Detailed neuropathological information on the structural brain lesions underlying seizures is valuable for understanding drug-resistant focal epilepsy.We report the diagnoses made on the basis of resected brain specimens from 9523 patients who underwent epilepsy surgery for drug-resistant seizures in 36 centers from 12 European countries over 25 years. Histopathological diagnoses were determined through examination of the specimens in local hospitals (41%) or at the German Neuropathology Reference Center for Epilepsy Surgery (59%).The onset of seizures occurred before 18 years of age in 75.9% of patients overall, and 72.5% of the patients underwent surgery as adults. The mean duration of epilepsy before surgical resection was 20.1 years among adults and 5.3 years among children. The temporal lobe was involved in 71.9% of operations. There were 36 histopathological diagnoses in seven major disease categories. The most common categories were hippocampal sclerosis, found in 36.4% of the patients (88.7% of cases were in adults), tumors (mainly ganglioglioma) in 23.6%, and malformations of cortical development in 19.8% (focal cortical dysplasia was the most common type, 52.7% of cases of which were in children). No histopathological diagnosis could be established for 7.7% of the patients.In patients with drug-resistant focal epilepsy requiring surgery, hippocampal sclerosis was the most common histopathological diagnosis among adults, and focal cortical dysplasia was the most common diagnosis among children. Tumors were the second most common lesion in both groups. (Funded by the European Union and others.).
Rasmussen encephalitis (RE) is a rare but devastating unihemispheric brain disorder that often affects children.1 The clinical picture is characterized by intractable focal epilepsy and progressive decline of functions associated with the affected hemisphere.2 Despite its known inflammatory background and T-cell involvement, immunotherapy appears to slow rather than halt disease progression, and hemispherotomy appears to be the only solution for intractable epilepsy.1–4 A potential early therapeutic window has been suggested, and new therapeutic agents have become available.1 A monoclonal antibody targeting CD52 that leads to long-term depletion of lymphocytes (alemtuzumab) has previously been considered as a possible treatment option for RE, but clinical data are limited.1,5 Acknowledgment: The authors thank Michal Tichy for management of neurosurgery and Vladimir Komarek for his support.
Chirurgická léčba epilepsie představuje významnou terapeutickou alternativu u pacientů s farmakorezistentní epilepsií.Klinika dětské neurologie 2. LF UK a FN Motol se v rámci Centra pro epilepsie Motol zabývá chirurgickou léčbou epilepsie
ABSTRACT We report a girl with a drug‐resistant frontal lobe epilepsy caused by focal cortical dysplasia, who exhibited uncommon seizures. The seizures consisted of shoulder or whole body shuddering after a short psychic aura and face grimacing. Consciousness was fully preserved. The seizures resembled “wet dog shake” seizures described in rat models of epilepsy or shuddering attacks in infants. EEG findings were inconclusive, however, MRI showed a clear dysplastic lesion in the right frontal mesial and polar structures. The patient underwent an extended lesionectomy guided by neuronavigation and intraoperative electrocorticography. Focal cortical dysplasia type Ib was histologically confirmed and the patient has been seizure‐free for the three years following resection. [ Published with video sequences ]
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