scholarly journals Cognitive functions in myoclonic epilepsy with ragged red fibres – a case report

2015 ◽  
Vol 23 (1) ◽  
pp. 69-74
Author(s):  
Martyna Domańska ◽  
Emilia J. Sitek ◽  
Michał Schinwelski ◽  
Maria Mazurkiewicz-Bełdzińska ◽  
Agnieszka Matheisel ◽  
...  

SUMMARY Introduction. Myoclonic epilepsy with ragged red fibers (MERRF) is a rare, progressive mitochondrial disease affecting multiple systems, including the central nervous system. Typical MERRF symptoms include: myoclonus, epileptic seizures, ataxia and cognitive decline. In mitochondrial diseases selective cognitive impairment or generalized decline, called mitochondrial dementia, is usually diagnosed. Description of case. We present the case of an 18-year-old patient with progressive neurological symptoms such as multifocal myoclonus, cerebellar syndrome (gait impairment, intention tremor, ataxia and dysmetria). The diagnosis of MERRF was confirmed at the age of 16. Neuropsychological examination showed slowing of verbal learning and deficient spontaneous recall with improvement on recognition as well as low verbal fluency. Discussion. The authors discuss differential diagnosis of mitochondrial diseases (MIDs) in respect to cognitive function impairment and, in particular, to dementia: MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes), KSS (Kearns-Sayre syndrome), NARP syndrome (neuropathy, ataxia, and retinitis pigmentosa and ptosis). The authors emphasize importance of comprehensive neuropsychological assessment in differential diagnosis of MIDs. Conclusion. Mild and selective cognitive impairment was identified. The type and degree of cognitive function impairment is not sufficient to diagnose dementia in this particular case of MERRF. Comprehensive neuropsychological assessment is crucial in MID in order to provide the patient with useful recommendations for education planning.

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Riwanti Estiasari ◽  
Yuhyi Fajrina ◽  
Diatri Nari Lastri ◽  
Syarli Melani ◽  
Kartika Maharani ◽  
...  

Introduction. Multiple Sclerosis (MS) can affect cognitive function that might interfere with quality of life. Processing speed and memory are the most common area of cognitive impairment. Cognitive evaluation in daily practice is often difficult to be performed since it needs neuropsychological expert and is time-consuming. Brief International Cognitive Assessment for MS (BICAMS) is valid and practical for cognitive evaluation. This study aims to validate BICAMS in Indonesian MS patients and healthy controls (HC) and to analyse the effect of cognitive impairment on quality of life. Methods. BICAMS, which composes Symbol Digits Modalities Test (SDMT), California Verbal Learning Test-Second Edition (CVLT-II), and Brief Visuospatial Memory Test-Revised (BVMT-R), was translated and cross-culturally adapted to Indonesian from the original BICAMS and then administered to 40 Indonesian MS patients and 66 HC matched by sex, age, and education. Test-retest reliability was performed on 16-MS patients and 42 HC. Quality of life was measured using Multiple Sclerosis Quality of Life (MSQOL-54) instrument. Results. The SDMT, CVLT-II, and BVMT-R score in MS patients were significantly lower than those in HC (effect size, r: 0.61, 0.36, and 0.47, respectively). Test-retest reliability for all tests was satisfactory with correlation coefficient for SDMT, CVLT-II, and BVMT-R in MS subjects 0.86, 0.81, and 0.83, respectively. Using 5th percentile of HC score as cut-off, 15% MS subjects had impairment in one test, 27.5% in two tests, and 40% in three tests. BICAMS was moderately correlated with EDSS but was not correlated with disease duration and relapse rate. SDMT score correlated with physical function and physical and mental role limitation. Conclusion. BICAMS is valid and reliable for assessing cognitive function of Indonesia MS patients.


