Key result
Long-standing SSPE shows severe neuronal loss, tangles, and Lewy bodies in the substantia nigra.
Case Report (n=3)
Lewy bodies can be found in the substantia nigra and locus coeruleus in long-standing cases of subacute sclerosing panencephalitis, suggesting they may be by-products of attempted regeneration.
Case reports of Lewy pathology in long-standing SSPE are hypothesis-generating; should not change practice and require validation in larger cohorts.
coeruleus, but not in the dorsal vagal nucleus, nucleus basalis, cerebral cortex, spinal cord, superior cervical sympathetic ganglia, and coeliac ganglia.The second case was a nine year old boy presenting with intellectual deterioration and an epileptic seizure.A month later, when admitted to H6pital Ste-Justine, Montreal, he was unable to obey simple commands, his affect was inappropriate and there was mild orofacial chorea.The left plantar was exten- sor and his gait ataxic.He developed general- ised myoclonus with occasional opisthotonus, and within weeks showed decorticate postur- ing.Serum measles virus titre was 1024.CSF protein was 0-54 g/l, with no cells, and the measles complement fixation titre was 128. An EEG was dominated by low voltage slowing and periodic high voltage slow-wave complexes.His condition stabilised, but he remained bed-bound and died at the age of 19 years.The brain showed generalised atrophy, with profound neuronal loss and gliosis in the cerebral cortex and hippocampus with a few microglial nodules and tangles.Some remaining neurons showed intranuclear inclusions.All central grey nuclei showed severe neuronal loss with glial and microglial re- actions and several tangles.There was severe neuronal loss in the substantia nigra and locus coeruleus with tangles and Lewy bodies (fig).Lewy bodies were not found in the dorsal vagal nucleus, cerebral cortex, spinal cord or autonomic nervous system.These patients with SSPE showed long survival of 13 and 10 years respectively.Tangles occur even in patients dying at a young age,3 but Lewy bodies have not been described in ot-her cases.They were present in the substantia nigra and locus coeruleus, but not in other areas usually susceptible to Lewy bodies.We know of a third case of SSPE with onset at 21 years, and a nine year survival.In this case there were very few nerve cells in the substantia nigra, and some pale bodies, which are normally seen in Lewy body diseases.4Apart from the Lewy body-Parkinson's disease spectrum Lewy bodies are confined to a small group of rare degenerative disorders.'They may be by-products of attempted regeneration, rather than of degeneration, thus explaining their occurrence in longstanding cases of SSPE showing periods of relative stability.SSPE is a destructive in- flammatory process due to persistent measles infection, but the aberrant immunological mechanisms are not fully understood.Although there is no direct evidence for an infectious aetiology for Parkinson's disease, there are parallels with SSPE which results from an infectious agent acquired in early life, leading to a progressive disorder associated with disconcordance in identical twins,5 and Lewy bodies pathologically.
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D A Bennett (1990) conducted a case report in Subacute Sclerosing Panencephalitis (SSPE) (n=3). Subacute Sclerosing Panencephalitis (SSPE) was evaluated on Neuropathological findings. Patients with long-standing Subacute Sclerosing Panencephalitis (SSPE) demonstrated severe neuronal loss, tangles, and Lewy bodies in the substantia nigra and locus coeruleus.
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