Sonographic detection of basal ganglia abnormalities in spasmodic dysphonia.

BACKGROUND AND PURPOSE: Abnormalities of the lenticular nucleus (LN) on transcranial sonography (TCS) are a characteristic finding in idiopathic segmental and generalized dystonia. Our intention was to study whether TCS detects basal ganglia abnormalities also in spasmodic dysphonia, an extremely focal form of dystonia. METHODS: Transcranial sonography of basal ganglia, substantia nigra and ventricles was performed in 14 patients with spasmodic dysphonia (10 women, four men; disease duration 16.5 ± 6.1 years) and 14 age- and sex-matched healthy controls in an investigator-blinded setting. RESULTS: Lenticular nucleus hyperechogenicity was found in 12 spasmodic dysphonia patients but only in one healthy individual (Fisher's exact test, P < 0.001) whilst other TCS findings did not differ. The area of LN hyperechogenic lesions quantified on digitized image analysis correlated with spasmodic dysphonia severity (Spearman test, r = 0.82, P < 0.001). CONCLUSION: Our findings link the underlying pathology of spasmodic dysphonia to that of more widespread forms of dystonia.

Spasmodic dysphonia and botulinum toxin: experience from the largest treatment series.

Spasmodic dysphonia is a focal laryngeal dystonia, with adductor, abductor, mixed, respiratory and singer's types. Our series over 24 years includes 1300 patients. 82% are of the adductor type; 63% were female; 12% had a positive family history and 82.4% had a focal distribution. All of the patients were managed with varying degrees of success with individualised dosing of botulinum neurotoxin A injected into the laryngeal musculature under EMG guidance.

Rhinocerebral mucormycosis: premortem diagnosis and therapy.

The diagnosis of rhinocerebral mucormycosis is most often made at autopsy. We report a series of nine patients in whom the diagnosis was established premortem. Six of the patients had underlying diabetes mellitus and three had acute leukemia. Facial or ocular pain was the complaint found in all patients, and frequently was the initial symptom. The diagnosis was established by examination and culture of infected tissue obtained by biopsy. In seven patients, identification of hyphal elements in smears of biopsy material allowed the immediate institution of amphotericin B therapy. Four of the seven patients treated with amphotericin B survived. All surviving patients had underlying diabetes mellitus and had undergone surgical debridement. Early diagnosis leading to immediate institution of appropriate therapy is most important for survival of patients with mucormycosis.

Laryngeal botulinum toxin injections for disabling stuttering in adults.

Stuttering is an action-induced speech disorder with involuntary, audible, or silent repetitions or prolongations in the utterance of short speech elements (sounds, syllables) and words. Symptomatic treatment programs frequently have initial success; persistent benefit is variable and many patients remain disabled. Stuttering has many characteristics similar to spasmodic dysphonia (laryngeal dystonia), often including the presence of adductor laryngeal spasms that obstruct airflow (glottal block). We hypothesized that relief of the spasmodic dysphonic glottal blocks in stutterers would modify the stuttering phenomenon and increase fluency. We therefore studied the effects of bilateral vocal fold injections of botulinum toxin type A (BTX) on dysfluency and speech characteristics in stuttering. We treated 14 adult patients (12 men, 2 women) with persistent stuttering and glottal block who previously failed standard speech therapy with 1.25 U BTX into each thyroarytenoid (vocalis) muscle. Fluency evaluations included the Stuttering Severity Instrument, the Perceptions of Stuttering Inventory, and a global rating scale (percent of normal function). Patients were evaluated at baseline and at 2-, 6-, and 12-week follow-up visits. Improvement in fluency documented by each rating instrument occurred at 2 and 6 weeks, with functional relapse by 12 weeks in most patients. We conclude that therapeutic laryngeal injections of botulinum toxin are useful in the management of stuttering with glottal block and result in a moderate improvement in fluency. When an adult patient with developmental stuttering with glottal blocks has failed speech interventional therapy and presents for treatment, a trial of BTX can be considered early.(ABSTRACT TRUNCATED AT 250 WORDS)

Postsurgical evaluation of uvulopalatopharyngoplasty: two case reports.

Uvulopalatopharyngoplasty (UPPP) has become a widely practiced procedure for the treatment of snoring and obstructive sleep apnea. Surgical outcome with UPPP has heretofore been assessed by investigators using the number of apneas per hour of sleep (apnea index) as the sole or primary criterion. We present two cases in which UPPP outcome was inadequately represented by the apnea index. It is suggested that the apnea/hypopnea index, extent of oxygen desaturations, degree of sleep fragmentation, level of daytime somnolence, interaction between respiratory function and sleep position and stages, and degree of respiratory strain be considered in the postsurgical evaluation.

Dyspnea in dystonia. A functional evaluation.

