eMedicine Specialties > Neurology > Behavioral Neurology and Dementia
Pick Disease: Treatment & Medication
Updated: Jun 18, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- 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 IV/IM).
- As dysfunction of cortical cholinergic systems does not occur in Pick disease, use of acetyltransferase inhibitors makes less sense than it does in the treatment of Alzheimer disease or dementia with Lewy bodies. Nonetheless, class II studies have suggested 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 EEG 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 results for the patient and/or family with a tissue diagnosis.
Consultations
- Geriatric or psychiatric case manager (social worker) or nurse practitioner
- Neuropsychologist, behavioral neurologist, geriatric psychiatrist, or neuropsychiatrist
- For patients with progressive aphasia, speech pathologist for family and patient education and, in rare cases, referral for a computerized communication assistive device
Diet
High sugar content foods may need to be restricted in some patients with carbohydrate craving, which may indicate Klüver-Bucy syndrome.
Activity
No restrictions on activity are necessary.
Medication
Unfortunately, no available drugs arrest or reverse the condition. Currently, practitioners use a combination of neuroprotective and symptomatic therapies. Research studies suggest that a number of agents may actively inhibit neurodegeneration in animals, cellular models, or other disorders (see Scott and Barrett, 2007 for a review27 ), but none of these drugs are currently standard for this disease.
Vitamins
Cofactors necessary in metabolic reactions and essential for normal DNA synthesis, with some vitamins providing antioxidant effects.
Vitamin E (Vita-Plus E Softgels)
May protect polyunsaturated acid in membranes from attack by free radicals.
Adult
Although 1000 IU PO bid was used in a multicenter study showing potential effect to delay adverse events in Alzheimer disease, this dosage has not been compared with lower doses; recently, studies suggested that high doses of vitamin E may increase risk of death by other causes, and 200-400 IU daily is now a standard regimen for people with dementia.
Pediatric
Not established
Anecdotal reports suggest addition of vitamin E causes increased INR in individuals taking Coumadin; in such patients, clinicians may wish to start at 800 IU and check INR after 4-5 d, adjust Coumadin as necessary, and then increase vitamin E by 800 IU until 2000 IU/d total dose is reached, monitoring INR
Documented hypersensitivity
Pregnancy
A - Fetal risk not revealed in controlled studies in humans
Precautions
Large doses have been associated with a high incidence of necrotizing enterocolitis
Thiamine (Thiamilate)
Essential coenzyme that combines with ATP to form thiamine pyrophosphate.
Adult
100-300 mg IV/IM
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
A - Fetal risk not revealed in controlled studies in humans
Precautions
Sensitivity reactions can occur (intradermal test-dose recommended in suspected sensitivity); deaths have resulted from IV use; sudden onset or worsening of Wernicke encephalopathy, following glucose, may occur in thiamine-deficient patients; administer before or together with dextrose-containing fluids in suspected thiamine deficiency
Antidepressants
Although SSRIs have been suggested for behavioral symptoms in these patients (eg, crave sweets, hypersexual)28,29 , exercise care in using these agents in patients with parkinsonism, who may develop adverse effects of akathisia or dyskinesias.
Agents with mixed noradrenergic and serotonergic action may be helpful in treating patients with depression and frontal cognitive disorder.
Mirtazapine (Remeron)
May be sedating, especially at the lower 15-mg dose, and may be useful for patients with agitation or disinhibition and depression.
Adult
15 mg PO initially
Pediatric
Not established
Increases sedative effects of alcohol, MAOIs, benzodiazepines, and other CNS depressants
Documented hypersensitivity; MAOIs
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Exercise caution in patients with renal or hepatic dysfunction, seizure disorders, and in immunocompromised patients; inquire for symptoms of REM sleep behavior disorder, which may be triggered by SSRIs
Venlafaxine (Effexor)
May be helpful for abulic patients who also have symptoms of depression or decreased initiative.
Adult
37.5 mg PO bid initially
Pediatric
Not established
Cimetidine, MAOIs, sertraline, fluoxetine, class I-C antiarrhythmics, TCAs, and phenothiazine may increase effects
Documented hypersensitivity; MAOIs within 14 d
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Exercise caution in patients diagnosed with hepatic impairment, migraine, seizure disorders, cerebrovascular disorders, breast cancer, or thromboembolic disease; inquire for symptoms of REM sleep behavior disorder, which may be triggered by SSRIs
Trazodone (Desyrel)
5-HT2–receptor antagonist that inhibits reuptake of 5-HT. Negligible affinity for cholinergic, adrenergic, dopaminergic, or histaminic receptors. Good hypnotic properties. Effective in reducing agitation in patients with head trauma or dementia. Useful for sleep disturbances. Structurally unrelated to TCAs, tetracyclics, or MAOIs. Cardiac conduction effects of trazodone are qualitatively dissimilar and quantitatively less pronounced than TCAs and therefore are less toxic in overdose.
