eMedicine Specialties > Neurology > Behavioral Neurology and Dementia

Pick Disease

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

Updated: Jun 18, 2008

Introduction

Background

Pick disease (named after Arnold Pick) is a progressive dementia defined by clinical and pathologic criteria. Unlike Alzheimer disease, which typically presents with impairment of recent memory associated with entorhinal cortex and hippocampal dysfunction, Pick disease typically affects the frontal and/or anterolateral temporal lobes. First described in 1892, with the defining pathologic characteristics first reported by Alois Alzheimer in 1911, Pick disease is now considered by some to be part of a "complex" of neurodegenerative disorders with similar or related histopathologic and clinical features.^1,2

Nomenclature history

Frontotemporal dementia (of which Pick disease is an example) is a broader term including Pick disease. Frontal lobe dementia is a term signifying neuropsychological features localizing to the frontal lobes. Clinically, Pick disease may be identical or very similar to frontal lobe degeneration.³

Some cases diagnosed premorbidly as Pick disease are shown pathologically to be progressive subcortical gliosis.⁴ Other cases may be diagnosed pathologically as dementia lacking distinctive histopathology.⁵ A clinical/genetic nosology includes frontotemporal dementia linked to chromosome 17.⁶ Primary progressive aphasia is a focal atrophy syndrome that may be associated with Pick, Alzheimer, or other pathology; clinically the deficit appears restricted to the frontal and/or temporal lobes.⁷

In a recent clinicopathological series, only 5% of patients with clinically diagnosed frontotemporal dementia had classical Pick disease with Pick bodies at postmortem evaluation.⁸

Pathophysiology

Pick disease is defined pathologically by severe atrophy, neuronal loss, and gliosis. Classified as a tauopathy, Pick disease is always accompanied by the occurrence of tau-positive inclusions.⁹ Swollen (ballooned) neurons (Pick cells) and argentophilic neuronal inclusions known as Pick bodies^10,11 disproportionally affect the frontal and temporal cortical regions.

Images of these abnormal findings can be viewed online at Internet Pathology Laboratory, University of Utah, CNS Degenerative Diseases.

Frequency

United States

Frontotemporal dementias as a group are the fourth most common cause of dementia. In most parts of the United States, among patients younger than 60 years, the frontotemporal dementias are the first or second most common cause of dementia. In patients older than 60 years, the incidence and prevalence of Alzheimer disease begins to take off and Alzheimer disease becomes by far the most prevalent form of dementia.⁸

Pick disease is sometimes used to refer to the clinical phenotype of the frontotemporal dementias. This group of disorders has a variety of pathological substrates, the most prevalent is frontotemporal lobar degeneration with ubiquitin inclusions. Pick disease itself (as defined by the presence of tau-positive, silver staining, cytoplasmic inclusions) accounted for only 5% of all cases of frontotemporal dementias in a recent pathological series.⁸

International

Familial forms of Pick disease may occur more frequently in Europe (particularly in Scandinavian nations). In a recent study in the Netherlands, the prevalence was only 28 per 100,000 persons.¹²

Mortality/Morbidity

• The disorder is progressive and invariably leads to increasing disability. The disease runs a shorter course than Alzheimer disease, on average about 6 years.^13,14
• In some individuals whose main symptoms are a disturbance of speech and language (primary progressive aphasia), the clinical course can be slow. The patient's ability to function at home may be spared for 10 or more years after onset.

Race

• Familial forms of Pick-complex dementias, linked to chromosome arm 17q, may be particularly common in people of Scandinavian origin/descent.
• It may represent as many as 17% of dementias in these populations.

Sex

More men than women may be affected.^13,15

Age

• Pick disease occurs in a younger age group than dementia of the Alzheimer type.
• Peak incidence occurs in individuals aged 55-65 years, and in most patients, Pick disease often presents when younger than 70 years.

Clinical

History

• The onset of behavioral and cognitive dysfunction in individuals with Pick disease is insidious.
• The primary impairment in cognition normally does not involve an abnormal level of consciousness or distractibility. Such a finding is more consistent with an attentional dementia¹⁶ or a confusional state and/or dementia.
• Clinical course during the first 2 years is as follows:
  • Psychiatric abnormalities that seem to respect the pattern of the classic frontal lobe syndromes are present.¹⁷
    • Patients with orbitofrontal dysfunction become aggressive and socially inappropriate. They may steal or demonstrate obsessive or repetitive stereotyped behaviors.
    • Patients with dorsomedial or dorsolateral frontal dysfunction may demonstrate a lack of concern, apathy, or decreased spontaneity.
    • Patients may be depressed early in the disease.
    • These mood changes can predate amnesia.
  • Speech and language abnormalities often begin early and progress rapidly.
    • Patients usually have relatively little limb apraxia and/or visuospatial dysfunction, thus distinguishing them from patients with diffuse bihemispheric impairment.
    • Even memory impairment is relatively less severe than speech/language and behavioral changes.
  • Incontinence can occur early. In contrast, continence generally is preserved in mild-to-moderate Alzheimer disease.
  • Parkinsonism, with its concomitant history of rigidity and gait impairment, can occur. Severe parkinsonism suggests an alternate diagnosis such as corticobasal ganglionic degeneration, diffuse Lewy body disease, or progressive supranuclear palsy.

