Kostas Konstantopoulos

Normative Data of the Montreal Cognitive Assessment in the Greek Population and Parkinsonian Dementia

The Montreal Cognitive Assessment (MoCA) is a brief cognitive instrument for the measurement of dementia. The aim of the present study is to provide normative data for the MoCA test in the Greek speaking population and to measure its validity in a clinical group of parkinsonian dementia participants. A total of 710 healthy Greek speaking participants and 19 parkinsonian dementia participants took part in the study. Both, the MoCA test and a neuropsychological test battery (digit span, semantic verbal fluency, phonemic verbal fluency, Color Trails Test) were administered to the normative and clinical samples. The test was found to correlate with all neuropsychological tests used in the test battery and it showed high discriminant validity (optimal screening cutoff point = 21, sensitivity = 0.82, specificity = 0.90) in the parkinsonian dementia participants. Further research is needed to use it in larger clinical samples and in different neurological diseases.

A quantitative method for the assessment of dysarthrophonia in myasthenia gravis

Speech and voice symptomatology (dysarthrophonia) are often reported by patients with myasthenia gravis (MG). However, they have been poorly investigated despite their significant impact on quality of life. Quantitative methods for the assessment of dysarthrοphonia could facilitate the evaluation of these common MG symptoms. The goal of this study was to investigate the phonatory (sustained phonation and reading) and speech (diadochokinesis) function in MG patients using quantitative measures. The voice/speech of 12 MG patients (7 with anti-AchR and 5 with anti-MuSK antibodies) and 24 age-matched healthy controls was recorded and analyzed using electroglottography (EGG) and speech acoustics. For the analysis of voice, the variables that were found to distinguish MG patients compared to healthy controls were a higher average fundamental frequency (P<0.05), a higher standard deviation of the average fundamental frequency (P<0.001), a higher mean fundamental frequency of the vibrating vocal folds (P<0.005) and a higher fundamental frequency range (P<0.005). The analysis of diadochokinesis showed that MG patients had a higher mean duration of the silent interval between a series of repetitive /pa/ syllables (P<0.05), of the sound /t/ (P=0.05) and of the silent interval between a series of repetitive /ka/syllables (P<0.05). No statistical differences were found in any of these variables between the MG subgroups with anti-AchR or anti-MuSK antibodies. This study demonstrates that non-invasive physiological methods (EGG and speech acoustics) offer essential tools for the assessment of dysarthrophonia in MG patients.

A Normative Study of the Color Trails Test in the Greek Population

The Color Trails Test (CTT) is a neuropsychological test that measures perceptual tracking and sustained and divided attention. Recently, its use has been increased in neurological diseases such as Alzheimers disease, brain injury, and subclinical infarction. The aim of the present study was to provide normative data for the CTT in the Greek population. A total of 321 native Greek speakers with various professional backgrounds took part in the study. Exclusion criteria involved the existence of neurological and psychiatric diseases, history of alcohol and/or drug dependence, traumatic brain injury, and a Mini-Mental State Examination score below 27. Our results showed that age and education are important factors to consider for the interpretation of scores in all CTT variables. Older participants took more time and exhibited more errors, more near-misses, and more prompts compared with younger participants. Also, more-educated participants took less time, made fewer errors, had fewer near-misses, and rewired fewer prompts compared with less educated participants. CTT is a promising tool for the measurement of attention in the Greek population. Further research is needed to use this test in Greek patients with various neurological diseases.

A normative study of the Children’s Color Trails Test (CCTT) in the Cypriot population

Background: The Children’s Color Trails Test (CCTT) is a neuropsychological test that measures attention, divided attention, and speed of mental processing. It has been increasingly used in the assessment of children in cross-cultural environments for neurological and psychiatric disorders such as seizures and closed head injuries, learning and/or language disabilities, attention deficit/hyperactivity disorder, children with manganese exposure, and children diagnosed with HIV virus. However, there is a paucity of studies presenting normative data. The aim of the present study was to provide normative data for the CCTT in the Cypriot population. Methods: A total of 709 native Cypriot children aged 7–16 years, recruited from various public schools across the island, took part in the study. Exclusion criteria involved the existence of neurological, psychiatric, cardiological, and metabolic diseases, premature birth, history of maternal alcohol and drug abuse during pregnancy, low birth weight, hearing loss, visual problems, native language other than Greek, and abnormality in fine-motor movements. Results: Age and gender were found to be important factors for the interpretation of scores in all CCTT variables. Older children required less time and exhibited fewer errors, near misses, and prompts compared to younger children. There was a consistent pattern of a 3–4 seconds improvement (less time in seconds) in the CCTT completion time as age increased. Conclusions: CCTT is a promising tool for the measurement of attention in the native Cypriot population. Further research is needed in children diagnosed with various neurological and psychiatric diseases in order to estimate validity of the CCTT in clinical populations.