2019 ◽  
Vol 7 (9) ◽  
pp. 1440-1445 ◽  
Author(s):  
Endy Juli Anto ◽  
Laura Oktavina Siagian ◽  
Jekson Martiar Siahaan ◽  
Hendrika Andriana Silitonga ◽  
Sony Eka Nugraha

BACKGROUND: Hypertension is still a health problem both in developed and developing countries. Hypertension can cause various complications; one of them is cognitive function impairment. AIM: This study aimed to look at the relationship of hypertension with cognitive function. This research can also be useful to help optimise the health of the elderly, maximise quality of life and avoid hypertension as a risk factor for cognitive impairment in the elderly at the Karya Kasih Nursing Homes, Medan from May to June 2018. METHODS: This research was carried out by analytic observational with cross-sectional research approach. In this study, 57 elderly from Karya Kasih Nursing Homes Medan who met the inclusion and exclusion criteria participated. Assessment of cognitive function used Mini-Mental State Examination (MMSE), Six Item Cognitive Impairment Test (6CIT) and Abbreviated Mental Test Score (AMT) instruments. RESULT: This study obtained a significant relationship between the history of hypertension with impaired cognitive function (p = 0.003). The results of the cognitive function examination with MMSE showed that among 57 elderly, 16 people (43.2%) were normal and 21 people (56.8%) had impaired cognitive function in the first degree hypertension group, besides that, 3 people were normal (15%) and 7 people (85%) had impaired cognitive function in the second degree hypertension group (p = 0.031). Based on the result of mild and severe cognitive function impairment, among 12 people (57.1%) and 9 people (42.9%) had a mild and severe cognitive function impairment, respectively, in first-degree hypertension. 3 people (17.6%) and 14 people (82.4%) had a mild and severe cognitive function impairment, respectively, in the second-degree hypertension (p = 0.013). The 6-CIT instrument also showed a significant relationship between the severity of hypertension and impaired cognitive function (p = 0.027), and there was no significant relationship with AMT instruments (p = 0.078). CONCLUSION: There was a relationship between the history or duration and degree of hypertension with cognitive dysfunction in the elderly at the Karya Kasih Nursing Home Medan.


2019 ◽  
Vol 75 (6) ◽  
pp. 1214-1221 ◽  
Author(s):  
Pei-Dong Zhang ◽  
Yue-Bin Lv ◽  
Zhi-Hao Li ◽  
Zhao-Xue Yin ◽  
Fu-Rong Li ◽  
...  

Abstract Background The aim of this study was to examine the trends in impairment regarding activities of daily living (ADL), physical performance, and cognitive function among the oldest-old (those aged 80 and older) in China between 1998 and 2014. Methods We used data on 34,297 oldest-old individuals from the seven waves of the Chinese Longitudinal Healthy Longevity Study. We estimated age, period, and cohort effects on the prevalence of self-reported ADL impairment, tested physical performance and cognitive function impairment using the age–period–cohort model. Results Regarding age, the prevalence of ADL, physical performance, and cognitive function impairment were highest in the centenarians, but they did not increase with age in this population. Among the literate subgroup, the prevalence of cognitive impairment increased more rapidly with age than that in the illiterate subgroup. Regarding period, the prevalence of self-reported and tested physical impairment slowly increased between 1998 and 2014, but cognitive impairment remained stable. Regarding cohort, ADL impairment continuously decreased. However, physical and cognitive impairment remained stable after a brief decline in the early birth cohorts. Conclusions The results suggest that the age effect is still the most obvious effect regarding several types of functional impairment. The likelihood of a younger person experiencing functional impairment may not change significantly, but ADL is likely to be amenable to improvement resulting from improved medical and social care. Therefore, increased care for the oldest-old may considerably improve their quality of life, particularly regarding their basic ADL.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Mick Kumwenda ◽  
Peter Hobson

