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Ultrastructural analysis of the chorda tympani nerve in facial paralysis from different etiologies.

Twelve chorda tympani segments, removed from as many patients (7 females, 5 males) affected by otosclerosis (3, as controls) and from either idiopathic (4), or traumatic (3) or herpetic (2) facial paralysis, were examined by a transmission electron microscope, in order to verify chordal ultrastructural changes, the gravity of neural damage in the various types of facial paralysis and the role played by chorda tympani on the etiopathogenesis of the idiopathic form. The segments were obtained, while performing a stapedectomy in otosclerotic patients and, in facial palsy, while performing chordal neurotomy or direct decompression of the II and III portio of the VII nerve. The obtained data showed no noteworthy alterations concerning myelinated and unmyelinated nerve fibers and chordae stroma in otosclerotic patients, confirming their reliability as controls. In facial paralysis, both normal ultrastructures and alterations were noticed, even though polymorphic: unravelling and dissociation of the lamellae sheaths up to a point of degeneration with a more or less complete resorption of myelin, accumulation of dense bodies and electron-dense formation, particularly in unmyelinated fibers, vacuoles in myelinated fibers, intracytoplasmic vacuoles in Schwann cells and, in herpetic forms, an occasional presence of inflammatory cells. Such results demonstrate that, in any type of facial paralysis, the ultrastructure of the chorda tympani is constantly involved, probably due to main trunk involvement. In the idiopathic forms the alterations of the chorda look like to degenerative alterations caused by traumato-ischemic events, rather than to cytopathic lesions induced by herpetic virus.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[An unusual complication of cervicofacial lift: paralysis of the external branch of the accessory spinal nerve].

Paralysis of the lateral branch of the accessory nerve is an extremely rare lesion during cervico-facial facelift. Based on 3 detailed and published personal cases observed over a period of 15 years, the authors review the literature, revealing a total of 9 cases over a period of 30 years, but other cases have probably not been published. Their review reveals the following points. This is an exceptional lesion. However, the nerve can be damaged as it emerges from the posterior border of the sternocleidomastoid muscle during slightly deep dissection and the electrical scalpel must be used cautiously. Even, partial paralysis of the trapezius muscle induces pain and considerable functional impairment with partial permanent disability. The natural history of this paralysis is very long and may be followed by regression after several years. Although there is an obvious cause-and-effect relationship between the operation and accessory nerve paralysis, it is always difficult to attribute this lesion to a possible surgical error, as the exact mechanism of the nerve lesion remain hypothetical and once again raises the problem of therapeutic risk.

Accessory Nerve Injuries↗

Tourniquet paralysis in microsurgery.

Tourniquet paralysis is a serious complication after surgery. The authors report 15 cases recorded in their institution from 1972 to 1992. Seven were a consequence of routine operations on the upper limb and 8 were after microsurgical procedures. In the first group, the incidence of tourniquet paralysis was 1 in 7000 and this does not differ from published reports. In the second group, the incidence of complications is higher: 1 in 4300 cases. In all 15 cases the Tinel sign was absent. Great care was taken to distinguish all the painful conditions which ranged from simple hyperalgesia to allodynia and causalgia. The distressing aspect of tourniquet paralysis is that all the main nerves of the upper limb (median, radial and ulnar), distally to the elbow, are usually affected by the paralysis. In the two groups, the three nerves were affected in 13 cases and the ulnar nerve was partially spared in 3 cases. In both groups the radial nerve was more severely affected. It took an average of 105 days for complete recovery for the first group (range, 30-210 days) and 115 days for the second group (range, 20-180 days). Only in 1 patient in the first group was functional recovery incomplete. According to the authors' experience, general safety factors should be considered before using tourniquet. The tourniquet should be prohibited in patients with congenital susceptibility to nerve compression and should be used with caution in cases of underlying coagulation disorders and neuropathies, in tiny cachetic patients and in patients with systemic lupus erythematosus. In microvascular procedures its use should be limited to the first phase of replantation.

Adolescent↗

[Laryngeal paralysis and laryngospasm].

