Pick Disease Clinical Presentation

  • Author: Anna M Barrett, MD; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA   more...
 
Updated: May 14, 2012
 

History

The onset of behavioral and cognitive dysfunction in individuals with Pick disease can be difficult to identify.

The primary impairment in cognition normally does not involve an abnormal level of consciousness or distractibility. Such a finding is more consistent with an attentional dementia[13] or a confusional state and/or dementia. In many cases, individuals with Pick disease perform well on quick cognitive screening tests, such as the Mini-Mental Status Examination (MMSE).[9] However, these studies omit assessment of behavioral and social judgment or response inhibition, and, thus, this kind of quick screening is not sensitive to early Pick disease impairment that can still be disabling.

Clinical course during the first 2 years

Behavioral variant

Psychiatric abnormalities that seem to respect the pattern of the classic frontal lobe syndromes are present.[14] Patients with orbitofrontal dysfunction can become aggressive and socially inappropriate. They may steal or demonstrate obsessive or repetitive stereotyped behaviors. Patients with dorsomedial or dorsolateral frontal dysfunction may demonstrate a lack of concern, apathy, or decreased spontaneity. Patients can demonstrate an absence of self-monitoring, abnormal self-awareness, and an inability to appreciate meaning.[14] Patients with gray matter loss in the bilateral posterolateral orbitofrontal cortex and right anterior insula may demonstrate changes in eating behaviors, such as a pathologic sweet tooth. Patients with more focal gray matter loss in the anterolateral orbitofrontal cortex may develop hyperphagia.[15]

Primary progressive aphasia

Speech and language abnormalities often begin early and progress rapidly. Memory impairment is relatively less severe than speech/language and behavioral changes.

Across subtypes of FTD, including Pick disease, patients can demonstrate apathy, irritability, and agitation.[16] Patients may be depressed early in the disease. These mood changes can predate amnesia.

Patients usually have relatively little limb apraxia and/or visuospatial dysfunction, thus distinguishing them from patients with diffuse, bihemispheric impairment.

Incontinence can occur early. In contrast, continence generally is preserved in mild-to-moderate Alzheimer disease.

Parkinsonism, with its concomitant history of rigidity and gait impairment, can occur. Severe parkinsonism suggests an alternate diagnosis, such as corticobasal ganglionic degeneration, diffuse Lewy body disease, or progressive supranuclear palsy.

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Physical Examination

The general physical examination often shows the patient to be unkempt at an earlier stage than in comparably impaired patients with Alzheimer disease.

Abnormal spontaneous behaviors observed during examination may include the following:

  • Witzelsucht or inappropriate jocularity
  • Echolalia (repeating the examiner's words), echopraxia (imitating the examiner's gestures),[17, 18] and other disinhibited approach or utilization behaviors

A general neurologic examination may include some of the following abnormalities:

  • Primitive reflexes, such as grasp, suck, and snout (not the palmomental reflex, which is often present in healthy individuals[16] )
  • Akinesia, plastic rigidity, or paratonia on motor examination[19]
  • Resting tremor - Uncommon; its presence suggests Parkinson disease or a Parkinson-plus syndrome

Mental status/neuropsychological examination may reveal the following:

  • Verbal output that is often nonfluent - Most patients have difficulty in naming common objects or pictures (anomia); spontaneous speech can be sparse, yet fluent, in character, with preserved grammar (logopenia)
  • Perseveration - Cognitive and motor (see the image below)Motor perseveration in a patient with Pick diseaseMotor perseveration in a patient with Pick disease. The patient was asked to copy loops (as demonstrated by the examiner in the first line).
  • Relatively preserved visuospatial and visual orientation skills
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Contributor Information and Disclosures
Author

Anna M Barrett, MD  Director, Stroke Rehabilitation Research Program, Kessler Foundation Research Center; Chief, Neurorehabilitation Program Innovation, Kessler Institute of Rehabilitation; Professor of Physical Medicine and Rehabilitation and Neurology and Neurosciences, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Anna M Barrett, MD is a member of the following medical societies: American Academy of Neurology, American Society of Neurorehabilitation, and International Neuropsychological Society

Disclosure: Pfizer/Eisai Grant/research funds Other; Wallerstein Foundation for Geriatric Improvement Grant/research funds Speaking and teaching; OBrien Technologies Grant/research funds Independent contractor; Kessler Foundation Salary Employment; National Institutes of Health Grant/research funds Other

Chief Editor

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA  Professor of Neurology, University of Central Florida College of Medicine; Director of Cognitive Neurology, Director of Stroke Program, James A Haley Veterans Affairs Hospital

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA is a member of the following medical societies: American Academy of Neurology, American Headache Society, American Heart Association, and American Society of Neuroimaging

Disclosure: Nothing to disclose.

