Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome




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Details zur Publikation

Autorenliste: Maestu F
Verlag: Wiley: 12 months
Jahr der Veröffentlichung: 2001
Bandnummer: 42
Heftnummer: 9
Erste Seite: 1198
Letzte Seite: 1203
Seitenumfang: 6
ISSN: 0013-9580
Sprachen: Englisch-Vereinigtes Königreich (EN-GB)


Purpose: The Parry-Romberg syndrome is an unusual disorder frequently
associated with epilepsy. The origin of this disease, and the cause of
epilepsy, are unknown. This study is the first reported case of the
Parry-Romberg syndrome, with intractable temporal lobe epilepsy, in
which detailed microanatomic analyses have been performed on resected
brain tissue obtained after surgical intervention.

Methods: Standard histopathologic methods and correlative light and
electron microscopy, combined with immunocytochemical techniques, were
used to study in detail the synaptic microorganization of the resected
hippocampal formation.

Results: After surgery, the patient was seizure free (followup
period of 4 years and 7 months). The resected temporal lobe showed a
variety of dramatic microanatomic alterations (small groups of ectopic
cells, neuronal loss, gliosis, and activated microglial cells) in mesial
structures, including the entorhinal cortex, subiculum, and dentate
gyrus. At the electron-microscopic level, we found that in the dentate
gyrus, the number of synapses in the cell-sparse region adjacent to the
ectopic mass of neurons was almost twice that found in the molecular and
polymorph cell layers, indicating the intrusion of neuritic processes
and synapse formation. In addition, the symmetrical axosomatic synapses
characteristically found on granule cells, which are likely derived from
gamma -aminobutyric acid (GABA)ergic inhibitory basket cells, were not

Conclusion: The complete seizure relief after surgery suggests that
the pacemaker region(s) of seizure activity were within the resected
tissue. However, we do not know which of the multiple neuropathologic
Findings reported here were the primary cause of seizure activity.
Nevertheless, the changes found in the dentate gyrus circuitry appear to
be among the most important alterations that would lead to epilepsy.


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