A-36 Case Study of Cerebellar Cognitive Affective Syndrome in 17-Year-Old Male with Cerebellar Arachnoid Cyst

2021 ◽  
Vol 36 (6) ◽  
pp. 1077-1077
Author(s):  
Laura Nicholson ◽  
Elizabeth Marston ◽  
Christopher Haak ◽  
Nicole Cruz

Abstract Objective Arachnoid cysts are often congenital and asymptomatic, however, they may contribute to neurocognitive symptoms when there is a mass effect on the brain. This case study describes how a 17-year-old with cerebellar arachnoid cyst, and minimal mass effect exhibited symptoms of cerebellar cognitive affective syndrome (CCAS), which has been mostly associated with brain tumor or injury in prior research. Method In this case study, a posterior cerebellar arachnoid cyst (midline) was identified incidentally (via CT scan and later MRI) following a panic attack associated with face twitching, motor tremors, and clumsiness. Background history was collected via medical records, neurologist consultation, and parent and patient interview. Patient history was significant for speech, language, motor, and academic delays. Results Results of a comprehensive evaluation revealed deficits in perceptual reasoning, visual memory, fine motor functioning, attention, processing speed, executive functioning, social cognition and receptive/expressive language. Multi-informant ratings indicated anxiety. Reading, writing, and mathematics fell several years below grade- level in the context of low average intellectual ability (WAIS-IV: GAI = 88). Conclusions Many deficits in this neuropsychological profile are typically subsumed by the cerebellum or its associated networks, and overlap with CCAS. The findings of this case study warrant consideration by clinicians of functional impairment related to cerebellar cysts and expansion of our knowledge-base of cerebellar function.

2019 ◽  
Vol 34 (6) ◽  
pp. 965-965
Author(s):  
P Figueroa ◽  
C Barbeito ◽  
S Ireland

Abstract Objective Mitochondrial Encephalopathy, Lactic acidosis, and Stroke-like episodes (MELAS) is a rare and debilitating mitochondrial disease that affects bodily systems at the neurological level. The purpose of this study is to contribute to the paucity of research regarding the neuropsychological presentation and functional impact of MELAS. Method Mr. James is a 23-year-old male of Thai descent who initially presented with 2-3 days of headaches followed by generalized seizures and altered mental status. Over the course of one year, Mr. James experienced recurrent seizures with postictal confusion, and transient aura-like episodes. Patient was diagnosed with MELAS based off a combination of clinical findings, including magnetic resonance imaging (MRI) spectroscopy (SPECT) and molecular genetic testing. Extensive evaluation revealed persistent bilateral multifocal cortical/subcortical lesions, diffuse edema like signal and expansion involving the bifrontal, bitemporal, and bilateral subinsular cortices. Neuropsychological sequalae included physical, cognitive, and emotional changes from baseline. Results Mr. James was given a comprehensive neuropsychological battery in order to identify marked changes from his pre-morbid levels of neuropsychological functioning. Consistent with the literature, his profile revealed hallmark expressive and receptive language difficulties and markedly reduced bilateral fine motor functioning. In addition, problems with higher order executive skills (i.e., cognitive flexibility, problem-solving, and hypothesis testing) were elucidated, correlating with individual findings on neuroimaging. Conclusions Neuropsychological evaluation of Mr. James contributes to the dearth of research surrounding MELAS. These findings also highlight the importance of neuropsychological testing as part of the comprehensive evaluation for diagnostic clarification, and provide recommendations of the functional impact for others affected with this disease.


2021 ◽  
Vol 36 (6) ◽  
pp. 1106-1106
Author(s):  
Melissa Grow ◽  
Stephanie Culotta

Abstract Objective This case study examined neurocognitive features associated with a rare genetic/neurological disorder impacting the cerebral cortex. Bilateral perisylvian polymicrogyria (BPP) is characterized by abnormal embryonic growth affecting both sides of the brain near the Sylvian fissure. BPP occurs randomly though researchers suspect an underlying genetic abnormality that may have autosomal recessive inheritance. Neurodevelopmental manifestations include neurocognition marked by EEG abnormalities, impaired motor function, and deficits in speech articulation, language development, learning and memory, and adaptive skills. Method A 13-year 7-month-old male with BPP, detected at 24 months by MRI, was seen for neuropsychological assessment to guide habilitative, academic and adaptive interventions. Relevant medical history includes stimulant medication and pharyngeal flap surgery. Parental concerns include the provision of appropriate special education services and withdrawal behaviors. Results Assessment revealed a complex neuropsychological profile characterized by nonverbal versus verbal intellectual strengths, receptive-expressive language impairment, underlying deficits in phonemic awareness, diminished fine motor speed, and diminished verbal fluency. Verbal list learning, semantic memory, and attention were intact. Mild elevations were evident for anxiety symptoms. Conclusion BPP, a rare genetic disorder, represents significant challenges for affected children. In this case study, a 13-year 7-month-old youngster’s assessment revealed underlying neurocognitive and neuromotor deficits most pronounced in aspects of language, speech articulation, select domains of executive functioning and academic skills. Areas of strength, including verbal memory and semantic recall, were indicated and merit consideration in developing appropriate interventions. This case study illustrates the value of neuropsychological assessment in guiding therapeutic interventions, pharmacotherapy, and appropriate educational services in this rare neurological disorder.