The intrinsic laryngeal muscle potential was recorded with electromyography. Vocal cord movements were observed by a video stroboscope. Laryngeal paralysis is divided into mild, moderate, and severe degrees based on the potentials of intrinsic laryngeal muscles and on the status of vocal cord movements. In the past 11 years (from 1983 to 1993) 1211 cases were diagnosed as having mild, moderate, and severe laryngeal paralysis. Among them, there were 11 cases with laryngospasm including 3 mild, 2 moderate, and 6 severe cases. The findings obtained from careful observation on these 11 cases of laryngospasm demonstrated that there were certain relationships between laryngeal paralysis and laryngospasm. During the course of exacerbation or restoration of paralysis, the laryngospasm may occur.

Adult↗

Transcutaneous intracordal silicon injection for unilateral vocal cord paralysis.

Unilateral recurrent laryngeal nerve paralysis causes the vocal cord to atrophy, leading to glottic incompetence. The voice is characterized by hoarseness, breathlessness, rapid air escape, ineffective cough and aspiration. Traditional treatments of unilateral vocal cord paralysis include intracordal injection, laryngeal framework surgery, and laryngeal reinnervation for medialization of the vocal cord. In this paper we report on a new technique of transcutaneous intracordal silicon injection in which the injection is made through the thyroid lamina under local anesthesia monitoring fiberscopy. We performed this procedure on 30 patients with unilateral vocal cord paralysis and later evaluated their post-operative voice. The silicon injection resulted in improvement of vocal quality, favorable clearance of sputum, and aspiration control. We found that, since it can be performed under local anesthesia without hospitalization, transcutaneous intracordal silicon injection should be performed as a therapy for treating unilateral vocal cord paralysis.

Administration, Cutaneous↗

[A case of X-linked bulbospinal muscular atrophy with bilateral abductor vocal cord paralysis].

We report a 54-year-old man with X-linked bulbospinal muscular atrophy (BSMA) with bilateral abductor vocal cord paralysis. He noticed distal weakness in the lower limbs at age 20. In the following 18 years the weakness and atrophy of his leg muscles increased gradually. He has complained of stridors during respiratory tract infection and snored heavily during sleep since his age of 50. He was admitted to our hospital for the progressive stridors during meals. His two brothers were said to have similar complaints. Physical examination showed gynecomastia, hypertension and inspiratory stridor. Neurologic examination revealed distal muscular atrophy in his four extremities, especially more severe in bilateral lower limbs. Deep tendon reflexes were absent in all extremities. His tongue was slightly atrophic with fasciculation. Neurological diagnosis was made by family history, neurological findings, electromyography and a CAG repeat expansion in the androgen receptor gene. Lungs and diaphragm were normal on the chest radiograph. Cranial MRI including brain stem was also normal. Direct laryngoscopy showed a complete paralysis of both vocal cords in paramedian position. Tracheostomy was done right away; his respiratory distress showed prompt improvement after the tracheostomy. No previous report of bilateral vocal cord paralysis in BSMA has been found. Life expectancy in BSMA patients with vocal cord paralysis may be shortened because of respiratory distress or asphyxia. Of clinical importance is a careful assessment of vocal cord function in BSMA patients.

Endoscopy↗

[Infantile peripheral facial paralysis].

Peripheral facial paralysis is a common mononeuropathy in adults. It is less frequent in children, but is very upsetting for parents when it occurs. Its causes in children are similar to those found in adults, the most common being idiopathic. However, secondary facial paralysis is the most frequent cause in children. The results of a 4-year follow-up of 29 cases of peripheral facial paralysis in 26 children age 14 and under are reported. The causes, evolution, treatment, recuperation, care, and rate of admission are reviewed. Results are compared to those found in the follow-up of 196 adult cases of peripheral facial paralysis made by the authors.

Adolescent↗

[Diaphragm paralysis following pediatric heart surgery. Review of 25 treated cases].

