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Neurobehavioral Assessment Review Article Address correspondence to Dr Daniel I. Kaufer, CB 7025, Neurobehavioral 3129 POB, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, Assessment [email protected]. Relationship Disclosure: Daniel I. Kaufer, MD, FAAN Dr Kaufer serves on the board of Alzheimers North Carolina and the scientific advisory council of the Lewy Body ABSTRACT Dementia Association, Inc; served as a symposium director Purpose of Review: This article presents a multidimensional, integrative approach for the American Neuropsychiatric to clinical assessment and management of neurobehavioral disorders. Association’s Annual Meeting; Recent Findings: Behavioral neurology and neuropsychiatry has grown as a subspecialty provides study design support for Johnson & Johnson along with increased recognition of two common brain disorders: dementia and traumatic Services, Inc; and provided brain injury. Alzheimer disease is a highly prevalent dementia and a prototypical mem- independent medical review ory disorder, which has led to a primary focus on cognitive screening and assessment. and deposition for HensonFuerst. By contrast, recent attention concerning possible long-term sequelae of repetitive trau- Unlabeled Use of Products/Investigational matic brain injury has emphasized aberrant behavior (eg, depression, impulsivity, aggres- Use Disclosure: sion). Clinical phenotyping across cognitive and behavioral dimensions, in conjunction Dr Kaufer reports no disclosure. with advancements in structural and functional neuroimaging, brain electrophysiologic * 2015, American Academy techniques, and molecular genetics, is essential to improve diagnostic precision and of Neurology. therapeutic targeting along the spectrum of CNS disorders. Summary: All neurologists benefit from honing their clinical skills in neurobehavioral assessment. A systematic approach to cognitive and behavioral assessment increases differential diagnostic specificity, helps focus appropriate therapeutic interventions, and improves the quality of life for patients and their families. This article highlights practical approaches to neurobehavioral assessment in support of differential diagnosis and therapeutic monitoring in general neurology practice. Continuum (Minneap Minn) 2015;21(3):597–612. INTRODUCTION tive biomarker data in dementia, traumatic The seeds of contemporary cognitive brain injury, and other neuropsychiatric and behavioral neurology arose about disorders. In current neurology practice, a half-century ago in the fertile soil of cognitive, functional, and behavioral as- Boston led by Norman Geschwind. This sessment is far from standardized. In a renaissance in behavioral neurology tertiary memory disorder clinical practice advanced work from the middle to late setting, there is much greater diagnostic 19th century, first in aphasiology (Broca yield from obtaining a detailed and com- and Wernicke) and later in dementia (ie, prehensive history than from ordering a Pick and Alzheimer). The necessity of a battery of esoteric tests. Accordingly, this detailed neurologic and neurobehavioral article focuses on the basic elements of a examination accrued from a forced de- comprehensive neurobehavioral assess- pendence on inferential data in the ab- ment that are amenable to general neu- sence of direct visualization of the brain. rology practice settings. Over the last several decades, brain imag- ing has revolutionized the daily practice HISTORY of neurology and is on the cusp of advanc- The primary focus of clinical neurobe- Supplemental digital content: ing structural, functional, and molecular havioral assessment is a change from a Direct URL citations appear in the printed text and are provided diagnostic tools that will marry clin- previous level of functioning, which in- in the HTML, PDF, and app ver- ical diagnostic assessment with objec- cludes cognitive and functional abilities, sions of this article. Continuum (Minneap Minn) 2015;21(3):597–612 www.ContinuumJournal.com 597 Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Neurobehavioral Assessment KEY POINTS 1 h The primary focus of mood, emotional responsiveness, and often compromised. Impaired aware- clinical neurobehavioral social behavior. The clinical history is ness of cognitive deficits is difficult to assessment is a often the most critical data source for a measure clinically but has important im- change from a previous neurobehavioral evaluation and typically plications for a patient’s ability to comply level of functioning, requires an informant who knows the pa- with appropriate restrictions on driving which includes cognitive tient well in order to ensure an accurate or accept supervision for activities such and functional