The multidisciplinary evaluation of the atypical dementia patient
Michael D. Geschwind
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorCaroline Racine Belkoura
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorMichael D. Geschwind
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorCaroline Racine Belkoura
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorMichael D. Geschwind MD PhD
Memory and Aging Center, Department of Neurology, University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorCaroline Racine Belkoura PhD
Department of Neurological Surgery, University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorSummary
This chapter presents an approach to assessing a patient with dementia, particularly those with non-AD or atypical dementias. We provide a multidisciplinary format used in our own clinic for assessing patients with cognitive impairment due to a wide variety of etiologies, with emphasis on cognitive, behavioral, and other neurological domains that often are affected with non-AD dementias, including frontal-executive, language, visuospatial, and motor functions. We stress the importance of key aspects of the history, such as identifying the very first symptom(s) and neurological exam, as well as recommend specific tools for testing various cognitive and behavioral domains. Throughout the chapter, clues to diagnosis of atypical dementias are provided. Lastly, various laboratory and imaging tests that might be helpful in diagnosing atypical dementias are discussed.
References
- Delis DC, Kramer JH, Kaplan E, et al. (2000) California Verbal Learning Test. 2nd edn., The Psychological Corporation; San Antonio, TX.
- Brandt J, Benedict R. 2001 Hopkins Verbal Learning Test-Revised: Professional Manual. Psychological Assessment Resources (PAR); Odessa, FL.
- Wechsler D. (2009) Wechsler Memory Scale. 4th edn., The Psychological Corporation; San Antonio, TX.
- Krueger CE, Kramer JH. (2010) Neurocognitive assessment. Continuum (Minneap Minn) 16:2, 176–90.
- Bonelli RM, Cummings JL. (2008) Frontal-subcortical dementias. Neurologist 14:2, 100–7.
- Lee SE, Rabinovici GD, Mayo MC, et al. (2011) Clinicopathological correlations in corticobasal degeneration. Ann Neurol 70:2, 327–40.
- Schneider SA, Walker RH, Bhatia KP. (2007) The Huntington's disease-like syndromes: what to consider in patients with a negative Huntington's disease gene test. Nat Clin Pract Neurol 3:9, 517–25.
- Lorincz MT. (2006) Geriatric chorea. Clin Geriatr Med 22:4, 879–97, vii.
- Baek WS, Desai NP. (2007) ALS: pitfalls in the diagnosis. Pract Neurol 7:2, 74–81.
- Cummings JL. (1997) The Neuropsychiatric Inventory: assessing psychopathology in dementia patients. Neurology 48:5 Suppl 6, S10–S6.
- Claassen DO, Josephs KA, Ahlskog JE, et al. (2010) REM sleep behavior disorder preceding other aspects of synucleinopathies by up to half a century. Neurology 75:6, 494–9.
- Rascovsky K, Hodges JR, Knopman D, et al. (2011) Sensitivity of revised diagnostic criteria for the behavioral variant of frontotemporal dementia. Brain 134:Pt 9, 2456–77.
- Kertesz A, Jesso S, Harciarek M, et al. (2010) What is semantic dementia?: a cohort study of diagnostic features and clinical boundaries. Arch Neurol 67:4, 483–9.
- Rosen HJ, Allison SC, Ogar JM, et al. (2006) Behavioral features in semantic dementia vs. other forms of progressive aphasias. Neurology 67:10, 1752–6.
- Seeley WW, Bauer AM, Miller BL, et al. (2005) The natural history of temporal variant frontotemporal dementia. Neurology 64:8, 1384–90.
- Rankin KP, Gorno-Tempini ML, Allison SC, et al. (2006) Structural anatomy of empathy in neurodegenerative disease. Brain 129:Pt 11, 2945–56.
- Gerstenecker A, Duff K, Mast B, et al. (2013) Behavioral abnormalities in progressive supranuclear palsy. Psychiatry Res 210:3, 1205–10.
- Yatabe Y, Hashimoto M, Kaneda K, et al. (2011) Neuropsychiatric symptoms of progressive supranuclear palsy in a dementia clinic. Psychogeriatrics 11:1, 54–9.
- Stanton BR, Leigh PN, Howard RJ, et al. (2013) Behavioral and emotional symptoms of apathy are associated with distinct patterns of brain atrophy in neurodegenerative disorders. J Neurol 260:10, 2481–90.
- Rosen HJ, Allison SC, Schauer GF, et al. (2005) Neuroanatomical correlates of behavioral disorders in dementia. Brain 128:11, 2612–25.
- Landes AM, Sperry SD, Strauss ME, et al. (2001) Apathy in Alzheimer's disease. J Am Geriatr Soc 49:12, 1700–7.
- Levenson RW, Sturm VE, Haase CM. (2014) Emotional and behavioral symptoms in neurodegenerative disease: a model for studying the neural bases of psychopathology. Annu Rev Clin Psychol 10: 581–606.
- Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. (2004) Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 55:3, 335–46.
- Gorno-Tempini ML, Brambati SM, Ginex V, et al. (2008) The logopenic/phonological variant of primary progressive aphasia. Neurology 71:16, 1227–34.
- Rohrer JD, Knight WD, Warren JE, et al. (2008) Word-finding difficulty: a clinical analysis of the progressive aphasias. Brain 131:Pt 1, 8–38.
- Gorno-Tempini ML, Hillis AE, Weintraub S, et al. (2011) Classification of primary progressive aphasia and its variants. Neurology 76:11, 1006–14.
- Crutch SJ, Lehmann M, Schott JM, et al. (2012) Posterior cortical atrophy. Lancet Neurol 11:2, 170–8.
- Jacobs DA, Lesser RL, Mourelatos Z, et al. (2001) The Heidenhain variant of Creutzfeldt-Jakob disease: clinical, pathologic, and neuroimaging findings. J Neuroophthalmol 21:2, 99–102.
- McMonagle P, Deering F, Berliner Y, et al. (2006) The cognitive profile of posterior cortical atrophy. Neurology 66:3, 331–8.
- Zakzanis KK, Boulos MI. (2001) Posterior cortical atrophy. Neurologist 7:6, 341–9.
- McKeith IG, Dickson DW, Lowe J, et al. (2005) Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology 65:12, 1863–72.
- Chow TW, Cummings JL. (2007) Frontal-subcortical circuits. In: B Miller, editor. The Frontal Lobes, 2nd edn., The Guilford Press; New York, pp. 3–26.
- Tekin S, Cummings JL. (2002) Frontal-subcortical neuronal circuits and clinical neuropsychiatry: an update. J Psychosom Res 53:2, 647–54.
- Koziol LF, Budding D, Andreasen N, et al. (2014) Consensus paper: the cerebellum's role in movement and cognition. Cerebellum 13:1, 151–77.
- Mioshi E, Kipps CM, Dawson K, et al. (2007) Activities of daily living in frontotemporal dementia and Alzheimer disease. Neurology 68:24, 2077–84.
- Knopman DS, Kramer JH, Boeve BF, et al. (2008) Development of methodology for conducting clinical trials in frontotemporal lobar degeneration. Brain 131:Pt 11, 2957–68.
- Mehta KM, Ott A, Kalmijn S, et al. (1999) Head trauma and risk of dementia and Alzheimer's disease: the Rotterdam study. Neurology 53:9, 1959–62.
- Janelsins MC, Kesler SR, Ahles TA, et al. (2014) Prevalence, mechanisms, and management of cancer-related cognitive impairment. Int Rev Psychiatry 26:1, 102–13.
- Tallet AV, Azria D, Barlesi F, et al. (2012) Neurocognitive function impairment after whole brain radiotherapy for brain metastases: actual assessment. Radiat Oncol 7, 77.
- Greene-Schloesser D, Robbins ME. (2012) Radiation-induced cognitive impairment—from bench to bedside. Neuro Oncol 14 Suppl 4, iv37–44.
- Jicha GA, Schmitt FA, Abner E, et al. (2010) Prodromal clinical manifestations of neuropathologically confirmed Lewy body disease. Neurobiol Aging 31:10, 1805–13.
- Moretti R, Torre P, Antonello RM, et al. (2004) Cholinesterase inhibition as a possible therapy for delirium in vascular dementia: a controlled, open 24-month study of 246 patients. Am J Alzheimers Dis Other Demen 19:6, 333–9.
- Girard C, Simard M, Noiseux R, et al. (2011) Late-onset-psychosis: cognition. Int Psychogeriatr 23:8, 1301–16.
- Castle DJ, Murray RM. (1993) The epidemiology of late-onset schizophrenia. Schizophr Bull 19:4, 691–700.
- Viswanath B, Harihara SN, Nahar A, et al. (2013) Battery for ECT Related Cognitive Deficits (B4ECT-ReCoDe): development and validation. Asian J Psychiatr 6:3, 243–8.
- Rose D, Fleischmann P, Wykes T, et al. (2003) Patients' perspectives on electroconvulsive therapy: systematic review. BMJ 326:7403, 1363.
- Bergsholm P. (2012) Patients’ perspectives on electroconvulsive therapy: a reevaluation of the review by Rose et al. on memory loss after electroconvulsive therapy. J ECT 28:1, 27–30.
- Jacquemont S, Hagerman RJ, Hagerman PJ, et al. (2007) Fragile-X syndrome and fragile X-associated tremor/ataxia syndrome: two faces of FMR1. Lancet Neurol 6:1, 45–55.
- Bugiani O, Murrell JR, Giaccone G, et al. (1999) Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau. J Neuropathol Exp Neurol 58:6, 667–77.
- Rademakers R, Cruts M, van Broeckhoven C. (2004) The role of tau (MAPT) in frontotemporal dementia and related tauopathies. Hum Mutat 24:4, 277–95.
