eMedicine Specialties > Neurology > Behavioral Neurology and Dementia

Pick Disease: Follow-up

Author: Anna M Barrett, MD, Associate Professor of Physical Medicine and Rehabilitation and Neurology and Neurosciences, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Director, Stroke Rehabilitation Research Program, Kessler Medical Rehabilitation Research and Education Center
Contributor Information and Disclosures

Updated: Jun 18, 2008

Follow-up

Further Inpatient Care

  • The second and third steps of the 3-step dementia workup also can be accomplished as an inpatient procedure.
    • This is completed over 2 days with initial contact made by a case manager.
    • It is particularly useful if the diagnosis is unclear, since referral for brain biopsy may be expedited.

Further Outpatient Care

  • Periodic follow-up care is indicated to manage problem behaviors or clinical problems of the patient, to support the caregiver, or to re-evaluate the diagnosis if it is in doubt.
  • If paranoia, depression, or other behavioral problems manifest, pharmacologic treatments can be tailored to address these problems.

Complications

  • After diagnostic lumbar puncture, some patients with Pick disease and cerebral atrophy can develop a subdural hematoma.
  • Observe for headache or change in mental status for several days after this procedure (some physicians admit for 23-h observation). If a subdural hematoma is suspected, perform a CT scan with and without contrast or an MRI scan with contrast.

Prognosis

  • Like most dementias, Pick disease is slowly progressive, leading to increased vocational and personal disability.
  • Some patients can progress slowly over extremely long periods.
  • Some may develop artistic or other talents during the course of their dementia, perhaps related to disinhibition of "creative" brain areas. Musical or artistic tastes also may change (eg, sudden interest in music intended for much younger listeners).
  • Some may be capable of acquiring new knowledge or skills such as learning to use a computer-assisted simple communication system.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Patients with progressive dementia should undergo neuroimaging sensitive enough to detect a structural lesion (eg, MRI in most patients, rather than a CT scan). Serologic testing for syphilis also should be strongly considered. Interim progression of these potentially treatable disorders can result in legal culpability.

Special Concerns

  • Consultation with a nurse practitioner or case manager experienced with dementia is indicated. If needed, such a person can be located through a local chapter of the Alzheimer's Association or the state Department of Aging.
    • While the patient is able to participate, a family contact (eg, durable power of attorney) can be designated to decide care-giving and/or end-of-life issues.
    • The nurse or case manager also can assist caregivers with stress management, teach behavioral techniques, refer to day programs, and assess a patient who may need to be admitted for short- or long-term management of behavioral problems.
  • In situations where a strong family history of frontotemporal dementia or Pick disease is present, unaffected family members may desire genetic testing. It cannot be overstressed that this should only be performed after informed genetic counseling, preferably in a specialty center familiar with the genetics of dementing disorders. In this setting, testing may be of benefit.36
 
Acknowledgments

The author would like to thank Shaan Khurshid for help in research and preparation of the most recent revision of this article. 



More on Pick Disease

Overview: Pick Disease
Differential Diagnoses & Workup: Pick Disease
Treatment & Medication: Pick Disease
Follow-up: Pick Disease
Multimedia: Pick Disease
References
Further Reading
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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. Miller BL, Ikonte C, Ponton M, Levy M, Boone K, Darby A, et al. A study of the Lund-Manchester research criteria for frontotemporal dementia: clinical and single-photon emission CT correlations. Neurology. Apr 1997;48(4):937-42. [Medline].

  4. Neumann MA, Cohn R. Progressive subcortical gliosis, a rare form of presenile dementia. Brain. Jun 1967;90(2):405-18. [Medline].

  5. 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].

  6. Foster NL, Wilhelmsen K, Sima AA, Jones MZ, D'Amato CJ, Gilman S. Frontotemporal dementia and parkinsonism linked to chromosome 17: a consensus conference. Conference Participants. Ann Neurol. Jun 1997;41(6):706-15. [Medline].

  7. 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].

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

  9. 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].

  10. 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.

  11. Jackson M, Lowe J. The new neuropathology of degenerative frontotemporal dementias. Acta Neuropathol (Berl). 1996;91(2):127-34. [Medline].

  12. Stevens M, van Duijn CM, Kamphorst W, de Knijff P, Heutink P, van Gool WA, et al. Familial aggregation in frontotemporal dementia. Neurology. Jun 1998;50(6):1541-5. [Medline].

