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Biomedical subjects

J R Duffy

Publications and source records attributed to J R Duffy.

At least 19 recordsLinked to original sources

Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP.

OBJECTIVE: To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). METHODS: The authors conducted pathologic reexamination with the most recent immunohistochemistry of all cases diagnosed with FTLD, PSP, and CBD between 1970 and 2004. The authors also reviewed the early clinical features for clinical diagnosis and application of published research criteria. RESULTS: Of 127 cases analyzed, 57 had a pathologic diagnosis of FTLD, 49 PSP, and 21 CBD. Of these, 38 were clinically reclassified as frontal variant frontotemporal dementia (FTD), 13 as progressive non-fluent aphasia (PNFA), 21 as CBD-like, 33 as PSP-like, and 13 with frontotemporal dementia with coexisting motor neuron disease (FTD-MND). The authors were unable to classify nine cases. All cases of FTD-MND were tau-negative and had pathologic evidence of motor neuron degeneration. All cases classified as PSP-like or CBD-like had tau-positive pathology. Of the 13 cases with PNFA, PSP and CBD accounted for almost 70% of the cases, while FTD was almost equally divided between tau-positive and tau-negative diseases. CONCLUSION: Frontotemporal lobar degeneration, corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP) have overlapping clinical features. The prediction of tau-positive pathology from a CBD or PSP-like presentation is good, while the frontotemporal dementia (FTD)-motor neuron disease syndrome almost certainly predicts motor neuron degeneration. Surprisingly, PSP and CBD accounted for most cases classified as progressive non-fluent aphasia. Frontal variant FTD is an unpredictable disease in terms of its biochemistry.

Aged↗

Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia.

Progressive supranuclear palsy (PSP) is a clinicopathological entity typically presenting as an akinetic rigid syndrome with early falls, axial rigidity, vertical supranuclear gaze palsy and levodopa resistance. Pathological features consist of tau deposition in neuronal and glial cells located mainly in subcortical and brainstem structures. Rare cases with the pathological diagnosis of atypical PSP have been described in which neocortical tau deposition is more widespread than what is usually seen in typical PSP. Progressive nonfluent aphasia (PNFA) is a syndrome characterized by spontaneous nonfluent speech and early preserved comprehension of language. Apraxia of speech (AOS) is a motor speech disorder that may be a feature of PNFA. We report the clinical and pathological findings of four cases that presented with features most consistent with PNFA predominated by AOS. Pathological features in these four cases included the typical features of PSP subcortically and in brainstem structures, but combined with tau-positive neuronal and glial pathology in the neocortex. Comprehensive semiquantitative analyses of tau burden including neurofibrillary tangles and pretangles, coiled bodies, tufted astrocytes and threads were undertaken in the four cases of atypical PSP and compared to 10 cases of typical PSP. Semiquantitative analysis demonstrated that in atypical PSP, the pathology shifts from subcortical grey and brainstem regions, commonly affected in typical PSP, towards neocortical regions. This shift in pathology accounts for the presentation of PNFA and AOS observed in our patients, as well as the lack of classic features of PSP. These cases demonstrate that atypical PSP can present as AOS and PNFA without the classic features of PSP.

Adult↗

Yes/no reversals as neurobehavioral sequela: a disorder of language, praxis, or inhibitory control?

This study identifies a linguistic phenomenon suggestive of damage to fronto-subcortical circuitry. Our objective was to determine the occurrence and neuroradiological/neurobehavioral correlates of yes/no reversals in corticobasal degeneration (CBD), and document occurrence of reversals in other neurological conditions. In a prospective study, we evaluated 34 CBD patients using a neuropsychologic battery and magnetic resonance imaging. Patients were subdivided into two groups: those with (n = 11) and without (n = 23) yes/no reversals. In a retrospective study conducted during the period of 1991-2001, we identified 33 patients for whom yes/no reversals occurred to compare correlates with prospective study findings. In the prospective study, 11 patients (32.3%) had yes/no reversals. Significant between-group differences were found in scores of lexical fluency (P = 0.02) and prehension (P = 0.03). Prehension scores correlated with facial praxis (P < 0.0001) and upper limb praxis scores (P < 0.0001) in the yes/no reversal group only. In the retrospective study, nine CBD patients and 24 non-CBD patients had yes/no reversals, with damage to fronto-subcortical areas present in all patients. Results suggest an association with deficits in mental flexibility and inhibitory control. High within-group correlations of lexical fluency and prehension with praxis scores suggest a relationship of yes/no reversals with multiple factors.

Aged↗

Clinicoanatomic studies in dysarthria: review, critique, and directions for research.

