Pick Disease Treatment & Management

Updated: Nov 18, 2019
  • Author: Monica Saini, MD, MBBS, MRCP(UK); Chief Editor: Jasvinder Chawla, MD, MBA  more...
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Treatment

Approach Considerations

Currently, there are no treatments available for changing the course of bvFTD and svPPA. There is no class I evidence regarding any drug in bvFTD or svPPA.

The aim of medical therapy is symptomatic relief.

Individualized management plans, including nonpharmacologic and pharmacologic interventions, are best suited for patients with Pick disease.

Non-pharmacological treatment

This requires a multidisciplinary approach.

  • Patient safety and well-being: This may include driving restrictions, restriction of financial access (where required), environmental safety (home modification) and adaptation, caregiver training and counseling for families.
  •  Exercises and physical therapy: These are useful especially for patients with concomitant problems with movements. Appropriate referrals to physical and occupational therapists should be considered. Such interventions may also improve mood.
  • Dietary interventions: High-sugar-content foods may need to be restricted in some patients with carbohydrate craving. Some patients may require special care to prevent placement of inedible objects in the mouth. At initial presentation and follow-up the weight of the patient should be monitored.
  • Speech therapy: Though not curative, speech interventions have been shown to help patients with PPA cope with their speech deficits. Speech and language therapists have developed several impairment-based interventions and compensatory strategies for use. Speech therapist assessment is also indicated for swallowing assessment and management of dysphagia.

Pharmacological treatment

Because dysfunction of cortical cholinergic systems does not occur in Pick disease, the use of acetyltransferase inhibitors in this condition is not indicated. Cholinesterase inhibitors can even exacerbate behavioral disturbance in patients with FTD. Memnatine, though tolerated well, has no clinical benefit in FTD. [51]

Studies in the neurochemistry of bvFTD have shown a serotoninergic and dopaminergic network disruption. Consequently, studies have shown that serotonin selective reuptake inhibitors (SSRIs) (fluoxetine, sertraline, paroxetine, fluvoxamine, citalopram) are effective in helping with various symptoms of FTD (disinhibition, impulsivity, eating disorders). For  severe neurobehavioral symptoms where SSRIs are not useful, careful and limited use of antipsychotic medications (preferably quetiapine or olanzapine) may be considered, with close monitoring for extrapyramidal side effects. However, avoid the use of neuroleptics as much as possible.

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. Occasionally, hospitalization may be required to an appropriate ward in case of violent or totally disruptive behavior.

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.

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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. [52]

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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