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Case Report

Subacute sclerosing panencephalitis after measlesvaccination and without clinical measles, vaccineinduced or something else: A case report

Nikhil Bansal a, Raghvendra Narayan b,*aResident, Department of Pediatrics, Maharishi Markandeshwar Institute of Medical Sciences and Research,

Mullana, Ambala, IndiabAssociate Professor, Department of Pediatrics, Maharishi Markandeshwar Institute of Medical Sciences and

Research, Mullana, Ambala, India

a r t i c l e i n f o

Article history:

Received 27 November 2012

Accepted 11 October 2013

Available online xxx

Keywords:

Measles

SSPE

Vaccine associated

Case register

* Corresponding author. Tel.: þ91 (0) 8295352E-mail addresses: drrgh71@gmail.com, dr

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2212-8328/$ e see front matter Copyright ªhttp://dx.doi.org/10.1016/j.pid.2013.10.001

a b s t r a c t

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder charac-

terized by behavioral changes, myoclonus, dementia, visual disturbances, pyramidal and

extra pyramidal signs. The disease has a gradual progressive course leading to death

within few years of its onset.

SSPE in a child who has received measles vaccine and having no clinically apparent

measles in the past is a rare occurrence. Although these type of cases have been reported

worldwide its pathogenesis is still not well established. Many authors linked it to be due to

mutant strain of measles virus and some it to its live vaccines. To our knowledge there are

very scant literature from India about such cases so here we are reporting this case in an

8-year-old girl child.

Copyright ª 2013, Indian Academy of Pediatrics, Infectious Disease Chapter. All rights

reserved.

1. Introduction quoted as 0.14 per 100,000.3 Many authors were able to isolate

Acute measles infection is responsible for an estimated

164,000 deaths/year worldwide and is therefore a major vac-

cine preventable cause of death worldwide.1,2 Subacute scle-

rosing panencephalitis (SSPE) is a rare but usually fatal late

complication of measles, which presents 3e10 years after the

acute infection. SSPE after measles vaccination and without

clinically apparentmeasles have been reportedworldwide but

the pathogenesis in these type of cases are still not estab-

lished. It has also been linked with its live vaccine and the

estimated risk of SSPE after vaccination is often erroneously

890.rghvendra@rediffmail.co

l N, Narayan R, Subacuor something else: A cas

2013, Indian Academy of

viral antigens from brain tissue of patients and reported as

causative agent in these type of cases.4,5 Keeping in view of

rare presentation of rare disease we are reporting this case

and also intended to search the pathogenesis in available

literature.

2. Case report

An 8 years old female child born of non-consanguineous

marriage presented with myoclonic convulsions and tremors

m (R. Narayan).

te sclerosing panencephalitis after measles vaccination ande report, Pediatric Infectious Disease (2013), http://dx.doi.org/

Pediatrics, Infectious Disease Chapter. All rights reserved.

p e d i a t r i c i n f e c t i o u s d i s e a s e x x x ( 2 0 1 3 ) 1e32

since last 6 months. Her birth history, developmental history

and immunization history were normal. She had received

measles vaccine according to Indian Academy of Pediatrics

immunization schedule (measles vaccine at 9 months and

MMR at 15 months of the age). There was no history of fever

with rash prior to the illness or in the past. The child was

apparently well about 6 months prior to the admission when

the parents observed that the child had become unusually

quiet and withdrawn. She began to loose interest in her

studies and surroundings. The parents later noticed sudden

jerky movements of the upper limbs, which gradually pro-

gressed over the time and involved all the four limbs. Later

duration and frequency of these movements increased and

continued through out the day, each episode lasting for

30e40 min and finally the child became bed ridden. On ex-

amination, vitals of the child were within normal limits. The

child was poorly nourished with weight below the 3rd

percentile. She was not able to sit and also not able to control

her head. Glasgow coma scale was 10/15 (E4, V2, M4). Power

was 3/5 in both the upper limbs 1/5 in both the lower limbs. All

the four limbs were hypotonic and superficial and deep re-

flexes were absent. Response to pain was there in the form of

crying and rest of the sensations were absent. Apart from

theses clinical findings rest of the clinical examination were

normal. There were raised titers of anti-measles antibodies in

the cerebrospinal fluid suggesting of SSPE. The EEG picture

was characterized by periodic complexes consisting of bilat-

erally symmetrical, synchronous, high voltage bursts of

polyphasic, stereotyped delta waves.

