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[Hypokalemic periodic paralysis: new advances].

We present a 56-year-old man with an episode of hypokalemic periodic paralysis. There are several interesting aspects of this rare disease, such as the suggestive history and clinical signs (sudden onset, symmetrical paralysis mainly of the lower extremities, "walking-off" phenomenon, no symptoms or signs between attacks) or the unexplained episodic character of the attacks. It belongs to the recently recognized ion channel diseases, a group of disorders in which mutations of genes encoding the channel protein are responsible for defective channel function. Molecular studies of mutated channels or channel subunits should provide insight into channel functioning and help to develop effective drug treatment. Once diagnosis of hypokalemic periodic paralysis is established, treatment and regular follow-up are necessary to prevent the development of permanent muscle weakness. The increasing mobility of the world's population will add to the frequency of thyrotoxic hypokalemic periodic paralysis (which is relatively frequent in Asian populations) even in Western countries.

Calcium Channels↗

Cricopharyngeal myotomy and arytenoid adduction in the management of combined laryngeal and pharyngeal paralysis.

Aspiration caused by unilateral combined laryngeal and pharyngeal paralysis is a serious clinical problem. This article reviews the abnormal physiology, which includes glottal incompetence, impaired pharyngeal propulsion, and sensory loss, and reports the results of surgical treatment of, 13 patients who had significant dysphagia and aspiration caused by unilateral laryngeal and pharyngeal paralysis. In eight patients the cause was surgical resection or trauma of the tenth cranial nerve, with concomitant twelfth nerve injury in four. Four patients had central lesions. One patient had a surgical tenth cranial nerve injury as well as a stroke. Thyroplasty, alone in two patients, and combined with cricopharyngeal myotomy in a third, improved voice, but not swallowing. In all patients treated by arytenoid adduction plus cricopharyngeal myotomy, aspiration was eliminated, and patients gained weight. Six of these patients had been dependent on enteral tube feedings. The results support the safety and efficacy of simultaneous arytenoid adduction and cricopharyngeal myotomy for dysphagia caused by combined laryngeal and pharyngeal paralysis.

Adolescent↗

Impairment of muscle stretch reflexes in tick paralysis.

Experiments with tick-paralyzed dogs and woodchucks have shown impairment of stretch reflexes in addition to partial paralysis at the neuromuscular junction. Stretch reflexes disappear very early during paralysis, whereas nociceptive reflexes do not appear to be directly affected. The early incoordination and the ascending nature of the paralysis may be related to impairment of stretch reflex pathways.

Animals↗

Observations on so-called idiopathic vocal cord paralysis.

A series of 238 patients with laryngeal paralysis seen during a six-year period (1976-1982) were studied with serial 16-mm motion picture filmstrips. There were 39 cases in which no etiological disease process could be found responsible for the paralysis; these were called "idiopathic." In all 39 cases, both the superior and recurrent laryngeal nerves were paralyzed indicating that the lesion involved the central (nuclear) lower motor neurons of the dorsal and ventral nucleus ambiguus. The high incidence of upper respiratory infections suggests a viral etiology in many cases. The ability to separate a pure recurrent from a combined superior and recurrent laryngeal paralysis on morphological indirect endoscopic appearance has significant diagnostic and therapeutic implications.

Diagnosis, Differential↗

Lack of association of the potassium channel-associated peptide MiRP2-R83H variant with periodic paralysis.

A missense variant (R83H) of the gene (KCNE3) encoding a potassium channel-associated peptide, MinK-related peptide 2 (MiRP2), has been reported in periodic paralysis patients. In the current study, no difference in the frequency of the MiRP2-R83H variant between periodic paralysis patients and healthy individuals was found. Furthermore, there was no segregation of this gene variant with the disease. These observations weaken the proposal that MiRP2-R83H causes periodic paralysis.

Adult↗

Early perioperative use of polytef suspension for the management of facial paralysis after extirpative skull base surgery.

BACKGROUND: The satisfactory management of facial paralysis after extirpative skull base surgery has been notoriously difficult. To optimize physical and psychological recovery, early perioperative use of polytef (polytetrafluoroethylene [PTFE]) facial suspension has been used in patients with either profound electrophysiological or anatomical disruption of the facial nerve. OBJECTIVE: To review the efficacy of this clinical algorithm. STUDY DESIGN: Retrospective medical record review. SETTING: Tertiary care University Hospital Inc, University of Cincinnati College of Medicine, Cincinnati, Ohio. PATIENTS AND METHODS: Medical records review of 32 patients who underwent lateral skull base surgery with resultant facial paralysis who had facial rehabilitation using polytef suspension. RESULTS: All patients who underwent polytef facial suspension reported improvement in both facial function as well as aesthetics. One patient had a late extrusion of the polytef implant. CONCLUSION: The early peri-extirpative application of this technique provides psychological and physical support to patients with facial paralysis who are recovering from lateral skull base surgery. Arch Facial Plast Surg. 2000;2:243-248

Adult↗

Minimally invasive brow suspension for facial paralysis.

