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.

Dysarthria and apraxia of speech associated with FK-506 (tacrolimus).

The immunosuppressive agent FK-506 (tacrolimus) is one of the agents most commonly used to prevent rejection after liver transplantation. Neurologic toxicity related to FK-506 has been reported, including speech disorders; however, a detailed analysis of the speech disorder associated with use of FK-506 has not been presented. Herein we describe a patient who exhibited mutism, then severe apraxia of speech with a concomitant hypokinetic, spastic, and ataxic dysarthria after administration of FK-506. His residual mixed dysarthria, without radiographic evidence of a structural lesion, suggests dysfunction of one or more neurochemical systems. The pathophysiologic mechanisms underlying this intriguing entity remain obscure.

Hereditary motor and sensory neuropathy with diaphragm and vocal cord paresis.

We describe two kindreds with an autosomal dominant inherited disorder characterized by a variable degree of muscle weakness of limbs, vocal cords, and intercostal muscles and by asymptomatic sensory loss, beginning in infancy or childhood in severely affected persons. Life expectancy in severely affected patients is shortened because of respiratory failure. Because nerve conduction velocities are normal and it is an inherited axonal neuropathy, we classify the disorder as a variety of hereditary motor and sensory neuropathy type II (HMSN II) (HMSN IIc). The present report provides further evidence for heterogeneity among the hereditary motor and sensory neuropathy type II disorders. In one large pedigree with the type IIc disorder, no linkage to DNA markers known to map near the HMSN IA locus on chromosome 17p or the HMSN IB locus on chromosome 1q was demonstrated.

Botulinum toxin injection for adductor spastic dysphonia: patient self-ratings of voice and phonatory effort after three successive injections.

Ten patients (aged 35 to 70 years) with neurologic adductor spastic dysphonia rated themselves on a 7-point scale of severity for degree of voice improvement and physical effort after a series of three injections of botulinum toxin. Symptoms were noticeably reduced 24 and 48 hours after injection; this improvement was followed by considerable fluctuations in voice quality and phonatory effort. With successive injections, patients differed in their post-injection experiences, the time required to reach optimal voice, and the total duration of benefit. The study shows that the course of voice change after botulinum toxin injection is not predictable, uniform, or equal among patients with spastic dysphonia.

Rapid voice tremor, or "flutter," in amyotrophic lateral sclerosis.

In an attempt to clarify the origin and frequency characteristics of a rapid voice tremor, or "flutter," in patients with amyotrophic lateral sclerosis (ALS), eight patients (four men and four women; ages 42 to 70 years) who had ALS and rapid voice tremor and an age- and sex-matched control group of eight subjects were asked to sustain the vowel /a/ and their voices were recorded for later analysis. Each segment of phonation was demodulated into amplitude and frequency components. From each subject's 8-second amplitude and frequency signals, a fast Fourier transform analysis was done on a 1-second segment previously identified perceptually as having the most apparent tremor or flutter. The results showed that patients with ALS had multiple combinations of levels and frequencies for amplitude and frequency modulations in comparison with control subjects, who had consistently low levels of modulations. In an attempt to quantify the tremor or flutter in ALS, amplitude and frequency modulations were not clearly or predominantly represented at one point along the spectrum. Nevertheless, these frequency and amplitude modulations are more prominent in patients with ALS than in normal subjects. The origins of these aberrant frequency and amplitude modulations in ALS patients remain obscure, although speculation is that they are of peripheral rather than central nervous system origin.

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.

Double-blind controlled study of botulinum toxin in adductor spasmodic dysphonia.

The treatment of adductor spasmodic dysphonia using botulinum toxin A was conducted in 13 patients as a double-blind, placebo-controlled study. Patients were diagnosed independently by an interdisciplinary team consisting of speech pathologists, an otolaryngologist, and a neurologist. The toxin or saline was injected into each thyroarytenoid muscle under electromyographic and laryngoscopic guidance. Botulinum toxin A markedly reduced perturbation, decreased fundamental frequency range, and improved the spectrographic characteristics of the voice. Fundamental frequency and phonation time remained unchanged. Patients injected with botulinum toxin A noticed significant improvement in their voices in comparison with the placebo-treated group. Excessive breathiness of the voice occurred in two patients, and mild bleeding in one patient in the botulinum toxin A-treated group. Injection with saline resulted in edema of the vocal cord in one patient. Botulinum toxin A proved to be an effective and safe treatment of adductor spasmodic dysphonia.