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

  14. 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].

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

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

  17. Gregory CA, Hodges JR. Frontotemporal dementia: use of consensus criteria and prevalence of psychiatric features. Neuropsychiatry Neuropsychol Behav Neurol. 1996;9(3):145-153.

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

  19. 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].

  20. Sjogren M, Wallin A, Edman A. Symptomatological characteristics distinguish between frontotemporal dementia and vascular dementia with a dominant frontal lobe syndrome. Int J Geriatr Psychiatry. Jun 1997;12(6):656-61. [Medline].

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

  22. Lynch T, Sano M, Marder KS, Bell KL, Foster NL, Defendini RF, et al. Clinical characteristics of a family with chromosome 17-linked disinhibition-dementia-parkinsonism-amyotrophy complex. Neurology. Oct 1994;44(10):1878-84. [Medline].

  23. Rogaeva EA, Fafel KC, Song YQ, Medeiros H, Sato C, Liang Y, et al. Screening for PS1 mutations in a referral-based series of AD cases: 21 novel mutations. Neurology. Aug 28 2001;57(4):621-5. [Medline].

  24. Raux G, Gantier R, Thomas-Anterion C, Boulliat J, Verpillat P, Hannequin D, et al. Dementia with prominent frontotemporal features associated with L113P presenilin 1 mutation. Neurology. Nov 28 2000;55(10):1577-8. [Medline].

  25. 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].

  26. 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].

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

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

  29. 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].

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

  31. 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.

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

  33. 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].

  34. 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].

  35. 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].

  36. 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].

  37. Coleman LW, Digre KB, Stephenson GM, Townsend JJ. Autopsy-proven, sporadic pick disease with onset at age 25 years. Arch Neurol. May 2002;59(5):856-9. [Medline].

  38. Freedman M. Frontotemporal dementia: recommendations for therapeutic studies, designs, and approaches. Can J Neurol Sci. Mar 2007;34 Suppl 1:S118-24. [Medline].

  39. Ghika-Schmid F, Ghika J, Regli F, Dworak N, Bogousslavsky J, Stadler C, et al. Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?. Mov Disord. Sep 1996;11(5):555-62. [Medline].

  40. Kertesz A, Munoz DG. Pick's Disease and Pick Complex. NY: Wiley-Liss: 1998.

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

  42. 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.

  43. Litvan I, Agid Y, Sastry N, Jankovic J, Wenning GK, Goetz CG, et al. What are the obstacles for an accurate clinical diagnosis of Pick's disease? A clinicopathologic study. Neurology. Jul 1997;49(1):62-9. [Medline].

  44. Neary D, Snowden JS, Gustafson L. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. Dec 1998;51(6):1546-54. [Medline].

  45. Perry RJ, Miller BL. Behavior and treatment in frontotemporal dementia. Neurology. Jun 2001;56(11 Suppl 4):S46-51. [Medline].

  46. Pick A. Ueber die Beziehungen der senilen Hirnatrophie zur Aphasie. Pragmatische Medizinsche Wehnschrift. 1892;17:165-167.

  47. Roman GC, Tatemichi TK, Erkinjuntti T, Cummings JL, Masdeu JC, Garcia JH, et al. Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop. Neurology. Feb 1993;43(2):250-60. [Medline].

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Further Reading

See Medscape CME activity Alzheimer's Disease and Dementia.

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Keywords

dementia lacking distinctive histopathology, frontal lobe degeneration, frontal lobe dementia, frontotemporal dementia, FTD, frontotemporal dementia linked to chromosome 17, primary progressive aphasia, progressive subcortical gliosis, Pick disease, Pick's disease, progressive dementia

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Contributor Information and Disclosures

Author

Anna M Barrett, MD, Associate Professor of Physical Medicine and Rehabilitation and Neurology and Neurosciences, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Director, Stroke Rehabilitation Research Program, Kessler Medical Rehabilitation Research and Education Center
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: Nothing to disclose.

Medical Editor

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

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Richard J Caselli, MD, Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale
Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Howard A Crystal, MD, Professor, Departments of Neurology and Pathology, State University of New York Downstate; Consulting Staff, Department of Neurology, University Hospital and Kings County Hospital Center
Howard A Crystal, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association
Disclosure: Pfizer Honoraria Speaking and teaching; Myriad Honoraria Consulting

 
 
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