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Bilateral Neuroretinitis Associated With Mumps
Raju Khubchandani, MD;
Tej Rane, DNB;
Premlata Agarwal, MBBS;
Fazal Nabi, DCH;
Phiroze Patel, MS, FRCS;
Avinash K. Shetty, MD
Arch Neurol. 2002;59:1633-1636.
ABSTRACT
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Background Involvement of the optic nerve is a rare complication of mumps infection.
Objectives To report a case of bilateral neuroretinitis complicating a mumps infection
and to review 5 previously reported cases.
Design Case report and literature review.
Setting Tertiary hospital.
Patient A 7-year-old girl had sudden-onset blindness due to bilateral neuroretinitis.
Approximately 3 weeks prior to the initial examination, she developed a self-limited
febrile illness with parotid swelling and subsequent meningoencephalitis.
Results Mumps was determined to be the underlying cause of the meningoencephalitis
and bilateral optic neuritis because of the exposure history in this nonvaccinated
child, the typical clinical signs and symptoms, and the positive serologic
test results. Recovery of visual function was gradual but nearly complete.
Conclusions Physicians should be aware that optic nerve involvement may be a manifestation
of mumps infection. The delayed onset of optic neuritis, the bilateral involvement,
and the near complete recovery suggest an immune-mediated pathogenesis.
INTRODUCTION
MUMPS (epidemic parotitis) is an acute communicable disease caused by
a paramyxovirus, characterized by painful enlargement of the salivary glands,
particularly the parotid glands.1 Neurologic
and ocular manifestations of mumps are well documented2-13
but seldom appear concomitantly.6, 8-9,11-12
Meningitis and mild meningoencephalitis are the most frequent complications
of mumps in children.1 Optic neuritis is a
rare neurologic complication following mumps infection and is manifested clinically
by decreased visual acuity, decreased color perception, and visual field defects.5, 7 Funduscopic examination may show marked
edema of the head of the optic nerve (papillitis) and retina (neuroretinitis)
or an entirely normal appearance (retrobulbar neuritis).5-6,8-9,11-12
We describe a 7-year-old girl in whom meningoencephalitis was followed
by bilateral neuroretinitis secondary to a mumps infection. We also review
the clinical spectrum, response to therapy, and visual sequelae of previously
reported pediatric cases of optic neuritis complicating mumps.
REPORT OF A CASE
A 7-year-old girl from Mumbai, India, was admitted to our hospital with
a 1-day history of sudden-onset blindness. Three days prior to admission,
she was discharged from a local hospital after a 2-week stay for management
of acute bacterial meningitis. Her clinical signs and symptoms included severe
headache, vomiting, and altered mental status. There was no history of trauma,
seizure, or skin rash. Therapy with intravenous ampicillin (200 mg/kg per
day) and cefotaxime (300 mg/kg per day) for 2 weeks and mannitol for the initial
3 days of hospitalization resulted in complete recovery. The cerebrospinal
fluid (CSF) profile was consistent with viral meningitis rather than bacterial
meningitis. The CSF analysis revealed a white blood cell count of 15 x
103/µL (20% neutrophils and 80% lymphocytes), glucose level
of 71 mg/dL (3.94 mmol/L), and protein level of 46 mg/dL. The gram stain was
negative for bacteria and the CSF culture was sterile. A detailed history
from her parents revealed that the patient had developed a febrile illness
with bilateral submandibular swelling 1 week prior to the earlier hospitalization.
On evaluation by a family physician, a diagnosis of submandibular adenopathy
secondary to pharyngitis was made, and the patient had received an oral antibiotic
(drug unknown). On further probing it emerged that while our patient was being
treated for meningitis in the hospital, her older sibling had developed mumps
characterized by fever and bilateral parotid enlargement. Both children were
unimmunized against mumps.
Physical examination findings revealed a conscious but irritable child
with a temperature of 36.7°C, heart rate of 110 beats/min, respiratory
rate of 22/min, and a blood pressure of 110/74 mm Hg. Examination of visual
acuity revealed no perception of light in both eyes. Both pupils were fixed,
dilated, and not reactive to light. The anterior chambers appeared normal.
