Hughlings Jackson and the role of the entorhinal cortex in temporal lobe epilepsy: from patient A to Doctor Z.
Hughlings Jackson's insightful bedside observations of patients with epilepsy paved the way for the first effective surgical epilepsy treatments. Jackson's most famous case, that of Doctor Z, concerned a medical doctor with partial complex seizures who was reported to have a discrete and circumscribed medial temporal lobe (mTL) lesion on autopsy. Although integral to Jackson's argument for mTL resection, the case remains controversial due to inadequate pathological descriptions of Doctor Z's lesion. This motivated us to describe the case of a patient, whom we call Patient A, who suffered from a form of epilepsy similar to that of Doctor Z, accompanied by a discrete and circumscribed mTL lesion in the exact same location. The lesion, a cavernous hemangioma, spared the hippocampus and was restricted to the lateral aspect of the entorhinal cortex. This finding validates Jackson's original description and suggests that the entorhinal cortex can play a role in seizure genesis.
Khalsa
Sahib S
SS
Department of Neurology, 1216 MERF, University of Iowa, Iowa City, IA 52242, USA. sahib-khalsa@uiowa.edu <sahib-khalsa@uiowa.edu>
Moore
Steven A
SA
Van Hoesen
Gary W
GW
D000368:Aged; D001932:Brain Neoplasms / Q000150:complications / Q000473:pathology*; D004569:Electroencephalography; D018728:Entorhinal Cortex / Q000473:pathology*; D004833:Epilepsy, Temporal Lobe / Q000150:complications / Q000266:history / Q000473:pathology*; D020786:Hemangioma, Cavernous, Central Nervous System / Q000150:complications / Q000473:pathology*; D049672:History, 19th Century; D006801:Humans; D008297:Male; D008569:Memory Disorders / Q000209:etiology / Q000473:pathology