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Pathophysiology of facial nerve paralysis induced by herpes simplex virus type 1 infection.

Herpes simplex virus type 1 (HSV-1) has been proven to be a cause of Bell's palsy; however, the underlying pathophysiology of the facial nerve paralysis is not fully understood. We established a mouse model with facial nerve paralysis induced by HSV-1 infection simulating Bell's palsy and investigated the pathophysiology of the facial nerve paralysis. The time course of the R1 latency in the blink reflex tests paralleled the recovery of the facial nerve paralysis well, whereas electroneurographic recovery tended to be delayed, compared to that of the paralysis; these responses are usually seen in Bell's palsy. On histopathologic analysis, intact, demyelinated, and degenerated nerves were intermingled in the facial nerve in the model. The similarity of the time course of facial nerve paralysis and the electrophysiological results in Bell's palsy and the model strongly suggest that the pathophysiological basis of Bell's palsy is a mixed lesion of various nerve injuries.

Animals↗

Role of herpes simplex virus infection in the pathogenesis of facial paralysis in mice.

To clarify the role and site of herpes simplex virus (HSV) infection in the pathogenesis of facial paralysis, we examined the viral genome by the polymerase chain reaction and the neutralization antibody titer using microplates in an animal model. Following inoculation with HSV type 1 of the KOS strain into mouse auricles, HSV DNA appeared in the ipsilateral facial nerve on the 3rd day, and in bilateral facial nerves and the brain stem on the 10th day only in animals with facial paralysis. In animals without facial paralysis, no HSV DNA was detected in these tissues. The neutralization antibody titer was elevated between 4 and 20 days in all animals, with or without facial paralysis. Facial paralysis developed only on the inoculated side, even though HSV DNA was also present in the contralateral facial nerve. We conclude that HSV infection in the facial nerve and brain stem is prerequisite for facial paralysis, and suggest that an immunologic reaction following viral infection plays a key role in the pathogenesis.

Animals↗

Auditory brain stem response and audiologic tests in idiopathic facial nerve paralysis.

Idiopathic facial nerve paralysis is believed by some to represent one aspect of a polyneuropathy. Conventional audiologic tests have not demonstrated involvement of the auditory portion of the eighth cranial nerve in this disorder. A case history is given of a patient with this disorder and an associated abnormal auditory brain stem response (ABR). Our study involved onetime evaluations of 17 patients with idiopathic facial nerve paralysis of varying durations. Each examination included clinical presentation and history, an audiogram, acoustic reflex, and ABR. Another patient had abnormal acoustic reflex test results ("unibox" pattern) suggestive of a brain stem lesion. On reexamination concurrent recovery of the paralysis and acoustic reflex was demonstrated. No auditory system disturbances secondary to the paralysis were detectable by ABR in this survey. The concurrent recovery of the "unibox" acoustic reflex and the facial paralysis suggests the association of a brain stem lesion with idiopathic facial nerve paralysis in this case.

Adolescent↗

Sleep paralysis, sexual abuse, and space alien abduction.

Sleep paralysis accompanied by hypnopompic ('upon awakening') hallucinations is an often-frightening manifestation of discordance between the cognitive/perceptual and motor aspects of rapid eye movement (REM) sleep. Awakening sleepers become aware of an inability to move, and sometimes experience intrusion of dream mentation into waking consciousness (e.g. seeing intruders in the bedroom). In this article, we summarize two studies. In the first study, we assessed 10 individuals who reported abduction by space aliens and whose claims were linked to apparent episodes of sleep paralysis during which hypnopompic hallucinations were interpreted as alien beings. In the second study, adults reporting repressed, recovered, or continuous memories of childhood sexual abuse more often reported sleep paralysis than did a control group. Among the 31 reporting sleep paralysis, only one person linked it to abuse memories. This person was among the six recovered memory participants who reported sleep paralysis (i.e. 17% rate of interpreting it as abuse-related). People rely on personally plausible cultural narratives to interpret these otherwise baffling sleep paralysis episodes.

Humans↗

New mutations of SCN4A cause a potassium-sensitive normokalemic periodic paralysis.