Funduscopic examination showed bilateral optic disc edema with obliteration
of the disc margins. There were scattered hemorrhages against a hyperemic
background. The ocular findings were confirmed by an ophthalmologist. There
were no signs of meningeal irritation and no focal neurologic deficits.
In view of the exposure history in a nonvaccinated child with subsequent
development of self-limited febrile illness with parotid swelling, mumps was
suspected as the underlying cause of meningoencephalitis and bilateral optic
neuritis. Pertinent diagnostic evaluation included a repeated CSF examination,
which was unremarkable and showed complete resolution of the pleocytosis.
The CSF gram stain was negative for organisms and the culture was sterile.
A polymerase chain reaction test for Mycobacterium tuberculosis on the CSF specimen was negative. Results of a chest radiograph were
unremarkable. Magnetic resonance imaging (MRI) of the brain showed a focal
linear hyperintense lesion in the posterior part of the left optic radiation
(Figure 1). The rest of the study
results were reported as normal. A mumps-specific IgM antibody titer measured
by enzyme-linked immunosorbent assay was positive at .125 mg/dL (1.25 gL)
(normal reference range, 54-222 mg/dL [<0.9 g/L]), confirming recent mumps
infection. No specific therapy was offered and the patient was discharged
to home after the prognosis was explained to the parents along with a plan
for close follow-up. A follow-up specimen for mumps serologic analysis was
not obtained. A repeated ophthalmologic evaluation performed 2 weeks later
showed improvement of visual acuity, with presence of perception and projection
of light in both eyes. Both pupils were normal in size but reacted slowly
to light. Follow-up at the end of 6 weeks revealed a visual acuity of 6/60
OU. Direct and consensual reactions to light were normal, with no evidence
of rapid afferent pupillary defect in either eye. Funduscopic examination
showed residual pallor of optic discs, with resolution of bilateral retinal
hemorrhages. Gradually, her vision improved, and after 3 months the visual
acuity was 20/60 OD and 20/40 OS. There was, however, residual pallor of both
optic discs. Findings from a visual evoked potential examination were normal
and a repeated MRI study showed resolution of the hyperintense lesion along
the left optic radiation.
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Magnetic resonance image of the brain (T2-weighted image) showing
signal hyperintensity in the left optic radiation (arrow).
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COMMENT
The incidence of neurologic involvement in mumps is difficult to estimate,
but CSF pleocytosis has been reported in 56% of patients with parotitis, while
10% have symptoms of meningitis.2 In typical
cases, mumps meningitis follows parotitis by 3 to 10 days,14
but neurologic symptoms may precede or even occur in the absence of salivary
gland involvement. The illness is characterized by fever, headache, nausea,
vomiting, signs of meningeal irritation, and only rarely, seizures. The outcome
is generally benign, and the CSF usually shows pleocytosis, with a predominance
of lymphocytes, and an elevated protein level, which in about 10% of cases
is accompanied by hypoglycorrhachia.15 True
encephalitis is rare, developing in about 0.2% of cases, although it is responsible
for most of the central nervous sysytem sequelae, including behavioral disturbances,
persistent headache, seizure disorder, deafness, and optic atrophy.16
Ocular manifestations in mumps are rare but well recognized.7 Various parts of the ocular apparatus may be involved,
such as dacroadenitis, optic neuritis, keratitis, iritis, conjunctivitis,
and episcleritis.5-13
Optic nerve involvement ranks next to that of the lacrimal gland as the most
common ocular manifestation of mumps7, 14
and is usually a complication of a central nervous system infection.11 The optic neuritis is usually bilateral, and may
occur in 3 forms: papillitis, neuroretinitis, and the retrobulbar type.7 Papillitis is characterized by hyperemia of the optic
disc, with edema. The nerve fiber layer of the retina is involved in neuroretinitis.
Visual loss is the predominant clinical manifestation and may be accompanied
by visual field defects, decreased color perception, and painful globe motion.
In retrobulbar neuritis, the optic disc is spared and only the axial portion
of the optic nerve is involved. The clinical signs and symptoms include central
scotoma, leading to visual loss, and normal ophthalmoscopic examination results.
