Laboratory Studies

Choice of blood tests depends on clinical setting.

In many cases, tests of thyroid function, B-12 level, and serology for syphilis are appropriate.

In other instances, testing for HIV or connective tissue disorders is indicated.

Imaging Studies

CT scanning is adequate to diagnose acute bleeds and ventriculomegaly (hydrocephalus).

MRI is more sensitive and specific than CT for showing tumors, focal or diffuse atrophy, subdural hematomas, or vascular and microvascular pathology.

Many behavioral neurology specialists would obtain a deoxyglucose PET scan in patients with a clinical diagnosis of frontotemporal dementia. A pattern of decreased frontal lobe glucose utilization with preserved temporal-parietal glucose utilization would favor the diagnosis of a frontotemporal dementia. The opposite pattern is characteristic of Alzheimer disease.^[16]

In one study, 18-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) was able to identify nearly half of the cases of behavioral variant of frontotemporal dementia (bvFTD) that were not detected by magnetic resonance imaging. According to the authors, high specificity of 18F-FDG-PET can enable exclusion of psychiatric and other neurodegenerative disorders. The 18F-FDG-PET study was performed in 52 patients with suspected bvFTD who lacked characteristic structural neuroimaging results.^[17]

Other Tests

Neuropsychology: Many tests are described in Physical.

EEG may be exceptionally considered if evidence of subclinical seizure activity is suspected, particularly in rapidly progressive symptomatic cases.

Procedures

Lumbar puncture may be needed to look for signs of occult infection.

Treatment & Management

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Media Gallery

Axial brain MRI of a patient with progressive tremorless parkinsonism and frontal-predominant dementia (Mini Mental State Examination = 23/30; Frontal Assessment Battery = 10/18; abnormal clock drawing task and additional constructional impairment) with moderate ideomotor apraxia. The MRI demonstrates predominantly frontal (A) and anterior temporal atrophy (B) suggestive of frontotemporal dementia.

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Author

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Medical Director, Tulane Center for Autism and Related Disorders, Tulane University School of Medicine; Pediatric Neurologist and Epileptologist, Ochsner Hospital for Children; Professor of Neurology, Louisiana State University School of Medicine

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, American Neurological Association, The Society of Federal Health Professionals (AMSUS), Child Neurology Society, Southern Pediatric Neurology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Jasvinder Chawla, MD, MBA Chief of Neurology, Hines Veterans Affairs Hospital; Professor of Neurology, Loyola University Medical Center

Jasvinder Chawla, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

Joseph Quinn, MD, MD Assistant Professor, Department of Neurology, Portland VA Medical Center, Oregon Health Sciences University

Joseph Quinn, MD, MD is a member of the following medical societies: American Academy of Neurology, Society for Neuroscience, Society for Pediatric Radiology

Disclosure: Nothing to disclose.

Acknowledgements

Alberto J Espay, MD, MSc Associate Professor, Director of Clinical Research, Gardner Family Center for Parkinson's Disease and Movement Disorders, University of Cincinnati College of Medicine

Alberto J Espay, MD, MSc is a member of the following medical societies: American Academy of Neurology and Movement Disorders Society

Disclosure: Abbott Consulting fee Consulting; Chelsea therapeutics Consulting fee Consulting; Novartis Honoraria Speaking and teaching; TEVA Consulting fee Consulting; NIH Grant/research funds K23 Career Development Award; Eli Lilly Consulting fee Consulting; Great Lakes Neurotechnologies Other; Michael J Fox Foundation Grant/research funds Other; Lippincott Williams & Wilkins Royalty Book; American Academy of Neurology Honoraria Speaking and teaching