More than 30 years ago, Darley, Aronson, and Brown (1969) proposed clinicoanatomic correlations for seven perceptual types of dysarthria. These correlations have not been systematically re-examined even though imaging technologies developed in recent years provide the means to do so. This review considers data from published imaging studies as well as data from selected medical interventions to evaluate the current state of knowledge that relates lesion site to the nature of a speech disturbance. Although the extant data are not sufficient to allow a complete evaluation of the seven types of dysarthria described by Darley et al., relevant information has been reported on lesions of the pyramidal pathway, extrapyramidal pathway, and cerebellum. In general, the results are best explained by an equivalence mode of brain-behavior relationship in which a type of dysarthria is associated with a lesion in one of two or more brain structures. Criteria also are proposed for future studies of clinicoanatomic relationships in neurogenic communication disorders.

Brain↗

Awake craniotomy for aggressive resection of primary gliomas located in eloquent brain.

OBJECTIVE: To determine with intraoperative neurologic and language examinations the maximal tumor resection achievable with acceptable postoperative neurologic dysfunction in patients undergoing awake stereotactic glial tumor resection in eloquent regions of the brain. PATIENTS AND METHODS: Between October 1995 and December 2000, 65 patients underwent frameless stereotactic resection of glial tumors located in functioning tissue. During the resection, continuous examinations by a neurologist and speech pathologist were performed. The goal of surgery was to resect the maximum neurologically permissible tumor volume defined on preoperative T2 imaging. Tumor resection was stopped at the onset of neurologic dysfunction. Novel segmentation software was used to measure tumor cytoreduction based on pre- and postoperative magnetic resonance imaging. All patients underwent 3-month postoperative neurologic examinations to determine functional outcomes. RESULTS: The cortical and subcortical white matter tracts at risk for injury were the left frontal operculum in 15 patients, the central lobule in 38, the insula in 11, and the left angular gyrus in 1. Thirty-four (52%) had a greater than 90% reduction in T2 signal postoperatively. In 26 patients thought to have low-grade tumors based on preoperative imaging, 12 proved to have grade 3 gliomas. Forty-eight patients (74%) developed intraoperative deficits; 34 (71%) recovered to a modified Rankin grade of 0 or 1 at 3 months postoperatively, 11 (23%) achieved a modified Rankin grade of 2, and 3 patients (6%) achieved a modified Rankin grade of 3 or 4 at 3-month follow-up. There was no operative mortality; 17 patients (26%) died from tumor progression during the follow-up period. CONCLUSIONS: Combining frameless computer-guided stereotaxis with cortical stimulation and repetitive neurologic and language assessments facilitates tumor resection in functioning brain regions. Resecting tumor until the onset of neurologic deficits allows for a good functional recovery. Imaging software can objectively and accurately measure preoperative and postoperative tumor volumes.

Adult↗

Ataxic dysarthria.

Although ataxic dysarthria has been studied with various methods in several languages, questions remain concerning which features of the disorder are most consistent, which speaking tasks are most sensitive to the disorder, and whether the different speech production subsystems are uniformly affected. Perceptual and acoustic data were obtained from 14 individuals (seven men, seven women) with ataxic dysarthria for several speaking tasks, including sustained vowel phonation, syllable repetition, sentence recitation, and conversation. Multidimensional acoustic analyses of sustained vowel phonation showed that the largest and most frequent abnormality for both men and women was a long-term variability of fundamental frequency. Other measures with a high frequency of abnormality were shimmer and peak amplitude variation (for both sexes) and jitter (for women). Syllable alternating motion rate (AMR) was typically slow and irregular in its temporal pattern. In addition, the energy maxima and minima often were highly variable across repeated syllables, and this variability is thought to reflect poorly coordinated respiratory function and inadequate articulatory/voicing control. Syllable rates tended to be slower for sentence recitation and conversation than for AMR, but the three rates were highly similar. Formant-frequency ranges during sentence production were essentially normal, showing that articulatory hypometria is not a pervasive problem. Conversational samples varied considerably across subjects in intelligibility and number of words/ morphemes in a breath group. Qualitative analyses of unintelligible episodes in conversation showed that these samples generally had a fairly well-defined syllable pattern but subjects differed in the degree to which the acoustic contrasts typical of consonant and vowel sequences were maintained. For some individuals, an intelligibility deficit occurred in the face of highly distinctive (and contrastive) acoustic patterns.

Adolescent↗

Stroke with dysarthria: evaluate and treat; garden variety or down the garden path?

