Pick Disease Treatment & Management

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

Approach Considerations

On first evaluation, discontinue medications that can impair memory or cause confusion (eg, anticholinergic drugs, sedatives, benzodiazepines). Consider empiric treatment for symptoms that are consistent with depression and/or sleep disorders. Consider administering thiamine empirically (100-300 mg intravenous [IV]/intramuscular [IM]).

Because dysfunction of cortical cholinergic systems does not occur in Pick disease, the use of acetyltransferase inhibitors in this condition makes less sense than it does in the treatment of Alzheimer disease or dementia with Lewy bodies. Nonetheless, class II studies have suggested that they might be of some benefit. The few published studies assessing the efficacy of memantine in frontotemporal dementias have had equivocal results. Some research has indicated that drugs that modulate the serotonergic system may be helpful for treating behavioral symptoms in frontotemporal dementias.

On second evaluation, treat any systemic conditions that were identified and discuss performing a lumbar puncture and HIV testing with the patient and family. If prominent inattention is present and epilepsy is considered, consider further second-line workup, including electroencephalography or an empiric trial of an anticonvulsant. Consider referral to a case manager, geriatric nurse practitioner, or other dementia resource person or group for social-family issues.

On third evaluation, perform a lumbar puncture, treat any conditions identified on testing, and consider consultation with a behavioral neurologist or geriatric psychiatrist. Share dementia information and reading material with the family. Consider a brain biopsy if the diagnosis is in doubt or if substantial benefit will result for the patient and/or family with a tissue diagnosis.

Inpatient care

The second and third steps of the 3-step dementia workup could be accomplished as an inpatient procedure. Be wary of hospitalization-related delirium, sundowning, or agitation. Such a hospitalization could be completed over 2 days, with initial contact made by a case manager. This could be particularly useful if the diagnosis is unclear or if the patient lives in a rural location remote from dementia specialty services, since referral for brain biopsy may be expedited.

Outpatient care

Periodic follow-up care is indicated to manage problem behaviors or clinical problems of the patient, to support the caregiver, or to reevaluate the diagnosis if it is in doubt.

If paranoia, depression, or other behavioral problems manifest, pharmacologic treatments can be tailored to address these problems.

Diet

High sugar content foods may need to be restricted in some patients with carbohydrate craving, which may indicate Klüver-Bucy syndrome.

Decompensation

People with Pick Disease who are relatively high functioning may decompensate while in a hospital or other unfamiliar setting during illness or medical intervention. This should be considered when contemplating elective surgery or other nonessential interventions (eg, cataract removal).

Next

Consultations

Patient care can include consultations with a neuropsychologist, behavioral neurologist, geriatric psychiatrist, or neuropsychiatrist. For patients with progressive aphasia, consultation with a speech pathologist for family and patient education and, in rare cases, referral for a computerized communication assistive device, can be beneficial.

Consultation with a nurse practitioner or a geriatric or psychiatric case manager (social worker) experienced with dementia is indicated. If needed, such professionals 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, provide referral to day programs, and assess a patient who may need to be admitted for short- or long-term management of behavioral problems.

In situations in which 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 be performed only after informed genetic counseling, preferably in a specialty center familiar with the genetics of dementing disorders. In this setting, testing may be of benefit.[23]

Previous
Proceed to Medication
 
 
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].

 
Previous
Next
 
Motor perseveration in a patient with Pick disease. The patient was asked to copy loops (as demonstrated by the examiner in the first line).
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.