Objective Functional neurologic disorder (FND) is a broad diagnostic construct that encompasses m... more Objective Functional neurologic disorder (FND) is a broad diagnostic construct that encompasses many heterogeneous conditions. The defining characteristic of FND is that the affected individual experiences symptoms for which there is no known biological mechanism. Often the symptoms are attributed to psychological factors. FND remains poorly understood, despite increased efforts to elucidate neural and neuropsychological correlates. To date, there have been only a few case studies published on acute onset of dysfluent writing that does not result in dysgraphia and is not associated with a major neurological event (i.e., stroke, or traumatic brain injury). Methods A 17-year-old, right-handed, White, cis-gender boy was referred to an outpatient neuropsychology private practice with a complaint of acute onset and progressive dysfluent and effortful writing without dysgraphia. A comprehensive neuropsychological evaluation was undertaken, and the patient’s mother served as a collateral i...
Objective The non-fluent/agrammatic variant of primary progressive aphasia (naPPA) is a neurodege... more Objective The non-fluent/agrammatic variant of primary progressive aphasia (naPPA) is a neurodegenerative syndrome associated with agrammatism in language production and effortful speech (Gorno-Tempini et al., 2011). There is limited literature on PPA in non-English speakers and few clinical cases of bilingual naPPA have been described (Tee et al., 2022; Zanini et al., 2011). Characteristics of symptoms in orthographic languages, such as Chinese, are not well understood (Ting et al., 2017). This case describes the assessment of naPPA in a bilingual Mandarin Chinese speaker. Method A 66-year-old Asian male with 16 years of education complained of worsening aphasia in both languages over two years with initial preferential sparing of Chinese. A comprehensive neuropsychological battery was administered mostly in Chinese with select subtests in English. Language functioning was assessed with the Bilingual Aphasia Screening Test (Paradis & Libben, 1987). Results Agrammatism and apraxia o...
Background: Cognitive impairment is widely considered the main cause of disability and handicap a... more Background: Cognitive impairment is widely considered the main cause of disability and handicap after subarachnoid hemorrhage (SAH). The impact of depression on recovery after SAH remains poorly defined. We sought to determine the frequency of post-SAH depression, identify risk factors for its development, and evaluate the impact of depression on quality of life (QOL) during the first year of recovery. Methods: We prospectively studied 216 of 534 SAH patients treated between July 1996 and December 2001 with complete one-year follow-up data. Depression was evaluated with the Center for Epidemiological Studies Depression (CES-D) scale, cognitive status with the Telephone Interview for Cognitive Status (TICS), and QOL with the Sickness Impact Profile (SIP) 3 and 12 months after SAH. Results: Depressed mood occurred in 47% of patients during the first year of recovery; 26% were depressed at both 3 and 12 months. Non-white ethnicity predicted early (3 month) and late (12 month) depressions; early depression was also predicted by previously-diagnosed depression, cigarette smoking, and cerebral infarction, whereas late depression was predicted by prior social isolation and lack of medical insurance. Depression was associated with inferior QOL in all domains of the SIP, and changes in depression status were associated with striking parallel changes in QOL, disability, and cognitive function during the first year of recovery. CES-D scores accounted for over 60% of the explained variance in SIP total scores, whereas TICS performance accounted for no more than 6%. Conclusion: Depression affects nearly half of SAH patients during the first year of recovery, and is associated with poor QOL. Systematic screening and early treatment for depression are promising strategies for improving outcome after SAH.
Studies of saccadic suppression and induced motion have suggested separate representations of vi-... more Studies of saccadic suppression and induced motion have suggested separate representations of vi-sual space for perception and visually guided behavior. Because these methods required stimulus mo-tion, subjects might have confounded motion and position. Weseparated cognitive and sensorimotor maps without motion of target, background, or eye, with an "induced Roelofs effect": a target inside an off-center frame appears biased opposite the direction of the frame. A frame displayed to the left of a subject's center line, for example, will make a target inside the frame appear farther to the right than its actual position. The effect always influences perception, but in half of our subjects it did not influ-ence pointing. Cognitive and sensorimotor maps interacted when the motor response was delayed; all subjects now showed a Roelofs effect for pointing, suggesting that the motor system was being fed from the biased cognitive map. A second experiment showed similar result...