The retrobulbar type of neuritis is less common than the neuroretinitic type.5, 7
A review of the English-language literature revealed 5 well-described
pediatric case reports of optic neuritis complicating mumps5-6,9, 11-12
(Table 1). Ocular
manifestations were limited to papillitis in 1 case, neuroretinitis in 3 cases,
and retrobulbar neuritis in 2 cases. There were 3 boys and 3 girls, with ages
ranging from 7 to 15 years. Five cases had severe visual loss, including sudden
onset blindness in 2 cases. One patient was asymptomatic. The onset of ocular
symptoms began between 2 and 5 weeks after parotitis. Funduscopic examination
results were unremarkable for 2 cases, and bilateral involvement occurred
in 3 patients. Accompanying neurologic manifestation included meningitis in
3 cases and encephalitis in 2 cases. One patient had no visual symptoms, and
an MRI study with short inversion time recovery sequences in conjunction with
visual evoked potentials proved useful in the detection of asymptomatic optic
neuritis.12 Four cases had near complete recovery
of visual function, indicating a temporary block in conduction of action potentials
and not structural disruption of optic nerve axons. Pallor of the optic discs
was a common sequela. One case developed secondary optic atrophy.5 Our patient was nonvaccinated, had history of exposure
to mumps, and subsequently developed clinical findings consistent with mumps,
including acute parotitis followed by aseptic meningitis. Three weeks after
the onset of parotitis, she developed bilateral neuroretinitis with serologic
evidence of mumps infection.
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Clinical Characteristics and Outcome of Optic Neuritis Associated With
Mumps*
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Meningoencephalitis followed by parainfectious optic neuritis has been
reported in several other viral infections, including measles, rubella, varicella,
Epstein-Barr virus, and other pathogens, such as Coxiella
burnetti, toxoplasmosis, cat-scratch disease, and Lyme disease.17-18 The pathogenesis of parainfectious
optic neuritis is not well understood, but an immunologic basis has been postulated.17 The delayed onset of optic neuritis in mumps infection,
bilateral involvement of the optic nerves, and profound loss of visual function
with often complete recovery support a systemic autoimmune demyelination process
rather than random viral invasion of each optic nerve.17
Additionally, the temporal framework and immunopathologic features of postinfectious
encephalitis closely resemble those of experimental models of virus-induced
demyelination of the central nervous system.19
The role of corticosteroids is controversial. A randomized controlled
trial of intravenous methyprednisolone followed by oral prednisone in acute
optic neuritis in the adult population showed quicker recovery from visual
loss and better vision at 6 months.20 However,
it is unclear whether corticosteroids affect the natural history of the disease
in children, and this merits further investigation.21
The experience with corticosteroids in mumps is anecdotal and limited to a
few case reports.9, 11-12
Although regarded as a benign infectious disease, physicians should
be aware of optic neuritis as a potentially serious ocular complication of
mumps infection. In resource-poor countries, mumps immunization should be
included in the routine vaccination program.
AUTHOR INFORMATION
Accepted for publication March 13, 2002.
Author contributions: Study concept and design (Drs Khubchandani, Rane, and Nabi); acquisition of data (Drs Khubchandani, Rane, and Nabi, and Messrs Agarwal and Patel); analysis and interpretation of data (Drs Khubchandani,
Nabi, and Shetty); drafting of the manuscript (Drs
Khubchandani, Rane, and Nabi, and Messrs Agarwal and Patel); critical
revision of the manuscript for important intellectual content (Drs Khubchandani and Shetty); administrative, technical, and material
support (Drs Khubchandani, Rane and Nabi, and Messrs Agarwal
and Patel); study supervision (Drs Khubchandani,
Nabi, and Shetty).
We thank S. B. Desai, MD, from the department of imaging for help with
the MRI scanning and Lt Gen P. K. Chakraborty, MD, Chief Executive Officer,
Jaslok Hospital and Research Center for permission to publish the report.
Corresponding author and reprints: Avinash K. Shetty, MD, Department
of Pediatrics, Wake Forest University School of Medicine, Medical Center Blvd,
Winston-Salem, NC 27157 (e-mail: avishetty{at}pol.net).
From the Departments of Pediatrics (Drs Khubchandani and Nabi and Mr
Agarwal) and Ophthalmology (Dr Rane and Mr Patel), Jaslok Hospital and Research
Center, Mumbai, India; and the Department of Pediatrics, Wake Forest University
School of Medicine, Winston-Salem, NC (Dr Shetty).
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ABSTRACT
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