Open-Label Fosmetpantotenate, a Phosphopantothenate Replacement Therapy in a Single Patient with Atypical PKAN

Objective. Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disorder with variable onset, rate of progression, and phenotypic expression. Later-onset, more slowly progressive PKAN often presents with neuropsychiatric as well as motor manifestations that include speech difficulties, progressive dystonia, rigidity, and parkinsonism. PKAN is caused by biallelic PANK2 mutations, a gene that encodes pantothenate kinase 2, a regulatory enzyme in coenzyme A biosynthesis. Current therapeutic strategies rely on symptomatic relief. We describe the treatment of the first, later-onset PKAN patient with oral fosmetpantotenate (previously known as RE-024), a novel replacement therapy developed to bypass the enzymatic defect. Methods. This was an open-label, uncontrolled, 12-month treatment with fosmetpantotenate of a single patient with a later-onset, moderately severe, and slowly progressive form of PKAN. Results. The patient showed improvement in all clinical parameters including the Unified Parkinsons Disease Rating Scale (UPDRS), Barry-Albright Dystonia Scale, the EuroQol five-dimensional three-level (EQ-5D-3L) scale, timed 25-foot walk test, and electroglottographic speech analysis. Fosmetpantotenate was well-tolerated with only transient liver enzyme elevation which normalized after dose reduction and did not recur after subsequent dose increases. Conclusions. Fosmetpantotenate showed promising results in a single PKAN patient and should be further studied in controlled trials.

The Predictive Nature of Age and Gender in the Verbal Fluency Test in the Greek Cypriot Children: Normative Data

Objective: The Children’s Verbal Fluency test (VF) is a neuropsychological test that measures executive function, vocabulary storage, and speed of mental processing. It has been increasingly used in the assessment of children in neurological and psychiatric disorders such as Batten disease, frontal lobe epilepsy, cerebellar tumor, Attention-Deficit Hyperactivity Disorder (ADHD) and dyslexia. However, there is a paucity of studies presenting normative data. The aim of the present study was to provide normative data for the children’s verbal fluency test in the Cypriot population and to test the criterion validity of the children’s VF in children with ADHD. Method: A total of 749 native Cypriot children aged 7-16 years, recruited from various public schools across the island, took part in the study. Exclusion criteria involved the existence of neurological, psychiatric, cardiological and metabolic diseases and native language other than Greek. Results: Age but not gender was found to be an important factor for the interpretation of scores in the verbal fluency variables. Older children produced more words in both, semantic and phonemic fluency test compared to younger children. Also, the test seemed to discriminate the clinical group of children exhibiting ADHD in semantic verbal fluency as compared to pair-matched controls. Conclusions: Children’s verbal fluency test is a promising tool for the measurement of executive function in the Cypriot population. Further research is needed in children diagnosed with various neurological and psychiatric diseases in order to estimate the validity of the children’s verbal fluency in other clinical populations.

Effects of video modelling on emerging speech in an adult with traumatic brain injury: Preliminary findings

Abstract Primary objective: Research has shown that traumatic brain injury (TBI) can affect a person’s ability to perform previously learned skills. Dysexecutive syndrome and inattention, for example, alongside a number of other cognitive and behavioural impairments such as memory loss and lack of motivation, significantly affect day-to-day functioning following TBI. This study examined the efficacy of video modelling in emerging speech in an adult male with TBI caused by an assault. Research design: In an effort to identify functional relations between this novice intervention and the target behaviour, experimental control was achieved by using within-system research methodology, overcoming difficulties of forming groups for such an highly non-homogeneous population. Methods and procedures: Across a number of conditions, the participant watched a videotape in which another adult modelled a selection of 19 spoken words. When this modelled behaviour was performed in vivo, then generalization across 76 other words in the absence of a videotape took place. Main outcomes and results: It was revealed that video modelling can promote the performance of previously learned behaviours related to speech, but more significantly it can facilitate the generalization of this verbal behaviour across untrained words. Conclusions: Video modelling could well be added within the rehabilitation programmes for this population.