Abstract Background and Aims Chronic kidney disease (CKD) is a global healthcare problem associated with several comorbid conditions including, cardiovascular disease, and diabetes, all of which are strong risk factors for the development of significant cognitive impairment. We recently reported the baseline outcomes of our older adult cohort with mild to moderate CKD (stages 3-4) and diabetes, where 48% were found to have a cognitive impairment ranging from mild to severe symptoms. [1] This study reports the outcomes at 36-months post baseline of the prevalence and incidence of cognitive impairment in this cohort the patients who at baseline were considered to have normal cognitive function. Method Cognitive function was assessed in patients over aged 55 years, with an estimated glomerular filtration rate < 45ml/min/1.73m 2, attending a renal and diabetes outpatient clinic, at baseline and at 36-months. The diagnosis of cognitive impairment was based upon patient and informant interview, case note review, neuropsychological assessment and application of Diagnostic and Statistical Manual of Mental disorders version 5 (DSM-5) and Petersen’s criteria for mild cognitive impairment (MCI). The incidence of cognitive impairment was calculated by dividing the number of new cases during study follow-up, by the person-time at risk throughout the observation period. Since it is not possible to precisely determine when a person actually develops cognitive impairment between baseline and follow-up, the midpoint of time between having normal cognition and becoming a case is assumed. Results Ninety-two patients without cognitive impairment at baseline, were included in this investigation (mean age of 75.8 + 9.1; 49 males: 43 female). Upon neuropsychological assessment and the application of DSM-5 criteria at follow-up, it was revealed that 25/92 (27%) of the cohort had developed a cognitive impairment ranging from MCI (n=19), to major symptoms (n = 6). The crude prevalence for MCI was 20.6%, and for dementia it was 6.5%. The total person years in the study were 237.38 years, with an overall incidence rate of 10.53 (95% Confidence interval 6.82-15.55) per 100 person-years. Conclusion This longitudinal cohort investigation reports the prevalence and incidence of new cases of cognitive impairment ranging from MCI to dementia in CKD. The crude prevalence in the current investigation for the development of cognitive impairment was 27%. The overall incidence rate for new cases of NCD was just over 10%. In other words, for every 100 patients in our current investigation we would estimate that around 10 will develop some a cognitive impairment ranging from MCI to dementia per year. Our findings from this and our previous investigation suggest that a significant number of CKD patients are at risk for the development of significant neurodegenerative conditions and in view of this cognitive function should be screened and monitored routinely in clinical practice. This will assist with appropriate health service planning, service development, clinical interventions and the assessment of the effectiveness of new and existing treatments services.


2016 ◽  
Vol 15 (4) ◽  
pp. 424-434 ◽  
Author(s):  
Yingchun Zeng ◽  
Andy S. K. Cheng ◽  
Chetwyn C. H. Chan

Background. Cognitive impairment is a common complaint among cancer survivors, significantly impacting working memory, attention, executive function, and information processing speed. This meta-analysis aims to evaluate the effect of neuropsychological interventions on the cognitive function of non–central nervous system (non-CNS) cancer survivors. Methods. Three databases (PubMed, PsycInfo, and CAJ Full-text Database) were searched from January 2010 to September 2015. Controlled clinical trials of neuropsychological interventions for the treatment of cognitive impairment in cancer survivors were considered for inclusion. Results. A total of 10 eligible trials were included in this meta-analysis. Three trials assessed the effects of cognitive rehabilitation (CR) interventions, and the weighted mean difference (WMD) for the overall intervention effect was −0.19 (95% confidence interval [CI] = −2.98 to 2.61). Two trials examined the effects of cognitive training (CT) interventions on the cognitive function of cancer survivors; the standardized mean difference (SMD) for the overall effect was 0.52 (95% CI = 0.06 to 0.98). The overall effect of CR interventions on neuropsychological status at postintervention was 5.66 (95% CI = 2.97 to 8.35). The SMD of CR and CT intervention for objective function by verbal learning tests was 0.50 (95% CI = 0.19 to 0.81) at postintervention, and 0.58 (95% CI = 0.19-0.98) at follow-up assessment within 6 months. Conclusion. Findings from this meta-analysis indicate that neuropsychological interventions can improve cognitive function in non-CNS cancer survivors, and support the need for future research. However, the conclusion from this meta-analysis was based on trials with small sample sizes. Future research should be conducted using a larger sample size. Relevant clinical implications were discussed accordingly.


2010 ◽  
Vol 197 (2) ◽  
pp. 135-140 ◽  
Author(s):  
Jane A. Lonie ◽  
Mario A. Parra-Rodriguez ◽  
Kevin M. Tierney ◽  
Lucie L. Herrmann ◽  
Claire Donaghey ◽  
...  