Additional Contributors

Daniel H Jacobs, MD, FAAN Associate Professor of Neurology, University of Florida College of Medicine

Daniel H Jacobs, MD, FAAN is a member of the following medical societies: American Academy of Neurology, American Society of Neurorehabilitation, and Society for Neuroscience

Disclosure: Teva Pharmaceutical Grant/research funds Consulting; Biogen Idex Grant/research funds Independent contractor; Serono EMD Royalty Speaking and teaching; Pfizer Royalty Speaking and teaching; Berlex Royalty Speaking and teaching

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

  1. Kertesz A, Hudson L, Mackenzie IR, Munoz DG. The pathology and nosology of primary progressive aphasia. Neurology. Nov 1994;44(11):2065-72. [Medline].

  2. Kertesz A. Pick Complex: an integrative approach to frontotemporal dementia: primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy. Neurologist. Nov 2003;9(6):311-7. [Medline].

  3. Haugarvoll K, Wszolek ZK, Hutton M. The genetics of frontotemporal dementia. Neurol Clin. Aug 2007;25(3):697-715, vi. [Medline].

  4. Weintraub S, Rubin NP, Mesulam MM. Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features. Arch Neurol. Dec 1990;47(12):1329-35. [Medline].

  5. Forman MS, Farmer J, Johnson JK, Clark CM, Arnold SE, Coslett HB, et al. Frontotemporal dementia: clinicopathological correlations. Ann Neurol. Jun 2006;59(6):952-62. [Medline].

  6. Jellinger KA. Neuropathological criteria for Pick disease and frontotemporal lobe dementia. In: Cruz-Sanchez et al, eds. Neuropathological Diagnostic Criteria. Amsterdam: IOS Press; 1995:35-54.

  7. Piguet O, Halliday GM, Reid WG, Casey B, Carman R, Huang Y. Clinical phenotypes in autopsy-confirmed Pick disease. Neurology. Jan 18 2011;76(3):253-9. [Medline].

  8. Graff-Radford NR, Woodruff BK. Frontotemporal dementia. Semin Neurol. Feb 2007;27(1):48-57. [Medline].

  9. Graham A, Hodges J. Frontotemporal dementia. Psych. 2008;7(1):24-8.

  10. Hodges JR, Davies R, Xuereb J, Kril J, Halliday G. Survival in frontotemporal dementia. Neurology. Aug 12 2003;61(3):349-54. [Medline].

  11. Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology. Jun 11 2002;58(11):1615-21. [Medline].

  12. Rascovsky K, Salmon DP, Lipton AM, Leverenz JB, DeCarli C, Jagust WJ, et al. Rate of progression differs in frontotemporal dementia and Alzheimer disease. Neurology. Aug 9 2005;65(3):397-403. [Medline].

  13. Nadeau SE. Multi-infarct dementia, subcortical dementia, and hydrocephalus. South Med J. May 1991;84(5 Suppl 1):S41-52. [Medline].

  14. Banks S, Weintraub S. Self-awareness and self-monitoring of cognitive and behavioral deficits in behavioral variant frontotemporal dementia, primary progressive aphasia and probable Alzheimer's disease. Brain Cogn. Jun 2008;67(1):58-68. [Medline]. [Full Text].

  15. Whitwell JL, Sampson EL, Loy CT, et al. VBM signatures of abnormal eating behaviours in frontotemporal lobar degeneration. Neuroimage. Mar 2007;35(1):207-13. [Medline].

