Focal chronic inflammatory epileptic encephalopathy in a patient with malformations of cortical development, with a review of the spectrum of chronic inflammatory epileptic encephalopathy.

PURPOSE

Chronic cellular inflammation closely associated with epilepsy without an active infection is a hallmark of Rasmussen encephalitis (RE). RE has typical and defining features lacking in other rare epilepsy patients who also have neocortical lymphocytes without an identifiable cause. A patient with malformations of cortical development had an abrupt change in frequency and epileptic focus after 22 years of a stable seizure disorder. Functional neurosurgery yielded a specimen showing a mixed cellular meningoencephalitis in the absence of a demonstrable infection.

METHODS

Historical, neurologic, electroencephalographic, pathologic, and literature data were correlated.

RESULTS

There was a subarachnoid mixed infiltrate including evidence of dendritic cells in our patient and also cytotoxic T lymphocytes adjacent to karyolytic neurons that corresponded to cells previously demonstrated to damage neurons in RE. Literature review disclosed 42 other cases similar to RE but with heterogeneous findings. The course was more protracted and often more benign than in RE. The inflammation that would have markedly decreased or disappeared in RE over that period was generally still well represented.

CONCLUSIONS

Our patient has heterogeneous features similar to, yet with differences from, RE. Literature review of chronic cellular inflammatory epileptic encephalopathy cases also similar to RE discloses important differences that may reflect idiosyncratic reactions and pace of the disease rather than a different disease. Comorbidity factors, genetic population traits, and secondary effects of the seizure disorder may lead to an expansion of the initial site of damage by an autoimmune reaction. These cases might best be grouped, probably along with RE, as secondary autoimmune diseases.