abilities, rendering. Knowing the reason for the as managing finances. Conversely, a pa- mood, emotional evaluation helps determine strategy re- tient may report a greater cognitive symp- responsiveness, and garding how to engage the patient and tom burden than a reliable informant, social behavior. informant. For example, a patient who is with cognitive testing aligning more h A marked discrepancy unaware that he or she is being evaluated with the latter’s assessment. This profile between a patient’s and for a memory problem needs to be inter- is more likely to be associated with a informant’s portrayal of viewed (and debriefed) differently from mood disturbance, anxiety symptoms, clinical cognitive changes one who is concerned about his or her or elevated level of stress. Although the sets the stage for clinical own memory functioning. In the former term ‘‘worried well’’ is sometimes used hypothesis testing case, it is often helpful to speak with the in this context, in certain cases it may be based on the degree of agreement with informant separately or, alternatively, al- appropriate to pursue a more in-depth formal cognitive testing. low the informant an opportunity to neuropsychological evaluation to en- discretely communicate sensitive infor- sure that early signs of a neurocognitive h Impaired awareness of mation. Although informants generally disorder are not being missed. This is cognitive deficits is difficult to measure provide a reliable history, at times their particularly true in highly educated indi- clinically but has frustration and distress may lead to their viduals, where ‘‘normal’’ performance on important implications overstating symptom severity in patients. standard cognitive screening tests may for how challenging In such circumstances it is often produc- be misleading. complying with tive to focus attention on helping them Other historical data may help elab- appropriate restrictions cope with caregiving demands. Rarely, orate a differential diagnosis, including or supervision for informants may have nefarious financial temporal onset and course, hereditary activities, such as driving motives for exaggerating clinical symp- factors, and comorbid medical condi- and managing finances, toms and associated disability in order tions (Table 1-1). A positive family his- can be for the patient. to obtain guardianship. Conversely, pa- tory deserves close attention, particularly tients with either depression or morbid if autosomal dominant inheritance is anxiety may be hypercritical of their self- suspected. Within a given family, a wide perceived cognitive status and exagger- range of phenotypic variability may be ate cognitive changes. seen with autosomal dominant mutations. A marked discrepancy between a pa- For example, an autosomal dominant mu- tient’s and informant’s portrayal of clin- tation on chromosome 9 associated with ical cognitive changes sets the stage for the expansion of a hexanucleotide repeat clinical hypothesis testing based on the (chromosome 9 open reading frame 72 degree of agreement with formal cog- [c9orf72]) is the most common cause nitivetesting.Forexample,ifan of both familial ALS and familial fronto- informant’s report of the patient’s deficits temporal degeneration.2 Affected mem- is supported by cognitive testing and the bers within a family can exhibit clinical deficits reported are greater than the pa- features on a spectrum between these tient’s self-report, it is reasonable to infer two disorders. Sleep disturbances such that the patient has impaired awareness as insomnia, restless leg syndrome, and of his or her deficits. This finding is sug- sleep apnea are common comorbidities gestive of a neurodegenerative disorder in neurobehavioral disorders, and rapid such as Alzheimer disease or traumatic eye movement (REM) behavior disor- brain injury, where deficit awareness is der is a hallmark of synucleinopathies.3 598 www.ContinuumJournal.com June 2015 Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited. KEY POINTS h TABLE 1-1 Diagnostic Elements in the Clinical History Documenting a careful history of head injuries b Initial/Concomitant Symptoms and their associated acute (eg, alteration or Cognitive: Memory, language, visuospatial, executive (eg, judgment) loss of consciousness) Functional: Work, managing finances, driving, shopping, household chores and chronic (eg, Neuropsychiatric: Apathy, depression, anxiety, psychosis, impulsivity, disinhibition headaches, vertigo) Motor: Tremor, rigidity, incoordination, balance difficulty, dysarthria, weakness accompaniments will b Temporal likely play an increasing Onset: Abrupt, subacute, insidious role in understanding complex relationships Course: Static, progressive,
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