A painting is shown that depicts the seizure semiology of a patient, i.e., Martina Schiele the painter and co-author of this article. It is illustrated that the artistic act helped to express and better understand her symptoms, which were hardly describable in words. A contextualisation and perspective on epilepsy and art is given.

BACKGROUND:Deterioration in naming function is a common sequelae after epilepsy surgery in the language-dominant temporal lobe but information on recovery and long-term outcome is scarce. We, therefore, assessed short-term and long-term outcome of object naming in patients undergoing surgery in the temporal lobe and determined factors affecting deterioration and recovery of naming function. METHOD:Object naming (Boston naming test) before surgery, at early follow-up (FU, 6-12 months) and late FU (≥2 years) was assessed in people with epilepsy (PWE) undergoing resections in the language-dominant left and non-dominant right temporal lobe. RESULTS:Sixty-six patients with left temporal lobe epilepsy (LTLE) and 87 control patients with right temporal lobe epilepsy (RLTE) were included. At early FU, 28 patients with LTLE (42%) and three patients with RTLE (3%) showed a significant naming decline. In patients with LTLE, risk for deterioration increased with lower verbal memory before surgery, older age at seizure onset and was particularly high with posterior temporal resections (≥40 mm from the temporal pole) and seizure onset >16 years. Of the patients with LTLE with early naming decline, 11 patients (39%) recovered fully in their naming abilities at late FU, averaging almost 10 years. Recovery was associated with the degree of postoperative naming decline at early FU. PWE with a decline of less than 10 items (<20%) had a good prognosis of recovery at late FU. Postoperative seizure control had no significant effect on recovery. CONCLUSIONS:In our cohort, less than 50% of PWE showed significantly deteriorated naming function after resection of the dominant temporal lobe. If a decline occurred, it appeared to recover to a certain degree and remained as a permanent deficit in 26% of the patients. Long-term outcome of visual object naming can be predicted by the degree of early postoperative decline.

Zusammenfassung Cécile und Oskar Vogt waren Gehirnforscher, die Struktur und Funktion des Zentralnervensystems über Jahrzehnte gemeinsam systematisch studierten. Im Berlin der Jahrhundertwende begannen sie noch vor 1900 ihre umfassenden zyto- und myeloarchitektonischen Studien an humanen Ganzhirnschnitten und mit Stimulationsexperimenten der Hirnrinde verschiedener Species, die sie bis zu ihrem Tod fortsetzen. Unter einfachen Bedingungen in einer Berliner Privatwohnung begannen sie ihre „Neurologische Centralstation“, die 1902 zum Neurologischen Laboratorium der Universität und 1914 zum Kaiser-Wilhelm-Institut (KWI) für Hirnforschung umgewidmet wurde. 1931 erst bezogen sie das neu gebaute KWI in Berlin-Buch, dem ersten Hirnforschungsinstitut seiner Art weltweit. Dies war wesentlich ermöglicht durch Zuwendungen von Krupp und der Rockefeller-Stiftung. In der NS-Zeit aus Berlin und dem Institut gedrängt, führten sie ihre Arbeit an einem neuen Institut im Schwarzwald fort, dessen Nachfolge heute das Cécile & Oskar Vogt Institut für Hirnforschung in Düsseldorf darstellt. In der Gruppe um die Vogts erarbeitete Korbinian Brodmann die berühmte Rindenkarte. Ihre Stimulationserkenntnisse diskutierten sie und glichen diese u. a. mit Otfried Foerster und Wilder Penfield ab. Die Vogts selbst arbeiteten an vielfältigen Themen. Wenngleich sie nicht direkt die Epilepsie studierten, so stellt die Ammonshornsklerose (AHS) eine zentrale Läsion in ihrer Überlegung zu ihrer Beobachtung der selektiven Vulnerabilität bestimmter Rindenregionen dar. Diese formulierten sie in der sog. Pathoklisetheorie, die gerade für Epilepsie und AHS kontrovers diskutiert wurde, sich aber als weitsichtig und i. W. als richtig erwies, wenngleich die Vogts sie mit den Mitteln ihrer Zeit noch nicht verifizieren konnten. Ihre Erkenntnisse wirken bis heute nach und stellen ein Fundament der modernen Epileptologie dar.