This study examined the effect of demographic and clinical variables on recovery of neuropsycholo... more This study examined the effect of demographic and clinical variables on recovery of neuropsychological functioning after SAH. Medical condition and hospital course, including disease severity and complications, were recorded prospectively. Demographics including age, sex, race/ethnicity, primary language, and years of education were obtained via interview. Three and 12 months after the SAH, participants were invited to complete a comprehensive neuropsychological battery in the patient’s preferred language, English or Spanish. Overall cognition and 7 cognitive domains were assessed: attention, psychomotor speed, visuospatial skills, language, verbal and visual memory, and executive functions. Psychomotor speed and attention recovered over time. Global mental status 3 months after SAH was the best correlate of neuropsychological functioning at one year. Factors that contributed to greater cognitive impairment after SAH included older age, anterior aneurysm location, amount and locatio...
Objective: In cases of insult to the left temporal lobe, particularly during periods of early dev... more Objective: In cases of insult to the left temporal lobe, particularly during periods of early development, language may lateralize to the right hemisphere, thus crowding out visual function. This is referred to as the "crowding out" hypothesis (Strauss, E., Satz, P., & Wada, J., 1990). However, comprehensive neuropsychological evidence of the hypothesis has not been presented. This study provides evidence of the "crowding out" phenomenon in a child six years status-post tumor resection. Method: An 8-year-old right-handed White female presented with academic concerns. In infancy, she was diagnosed with a left temporal primary central nervous system neoplasm (most consistent with desmoplastic infantile astrocytoma) and underwent a gross total resection at 31 months with subsequent chemotherapy. Pre-operative magnetic resonance imaging (MRI) revealed a cystic mass lesion of the medial left temporal lobe. The 4-year post-operative MRI revealed no evidence of tumor recurrence. A comprehensive neuropsychological battery was administered. Results: Strengths in verbal functioning, learning, and recall were demonstrated (Verbal Comprehension Index = 95th percentile). Average performance on visuospatial tasks that allow for verbal mediation strategies was demonstrated; however, performance was impaired on perceptual reasoning and visual memory tasks in which verbal mediation was not possible (Visual Spatial Index = 23rd percentile). Conclusions: Neuropsychological performance was consistent with strong verbal skills, with deficits in pure perceptual reasoning. Considering the utility of language, language functions may have extended to the right hemisphere. These deficits suggest the presence of "crowding out", as the right lobe appears to be compensating some visual function for verbal. References:
Objective: This case characterizes the longitudinal neuropsychological outcome of pediatric cereb... more Objective: This case characterizes the longitudinal neuropsychological outcome of pediatric cerebellar cognitive affective syndrome (CCAS), prior to and status-post cerebellar tumor resection, at three separate time points between ages 7 and 11. Method: A Chinese-American, bilingual girl underwent gross resection of cerebellar vermis astrocytoma at age seven. Initial magnetic resonance imaging (MRI) revealed left-to-right midline shift with partial effacement of the left lateral ventricle. A comprehensive neuropsychological battery was administered prior to resection, and at ages 9 and 11. An average Z score was calculated for each neuropsychological domain. Results: Across all sessions, the child showed blunted affect and impaired verbal initiation. The pre-operative evaluation revealed expressive and receptive language difficulties (Z = −1.70 and −1.00, respectively) and slow processing speed (Z = −1.09). Post-operative evaluation two years later revealed persistent difficulties with expressive language (Z = −1.06) and processing speed (Z = −1.22), as well as lagging social skills (Z = −0.91) and executive functions (Z = −2.23), with improved receptive language (Z = −0.25). Testing four years post-resection revealed persistent deficits of expressive language (Z = −1.78), processing speed (−1.00), and social communication with improved receptive language (Z = 0.16) and executive functioning (Z = −0.29). Conclusions: Serial neuropsychological evaluations revealed persistent deficits of expressive language, processing speed, and social skills, with improved receptive language. The child "grew into her deficits" of executive functioning prior to improving. Although affective disturbance and expressive language difficulties were seen across all evaluations, this case demonstrates gradual resolution of symptoms of CCAS.
Performance validity testing in children undergoing neuropsychological testing is a growing resea... more Performance validity testing in children undergoing neuropsychological testing is a growing research area. Accurate identification of performance validity is necessary to avoid invalid assessment conclusions. In the present research, a forced choice (FC) trial was created for the California Verbal Learning Test - Children's Edition (CVLT-C), modeled after the established California Verbal Learning Test - Second Edition (CVLT-II) FC trial. Distractor words were taken directly from the CVLT-II FC Standard Form, with about half being concrete (n = 8) and half abstract (n = 7). The order of the items was organized similarly to the CVLT-II FC to ensure that items from within the same category were not sequential. The Test of Memory Malingering was administered for comparison, and three embedded measures that have previously been validated in adults were also calculated. The CVLT-C FC trial was administered to 40 children, aged 6-16 (M = 12.08, SD = 3.13). Average full scale IQ was 97...
