From MMR to PDD via Acute Disseminated Encephalomyelitis (ADEM), sometimes an SSPE variant

Teresa Binstock
Researcher in Developmental & Behavioral Neuroanatomy
March 05, 2009

During the last 10 years or so, various researchers have considered that some cases of MMR-related autism may be an atypical variant of SSPE, otherwise known as subacute sclerosing panencephalitis (1). Evidence consistent with this possibility is increasing.

A recently published study described atypical SSPE in a child with Acute Disseminated Encephalomyelitis (2; ADEM). Importantly, ADEM can be induced by the MMR, as admitted by the Vaccine Court in regard to a child who developed PDD, one of the Autism-Spectrum Disorders (3). The new SSPE finding (2) is not the only report of ADEM being linked with SSPE and with demyelination (eg, 4-11). If an infant or toddler has hyper-inflammatory alleles (12) or impaired immunity, increased risk towards ADEM, atypical SSPE, and an ASD seems biologically plausible.


References

1. SSPE overview

http://en.wikipedia.org/wiki/Subacute_sclerosing_panencephalitis

2. Subacute Sclerosing Panencephalitis (SSPE) Presenting as Acute Disseminated Encephalomyelitis in a Child.

Goraya JS et al. J Child Neurol. 2009 Feb 9. [Epub ahead of print] PMID: 19204320

Subacute sclerosing panencephalitis (SSPE) typically presents with progressive mental deterioration, behavioral changes, and myoclonic jerks. Atypical presentations are not unknown and may result in diagnostic delays. A 9-year-old girl presented with poor balance and ataxia following an episode of upper respiratory tract infection. Neurological examination revealed mild hemiparesis and ataxia. Brain magnetic resonance imaging revealed scattered areas of T2 and fluid-attenuated inversion recovery hyperintensities in the white matter consistent with acute disseminated encephalomyelitis.... Later during the course of her illness, subacute sclerosing panencephalitis was suspected from the appearance of burst-suppression pattern on electroencephalogram, and the diagnosis confirmed by elevated titers of measles antibodies in cerebrospinal fluid. Physicians taking care of children need to be aware of atypical presentations of subacute sclerosing panencephalitis and must have a high index of suspicion to prevent diagnostic delays and avoid unnecessary diagnostic and therapeutic interventions.

3. ADEM --> PDD/ASD
Another ruling in the US vaccine court
Melanie Phillips, The Spectator
http://www.spectator.co.uk/melaniephillips/3395891/another-ruling-in-the-us-vaccine-court.thtml

4. PubMed Search:
Acute AND Disseminated AND Encephalomyelitis AND sspe

5: Involvement of morbilliviruses in the pathogenesis of demyelinating disease.
Rev Med Virol. 2007 Jul-Aug;17(4):223-44. PMID: 17410634

6: Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis.
Indian J Pediatr. 2006 Dec;73(12):1119-21. PMID: 17202644

7: Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis: a case report.
J Neurol Sci. 1997 Feb 27;146(1):13-8. PMID: 9077490

8: Immune responses during measles virus infection.
Griffin DE. Curr Top Microbiol Immunol. 1995;191:117-34. PMID: 7789155

The characteristic disease features of measles--fever and rash--are associated with the immune response to infection and are coincident with virus clearance. MV-specific antibody and CD4 and CD8 T cell responses are generated and contribute to virus clearance and protection from reinfection. During this same phase of immune activation immunologic abnormalities are also apparent. There is a generalized suppression of cellular immune responses that may contribute to increased susceptibility to other infections. Autoimmune disease may appear in the form of acute disseminated encephalomyelitis. If virus-specific immune responses are inadequate infection may progress with pulmonary or CNS manifestations, but without a rash. The pathogenesis of the rare disease SSPE, that occurs many years after primary infection is not clear, but immune responses show increased antibody to measles and cellular immune responses similar to those seen after uncomplicated infection.

9: Delayed acute measles inclusion body encephalitis in a 9-year-old girl: ultrastructural, immunohistochemical, and in situ hybridization studies.
Mod Pathol. 1992 May;5(3):348-52. PMID: 1353879

10: Magnetic resonance imaging of inflammatory and demyelinating white-matter diseases of childhood.
Dev Med Child Neurol. 1990 Feb;32(2):97-107. PMID: 2338183

11: Measles and the central nervous system.
Lancet. 1983 Dec 17;2(8364):1406-10. PMID: 6140503

12. Vaccination policy lags behind vaccine science
Teresa Binstock, Dec 2007
http://ravenintellections.typepad.com/from_the_desk_of_teresa_b/2007/12/vaccination-pol.html

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