BACKGROUND: Dystonia consists of action-triggered sustained focal muscle contractions, worsened by effort, and resulting in voice changes, abnormal posturing, and dyspnea. The cause of dyspnea, previously unexplained, is the basis of this report. METHODS: Since the maximal efforts required to perform pulmonary function testing (PFT) could worsen the muscular contractions in dystonic patients, we used several tests to identify possible causes of dyspnea. These included spirometry with flow volume loops (FVL), tidal volume breathing, maximum voluntary ventilation (MVV), and inspiratory and expiratory muscle pressures (PImax, PEmax), sitting and supine. We used cycle ergometry with arterial blood gas (ABG) values to detect cardiac/pulmonary limitations and respiratory inductive plethysmography (RIP) to assess chest wall/abdominal movements for synchrony. Dynamic videofluoroscopy (VF) assessed and recorded the action-triggered muscle activity of the upper airways and the diaphragm during quiet breathing, speech, swallowing, and maximal respiratory maneuvers similar to the efforts required during PFT. RESULTS: Twenty-six dystonic patients, 12 women and 14 men, ages 14 to 70 years (mean age, 52.3 years) were evaluated. Their neurologic classification included 22 primary (idiopathic) and 4 secondary (2 postneuroleptic use, 2 posstraumatic). Four patients originally classified as having focal dystonia had dyspnea and were found to have diaphragmatic and/or upper airway dysfunction too. The PFTs showed abnormal FVL and/or tidal volume breathing patterns, with intermittent interruptions of air flow during inspiration or expiration in 20 of 24 patients. The VF was abnormal in 24 of 26 patients: 19 patients had combined upper airway (UA) and diaphragmatic dysfunction (DD); 1 patient had UA dysfunction alone, and 4 patients had DD alone. Except for poor effort and/or dystonic movements, cycle ergometry was normal in 18 of 21 patients. The ABG values and/or pulse oximetry were normal in 19 of 22 patients. CONCLUSION: Dyspnea in dystonia appears to be due to excessive and/or dysynchronized contractions of the upper airways and/or diaphragm, with usually normal gas exchange. These spasmodic and irregular muscular contractions during speech and daily activities are associated with the sensation of excessive effort to overcome the spasms. Excessive spasms can be triggered during PFT and are best detected on FVL patterns coupled with dynamic VF.

Inflammatory and obstructive disorders of salivary glands.

Obstructive and inflammatory diseases of the salivary glands can have a congenital, traumatic, metabolic, or infectious-inflammatory cause. The acute inflammatory conditions include bacterial and viral infections, and the chronic conditions include sialoliths, strictures, chronic sialadenitis, sialectasis, and lymphoepithelial disease. The neoplastic disease can cause obstruction and/or infection and often make the diagnosis elusive. In addition to a working knowledge of possible etiology, one needs experience with clinical examination, salivary analysis, sialography, CT scans, MRI, and fine-needle aspiration and cytology in order successfully to evaluate and manage patients with these conditions.

Computed tomography of the cervical lymph nodes: use of intravenous contrast enhancement.

Intravenous contrast administration increases the sensitivity of computed tomographic scanning for enlarged cervical lymph nodes but requires a detailed knowledge of neck anatomy, especially in order to distinguish certain normal vessels from involved nodal groups. Along the collar chain, relations between the parotid and submandibular salivary glands and the posterior and anterior facial veins and facial artery are analyzed. The digastric muscle is defined as a transitional landmark between collar and deep cervical nodes. Along the deep cervical chain, emphasis is on the internal jugular vein, its variability in size, and its relations to the anterior scalene and omohyoid muscles.

Regional behavioral variations of epidermoid carcinoma of the head and neck: a study in an animal model.

Regional behavioral differences have been observed clinically in epidermoid carcinoma of the head and neck. An animal model was developed for study, using homograft, transplanted epidermoid carcinoma in Fisher rats. Histopathologic review of tumors of the palate, base of tongue and larynx shows cartilage, arterial wall, surface epithelium and dense connective tissue containing elastic fibers to be relatively resistant to tumor invasion. Muscle, bone, vein and loose connective tissues were easily invaded. This correlates well with the known biochemical and immunological events in tumor invasion and host-tumor interactions. Regional metastasis occurred in two animals with base of tongue tumors. No distant metastases were found.

Botulinum toxin: basic science and clinical uses in otolaryngology.