The case of a man who initially presented to an emergency room with an isolated dysarthria is reviewed in order to demonstrate how the history, clinical context, and clinical observations can lead to accurate or inaccurate diagnosis. The diagnostic process in this case illustrates (1) the value of experience in clinical practice; (2) the role of the history and clinical context in diagnosis, but their occasional capacity to mislead; (3) the importance of distinguishing evidence from inference; (4) the use of deductive strategies and pattern recognition in speech diagnosis; (5) the importance of diagnostic vigilance; (6) the value of distinguishing among dysarthria types; and (7) the contribution that the diagnosis of speech disorders can make to the localization and diagnosis of neurologic disease.

Aged↗

Telemedicine and the diagnosis of speech and language disorders.

OBJECTIVE: To summarize results of telemedicine evaluations of speech and language disorders in patients in a small, rural hospital and in large multidisciplinary medical practices. MATERIALS AND METHODS: Eight patients underwent assessment as part of experiments with the National Aeronautics and Space Administration-launched Advanced Communications Technology Satellite. A second clinician was on-site with patients to assess the reliability of satellite observations. Twenty-four previously videotaped samples of speech disorders were also transmitted to assess agreement with original face-to-face clinical diagnoses. In addition, results of 150 telemedicine evaluations among Mayo Clinic practices in Minnesota, Arizona, and Florida were examined retrospectively. RESULTS: Evaluations were reliable, and patient satisfaction was good. Diagnoses were consistent with lesion localization and medical diagnosis when they were known, and they frequently had implications for lesion localization and medical diagnosis and management when they were previously unknown. The frequency of uncertain diagnosis (13%) for evaluation among the Mayo practices was only slightly higher than that encountered in face-to-face practice. Face-to-face evaluations were considered necessary for only 6 of the 150 patients (4%). CONCLUSION: Telemedicine evaluations can be reliable, beneficial, and acceptable to patients with a variety of acquired speech and language disorders, both in rural settings and within large multidisciplinary medical settings.

Adult↗

A cardiac service line approach to patient-centered care.

The traditionally organized, functionally structured nature of hospitals is no longer affordable in today's economic environment. Managed care, the outcomes movement, and changed stakeholders have challenged hospitals to become more patient centered with seamless, streamlined operations. A service line approach focusing on patient populations across a broad continuum of care offers a system that maximizes results. Strong leadership that emphasizes a clear vision of the future, a process-oriented organizational structure, multidisciplinary clinical practice, performance-based management, and broadened roles that emphasize lifelong learning have contributed to the success of the cardiac service at Virginia Heart Center. Nurse managers have taken the lead at this center in achieving superior performance.

Cardiology Service, Hospital↗

Associative anomia: dissociating words and their definitions.

A 60-year-old woman, in whom magnetic resonance imaging and single photon emission computed tomography confirmed the presence of chronic damage to the left temporal lobe, had pure anomia with sparing of other language and cognitive functions. She could independently access lexical knowledge (words) and semantic knowledge (word definitions). We postulate that her anomia was due to a failure to associate lexical and semantic knowledge.

Anomia↗

Speech disorders in systemic amyloidosis.

The purpose of this study was to define more completely the speech disorders that can occur in systemic amyloidosis. Fifty-nine patients with histologically established primary systemic amyloidosis were studied. Of this group, 81% had abnormal voice quality, 43% had abnormal pitch, 7% had abnormal resonation and 15% had abnormal articulation. Twenty-two patients also had abnormal findings at laryngeal examination, consisting of bowing, oedema, thickening, dry irritation and polypoid changes of the vocal folds. Macroglossia was found in 17% of the patients and submandibular swelling in 10%. These results support previous studies which showed that abnormal phonation is a sign of systemic amyloidosis and that abnormal voice is by far the most common abnormal speech sign. The relationship between amyloid deposit and dysphonia was not clear. In 23 patients who had laryngeal examinations, no evidence of actual amyloid deposits was documented.

Adult↗

An investigation of body part as object (BPO) responses in normal and brain-damaged adults.

A test of simple pantomime was administered to three groups of adults and comparisons were made across groups of the incidence of subjects who exhibited body part as object (BPO) responses and of the mean frequency of occurrence of BPO in each group. The three groups were left-hemisphere-damaged aphasics (N = 28), right-hemisphere-damaged (N = 24), and normal controls (N = 28). The results indicated no significant differences among groups on the BPO measures. Also, to test the strength of association between the frequency of occurrence of BPO and measures of limb apraxia and severity of aphasia for the left-hemisphere-damaged aphasic group, correlation coefficients were obtained. The correlations were low and nonsignificant. The results of this investigation do not support the common clinical assumption that the occurrence of BPO during the performance of simple pantomimes is pathognomic for left-hemisphere pathology or associated with limb apraxia.

