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CLINICAL CRITERIA FOR DIAGNOSIS OF FRONTOTEMPORAL LOBAR DEGENERATION (FTD)
(Neary D. et al, Neurology 1998;51:1546-54)
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List 1 The clinical diognostic features of frontotemporal lobar degeneration: clinical profile
Character change and disordered social conduct are the dominant fcatures initially and throughout the disease course. Instrumental functions of perception, spatial skills, praxis, and memory are intact or relatively well preserved.
I. Core diagnostic features
A. Insidious onset and gradual progression
B. Early decline in social interpersonal conduct
C. Early impairment in regulation of personal conduct
D. Early emotional blunting
E. Early loss of insight
II. Supportive diagnostic features
A. Behavioral disorder
1. Decline in personal hygiene and grooming
2. Mental rigidity and inflexibility
3. Distractibility and impersistence
4. Hyperorality and dietary changes
5. Perseverative and stereotyped behavior
6. Utilization behavior
B. Speech and language
1. Altered speech output
a. Aspontaneity and economy of speech
b. Press of speech
2. Stereotypy of speech
3. Echolalia
4. Perseveration
5. Mutism
C. Physical signs
1. Primitive reflexes
2. Incontinence
3. Akinesia, rigidity, and tremor
4. Low and labile blood pressure
D. Investigations
1. Neuropsychology: significant impairment on frontal lobe tests in the absence of severe amnesia, aphasia, or perceptuospatial disorder
2. Electroencephalography: normal on conventional EEG despite clinically evident dementia
3. Brain imaging (structural and/or functional): predominant frontal and/or anterior temporal abnormality
Features common to clinical syndromes of frontotemporal lobar degeneration
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List 2 The clinical diagnostic features of progressive non fluent aphasia: clinical profile
Disorder of expressive language is the dominant feature initially and throughout the disease course. Other aspects of cognition are intact or relativeby well preserved.
I. Core diagnostic features
A. Insidious onset and gradual progression
B. Nonfluent spontaneous speech with at least one of the following: agrammatism, phonemic paraphasias, anomia
II. Supportive diagnostic features
A. Speech and language
1. Stuttering or oral apraxia
2. Impaired repetition
3. Alexia, agraphia
4. Early preservation of word meaning
5. Late mutism
B. Behavior
1. Early preservation of social skills
2. Late behavioral changes similar to FTD
C. Physical signs: late contralateral primitive reflexes, akinesia, rigidity, and tremor
D. Investigations
1. Neuropsychology: nonfluent aphasia in the absence of severe amnesia or perceptuospatial disorder
2. Electroencephalography: normal or minor asymmetric slowing
3. Brain imaging (structural and/or functional): asymmetric abnormality predominantly affecting dominant (usually left) hemisphere
Features common to clinical syndromes of frontotemporal lobar degeneration
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List 3 The clinical diagnostic features of semantic aphasia and associative agnosia (SD): clinical profile
Semantic disorder (impaired understanding of word meaning and/or object identity) is the dominant feature initially and throughout the disease course. Other aspects of cognition, including autobiographic memory, are intact or relatively well preserved.
I. Core diagnostic features
A. Insidious onset and gradual progression
B. Language Disorder characterized by
1. Progressive, fluent, empty spontaneous speech
2. Loss of word meaning, manifest by impaired naming and comprehension
3. Semantic paraphasias and/or
C. Perceptual disorder characterized by
1. Prosopagnosia: impaired recognition of identity of familiar faces and/or
2. Associative agnosia: impaired recognition of object identity
D. Preserved perceptual matching and drawing reproduction
E. Preserved single-word repetition
F. Preserved ability to read aloud and write to dictation orthographically regular words
II. Supportive diagnostic features
A. Speech and language
1. Press of speech
2. Idiosyncratic word usage
3. Absence of phonemic paraphasias
4. Surface dyslexia and dysgraphia
5. Preserved calculation
B. Behavior
1. Loss of sympathy and empathy
2. Narrowed preoccupations
3. Parsimony
C. Physical signs
1. Absent or late primitive reflexes
2. Akinesia, rigidity, and tremor
D. Investigations
E. Neuropsychology
1. Profound semantic loss, manifest in failure of word comprehension and naming and/or face and object recognition
2. Preserved phonology and syntax, and elementary perceptual processing, spatial skills, and day-to-day memorizing
F. Electroencephalography: normal
G. Brain imaging (structural and/or functional): predominant anterior temporal abnormality (symmetric or asymmetric)
Features common to clinical syndromes of frontotemporal lobar degeneration
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List 4 Features common to clinical syndromes of frontotemporal lobar degeneration (extension of lists 1 through 3)
III. Supportive features
A. Onset before 65 years: positive family history of similar disorder in first-degree relative
B. Bulbar palsy, muscular weakness and wasting, fasciculations (associated motor neuron disease present in a minority of patients)
IV. Diagnostic exclusion features
A. Historical and clinical
1. Abrupt onset with ictal events
2. Head trauma related to onset
3. Early, severe amnesia
4. Spatial disorientation
5. Logoclonic, festinant speech with loss of train of thought
6. Myoclonus
7. Corticospinal weakness
8. Cerebellar ataxia
9. Choreoathetosis
E. Investigations
1. Brain imaging: predominant postcentral structural or functional deficit;
multifocal lesions on CT or MRI
2. Laboratory tests indicating brain involvement of metabolic or inflammatory disorder such as MS,
syphilis, AIDS, and herpes simplex encephalitis
V. Relative diagnostic exclusion features
A. Typical history of chronic alcoholism
B. Sustained hypertension
C. History of vascular disease (e.g., angina, claudication)
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