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Electrocardiographic changes in thyrotoxic periodic paralysis.

The electrocardiograms (ECGs) of 30 patients with hypokalaemic thyrotoxic periodic paralysis during and after paralysis were studied. During paralysis, typical features of hypokalaemia were seen in all patients with serum potassium levels of 2.8 mmol/l or less; above this level, the ECGs varied from non-diagnostic to those showing typical features of hypokalaemia. It was not possible to accurately predict the serum potassium level from the ECG except when either sinus arrest or heart block was present. Although extrasystoles have been reported to be common in hypokalaemia, none of the patients in this study had extrasystoles. Sinus arrest occurred in two patients and second degree atrio-ventricular block occurred in three patients, a finding which has not been reported in hypokalaemia.

Adult↗

Plication of the diaphragm for symptomatic phrenic nerve paralysis.

Paralysis of the diaphragm in infants may produce severe respiratory difficulty because of the paradoxic motion of the affected diaphragm and shift of a mobile mediastinum to the contralateral side. Six infants with diaphragmatic paralysis and severe respiratory distress underwent plication of the diaphragm by a simple technique. Five of the six infants had significant improvement in respiratory effort and were ultimately weaned from ventilatory support. One patient with bilateral paralysis had only minimal improvement. Diaphragmatic function returned in two patients after plication. Plication of the diaphragm is a safe and useful procedure to improve ventilation in infants with a paralyzed diaphragm. Since this technique does not prevent return of diaphragmatic function, it should be employed prior to the development of sequelae of prolonged assisted ventilation and sooner if the phrenic nerve is permanently injured.

Diaphragm↗

Respiratory obstruction and apnea in infants with bilateral abductor vocal cord paralysis, meningomyelocele, hydrocephalus, and Arnold-Chiari malformation.

This report describes 21 infants and children with bilateral abductor vocal cord paralysis and associated meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. Two life-threatening forms of respiratory distress are distinguished: (1) upper airway obstruction due to bilateral abductor cord paralysis and (2) apnea. Clinically significant episodes of apnea were documented in 13 infants. Ten infants had evidence of aspiration and dysphagia. Vocal cord paralysis, apnea, aspiration, and dysphagia were frequently temporally related to increased intracranial pressure.

Airway Obstruction↗

Vocal cord medialization for unilateral paralysis associated with intrathoracic malignancies.

Patients with unilateral vocal cord paralysis from intrathoracic malignancies may have significant dysfunctions of speech, swallowing, ventilation, and effective coughing as a result of inadequate compensation of the nonparalyzed cord. In patients with already compromised pulmonary function, aspiration can be a life-threatening event. Sixty-three patients with intrathoracic malignancies required surgical correction of vocal cord paralysis. Primary pathology included lung cancer (49), esophageal cancer (nine), and miscellaneous tumors (five). Symptoms included hoarseness (62), dyspnea (21), aspiration (26), weight loss (19), dysphagia (14), and pneumonia (14). The surgical procedures included medial displacement of the vocal cord with silicone elastomer (48), temporary Gelfoam injection (seven), and Teflon (polytetrafluoroethylene) injection (eight) to move the affected cord to a medial position. In 11 patients, the operation was performed in the acute postoperative setting to improve pulmonary toilet. Symptomatic improvement was noted in the following proportions of affected patients: hoarseness, 92%; dyspnea, 90%; dysphagia, 93%; aspiration, 92%; pneumonia, 93%; and weight loss, 47%. Overall success rate of the intervention was 57 of 63 patients (90%). All 11 patients treated in the acute setting had immediate improvement. A variety of complications occurred in 17% of patients. Surgical management of vocal cord paralysis in patients with intrathoracic malignancies prevents life-threatening pulmonary complications in the acute postoperative setting. In chronic situations, it provides patients with improved speech, swallowing, and pulmonary function, resulting in improved quality of life, even for patients not cured of their disease.

Adult↗

Respiratory distress in a pregnant woman with congenital vocal cord paralysis.