Physical

The general physical examination often shows the patient to be unkempt at an earlier stage than in comparably impaired patients with Alzheimer disease.

• Abnormal spontaneous behaviors observed during examination may include the following:
  • Witzelsucht or inappropriate jocularity
  • Echolalia (repeating the examiner's words), echopraxia (imitating the examiner's gestures)^18,19 , and other disinhibited approach or utilization behaviors
• General neurologic examination may include some of the following abnormalities:
  • Primitive reflexes such as grasp, suck, and snout (not palmomental reflex, which is often present in healthy individuals²⁰ )
  • Akinesia, plastic rigidity, or paratonia on motor examination²¹
  • Resting tremor (uncommon; its presence suggests Parkinson disease or a Parkinson-plus syndrome)
• Mental status/neuropsychological examination may reveal the following:
  • Verbal output that is often nonfluent
    • Most patients have difficulty in naming common objects or pictures (anomia).
    • Spontaneous speech can be sparse yet fluent in character, with preserved grammar (logopenia).
  • Perseveration (cognitive and motor; see Media file 1)
  • Relatively preserved visuospatial and visual orientation skills

Causes

• The specific cause of Pick disease is unknown.
• In families with an inherited frontal lobe dementia (some of which pathologically or clinically were indistinguishable from Pick disease), linkage to markers on band 17q21-22 coding tau protein has been reported ^22,6 as have presenilin-1 mutations 14q21.^23,24
  • These familial disorders are heterogenous in different family members.
  • Some members may present primarily with amyotrophy, others with primary supranuclear gaze palsy, Parkinsonism, schizophrenialike thought disorder, or progressive aphasia and/or apraxia.

Differential Diagnoses

Other Problems to Be Considered

Adult polyglucosan body disease
Chronic meningitis
Hashimoto encephalopathy
Hemochromatosis (controversial)
Neurosarcoidosis
Other frontal lobe tumors
Dementia in Parkinson disease
Dementia in progressive supranuclear palsy
Creutzfeldt-Jakob disease
Olfactory groove meningioma
Tertiary neurosyphilis
Sequential bilateral thalamic strokes

Workup

Laboratory Studies

• As for any dementia evaluation, initial workup includes a vitamin B-12 level, thyroid function studies, antinuclear antibodies¹⁶ , and possibly fluorescent treponemal antibody testing for syphilis. The American Academy of Neurology in their practice guidelines for the evaluation of dementia indicate that the incidence of syphilis as a cause of dementia is so low in the typical American patient that routine screening for syphilis is not necessary.⁵ However, index of suspicion should be high in patients with HIV and in patients from geographic regions where the prevalence of syphilis is high.
  • If prominent inattention is observed, the patient may have a toxic and/or metabolic encephalopathy rather than a true dementia. In such patients, obtain a urine toxicology screen, serum chemistry panel, complete blood count and differential count, liver function tests, ammonia level, and erythrocyte sedimentation rate.
  • If the patient has parkinsonism or a movement disorder, the following tests can be added:
    • Ceruloplasmin (serum) and serum or urinary copper to exclude Wilson disease
    • Manual peripheral blood smear for acanthocytes
    • Genetic testing is not commercially available for screening for mutations associated with frontotemporal dementias, but may be obtained from research laboratories.
• Second-line workup includes cerebrospinal fluid examination (for chronic meningitis or elevated pressure) and HIV serology.
  • As Alzheimer disease is almost always in the differential diagnosis, cerebrospinal fluid analysis for Abeta1-42 may be indicated (see Procedures).
  • If prominent inattention is present, obtain a metastatic cancer workup, electroencephalogram (EEG), and Lyme disease serology.
  • Consider consultation by a social worker, geriatric case manager, or nurse practitioner.
• Third-line workup includes the following:
  • Obtain evaluation by neuropsychologist, behavioral neurologist, or neuropsychiatrist.
  • Consider and discuss brain biopsy in very select patients.