BACKGROUND: Periodic paralysis is classified into hypokalemic (hypoPP) and hyperkalemic (hyperPP) periodic paralysis according to variations of blood potassium levels during attacks. OBJECTIVE: To describe new mutations in the muscle sodium channel gene SCN4A that cause periodic paralysis. METHODS: A thorough clinical, electrophysiologic, and molecular study was performed of four unrelated families who presented with periodic paralysis. RESULTS: The nine affected members had episodes of muscle weakness reminiscent of both hyperPP and hypoPP. A provocative test with potassium chloride was positive in two patients. However, repeated and carefully performed tests of blood potassium levels during attacks resulted in normal potassium levels. Remarkably, two patients experienced hypokalemic episodes of paralysis related to peculiar provocative factors (corticosteroids and thyrotoxicosis). Similarly to hyperPP, electromyography in nine patients revealed increased compound muscle action potentials after short exercise and a delayed decline during rest after long exercise as well as myotonic discharges in one patient. With use of molecular genetic analysis of the gene SCN4A, three new mutations were found affecting codon 675. They resulted in an amino acid substitution of a highly conserved arginine (R) to either a glycine (G), a glutamine (Q), or a tryptophan (W). Interestingly, hypoPP is caused by both mutations affecting nearby codons as well as the change of an arginine into another amino acid. CONCLUSION: A potassium-sensitive and normokalemic type of periodic paralysis caused by new SCN4A mutations at codon 675 is reported.

Acetazolamide↗

Auditory symptoms associated with herpes zoster or idiopathic facial paralysis.

Auditory symptoms (hyperacusis, tinnitus, decreased hearing) have long been recognized to accompany herpetic or idiopathic facial paralysis. Twenty-nine percent of 1,080 patients with idiopathic facial paralysis and 37 percent of 172 with herpes zoster oticus facial paralysis had auditory symptoms. Abnormal related sensori-neural hearing loss was documented in only 11 of these 377 patients with auditory complaints. All of the 11 had a diagnosis of herpes zoster oticus. Sensori-neural hearing loss occurs in only about 6.5 percent of patients with herpes zoster facial paralysis, and no confirmed case of such loss in idiopathic facial paralysis has been reported. In patients presenting with sensori-neural hearing loss accompanying facial paralysis believed to be idiopathic, herpes zoster should be suspected even in the absence of vesicles. Factors favorable for recovery of auditory function include age 64 years or younger, mild initial hearing loss, a cochlear pattern of hearing loss, and absence of vertigo. Recovery of auditory function does take place; however, a high-tone sensori-neural loss may persist except in younger patients.

Adolescent↗

Glottographic measures of vocal fold vibration: an examination of laryngeal paralysis.

Photoglottography and electroglottography were applied to groups of patients with recurrent laryngeal nerve paralysis, superior laryngeal nerve paralysis, and combined recurrent and superior laryngeal nerve paralyses of idiopathic causes. Individual patients with resection of the vagal nerve above the origin of the superior laryngeal nerve were also studied. Open Quotient and Speed Quotient were calculated from the photoglottography signals. Speed Quotient values significantly differentiated recurrent laryngeal nerve paralysis from idiopathic paralysis and superior laryngeal nerve paralysis, as well as from normal function. Data from patients with vagal resection clearly differentiated them from patients with recurrent laryngeal nerve paralysis. The measure of Open Quotient distinguished pathological phonation from normal, but was not as useful for separation of differing lesions. Electroglottography appeared to be less useful than photoglottography. The pathophysiology underlying the observed glottographic signals is discussed.

Adult↗

Iatrogenic serratus anterior paralysis. Long-term outcome in 26 patients.

STUDY OBJECTIVE: To evaluate the treatment, extent of recovery, and residual disability in 26 iatrogenic cases of serratus paralysis. PATIENTS AND STUDY DESIGN: Seventeen cases of serratus anterior paralysis had occurred following a local invasive procedure along the course of the long thoracic nerve, including seven first-rib resections, four mastectomies with axillary dissection, two scalenotomies, two surgical treatments of spontaneous pneumothorax, and two infraclavicular plexus anesthesia. Eight cases of paralysis had occurred after general anesthesia for patients who had undergone surgery for diverse clinical reasons. One case of paralysis occurred after spinal anesthesia. The length of sick leave, treatment with a shoulder brace, amount of physical therapy, long-term symptoms, and residual disability were evaluated from the medical records and from the questionnaire sent to the patients on average 6 years (range, 2 to 11 years) after the onset of the paralysis. RESULTS: Despite comprehensive and lengthy treatment, all but one had residual symptoms, as well as limitations in the use of the affected limb. Twenty-one (81%) of the patients could not lift or pull heavy objects, 15 (58%) could not play sports, such as tennis or golf, and 14 (54%) found it impossible to work with hands above shoulder level. CONCLUSION: Serratus anterior paralysis, following anesthesia or local invasive procedures on the anterolateral aspect of the thorax, may cause considerable and long-term dysfunction of the shoulder girdle and affect the function of the whole upper limb.