BackgroundCognitive impairment precedes the diagnosis of Alzheimer's disease. It is unclear which psychometric measures predict dementia, and what cut-off points should be used. Replicable cognitive measures to provide information about differential diagnosis and prognosis would be clinically useful.AimsIn a prospective cohort study we investigated which measures distinguish between individuals with amnestic mild cognitive impairment (aMCI) that converts to dementia and those whose impairment does not, and which combination of measures best predicts the fate of people with aMCI.MethodForty-four participants with aMCI underwent extensive neuropsychological assessment at baseline and annually thereafter for an average of 4 years. Differences in baseline cognitive performance of participants who were converters and non-converters to clinically diagnosed dementia were analysed. Classification accuracy was estimated by sensitivity, specificity, positive and negative predictive values and using logistic regression.ResultsForty-one percent of participants had progressed to dementia by the end of study, with a mean annual conversion rate of 11%. Most (63%) showed persisting or progressive cognitive impairment, irrespective of diagnosis. The Addenbrooke's Cognitive Examination together with the discrimination index of the Hopkins Verbal Learning Test – Revised (but none of the demographic indices) differentiated the participants who were converters from the non-converters at baseline with 74% accuracy.ConclusionsTargeted neuropsychological assessment, beyond simple cognitive screening, could be used in clinical practice to provide individuals with aMCI with prognostic information and aid selective early initiation of monitoring and treatment among those who progress towards a clinically diagnosable dementia.


2002 ◽  
Vol 8 (7) ◽  
pp. 934-942 ◽  
Author(s):  
KEJAL KANTARCI ◽  
GLENN E. SMITH ◽  
ROBERT J. IVNIK ◽  
RONALD C. PETERSEN ◽  
BRADLEY F. BOEVE ◽  
...  

The aim of this study was to examine the associations of apolipoprotein E (APOE) genotype, metabolic changes in the posterior cingulate detected by 1H magnetic resonance spectroscopy (MRS), and neuropsychologic measures of memory and cognition both in normally aging elderly, and in patients with mild cognitive impairment (MCI) and AD. We studied 67 controls, 18 MCI and 33 AD patients. We used the Dementia Rating Scale total score (DRSTOT) as a measure of general cognitive function and the total learning from the Auditory Verbal Learning Test (AVTOT) as a measure of memory performance. No differences were noted on 1H-MRS metabolite ratios or cognitive measures across APOE genotype within control and patient groups. In controls, age was a significant predictor of both cognitive test scores, and NAA/Cr was a univariate associate of DRSTOT. All 3 1H-MRS metabolite ratios, N-acetylaspartate (NAA)/creatine (Cr), myoinositol (MI)/Cr, and NAA/MI, were univariate associates of AVTOT and DRSTOT scores in the combined MCI and AD group. In stepwise regression analyses in the combined patient group only NAA/MI entered the models. These data suggest NAA/Cr could be a modest predictor of general cognitive function in both healthy elderly and impaired patients, while MI/Cr is a more specific marker for neuropsychologic dysfunction associated with neurodegenerative disease. Among 1H-MRS measurements, the NAA/MI ratio maybe the most efficient predictor of memory and cognitive function in patients with MCI and AD. (JINS, 2002, 8, 934–942.)


2009 ◽  
Vol 3 (3) ◽  
pp. 214-221 ◽  
Author(s):  
Érica Maria Lima Pimentel

Abstract The prevalence of dementia increases significantly from the age of 65 years, doubling every five years thereafter. Alzheimer's disease (AD) and vascular dementia (VaD) constitute the two main dementia types. Differentiating them encompasses anamnesis, neurological examination, laboratory and neuroimaging exams and neuropsychological assessment. Neuropsychological assessment produces different findings for each dementia type, and reveals those areas most impaired as well as those most preserved. The aim of the present article was to describe the role of neuropsychology in diagnosing dementia and achieving a differential diagnosis between AD and VaD. A general overview follows of the most widely known instruments used to assess cognitive function in dementia, and the cognitive changes seen in AD and VaD. The conclusion drawn was that there is significant overlap in cognitive changes between both these dementia types, while each type has its own specific characteristics which are identifiable and quantifiable on neuropsychological assessments and provide the basis for reaching a differential diagnosis.