Author(s):  
Mikhail Bunin ◽  

The issues of methodological approaches toward and criteria of publication selection for rare and valuable book collections of sci-tech libraries are discussed. Based on the experience of CSAL, the need for comprehensive evaluation of books is substantiated: by materials and facilities, level of performance, physical condition and age, circulation, periodicity, and by value in scientific and sociocultural aspects.


Author(s):  
Haddad Gaelle ◽  
Alam Raquelle ◽  
Atweh Lamya Ann ◽  
Hourani Mukbil

2011 ◽  
Vol 17 (4) ◽  
pp. 449-456 ◽  
Author(s):  
AM Smerbeck ◽  
J Parrish ◽  
D Serafin ◽  
EA Yeh ◽  
B Weinstock-Guttman ◽  
...  

Background: Children with multiple sclerosis (MS) can suffer significant cognitive deficits. This study investigates the sensitivity and validity in pediatric MS of two visual processing tests borrowed from the adult literature, the Brief Visuospatial Memory Test-Revised (BVMTR) and the Symbol Digit Modalities Test (SDMT). Objective: To test the hypothesis that visual processing is disproportionately impacted in pediatric MS by comparing performance with that of healthy controls on the BVMTR and SDMT. Methods: We studied 88 participants (43 MS, 45 controls) using a neuropsychological assessment battery including measures of intelligence, language, visual memory, and processing speed. Patients and demographically matched controls were compared to determine which tests are most sensitive in pediatric MS. Results: Statistically significant differences were found between the MS and control groups on BVMTR Total Learning ( t (84) = 4.04, p < 0.001, d = 0.87), BVMTR Delayed Recall ( t (84) = 4.45, p < 0.001, d = 0.96), and SDMT ( t (38) = 2.19, p = 0.035, d = 0.69). No significant differences were found between groups on confrontation naming or general intellectual ability. Validity coefficients exploring correlation between BVMTR, SDMT, and disease characteristics were consistent with the adult literature. Conclusions: This study found that BVMTR and SDMT may be useful in assessing children and adolescents with MS.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cui Wang ◽  
Zhouhua Guo ◽  
Qingsheng Li ◽  
Jing Fang

AbstractIn order to protect the offshore environment and strengthen the comprehensive rectification of sewage outfalls, an evaluation method of regional sewage outfalls by combining the marine numerical simulation and comprehensive evaluation technology was constructed, considering the marine environmental capacity and the ecological impact of sewage discharge from outfalls on the marine eco-environment sensitive areas. Then the layout rationality of each outfall was evaluated and the discharge scale was optimized with a case study of existing sewage outfalls in Xiamen. The results show that, the comprehensive evaluation score of Yundang outfall was 3 points in 2025, evaluated as the outfall with irrational layout. In 2035, the comprehensive evaluation scores of Fenglin and Dalipu outfalls were 3 and 2 points respectively, evaluated as the outfall with irrational discharge scale. It is suggested to control the scale of expansion or increase the reclaimed water reuse rate in Jimei and Gaoqi Wastewater Treatment Plants. This method has enriched the evaluation system for layout optimization of sewage outfalls, providing scientific supports for comprehensive improvement of sewage outfalls and marine environmental management.


Author(s):  
Iveta Vrabková ◽  
Pavel Šaradín

Local Action Groups (LAGs) represent a dynamic platform for inter-municipal cooperation in Europe. Their principal advantages include EU funding and the capacity to generate economic returns and stimulate the development of local communities. The methodology used for the evaluation of the performance of LAGs is defined by the EU on the one hand and by national authorities on the other. Furthermore, there are an entire array of evaluation tools and academic experiments available. The present paper does not aim at a comprehensive evaluation of LAGs, but instead only examines the technical efficiency of LAGs. Using the Czech Republic as an example, the paper introduces an evaluation tool to measure the technical efficiency of LAGs and describes how it can be applied. The adoption of this tool is seen as a means of improving one of the parameters of the performance of LAGs.


2021 ◽  
Vol 2021 ◽  
pp. 1-12
Author(s):  
Lili Wan ◽  
Qiuping Peng ◽  
Tianci Zhang ◽  
Zhan Wang ◽  
Yong Tian

In order to clarify the comprehensive operational capabilities of the airport and better plan the sustainable development mode of the airport, this paper studies the evaluation method of airport environmental carrying capacity. First, this paper proposes the concept of airport environmental carrying capacity by taking into account the complex characteristics of airports affected by multiple factors and then selects 16 representative evaluation indicators to construct an indicator system based on the Driving Force-Pressure-State-Response (DPSR) framework. Finally, the accelerated genetic algorithm-projection pursuit model is established to model a comprehensive evaluation index, which is used to calculate the airport environmental carrying capacity (AECC). The results of the case study show that the AECC of Guangzhou Baiyun International Airport (CAN) decreased year by year from 2008 to 2017, which is in line with the coordinated development level of CAN. By analysing the changing mechanism of AECC and indicators, we get 6 key influencing indicators that led to the continuous decline of AECC and put forward some political suggestions to improve the AECC.


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