Postoperative diaphragm paralysis after pediatric heart surgery is the cause of respiratory insufficiency for which there is currently no agreed treatment. The two hypotheses now considered are: early folding of the diaphragm or longterm assisted ventilation. A retrospective study from 1988 to January 1995 on the incidence of diaphragm paralysis and the type of treatment performed was carried out by the Department of Pediatric Heart Surgery of the Bambino Gesù Hospital in Rome. A total of 25 patients presented diaphragm paralysis out of 3400 operations performed and the clinical records of these patients in intensive care were reexamined. The patients were aged between 2 days and 5 years (mean 3 years) and weighed between 3 kg and 15 kg (mean 6.5). The diagnosis of diaphragm paralysis was made using fluoroscopy of the pulmonary fields during spontaneous breathing; 13 patients were extubated 7 days after diagnosis, 12 underwent surgical folding and were extubated immediately after surgery. The authors underline the particular intolerance to ventilatory insufficiency of pediatric patients after the correction of congenital cardiopathies and the need for early intervention to shorten the stay in intensive care and to avoid the onset of other severe complications such as infection. In particular, in patients undergoing Glenn's or Fontan's modified operation, the integrity of the ventilatory apparatus is essential for the correct function of the new hemodynamic status.

Cardiac Surgical Procedures↗

Bilateral recurrent laryngeal neurectomy as a model for the study of idiopathic canine laryngeal paralysis.

The purposes of this study were to develop an experimental model of canine laryngeal paralysis that mimicked the naturally occurring disease and to document the upper airway changes produced, both clinically and with pulmonary function testing. Ten dogs had bilateral recurrent laryngeal neurectomy performed and were recovered from anesthesia. Tidal breathing flow-volume loop analysis and upper airway resistance measurements were taken before and after the development of clinical laryngeal paralysis while dogs breathed room air and after the individual administration of 2 respiratory stimulants. Clinical signs of laryngeal paralysis developed 38 days (median) following denervation. Although some variations were present, tidal breathing flow-volume loop analyses on room air, following denervation, were similar to those reported in naturally occurring cases. Upper airway resistance increased following denervation and was significantly increased with both respiratory stimulants. We concluded that bilateral recurrent laryngeal neurectomy resulted in clinical signs and respiratory changes similar to those of idiopathic canine laryngeal paralysis.

Animals↗

[Criterion on the screening for acute flaccid paralysis (AFP) surveillance].

Using AFP as the criterion for surveillance of children aged < 15 years on polio eradication programme, the sensitivity is 100%. But specificity is low, with high false positivity. Through analysing all 818 cases of AFP occurred in 1991 in Anhui province, we were trying to find out a more specific criterion for the purpose of screening polio cases from AFP. Among 818 cases of AFP aged < 15 years, most polio and non-polio AFP cases occurred under 5 years. At the onset of paralysis, 87.63% of the polio cases presented with fever, compared with 45.99% of non-polio AFP cases did. There fore we chose age and fever at the onset of paralysis as the criterion for screening tests. In parallel tests, the sensitivity was very high, but the specificity was still low. In serial tests, the combination of age under < 6 and fever at onset of paralysis resulted in a sensitivity of 81.44% and specificity of 65.54%. This results suggested that by screening the cases of AFP aged < 6 years who had fever at the onset of paralysis, the number of sample size under testing can be largely reduced, with a minimal compromise in the sensitivity. As for surveillance purpose, the criterion can be used to identify more critical cases followed by urgent attention.

Adolescent↗

Bell's palsy: an update on idiopathic facial paralysis.

Patients with Bell's palsy, or idiopathic facial paralysis, present sporadically in the primary care setting. New evidence implicates reactivated herpes simplex virus (HSV) as the etiologic agent in greater than 70% of cases diagnosed as Bell's palsy. Careful evaluation of the patient with facial paralysis, including history, physical examination, and diagnostic assessment, may mandate the expeditious treatment of facial paralysis to prevent faulty nerve regeneration during the recovery period. Using the results of an objective tool for grading resting facial symmetry, symmetry of voluntary movement, and synkinesis can provide a quantitative measurement for decision making. These data are also useful in documenting progression or regression of the patient's facial paralysis. Administration of acyclovir with prednisone improves the recovery of complete facial functioning following an episode of Bell's palsy. During the acute and convalescent stages, the eye on the affected side must be protected until function is restored to the facial nerve. Residual effects of Bell's palsy lasting more than 6 months may indicate another diagnosis and the need to refer the patient to a specialist.

