Abstract
Progressive nonfluent aphasia (PNFA) is a clinical syndrome characterized by motor speech impairment and agrammatism, with relative sparing of single word comprehension and semantic memory. PNFA has been associated with the characteristic pattern of left anterior insular and posterior frontal atrophy, including the motor and premotor regions and Broca's area. Postmortem histopathologic evidence has shown that PNFA is usually associated with tau pathology, although focal Alzheimer disease pathology and tau-negative, ubiquitin-TDP-43 inclusions also have been reported in association with this clinical syndrome. We performed a detailed analysis of motor speech errors in 18 patients with PNFA and investigated their neural correlates using voxel-based morphometry on magnetic resonance imaging scans. Seven patients demonstrated only apraxia of speech (AOS) errors, whereas 11 showed AOS along with dysarthria. Slow rate of speech, effortful articulation with groping, and consonant distortions were the most common AOS errors. Hypernasality was the most represented dysarthric feature and dysarthria was most often classified as spastic, hypokinetic, or mixed spastic-hypokinetic. Neuroimaging results demonstrated that patients with AOS-only and AOS plus dysarthria showed atrophy in the left posterior frontal, anterior insular, and basal ganglia regions when compared with controls. Patients with AOS plus dysarthria showed greater damage than patients with AOS-only in the left face portion of primary motor cortex and left caudate. PNFA is a distinct frontotemporal lobar degeneration clinical syndrome associated with characteristic clinical, neuroimaging, and pathologic features. The clinical features are driven by the severity of left frontal and caudate damage.
| Original language | English (US) |
|---|---|
| Journal | Alzheimer Disease and Associated Disorders |
| Volume | 21 |
| Issue number | 4 |
| DOIs | |
| State | Published - Oct 2007 |
| Externally published | Yes |
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Keywords
- Apraxia of speech
- Dysarthria
- FTLD
- Progressive aphasia
- Progressive nonfluent aphasia
- VBM
ASJC Scopus subject areas
- Geriatrics and Gerontology
- Psychiatry and Mental health
- Neuroscience(all)
Cite this
Progressive nonfluent aphasia and its characteristic motor speech deficits. / Ogar, Jennifer M.; Dronkers, Nina; Brambati, Simona M.; Miller, Bruce L.; Gorno-Tempini, Maria Luisa.
In: Alzheimer Disease and Associated Disorders, Vol. 21, No. 4, 10.2007.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Progressive nonfluent aphasia and its characteristic motor speech deficits
AU - Ogar, Jennifer M.
AU - Dronkers, Nina
AU - Brambati, Simona M.
AU - Miller, Bruce L.
AU - Gorno-Tempini, Maria Luisa
PY - 2007/10
Y1 - 2007/10
N2 - Progressive nonfluent aphasia (PNFA) is a clinical syndrome characterized by motor speech impairment and agrammatism, with relative sparing of single word comprehension and semantic memory. PNFA has been associated with the characteristic pattern of left anterior insular and posterior frontal atrophy, including the motor and premotor regions and Broca's area. Postmortem histopathologic evidence has shown that PNFA is usually associated with tau pathology, although focal Alzheimer disease pathology and tau-negative, ubiquitin-TDP-43 inclusions also have been reported in association with this clinical syndrome. We performed a detailed analysis of motor speech errors in 18 patients with PNFA and investigated their neural correlates using voxel-based morphometry on magnetic resonance imaging scans. Seven patients demonstrated only apraxia of speech (AOS) errors, whereas 11 showed AOS along with dysarthria. Slow rate of speech, effortful articulation with groping, and consonant distortions were the most common AOS errors. Hypernasality was the most represented dysarthric feature and dysarthria was most often classified as spastic, hypokinetic, or mixed spastic-hypokinetic. Neuroimaging results demonstrated that patients with AOS-only and AOS plus dysarthria showed atrophy in the left posterior frontal, anterior insular, and basal ganglia regions when compared with controls. Patients with AOS plus dysarthria showed greater damage than patients with AOS-only in the left face portion of primary motor cortex and left caudate. PNFA is a distinct frontotemporal lobar degeneration clinical syndrome associated with characteristic clinical, neuroimaging, and pathologic features. The clinical features are driven by the severity of left frontal and caudate damage.
AB - Progressive nonfluent aphasia (PNFA) is a clinical syndrome characterized by motor speech impairment and agrammatism, with relative sparing of single word comprehension and semantic memory. PNFA has been associated with the characteristic pattern of left anterior insular and posterior frontal atrophy, including the motor and premotor regions and Broca's area. Postmortem histopathologic evidence has shown that PNFA is usually associated with tau pathology, although focal Alzheimer disease pathology and tau-negative, ubiquitin-TDP-43 inclusions also have been reported in association with this clinical syndrome. We performed a detailed analysis of motor speech errors in 18 patients with PNFA and investigated their neural correlates using voxel-based morphometry on magnetic resonance imaging scans. Seven patients demonstrated only apraxia of speech (AOS) errors, whereas 11 showed AOS along with dysarthria. Slow rate of speech, effortful articulation with groping, and consonant distortions were the most common AOS errors. Hypernasality was the most represented dysarthric feature and dysarthria was most often classified as spastic, hypokinetic, or mixed spastic-hypokinetic. Neuroimaging results demonstrated that patients with AOS-only and AOS plus dysarthria showed atrophy in the left posterior frontal, anterior insular, and basal ganglia regions when compared with controls. Patients with AOS plus dysarthria showed greater damage than patients with AOS-only in the left face portion of primary motor cortex and left caudate. PNFA is a distinct frontotemporal lobar degeneration clinical syndrome associated with characteristic clinical, neuroimaging, and pathologic features. The clinical features are driven by the severity of left frontal and caudate damage.
KW - Apraxia of speech
KW - Dysarthria
KW - FTLD
KW - Progressive aphasia
KW - Progressive nonfluent aphasia
KW - VBM
UR - http://www.scopus.com/inward/record.url?scp=37349031049&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=37349031049&partnerID=8YFLogxK
U2 - 10.1097/WAD.0b013e31815d19fe
DO - 10.1097/WAD.0b013e31815d19fe
M3 - Article
VL - 21
JO - Alzheimer Disease and Associated Disorders
JF - Alzheimer Disease and Associated Disorders
SN - 0893-0341
IS - 4
ER -