body temperature was 38.5°C, blood pressure was 155/80
mmHg, and pulse was regular (100/min). There
were no cardiac murmurs, rhonchus or crackles.
Neurological examination revealed disorientation
and a stiff neck. He also could not walk becauseof muscle weakness in his lower limbs. Deep
tendon reflexes were absent in his lower limbs.
Neither sensory loss nor paresthesia was observed. A urinary catheter was inserted because of difficult micturition.
A hematological investigation showed anelevated white blood cell count of 15,440/μL.
The blood urea nitrogen level was 65.6 mg/dL,
creatinine level was 1.71 mg/dL, natrium levelwas 131 mEq/L, and C-reactive protein level was
2.78 mg/dL. Titers of antibody herpes simplex
virus (HSV), varicella-zoster virus (VZV) andcytomegalovirus (CMV) were not elevated in
his serum. Titers of Epstein-Barr virus (EBV)virus capsid antigen immunoglobulin M (IgM)
were < 10 (normal range < 10), virus capsid
antigen IgG 80 (normal range < 10), early antigen IgG < 10 (normal range < 10), Epstein-Barr
nuclear antigen 20 (normal range < 10) in his
serum. Results of cerebrospinal fluid (CSF) examination revealed the following: 492 cells/mm3(polymorphonuclear : mononuclear = 1:122), protein level of 299 mg/dL, IgG index 0.79, myelin
basic protein (343 pg/mL, control < 102). Oligoclonal band was positive. Viorogical analysis of

nervous system, which would include “encephalomyelo-radiculitis”. We use the term meningoencephalo-radiculitis (MER), because meningoencephalitis and radiculitis was obvious and myelitis was uncertain in our patient.
During the course of ADEM and GBS, micturitional disturbance is commonly seen. Lumbosacral myelitis in ADEM and lumbosacral radiculoneuritis in GBS appear to be the main causesof micturitional disturbance. Local lumbosacral
radiculitis can cause micturitional disturbance, acondition known as Elsberg syndrome (Hemrika
et al., 1985). Even in the presence of Elsberg syndrome, micturitional disturbance as a first symptom of MER is rare (Laarman et al., 1987; Tsugawa
et al., 2004; Sasaki et al., 2006). We report a caseof MER with Epstein-Barr virus (EBV) infection
presenting urinary retention as an initial symptom.

Patient Report
A 48-year-old Japanese man had joint pain in his
knee and elbow, lumbago and headache in midMarch, 2006. Five days after the onset, he had
urinary retention, and a urinary catheter wasinserted intermittently. He was taken to hospital
because of the presenting urinary retention andhigh fever nine days after onset. He developed
consciousness disturbance on Day 2 after hospitalization (10 days after onset).
84

Urinary retention of acute MER

Aministration
IVIg
MP
Ara-A

IVIg
30

60

IVIg

IVIg

90

120 150 (day)Urinary retention
Ventilator

Conscious disturbanceFig. 2. The clinical course of
the present patient. Ara-A,
vidarabine; IVIg, intravenous
immunoglobulin; MP, methylprednisolone.

Motor weakness (lower limbs)
Eye movement disoder
Motor weakness (upper limbs)