The ILAE Neuroimaging Task Force publishes educational case reports that highlight basic aspects of neuroimaging in epilepsy consistent with the ILAE's educational mission. Subcortical laminar heterotopia, also known as subcortical band heterotopia (SBH) or "double cortex," is an intriguing and rare congenital malformation of cortical development. SBH lesions are part of a continuum best designated as agyria-pachygyria-band-spectrum. The malformation is associated with epilepsy that is often refractory, as well as variable degrees of developmental delay. Moreover, in an increasing proportion of cases, a distinct molecular-genetic background can be found. Diagnosing SBH can be a major challenge for many reasons, including more subtle lesions, and "non-classic" or unusual MRI-appearances. By presenting an illustrative case, we address the challenges and needs of diagnosing and treating SBH patients in epilepsy, especially the value of high-resolution imaging and specialized MRI-protocols.

On February 23rd 1936, a boy-child ("Kn") died in an asylum near Munich after years of severe congenital disease, which had profoundly impaired his development leading to inability to walk, talk and see as well as to severe epilepsy. While a diagnosis of "Little's disease" was made during life, his postmortem brain investigation at Munich neuropathology ("Deutsche Forschungsanstalt für Psychiatrie") revealed the diagnosis of "amaurotic idiocy" (AI). AI, as exemplified by Tay-Sachs-Disease (TSD), back then was not yet understood as a specific inborn error of metabolism encompassing several disease entities. Many neuropathological studies were performed on AI, but the underlying processes could only be revealed by new scientific techniques such as biochemical analysis of nervous tissue, deciphering AI as nervous system lipid storage diseases, e.g. GM2-gangliosidosis. In 1963, Sandhoff & Jatzkewitz published an article on a "biochemically special form of AI" reporting striking differences when comparing their biochemical observations of hallmark features of TSD to tissue composition in a single case: the boy Kn. This was the first description of "GM1-Gangliosidosis", later understood as resulting from genetically determined deficiency in beta-galactosidase. Here we present illustrative materials from this historic patient, including selected diagnostic slides from the case "Kn" in virtual microscopy, original records and other illustrative material available. Finally, we present results from genetic analysis performed on archived tissue proving beta-galactosidase-gene mutation, verifying the 1963 interpretation as correct. This synopsis shall give a first-hand impression of this milestone finding in neuropathology. Original paper: On a biochemically special form of infantile amaurotic idiocy. Jatzkewitz H., Sandhoff K., Biochim. Biophys. Acta 1963; 70; 354-356. See supplement 1.

Epilepsy surgery is a viable therapy option for patients with pharmacoresistant focal epilepsies. A prerequisite for postoperative seizure freedom is the localization of the epileptogenic zone, e.g., using electro- and magnetoencephalography (EEG/MEG). Evidence shows that resting state MEG contains subtle alterations, which may add information to the workup of epilepsy surgery. Here, we investigate node degree (ND), a graph-theoretical parameter of functional connectivity, in relation to the seizure onset zone (SOZ) determined by invasive EEG (iEEG) in a consecutive series of 50 adult patients. Resting state data were subjected to whole brain, all-to-all connectivity analysis using the imaginary part of coherence. Graphs were described using parcellated ND. SOZ localization was investigated on a lobar and sublobar level. On a lobar level, all frequency bands except alpha showed significantly higher maximal ND (mND) values inside the SOZ compared to outside (ratios 1.11–1.20, alpha 1.02). Area-under-the-curve (AUC) was 0.67–0.78 for all expected alpha (0.44, ns). On a sublobar level, mND inside the SOZ was higher for all frequency bands (1.13–1.38, AUC 0.58–0.78) except gamma (1.02). MEG ND is significantly related to SOZ in delta, theta and beta bands. ND may provide new localization tools for presurgical evaluation of epilepsy surgery.