  16. Srikanth S, Nagaraja AV, Ratnavalli E. Neuropsychiatric symptoms in dementia-frequency, relationship to dementia severity and comparison in Alzheimer's disease, vascular dementia and frontotemporal dementia. J Neurol Sci. Sep 15 2005;236(1-2):43-8. [Medline].

  17. Lhermitte F. 'Utilization behaviour' and its relation to lesions of the frontal lobes. Brain. Jun 1983;106 (Pt 2):237-55. [Medline].

  18. Shimomura T, Mori E. Obstinate imitation behaviour in differentiation of frontotemporal dementia from Alzheimer's disease. Lancet. Aug 22 1998;352(9128):623-4. [Medline].

  19. Beversdorf DQ, Heilman KM. Facilitory paratonia and frontal lobe functioning. Neurology. Oct 1998;51(4):968-71. [Medline].

  20. Knopman DS, Mastri AR, Frey WH 2nd, Sung JH, Rustan T. Dementia lacking distinctive histologic features: a common non- Alzheimer degenerative dementia. Neurology. Feb 1990;40(2):251-6. [Medline].

  21. Grossman M, Farmer J, Leight S, Work M, Moore P, Van Deerlin V. Cerebrospinal fluid profile in frontotemporal dementia and Alzheimer's disease. Ann Neurol. May 2005;57(5):721-9. [Medline].

  22. Verbeek MM, Pijnenburg YA, Schoonenboom NS, Kremer BP, Scheltens P. Cerebrospinal fluid tau levels in frontotemporal dementia. Ann Neurol. Oct 2005;58(4):656-7; author reply 657. [Medline].

  23. Steinbart EJ, Smith CO, Poorkaj P, Bird TD. Impact of DNA testing for early-onset familial Alzheimer disease and frontotemporal dementia. Arch Neurol. Nov 2001;58(11):1828-31. [Medline].

  24. Scott KR and Barrett AM. Dementia Syndromes: Evaluation and Treatment. Expert Review of Neurotherapeutics. 2007;7:407-422.

  25. Litvan I. Therapy and management of frontal lobe dementia patients. Neurology. Jun 2001;56(11 Suppl 4):S41-5. [Medline].

  26. Swartz JR, Miller BL, Lesser IM, Schuman S. Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors. J Clin Psychiatry. May 1997;58(5):212-6. [Medline].

  27. Lebert F, Stekke W, Hasenbroekx C, Pasquier F. Frontotemporal dementia: A randomised, controlled trial with trazodone. In: Dementia & Geriatric Cognitive Disorders. Vol 17(4). Jun 2004: 355-59.

  28. Tanaka Y, Miyazaki M, Albert ML. Effects of increased cholinergic activity on naming in aphasia. Lancet. Jul 12 1997;350(9071):116-7. [Medline].

  29. Kertesz A, Blair M, Davidson W. A Pilot Study of the Safety and Efficacy of Galantamine for Pick Complex/Frontotemporal Dementia (FTD). Abstracts of the 130th Annual Meeting of the American Neurological Association. 2005;61.

  30. Lampl Y, Sadeh M, Lorberboym M. Efficacy of acetylcholinesterase inhibitors in frontotemporal dementia. Ann Pharmacother. Nov 2004;38(11):1967-8. [Medline].

  31. Kilgard MP, Merzenich MM. Cortical map reorganization enabled by nucleus basalis activity. Science. Mar 13 1998;279(5357):1714-8. [Medline].

  32. Imamura T, Takanashi M, Hattori N, Fujimori M, Yamashita H, Ishii K, et al. Bromocriptine treatment for perseveration in demented patients. Alzheimer Dis Assoc Disord. Jun 1998;12(2):109-13. [Medline].

  33. McDowell S, Whyte J, D'Esposito M. Differential effect of a dopaminergic agonist on prefrontal function in traumatic brain injury patients. Brain. Jun 1998;121 (Pt 6):1155-64. [Medline].

  34. Drayton SJ, Davies K, Steinberg M, Leroi I, Rosenblatt A, Lyketsos CG. Amantadine for executive dysfunction syndrome in patients with dementia. Psychosomatics. May-Jun 2004;45(3):205-9. [Medline].

 
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Motor perseveration in a patient with Pick disease. The patient was asked to copy loops (as demonstrated by the examiner in the first line).
 
 
 
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