CSF revealed the presence of EBV DNA by PCR,but no HSV, VZV and CMV DNA in the CSF.
Magnetic resonance imaging (MRI) showed anormal brain on Day 9 after hospitalization (17days after onset). Gadolinium enhancement was
not done. Single photon emission tomography
did not show hypoperfusion or hyperperfusion.
Electroencephalography showed a 6- to 7-Hz slow
wave. On Day 2 (10 days after onset), viral encephalitis was the tentative diagnosis, and vidarabine was administered intravenously. However, hedeveloped consciousness disturbance and presented tonic seizure and tetraplegia, so steroid pulse
therapy (methylprednisolone 1000 mg × 3 days)
and immunoglobulin (2.5 g × 5 days) were added.
He was incubated and required mechanical ventilation. On Day 5 (13 days after onset), total ophthalmoplegia presented, and deep tendon reflexes
in all limbs and bilateral oculocephalic reflexes
were absent. Immunoglobulin (27.5 g × 5 days)
was administered because of the suspected Fisher
syndrome on Day 12 (20 days after onset). In theclinical course, diffuse muscle atrophy in the upper and lower limbs was present. On Day 34 (42days after onset), his consciousness gradually recovered. Ophthalmoplegia and muscle weakness
were almost full recovered and he could walk a
few steps. However, he performed intermittent
self-catheterization because of voiding difficulties.
Orthostatic hypotension was not shown in a headup tilt test. Coefficient of variation of RR inter-vals was 1.22%. A sweat test indicated no sweat
on his trunk and lower limbs. Phenylephrine
eye drops test showed mild mydriasis. Antibody
activities against ganglioside GM1, GM2, GM3,
GD1a, GD1b, GD3, GT1b, GQ1b, GA1, Gal-C and
GalNAc-GD1a were all negative. Fifty days after
onset, an electrophysiological examination was
performed. Distal complex motor action potential
amplitudes were 11.7/8.3 (right/left) mV in his median nerve and 0.8/0.7 mV in his tibial nerve. Motor nerve conduction velocities were 56.5/57.5 mV
in his median and 48.6/45.0 mV in his tibial nerve.
Terminal latencies were 3.9/4.2 m/s in his medianand 6.6/6.8 m/s in his tibial nerve. Sensory nerve
action potential velocities were 69.9/64.2 m/s in
his median and 38.0/44.0 m/s in his sural nerve.
F-wave latencies were 28.7/27.9 ms in his median
nerve and 53.9/42.6 ms in his right tibial nerve.
Urodynamic study was performed for 82 days
after onset. When 210 mL of water was infusedhe complained first of a desire to void. Bladdervolume at maximum desire to void was 430 mL.
Atonic cystometrogram was presented.
He was transferred to another hospital forfurther rehabilitation for 168 days after onset.
Brain MRI showed high signal intensity in thepons, basal ganglia, cerebral white matter on T2
weighted imaging and fluid attenuated imaging 6
months after onset (Fig. 1). Cervical, thoracic andlumbar MRI were normal. Clinical course wasshown in Fig. 2.
85

Y. Asai et al.

Table 1. Clinical comparison among our patient and those reported in literature on urinary retention
preceding meningoencephalitis with GBS or meningo-encephalo-radiculitis as the first symptom

Age (year)
Sex
Cerebrospinal fluid
Precedent infection
Cell (/mm3)/protein (mg/dL)
Ganglioside antibody
Oligoclonal band
Myelin basic protein
NCV and/or EMG
EMG
Motor conduction velocity
Sensory conduction velocity
F waveBrain MRIHigh intensity area
Spinal MRI
Enhanced

Laarmanet al.
(1987)

Tsugawa
et al.
(2004)

Patient
1

Sasaki et al. (2006)		
Patient
Patient
2
3

30
Male

14
Male

34
Male

51
Male

46
Male

48
Male

MC
145/100
ND
ND
+

CPB
53/51
+
–
+

ND
704/274––+ND
387/805––+

ND
227/173–
ND+

EBV
492/299–++

Denervation
		
ND
NP
			
NP
			
ND
ND
NP
NP
				
				
ND
NP
		
Conus, equina

NP		
NP
NP		
BG,
brain stemNP

BG,
thalamus
ND

Present
patient

Low amplitude
Delay of latency
BG,
DWM
NP

BG, basal ganglia; CPB, campylobactor; DWM, deep white matter; EBV, Epstein-Barr virus; EMG, electromyography; GBS, GuillainBarré syndrome; MC, mycoplasma; MRI, magnetic resonance imaging; NCV, nerve conduction velocity; ND, not detected; NP, not performed.