The relationship between psychopathology and speech and language disorders in neurologic patients.

Depression, anxiety, and conversion reaction are common in neurologic patients. These disorders can produce neurologic-like symptoms that either mask or intensify those produced by the neurologic disease. This paper reviews clinical and research findings relating psychopathology to the formation and remediation of speech and language disorders in neurologic patients. The need to consider the psychosocial and psychopathological aspects of neurologic communicative disorders, and the link between emotional and communicative processes, are emphasized. Diagnostic criteria for the identification of psychogenic communicative disorders are outlined.

Clinical studies in psychogenic stuttering of adult onset.

Twelve patients (6 men and 6 women), ages 21-79 years, who, as far as it could be determined from their case histories, began to stutter for the first time in adulthood were classified by examining physicians and speech-language pathologists as having psychogenic stuttering. The case histories of these patients were examined to determine the details of their speech and allied complaints, the course and duration of stuttering, and if any psychologically stressful events surrounded the onset and development of the disorder. Minnesota Multiphasic Personality Inventory (MMPI) data on 10 of the 12 patients were analyzed independently. The main findings were (a) stuttering was the chief complaint of 11 patients; (b) all 12 patients had additional neurologic-like complaints that turned out to be nonorganic; (c) duration of stuttering from onset to examination ranged from 4 hr to 1.5 years; (d) the stuttering of most patients was variable in severity and returned to normal either spontaneously or as a result of speech therapy or psychotherapy; (e) psychologic stress was associated with the onset in 10 patients: the stress was chronic in 4 patients and acute in 6; (f) MMPI profiles of 9 of 10 patients were abnormal, with four profiles reflective of a conversion reaction; (g) differentiating neurologic from psychogenic stuttering and the need for interdisciplinary collaboration were essential in all 12 cases; and (h) speech therapy and psychotherapy are justified when psychogenic stuttering is diagnosed.

Tongue strength and alternate motion rates in normal and dysarthric subjects.

Tongue strength and alternate motion rates were measured in 50 normal subjects and in 18 subjects who had different neuropathologic types of dysarthria. The dysarthric subjects did not differ significantly from one another on any of the test measures. However, as a group they differed significantly from their normal counterparts in that they demonstrated weaker tongue strength, reduced and unsustained levels of maximum tongue strength effort, and slower alternate motion rates. Clinical implications are discussed.

Judgment of voice improvement after recurrent laryngeal nerve section for spastic dysphonia: clinicians versus patients.

Four or more years after recurrent laryngeal nerve section for adductor spastic dysphonia, 25 patients assessed their voice quality and phonatory effort in relation to presurgical status, using categorical ratings (gradations of better or worse, and of easier and harder) and also numerical ratings. Quality was judged better by 88%, and effort easier by 84%; but many thought the improvement was only moderate or slight. Three speech pathologists, assessing presurgical and current recordings of the patient's voices numerically, rated the majority of the better and easier voices much closer to their presurgical status than to normal. Clinicians were highly consistent and reliable in their assessments. Patients were often in disagreement with clinician ratings and generally rated their dysphonia as less severe than the clinicians did. These discrepancies are discussed within the context of contradictory opinions regarding the efficacy of recurrent laryngeal nerve section for the treatment of adductor spastic dysphonia.

Clinician reliability in rating voice improvement after laryngeal nerve section for spastic dysphonia.

A recent study by Aronson and DeSanto (1983) showed that, although section of the recurrent laryngeal nerve to relieve adductor spastic dysphonia effected considerable improvement in nearly 100% of the voices immediately after surgery, within the next 3 years 64% had returned to their preoperative status or worse. These findings were based on ratings by one speech pathologist, and the study was not designed to measure the reliability of the rater's judgments.