NEUROLOGY 1991;41:1374
© 1991 American Academy of Neurology
Frontal lobe degeneration
Clinical, neuropsychological, and SPECT characteristics
B. L. Miller, MD,
J. L. Cummings, MD,
J. Villanueva-Meyer, MD,
K. Boone, PhD,
C. M. Mehringer, MD,
I. M. Lesser, MD and
I. Mena, MD
Department of Neurology (Dr. Miller), Harbor-UCLA Medical Center; Departments of Neurology, and Psychiatry and Biobehavioral Services (Dr. Cummings), UCLA Medical Center; Departments of Nuclear Medicine (Drs. Villanueva-Meyer and Mena), Psychiatry (Drs. Boone and Lesser), and Radiology (Dr. Mehringer), Harbor-UCLA Medical Center, Torrance, CA.
The clinical, neuropsychological, and cerebral blood flow characteristics of eight patients with frontal lobe degeneration (FLD) were studied. Social withdrawal and behavioral disinhibition were the earliest and most common clinical presentations, and psychiatric symptoms typically preceded the onset of dementia by several years. Neuropsychological testing showed selective impairment of frontal and memory tasks with relative sparing of attention, language, and visuospatial skills. Single-photon emission computerized tomography demonstrated frontal and temporal hypoperfusion with relative sparing of parietal and occipital blood flow. Previous studies suggest that the neuropathologic findings in patients with FLD are varied; some demonstrate frontal gliosis, neuronal loss, and Pick bodies while others show only gliosis and neuronal loss.
Address correspondence and reprint requests to Dr. Bruce L. Miller, Department of Neurology B-4, Harbor-UCLA Medical Center, Torrance, CA 90509.
Supported by a Physician-Scientist-Award from the National Institute of Aging, USPHS A K11 G 00284, and a grant from the Mental Health Branch of the State of California.
Received July 16, 1990. Accepted for publication in final form February 6, 1991.
This article has been cited by other articles:

