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  • Author or Editor: Johannes Schramm x
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Intravascular papillary endothelial hyperplasia of the sellar region

Report of three cases and review of the literature

Rudolf A. Kristof, Dirk Van Roost, Helmut K. Wolf and Johannes Schramm

✓ Intravascular papillary endothelial hyperplasia (IPEH) is considered a reactive proliferation of endothelium associated with thrombosis. The occurrence of IPEH in the cranial cavity is exceedingly rare. In this article, the authors report three cases of IPEH that originated from the cavernous sinus and extended into the sellar contents. The lesions were resected incompletely in two cases and completely in one case. The IPEH in one of the patients was incompletely resected and exhibited further growth on magnetic resonance imaging 3 months postoperatively; local radiation therapy was instituted. This led to shrinkage of the lesion over an additional follow-up period of 3.5 years. In a review of the literature, the authors located seven other cases of intracranial IPEH. The authors conclude that clinically symptomatic intracranial IPEH should be completely resected whenever possible, because it can cause considerable morbidity and mortality and because it is prone to progression or recurrence.

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Josef Zentner, Andreas Hufnagel, Ulrich Pechstein, Helmut K. Wolf and Johannes Schramm

✓ In this article, the authors report their experience with surgically induced supplementary motor area (SMA) deficiency syndrome in a prospective clinical trial of 28 patients who underwent surgery for tumorous (19 patients) or nontumorous (nine patients) lesions. The dominant side was affected in 17 patients and the nondominant side in 11 patients. The primary presenting symptoms included seizure activity (23 patients), hemiparesis (four patients), and aphasia (one patient). Functional topographic mapping, achieved by phase reversal of somatosensory evoked potentials, allowed precise localization of the central sulcus in 25 of the 28 patients. Motor evoked potential (MEP) monitoring, which was performed successfully in 13 of 15 cases during the resective procedure, showed no significant changes in the potentials in any patient. Immediately after surgery, 25 (89%) of the 28 patients displayed additional neurological deficits (aphasia and/or hemiparesis) that depended on the extent of the SMA resection. In 12 patients the SMA was resected completely: nine of these patients demonstrated a complete and three an incomplete deficit. In 16 patients the SMA resection was incomplete: 13 of these patients displayed an incomplete deficit, whereas three had no deficit. Neurological disorders resolved completely within 3 to 42 days (mean 11 days), except for a minimal disturbance of fine motor and/or speech function in complex tasks or at high speed. Electromagnetically elicited MEPs, examined postoperatively in five patients, were initially absent but recovered with improvement of motor function. In conclusion, although the SMA is known to control important functions such as initiation of motor activity or speech, our findings show that unilateral SMA removal can be accomplished without resulting in significant permanent deficits. Functional topographic mapping and monitoring facilitate the exact delineation of the adequate resection plane along the precentral sulcus, and postoperative magnetic resonance imaging allows precise correlation of clinical and anatomical data.

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Josef Zentner, Helmut K. Wolf, Christof Helmstaedter, Thomas Grunwald, Ales F. Aliashkevich, Otmar D. Wiestler, Christian E. Elger and Johannes Schramm

Object. The goal of this study was to define the incidence and clinical significance of amygdala sclerosis (AS) in patients with temporal lobe epilepsy (TLE).

Methods. Surgical specimens of the lateral amygdaloid nucleus and the hippocampus excised from 71 patients who were treated for medically intractable TLE were quantitatively evaluated using a computer-assisted image-analysis system and compared with 10 normal autopsy specimens. Densities of neurons and reactive astrocytes in the patients with TLE were correlated with clinical, neuropsychological, and depth-electroencephalography data. The neuron counts of the lateral amygdaloid nucleus did not correlate with various presumed etiological factors of TLE including hereditary seizures, birth complications, febrile convulsions, traumatic brain injury, infections, seizure semiology, and epileptological outcome. However, patient age at surgery was significantly higher (mean difference 10 years) when AS was present, as compared with patients without AS (p < 0.01). Seizure origin, as determined by using amygdalohippocampal depth electrodes, did not correlate with the presence or absence of AS. Neuropsychologically, there was a significant correlation between the neuronal densities of the lateral amygdaloid nucleus and both preoperative visual recognition and postoperative deterioration of short-term verbal memory performance (p < 0.05).

Conclusions. Except for the relatively long history of epilepsy, the presence of AS is not associated with specific clinical or electrocorticographic features of mesial TLE. However, patients without AS are particularly at risk for deterioration of short-term verbal memory following amygdalohippocampectomy.