The role of botulinum toxin as a therapeutic agent is expanding rapidly in otolaryngology. Botulinum toxin is a protease that blocks the release of acetylcholine from nerve terminals. Its effects are transient and nondestructive, and largely limited to the area in which it is administered. These effects are also graded according to dose, allowing for individualized treatment of patients and disorders. Botulinum toxin has been used primarily to treat disorders of excessive or inappropriate muscle contraction. In the field of otolaryngology, these include spasmodic dysphonia, oromandibular dystonia, and blepharospasm; vocal tics and stuttering; cricopharyngeal achalasia; various tremors and tics; hemifacial spasm; temporomandibular joint disorders; and a number of cosmetic applications. Botulinum toxin treatment has recently begun to show some benefit in the control of pain from migraine and tension headache. It may also prove useful in the control of autonomic dysfunction, as in Frey syndrome, sialorrhea, and rhinorrhea. In over 20 years of use in humans, botulinum toxin has accumulated a considerable safety record, and in many cases represents relief for thousands of patients unaided by other therapy.

Malignant fibrous histiocytoma of the head and neck.

Review of the literature reveals of 29 cases of fibrous histiocytoma of the deep structures of the head and neck. Three additional cases are reported: a multicentric case involving the submandibular gland with metastases to the lungs; and intraosseous tumor of the mandible locally invasive; and a submental tumor which rapidly recurred after simple excision. A metastatic potential of 22% is established. The historm, nomenclature, histopathology and clinical features are reviewed. The value of electron microscopy and tissue culture in arriving at the current diagnosis are stressed. Wide excision is the treatment of choice.

Botulinum toxin management of spasmodic dysphonia (laryngeal dystonia): a 12-year experience in more than 900 patients.

OBJECTIVES: This paper reviews a 12-year experience in more than 900 patients with spasmodic dysphonia who have been treated with botulinum toxin. STUDY DESIGN: This is a retrospective analysis of patients with adductor spasmodic dysphonia (strain-strangled voice), abductor spasmodic dysphonia (whispering voice), and adductor breathing dystonia (paradoxical vocal fold motion), all of whom have been treated with botulinum toxin injections for relief of symptom. METHODS: All of the patients were studied with a complete head and neck and neurologic examination; fiberoptic laryngostroboscopy; acoustic and aerodynamic measures; and a speech evaluation including the Universal spasmodic dysphonia rating scale. Some were given electromyography. All patients received botulinum toxin injections into the affected muscles under electromyographic guidance. RESULTS: The adductor patients had an average benefit of 90% of normal function lasting an average of 15.1 weeks. The abductor patients had an average benefit of 66.7% of normal function lasting an average of 10.5 weeks. Adverse effects included mild breathiness and coughing on fluids in the adductor patients, and mild stridor in a few of the abductor patients. CONCLUSION: Botulinum toxin A injection of the laryngeal hyperfunctional muscles has been found over the past 12 years to be the treatment of choice to control the dystonic symptoms in most patients with spasmodic dysphonia.

Patient survival factors in paranasal sinus mucormycosis.

Analysis of 170 cases of paranasal sinus mucormycosis collected from the literature and 9 cases of our own revealed a 50% mortality for this disease. When analyzed according to decade, survival has increased to 70% in the cases reported from 1970-1979. There were no significant differences between the survivors and the fatalities when evaluated according to age, sex, laterality, or radiographic findings. There was a markedly poorer prognosis for those patients with hemiplegia, facial necrosis, and nasal deformity. The underlying disease was an important determinant of survival: 75% of patients with no systemic disease, 60% of diabetics, and 20% of patients with other disorders survived. Surgical debridement or radical resection and the use of amphotericin B significantly increased survival. Their combination further enhanced survival, especially in the diabetic.

Localized injections of botulinum toxin for the treatment of focal laryngeal dystonia (spastic dysphonia).

Spastic dysphonia is a condition producing a strain-strangle phonation. We have previously classified most of these patients as having focal laryngeal dystonia, a disorder of central motor processing. The initial success of recurrent nerve section in many of these patients has been followed by recurrence of symptoms in months to years. Bilateral involvement of the vocal cords with hyperfunction of the nonparalyzed vocal cord could explain these failures. Injection of botulinum toxin (BOTOX) has been effective treatment for many focal dystonias. We have treated more than 100 patients with dystonia including five with laryngeal dystonia. All of the patients laryngeal had dramatic improvement after 48 to 72 hours; benefit lasted 3 to 9 months for each injection period. BOTOX injection can be performed on awake, ambulatory patients. Bilateral treatment and titration of dose can achieve the desired degree of weakness.

Clinical and laboratory characteristics of focal laryngeal dystonia: study of 110 cases.

