eMedicine Specialties > Neurology > Behavioral Neurology and Dementia

Pick Disease: Differential Diagnoses & Workup

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
Contributor Information and Disclosures

Updated: Jun 18, 2008

Differential Diagnoses

Alzheimer Disease
Huntington Disease
Anterior Circulation Stroke
Hydrocephalus
Cardioembolic Stroke
Hyperammonemia
Cortical Basal Ganglionic Degeneration
Inherited Metabolic Disorders
Dementia in Motor Neuron Disease
Lyme Disease
Frontal and Temporal Lobe Dementia
Marchiafava-Bignami Disease
Frontal Lobe Epilepsy
Multiple Sclerosis
Frontal Lobe Syndromes
Multiple System Atrophy
Head Injury
Neuroacanthocytosis
Herpes Simplex Encephalitis
Prion-Related Diseases
HIV-1 Encephalopathy and AIDS Dementia Complex

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.

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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Further Reading

See Medscape CME activity Alzheimer's Disease and Dementia.

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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

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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

 
 
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