Electroencephalogram criticality in cognitive impairment: a monitoring biomarker?

Unlabelled: Critical states present scale-free dynamics, optimizing neuronal complexity and serving as a potential biomarker in cognitively impaired patients. We explored electroencephalogram (EEG) criticality in amnesic Mild Cognitive Impairment patients with clinical improvement in working memory, verbal memory, verbal fluency and overall executive functions after the completion of a 6-month prospective memory training. We compared "before" and "after" stationary resting-state EEG records of right-handed MCI patients (n = 17; 11 females), using the method of critical fluctuations and Haar wavelet analysis.

How Encephalopathy Impacts Language Ability: A Scoping Review of the Linguistic Abilities of Adults with Developmental and Epileptic Encephalopathy.

: Developmental and epileptic encephalopathy refers to a group of conditions where patients experience abnormal development due to various causes as well as frequent epileptiform discharges that ultimately contribute, in an independent and additive fashion, to cognitive and linguistic impairments. The language and cognition outcome of these patients in adulthood has been understudied. This paper aims to present a scoping review of linguistic abilities in adults with developmental and epileptic encephalopathy to determine the extent to which language outcomes in adulthood and their relation to cognitive outcomes have been studied.

The Pre-Interictal Network State in Idiopathic Generalized Epilepsies.

Generalized spike wave discharges (GSWDs) are the typical electroencephalographic findings of Idiopathic Generalized Epilepsies (IGEs). These discharges are either interictal or ictal and recent evidence suggests differences in their pathogenesis. The aim of this study is to investigate, through functional connectivity analysis, the pre-interictal network state in IGEs, which precedes the formation of the interictal GSWDs.

Branched-chain amino acids as adjunctive-alternative treatment in patients with autism: a pilot study.

The branched-chain amino acid (BCAA) is a group of essential amino acids that are involved in maintaining the energy balance of a human being as well as the homoeostasis of GABAergic, glutamatergic, serotonergic and dopaminergic systems. Disruption of these systems has been associated with the pathophysiology of autism while low levels of these amino acids have been discovered in patients with autism. A pilot open-label, prospective, follow-up study of the use of BCAA in children with autistic behaviour was carried out.

Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis.

Background: There are limited data regarding the prevalence of distinct clinical, neuroimaging and genetic markers among patients diagnosed with cerebral amyloid angiopathy-related inflammation (CAA-ri). We sought to determine the prevalence of clinical, radiological, genetic and cerebrospinal fluid biomarker findings in patients with CAA-ri.

Methods: A systematic review and meta-analysis of published studies including patients with CAA-ri was conducted to determine the prevalence of clinical, neuroimaging, genetic and cerebrospinal fluid biomarker findings.

EEG Network Analysis in Epilepsy with Generalized Tonic-Clonic Seizures Alone.

Many contradictory theories regarding epileptogenesis in idiopathic generalized epilepsy have been proposed. This study aims to define the network that takes part in the formation of the spike-wave discharges in patients with generalized tonic-clonic seizures alone (GTCSa) and elucidate the network characteristics. Furthermore, we intend to define the most influential brain areas and clarify the connectivity pattern among them.

Restricted Ketogenic Diet Therapy for Primary Lung Cancer With Metastasis to the Brain: A Case Report.

A high-fat and low-carbohydrate diet was administered as a complementary and alternative therapy to a 54-year-old man suffering from non-small-cell lung cancer (NSCLC) with brain metastasis. Three months after the cessation of chemotherapy and radiotherapy, a ketogenic diet (KD) was initiated. This approach was an attempt to stabilize the disease progression after chemotherapy and radiotherapy.

Nonparaneoplastic Anti-GAD Limbic Encephalitis: Seizure Outcome and Long-term Neuropsychological Follow-up After Immunotherapy.

Antibodies against glutamate decarboxylase (GAD-Abs), especially GAD65 antibodies, are associated with limbic encephalitis (LE) manifested by temporal lobe epilepsy and neuropsychological deficits. We present the case of a 42-year-old Greek woman with nonparaneoplastic anti-GAD LE, discussing the therapeutic management and highlighting the role of neuropsychological assessment. The patient underwent functional and structural brain studies and was investigated longitudinally over a 6-year period with a battery of neuropsychological tests that were designed to document her intellectual function and verbal and visual memory.

Primary Sjögren's Syndrome Presenting with Rapidly Progressive Dementia: A Case Report.

Background: Rapidly progressive dementias (RPDs) are dementias that progress subacutely over a time period of weeks to months. Primary Sjögren's syndrome (pSS) is an autoimmune disease that can affect any organ system and may present with a wide range of clinical features that may mimic a plethora of medical conditions and, in rare cases, may manifest as RPD. We describe a unique case of pSS, in which rapidly progressive dementia (RPD) was the first disease manifestation, and the patient's radiological and electroencephalogram findings were compatible with Creutzfeldt- Jakob disease (CJD).

Neuroimaging and Electroencephalographic Correlation in Patients with Transient Global Amnesia: Clinical Case Series.

. To determine if there is any correlation between the electroencephalographic and neuroimaging findings in patients with Transient Global Amnesia (TGA). We retrospectively reviewed files of the First Department of Neurology of AHEPA University Hospital, including patients with a clinical diagnosis of TGA.

The Efficacy and Safety of Ketogenic Diets in Drug-Resistant Epilepsy in Children and Adolescents: a Systematic Review of Randomized Controlled Trials.

Purpose Of Review: Drug-resistant epilepsy represents around one-quarter of epilepsies worldwide. Although ketogenic diets (KD) have been used for refractory epilepsy since 1921, the past 15 years have witnessed an explosion of KD use in the management of epilepsy. We aimed to review evidence from randomized controlled trials (RCTs) regarding the efficacy and safety of KD in drug-resistant epilepsy in children and adolescents.

