EEG at onset and MRI predict long-term clinical outcome in Aicardi syndrome.

OBJECTIVE: Descriptions of electroencephalographic (EEG) patterns in Aicardi syndrome (AIC) have to date referred to small cohorts of up to six cases and indicated severe derangement of electrical activity in all cases. The present study was conducted to describe the long-term EEG evolution in a larger AIC cohort, followed for up to 23 years, and identify possible early predictors of the clinical and EEG outcomes. METHODS: In a retrospective study, two experienced clinical neurophysiologists systematically reviewed all EEG traces recorded in 12 AIC cases throughout their follow-up, from epilepsy onset to the present. Clinical outcome was assessed with standardized clinical outcome scales. RESULTS: Analysis of the data revealed two distinct AIC phenotypes. In addition to the "classical severe phenotype" already described in the literature, we identified a new "mild phenotype". The two phenotypes show completely different EEG features at onset of epilepsy and during its evolution, which correspond to different clinical outcomes. CONCLUSIONS: Data from our long-term EEG and clinical-neuroradiological study allowed us to describe two different phenotypes of AIC, with different imaging severity and, in particular, different EEG at onset, which tend to remain constant over time. SIGNIFICANCE: Together, these findings might help to predict long-term clinical outcomes.

[Radiotherapy in benign diseases: Morbus Peyronie]. / Strahlentherapie benigner Erkrankungen: Morbus Peyronie.

PURPOSE: The induratio penis plastica (IPP) or the so-called Morbus Peyronie is a burdening disease for patients with three main symptoms, induration, deviation and pain. The etiology of this fibrosing and plaque forming disease is largely unknown up to now. PATIENTS AND METHODS: The presented data refer to a retrospective univariant examination of 67 patients, which have been treated for IPP with soft X-rays in the Dermatological Clinic of the Technical University of Munich between 1990 and 1995. The aim of the study was to examine, how far a progression of the disease can be stopped with soft X-rays and how the pain symptomatic is reduced. RESULTS: In 58 of 67 examined patients (86.6%) a progression of the disease could be stopped. 25 out of 67 patients (37.3%) complained of pain before therapy. This symptomatic mostly improved totally in 21 patients (84% of the patients with pain). A complete or partial dissolution of the indurations was to be noticed in 41 of 70 indurations (58.6%). In 23 of 60 patients (38.3%) an improvement or totally regression of the deviation was observed. It could be shown that therapeutic outcome significantly correlates to a shorter duration of anamnesis (p < 0.05), smaller plaque size (p < 0.025) and a tendency to lower age of the patients. CONCLUSION: Radiotherapy of Morbus Peyronie is extremely effective and has a low rate of side effects.