not emphasized in these previous reports. Sasaki et al. (2006) reported 3 male patients withmeningo-encephalo-radiculitis presenting withacute urinary retention. It was inferred that they
may represent a variant of ADEM. The reports of
Laarman et al. (1987) and Tsugawa et al. (2004)
also showed peripheral disorder, suggesting theco-existence of GBS. However, demyelination ofcentral nervous system by brain and spinal MRI
was uncertain. On the other hand, in the reportby Sasaki et al. (2006), central nervous systemdisorder was presented by MRI, but disorder of
peripheral nervous system was uncertain. Our patient presented muscle atrophy during the courseof his condition, low amplitude of tibial, delayof right sural sensory nerve and tibial F-wave latency. Brain MRI showed high signal intensity inthe pons, basal ganglia and cerebral white matteron T2 weighted imaging and fluid attenuated imaging. Thus disorder of both the central nervous
system and peripherall nervous system was cer-Discussion
The patient presented urinary retention as the firstsymptom, followed by MER. There are some
reports that urinary retention may precede meningoencephalitis in GBS or meningo-encephaloradiculitis as an initial symptom (Laarman et al.,
1987; Tsugawa et al., 2004; Sasaki et al., 2006)(Table 1). Tsugawa et al. (2004) reported GBS
with meningo-encephalitis in a patient presenting urinary retention and muscle weakness as ininitial symptoms. Laarman et al. (1987) reported
a case of meningo-encephalitis and GuillainBarré type ascending sensorimotor paralysis. In
their case, headache and fever were shown as firstsymptoms, followed by difficult micturition 10
days after onset and consciousness disturbance
12 days after onset. The condition of our patient
was similar to that described in these 2 reports;
however, urinary retention as a first symptom was86

Urinary retention of acute MER

tainly shown in the present patient.
The etiology of urinary retention in ourpatient is thought to be a variant of the Elsberg
syndrome that is generally regarded as a form oflumbosacral radiculopathy (Hemrika et al., 1985).
The most common etiologies are viral infections,particularly genital herpes (HSV type 2) (Black etal., 1983; White et al., 1984; Hemrika et al., 1996;
Lepori et al., 1992; Herbaut et al., 1990; Eberhardt et al., 2004). EBV has also been reported asa cause of sacral myeloradiculitis (Sperber et al.,1973). Additionally, EBV has also been reported
as a cause of myeloradiculitis and encephalomyeloradiculitis (Merelli et al., 1997; Majid et al.,
2002; Phowthongkum et al., 2007). Elevated
CSF cell count is often observed in these conditions as in our case. To the best of our knowledge
this is the first reported case of acute meningoencephalo-radiculitis in EBV, in which urinary
retention was an initial symptom. Although it
remains to be determinated the pathomechanismof radiculitis and/or encephalitis in Epstein-Barrvirus infection, our case report would serve as an
aid to clarify this pathomechanism.
In the autonomic system examination, thesympathetic and parasympathetic nervous systems
were both impaired. Postsynaptic impairment
was suggested from the eye drops test. However,brain stem disorder also might affect urinary
retention. It is important to keep in mind thaturinary retention can be the initial sign of acutecentral or peripheral demyelination.

