Pick Disease Workup

  • Author: Anna M Barrett, MD; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA   more...
 
Updated: May 14, 2012
 

Approach Considerations

As in any dementia evaluation, initial workup includes a vitamin B-12 level, thyroid function studies, antinuclear antibodies,[13] and, possibly, fluorescent treponemal antibody testing for syphilis. The American Academy of Neurology, in their practice guidelines for the evaluation of dementia, indicates 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.[20] However, the index of suspicion should be high in patients with human immunodeficiency virus (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 (CBC) 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 - Not commercially available for screening for mutations associated with frontotemporal dementias, but genetic testing may be obtained from research laboratories.

Second-line workup

A second-line workup includes cerebrospinal fluid (CSF) examination (for chronic meningitis or elevated pressure) and HIV serology. As Alzheimer disease is almost always in the differential diagnosis, CSF analysis for Abeta1-42 may be indicated.

If prominent inattention is present, obtain a metastatic cancer workup, electroencephalogram (EEG), and Lyme disease serology. Consider consultation with a social worker, geriatric case manager, or nurse practitioner.

Third-line workup

A third-line workup includes the following:

  • Evaluation by a neuropsychologist, behavioral neurologist, or neuropsychiatrist
  • Consideration and discussion of genetic counseling and testing if a familial syndrome is suspected-- excellent information is available at Association for FTD
  • Consideration of a brain biopsy in very select patients
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CT Scanning, MRI, and PET Scanning

Brain CT scanning and MRI

Order a computed tomography (CT) scan if magnetic resonance imaging (MRI) is contraindicated in the patient (eg, if the patient has a pacemaker or metallic ocular implants).

If not contraindicated, an MRI scan is preferred. Metastatic lesions and subcortical infarction (eg, caudate, thalamic) can easily be missed on a 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 fluid-attenuated inversion recovery (FLAIR) sequences.[8]

Functional brain and physiologic imaging

Functional brain imaging, eg, with single-photon emission CT (SPECT) scanning, or physiologic imaging with positron emission tomography (PET) scanning 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.

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Lumbar Puncture

Lumbar puncture (CSF 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 the CSF of patients with Alzheimer disease. Data on levels of tau and beta-amyloid in CSF from patients with frontotemporal dementias has been inconsistent.[21, 22]

Markers for Creutzfeldt-Jakob disease, central nervous system (CNS) Whipple disease, progressive multifocal leukoencephalopathy, and herpes encephalitis also can be ordered from the spinal fluid.

Complications

After diagnostic lumbar puncture, some patients with Pick disease and cerebral atrophy can develop a subdural hematoma.

Observe the patient for headache or change in mental status for several days after this procedure (some physicians admit the patient for 23 h observation). If a subdural hematoma is suspected, perform a CT scan with and without contrast or an MRI scan with contrast.

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Brain Biopsy

Brain biopsy may be considered in exceptional circumstances if the diagnosis is in doubt and use of 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 the 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 a treatment that has significant adverse effects is being considered
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Contributor Information and Disclosures
Author

Anna M Barrett, MD  Director, Stroke Rehabilitation Research Program, Kessler Foundation Research Center; Chief, Neurorehabilitation Program Innovation, Kessler Institute of Rehabilitation; Professor of Physical Medicine and Rehabilitation and Neurology and Neurosciences, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

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: Pfizer/Eisai Grant/research funds Other; Wallerstein Foundation for Geriatric Improvement Grant/research funds Speaking and teaching; OBrien Technologies Grant/research funds Independent contractor; Kessler Foundation Salary Employment; National Institutes of Health Grant/research funds Other

Chief Editor

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA  Professor of Neurology, University of Central Florida College of Medicine; Director of Cognitive Neurology, Director of Stroke Program, James A Haley Veterans Affairs Hospital

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA is a member of the following medical societies: American Academy of Neurology, American Headache Society, American Heart Association, and American Society of Neuroimaging

Disclosure: Nothing to disclose.

Additional Contributors

Daniel H Jacobs, MD, FAAN Associate Professor of Neurology, University of Florida College of Medicine

Daniel H Jacobs, MD, FAAN 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

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Motor perseveration in a patient with Pick disease. The patient was asked to copy loops (as demonstrated by the examiner in the first line).
 
 
 
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