Acute Disease↗

Prediction of prognosis in peripheral facial nerve paralysis using submandibular gland scintigraphy.

UNLABELLED: In this study, we evaluated the ability of submandibular gland scintigraphy to predict the prognosis of peripheral facial nerve paralysis. METHODS: Submandibular gland scintigraphy was performed in 78 patients with acute peripheral facial nerve paralysis. After injection of 180-370 MBq [99mTc]pertechnetate, serial 1-min images were acquired for 25 min. At 15 min after injection of radionuclide, ascorbic acid was administered intraorally to stimulate salivary secretion. Regions of interest were set manually on both submandibular glands, and time-activity curves were generated. The ratios of peak count density (PCR) and washout (WR) of the affected side to the normal side were calculated. Parameters of > or = 0.8 suggested normal affected submandibular function and indicated a good prognosis. RESULTS: Complete recovery of facial nerve paralysis was observed in 52 of 78 patients. The sensitivity, specificity and accuracy of PCR for a good prognosis were 79%, 50% and 69%, and those of WR were 85%, 77% and 82%, respectively. Positive and negative predictive values for a good prognosis were 76% and 54% in PCR and 88% and 71% in WR, respectively. When WR obtained within 14 days of the onset was used, positive and negative predictive values for a good prognosis were 94% and 73%, respectively. None of the eight patients who had values of <0.8 for both parameters within 14 days of the onset recovered completely. CONCLUSION: Submandibular gland scintigraphy can serve as a reliable indicator to predict the prognosis of acute peripheral facial nerve paralysis in its early symptomatic period.

Acute Disease↗

[Moebius syndrome with pharyngo-laryngeal paralysis in a premature infant].

A case of Moebius syndrome in a premature baby is reported. After a phase of neonatal severe respiratory distress syndrome, the baby presented with a persistent facial paralysis, already present at birth and inability to close the eyes (also present in her father), without ophthalmoplegia. An unusual pharyngeal and laryngeal paralysis was also present: it led to tracheal intubation then tracheostomy and gastrostomy. CT scan at 15 months of age showed hypoplasia of brain stem. The difficulties of managing bulbar paralysis in such a premature baby are emphasized.

Facial Paralysis↗

Thyrotoxic periodic paralysis. Report of 10 cases and review of electromyographic findings.

We reviewed the clinical characteristics of 10 patients with thyrotoxic periodic paralysis. In these patients, a relatively uniform group of young men, the periodic paralysis developed nearly concurrently with the onset of hyperthyroidism. The attacks were precipitated most frequently by rest and by exercise and, occasionally, by ingestion of a large carbohydrate load. In each patient, the paralysis resolved on return of euthyroidism. The approximate incidence rate for thyrotoxic periodic paralysis in our largely white North American patient population (all hyperthyroidism cases) ranged from 0.1% to 0.2%, which is one tenth the rate reported for Oriental populations. In 7 patients, electrodiagnostic testing revealed characteristic changes in compound muscle action potential amplitude in response to exercise of the muscle being tested.

Adult↗

Hypermagnesemic periodic paralysis: treatment with digitalis and lithium carbonate.

An 18-year-old man suffering from a hereditary hyperkalemic periodic paralysis showed, in addition, a hypermagnesemic periodic paralysis with a 2.5-fold increase in serum magnesium level. All other serum electrolyte levels were normal. The hypermagnesemic periodic paralysis seems to have been caused by a disturbance of the renal tubular system. Thus, magnesium-stimulated paralysis was prevented by treatment with lithium carbonate and digitalis, which blocked the active ion transport.

Adolescent↗

Superior oblique paralysis. A review of 270 cases.

