Since toxic effects of copper to the nervous system are widely known, copper deficiency is less well known and difficult to diagnose (Prodan et al., 2002; Kumar, 2006; Solioz et al., 2010). We present a case highly suggestive of copper deficiency myelopathy with stable disease since start of copper substitution 18 months ago. The 69 year old patient first presented in the department of neurosurgery in 2003 with a progressive gait disorder resembling a claudicatio spinalis. MRI exhibited a narrowing of the spinal canal LWK 3 to SWK 1 without myelopathy. Decompressive surgery was performed. However, no clear improvement after surgery occurred. Over the course of the years the symptoms deteriorated and in 2005 a spinal cord lesion at BWK 10 without contrast enhancement was seen. Contrast enhancement in 2010 was first detected in 2010. Then a spinal glioma was assumed and the patient was referred to the neurology department for a workup prior to the considered spinal biopsy. On clinical examination the patient had a spastic paraparesis, still being able to walk with walking aids. On MRI, not only a lesion of the spine, but also of the corpus callosum (T2, no contrast enhancement) could be found. Visual evoked potentials presented with delayed latencies. Laboratory findings showed a microcytic anemia without iron-deficiency. No other relevant changes were seen on extensive laboratory work up, especially no copper or zinc abnormalities or vitamin B12 – or folate deficiency. CSF showed a blood–brain-barrier dysfunction, but no other significant findings. We started copper substitution and refrained from spinal biopsy for the time being. Ever since the patient has remained clinically stable, the lesion on MRI is not progressive, and microcytosis disappeared. Copper-deficiency should be taken into consideration in case of a combination of myelopathy and microcytic anemia of unknown origin. Furthermore, pathologic visual evoked potentials and lesions of the corpus callosum have been described in copper deficiency. There is a risk of underdiagnosing copper deficiency since there is no definite laboratory marker, especially as normal copper serum-level does not rule out copper deficiency.

Cortical spreading depression (CSD) – initially considered a correlate for migraine auras – has also been related to other neurologic diseases in vitro. In vivo it has been shown in stroke and trauma patients and was considered to be a sign of poor outcome (Strong, 2002; Fabricius 2006; Dreier et al., 2012). The aim of our study was to investigate CSD in epilepsy patients undergoing invasive presurgical video-EEG-monitoring, allowing to collect CSD-data in patients without severe brain damage. Data of 18 patients with pharmacoresistant epilepsy undergoing invasive video-EEG-monitoring with 44 up to 106 subdural electrodes and with a high pass filter of 0,02 Hz filter settings were evaluated. CSD was defined as a slow wave in the range of 1–5 mm/min with suppression of the normal EEG-activity. Data were related to the extent of periprocedural hemorrhage, the origin and number of seizures and histopathologic findings. In 7/18 patients we could detect CSD. Two of them developed a clinically symptomatic subdural hematoma (SDH), paralleled by an increase of CSD-frequency; in 4/7 subclinical hemorrhage or minor lesions could be demonstrated on MRI, while one patient showed no abnormality on MRI. In comparison, minor hemorrhage was documented in 5/11 patients without CSD. There was no obvious spatial or temporal relation of CSD to seizure onset. The main histopathological finding both in patients with and without CSD was focal cortical dysplasia. Our data show CSD for the first time in vivo in patients without major brain trauma in patients with epilepsy. The main finding was that CSD is not limited to major brain damage but might also appear after mild trauma without clinical relevance or even without detectable trauma. Out data did not show a temporal or spatial correlation of CSD to seizure onset or histopathological findings. However, larger numbers and a longer follow-up are necessary to draw conclusions about the clinical relevance of CSD in epilepsy patients.

Extratemporal epileptic foci are increasingly treated successfully with surgery. This study assesses the effect of extratemporal epilepsy surgery on the subjective handicaps of epilepsy patients. Twenty-one adults with pharmacoresistant focal epilepsy undergoing extratemporal surgical interventions were analyzed compared to an age-, gender-, and IQ-matched patient group with temporal lobe epilepsy. A questionnaire on subjective handicaps was given prior to surgery and after 1 year of follow-up. There were significant postoperative improvements in the domains of work and activities, self perception, and judgement of changes in handicap. These changes were similar to those in patients undergoing temporal lobe surgery and not restricted to those achieving complete seizure remission. Epilepsy surgery thus improves not only seizure control but also quality of life in patients with extratemporal focus localization.

Quality of life (QOL) with respect to seizure outcome, cognitive performance, and depressive symptoms was analyzed in 21 adult patients undergoing extratemporal epilepsy surgery (EXTLE). The Subjective Handicap of Epilepsy questionnaire was administered before and 1 year after surgery. Additionally, cognitive performance, depressive symptoms (Beck Depression Inventory), and seizure frequency were assessed. After surgery, seizure frequency decreased and various aspects of QOL significantly improved. Improvements in the domain of Work and Activities correlated with a reduction in complex partial seizure frequency. Alterations in the subscale change correlated with a decrease in simple partial seizure frequency. The present results provide detailed evidence that patients with extratemporal epilepsy benefit from epilepsy surgery.

Extratemporale epileptische Foci werden zunehmend erfolgreich epilepsiechirurgisch behandelt. In der vorliegenden Studie wurde der Effekt extratemporaler epilepsiechirurgischer Eingriffe auf das subjektiv empfundene Handicap von Epilepsiepatienten untersucht. 21 erwachsene Patienten mit pharmakoresistenter fokaler Epilepsie, die sich extratemporalen epilepsiechirurgischen Eingriffen unterzogen, wurden im Vergleich zu hinsichtlich Alter, Geschlecht und IQ gematchten Patienten mit Temporallappenepilepsie mittels eines Fragebogens zur Erfassung des subjektiven Handicaps vor Durchführung der Operation und ein Jahr nach Operation untersucht. Postoperativ zeigten sich signifikante Verbesserungen in den Bereichen Arbeit und Aktivitäten, Selbstwahrnehmung und in der Einschätzung der Veränderung des Handicaps. Diese Veränderungen waren ähnlich denen bei temporalen Resektionen und traten nicht nur bei anfallsfreien Patienten auf. Die epilepsiechirurgische Therapie stellt somit auch bei extratemporaler Fokuslokalisation eine Behandlungsoption dar, die sich über eine Anfallskontrolle hinaus positiv auf die Lebensqualität der Patienten auswirkt.

Question: Are there differences in seizure semiology in patients with hypothalamic hamartoma due to age and duration of epilepsy?