BACKGROUND: Vocal cord paralysis is one of the most common congenital laryngeal lesions. Hoarseness and dysphonia are common in unilateral cases. Stridor and respiratory distress are seen in bilateral cases. CASE: A 33-year-old primigravida with bilateral congenital vocal cord paralysis presented in the third trimester with a 2-week history of bronchitis and progressive shortness of breath. She developed stridor, her respiratory status deteriorated, and she was intubated with difficulty. She went into cardiac arrest, was resuscitated and placed on a ventilator, and a tracheotomy was performed. Her condition improved, she remained stable until 38 weeks, and was delivered by cesarean. CONCLUSION: Congenital vocal cord paralysis may result in life-threatening respiratory distress during pregnancy. Tracheotomy placement may be useful in the management of these patients.

Adult↗

Management of facial nerve paralysis.

Facial paralysis is a dreaded complication of parotid surgery. It can lead to a variety of troubling symptoms in the patient, such as ocular problems and nasal obstruction. It can also have a significant emotional impact on the patient because of facial disfigurement and difficulties with communication, eating, and drinking. Successful outcome for patients inflicted with facial paralysis depends on proper selection of the appropriate rehabilitation technique. This article discusses the acute and late management of facial paralysis resulting from parotid surgery.

Facial Paralysis↗

Acute partial paralysis alters perceptions of air hunger, work and effort at constant P(CO(2)) and V(E).

Breathing sensations of AIR HUNGER, WORK and EFFORT may depend on projections of central motor discharge (corollary discharge) to the forebrain. Source of motor drive (brainstem or cortex) may determine what is perceived. To test the effect of changing motor discharge at constant ventilation, we induced partial neuromuscular blockade during hypercapnic hyperpnea (31 + or - 9 L min(-1); PET(CO(2))=49 + or - 2 Torr) and during matched volitional hyperpnea (34 + or - 5 L min(-1); PET(CO(2))=41 + or - 1 Torr). Decline of vital capacity was similar between conditions (39%). Ventilation was unchanged with paralysis, indicating increased respiratory motor drive to maintain hyperpnea. Sensations were rated on a seven point ordinal scale. Median EFFORT and WORK increased 3-3.5 points with paralysis during both forms of hyperpnea (P<0.02, Wilcoxon signed rank). Median AIR HUNGER increased 2.5 points with paralysis during hypercapnic (P<0.02) but not during volitional hyperpnea. Data suggests that EFFORT and WORK arise from motor cortex activity (subjects reported engaging volitional control when paralyzed even during hypercapnia) and suggests that AIR HUNGER arises from medullary motor activity.

Adult↗

[Andersen syndrome: a particular form of paralysis with cardiac dysrhythmia].

Andersen syndrome includes a clinical triad with periodic paralysis, cardiac arrhythmia and dysmorphic features most often mild but relevant. It is a potassium channelopathy due to mutation of KCJN2 gene coding for Kir 2.1 protein. We report a familial case with mutation R218W of Kir 2.1 and discuss the main phenotypic and genetic aspects of Andersen syndrome. Muscle manifestations are essentially a periodic paralysis most often of hypokaliemic type. Muscle biopsy reveals tubular aggregates but can be normal as it is shown in the same patient in our kindred. Our proband complained of paralytic attacks since childhood and at adult age she demonstrated a mild permanent deficit of pelvic girdle muscles as it has been described in other types of periodic paralysis after a long duration course. Cardiac manifestations may include in a variable manner a long QT syndrome, premature ventricular contractions, complex ventricular ectopy, polymorphic or bidirectional ventricular tachycardia. Imipramine had a positive effect on arrhythmia in our case. Dysmorphic features are often mild and have to be cautiously looked for as a clue to the diagnosis of Andersen syndrome. They can be easily overlooked if not systematically looked for. Clinical expressivity is variable including in the same family. In our observation, the daughter showed a complete triad, early expressed, which allowed the diagnosis. Her father was late diagnosed on ventricular dysrhytmia but without muscle manifestations and dysmorphic features. Since KCJN2 gene mutation identification, locus heterogeneity of Andersen syndrome was shown. Andersen syndrome kindreds without mutations in KCNJ2 were clinically indistinguishable from KCNJ2-associated subjects. KCNJ2 gene encodes the inward rectifier K+ channel Kir2.1 which plays an important role in maintaining membrane potential and during the terminal phase of cardiac action potential repolarization. Several studies showed a dominant negative effect of the mutation on Kir 2.1 channel function.

