Objectives: This study provides a standardized Arabic language neuropsychological test battery and tests its ability to distinguish patients with left and right hemisphere epileptic foci who are candidates for surgical resection. Methods: An Arabic language battery of 15 tests was developed based on the neuropsychological test battery used at the Johns Hopkins Hospital for surgical evaluation of patients undergoing temporal lobe resection. With modifications where culturally required, 11 tests were translated to Arabic by the principal investigator and back-translated by two bilingual health professionals; four tests were available in Arabic and added to the battery. The battery was administered to 21 Arabic-speaking patients with left temporal epileptic foci, 21 with right temporal epileptic foci, and 46 neurologically and psychiatrically healthy adults. Results: Nearly all the Arabic test versions were capable of differentiating healthy controls and the temporal lobe epilepsy (TLE) groups. Tests known to distinguish left and right temporal lobectomy candidates, such as wordlist memory and prose recall, were able to do so as accurately as the English versions. Also, a roughly “culturally free” task (the Baltimore Board) and a newly developed version of the Boston Naming Test demonstrated some sensitivity to left temporal lobe involvement. Conclusions: Arabic-language neuropsychological tests for epilepsy surgical evaluations are made available, demonstrate cultural sensitivity and clinical validity, and require further psychometric property and normative research. (JINS, 2019, 25, 761–771)

Background: To estimate the 12-month incidence, prevalence, and persistence of mental disorders among recently admitted assisted living (AL) residents and to describe the recognition and treatment of these disorders.

Methods: Two hundred recently admitted AL residents in 21 randomly selected AL facilities in Maryland received comprehensive physician-based cognitive and neuropsychiatric evaluations at baseline and 12 months later. An expert consensus panel adjudicated psychiatric diagnoses (using DSM-IV-TR criteria) and completeness of workup and treatment. Incidence, prevalence, and persistence were derived from the panel's assessment. Family and direct care staff recognition of mental disorders was also assessed.

Results: At baseline, three-quarters suffered from a cognitive disorder (56% dementia, 19% Cognitive Disorders Not Otherwise Specified) and 15% from an active non-cognitive mental disorder. Twelve-month incidence rates for dementia and non-cognitive psychiatric disorders were 17% and 3% respectively, and persistence rates were 89% and 41% respectively. Staff recognition rates for persistent dementias increased over the 12-month period but 25% of cases were still unrecognized at 12 months. Treatment was complete at 12 months for 71% of persistent dementia cases and 43% of persistent non-cognitive psychiatric disorder cases.

Conclusions: Individuals recently admitted to AL are at high risk for having or developing mental disorders and a high proportion of cases, both persistent and incident, go unrecognized or untreated. Routine dementia and psychiatric screening and reassessment should be considered a standard care practice. Further study is needed to determine the longitudinal impact of psychiatric care on resident outcomes and use of facility resources.

Impairments in executive cognition (EC) may be predictive of incident dementia in patients with mild cognitive impairment (MCI). The present study examined whether specific EC tests could predict which MCI individuals progress from a Clinical Dementia Rating (CDR) score of 0.5 to a score ≥1 over a 2-year period. Eighteen clinical and experimental EC measures were administered at baseline to 104 MCI patients (amnestic and non-amnestic, single- and multiple-domain) recruited from clinical and research settings. Demographic characteristics, screening cognitive measures and measures of everyday functioning at baseline were also considered as potential predictors. Over the 2-year period, 18% of the MCI individuals progressed to CDR ≥ 1, 73.1% remained stable (CDR = 0.5), and 4.5% reverted to normal (CDR = 0). Multiple-domain MCI participants had higher rates of progression to dementia than single-domain, but amnestic and non-amnestic MCIs had similar rates of conversion. Only three EC measures were predictive of subsequent cognitive and functional decline at the univariate level, but they failed to independently predict progression to dementia after adjusting for demographic, other cognitive characteristics, and measures of everyday functioning. Decline over 2 years was best predicted by informant ratings of subtle functional impairments and lower baseline scores on memory, category fluency, and constructional praxis. (JINS, 2011, 17, 277–288)

