The Superior Frontal Transsulcal Approach to the Anterior Ventricular System: Exploring the Sulcal and Subcortical Anatomy Using Anatomic Dissections and Diffusion Tensor Imaging Tractography.

OBJECTIVE: To explore the superior frontal sulcus (SFS) morphology, trajectory of the applied surgical corridor, and white matter bundles that are traversed during the superior frontal transsulcal transventricular approach. METHODS: Twenty normal, adult, formalin-fixed cerebral hemispheres and 2 cadaveric heads were included in the study. The topography, morphology, and dimensions of the SFS were recorded in all specimens. Fourteen hemispheres were investigated through the fiber dissection technique whereas the remaining 6 were explored using coronal cuts. The cadaveric heads were used to perform the superior frontal transsulcal transventricular approach. In addition, 2 healthy volunteers underwent diffusion tensor imaging and tractography reconstruction studies. RESULTS: The SFS was interrupted in 40% of the specimens studied and was always parallel to the interhemispheric fissure. The proximal 5 cm of the SFS (starting from the SFS precentral sulcus meeting point) were found to overlie the anterior ventricular system in all hemispheres. Five discrete white matter layers were identified en route to the anterior ventricular system (i.e., the arcuate fibers, the frontal aslant tract, the external capsule, internal capsule, and the callosal radiations). Diffusion tensor imaging studies confirmed the fiber tract architecture. CONCLUSIONS: When feasible, the superior frontal transsulcal transventricular approach offers a safe and effective corridor to the anterior part of the lateral ventricle because it minimizes brain retraction and transgression and offers a wide and straightforward working corridor. Meticulous preoperative planning coupled with a sound microneurosurgical technique are prerequisites to perform the approach successfully.

Determinants of stigma in a cohort of hellenic patients suffering from multiple sclerosis: a cross-sectional study.

BACKGROUND: Patients suffering from several neurologic disorders may bear the "stigma" of their disease, being disqualified from full social acceptance. Although stigma is considered to be present in Multiple Sclerosis (MS), the factors that influence its levels are ambiguous. Aim of our study was to examine, for the first time in the literature, the basic determinants of stigma in a Hellenic MS-patients cohort, as well as how stigma affects their Quality-of-Life (QoL) profiles. METHODS: Three hundred forty two patients were recruited in this study. Data collected concerned sociodemographic and disease-related variables, mental illness assessment, Multiple-Sclerosis-QoL-54 (MSQoL-54) and Stigma-Scale-for-Chronic-Illness-24 (SSCI-24) questionnaires. Potential determinants were evaluated with univariate statistical analyses for their contribution to total, internalized (inner-self derived) and externalized (society derived) stigma. Important findings were further evaluated on hierarchical regression models. RESULTS: Disability levels were found to be the most powerful predictor in all stigma categories, followed by the presence of mental illness. Working and caregiving status were also ascertained as determinants of internalized stigma. Stigma levels displayed strong negative correlation with all composites of MSQoL-54. CONCLUSIONS: Stigma is present in the social environment of MS patients and was confirmed as a barrier (according to the International Classification of Functioning, Disability and Health), with detrimental effects on their QoL levels and functioning performances. Disability and mental illness were shown as the principal determinants of stigma, while financial characteristics were not as equally involved. Further validation of these results in other MS populations may provide safer conclusions, towards more efficacious patient-centered care outcomes.

Auditory event-related potentials (P300) and mesial temporal sclerosis in temporal lobe epilepsy patients.

AIMS: To investigate the role of centrally recorded P300 in patients suffering from mesial temporal sclerosis-temporal lobe epilepsy (MTS-TLE). METHODS: Sixteen patients (3 men and 13 women; median age: 32.5 years old) suffering from TLE with MTS and 43 healthy controls (12 men and 31 women; median age: 35 years old) participated in the study. P300 was elicited using an auditory two-stimulus oddball paradigm. In order to address the aim of the study, we adopted two statistical approaches; hierarchical linear regression analyses and ROC curves. RESULTS: After adjusting for age, MTS patients had a mean reduction of P300 amplitude by 6.93 µV and a mean increase of P300 latency by 38.78 ms, compared to controls. Age and MTS-TLE status accounted for 32 and 16% of the variance of latency and amplitude, respectively. Diagnostic analyses to detect MTS-TLE status revealed a sensitivity and specificity of 88 and 65% for amplitude and 81 and 70% for latency, respectively. No association between duration of disease and P300 characteristics were found. CONCLUSIONS: This study, along with other studies, contributes to our understanding and clinical significance of centrally recorded P300s in MTS-TLE patients. Future studies should focus on the association of these P300s with cognition in such patients.

Monosymptomatic clinically isolated syndrome with sudden sensorineural hearing loss: case report and critical review of the literature.