Adolescent↗

[Symptomatic periodic paralysis secondary to primary Sjogren's syndrome].

INTRODUCTION: Hypokalaemic periodic paralysis can be primitive or secondary to potassium deficiency which can arise from several causes. Primary Sjogren's syndrome is a rare cause related to kidney involvement. CASE REPORT: A 50-year-old woman has been admitted for hypotonic tetraparesis which had appeared a few days earlier. History taking revealed three previous similar episodes with a notion of oral and lacrimal dryness. Laboratory tests revealed severe hypokalaemia, hyperchloremia, alkaline urinary pH and a minima 24h proteinuria. Additional investigations led to the diagnosis of a primary Sjogren's syndrome defined on the basis of international criteria. Kidney biopsy revealed tubular-interstitial nephritis. Oral corticosteroïd therapy and potassium supplementation led to symptom improvement. A recurrent episode also responded to treatment. Additional urinary alkalinisation has prevented further relapse. DISCUSSION: Primary Sjogren's syndrome is an exocrine disease causing systemic disorders. Tubular-interstitial nephropathy may occur in 25 percent of patients leading to distal tubular acidosis defined by the association of hypokalaemia, hyperchloremia and alkaline urinary pH. When hypokalaemia is severe, periodic paralysis may occur. CONCLUSION: Primary Sjogren's syndrome can lead to nephropathy and subsequent hypokalaemic periodic paralysis. Urinary alkalinisation is essential to prevent this catastrophic presentation from recurring.

Adrenal Cortex Hormones↗

Neonatal paralysis of the rat soleus muscle selectively affects motoneurones from more caudal segments of the spinal cord.

Transient paralysis of the rat soleus muscle shortly after birth leads to permanent muscle weakness, loss of muscle fibres and the death of motoneurones. The soleus muscle receives its innervation from motoneurones whose axons exit the spinal cord either via the L4 ventral ramus or in the more caudal part of the cord via the L5 ventral ramus. Whether both populations of motoneurones are equally affected by neonatal paralysis was studied here. In soleus muscles paralysed with alpha-bungarotoxin shortly after birth and examined 8-10 weeks later, there is no loss of force or muscle fibres in the part of the muscle supplied by axons in the L4 ventral ramus. Loss of force and muscle fibre numbers occurs only in the part of the muscle supplied by axons in the L5 ventral ramus. In a normal adult soleus 30.3 +/- 2.4% of muscle force is produced by stimulating the L4 ventral ramus and 69.0 +/- 5.5% by stimulating the L5 ventral ramus. In soleus muscles treated with alpha-bungarotoxin 28 +/- 1.4% of the force produced by the contralateral control soleus was generated by axons in the L4 ventral ramus and only 20.3 +/- 5.6% by stimulating the L5 spinal nerve. The number of muscle fibres supplied by either ventral ramus in control and experimental muscles confirmed that the decrease of force after treatment with alpha-bungarotoxin can be accounted for by loss of muscle fibres supplied by axons in the L5 ventral ramus. The reduced force production and muscle fibre numbers was due to a selective loss of motoneurones that had their axons in the L5 ventral ramus. The number of axons to soleus in the L4 ventral ramus was 9.3 +/- 0.7 in controls and 10.3 +/- 0.9 in the experimental animals, whereas the L5 ventral ramus contained 17.2 +/- 0.7 in controls and only 4.7 +/- 1.7 in the experimental animals. Thus paralysis of the soleus muscle at birth selectively affects motoneurones in the more caudal part of the spinal cord, suggesting that the more cranial motoneurones are more mature and less likely to be influenced by lack of neuromuscular interaction at the time of birth.

