CASE REPORT |
https://doi.org/10.5005/jp-journals-11001-0078 |
A Case Study Report on Subacute Sclerosing Panencephalitis from an Indian Tertiary Care System
1,2Department of Psychiatry, Silchar Medical College and Hospital, Silchar, Assam, India
Corresponding Author: Navanita Sarma, Department of Psychiatry, Silchar Medical College and Hospital, Silchar, Assam, India, e-mail: yutika.sarma555@gmail.com
Received on: 25 June 2024; Accepted on: 20 October 2024; Published on: 16 November 2024
ABSTRACT
A prolonged measles virus infection causes the unusual slow-progressing degeneration of the central nervous system known as subacute sclerosing panencephalitis (SSPE). This illness usually manifests in early stages of adolescence, progresses slowly, and has a dismal prognosis. Usually, there is a 6–8 years latent interval between measles infection and SSPE. It progresses gradually and finally results in death. The measles virus may induce an aberrant immune response that results in brain inflammation and this serious disease. Because SSPE is uncommon and sometimes misunderstood, it can be extremely challenging to diagnose. To arrive at a diagnosis, a complete examination and full history are required. Here we present a case of a 12-year-old girl with SSPE who visited a tertiary care hospital in Assam, India.
How to cite this article: Sarma N, Das H. A Case Study Report on Subacute Sclerosing Panencephalitis from an Indian Tertiary Care System. East J Psychiatry 2024;24(2):58-61.
Source of support: Nil
Conflict of interest: None
Keywords: Case report, Diagnosis, Measles virus, Immune response.
INTRODUCTION
A persistent measles infection causes subacute sclerosing panencephalitis (SSPE), a slowly progressing neurological illness. This condition has a significant fatality rate, progresses slowly, and usually manifests in early adolescence.1 It is caused by inflammation and brain deterioration, which impair cognitive and motor abilities.
PATHOGENESIS
The measles virus, a single-stranded ribonucleic acid (RNA) virus belonging to the Paramyxoviridae family, causes SSPE. Complications can be both short-term and long-term. Adults over 20 and children under five exhibit higher mortality rates.2,3 New study indicates that the pathogenesis has been attributed to both virus reproduction and host factors. Previous studies linked it to the virus’s inability to make the measles M protein, while more recent studies link it to changes in the gene that codes for this protein. It has been suggested that reduced cellular immunity and limiting the virus to intracellular multiplication by certain antibodies are host characteristics that contribute to the illness. Evidence suggests that patients with SSPE have an increased humoral immune response and a decreased cellular immunological response.4,5
Although brain biopsy is the gold standard for diagnosis, clinicians mostly use the Dyken’s criteria for it.
CASE DESCRIPTION
A 12-year-old girl, from a rural background, belonging to a lower socioeconomic status and studying in class VII, visited our hospital’s psychiatry outpatient department at Silchar Medical College and Hospital (SMCH) presenting with memory loss, intellectual deterioration, and unexplained sudden decline in her scholastic performance.
The girl was apparently well about 3 months prior when her mother noticed that the child had started having difficulty memorizing her schoolwork and began taking much longer time to finish assignments. She would sit down with her homework and fiddle with the pen only to leave the pages blank. There were also multiple complaints from the school regarding her falling grades. It was completely contrary to her earlier academic performance.
It was associated with decreased interaction, and she showed little inclination to talk to her parents and friends. Most of the days she skipped school only to remain confined within her room. When friends and relatives came to meet her, she only responded with a smile. It was also seen that she had difficulty recognizing her friends.
A few weeks later, she started developing progressive weakness on the left side of her body and drooping of the head along with an unsteady gait. Earlier, her mother thought the swaying of her body to one side was due to her fear of getting punishment, but gradually it was seen that there was also difficulty in holding objects, and she needed support to walk. There were progressive gait disturbances. With time, she became more withdrawn and looked confused all the time. She also developed some abnormal stereotyped movements. She was seen making fists, fixing her hair, or smelling her fingers. The patient was admitted to the psychiatry ward, SMCH, for further evaluation and treatment.
Her physical examination revealed no abnormality but on central nervous system (CNS) examination, tone and power were reduced in the right lower limb.
On mental status examination, eye-to-eye contact was inadequate, psychomotor activity was retarded, speech was monosyllabic with decreased tone and flow but coherent and relevant. Her effect was shallow, restricted to the lower side, stable, and appropriate with no hallucinatory behavior suggestive of perceptual disturbances. She was conscious and alert with comprehension intact but confused in left–right orientation and delayed in following commands.
On day 2 of admission, she had two episodes of urinary incontinence. When asked for some response, the patient only showed a brief hand movement. On day 3 of admission, she complained of slight blurriness of vision.
Her routine blood investigations and computed tomography (CT) scan brain reports were normal.
Her electroencephalogram (EEG) (Figs 1 and 2) showed characteristic bilateral periodic complexes.
Fig. 1: Electroencephalogram of the patient showing bilateral periodic complexes
Fig. 2: Electroencephalogram of the patient showing bilateral periodic complexes
Magnetic resonance imaging (MRI) of the brain (Fig. 3) showed T2/fluid-attenuated inversion recovery (FLAIR) hyperintensity with gyral swelling involving cortical and subcortical white matter of the right parieto-occipital region with diffuse restriction suggestive of acute infarct.