Adolescent↗

Thyrotoxic periodic paralysis associated with a mutation in the sodium channel gene SCN4A.

Thyrotoxic hypokalemic periodic paralysis (THypoKPP) is an uncommon disorder with an unknown etiology. We describe a family in which the proband presented with paralysis and thyrotoxicosis. Because of similarities between familial hypokalemic periodic paralysis (FHypoKPP) and THypoKPP, we sequenced exon 12 of the SCN4A gene, which is known to be mutated in FHypoKPP. We identified an Arg672Ser mutation in the proband and his affected father, as well as the proband's brother. As the brother has paralysis without thyrotoxicosis, our finding suggests that the genetic spectrum of FHypoKPP and THypoKPP overlap. We speculate that thyroid hormone may exert a threshold or permissive effect in hypokalemic periodic paralysis. Non-thyrotoxic family members of individuals with THypoKPP may have an unrecognized risk for paralysis.

Adolescent↗

Laryngeal paralysis: a study of 375 cases in a mixed-breed population of horses.

Referred cases (n = 375) of laryngeal paralysis (1985-1998) from a mixed-breed equine population included 351 (94%) cases of recurrent laryngeal neuropathy (RLN) (idiopathic laryngeal hemiplegia) and 24 cases (6%) of laryngeal paralysis from causes other than RLN. Laryngeal movements were classified endoscopically into one of 6 grades, in contrast to the usual 4 grades. The RLN cases had a median grade 4 laryngeal paralysis, of which 96% were left-sided, 2% right-sided and 2% bilaterally affected. RLN cases included 204 (58%) Thoroughbred, 96 (27%) Thoroughbred-cross, 23 (7%) draught, 16 (5%) Warmbloods and 10 (3%) other breeds, including only 4 (1%) ponies. The median age of RLN cases at referral was 6 years (range 2-12) and their median height was 170.2 cm. The work of RLN horses included National Hunt racing (42%), flat racing (1%), hunting (19%), eventing (16%) and miscellaneous work (22%). Reported presenting signs in RLN-affected horses included abnormal exercise-related respiratory sounds in 90% and reduced exercise tolerance in only 64%. However, many horses were referred before their exercise tolerance could be fully assessed. Forty percent of the RLN cases had intercurrent disorders, including 10% with additional upper respiratory and 7% with lower respiratory tract diseases. The 24 nonidiopathic RLN cases included 12 with bilateral laryngeal paralysis, 11 (92%) of which were ponies. Bilateral laryngeal paralysis occurred with hepatic encephalopathy in 7 cases and following general anaesthesia in 2 cases. The 12 cases of acquired unilateral laryngeal paralysis included 7 caused by guttural pouch mycosis.

Animals↗

Pediatric facial paralysis--a spirochetal infection with good prognosis?

The aim of the study was to investigate the possible signs of chronic spirochetal infection in 27 children with a history of facial paralysis with onset of symptoms during May-October 1985-1993. These children had not been studied at the time of facial paralysis for possible Lyme borreliosis and none of these children had received antimicrobial therapy at that time. The patients were interviewed with special reference to symptoms and signs of Lyme borreliosis and thereafter examined clinically and with laboratory tests. None of the patients showed signs or symptoms of chronic Lyme borreliosis. One child-now a 14-year old girl-had a 4-fold rise in serum Borrelia burgdorferi antibodies as a sign of a recent infection. According to our prospective studies on pediatric facial paralysis in Finland (over 50% of children presenting with facial paralysis during May-October have Lyme borreliosis), we have a reason to assume that at least some in our study group had a facial paralysis caused by Borrelia burgdorferi infection. The results of our study indicate that facial paralysis has a favorable prognosis and in short-term follow-up the children do not have symptoms or signs of chronic Lyme borreliosis.