Prevalence of Clinical and Neuroimaging Markers in Cerebral Amyloid Angiopathy: A Systematic Review and Meta-Analysis.

Background: Limited data exist regarding the prevalence of clinical and neuroimaging manifestations among patients diagnosed with cerebral amyloid angiopathy (CAA). We sought to determine the prevalence of clinical phenotypes and radiological markers in patients with CAA.

Methods: Systematic review and meta-analysis of studies including patients with CAA was conducted to primarily assess the prevalence of clinical phenotypes and neuroimaging markers as available in the included studies.

Genetic cause of epilepsy in a Greek cohort of children and young adults with heterogeneous epilepsy syndromes.

We describe a cohort of 10 unrelated Greek patients (4 females, 6 males; median age 6.5 years, range 2-18 years) with heterogeneous epilepsy syndromes with a genetic basis. In these patients, causative genetic variants, including two novel ones, were identified in 9 known epilepsy-related genes through whole exome sequencing.

Cerebral folate deficiency in two siblings caused by biallelic variants including a novel mutation of gene: Intrafamilial heterogeneity following early treatment and the role of ketogenic diet.

Mutations in the gene, encoding for the folate alpha receptor (FRa), represent a rare recessive genetic cause of cerebral folate deficiency (CFD), a potentially reversible neurometabolic condition. Patients typically present with developmental delay, seizures, abnormal movements, and delayed myelination. We hereby expand the phenotypic and genotypic spectrum of the disease with the report of the first two Greek siblings that were found compound heterozygous for one known gene mutation (p.

Genetic cause of heterogeneous inherited myopathies in a cohort of Greek patients.

Inherited muscle disorders are caused by pathogenic changes in numerous genes. Herein, we aimed to investigate the etiology of muscle disease in 24 consecutive Greek patients with myopathy suspected to be genetic in origin, based on clinical presentation and laboratory and electrophysiological findings and absence of known acquired causes of myopathy. Of these, 16 patients (8 females, median 24 years-old, range 7 to 67 years-old) were diagnosed by Whole Exome Sequencing as suffering from a specific type of inherited muscle disorder.

The nature, frequency and value of stimulation induced seizures during extraoperative cortical stimulation for functional mapping.

Purpose: The aim of this retrospective service evaluation was to determine the nature, frequency and clinical value of seizure occurrence during extraoperative direct cortical stimulation for functional mapping in patients undergoing invasive recordings (icEEG) for epilepsy surgery workup.

Methods: We reviewed 145 sequential cases of patients with refractory focal epilepsy who underwent intracranial electrode implantation and extraoperative direct cortical stimulation (CS) for functional mapping. CS intended for mapping can elicit as a by-product electrical or electroclinical events, such as afterdischarges, subclinical EEG seizures, and stimulation-induced seizures (SIS).

A probable role of copper in the comorbidity in Wilson's and Creutzfeldt-Jakob's Diseases: a case report.

Background: To the best of our knowledgedd, there is currently no case in the literature reporting the comorbidity of Wilson's and Creutzfeldt-Jakob disease (CJD), linked through copper.

Case Presentation: A 44-year-old male with a history of inherited Wilson's disease (hepatolenticular degeneration), which manifested as mild liver injury and psychiatric symptoms, was admitted to our department due to speech and cognitive disturbances. Upon his admission, he had motor aphasia as well as psychomotor retardation with an otherwise normal neurological examination.

West Nile neuroinvasive disease. Report of four cases in Northern Greece, 2018.

West Nile virus (WNV) is a mosquito-borne RNA flavivirus which caused several epidemics worldwide. The year 2018 was a WNV record year for Europe, including Greece, with earlier and longer transmission season with higher than the previous number of cases. It has been proposed that some simple biochemical markers may be helpful for the recognition of WNV neuroinvasive disease, its differential from other neurological infectious diseases and prognosis.

Is brain connectome research the future frontier for subjective cognitive decline? A systematic review.

Objective: We performed a systematic literature review on Subjective Cognitive Decline (SCD) in order to examine whether the resemblance of brain connectome and functional connectivity (FC) alterations in SCD with respect to MCI, AD and HC can help us draw conclusions on the progression of SCD to more advanced stages of dementia.

Methods: We searched for studies that used any neuroimaging tool to investigate potential differences/similarities of brain connectome in SCD with respect to HC, MCI, and AD.

Results: Sixteen studies were finally included in the review.

Theory of Mind impairment in focal versus generalized epilepsy.

Theory of Mind (ToM) is a critical component of social cognition, and thus, its impairment may adversely affect social functioning and quality of life. Recent evidence has suggested that it is impaired in epilepsy. What is not clear, however, is whether it is related to particular types of epilepsy or other factors.

Spine pathology in a girl with upper limb pain: A co-incidence or a causal relationship?

A 10-year-old girl was admitted to the Emergency Department due to a history of intermittent pain located in the left radiocarpal joint for a month, as well as in the interphalangeal joints of the left hand without any additional symptoms. Clinical examination revealed mild sensory deficits and diminished muscle strength of the left upper limb without any other pathologic findings. A Magnetic Resonance Imaging scan of the brain and spinal cord was performed, which confirmed a diagnosis of thoracic syringomyelia.

Genotype-phenotype correlations and expansion of the molecular spectrum of AP4M1-related hereditary spastic paraplegia.

Background: Autosomal recessive hereditary spastic paraplegia (HSP) due to AP4M1 mutations is a very rare neurodevelopmental disorder reported for only a few patients.

Methods: We investigated a Greek HSP family using whole exome sequencing (WES).

Results: A novel AP4M1A frameshift insertion, and a very rare missense variant were identified in all three affected siblings in the compound heterozygous state (p.