INTRODUCTION: Isolated cranial nerve involvement is rare in patients with multiple sclerosis (10.4%) and extremely rare is an eighth nerve palsy, especially in the context of a clinically isolated syndrome (<1%). CASE REPORT: A 34-year-old male presented with a history of left-sided tinnitus and sudden sensorineural hearing loss (SSNHL). Magnetic resonance imaging of the brain revealed >9, nonenhancing periventricular and corpus callosum lesions. Brainstem auditory evoked potentials were abnormal, ipsilateral to the affected ear, consistent with the presumed underlying demyelinating pathology. Visual evoked potentials showed bilateral prolonged P100 latencies. Oligoclonal bands were not detected in the cerebrospinal fluid, but IgG index was marginally elevated. After administration of corticosteroids, the patient recovered auditory function over a several month period. CONCLUSIONS: This report describes a case of SSNHL in the context of magnetic resonance imaging of the brain and electrophysiological findings consistent with a demyelinating etiology. SSNHL is a rare and possibly underrecognized manifestation of clinically isolated syndrome.

Toward understanding cognitive impairment in patients with myotonic dystrophy type 1.

Cognitive dysfunction and sleep disruption are two frequent but underestimated features of adult onset myotonic dystrophy type 1 (MD1). In order to investigate the MD1 cognitive profile and its relationship with sleep disruption, 23 patients with genetically proved MD1 (mild-moderate in severity) underwent neuropsychological (nps) and polysomnography assessment. Patients scored lower than controls on almost all nps tests but cognitive impairments were mostly observed in executive functions (z-score = -2.14), with complex attention (z-score = -1.04), memory (z-score = -0.65), constructions (z-score = -1.29), and reasoning (z-score = -0.75) being slightly affected. Moderate-severe sleep apnea (apnea-hypopnea index [AHI] > or =15) was very frequent with most of the apneas being of the obstructive type. However, we found hardly any evidence of association between subjective, objective sleep parameters, and nps performance (p > .001). Thus, in our cohort of 23 adult MD1 patients, mild cognitive dysfunction, which is mostly related to the dysfunction of frontal association cortex and its underlying neural networks, does not seem to be significantly influenced by sleep disruption, which is mainly caused by obstructive apnea events.

Cognitive deficits presenting as psychiatric symptoms in a patient with Moyamoya disease.

The case of a middle-aged man who had intraventricular hemorrhages secondary to Moyamoya disease (MMD), and initially, presented with psychiatric symptoms which did not respond to treatment is described. Neuropsychological assessment showed underlying significant cognitive deficits, mostly of complex attention and speed of information processing, visuospatial and constructional abilities, verbal and nonverbal memory, and executive functions. These deficits remained stable or slightly improved in follow-up assessments. Cognitive dysfunction should be suspected in case of psychiatric or psychiatric-related symptoms in MMD patients. This case study in Greece highlights the range of preserved and impaired cognitive functions in adult MMD by means of repeated neuropsychological evaluations in which a broad range of cognitive abilities were assessed.

Selective Reminding Test: demographic predictors of performance and normative data for the Greek population.

The Buschke Selective Reminding Test (SRT) measures verbal learning and memory during a multiple-trial list-learning task, which allows for analysis of encoding, storage, and retrieval data. This study of 443 healthy participants (ages 18 to 83 years with 3 to 18 years of education) presents normative data for the Greek population. Statistical analysis indicated that age and educational level were correlated with all the variables as well as sex, although to a considerably lesser extent. Performance on most of the measures decreased with increasing age and lower education, whereas sex differences favored women over men. Based on these results, the sample was stratified into six age groups and three levels of education, with mean and standard deviation for each group. Current norms for the Selective Reminding Test represent a useful neuropsychological tool in clinical practice for patients with memory dysfunction, irrespective of etiology.

The stroop effect in Greek healthy population: normative data for the Stroop Neuropsychological Screening Test.

The Stroop Test is a quick and frequently used measure in screening for brain damage, dysfunction of selective attention, and cognitive flexibility. The purpose of the present study is to provide normative data for Trenerry's Stroop Neuropsychological Screening Test (SNST) in a sample of 605 healthy Greek participants (age range: 18-84 years, education range: 6-18 years). Results revealed that age and education significantly contributed to SNST scores, accounting for a significant proportion of variance in time needed to complete the color task and in the interference Color-Word score. Performance on most of the measures decreases with increasing age and lower levels of education. Normative data stratified by age and education for the Greek adult population are provided as a useful set of norms for clinical practice.

Unusual cases of multiple symmetrical lipomatosis with neurological disorders.

Multiple symmetrical lipomatosis (MSL) is a rare disorder of unknown etiology defined as the presence of multiple and symmetrical fatty accumulations, usually involving the upper trunk, neck and head. Frequently associated findings include diabetes mellitus, hyperlipidemia, liver disease, hypothyroidism and polyneuropathy of unknown origin, but nevertheless, there are published reports of cognitive disorders in patients with MSL. We describe two unusual cases (38-year-old and 45-year-old Greek men) of MSL who presented with polyneuropathy and memory disorders. This is the first description of memory disorders in patients with MSL. We propose that Mini-Mental State Examination and assessment of cognitive functions should be performed for all patients with MSL. The underlying mechanism in our patients remains unknown, and this question should be the subject of a future study.