|
 |

|
 |
 
Y. Zhang, N. Schuff, A.-T. Du, H. J. Rosen, J. H. Kramer, M. L. Gorno-Tempini, B. L. Miller, and M. W. Weiner
White matter damage in frontotemporal dementia and Alzheimer's disease measured by diffusion MRI
Brain,
September 1, 2009;
132(9):
2579 - 2592.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. M. Kipps, P. J. Nestor, J. Acosta-Cabronero, R. Arnold, and J. R. Hodges
Understanding social dysfunction in the behavioural variant of frontotemporal dementia: the role of emotion and sarcasm processing
Brain,
March 1, 2009;
132(3):
592 - 603.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. F. Mendez, E. A. Licht, and J. S. Shapira
Changes in Dietary or Eating Behavior in Frontotemporal Dementia Versus Alzheimer's Disease
American Journal of Alzheimer's Disease and Other Dementias,
June 1, 2008;
23(3):
280 - 285.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
L. Massimo and M. Grossman
Patient Care and Management of Frontotemporal Lobar Degeneration
American Journal of Alzheimer's Disease and Other Dementias,
May 1, 2008;
23(2):
125 - 131.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
M. F. Mendez, E. C. Lauterbach, S. M. Sampson, and ANPA Committee on Research
An Evidence-Based Review of the Psychopathology of Frontotemporal Dementia: A Report of the ANPA Committee on Research
J Neuropsychiatry Clin Neurosci,
May 1, 2008;
20(2):
130 - 149.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. W. Levenson and B. L. Miller
Loss of Cells--Loss of Self: Frontotemporal Lobar Degeneration and Human Emotion
Current Directions in Psychological Science,
December 1, 2007;
16(6):
289 - 294.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. F. Mendez, J. S. Shapira, A. McMurtray, E. Licht, and B. L. Miller
Accuracy of the Clinical Evaluation for Frontotemporal Dementia
Arch Neurol,
June 1, 2007;
64(6):
830 - 835.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
P. J Eslinger, P. Moore, V. Troiani, S. Antani, K. Cross, S. Kwok, and M. Grossman
Oops! Resolving social dilemmas in frontotemporal dementia
J. Neurol. Neurosurg. Psychiatry,
May 1, 2007;
78(5):
457 - 460.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R McNeill, G M Sare, M Manoharan, H J Testa, D M A Mann, D Neary, J S Snowden, and A R Varma
Accuracy of single-photon emission computed tomography in differentiating frontotemporal dementia from Alzheimer's disease
J. Neurol. Neurosurg. Psychiatry,
April 1, 2007;
78(4):
350 - 355.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. J. Libon, S. X. Xie, P. Moore, J. Farmer, S. Antani, G. McCawley, K. Cross, and M. Grossman
Patterns of neuropsychological impairment in frontotemporal dementia
Neurology,
January 30, 2007;
68(5):
369 - 375.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. F. Mendez, A. K. Chen, J. S. Shapira, P.-H. Lu, and B. L. Miller
Acquired extroversion associated with bitemporal variant of frontotemporal dementia.
J Neuropsychiatry Clin Neurosci,
December 1, 2006;
18(1):
100 - 107.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
I. L. Ber, E. Guedj, A. Gabelle, P. Verpillat, M. Volteau, C. Thomas-Anterion, M. Decousus, D. Hannequin, P. Vera, L. Lacomblez, et al.
Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia.
Brain,
November 1, 2006;
129(Pt 11):
3051 - 3065.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. T. Du, G. H. Jahng, S. Hayasaka, J. H. Kramer, H. J. Rosen, M. L. Gorno-Tempini, K. P. Rankin, B. L. Miller, M. W. Weiner, and N. Schuff
Hypoperfusion in frontotemporal dementia and Alzheimer disease by arterial spin labeling MRI.
Neurology,
October 10, 2006;
67(7):
1215 - 1220.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. M. McMurtray, A. K. Chen, J. S. Shapira, T. W. Chow, F. Mishkin, B. L. Miller, and M. F. Mendez
Variations in regional SPECT hypoperfusion and clinical features in frontotemporal dementia
Neurology,
February 28, 2006;
66(4):
517 - 522.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. L. Chang, C. Lomen-Hoerth, J. Murphy, R. G. Henry, J. H. Kramer, B. L. Miller, and M. L. Gorno-Tempini
A voxel-based morphometry study of patterns of brain atrophy in ALS and ALS/FTLD
Neurology,
July 12, 2005;
65(1):
75 - 80.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
B. F. Boeve, I. W. Tremont-Lukats, A. J. Waclawik, J. R. Murrell, B. Hermann, C. R. Jack Jr, M. M. Shiung, G. E. Smith, A. R. Nair, N. Lindor, et al.
Longitudinal characterization of two siblings with frontotemporal dementia and parkinsonism linked to chromosome 17 associated with the S305N tau mutation
Brain,
April 1, 2005;
128(4):
752 - 772.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. Diehl, A. U. Monsch, C. Aebi, S. Wagenpfeil, S. Krapp, T. Grimmer, W. Seeley, H. Forstl, and A. Kurz
Frontotemporal Dementia, Semantic Dementia, and Alzheimer's Disease: The Contribution of Standard Neuropsychological Tests to Differential Diagnosis
J Geriatr Psychiatry Neurol,
March 1, 2005;
18(1):
39 - 44.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
Y. Jeong, S. S. Cho, J. M. Park, S. J. Kang, J. S. Lee, E. Kang, D. L. Na, and S. E. Kim
18F-FDG PET Findings in Frontotemporal Dementia: An SPM Analysis of 29 Patients
J. Nucl. Med.,
February 1, 2005;
46(2):
233 - 239.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
W. Liu, B. L. Miller, J. H. Kramer, K. Rankin, C. Wyss-Coray, R. Gearhart, L. Phengrasamy, M. Weiner, and H. J. Rosen
Behavioral disorders in the frontal and temporal variants of frontotemporal dementia
Neurology,
March 9, 2004;
62(5):
742 - 748.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. S. Knopman, B. F. Boeve, and R. C. Petersen
Essentials of the Proper Diagnoses of Mild Cognitive Impairment, Dementia, and Major Subtypes of Dementia
Mayo Clin. Proc.,
October 1, 2003;
78(10):
1290 - 1308.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
M. F. Mendez and K. M. Perryman
Neuropsychiatric Features of Frontotemporal Dementia: Evaluation of Consensus Criteria and Review
J Neuropsychiatry Clin Neurosci,
November 1, 2002;
14(4):
424 - 429.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
H. J. Rosen, K. M. Hartikainen, W. Jagust, J. H. Kramer, B. R. Reed, J. L. Cummings, K. Boone, W. Ellis, C. Miller, and B. L. Miller
Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD
Neurology,
June 11, 2002;
58(11):
1608 - 1615.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. Chan, N. C. Fox, R. Jenkins, R. I. Scahill, W. R. Crum, and M. N. Rossor
Rates of global and regional cerebral atrophy in AD and frontotemporal dementia
Neurology,
November 27, 2001;
57(10):
1756 - 1763.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
B. L. Miller, W. W. Seeley, P. Mychack, H. J. Rosen, I. Mena, and K. Boone
Neuroanatomy of the self: Evidence from patients with frontotemporal dementia
Neurology,
September 11, 2001;
57(5):
817 - 821.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. L. Laven and E. M. Bednarczyk
CNS Assessment Using Functional Neuro-PET Imaging
Journal of Pharmacy Practice,
August 1, 2001;
14(4):
308 - 331.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
A. M. Lipton, C. M. Cullum, S. Satumtira, E. Sontag, L. S. Hynan, C. L. White III, and E. H. Bigio
Contribution of Asymmetric Synapse Loss to Lateralizing Clinical Deficits in Frontotemporal Dementias
Arch Neurol,
August 1, 2001;
58(8):
1233 - 1239.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. R. Hodges
Frontotemporal dementia (Pick's disease): Clinical features and assessment
Neurology,
June 12, 2001;
56(90004):
S6 - 10.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
K. E. Nichol, R. Kim, and C. W. Cotman
Bcl-2 family protein behavior in frontotemporal dementia implies vascular involvement
Neurology,
June 12, 2001;
56(90004):
S35 - 40.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. J. Perry and B. L. Miller
Behavior and treatment in frontotemporal dementia
Neurology,
June 12, 2001;
56(90004):
S46 - 51.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. S. Knopman, S. T. DeKosky, J. L. Cummings, H. Chui, J. Corey-Bloom, N. Relkin, G. W. Small, B. Miller, and J. C. Stevens
Practice parameter: Diagnosis of dementia (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology
Neurology,
May 8, 2001;
56(9):
1143 - 1153.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
W. Jagust, R. Thisted, M.D. Devous Sr., R. Van Heertum, H. Mayberg, K. Jobst, A.D. Smith, and N. Borys
SPECT perfusion imaging in the diagnosis of Alzheimer's disease: A clinical-pathologic study
Neurology,
April 10, 2001;
56(7):
950 - 956.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
E. E. Camargo
Brain SPECT in Neurology and Psychiatry
J. Nucl. Med.,
April 1, 2001;
42(4):
611 - 623.
[Abstract]
[Full Text]
|
 |
|