Previous studies reveal significant relationships between some quantitative computed tomography (CT) measures and level of cognitive functioning in patients with Alzheimer's disease (AD). This study was designed to determine whether measurements from CT scans of AD patients could predict future rates of decline in cognitive function. Subjects were 8 men and 19 women diagnosed with probable AD. CT measures included bifrontal ratio, bicaudate ratio, and areas of lateral ventricles, third ventricle, and suprasellar cistern (SSC). Measures of cognitive and adaptive functioning were obtained at the time of the scan and on follow-up. Of the CT measures, the SSCR (SSC corrected for intracranial area) was the most highly correlated with Mini-Mental State Exam (MMSE) score and other cognitive measures at the time of the scan. Follow-up data were obtained for those 20 individuals who were mildly to moderately demented at the time of the scan (MMSE ≥ 10). Rate of change was calculated for each neuropsychological measure. The SSCR correlated significantly with rate of change for MMSE and other measures of cognitive and adaptive functioning. This study demonstrates that CT measurement of the SSC can predict the subsequent rate of neurocognitive decline in AD patients. (JINS, 1996, 2, 89–95.)

The contribution of neurologic, genetic, and demographic variables to decline in cognition was examined in 70 early- to mid-stage patients with Huntington's disease (HD) using random effects modeling. Study participants were followed prospectively at baseline and at four annual reevaluations. Only modest decline was noted on most neuropsychological variables. Neurologic dysfunction, assessed using the Quantified Neurologic Examination (QNE), proved to be the strongest predictor of cognitive decline. While significantly predictive of more rapid decline in neurologic functioning, CAG repeat length was not generally related to cognitive decline after adjusting for QNE, with the exception of performance on a single test of visual scanning and psychomotor speed (i.e., Trail Making Test, Part A). We propose that CAG repeat length is more closely linked with changes in basal ganglia that predominate in early- to mid-stage HD than with cortical degeneration seen later in disease progression. Such a relationship would explain the predictive value that CAG repeat length plays in changes associated with automatic motor response programs (e.g., QNE and Trail Making Test, Part A) but not in dysfunction on tasks requiring higher-order processing. (JINS, 2006, 12, 445–454.)

A triplet repeat (CAG) expansion mutation in the huntingtin gene on chromosome 4 is responsible for Huntington's disease (HD). Presymptomatic genetic testing for this mutation has identified clinically normal persons who are virtually certain to develop this dementing illness if they live a normal lifespan. The present study sought to determine whether these “mutation-positive” persons have impairments in cognitive functioning. Seventy-five mutation-positive persons did not differ from 128 mutation-negative persons on tests selected for their sensitivity to early-stage HD. Interestingly, however, those with the mutation viewed themselves as more likely to develop HD than did those without the mutation. Among mutation-positive subjects, having a longer CAG repeat mutation was likewise not associated with cognitive impairment. However, being closer to estimated disease onset (a product of repeat length and parent's age at onset) was associated with selected cognitive impairments. When viewed in light of previous studies showing atrophy of the caudate nucleus and putamen in mutation-carriers who are close to onset but not those far from onset, these results suggest that subtle changes in brain and behavior may be detected shortly before subjects with the HD mutation develop sufficient signs and symptoms for diagnosis. Conceptual and methodological problems associated with the search for presymptomatic cognitive and behavioral indicators of dementing illness are discussed. (JINS, 2002, 8, 918–924.)

Deficits on tasks requiring semantic memory in Alzheimer's disease (AD) may be due to storage loss, a retrieval deficit, or both. To address this question, we administered multiple tasks involving 9 exemplars of the category “animals,” presented as both words and pictures, to 12 AD patients and 12 nondemented individuals. Participants made semantic judgments by class (sorting task), similarity (triadic comparison task), and dimensional attributes (ordering task). Relative to control participants, AD patients were impaired on an unstructured sorting task, but did not differ on a constrained sorting task. On the triadic comparison task, the patients were as likely to make judgments based on size as domesticity attributes, whereas control participants made judgments based primarily on domesticity. The patients' judgments were also less consistent across tasks than those of control participants. On the ordering tasks, performance was generally comparable between groups with pictures but not words, suggesting that pictures enable AD patients to access information from semantic memory that is less accessible with lexical stimuli. These results suggest that AD patients' semantic judgments are impaired when the retrieval context is unstructured, but perform normally under supportive retrieval conditions. (JINS, 2002, 8, 83–94.)