Adolescent↗

[Facial paralysis evaluations using the Yanagihara method, the House-Brackmann method, and self-evaluation by patients].

The Yanagihara method and the House-Brackmann (H-B) method are widely used in Japan to evaluate facial paralysis. The present study focuses on the relationship between the evaluation of facial paralysis using these methods and self-evaluation by patients. One-hundred and thirty-one patients with facial paralysis were included in the study, consisting of 68 males and 63 females between the ages of 17 and 84 years (mean age: 41 +/- 18 years). In addition to the Yanagihara and H-B methods, two methods of self-evaluation were used by the patients. In the first self-evaluation method, the patient was asked to rate the degree of paralysis as a grade of one to six. In the second method, the patient was asked to rate the severity of the paralysis on a scale of 0-100. The paralysis scores and grades determined using the Yanagihara and H-B methods were correlated with the self-evaluations. However, the strength of the correlation varied among the patients, indicating that the evaluation of subjective symptoms differed among individuals. Even patients who were evaluated as either "completely paralyzed" or "cured" according to the Yanagihara and H-B methods did not always rate their subjective symptoms as being consistent with these scores. In particular, 20 to 30% of patients who were evaluated as "cured" complained of minor dyskinesia.

Adolescent↗

Results of transfer of the pectoralis major tendon to treat paralysis of the serratus anterior muscle.

BACKGROUND: Paralysis of the serratus anterior muscle can be functionally disabling. As a result of the scapular winging associated with such paralysis, the scapula does not remain apposed to the thorax when the upper extremity is elevated forward at the shoulder. This produces functional disability associated with pain and loss of a stable base for movement of the upper extremity. METHODS: We reviewed the results of transfer of the pectoralis major tendon with the addition of a fascial graft in sixteen patients who had paralysis of the serratus anterior. The average age of the patients at the time of the operation was thirty-three years (range, twenty to fifty-five years). Electrodiagnostic studies confirmed the presence of an isolated injury of the long thoracic nerve. The index operation was performed sixteen months to eleven years after the onset of pain and weakness. The etiology of the paralysis was idiopathic in two patients, traumatic in seven, and secondary to operative intervention in seven. All patients had pain in the shoulder on the side of the paralysis. RESULTS: The result was excellent for eight patients, good for five, and fair for one at an average of four years and three months (range, two years and one month to nine years) postoperatively. There were two failures, both of which occurred after a traumatic event. Of the fourteen patients in whom the procedure did not fail, eight were asymptomatic and had normal function, five had intermittent mild discomfort, and one had frequent mild pain without any winging of the scapula. The average Constant and Murley score for the fourteen patients in whom the procedure did not fail increased from 36 points preoperatively to 92 points postoperatively. CONCLUSIONS: The index procedure successfully alleviated the functional disability caused by paralysis of the serratus anterior muscle.

Adult↗

[A family with heat-sensitive myotonia alternating with hypokalemic periodic paralysis].

We report a 34-year-old man with evident family history of paralysis and myotonia. He has noticed episodic morning paralysis alternating with muscle myotonia since the age of 10 years. When an episode of paralysis occurred, his serum potassium level decreased to 2.3 mEq/L and tended to increase to about 4.0 mEq/L when he complained myotonia. This case is obviously different from several already-known diseases, as it is perhaps the first case thermo-sensitive disorder of muscle activity in that the myotonia is induced by warm environment but paralysis occurs in the setting of cold ambient temperature and hypokalemia. Therefore, we are underway to perform molecular genetic analysis of ion channels since paramyotonia/hyperkalemic periodic paralysis is known to be associated with Na(+)-channel missense mutations, while hypokalemic periodic paralysis is with Ca(++)-channel. Addendum: After submission of the manuscript, we identified a novel mutation in Na+ channel alpha subunit. The detail of molecular aspect will be reported elsewhere.

Adult↗

[Diaphragmatic paralysis: pathology at the reach of the pediatric surgeon].

