Pick Disease Treatment & Management

Updated: Jun 06, 2014
  • Author: A M Barrett, MD; Chief Editor: Jasvinder Chawla, MD, MBA  more...
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Treatment

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. Discuss the potential utility of lumbar puncture to identify rare treatable conditions.

On third evaluation, perform a lumbar puncture if elected by patient and family. 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.

Diet and activity

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

It is not clear whether a diet relatively low in carbohydrates, especially refined carbohydrates and sugar, and relatively high in fats and protein (a ketogenic diet), may be associated with improved cognition in Pick disease and FTDs. Such a diet has been associated with symptomatic improvement of both Alzheimer disease and Parkinson disease, but the results are very preliminary and should not outweigh a doctor's advice about an appropriate heart-healthy diet.

A regimen of appropriate therapeutic exercise (eg, brisk walking with a partner) can be helpful in these patients to help regulate mood and reduce akathisia. It is also possible exercise may have beneficial effects on cognitive function.

Inpatient care

Most of the dementia workup above is now recommended as outpatient management. However, a one-day hospital stay may accomplish the second and third steps above under rare conditions (eg, potential need for epilepsy monitoring, if the diagnosis might include chronic meningitis or encephalitis, or if the need for brain biopsy is suspected). However, think carefully about the high risk of hospital-acquired delirium, with potential sundowning and agitation; this complication (see below) is associated with increased mortality and poor quality of life.

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.

Decompensation/risk of hospital-acquired delirium

Like all people with dementia, people with Pick disease who are relatively high functioning are at very high risk of hospital-acquired delirium. If they decompensate while in a hospital or other unfamiliar setting during illness or medical intervention, this medical complication may adversely affect the outcome of the hospitalization as well as their general health. This should be considered when contemplating elective surgery or other nonessential interventions (eg, cataract removal).

Medicolegal concerns

Loss of social-emotional abilities must be taken into account when making recommendations about continuing work, especially when responsibility for others is part of the work role. People with FTD may retain intellectual capability of performing tasks, but they may make social or interpersonal errors that are unacceptable or even dangerous to themselves or others.

Surgical care

At this time, surgical treatments are not demonstrated to benefit people with frontotemporal dementias or Pick disease.

Next:

Consultations

Neuropsychologist, behavioral neurologist, geriatric psychiatrist, or neuropsychiatrist

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.

Genetic counselor

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. [25]

Brain injury specialist

If the patient and family have difficulty accepting the idea of social-emotional dysfunction and disability, and, for example, continue to assign the patient responsibility such as caring for small children, it can be helpful to consult with a job coach, cognitive specialist, or psychologist or social worker experienced with people with traumatic brain injury. Such specialists are experienced with recognizing and explaining the deficits encountered in people with FTD, because of the frequency of orbital-frontal cortical deficits after traumatic brain injury.

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