BACKGROUND:Epilepsy surgery offers an effective treatment to achieve seizure freedom in refractory temporal lobe epilepsy. Since left temporal lobe surgery can be associated with verbal memory deterioration, control of cognitive decline is a main goal of therapy. This study analyzes the prognostic value of intracarotid amobarbital procedure (Wada test) in addition to specific neuropsychological and clinical variables for postoperative memory changes.METHOD:Between 2013 and 2021 thirty-six patients (18 females, 18 males, mean age 41.0 years) from the Epilepsy Center Erlangen (ECE) with left hemispheric temporal lobe epilepsy underwent neuropsychological assessment preoperatively - including the Wada test - and six months postoperatively. In addition, a group of 92 patients (40 females, 52 males, mean age 36.1 years) with left or right hemispheric focus who underwent Wada test and surgery before 2013 was included as a standardization group. In all patients Wada test was carried out preoperatively to determine language dominance and memory capacity.RESULTS:Postoperative verbal memory scores showed no significant difference from preoperative performance. Preoperative verbal memory performance as well as the hippocampal resection extent is particularly important in predicting postoperative verbal memory change. After left temporal lobe surgery, a significantly higher postoperative functional level was shown for figural memory. Specifically, a good contralateral hemispheric performance level assessed by the Wada test proved to be a compensatory factor for postoperative losses.CONCLUSION:The Wada test is no longer necessary as a diagnostic tool for a broad group of patients with temporal lobe epilepsy. However, it can be useful for a subgroup of patients with clinical indicators such as nonspecific or incongruent preoperative verbal and figural memory impairments. In this study, Wada test data about the functional level of the contralateral hemisphere specifically allowed estimation of postoperative figural memory changes.

The objective of the current study was to optimize the postprocessing pipeline of 7 T chemical exchange saturation transfer (CEST) imaging for reproducibility and to prove this optimization for the detection of age differences and differences between patients with Parkinson's disease versus normal subjects. The following 7 T CEST MRI experiments were analyzed: repeated measurements of a healthy subject, subjects of two age cohorts (14 older, seven younger subjects), and measurements of 12 patients with Parkinson's disease. A slab-selective, B 1 + -homogeneous parallel transmit protocol was used. The postprocessing, consisting of motion correction, smoothing, B 0 -correction, normalization, denoising, B 1 + -correction and Lorentzian fitting, was optimized regarding the intrasubject and intersubject coefficient of variation (CoV) of the amplitudes of the amide pool and the aliphatic relayed nuclear Overhauser effect (rNOE) pool within the brain. Seven "tricks" for postprocessing accomplished an improvement of the mean voxel CoV of the amide pool and the aliphatic rNOE pool amplitudes of less than 5% and 3%, respectively. These postprocessing steps are: motion correction with interpolation of the motion of low-signal offsets (1) using the amide pool frequency offset image as reference (2), normalization of the Z-spectrum using the outermost saturated measurements (3), B 0 correction of the Z-spectrum with moderate spline smoothing (4), denoising using principal component analysis preserving the 11 highest intensity components (5), B 1 + correction using a linear fit (6) and Lorentzian fitting using the five-pool fit model (7). With the optimized postprocessing pipeline, a significant age effect in the amide pool can be detected. Additionally, for the first time, an aliphatic rNOE contrast between subjects with Parkinson's disease and age-matched healthy controls in the substantia nigra is detected. We propose an optimized postprocessing pipeline for CEST multipool evaluation. It is shown that by the use of these seven "tricks", the reproducibility and, thus, the statistical power of a CEST measurement, can be greatly improved and subtle changes can be detected.

There is a relevant risk of psychosocial as well as cognitive impairments in epilepsy patients with resective surgery in the left temporal lobe. Surgery in the speech-dominant hemisphere can be associated with deterioration of speech related functions including verbal memory. There are only limited studies addressing the impact of occupational therapy in postoperative rehabilitation of epilepsy patients. METHOD:In this study, a mixed-methods design based on the grounded theory concept was administered. Seven patients with left temporal lobe epilepsy and a risk profile for postoperative cognitive decline were investigated over various time points. Neuropsychological assessments occurred before surgery, 1 week, 3 months, and 6 months postoperatively. According to our rehabilitation concept, every patient started occupational therapy 1 month before surgery. Therapy lasted for at least 6 months after surgery. For all patients, extensive qualitative interviews with the occupational therapists and anamnestic data were analyzed. RESULTS:In all patients, postoperative psychosocial difficulties emerged. Language and memory tests showed a decline after 6 months specifically for name retrieval. Occupational therapy was adapted to find individual solutions for the patients problems and to implement effective coping strategies. CONCLUSION:Cognitive training lead to consistent improvements over time. The results show that occupational therapy can be an efficient tool in the treatment of psychosocial and cognitive impairments after epilepsy surgery.

The surgical treatment of frontal lobe epilepsy (FLE) has developed enormously during the last century. Milestones of understanding FLE and developing the surgical treatment are closely connected to important persons in the field of neurology and neurosurgery and their contributions to the knowledge about epilepsy. The main developments up to today and selected important protagonists are outlined in this article.