2021 ◽  
Vol 13 ◽  
Author(s):  
Luobing Wu ◽  
Caihong Wang ◽  
Jingchun Liu ◽  
Jun Guo ◽  
Ying Wei ◽  
...  

Recent neuroimaging studies have shown the possibility of cognitive impairment after pontine stroke. In this study, we aimed to use voxel-mirrored homotopic connectivity (VMHC) to investigate changes in the cognitive function in chronic pontine stroke. Functional MRI (fMRI) and behavioral assessments of cognitive function were obtained from 56 patients with chronic pontine ischemic stroke [28 patients with left-sided pontine stroke (LP) and 28 patients with right-sided pontine stroke (RP)] and 35 matched healthy controls (HC). The one-way ANOVA test was performed for the three groups after the VMHC analysis. Results showed that there were significant decreases in the bilateral lingual gyrus (Lingual_L and Lingual_R) and the left precuneus (Precuneus_L) in patients with chronic pontine ischemic stroke compared to HCs. However, in a post-hoc multiple comparison test, this difference remained only between the HC and RP groups. Moreover, we explored the relationship between the decreased z-values in VMHC and the behavior-task scores using a Pearson's correlation test and found that both scores of short-term memory and long-term memory in the Rey Auditory Verbal Learning Test were positively correlated with z-values of the left lingual gyrus (Lingual_L), the right lingual gyrus (Lingual_R), and the left precuneus (Precuneus_L) in VMHC. Besides that, the z-values of Precuneus_L in VMHC were also negatively correlated with the reaction time for correct responses in the Flanker task and the spatial memory task. In conclusion, first, the lingual gyrus played an important role in verbal memory. Second, the precuneus influenced the working memory, both auditory-verbal memory and visual memory. Third, the right-sided stroke played a greater role in the results of this study. This study provides a basis for further elucidation of the characteristics and mechanisms of cognitive impairment after pontine stroke.


e-CliniC ◽  
2016 ◽  
Vol 4 (1) ◽  
Author(s):  
Nurindah Athika ◽  
Junita Maja PS ◽  
Arthur H.P. Mawuntu

Abstract: Traumatic Brain Injury (TBI) is the most common case in hospital. TBI can caused cognitive impairment. This study aimed to obtain the description of cognitive function in patients with mild and moderate TBI that were admitted to Prof. Dr. R. D. Kandou Hospital Manado. This was a prospective descriptive study by conducting direct examination to the patients diagnosed with mild or moderate TBI by using MMSE and Ina MoCA instruments. Thee results showed that there of 50 subjects there were 74% with mild TBI and 26% with moderate TBI. MMSE showed 96% normal while Ina MoCA showed 76% normal. Cognitive function impairment was more visible on Ina MoCA examination. Conclusion: Ina MoCA was better than MMSE examination in description of cognitive function impairment.Keywords: head injury, cognitive function Abstrak: Cedera kepala merupakan suatu kegawatan yang paling sering dijumpai di Rumah Sakit. Cedera kepala dapat menyebabkan gangguan fungsi kognitif. Penelitian ini bertujuan untuk mengetahui gambaran skor MMSE dan MoCA-Ina pada pasien cedera kepala ringan dan sedang yang dirawat di RSUP Prof. Dr. R. D. Kandou Manado. Jenis penelitian ini ialah deskriptif prospektif dengan melakukan pemeriksaan langsung pada pasien yang didiagnosis cedera kepala ringan atau sedang menggunakan instrumen MMSE dan MoCA-Ina. Hasil penelitian mendapatkan subjek sebanyak 50 orang dengan persentase cedera kepala ringan sebanyak 74% dan cedera kepala sedang 26%. Pada MMSE didapatkan 96% normal sedangkan pada MoCA-Ina didapatkan 76% normal. Penurunan fungsi kognitif lebih terlihat pada pemeriksaan MoCA-Ina. Simpulan: MoCA-Ina lebih dapat menggambarkan gangguan fungsi kognitif daripada pemeriksaan MMSE. Kata kunci: cedera kepala, fungsi kognitif


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