Spastic dysphonia is a syndrome often producing a strain-strangle voice. We have previously classified most of these patients as having focal laryngeal dystonia, a disorder of central motor processing. In a study of 1,280 cases of dystonia registered at the Dystonia Clinical Research Center at the Columbia-Presbyterian Medical Center, we found 110 patients who had vocal cord involvement. These patients had historical information evaluated for age of onset (mean 34.6 years), duration of symptoms (mean 13.8 years), sex (1.4:1 female to male) family history (positive in 23%), and primary (66%) and secondary (34%) etiology; neurological evaluation for other dystonic involvement (25% with segmental cranial involvement, 23% with generalized dystonia) or tremor (irregular 23%, regular 6% on EMG). Treatment options were evaluated and included speech therapy, psychotherapy, biofeedback (with limited success), systemic medication (limited success except in abductor cases), nerve section (with late failure rate), and the use of botulinum toxin (improvement in all 34 injected patients).

Sleep-related laryngeal obstruction presenting as snoring or sleep apnea.

The difference between snoring (with or without sleep apnea) and laryngeal stridor resulting from laryngeal dysfunction may not be readily apparent. Two cases of Shy-Drager syndrome and one undiagnosed case in which laryngeal dysfunction was exacerbated by sleep are reported. Such dysfunction might create life-threatening situations for which emergency tracheostomy should be considered. The importance of differentiating stridor from snoring is discussed.

Delayed failure of myocutaneous flaps in head and neck reconstruction.

Since the concept of myocutaneous flap reconstruction of the head and neck region was introduced it has opened a new approach to surgical management in this area. This flap not only reduced the number of operations as compared to "staged procedure", but also reduced the costs of medical care. We are, however, experiencing significant failures as the applications of this flap are extended. This paper reviews our experience with myocutaneous flaps in 15 partial and total failures. An evaluation of these failures reveals that most occurred 1 1/2 to 3 weeks after reconstruction. The factors predisposing to failure seem related more to technical errors rather than to general factors. Diabetes, peripheral vascular disease, malnutrition and low hemoglobin, and low blood pressure were not major contributors to the failures in our series. Local factors predisposing to failure of myocutaneous flaps can be divided into two large categories--arterial failure and venous failure. In our series of unsuccessful myocutaneous flaps, the major factors appeared to be venous stasis leading to arterial insufficiency. All failures had developed after the initial critical period of flap survival (7-10 days). The following techniques showed an especially high rate of failure: 1. SCM--myocutaneous flap to resurface floor of mouth. 2. Tubed pectoralis myocutaneous flap to reconstruct pharynx and esophagus. 3. Flaps developed with very narrow vascular pedicles. Individual cases representing delayed failure are presented.

Clinical-pathological determinants in prognosis of fibrous histiocytomas of head and neck.

Review of the literature reveals 76 cases of fibrous histiocytoma of the deep structures of the head and neck. To this we have added 11 of our own cases, and analyzed the total group to determine clinical and histological features of a high risk population. Age, sex, presence of pain, location of tumor, size of tumor, and local invasion appear to be important clinical criteria. Bizarre giant cells, numerous and atypical mitoses, necrosis, and inflammation appear to be important histologic features. The treatment of choice is wide local excision. Chemotherapy appears to produce tumor regression.

Treatment of adductor laryngeal breathing dystonia with botulinum toxin type A.

Adductor laryngeal breathing dystonia (ALBD) is a rare disorder in which patients have persistent inspiratory stridor, usually normal voice, and cough. Physical exam is characterized by paradoxical movement of the vocal cords on inspiration. These patients have involuntary action-induced spasms of the adductor laryngeal muscles on inspiration. There has been no uniformly satisfactory treatment for the disease. Speech therapy, psychotherapy, and pharmacotherapy have all had limited success. We report the successful use of botulinum toxin type A in seven patients with adductor laryngeal breathing dystonia. All patients received bilateral thyroarytenoid injections. All patients had toxin effect within 72 hours, reaching maximal effect within 2 weeks with sustained improvement for an average of 13.8 weeks. Adverse effects included breathy voice and mild choking on liquids. Both resolved, on average, within 2 weeks. This retrospective study supports the safe and effective use of botulinum toxin type A in the treatment of adductor laryngeal breathing dystonia.

Abductor laryngeal dystonia: a series treated with botulinum toxin.

Abductor laryngeal dystonia (LD) is characterized by a hoarse voice quality which is broken up by breathy or whispered portions. Botulinum toxin injection (Botox) has been a safe and effective treatment for adductor laryngeal dystonia and is currently accepted medical therapy. As an extension of the established treatment program, in 1989 treatment of abductor LD was initiated. Thirty-two patients have been treated by sequential percutaneous electromyogram-guided (EMG) injections of the posterior cricoarytenoid (PCA) muscles. Most patients required treatment of both PCA muscles and improved to an average of 70% of normal voice. Patients who had a preexisting tremor, evidence of dystonia in other muscle groups, vocal tremor, or respiratory dysrhythmia had less improvement. Ten patients also required injection of the cricothyroid muscles and/or type I laryngoplasty.