Adult
50-75 mg PO qhs; increase to 200-300 mg PO qhs as tolerated
Pediatric
Not established
May enhance response to alcohol, barbiturates, and other CNS depressants; digoxin and phenytoin serum levels may increase in patients receiving trazodone, concurrently; may decrease hypoprothrombinemic effects of warfarin
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypotension, including orthostatic hypotension and syncope, has occurred; may produce drowsiness, dizziness, or blurred vision; patients taking this medication should observe caution while driving or performing other tasks requiring alertness, coordination, or dexterity; inquire for symptoms of REM sleep behavior disorder, which may be triggered by SSRIs
Cholinergic agents
Cholinergic therapy may be helpful for patients with aphasia30 , and preliminary studies indicate cholinesterase inhibitors may be useful for aphasia in Pick disease31 and for other dementia-related symptoms in these patients32 .
Donepezil (Aricept)
Acetylcholinesterase inhibitor used in dementia of the Alzheimer type. Cholinergic stimulation may improve naming (Tanaka, 1997) and increase neuronal plasticity (Kilgard, 1998); thus, reasonable to attempt therapy in patients with primary progressive aphasia. Unfortunately, no clinical studies are available on the effect of donepezil in patients with Pick disease.
Adult
5 mg PO qd initially
Pediatric
Not established
Effects of succinylcholine, cholinesterase inhibitors, or cholinergic agonists are increased when administered concurrently with donepezil
Documented hypersensitivity; concurrent anticholinergic treatment, which is expected to nullify effect
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Exercise caution in patients with seizures, asthma, sick sinus syndrome, or other supraventricular conduction abnormalities
Dopaminergic agents
Although these agents may worsen sexual or behavioral disinhibition, they may improve executive function, perseveration, and abulia.33,34,35
Bromocriptine (Parlodel)
Semisynthetic ergot alkaloid derivative. Strong dopamine D2-receptor agonist. Partial dopamine D1-receptor agonist. Inhibits prolactin secretion with no effect on other pituitary hormones. May be given with food to minimize possibility of GI irritation.
Approximately 28% absorbed from GI tract and metabolized in liver. Approximate elimination half-life is 50 h, with 85% excreted in feces and 3-6% eliminated in urine.
Initiate at low dosage; slowly increase dosage to individualize therapy. Assess dosage titration every 2 wk. Gradually reduce dose in 2.5-mg decrements if severe adverse reactions occur.
Adult
1.25 mg PO initially for 5-7 d, increase very slowly (over a month or more) to 2.5-5 mg PO bid
Pediatric
Not established
Toxicity may increase with ergot alkaloids; amitriptyline, butyrophenones, imipramine, methyldopa, phenothiazines, reserpine; may decrease bromocriptine effects
Documented hypersensitivity; ischemic heart disease; peripheral vascular disorders; psychosis; aggressiveness; violent behavior; uncontrolled hypertension; angina
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal or hepatic disease; rapid dose increase can be associated with nausea and vomiting; do not give after 6 pm (6 am/6 pm is a good schedule) as it will impair sleep and may be associated with vivid unpleasant dreams and disorganized nighttime behavior when taken at bedtime. Do not give for more than 2-3 months because of potential of inducing fibrotic complications with long-term use.
Amantadine (Symmetrel)
Inhibits N -methyl-D -aspartic acid (NMDA) receptor–mediated stimulation of acetylcholine release in rat striatum. May enhance dopamine release, inhibit dopamine reuptake, stimulate postsynaptic dopamine receptors, or enhance dopamine receptor sensitivity.
Adult
100 mg PO qd; increase slowly over weeks to 100 mg PO bid
Pediatric
Not established
Drugs with anticholinergic or CNS stimulant activity increase amantadine toxicity; the concurrent administration of hydrochlorothiazide plus triamterene with amantadine may increase plasma concentrations of amantadine
Documented hypersensitivity; renal failure
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in liver disease, uncontrolled psychosis, eczematoid dermatitis, and seizures and in those receiving CNS stimulant drugs; reduce dose in renal disease when treating Parkinson disease; do not discontinue this medication abruptly; give second dose no later than 6 pm; may produce peripheral edema or delirium (discontinue if these symptoms are noted); livedo reticularis may be associated with this medication
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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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
