A study of SSPE: early clinical features
Summary (2 min read)
Introduction
- Subacute sclerosing panencephalitis (SSPE) is a slowly progressive fatal inflammatory disease of the central nervous system, developing as a sequel to childhood measles infection (1,2).
- Typically it presents with myoclonus and dementia progressing to a mute, bed-ridden and incontinent state finally leading to death.
- It has been brought down following implementation of measles vaccination in the developed countries (4).
- SSPE still continues to take lives of children in developing countries (6); the uncommon modes of presentation of SSPE pose diagnostic difficulties and hence are being highlighted in this study.
Materials and Methods
- This study was carried out in the department of neurology of a tertiary care hospital during the period of five years from 1998 to 2003.
- Those patients fulfilling the above criteria were selected and enrolled for the study.
- These patients were then evaluated clinically to look for abnormalities of higher mental functions, visual acuity, presence of chorioretinitis on fundus examination, presence of long tract signs and motor or sensory deficit.
- Clinical examination was followed by investigations including EEG, cerebrospinal fluid and serum examination to look for measles antibody titers.
Results
- Twenty four of these patients were males and eight were females.
- In their series 26 patients presented with cognitive decline, while myoclonus was seen in 27 patients .
- Out of the four patients with onset of illness after the age of 20 years three were vaccinated and, the single unvaccinated patient had suffered from measles at the age of 5 years.
- Thirteen out of the 32 patients showed uncommon features at the onset of the disease .
- Out of these seven, visual loss was the first symptom in six patients.
Features Number of Cases
- Presenting with seizures had a prolonged history of seizures, with average duration of 15 months before cognitive decline set in.
- Twenty one patients were imaged in their series.
- Changes were seen in parieto-occipital cortical and subcortical areas with symmetric involvement of the periventricular white matter in 10 out of 13 patients.
- Three patients had involvement of white and grey matter together while isolated involvement of basal ganglia was seen in a single patient .
Discussion
- SSPE is a chronic inflammatory disease of the central nervous system following childhood measles infection which is invariably fatal.
- The authors observation of rapid evolution of SSPE in vaccinated patients will need further scrutiny with larger number of patients.
- Visual loss was seen as frequently with the young as with the older patients.
- All six patients experienced generalized seizures, out of which four patients began their illness with seizures alone and in the other two such seizures were either along with or following other symptoms respectively.
- Those social issues have much changed over the past 30 years and this observation is difficult to explain and may be related to hormonal influence as suggested by Dyken et al (4).
Conclusions
- The mean age of presentation has increased to 13.4 years, over the past 30 years.
- Almost 40% of their patients presented with one or more of uncommon features like vision loss, seizures, and behavioral disturbances.
- These presentations need emphasis in the early diagnosis of this devastating condition.
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Cites background from "A study of SSPE: early clinical fea..."
...Vaccinated children developing subacute sclerosing panencephalitis constituted around 28% of the total cases of subacute sclerosing panencephalitis in a study from India with a rapid clinical worsening as compared to the nonvaccinated group.(4) This has been attributed to various factors such as poor nutritional status of children in developing countries resulting in poor vaccine uptake, a different circulating strain in the environment, subclinical measles infection prior to measles vaccination, or due to faulty storage (cold chain) of the vaccine, acting singly or in combination....
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...Ocular manifestations occur in 10% to 50% of patients of subacute sclerosing panencephalitis and could precede neurologic features in 10% to 20% of patients by a few weeks to months.(3,4) These include chorioretinitis, retinal pigmentary changes, increased retinal vessel tortuosity, papilledema, papillitis and disc pallor, cortical blindness, nystagmus, ptosis, and gaze palsies....
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...This has been attributed to various factors such as poor nutritional status of children in developing countries resulting in poor vaccine uptake, a different circulating strain in the environment, subclinical measles infection prior to measles vaccination, or due to faulty storage (cold chain) of the vaccine, acting singly or in combination.(4) We believe that 1 or more of these factors could have played a role in our patient resulting in subacute sclerosing panencephalitis despite measles immunization....
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References
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"A study of SSPE: early clinical fea..." refers background or result in this paper
...A similar study regarding clinical aspects of SSPE was published from our department in 1974 (7)....
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...We have also compared the present results with a similar study carried out in the same department of neurology in 1974 (7)....
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