Adult↗

Computer-assisted stereotactic ventralis lateralis thalamotomy with microelectrode recording control in patients with Parkinson's disease.

Stereotactic ventralis lateralis thalamotomy can be performed in selected patients with medically intractable parkinsonian tremor and rigidity. New technology, including computed tomography-based stereotaxis and microelectrode recording techniques, provides a data base for precise localization of thalamic lesions tailored to each patient and thus reduces the risk associated with such a procedure. At our institution, 12 patients with medically intractable parkinsonian tremor have undergone this procedure; all experienced alleviation or cessation of the tremor and no permanent disabling neurologic sequelae.

Adult↗

Comparison of the performances of a fluent and a nonfluent aphasic on a pantomimic referential task.

The pantomimic performances of a typical Broca's (nonfluent) and Wernicke's (fluent) aphasic were compared with each other and with four normal control subjects on a simple task of nonverbal referential communication. Both aphasic subjects demonstrated only about 50% accuracy in their pantomimic communication. Also, measures were obtained of the motoric fluency of the pantomimes of all subjects and comparisons were made between the fluent and nonfluent aphasics. These measures demonstrated distinct differences in the fluency patterns of the pantomimes of the two aphasic subjects similar to the differences in speech fluency which distinguish and characterize these two types of aphasia; that is, the fluent aphasic pantomimed fluently and like the control subjects and the nonfluent aphasic pantomimed nonfluently. The quantitative and qualitative similarities in the fluency patterns of the speech and the pantomimic behaviors of the aphasic subjects are discussed in terms of their implications for a definition of aphasia.

Adult↗

The effect of response choice relatedness on pantomime and verbal recognition ability in aphasic patients.

Twenty aphasic and twenty control subjects were examined on two versions of a pantomime recognition and a verbal recognition test; one version containing response choices conceptually/semantically related to target responses, the other containing unrelated response choices. The aphasic group was inferior to controls on the pantomime and verbal recognition tests, although the magnitude and consistency of the differences between aphasics and controls were greater for the pantomime test. The presence of conceptually/semantically related response choices on the pantomime recognition test significantly reduced test scores of aphasic patients, and this reduction was related to the magnitude of the effect of response choice relatedness on their verbal recognition test performance. The findings are consistent with an interpretation of aphasia as an impairment of a central symbolic ability which can affect processing for meaning in both verbal and nonverbal modes of communication.

Adult↗

Three studies of deficits in pantomimic expression and pantomimic recognition in aphasia.

Studies were conducted to investigate aphasic deficits in pantomimic behaviors. Three groups of subjects were used: 47 aphasics; 27 right-hemisphere-damaged; and 11 controls. Study I replicates a previous study of pantomimic recognition deficits (Duffy, Duffy, & Pearson, 1975) and essentially duplicates the previous findings of significant deficits of pantomimic recognition in aphasic subjects that are highly correlated with their verbal deficits. Study II examines the relationship between deficits in pantomimic recognition and expression; and the relationships between these two nonverbal behaviors and aphasic verbal deficits. Zero order correlations, partial correlations, and multiple regression analyses are presented. The results show that aphasics exhibit significant deficits in both pantomimic expression and recognition; and, that both of these are highly correlated with aphasic verbal deficits. Study III is an investigation of four causal theories of aphasic deficits in pantomimic expression. Zero order correlations, partial correlations, and multiple regression analyses are presented. It is concluded that aphasic pantomimic expressive deficits are not caused by general intellectual deficit or limb apraxia; but, they are associated with a central symbolic disorder or a verbal mediation deficit. The implications of these studies for an understanding of the nature of aphasia as a syndrome which includes both verbal and nonverbal impairments are discussed.

Aphasia↗

Path analysis: a strategy for investigating multivariate causal relationships in communication disorders.

This tutorial paper explains, illustrates, and discusses path analysis, a powerful strategy for examining the plausibility and degree of causal relationships which are postulated to exist among a set of variables. In addition to its strength as a method of theory testing, path analysis is highly compatible with the kind of retrospective research which is so often used to study speech and language pathologies. One of its major advantages is that it allows explicit recognition of multiple, interacting causes of communication disorders and allows the researcher to evaluate the logical consequences of assumptions made about the specific nature of those causal relationships. A summary of the meaning of causal relationships is followed by a presentation of the principles underlying path analysis, as well as an outline of its basic procedures. Also presented is an illustrative example of the application of path analysis to the evaluation of some competing causal theories of a communication disorder. Finally, the general advantages of path analysis and its applicability to the investigation of causal relationships in speech and language pathologies are summarized and discussed.

Humans↗