In 270 patients with superior oblique paralyses treated between 1973 and 1984, congenital and traumatic causes were most frequent, and one fourth of all traumatic cases had bilateral involvement. Among the diagnostic features distinguishing bilateral from unilateral paralysis were a right hypertropia in left gaze and left hypertropia in right gaze, and a positive Bielschowsky test on tilting the head toward either shoulder. However, absence of either sign did not exclude bilateral paralysis. Large excyclotropia and a V-pattern esotropia are suggestive of but not diagnostic for bilateral paralysis. Complaints about cyclotropia are limited to acquired paralysis. Cyclotropia in the normal eye, head tilt toward the involved side, or absence of any abnormal head posture limits the diagnostic value of these associated signs. Overshoot of the contralateral superior oblique occurred in 19% of the patients and is thought to be caused by contracture of the ipsilateral superior rectus muscle. Surgical treatment in 112 patients resulted in an 85% cure rate with an average of 1.45 operations per patient.

Adolescent↗

Aciclovir or valaciclovir for Bell's palsy (idiopathic facial paralysis).

BACKGROUND: The most common disorder of the facial nerve is acute idiopathic facial paralysis or Bell's palsy and there may be significant morbidity or incomplete recovery associated with severe cases. OBJECTIVES: To assess the efficacy of aciclovir or similar agents for treating Bell's palsy. SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group register (searched April 2003), MEDLINE (from January 1966 to April 2003), EMBASE (from January 1980 to April 2003) and LILACS (from January 1982 to April 2003). We also contacted authors of identified trials. SELECTION CRITERIA: Randomised or quasi-randomised trials of aciclovir or valaciclovir therapy, alone or in combination with any other drug, in patients with Bell's palsy. DATA COLLECTION AND ANALYSIS: We identified six randomised trials. MAIN RESULTS: Three studies met our inclusion criteria, including 246 patients. One study evaluated aciclovir with corticosteroid versus corticosteroid alone, another study evaluated aciclovir alone versus corticosteroid and a further study evaluated valaciclovir with corticosteroid versus corticosteroid alone or versus placebo alone. Incomplete recovery after one year: data were not available. An analysis was performed on data reported at the end of the study period in each trial. The results from one study four months after the start of treatment significantly favoured the treatment group, whilst the results of the study three months after the start of treatment significantly favoured the control group. The results from the second study at four months showed no statistically significant difference between the three groups. Adverse events: relevant data were not reported in any of the three trials. Complete facial paralysis six months after start of treatment: only one patient had complete paralysis upon entering one of the studies. This patient was assigned to the control group and the level of recovery attained was not reported. Motor synkinesis or crocodile tears one year after start of treatment: data were available up to a maximum of four months after onset of paralysis. One study reported a significant difference between the treatment groups in favour of the aciclovir plus corticosteroid group over corticosteroid alone, another demonstrated an inconclusive result with no difference between the aciclovir and corticosteroid. The third study did not comment upon these sequelae. REVIEWERS' CONCLUSIONS: More data are needed from a large multicentre randomised controlled and blinded study with at least 12 months' follow up before a definitive recommendation can be made regarding the effect of aciclovir or valaciclovir on Bell's palsy.

Acyclovir↗

Andersen's syndrome: a distinct periodic paralysis.

A previous study of 4 patients defined Andersen's syndrome (AS) as a triad of potassium-sensitive periodic paralysis, ventricular dysrhythmias, and dysmorphic features. AS appears to be distinct in terms of its genetic defect from the alpha-subunit of skeletal muscle sodium channel and the cardiac potassium channel responsible for most long QT syndromes (LQT1). We studied 11 additional patients with AS from 5 kindreds. Spontaneous attacks of paralysis were associated with hypokalemia, normokalemia, or hyperkalemia. All 11 patients had similar dysmorphic features. The QT interval was prolonged in all patients although only 4 were symptomatic. Genetic linkage studies excluded linkage to the alpha-subunit of the skeletal muscle sodium channel and to four distinct LQT loci. In addition, none of the common dihydropyridine receptor mutations responsible for hypokalemic periodic paralysis were present. We conclude that (1) AS is a genetically unique channelopathy affecting both cardiac and skeletal membrane excitability, (2) attacks of paralysis may be either hypokalemic or hyperkalemic, (3) a prolonged QT interval is an integral feature of this syndrome, and (4) a prolonged QT interval may be the only sign in an individual from an otherwise typical AS kindred. This may be confused with more common, potentially lethal LQT syndromes.

Adolescent↗