Background — Cognitive dysfunction is a common and disabling sequela of subarachnoid hemorrhage (... more Background — Cognitive dysfunction is a common and disabling sequela of subarachnoid hemorrhage (SAH). Although several clinical and radiographic findings have been implicated in the pathogenesis of cognitive dysfunction after SAH, few prospective studies have comprehensively and simultaneously evaluated these risk factors. Methods — Between July 1996 and March 2000, we prospectively evaluated 113 of 248 consecutively admitted nontraumatic SAH patients alive at 3 months with a comprehensive neuropsychological evaluation. Summary scores for 8 cognitive domains were calculated to express test performance relative to the entire study population. Clinical and radiographic variables associated with domain-specific cognitive dysfunction were identified with forward stepwise multiple regression, with control for the influence of demographic factors. Results — The study participants were younger ( P =0.005), less often white ( P =0.006), and had better 3-month modified Rankin scores ( P =0....
Proceedings of the National Academy of Sciences, 2004
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a trip... more Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a triplet (CAG) expansion mutation. The length of the triplet repeat is the most important factor in determining age of onset of HD, although substantial variability remains after controlling for repeat length. The Venezuelan HD kindreds encompass 18,149 individuals spanning 10 generations, 15,409 of whom are living. Of the 4,384 immortalized lymphocyte lines collected, 3,989 DNAs were genotyped for their HD alleles, representing a subset of the population at greatest genetic risk. There are 938 heterozygotes, 80 people with variably penetrant alleles, and 18 homozygotes. Analysis of the 83 kindreds that comprise the Venezuelan HD kindreds demonstrates that residual variability in age of onset has both genetic and environmental components. We created a residual age of onset phenotype from a regression analysis of the log of age of onset on repeat length. Familial correlations (correlation ± SE...
Objective Functional neurologic disorder (FND) is a broad diagnostic construct that encompasses m... more Objective Functional neurologic disorder (FND) is a broad diagnostic construct that encompasses many heterogeneous conditions. The defining characteristic of FND is that the affected individual experiences symptoms for which there is no known biological mechanism. Often the symptoms are attributed to psychological factors. FND remains poorly understood, despite increased efforts to elucidate neural and neuropsychological correlates. To date, there have been only a few case studies published on acute onset of dysfluent writing that does not result in dysgraphia and is not associated with a major neurological event (i.e., stroke, or traumatic brain injury). Methods A 17-year-old, right-handed, White, cis-gender boy was referred to an outpatient neuropsychology private practice with a complaint of acute onset and progressive dysfluent and effortful writing without dysgraphia. A comprehensive neuropsychological evaluation was undertaken, and the patient’s mother served as a collateral i...
Objective The non-fluent/agrammatic variant of primary progressive aphasia (naPPA) is a neurodege... more Objective The non-fluent/agrammatic variant of primary progressive aphasia (naPPA) is a neurodegenerative syndrome associated with agrammatism in language production and effortful speech (Gorno-Tempini et al., 2011). There is limited literature on PPA in non-English speakers and few clinical cases of bilingual naPPA have been described (Tee et al., 2022; Zanini et al., 2011). Characteristics of symptoms in orthographic languages, such as Chinese, are not well understood (Ting et al., 2017). This case describes the assessment of naPPA in a bilingual Mandarin Chinese speaker. Method A 66-year-old Asian male with 16 years of education complained of worsening aphasia in both languages over two years with initial preferential sparing of Chinese. A comprehensive neuropsychological battery was administered mostly in Chinese with select subtests in English. Language functioning was assessed with the Bilingual Aphasia Screening Test (Paradis & Libben, 1987). Results Agrammatism and apraxia o...
