Impairment of cognitive functions in children and adolescents with focal epilepsy.

Introduction: Frontal Lobe Epilepsy (FLE) and Temporal Lobe Epilepsy (TLE) are the two most frequent types of focal epilepsies and they are connected with difficulties in cognitive functioning. Despite multiple trials to systematize profile of cognitive functioning among children with epilepsy by researchers, the available data are ambiguous. The aim of our study was to compare the cognitive function of children upon diagnosis of TLE and FLE and during follow-up and to compare with a control group of healthy children. Material and methods: Study included 39 patients with newly diagnosed TLE, 24 patients with FLE whose first epileptic seizure occurred between their 6th and 12th year of life and 24 healthy children matched by age, sex and IQ level. Neuropsychological examination was performed the moment of diagnosis and 2-3 years later using diagnostic tools validated and standardized to the patient's age. Intergroup comparison was conducted in both stages of study. Also, correlation between localization of epileptic focus and cognitive difficulties was analysed. Results: Children with FLE and TLE accomplished worse results in most of the cognitive tasks compared to the control group already in the initial examination. Patients with FLE presented difficulties in memorizing verbal and visual material, attention and in learning new information. Patients with TLE had difficulties in tasks engaging verbal and non-verbal memory and attention. In the follow-up, patients with FLE presented more severe cognitive impairment compared with the other groups. Despite similar tendencies among children with TLE significantly worse results in tasks engaging verbal memory and attention were observed among patients with FLE. It is noteworthy that patients suffering from FLE and TLE present deficits in many aspects of cognitive functioning already at the time of diagnosis. Conclusion: Children and adolescents suffering from epilepsy are at risk of psychosocial difficulties, emotional disorders and mental illnesses. Thus, full assessment of cognitive function is essential in this patient group not only at the moment of diagnosis but also during follow-up in order to quickly introduce an individual support system.

Does progressive aphantasia exist? The hypothetical role of aphantasia in the diagnosis of neurodegenerative diseases.

Aphantasia is a heterogeneous neuropsychological syndrome consisting of the inability to create mental images. We argue that its progressive form may be a harbinger of dementia. Aphantasia may manifest as the inability to create any mental images or to create complex scenes, inability to spontaneously initiate generation of mental images, and/or inability to visualize a sequence of events.

The prevalence of the restless legs Syndrome/Willis-Ekbom disease among teenagers, its clinical characteristics and impact on everyday functioning.

BACKGROUND AND AIMS: The data on the prevalence of the Restless Legs Syndrome/Willis -Ekbom disease (RLS/WED) in the population of teenagers is scarce. The aim of this study was to determine RLS/WED occurrence in adolescents, its diagnostic accuracy, family history, clinical characteristics and impact on everyday functioning. MATERIAL AND METHODS: A group of 2379 pupils (aged 13-18 y.o.) from 6 randomly selected secondary schools in Gdansk, Poland were screened for RLS/WED with the use of a questionnaire. In order to verify the diagnosis and perform additional tests (neurological examination, psychological evaluation, biochemical blood tests, demographic questionnaire, International RLS rating scale/IRLSS, Epworth daytime sleepiness scale). all of the respondents with RLS/WED suspicion and their parents were asked for a consultation by a child neurologist. Both children and parents with RLS/WED diagnosis were tested with actigraphy at home for at least two consecutive nights. RESULTS: Two thousand and ninety seven students (88,15%) filled the questionnaire correctly (1171 girls and 926 boys, 56% and 44%). Sixty four respondents were suspected of having RLS/WED (3,1%), however, 36 of them were diagnosed as RLS/WED-mimics (mainly positional discomfort). Finally, 21 (1%) were diagnosed with definite idiopathic RLS/WED. The average age of symptom onset was 10.96 years. The severity was moderate in the most of the cases (61.9%) and the course of the disease was intermittent in all of them. Family history was positive in 80%. Abnormal actigraphy (PLMS index >5/h) was present in 80%. Blood level of ferritin was low (<50 ng/ml) in 85%. Excessive daytime sleepiness and school problems affected almost half of them. The presence of RLS/WED symptoms was associated with disrupted sleep, behavioral problems (irritability, aggression, hyperactivity), attention deficit and lowered mood. No correlation between RLS/WED and attention deficit hyperactivity disorder (ADHD), nocturnal enuresis or primary headaches was found. Thirty eight percent of the patients sought medical help, but none of them obtained proper diagnosis nor treatment of RLS/WED. CONCLUSIONS: In this study restless legs syndrome affected 1% of Polish teenagers, in the majority of cases was idiopathic and associated with positive family history. It affected sleep and everyday functioning. Neurological consultation is essential to avoid false positive diagnoses of RLS/WED in teenagers.

Peri-ictal headaches in the paediatric population - prospective study.

INTRODUCTION: The co-occurrence of headache and epilepsy is well-documented in the adult population. The aim of the prospective study was to analyse in the paediatric population the correlations between the types of peri-ictal headaches and types of seizures. Furthermore, an attempt was made to find trends in characteristic features of peri-ictal headaches.Material: A total of 57 children with peri-ictal headache were enrolled in the study. The participants' guardians were asked to keep a diary of the seizure and peri-ictal headache episodes during a 180-day period. During follow-up visits, systematic history regarding peri-ictal headaches was taken. RESULTS: A total of 913 seizure and 325 peri-ictal headache episodes were noted during the study. Post-ictal headaches were most common, occurring in < 1 h after the seizure, lasting minutes to hours and more likely to occur after generalised seizures, whereas pre-ictal headaches occurred 30-240 min before the seizure. In the analysed group, peri-ictal headaches were most often moderate in intensity. Only 30% of patients took analgesic medication, usually to treat post-ictal headaches. CONCLUSION: Peri-ictal headaches are a significant health problem for patients with epilepsy. The most common type are post-ictal headaches, and they are most likely to appear after a generalised seizure.

Edward Flatau (1868-1932) - world-leading pioneer of neurology and neurosurgery.

Polish neurology and neurosurgery owe a very great deal to Edward Flatau. He was a pioneer of these disciplines in Poland whose influence extends worldwide, and we recently celebrated the 150th anniversary of his birth. Edward Flatau's grandson, an outstanding physicist who has been living in San Diego in the USA for many years, has published a book 'Edward Flatau and his comet: beginnings of Polish neurology'. Its promotion took place in the Flatau hometown of Plock in Poland. In 1894, Edward Flatau published his notable work: 'Atlas of the human brain and the trajectory of fibres', the result of arduous work on brain sections and images that were captured with an especially designed camera. These photographs caused a publishing sensation at the end of the 19th century. The atlas was published in German and almost immediately translated into Polish, Russian, French and English. The most distinguished work of Edward Flatau was his great 1912 study of migraine headaches entitled 'Migraine', which has still not become out-of-date and is regularly quoted in international literature today.

Agraphia in patients with frontotemporal dementia and parkinsonism linked to chromosome 17 with P301L MAPT mutation: dysexecutive, aphasic, apraxic or spatial phenomenon?

OBJECTIVES: Patients with frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) may be agraphic. The study aimed at characterizing agraphia in individuals with a P301L MAPT mutation. METHODS: Two pairs of siblings with FTDP-17 were longitudinally examined for agraphia in relation to language and cognitive deficits. RESULTS: All patients presented with dysexecutive agraphia. In addition, in the first pair of siblings one sibling demonstrated spatial agraphia with less pronounced allographic agraphia and the other sibling had aphasic agraphia. Aphasic agraphia was also present in one sibling from the second pair. CONCLUSION: Agraphia associated with FTDP-17 is very heterogeneous.