The ketogenic diet is increasingly used for the management of difficult-to-control seizures in children. Here, we describe the first prospective study of the effects of the diet on development, behavior, and parenting stress. Participants were 65 children (36 males, 29 females) with intractable seizures, ages 18 months to 14 years 6 months, enrolled in a prospective study at the Johns Hopkins Hospital, Baltimore, MD, USA, to study the diet's efficacy. Children were assessed before diet initiation and at 1-year follow-up. At follow-up, 52% (34 of 65) children remained on the diet. Mean seizure frequency decreased from 25 per day before diet initiation to less than two per day 1 year later. At follow-up, mean developmental quotient showed statistically significant improvement (p<0.05), with significant behavioral improvements in attention and social functioning. Parental stress was essentially unchanged. No baseline factor examined predicted diet adherence, and the primary reason for diet discontinuation was insufficient seizure control. These preliminary results support prior anecdotal reports of the beneficial effects of the diet on cognition and behavior.

Studies of cognitive functioning in patients with systemic lupus erythematosus (SLE) have found deficits even in patients without other evidence of neurologic involvement. The present study used scores on the 11 items of the Mini-Mental State Exam (MMSE) to classify the cognitive impairment of 93 SLE patients as suggestive of “cortical” or “subcortical” dysfunction using a validated statistical algorithm. Ninety-five percent of patients were categorized as having “subcortical” deficits, and 5% were categorized as having “cortical” deficits. When the analysis was limited to only those with total MMSE scores ≤ 24, 81% were classified as “subcortical” and 19% as “cortical.” These results suggest that SLE patients can have psychomotor and mental tracking deficits of a type seen in patients with subcortical brain disease, even in the absence of gross neurologic involvement. (JINS, 2000, 6, 821–825.)

Although a U.S. Presidential Proclamation designated the 1990s “The Decade of the Brain,” not all cerebral constituents shared equally in the limelight. By anyone's accounting, the prefrontal cortex was the darling of clinicians and neuroscientists throughout the '90s, with everything from schizophrenia and anorexia nervosa to pathological gambling and the emergence of artistic skill attributed to “frontal lobe dysfunction” (David, 1992; Miller et al., 1998; Rugle & Melamed, 1993). It should come as no surprise, then, that that most universal of cognitive afflictions, aging, should be linked to changes in frontal cortex.

Recent studies have detected basal ganglia atrophy in clinically asymptomatic persons with the genetic mutation that causes Huntington's disease (HD). Whether reductions in caudate and putamen volume on MRI scans are associated with changes in cognitive and neurologic functioning was examined in 13 healthy adults with the IT-15 mutation. Reduced striatal volume was found to correlate with greater neurologic (largely motor) impairment, slower mental processing speed, and poorer verbal learning, although none of the participants met even liberal criteria for clinical diagnosis of HD. These correlations are strikingly similar to those observed in symptomatic HD patients, possibly reflecting the earliest manifestations of disease. (JINS, 1998, 4, 467–473.)

Fortunately, it is the rare neuropsychologist who still uses the term “frontal lobe functions” to refer to the collection of supervisory and regulatory mental processes we now typically call the “executive functions.” Most of us have come to realize that simply using a neuroanatomic moniker for a set of cognitive processes does not a neuropsychology make.

This issue of JINS sees the merging of two of its departments: “Critical Reviews/Updates” and “Dialogues” have been combined to form “Critical Reviews & Dialogues.”

For too many members of our discipline, the practice of clinical neuropsychology is synonymous with the administration and interpretation of specialized tests of brain functioning. We have, in fact, become quite sophisticated in the assessment of brain-disordered patients. But the development of effective programs of neuropsychological treatment (which includes, but is not limited to, “cognitive rehabilitation”) remains one of the most pressing needs and awesome challenges for clinical and research neuropsychology.