Background: Cognitive impairment is widely considered the main cause of disability and handicap a... more Background: Cognitive impairment is widely considered the main cause of disability and handicap after subarachnoid hemorrhage (SAH). The impact of depression on recovery after SAH remains poorly defined. We sought to determine the frequency of post-SAH depression, identify risk factors for its development, and evaluate the impact of depression on quality of life (QOL) during the first year of recovery. Methods: We prospectively studied 216 of 534 SAH patients treated between July 1996 and December 2001 with complete one-year follow-up data. Depression was evaluated with the Center for Epidemiological Studies Depression (CES-D) scale, cognitive status with the Telephone Interview for Cognitive Status (TICS), and QOL with the Sickness Impact Profile (SIP) 3 and 12 months after SAH. Results: Depressed mood occurred in 47% of patients during the first year of recovery; 26% were depressed at both 3 and 12 months. Non-white ethnicity predicted early (3 month) and late (12 month) depressions; early depression was also predicted by previously-diagnosed depression, cigarette smoking, and cerebral infarction, whereas late depression was predicted by prior social isolation and lack of medical insurance. Depression was associated with inferior QOL in all domains of the SIP, and changes in depression status were associated with striking parallel changes in QOL, disability, and cognitive function during the first year of recovery. CES-D scores accounted for over 60% of the explained variance in SIP total scores, whereas TICS performance accounted for no more than 6%. Conclusion: Depression affects nearly half of SAH patients during the first year of recovery, and is associated with poor QOL. Systematic screening and early treatment for depression are promising strategies for improving outcome after SAH.
Studies of saccadic suppression and induced motion have suggested separate representations of vi-... more Studies of saccadic suppression and induced motion have suggested separate representations of vi-sual space for perception and visually guided behavior. Because these methods required stimulus mo-tion, subjects might have confounded motion and position. Weseparated cognitive and sensorimotor maps without motion of target, background, or eye, with an "induced Roelofs effect": a target inside an off-center frame appears biased opposite the direction of the frame. A frame displayed to the left of a subject's center line, for example, will make a target inside the frame appear farther to the right than its actual position. The effect always influences perception, but in half of our subjects it did not influ-ence pointing. Cognitive and sensorimotor maps interacted when the motor response was delayed; all subjects now showed a Roelofs effect for pointing, suggesting that the motor system was being fed from the biased cognitive map. A second experiment showed similar result...
This study examined the effect of demographic and clinical variables on recovery of neuropsycholo... more This study examined the effect of demographic and clinical variables on recovery of neuropsychological functioning after SAH. Medical condition and hospital course, including disease severity and complications, were recorded prospectively. Demographics including age, sex, race/ethnicity, primary language, and years of education were obtained via interview. Three and 12 months after the SAH, participants were invited to complete a comprehensive neuropsychological battery in the patient’s preferred language, English or Spanish. Overall cognition and 7 cognitive domains were assessed: attention, psychomotor speed, visuospatial skills, language, verbal and visual memory, and executive functions. Psychomotor speed and attention recovered over time. Global mental status 3 months after SAH was the best correlate of neuropsychological functioning at one year. Factors that contributed to greater cognitive impairment after SAH included older age, anterior aneurysm location, amount and locatio...
Objective: In cases of insult to the left temporal lobe, particularly during periods of early dev... more Objective: In cases of insult to the left temporal lobe, particularly during periods of early development, language may lateralize to the right hemisphere, thus crowding out visual function. This is referred to as the "crowding out" hypothesis (Strauss, E., Satz, P., & Wada, J., 1990). However, comprehensive neuropsychological evidence of the hypothesis has not been presented. This study provides evidence of the "crowding out" phenomenon in a child six years status-post tumor resection. Method: An 8-year-old right-handed White female presented with academic concerns. In infancy, she was diagnosed with a left temporal primary central nervous system neoplasm (most consistent with desmoplastic infantile astrocytoma) and underwent a gross total resection at 31 months with subsequent chemotherapy. Pre-operative magnetic resonance imaging (MRI) revealed a cystic mass lesion of the medial left temporal lobe. The 4-year post-operative MRI revealed no evidence of tumor recurrence. A comprehensive neuropsychological battery was administered. Results: Strengths in verbal functioning, learning, and recall were demonstrated (Verbal Comprehension Index = 95th percentile). Average performance on visuospatial tasks that allow for verbal mediation strategies was demonstrated; however, performance was impaired on perceptual reasoning and visual memory tasks in which verbal mediation was not possible (Visual Spatial Index = 23rd percentile). Conclusions: Neuropsychological performance was consistent with strong verbal skills, with deficits in pure perceptual reasoning. Considering the utility of language, language functions may have extended to the right hemisphere. These deficits suggest the presence of "crowding out", as the right lobe appears to be compensating some visual function for verbal. References:
Objective: This case characterizes the longitudinal neuropsychological outcome of pediatric cereb... more Objective: This case characterizes the longitudinal neuropsychological outcome of pediatric cerebellar cognitive affective syndrome (CCAS), prior to and status-post cerebellar tumor resection, at three separate time points between ages 7 and 11. Method: A Chinese-American, bilingual girl underwent gross resection of cerebellar vermis astrocytoma at age seven. Initial magnetic resonance imaging (MRI) revealed left-to-right midline shift with partial effacement of the left lateral ventricle. A comprehensive neuropsychological battery was administered prior to resection, and at ages 9 and 11. An average Z score was calculated for each neuropsychological domain. Results: Across all sessions, the child showed blunted affect and impaired verbal initiation. The pre-operative evaluation revealed expressive and receptive language difficulties (Z = −1.70 and −1.00, respectively) and slow processing speed (Z = −1.09). Post-operative evaluation two years later revealed persistent difficulties with expressive language (Z = −1.06) and processing speed (Z = −1.22), as well as lagging social skills (Z = −0.91) and executive functions (Z = −2.23), with improved receptive language (Z = −0.25). Testing four years post-resection revealed persistent deficits of expressive language (Z = −1.78), processing speed (−1.00), and social communication with improved receptive language (Z = 0.16) and executive functioning (Z = −0.29). Conclusions: Serial neuropsychological evaluations revealed persistent deficits of expressive language, processing speed, and social skills, with improved receptive language. The child "grew into her deficits" of executive functioning prior to improving. Although affective disturbance and expressive language difficulties were seen across all evaluations, this case demonstrates gradual resolution of symptoms of CCAS.
Performance validity testing in children undergoing neuropsychological testing is a growing resea... more Performance validity testing in children undergoing neuropsychological testing is a growing research area. Accurate identification of performance validity is necessary to avoid invalid assessment conclusions. In the present research, a forced choice (FC) trial was created for the California Verbal Learning Test - Children's Edition (CVLT-C), modeled after the established California Verbal Learning Test - Second Edition (CVLT-II) FC trial. Distractor words were taken directly from the CVLT-II FC Standard Form, with about half being concrete (n = 8) and half abstract (n = 7). The order of the items was organized similarly to the CVLT-II FC to ensure that items from within the same category were not sequential. The Test of Memory Malingering was administered for comparison, and three embedded measures that have previously been validated in adults were also calculated. The CVLT-C FC trial was administered to 40 children, aged 6-16 (M = 12.08, SD = 3.13). Average full scale IQ was 97...
Background — Cognitive dysfunction is a common and disabling sequela of subarachnoid hemorrhage (... more Background — Cognitive dysfunction is a common and disabling sequela of subarachnoid hemorrhage (SAH). Although several clinical and radiographic findings have been implicated in the pathogenesis of cognitive dysfunction after SAH, few prospective studies have comprehensively and simultaneously evaluated these risk factors. Methods — Between July 1996 and March 2000, we prospectively evaluated 113 of 248 consecutively admitted nontraumatic SAH patients alive at 3 months with a comprehensive neuropsychological evaluation. Summary scores for 8 cognitive domains were calculated to express test performance relative to the entire study population. Clinical and radiographic variables associated with domain-specific cognitive dysfunction were identified with forward stepwise multiple regression, with control for the influence of demographic factors. Results — The study participants were younger ( P =0.005), less often white ( P =0.006), and had better 3-month modified Rankin scores ( P =0....
Proceedings of the National Academy of Sciences, 2004
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a trip... more Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a triplet (CAG) expansion mutation. The length of the triplet repeat is the most important factor in determining age of onset of HD, although substantial variability remains after controlling for repeat length. The Venezuelan HD kindreds encompass 18,149 individuals spanning 10 generations, 15,409 of whom are living. Of the 4,384 immortalized lymphocyte lines collected, 3,989 DNAs were genotyped for their HD alleles, representing a subset of the population at greatest genetic risk. There are 938 heterozygotes, 80 people with variably penetrant alleles, and 18 homozygotes. Analysis of the 83 kindreds that comprise the Venezuelan HD kindreds demonstrates that residual variability in age of onset has both genetic and environmental components. We created a residual age of onset phenotype from a regression analysis of the log of age of onset on repeat length. Familial correlations (correlation ± SE...
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