The phrenic nerve is the only motor nerve in the diaphragm. The injury will lead to a diaphragmatic paralysis or eventration with paradoxical movements during breathing. The aim of this review is to analyze the diagnostic criteria, surgical indications, and the patients that are not treated by surgery. We review 53 cases with diaphragmatic pathology in the last 5 years; 19 females and 20 males. One was a diaphragmatic rupture, 13 were diaphragmatic hernias, and 39 had diaphragmatic paralysis. Diaphragmatic paralysis was diagnosed by fluoroscopy in 97%, electromiography 5.1% or ultrasounds. The ethiology was 64% after cardiac surgery and 10% after thoracic oncologic surgery. The age at diagnosis was less than 1 month in 41% cases. In 29 patients the paralysis was well tolerated and were extubated between 24 h and 4 days after the diagnosis, frequently was in the first 48 h. 4 patients need a traqueostomy and 2 went to another hospital. In 5 we performed a diaphragmatic plication because the inability to be extubated or the persistence of respiratory distress. The paralysis was demonstrated by fluoroscopy. 2 patients are asymptomatic, 2 are in mechanical ventilation and 1 died (sepsis). The most frequent cause of diaphragmatic paralysis was related to cardiac or thoracic surgery. In general, is well tolerated, almost asymptomatic, the patients were extubated between 24 or 48 h. In pediatric population they are few cases that need surgical treatment. The patients that we operated on were less than 2 months old, because is at that age were the repercussion is more important. We must follow strict criteria, without precipìtation for the surgical indications.

Child, Preschool↗

[Recent developments in electrical tests applied to the study of facial paralysis].

We have already shown the spontaneous development over a period of time of idiopathic facial paralysis, in relation to the extent of the resulting disfigurement, by means of analytical clinical measurement representing the sum of 19 factors constituting the force of contraction, tonus, coordination and hemispasm. This spontaneous development takes place in 3 stages: --an initial period up to the 15th day; --an intermediate period during which there is clinically total facial paralysis; --a period of recuperation. The duration of this development in relation to the extent of sequelae enables a rough distinction to be made between: a) benign facial paralysis in which disfigurement is nil or very slight (1 to 6 p. 100), incomplete paralysis or complete paralysis in which recuperation starts about the 15th day at the earliest, or at about the 4th month at the latest; c) very serious facial paralysis with moderate (38 to 66 p. 100), or considerable (66 to 100 p. 100) disfigurement and in which recuperation starts at the end of the 3rd month at the earliest. The authors have attempted to indicate how effective very prompt quantitative electrical procedures are in helping to assess the seriousness of ultimate permanent disfigurement (see article).

Electrodiagnosis↗

[A case of hypokalemic periodic paralysis: utility of exercise test for the assessment of therapeutic efficacy].

We report a 14-year-old male with hypokalemic periodic paralysis. He noticed periodic paralysis at the age of 11. Any complication did not accompany the symptom. At the age of 12, hypokalemia was found during an episode of paralysis, and he was diagnosed as hypokalemic periodic paralysis. The frequency of paralytic attack increased around April 2000. Although long-acting oral potassium (32 mEq/day) was administered, it did not give favorable effect. Therapeutic spironolactone trial also failed. After the reconfirmation of the diagnosis of periodic paralysis by an exercise test, oral acetazolamide (750 mg/day) was started. In subsequent exercise test, the increment of the CMAP amplitude of abductor digiti minimi during exercise became smaller and the decrement of CMAP amplitude after exercise disappeared thereafter, which was assumed to be related with clinical improvement. The noninvasive exercise test is useful not only to diagnose periodic paralysis but also to evaluate therapeutic efficacy.

Acetazolamide↗

[Congenital paralysis of the vocal cords: a review of 14 cases].

Were performed 220 direct laryngoscopys and bronchoscopys, from 1981 to 1990, in newborns and infants that showed as a main finding: stridor, voice change or aspiration syndromes. Fourteen (20.28%) of the 69 congenital laryngeal anomalies were vocal cord paralysis: 13 (92.8%) unilateral paralysis and 1 (7.2%) bilateral paralysis. Eleven (84.6%) of the 13 unilateral paralysis were left and 2 (15.4%) were right. The vocal cord position was noted to be median or paramedian in 13 (92.8%) patients. The etiology was idiopathic in 11 (78.5%) of the cases. The bilateral paralysis required tracheotomy and in the unilateral paralysis wasn't necessary it.

Female↗