OBJECTIVE: To report a new technique for unilateral brow suspension for facial paralysis that is minimally invasive, limits supraciliary scar formation, does not require specialized endoscopic equipment or expertise, and has proved to be equal to direct brow suspension in durability and symmetry. DESIGN: Retrospective survey of a case series of 23 patients between January 1997 and December 2000. SETTING: Metropolitan tertiary care center. PATIENTS: Patients with head and neck tumors and brow ptosis caused by facial nerve paralysis. MAIN OUTCOME MEASURE: The results of the procedure were determined using the following 3-tier rating system: outstanding (excellent elevation and symmetry); acceptable (good elevation and fair symmetry); and unacceptable (loss of elevation). RESULTS: The results were considered outstanding in 12 patients, acceptable in 9 patients, and unacceptable in only 1 patient. One patient developed a hematoma, and 1 patient required a secondary adjustment. CONCLUSIONS: The technique has proved to be superior to standard brow suspension procedures with regard to scar formation and equal with respect to facial symmetry and suspension. These results have caused us to abandon direct brow suspension and to use this minimally invasive method in all cases of brow ptosis due to facial paralysis.

Facial Paralysis↗

Left vocal cord paralysis as a primary manifestation of invasive pulmonary aspergillosis in a nonimmunocompromised host.

We report the first case (to our knowledge) of vocal cord paralysis as a primary manifestation of invasive pulmonary aspergillosis, which occurred in a 69-year-old woman without immunodeficiency. Her chest radiograph showed left upper lobe infiltration with pleural thickening, and a computed tomogram of her chest showed a thick pleural reaction and fibrosis around the arch of the aorta. A transbronchial biopsy specimen revealed Aspergillus infection. The patient was treated with oral itraconazole. However, since vocal cord paralysis persisted, the patient underwent type I thyroplasty to improve vocal function. A review of the literature showed that the incidence of invasive pulmonary aspergillosis has increased, even in nonimmunocompromised subjects, and that the disease has a potential for recurrent laryngeal nerve palsy. Therefore, invasive pulmonary aspergillosis should be considered in patients with vocal cord paralysis.

Administration, Oral↗

Three-dimensional endoscopic images of vocal fold paralysis by computed tomography.

OBJECTIVE: To describe characteristics of 3-dimensional (3-D) computed tomographic (CT) endoscopic images of the larynx in unilateral vocal fold paralysis and the changes of the paralyzed vocal fold after phonosurgery as indicated by 3-D CT endoscopy. SETTING: A university medical center. PATIENTS: Twelve consecutive patients with unilateral vocal fold paralysis who underwent helical CT examination. Five of them underwent the CT examination before and after phonosurgical treatment. INTERVENTIONS: Three patients underwent arytenoid adduction, and 2 underwent type 1 thyroplasty. MAIN OUTCOME MEASURES: "Sagging," defined as caudal displacement of the vocal fold; and "thinning," defined as a decrease in the vertical thickness of the vocal fold and expansion of the ventricle on the affected side, were evaluated. RESULTS: Excessive motion artifacts in one patient prevented detailed description of his 3-D images. Sagging and thinning of the vocal fold and expansion of the ventricle on the affected side were noted on 6, 11, and 8 occasions, respectively. Adduction or augmentation of the paralyzed vocal fold after phonosurgery was observed in 3-D CT endoscopic images when displayed with bony densities. CONCLUSIONS: The use of 3-D CT endoscopy enables description of 3-D characteristics of unilateral vocal fold paralysis and supplements stroboscopic findings. Furthermore, CT endoscopic images, when simultaneously displayed with bony densities, may help in evaluating the effects of phonosurgical treatment of the paralyzed vocal fold.

Adult↗

Medical malpractice and facial nerve paralysis.

BACKGROUND: Iatrogenic facial nerve paralysis is a devastating surgical complication that occasionally results in litigation. OBJECTIVE: To analyze litigation trends to better understand the causes and outcomes of suits involving facial nerve paralysis to prevent future litigation and improve physician education. DESIGN: Retrospective review. SETTING: All US civil trials. PARTICIPANTS: All state and federal civil trials alleging malpractice and facial nerve paralysis were reviewed. METHODS: Jury verdict reviews from January 1, 1985, to December 31, 2000, were obtained from a computerized legal database and analyzed. Reviews compile data on defendants, plaintiffs, allegations of wrong doing, and expert witness specialties, and provide case summaries. Fifty-three cases from 19 of the 50 states were obtained. Data were entered into a spreadsheet for analysis. MAIN OUTCOME MEASURES: Verdicts and indemnity payments. RESULTS: Suits reviewed were as follows: cosmetic, 12 (23%); otologic, 13 (25%); nonneoplastic disease of the parotid or other benign conditions of the head and neck, 15 (28%); benign neoplasms of the parotid, 9 (17%); malignant neoplasms of the parotid, 1 (2%); and temporomandibular joint operations, 3 (6%) (percentages do not total 100 because of rounding). Allegations of negligence were frequently multiple and included lack of informed consent (16 [30%] of 53), failure to diagnose (10 [19%] of 53), and surgical misadventures (47 [89%] of 53). Excluding failure to obtain consent or to diagnose, 28 suits still alleged negligence based solely on a surgical misadventure. Of these suits, 12 (63%) resulted in plaintiff awards. CONCLUSIONS: Surgeons must emphasize and document the likelihood and consequences of this devastating complication to all patients undergoing surgery in this area. Risk management goals include a thorough and timely examination and careful and thoughtful surgical approaches. However, patient rapport and bedside manner may be the only protection the surgeon has from litigation arising from this complication.