- Edwards-Lee T, Miller BL, Benson DF, et al. (1997) The temporal variant of frontotemporal dementia. Brain 120:Pt 6, 1027–40.
- McCarten JR. (2013) Clinical evaluation of early cognitive symptoms. Clin Geriatr Med 29:4, 791–807.
- Garbutt S, Matlin A, Hellmuth J, et al. (2008) Oculomotor function in frontotemporal lobar degeneration, related disorders and Alzheimer's disease. Brain 131:Pt 5, 1268–81.
- Hellmuth J, Mirsky J, Heuer HW, et al. (2012) Multicenter validation of a bedside antisaccade task as a measure of executive function. Neurology 78:23, 1824–31.
- Litvan I, Agid Y, Goetz C, et al. (1997) Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology 48:1, 119–25.
- Petersen RC. (1998) Clinical subtypes of Alzheimer's disease. Dement Geriatr Cogn Disord 9:Suppl 3, 16–24.
- Tartaglia MC, Johnson DY, Thai JN, et al. (2012) Clinical overlap between Jakob-Creutzfeldt disease and Lewy body disease. Can J Neurol Sci 39:3, 304–10.
- Tschampa HJ, Neumann M, Zerr I, et al. (2001) Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 71:1, 33–9.
- Geschwind MD, Tan KM, Lennon VA, et al. (2008) Voltage-gated potassium channel autoimmunity mimicking Creutzfeldt-Jakob disease. Arch Neurol 65:10, 1341–6.
- Yokoyama JS, Rosen HJ. (2012) Neuroimaging features of C9ORF72 expansion. Alzheimers Res Ther 4:6, 45.
- Mitchell AJ, Malladi S. (2010) Screening and case finding tools for the detection of dementia. Part I: evidence-based meta-analysis of multidomain tests. Am J Geriatr Psychiatry 18:9, 759–82.
- Folstein MF, Folstein SE, McHugh PR. (1975) “Mini-mental state.” A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:3, 189–98.
- Nasreddine ZS, Phillips NA, Bedirian V, et al. (2005) The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc 53:4, 695–9.
- Mitchell AJ. (2009) A meta-analysis of the accuracy of the mini-mental state examination in the detection of dementia and mild cognitive impairment. J Psychiatr Res 43:4, 411–31.
- Tan KS, Libon DJ, Rascovsky K, et al. (2013) Differential longitudinal decline on the mini-mental state examination in frontotemporal lobar degeneration and Alzheimer disease. Alzheimer Dis Assoc Disord 27:4, 310–5.
- Zadikoff C, Fox SH, Tang-Wai DF, et al. (2008) A comparison of the mini mental state exam to the Montreal cognitive assessment in identifying cognitive deficits in Parkinson's disease. Mov Disord 23:2, 297–9.
- Hoops S, Nazem S, Siderowf AD, et al. (2009) Validity of the MoCA and MMSE in the detection of MCI and dementia in Parkinson disease. Neurology 73:21, 1738–45.
- Mickes L, Jacobson M, Peavy G, et al. (2010) A comparison of two brief screening measures of cognitive impairment in Huntington's disease. Mov Disord 25:13, 2229–33.
- Yesavage JA, Brink TL, Rolse TL, et al. (1983) Development and validity of a Geriatric Depression Scale: a preliminary report. J Psychiatr Res 17, 37–49.
- Malloy P, Grace J. (2005) A review of rating scales for measuring behavior change due to frontal systems damage. Cogn Behav Neurol 18:1, 18–27.
- Marra CM. (2014) Neurosyphilis. In: WM Scheld, RJ Whitley, CM Marra, editors. Infections of the Central Nervous System, 4th edn., Wolters Kluwer Health; Philadelphia, PA, pp. 659–73.
- Paterson RW, Takada LT, Geschwind MD. (2012) Diagnosis and treatment of rapidly progressive dementias. Neurol Clin Pract 2:3, 187–200.
- Goldman JS, Miller BL, Safar J, et al. (2004) When sporadic disease is not sporadic: the potential for genetic etiology. Arch Neurol 61:2, 213–6.
- Hall DA, Berry-Kravis E, Jacquemont S, et al. (2005) Initial diagnoses given to persons with the fragile X associated tremor/ataxia syndrome (FXTAS). Neurology 65:2, 299–301.
- Fogli A, Rodriguez D, Eymard-Pierre E, et al. (2003) Ovarian failure related to eukaryotic initiation factor 2B mutations. Am J Hum Genet 72:6, 1544–50.
- Huntington's Disease Society of America I. Guidelines for genetic testing for Huntington's disease (Revised 1994). 1994 [cited May 8, 2003]; Available from: http://www.hdfoundation.org/html/hdsatest.php.
- Feldman EA. (2012) The Genetic Information Nondiscrimination Act (GINA): public policy and medical practice in the age of personalized medicine. J Gen Intern Med 27:6, 743–6.