Isolated evaluation of multiparametric in vivo chemical exchange saturation transfer (CEST) MRI often requires complex computational processing for both correction of B0 and B1 inhomogeneity and contrast generation. For that, sufficiently densely sampled Z‐spectra need to be acquired. The list of acquired frequency offsets largely determines the total CEST acquisition time, while potentially representing redundant information. In this work, a linear projection‐based multiparametric CEST evaluation method is introduced that offers fast B0 and B1 inhomogeneity correction, contrast generation and feature selection for CEST data, enabling reduction of the overall measurement time. To that end, CEST data acquired at 7 T in six healthy subjects and in one brain tumor patient were conventionally evaluated by interpolation‐based inhomogeneity correction and Lorentzian curve fitting. Linear regression was used to obtain coefficient vectors that directly map uncorrected data to corrected Lorentzian target parameters. L1‐regularization was applied to find subsets of the originally acquired CEST measurements that still allow for such a linear projection mapping. The linear projection method allows fast and interpretable mapping from acquired raw data to contrast parameters of interest, generalizing from healthy subject training data to unseen healthy test data and to the tumor patient dataset. The L1‐regularization method shows that a fraction of the acquired CEST measurements is sufficient to preserve tissue contrasts, offering up to a 2.8‐fold reduction of scan time. Similar observations as for the 7‐T data can be made for data from a clinical 3‐T scanner. Being a fast and interpretable computation step, the proposed method is complementary to neural networks that have recently been employed for similar purposes. The scan time acceleration offered by the L1‐regularization (“CEST‐LASSO”) constitutes a step towards better applicability of multiparametric CEST protocols in a clinical context.

Magnetoencephalography (MEG) offers considerable advantages for presurgical localization diagnostics of frontal and generalized extratemporal epilepsy. A number of retrospective and prospective studies show a significant impact on the planning of invasive recordings and epilepsy surgical procedures with marked improvement of postoperative seizure control. This article provides a concise overview of the current state of the evidence.

Zusammenfassung Die Magnetenzephalographie (MEG) bietet für die prächirurgische Lokalisationsdiagnostik frontaler und allgemein extratemporaler Epilepsien wesentliche Vorteile. Eine Reihe retro- und prospektiver Studien zeigt einen signifikanten Einfluss auf die Planung invasiver Ableitungen und epilepsiechirurgischer Eingriffe mit deutlicher Verbesserung der postoperativen Anfallskontrolle. Der Artikel gibt einen kurz gefassten Überblick über den aktuellen Stand der Evidenz.

Die chirurgische Therapie der Frontallappenepilepsie (FLE) hat sich in den letzten über 100 Jahren enorm entwickelt. Meilensteine des Verständnisses der FLE und der Entwicklung ihrer chirurgischen Therapie sind eng verbunden mit wichtigen Persönlichkeiten aus Neurologie und Neurochirurgie und deren Beiträgen zum Verständnis der Epilepsie. Grundzüge der Entwicklung bis heute sowie beteiligte Akteure werden in diesem Beitrag umrissen.

Berthold Kihn (1895-1964) was a German psychiatrist. This article describes his life and works with respect to his contributions to the treatment of epilepsy, for which he invented and broadly used his own pharmaceutical treatment, Kihn's powder. Particular attention is paid to an overt discrepancy in Kihn & apos;s actions when comparing his later and earlier career years, when he was a National Socialism ideologist and not only an advocate of sterilization and so-called euthanasia but also an active protagonist during the Nazi regime.

50 years ago back in 1971, David C. Taylor and colleagues from England reported on a small series of surgical epilepsy cases proposing a new type of tissue lesion as a cause of difficult-to-treat focal epilepsy: a localized malformation of cerebral cortex. The lesion is now known as focal cortical dysplasia (FCD) Type II or Taylor’s cortical dysplasia. FCD II is not rare, and today is a frequent finding in neurosurgical epilepsy specimens. Medical progress has been achieved in that the majority of FCD II is diagnosed non-invasively by magnetic resonance imaging today. Detailed studies on FCD revealed that the lesion belongs to a spectrum of mTOR-o-pathies, thereby confirming the authors´ initial hypothesis of a relationship to tuberous sclerosis. Here, selected original materials from Taylor´s series are presented as virtual slides, supplemented by original clinical records, in order to give a first-hand impression of this milestone finding in neuropathology of epilepsy.