simplex genitalis. Ann Neurol 1983;14:692.
Blennow G, Gamstorp I, Rosenberg R. Encephaloradiculo-neuropathy. Dev Med Child Neurol
1968;10:485–490.
6 Chikakiyo H, Kunishige M, Yoshino H, Asano A,
Sumitomo Y, Endo I, et al. Delayed motor and sensory neuropathy in a patient with brainstem encephalitis. J Neurol Sci 2005;234:105–108.
7 Eberhardt O, Kuler W, Dichgans J, Weller M. HSV2 sacral radiculitis (Elsberg syndrome). Neurology
2004;63:758–759.
8 Gamstorp I. Encephalo-myelo-radiculo-neuropathy:
involvement of the CNS in children with GuillainBarré-Strohl syndrome. Dev Med Child Neurol
1974;16:654–658.
9 Guillain G. Radiculoneuritis with acellular hyperalbuminosis of the cerebrospinal fluid. Arch Neurol
Psychiat 1936;36:975.
10 Hemrika DJ, Schutte MF, Bleker OP. Elsberg syndrome: a neurologic basis for acute urinary retention in patients with genital herpes. Obstet Gynecol
1986;68:37S–39S.
11 Herbaut AG, Nogueira MC, Wespes E. Urinary retention due to sacral myeloradiculitis: a clinical andneurophysiological study. J Urol 1990;144:1206–
1208.
12 Laarman GJ, Hoekstra JBL, Donker DNJ, Verbrugh
HA, Hekker AC. Meningo-encephalitis and Guillain-Barré type ascending sensorimotor paralysis
associated with mycoplasma pneumoniae infection.
Neth J Med 1987;31:66–71.
13 Lepori P, Marcacci G, Gaglianone S. Elsberg syndrome: radiculomyelopathy and acute urinary retention in patient with genital herpes. Ital J Neurol Sci
1992;13:373–375.
14 Majid A, Galetta S, Sweeney C, Robinson C,
Mahalingam R, Smith J, et al. Epstein-Barr virus
myeloradiculitis and encephalomyeloradiculitis.
Brain 2002;125:159–165.
15 Merelli E, Bedin R, Sola P, Gentilini M, Pietrosemoli P,
Meacci M, et al. Encephalomyeloradiculopathy associated with Epstein-Barr virus: primary infection orreactivation? Acta Neurol Scand 1997;96:416–420.
16 Morley JB, Reynolds EH. Papilledema and LandryGuillain-Barré syndrome. Case report and review.
Brain 1966; 89:205–222.
17 Okumura A, Ushida H, Maruyama K, Itomi K,
Ishiguro Y, Takahashi M, et al. Guillain-Barré
syndrome associated with central nervous system lesions. Arch Dis Child 2002;86:304–306.
18 Pfausler B, Engelhardt K, Kampf l A, Spiss H,
Taferner E, Schmutzhard E. Post-infectious central
and peripheral nervous system diseases complicating
Mycoplasma pneumoniae infection. Eur J Neurol
2002;9:93–96.
19 Phowthongkum P, Phantumchinda K, Jutivorakool K,
5References
1
2
3
4

Amit R, Shapira Y, Blank A, Aker M. Acute, severe,
central and peripheral nervous system combined demyelination. Pediatr Neurol 1986;2:47–50.
Amit R, Glick B, Itzchak Y, Dgani Y, Meyeir S.
Acute severe combined demyelination. Childs Nerv
Syst 1992;8:354–356.
Baker AB. Guillain-Barre’s disease (encephalomyelo-radiculitis): a review of 33 cases. Lancet
1943;63:384–398.
Black D, Stewart J, Melmed C. Sacral nerve dysfunction plus generalized polyneuropathy in herpes

87

Y. Asai et al.

Suankratay C. Basal ganglia and brainstem encephalitis, optic neuritis, and radiculomyelitis in Epstein–Barr virus infection. J Infect 2007;54:141–144.
20 Sa sa k i M, Oha ra S, Haya sh i R , Iwa ha sh i T,
Tsuyuzaki J. Aseptic meningo-encephalitis presenting initially with urinary retention. J Neurol
2006;253:908–913.
21 Sperber A, Tessler AN, Berezeller P. Infectious
mononucleosis with acute urinary retention. Urology 1973;2:456–457.
22 Tsugawa T, Nikaido K, Doi T, Koga M, Susuki K,
Kubota T, et al. Guillain-Barré syndrome withmeningoencephalitis after Campylobacter jejuni infection. Ped Infect Dis J 2004;23:966–968.
23 White WB, Hanna M, Stewart JA. Systemic herpes
simplex virus type 2 infection: proctitis, urinary retention, arthralgia, and meningitis in the absence ofprimary mucocutaneous lesions. Arch Intern Med
1984;144:826–827.
Received June 24, 2008; accepted August 22, 2008.
Corresponding author: Yasumasa Asai, MD

88