Acute Disease↗

Neuralgic amyotrophy presenting with bilateral vocal cord paralysis in a child: a case report.

Acute stridor and bilateral vocal cord paralysis is not uncommon in the neonate but is unusual in the older child. We report the first case of bilateral vocal cord paralysis secondary to neuralgic amyotrophy, a peripheral polyneuropathy, in a 5-year-old child. An extensive workup revealed a paralyzed right hemidiaphragm, arm weakness and an EMG pattern consistent with neuralgic amyotrophy. Neuralgic amyotrophy is an uncommon disorder in pediatric patients which may involve cranial and peripheral nerves including the phrenic nerves and rarely the recurrent laryngeal nerves. We propose that the diagnosis be considered in children who present with bilateral vocal cord paralysis and other associated neurologic findings.

Brachial Plexus↗

Left sided vocal cord paralysis: a newly recognized late complication of mediastinal irradiation.

BACKGROUND AND PURPOSE: To evaluate the incidence of vocal cord paralysis in a group of breast cancer patients treated with post operative radiation therapy after radical mastectomy. MATERIALS AND METHODS: A group of 150 patients operated with total mastectomy and axillary clearance received, in 1963-1965, post-operative radiotherapy to the parasternal, axillary and supraclavicular lymph node regions. The cohort has now been followed up to 34 years. RESULTS: Eleven of the 12 vocal cord paralyses were left sided, although the breast cancer was almost equally distributed as left- and right-sided (55 vs. 45%). The symptoms did not appear until 2-25 years after irradiation. Series 1 (treated with (60)Co photons) developed 5% recurrent nerve paralysis (RNP) after a median time of 19.0 years compared with 10% and a much earlier appearance (3.5 years) for series 2 (treated with both (60)Co photons and electrons). A reconstruction of the dose plan shows that an unintentional overlap of the fields resulted in hotspots in the tracheo-oesophageal groove, where parts of the tissue received 120-130% of the prescribed dose. CONCLUSIONS: A left-sided vocal cord paralysis of patients treated with mediastinal radiotherapy might not indicate only tumour recurrence but also mediastinal fibrosis. Small differences in patient positioning cannot be excluded as the cause of the difference in the two series. We postulate that other more subtle damage to the vagus nerve may occur without being recognized as late radiation injury.

Adult↗

The effectiveness of neuromuscular facial retraining combined with electromyography in facial paralysis rehabilitation.

OBJECTIVE: The study goal was to present the effectiveness of neuromuscular facial retraining techniques used in combination with electromyography for improving facial function even in cases of longstanding paralysis. STUDY DESIGN AND SETTING: We conducted a retrospective case review in a tertiary neurotology clinic. PATIENTS: Twenty-four patients with facial paralysis received neuromuscular facial retraining between April 1999 and April 2001. The patient sample included 6 males and 18 females, with an average age of 44 years. A control group consisted of 6 patients (4 females and 2 males). RESULTS: All patient groups made significant improvements in function with improved symmetry in dual-channel electromyographic readings and increased facial movement percentages. Some of the percentages of posttreatment facial function were as follows: acoustic neuromas, 93%; Bell's palsy/Ramsay Hunt syndrome, 80%; and facial nerve anastomosis, 71%. Synkinesis was reduced by at least 2 levels in patients who initially demonstrated synkinesis. CONCLUSIONS: Neuromuscular facial retraining exercises and electromyography are effective for improving facial movements. SIGNIFICANCE: Facial retraining is an excellent example of the plasticity of the central nervous system to reorganize, even in cases of longstanding paralysis.

Adolescent↗

An investigation of acute facial paralysis in animals induced by exposure of the tympanic membrane to cold air.