Fig. 3: Magnetic resonance imaging of the brain showing acute infarct involving parieto-occipital lobe on the right side
Her cerebrospinal fluid (CSF) analysis (Table 1) for measles immunoglobulin G (IgG) antibodies showed high titers of CSF total IgG and high CSF/serum quotient and significantly high titers of serum total IgG CSF/serum quotient 2.6.
| Serum IgG measles | 437.78 |
| CSF IgG measles | 488.87 |
| Serum total IgG | 1,673 |
| CSF total IgG | 45.5 |
| CSF/serum quotient | 2.6 |
References: CSFQ ref normal < 1.3; CSFQ ref equivocal 1.3–1.5; CSFQ ref positive > 1.5.
TREATMENT GIVEN
Before the final diagnosis was made, the patient was put on tablet brivaracetam 50 mg in two divided doses, tablet benfotiamine 100 mg, one tablet once daily, tablet clobazam 10 mg in two divided doses, and syrup L-carnosine was given.
After the final diagnosis, the patient was eventually referred to a neurology center.
PROGNOSIS
The mortality rate is quite high in the case of SSPE, about 95%, while the remaining cases undergo spontaneous remission.4-6
DISCUSSION
Our case puts emphasis over the fact that through proper, detailed, and thorough examination and necessary investigations with EEG, MRI brain, and CSF analysis, we can eventually reach a diagnosis. As the disease can mimic acute encephalopathy, we should include SSPE on the list of differential diagnoses of acute encephalopathy. A negative history of fever with rash in the past, as in our case, does not simply exclude the diagnosis of SSPE. When atypical symptoms such as psychiatric symptoms, uncontrolled seizures, or extrapyramidal symptoms are present, SSPE shows a fulminant course. Getting infected before the age of 2 years, increased viral load, and coinfection with other viruses puts patients at greater risk for fulminant, atypical course.3-5
Diagnostic Criteria
Dyken’s criteria are used for the diagnosis, which include two major and four minor criteria. Out of these, two major and one minor criteria are needed for the final diagnosis.7
Major Criteria
Possessing a typical or atypical presentation is one of the principal requirements. Acute, rapid, subacute, chronic progressive, or chronic relapsing-remitting are the categories used to describe the typical presentation. Seizures and a very unusual age of presentation fall into the category of being atypical.
An additional important criterion is elevated antimeasles antibodies, which need to be ≥1:4 in the CSF or 1:256 in the serum.
Minor Criteria
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Electrocardiogram (ECG) results in line with bilateral, synchronous, high-amplitude slow waves that occur at regular intervals. These are referred to as Radermecker complexes or slow-wave complexes.
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A higher than average concentration of globulin in the CSF, which accounts for over 20% of the total protein in the fluid.
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Measles virus detected in a brain biopsy.
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A molecular test that detects mutations in the genome of the wild strain of the measles virus.7
Progression of the Disease
Stage I: Symptoms include personality or behavioral changes, such as irritability, dementia, lethargy, social disengagement, or speech regression, as the illness progresses.
Stage II: Progressive loss of motor function, characterized by dyskinesia, dystonia, and myoclonus.
Stage III: Includes spasticity, posturing, and extrapyramidal symptoms.
Stage IV: Autonomic failure, akinetic mutism, or transition into a vegetative state.3
Treatment
The current options try to slow the disease’s progression, stabilize it, extend survival, or improve clinical outcomes.
Inosine pranobex is an oral antiviral that inhibits viral replication and functions as an immunomodulator. Three doses of 100 mg/kg are given throughout the day, with a maximum dosage of 3000 mg.
Isoprinosine and interferon-alpha (IFN-alpha) are commonly used together. It is an immunomodulator that is injected intrathecally.
Another nucleotide analog that has been tried is ribavirin. When combined with IFN-alpha, patients appeared to benefit only somewhat from it.4,5
Numerous case reports suggest a ketogenic diet as an alternative treatment, as it has been shown to be neuroprotective.
CONCLUSION
Antimeasles antibodies should be checked in the CSF whenever there are odd clinical signs and a neuroimaging picture. Psychiatrists ought to be cognizant of the diverse manifestations of SSPE and ought to incorporate it into their differential diagnosis when a young child exhibits depressive symptoms and cognitive decline.
REFERENCES
1. Upadhyayula PS, Yang J, Yue JK, et al. Subacute sclerosing panencephalitis of the brainstem as a clinical entity. Med Sci (Basel) 2017;5(4):26. DOI: 10.3390/medsci5040026
2. Ferren M, Horvat B, Mathieu C. Measles encephalitis: towards new therapeutics. Viruses 2019;11(11):1017. DOI: 10.3390/v11111017
3. Jafri SK, Kumar R, Ibrahim SH. Subacute sclerosing panencephalitis - current perspectives. Pediatric Health Med Ther 2018;9:67’71. DOI: 10.2147/PHMT.S126293
4. Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: an update. Dev Med Child Neurol 2010;52(10):901’907. DOI: 10.1111/j.1469-8749.2010.03717.x
5. Sato Y, Watanabe S, Fukuda Y, et al. Cell-to-cell measles virus spread between human neurons is dependent on hemagglutinin and hyperfusogenic fusion protein. J Virol 2018;92(6):e02166-17. DOI: 10.1128/JVI.02166-17
6. Nathan J, Khedekar Kale D, Naik VD, et al. Substantial remission in subacute sclerosing panencephalitis by following the ketogenic diet: a case report. Cureus 2019;11(8):e5485. DOI: 10.7759/cureus.5485
7. Chiu MH, Meatherall B, Nikolic A, et al. Subacute sclerosing panencephalitis in pregnancy. Lancet Infect Dis 2016;16(3):366’375. DOI: 10.1016/S1473-3099(15)00524-1
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