|
 |

|
 |
 
J S Snowden, D Bathgate, A Varma, A Blackshaw, Z C Gibbons, and D Neary
Distinct behavioural profiles in frontotemporal dementia and semantic dementia
J. Neurol. Neurosurg. Psychiatry,
March 1, 2001;
70(3):
323 - 332.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
P Charpentier, I Lavenu, L Defebvre, A Duhamel, P Lecouffe, F Pasquier, and M Steinling
Alzheimer's disease and frontotemporal dementia are differentiated by discriminant analysis applied to 99mTc HmPAO SPECT data
J. Neurol. Neurosurg. Psychiatry,
November 1, 2000;
69(5):
661 - 663.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. Bozeat, C. A Gregory, M. A L. Ralph, and J. R Hodges
Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease?
J. Neurol. Neurosurg. Psychiatry,
August 1, 2000;
69(2):
178 - 186.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. J. Perry and J. R. Hodges
Differentiating frontal and temporal variant frontotemporal dementia from Alzheimer's disease
Neurology,
June 27, 2000;
54(12):
2277 - 2284.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
H. M M Smeding and I. de Koning
Frontotemporal dementia and neuropsychology: the value of missing values
J. Neurol. Neurosurg. Psychiatry,
June 1, 2000;
68(6):
726 - 730.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. H. Growdin and J. M. Primavera
Case 11-2000- A 74-Year-Old Man with Memory Loss, Language Impairment, and Personality Changes
N. Engl. J. Med.,
April 13, 2000;
342(15):
1110 - 1117.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
P S Mathuranath, J. H Xuereb, T. Bak, and J. R Hodges
Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature
J. Neurol. Neurosurg. Psychiatry,
March 1, 2000;
68(3):
304 - 312.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
T. A. Ala, G. O. Beh, and W. H. Frey II
Pure hippocampal sclerosis: A rare cause of dementia mimicking Alzheimer's disease
Neurology,
February 22, 2000;
54(4):
843 - 848.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
I.-M. TALLBERG
Projection of Meaning in Fronto-Temporal Dementia
Discourse Studies,
November 1, 1999;
1(4):
455 - 477.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
S. Rahman, B. J. Sahakian, J. R. Hodges, R. D. Rogers, and T. W. Robbins
Specific cognitive deficits in mild frontal variant frontotemporal dementia
Brain,
August 1, 1999;
122(8):
1469 - 1493.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
T. Fladby, M. Schuster, O. Gronli, H. Sjoholm, S. Loseth, and H. Sexton
Organic Brain Disease in Psychogeriatric Patients: Impact of Symptoms and Screening Methods on the Diagnostic Process
J Geriatr Psychiatry Neurol,
April 1, 1999;
12(1):
16 - 20.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
P. R Talbot, J. J Lloyd, J. S Snowden, D. Neary, and H. J Testa
A clinical role for 99mTc-HMPAO SPECT in the investigation of dementia?
J. Neurol. Neurosurg. Psychiatry,
March 1, 1998;
64(3):
306 - 313.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. H. Craig, J. L. Cummings, L. Fairbanks, L. Itti, B. L. Miller, J. Li, and I. Mena
Cerebral Blood Flow Correlates of Apathy in Alzheimer Disease
Arch Neurol,
November 1, 1996;
53(11):
1116 - 1120.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
M. L. Levy, B. L. Miller, J. L. Cummings, L. A. Fairbanks, and A. Craig
Alzheimer Disease and Frontotemporal Dementias: Behavioral Distinctions
Arch Neurol,
July 1, 1996;
53(7):
687 - 690.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
I. M. Lesser, I. Mena, K. B. Boone, B. L. Miller, C. M. Mehringer, and M. Wohl
Reduction of Cerebral Blood Flow in Older Depressed Patients
Arch Gen Psychiatry,
September 1, 1994;
51(9):
677 - 686.
[Abstract]
[PDF]
|
 |
|
|