Imaging Studies

• Brain computed tomography (CT) or magnetic resonance imaging (MRI)
  • Order CT scan if MRI is contraindicated for the patient (eg, the patient has a pacemaker or metallic ocular implants).
  • If not contraindicated, order an MRI. Metastatic lesions and subcortical infarction (eg, caudate, thalamic) can easily be missed on CT scan. Frontal lobe atrophy out of proportion to atrophy in other brain regions can sometimes be detected. Some patients with frontotemporal dementia may have increased T2 signal in frontal lobe white matter, especially on FLAIR sequences.⁸
• Functional brain image (eg, single-photon emission computed tomography [SPECT] scan) or physiologic imaging with positron emission tomography (PET scan) may be appropriate in some patients.
  • In some patients with relatively isolated social-behavioral dysfunction, employers or others may require evidence of a medical disorder. Such patients may appear cognitively normal on objective neuropsychological tests, yet may be unable to function due to acquired brain disease.
  • A SPECT scan may demonstrate relative hypometabolism in frontal and temporal areas (when other neuroimaging is normal), thus providing evidence of brain dysfunction.

Procedures

• Lumbar puncture (cerebrospinal fluid examination) may be appropriate. Some memory disorder specialists perform this examination in every patient with frontal lobe or atypical dementia.
  • Check pressure, cultures, cryptococcal antigen, and large-volume tap for cytology or acid-fast bacillus (AFB) if the clinical situation warrants such testing. Elevated levels of phosphorylated tau protein and low levels of Abeta1-42 are found in CSF of patients with Alzheimer disease. Data on levels of tau and beta-amyloid in CSF from patients with frontotemporal dementias has been inconsistent.^25,26  Markers for Creutzfeldt-Jakob disease, CNS Whipple disease, progressive multifocal leukoencephalopathy (PML), and herpes encephalitis also can be ordered from the spinal fluid.
  • Brain biopsy may be considered in exceptional circumstances if the diagnosis is in doubt and a treatment depends on the results. Occasionally, spinal fluid markers can obviate the need for a brain biopsy, even in these patients. Some of the factors mitigating for a brain biopsy include the following:
    • If diagnosis is in doubt (eg, faced with second- or third-line autoimmune therapy for neurosarcoidosis)
    • If a familial frontotemporal dementia is suspected
    • If the family desires
    • If treatment with significant adverse effects is being considered

Histologic Findings

See Pathophysiology.

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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity

Precautions

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.

Dosing

Adult

100-300 mg IV/IM

Pediatric

Not established

Interactions

None reported

Contraindications

Documented hypersensitivity

Precautions

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.

Dosing

Adult

15 mg PO initially

Pediatric

Not established

Interactions

Increases sedative effects of alcohol, MAOIs, benzodiazepines, and other CNS depressants

Contraindications

Documented hypersensitivity; MAOIs

Precautions

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.

Dosing

Adult

37.5 mg PO bid initially

Pediatric

Not established

Interactions

Cimetidine, MAOIs, sertraline, fluoxetine, class I-C antiarrhythmics, TCAs, and phenothiazine may increase effects

Contraindications

Documented hypersensitivity; MAOIs within 14 d

Precautions

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.

Dosing

Adult

50-75 mg PO qhs; increase to 200-300 mg PO qhs as tolerated

Pediatric

Not established

Interactions

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

Contraindications

Documented hypersensitivity

Precautions

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.

Dosing

Adult

5 mg PO qd initially

Pediatric

Not established

Interactions

Effects of succinylcholine, cholinesterase inhibitors, or cholinergic agonists are increased when administered concurrently with donepezil

Contraindications

Documented hypersensitivity; concurrent anticholinergic treatment, which is expected to nullify effect

Precautions

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.

Dosing

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

Interactions

Toxicity may increase with ergot alkaloids; amitriptyline, butyrophenones, imipramine, methyldopa, phenothiazines, reserpine; may decrease bromocriptine effects

Contraindications

Documented hypersensitivity; ischemic heart disease; peripheral vascular disorders; psychosis; aggressiveness; violent behavior; uncontrolled hypertension; angina

Precautions

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.

Dosing

Adult

100 mg PO qd; increase slowly over weeks to 100 mg PO bid

Pediatric

Not established

Interactions

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

Contraindications

Documented hypersensitivity; renal failure

Precautions

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

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

• For excellent patient education resources, visit eMedicine's Dementia Center. Also, see eMedicine's patient education articles Pick Disease and Dementia Medication Overview.

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

Multimedia

Media file 1: Motor perseveration in a patient with Pick disease. The patient is asked to copy the loops (as demonstrated by the examiner in the first line).

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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

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

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

See Medscape CME activity Alzheimer's Disease and Dementia.

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