Adult↗

Causes of recurrent laryngeal nerve paralysis.

The recurrent laryngeal nerve in its course from the brain stem to the larynx follows a path that brings it in proximity to numerous structures. These structures can interfere with its function by pressure or by disruption of the nerve caused by disease invading the nerve. The various causes of recurrent laryngeal nerve paralysis that I have observed during the past 24 years are reported. In 134 patients with recurrent laryngeal nerve paralysis, the left recurrent nerve was most commonly involved. Malignant neoplasms of the lung and pulmonary tuberculosis were the most frequent causes of the paralysis.

Adolescent↗

Acoustic reflex and loudness discomfort in acute facial paralysis.

An extensive research protocol was used to evaluate the conditions of 48 consecutive patients with acute facial paralysis. The results indicated that after nerve excitability testing, the acoustic reflex was the most efficient indicator of impending nerve degeneration and predictor of recovery. The presence of increased sensitivity to intense acoustic stimuli (loudness discomfort level) indicated poor prognosis. The data demonstrate that dysacusis (hyperacusis) is not related to stapedial muscle paralysis and also question the validity of "topographic diagnosis" in determination of the site of the lesion in facial paralysis.

Adolescent↗

Bulbar changes with laryngeal paralysis.

There are two hypotheses that attempt to explain how a lesion in the lower part of the chest may cause paralysis of both laryngeal nerves with intermediate position of the afflicted cord. The first proposes retrograde degeneration of the vagus nerve up to its nucleus ambiguus, the second, ascending metastasis along the vagus nerve until the superior laryngeal nerve becomes paralyzed. In one case of inoperable lung cancer, the brain-stem was sectioned into a gapless series. The number of cells of the nucleus ambiguus on each side was equal, thus excluding an irreversible retrograde degeneration. However, central chromatolysis was found more than twice as often on the side of laryngeal paralysis. Intermediate vocal cord position was again associated with paralysis of both laryngeal nerves of that side.

Female↗

Bilateral vocal cord paralysis in Parkinson's disease.

We report two cases of bilateral vocal cord paralysis in patients with Parkinson's disease and review the possible causes of this condition. We suggest that the vocal cord muscle paralysis is a manifestation of advanced disease. Bilateral vocal cord paralysis in Parkinson's disease may be seen more commonly in the future as therapy enables more patients to live longer.

Aged↗

Metabolic facial paralysis in an infant.

A 2-month-old infant developed facial paralysis with a presentation that masqueraded as a possible middle ear tumor. The cause of the paralysis eventually was related to a metabolic consequence of his underlying disorder, cystic fibrosis. To our knowledge, the association of vitamin A imbalance, pseudotumor cerebri, and facial paralysis has not previously been discussed in the otolaryngologic literature.

Cystic Fibrosis↗

Laryngeal paralysis as the presenting sign of aortic trauma.

Disruption of the thoracic aorta is usually fatal without prompt surgical attention. Left recurrent laryngeal nerve paralysis has been reported in approximately 10% of patients who survive long enough to develop a pseudoaneurysm, but the time of onset has rarely been specified. In this study, 50 cases of thoracic aortic trauma were reviewed to determine the incidence and timing of laryngeal paralysis, compared with 50 cases of atherosclerotic aneurysms matched for location. Four patients in the traumatic group were hoarse, and the onset was immediate in 3. Six in the atherosclerotic group became hoarse. Results indicate that laryngeal paralysis following severe trauma can be a very early sign of aortic injury and requires prompt and thorough investigation.

Adolescent↗

Vestibular schwannoma presenting with sudden facial paralysis.

Facial paralysis is an unusual manifestation of vestibular schwannoma, and generally signifies an advanced stage of tumor growth. We describe a case of eighth-nerve schwannoma that presented initially with rapid-onset complete unilateral facial paralysis. At the time of operation the nerve was found to be electrically intact despite marked compression by tumor. The facial nerve was preserved and facial motion has partially recovered postoperatively. All unexplained persistent facial paralysis should be evaluated by magnetic resonance imaging with paramagnetic contrast enhancement.

Adult↗

Paralysis of the diaphragm.

Diaphragmatic paralysis has been mentioned infrequently as a cause of respiratory symptoms in children. During a three-year period, diaphragmatic paralysis has occurred in 12 neonates following birth trauma (six) or thoracotomy (six) and in four older children following thoracotomy. Spontaneous recovery occurred in all four older children and in five of the 12 neonates. Although two neonates became asymptomatic despite persistent paralysis, the remaining five neonates required prolonged intermittent positive-pressure breathing; two eventually died of secondary pneumonia, and three became asymptomatic only after plication of the affected diaphragm.

Adolescent↗