After history and clinical examination along with labora-

tory findings diagnosis of SSPE was made. Parents were coun-

seled about the illness and the prognosis explained to them.

3. Discussion

Subacute sclerosing panencephalitis (SSPE) is a progressive

neurological disorder of childhood and early adolescence

caused by persistent defectivemeasles virus. The incidence of

this disease was reported to be one case per million patients

withmeasles a year.6 The history of primarymeasles infection

before age 2 years were present in most patients. SSPE is an

incurable disease and usually causes death within 2e4 years

of onset.7The initial symptoms of SSPE usually involve

regressive changes in intellect and personality. Within few

months, neurologic symptoms, myoclonic jerks develop and

compound the psychological symptoms. Our patient clinical

course reflected this typical natural history.

The five diagnostic criteria of SSPE include clinical pre-

sentation, a characteristic EEG, abnormal CSF studies,measles

antibody in serum and CSF, and brain biopsy. The diagnosis

can be reliably established if the patient fulfils three of the five

criteria.8The Cerebrospinal Fluid (CSF) in SSPE will typically

have markedly elevated values of gamma globulin and anti-

measles antibodies with IgG oligo-clonal bands.7e9 The EEG

pattern is virtually diagnostic with periodic complexes con-

sisting of bilaterally symmetrical, synchronous, high voltage

(200-500 mv) bursts of polyphasic, stereotyped delta waves

which repeat at fairly regular 4e10 s intervals and have a 1:1

relationship with myoclonic jerks.8e10 All these characteristic

Please cite this article in press as: Bansal N, Narayan R, Subacuwithout clinicalmeasles, vaccine induced or something else: A ca10.1016/j.pid.2013.10.001

EEG and CSF findings were seen in our case. MRI findings are

not essential and specific for the diagnosis of SSPE; it is sen-

sitive in detecting early white matter abnormalities. MRI

profile of SSPE includes focal abnormalities in the sub-cortical

white matter early in the course of disease and diffuse cere-

bral atrophy at later stages.11,12The estimated risk of SSPE

after vaccination is often erroneously quoted as 0.14 per

100,000 based on Farrington’s 1991 paper.3

The cases of SSPE in vaccinated individuals without history

of clinicalmeasles couldmaybedue to a) poor sero-conversion

with subclinical infection leading to SSPE, b) Vaccine failure

with subclinical or undiagnosed measles leading to SSPE, c)

Subclinical infection caused by some mutant variant of mea-

sles virus13 d) Measles vaccine induced SSPE. Because RNA vi-

ruseshavemorepropensity formutation, for further reasoning

as towhy this type of cases occurwemayhave to domolecular

epidemiological surveys to get a clear picture of MV genotypes

circulating in our country. Regarding association of measles

vaccine and SSPEmany authors in their cases stated that there

is no any indication that measles vaccine can induce SSPE but

in contrary to this Okuno et al reported 5.4% of cases of SSPE

probably due to vaccine associated.14

Eleven cases of SSPE in the UK were investigated with

isolation of viral antigens frombrain tissue of the patients. The

entire or partial matrix (M), Hemagglutinin (H), and Nucleo-

protein (N) genes of measles virus were sequenced following

direct RT-PCR amplification from brain tissue. No vaccine

strains were detected although five of these patients had been

previously immunized. The sequence data obtained from

these strains do not support the view that vaccine strains are

associated with SSPE and provide further studies of MV

epidemiology, evolution and pathogenesis in SSPE.4 Hotta et al

isolated a measles virus mutant strain named SSPE Kobe1-

shortly after the clinical onset in an SSPE patient from brain

tissue that again support the same view.5 So there is a need of

morecases tobe followedwith isolationofvirusorviralantigen

from the brain so that exact casual relationship regarding

pathogenesis could be established. Till date evidence is not

adequate to accept or reject a casual relation betweenmeasles

vaccine and SSPE. Though this type of cases encountered in

clinical practice, to our knowledge there are very scant report

specially from Indian subcontinent. Our aim to report this case

is to give special emphasis on mutant strains if causing this

type of cases should be identified if feasible so that thismay be

a hope for new vaccine that will prevent such fatal disease.

There is also aneedof case registryof SSPE cases in India as it is

in many countries so that much studies can be done and an-

swers for these types of cases could be established.3

Conflicts of interest

All authors have none to declare.

r e f e r e n c e s

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