The goal of this investigation was to test the hypothesis that tympanic membrane exposure to cold air is a cause of acute facial palsy. A series of acute invasive experiments and a series of chronic noninvasive experiments were conducted in both cats and dogs. In the acute studies, stimulation was applied intracranially to the facial nerve root through a stereotaxically placed microelectrode and recordings of compound action potentials obtained extracranially from the facial nerve. Nerve conduction was monitored continuously during the application of cold air to the tympanic membrane. Nerve conduction disturbances were observed in all animals tested (8), and reduction in compound action potential amplitude ranged from 33% to 96%. Histologic analysis of the intratemporal portion of the facial nerve was performed in the animal exhibiting the greatest block in conduction, representative of a near-total paralysis. Axon swelling, demyelinization, and degeneration (Bungner's bands) without inflammation were apparent along the entire tympanic membrane segment. Interstitial swelling of nerve endoneurium was also present at the second genu and vertical segment. In the chronic studies, animals were exposed to cold air and monitored daily for facial paralysis after recovery from anesthesia. None of the animals demonstrated any detectable behavioral facial paralysis.

Action Potentials↗

Tick paralysis in North America and Australia.

The pathophysiology and clinical manifestations of tick paralysis in North America and Australia are reviewed. Clinical and electrodiagnostic findings in tick paralysis are contrasted with those that occur in other diffuse lower motor neuron disorders, and the disease in North America is compared with the more severe form of the disease that occurs along the east coast of Australia. A detailed account of the treatment of animals afflicted with tick paralysis is presented.

Animals↗

To study voice quality before and after thyroplasty type 1 in patients with symptomatic unilateral vocal cord paralysis.

PURPOSE: The purpose of this study was to evaluate voice quality before and after thyroplasty type 1 in patients with symptomatic unilateral vocal cord paralysis. METHODS: Fifteen consecutive cases of unilateral symptomatic vocal cord paralysis of a known or idiopathic etiology with the duration of 6 months who have failed conventional speech therapy. The voice quality was assessed by perceptual evaluation, acoustic measures, and aerodynamic measures. The position of the cord was assessed by using fiberoptic laryngoscope. The procedure was done under local anesthesia. RESULTS: Among 15 patients, on the first postoperative day, 7 patients were changed to moderate dysphonia and 8 patients had a near-normal voice. On the 30th postoperative day, 3 patients had persistent moderate dysphonia, nine patients had near-normal voice, and 3 patients had normal voice. CONCLUSION: Thyroplasty type 1 successfully reduces glottic gap and improves voice quality in patients with unilateral vocal cord paralysis. Improved maximum phonation time, signal-to-noise ratio, shimmer, jitter, fundamental frequency, and intensity of voice appear to be directly related to medialization of the paralyzed vocal cord.

Adult↗

Aspiration in unilateral recurrent laryngeal nerve paralysis after surgery.

PURPOSE: Dysphonia with hoarseness and breathiness are the key symptoms of unilateral recurrent laryngeal nerve (RLN) paralysis, whereas aspiration is not usually described. The aim of this prospective study was to assess the incidence of aspiration in patients with unilateral recurrent laryngeal nerve paralysis after head and neck or thoracic surgery. PATIENTS AND METHODS: Five patients were included and evaluated within the first week and again 2 months postoperatively. Position, tone, and tension of the true vocal cord as well as assessment of the glottic axis, arytenoid position, laryngeal sensation, status of the pyriform sinus, and salivary stasis were studied. Swallowing evaluation was performed using flexible fiberoptic laryngoscopy during dry swallowing, thick cream, methylene blue liquid as well as with swallowing videofluoroscopy. RESULTS: Three patients had no aspiration, one patient had silent aspiration, and one had symptomatic aspiration. CONCLUSION: We conclude that aspiration may occur in unilateral recurrent laryngeal nerve paralysis and have to be systematically evaluated after pneumonectomy.

Adult↗