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At least 1,531 records · Page 85Linked to original sources
PubMed2003

Severe infantile hyperkalaemic periodic paralysis and paramyotonia congenita: broadening the clinical spectrum associated with the T704M mutation in SCN4A.

The authors describe an Italian kindred with nine individuals affected by hyperkalaemic periodic paralysis associated with paramyotonia congenita (hyperPP/PMC). Periodic paralysis was particularly severe, with several episodes a day lasting for hours. The onset of episodes was unusually early, beginning in the first year of life and persisting into adult life. The paralytic episodes were refractory to treatment. Patients described minimal paramyotonia, mainly of the eyelids and hands. All affected family members carried the threonine to methionine substitution at codon 704 (T704M) in exon 13 of the skeletal muscle voltage gated sodium channel gene (SCN4A). The association between T704M and the hyperPP/PMC phenotype has been only recently revealed. Nevertheless, such a severe phenotype has never been reported so far in families with either hyperPP or hyperPP/PMC. These data further broaden the clinical spectrum of T704M and support the evidence that this mutation is a common cause of hyperPP/PMC.

Adolescent↗
PubMed2001

Angiotensin converting enzyme inhibitor induced hyperkalaemic paralysis.

Secondary hyperkalaemic paralysis is a rare condition often mimicking the Guillain-Barré syndrome. There have been a few case reports of hyperkalaemia caused by renal failure, trauma, and drugs where the presentation has been with muscle weakness. A case of hyperkalaemic paralysis caused by an angiotensin converting enzyme inhibitor is reported.

Aged↗
PubMed2004

Bilateral diaphragmatic paralysis and related respiratory complications in a patient with West Nile virus infection.

The case report is presented of a patient with West Nile virus infection and ventilator dependent respiratory failure in whom bilateral diaphragmatic paralysis developed. If the prevalence of West Nile virus infection continues to rise, recognition of diaphragmatic paralysis and related respiratory complications will become increasingly important to the pulmonary/critical care physician.

Humans↗
PubMed1978

Airflow in unilateral vocal cord paralysis before and after Teflon injection.

The effect of unilateral vocal cord paralysis and intracordal Teflon injection on maximum expiratory and inspiratory flows was studied in 15 consecutive patients. Ten patients had a ratio of forced expiratory flow to forced inspiratory flow at 50% vital capacity (Ve50/Vi50) more than one. Of the remaining five, four had low Ve50 consistent with underlying bronchial disease. Repeat studies were obtained in 10 patients two or more weeks after Teflon injection into a vocal cord for voice therapy. Maximum expiratory flow rates did not change (means 6.64 +/- 0.881/sec before and 6.47 +/- 1.101/s after injection). Inspiratory flow at 50% vital capacity improved in all six patients with a forced expiratory volume in one second (FEV1) greater than 75% of the forced vital capacity (FVC). In patients with an FEV1 less than 75% FVC, no consistent changes could be seen. We conclude that a high Ve50/Vi50 suggestive of variable extrathoracic airways obstruction is a frequent finding in the presence of unilateral vocal cord paralysis. Teflon injection does not cause a significant reduction in forced expiratory flows and improves inspiratory flows in subjects without evidence of underlying bronchial disease.

Airway Obstruction↗
PubMed1979

Effect of posture on lung volume: airway closure and gas exchange in hemidiaphragmatic paralysis.

The effects of posture on lung volume, airway closure, and gas exchange were studied in eight patients with hemidiaphragmatic paralysis. The mean vital capacity in the sitting position was 81% of predicted normal, and in the supine posture fell by a further 19% in right-sided but only 10% in left-sided paralysis. The mean arterial oxygen tension was less than predicted in the sitting posture and fell significantly on lying. Single breath gas transfer factor was normal in all cases whereas the diffusion coefficient was greater than predicted in the sitting posture and rose even further on lying. Closing volume showed no positional change but closing volume as a percentage of vital capacity was higher in the supine position. Regional airways closure was expressed as the relationship expiratory reserve volume minus closing volume. Negative values were found in only two of the subjects in the sitting position but seven had negative values supine, indicating significant airway closure during tidal breathing in this position.

Adult↗
PubMed1991

Bilateral diaphragm paralysis after cardiac surgery with topical hypothermia.

Bilateral diaphragm paralysis is a rare but important complication of open heart surgery. Two cases were found among 360 prospectively studied patients undergoing open heart surgery during one year. Both patients had insulin dependent diabetes with peripheral neuropathy and this may have contributed to their diaphragm paralysis. The patients were studied postoperatively for one year with measurements of lung function, nocturnal oximetry, diaphragmatic function, and phrenic nerve conduction. Treatment with intermittent positive airway pressure ventilation by nasal mask was effective in both patients. After nine months one patient had recovered completely with normal phrenic nerve conduction and diaphragmatic function; the other continues most of his normal daytime activities, but still requires nasal positive airway pressure ventilation for six hours at night.

Cardiac Surgical Procedures↗
PubMed1983

Recurrent laryngeal nerve paralysis: a laryngographic and computed tomographic study.

Vocal cord paralysis is a relatively common entity, usually resulting from a pathologic process of the vagus nerve or its recurrent laryngeal branch. It is rarely caused by intralaryngeal lesions. Fourteen patients with recurrent laryngeal nerve paralysis (RLNP) were evaluated by laryngography, computed tomography (CT), or both. In the evaluation of the paramedian cord, CT was limited in its ability to differentiate between tumor or RLNP as the cause of the fixed cord, but it yielded more information than laryngography on the structural abnormalities of the larynx and pre-epiglottic and paralaryngeal spaces. Laryngography revealed distinct features of RLNP and is the procedure of choice for evaluation of functional abnormalities of the larynx until further experience with faster CT scanners and dynamic scanning of the larynx is gained.

Adult↗
PubMed1993

Patterns of vertebral ossification and pelvic abnormalities in paralysis: a study of 200 patients.

To clarify the characteristics, extent, and frequency of spinal ossification and abnormalities of the sacroiliac joint, symphysis pubis, and hip in paralysis, routine radiographs of the lumbar spine, pelvis, and abdomen were retrospectively evaluated in 95 quadriplegic, 105 paraplegic, and 100 age-matched control patients, with attention to age and race and to spinal level and duration of paralysis. The spinal radiographs were evaluated for presence of osteophytes, syndesmophytes, paravertebral ossification, and flowing hyperostosis characteristic of diffuse idiopathic skeletal hyperostosis (DISH); the pelvic radiographs were used to measure the widths of the sacroiliac joint, hip, and symphysis pubis articulations and evaluate for presence of enthesopathy. Bone formation around vertebral bodies was often seen in quadriplegic (n = 41 [43%]) and paraplegic (n = 46 [44%]) patients. In quadriplegic patients, four distinct vertebral ossification patterns were evident: osteophytosis, paraspinal ossification resembling that found in psoriasis, syndesmophytosis, and flowing ossification similar to that in DISH. In paraplegic patients, osteophytosis was most frequent. Vertebral ossification in quadriplegic patients was significantly associated with age (P < .01) and increased in frequency and severity with increasing age.

Adult↗
PubMed1984

Canine gastrocnemius disuse atrophy: resistance to paralysis by dimethyl tubocurarine.

Ten dogs developed unilateral gastrocnemius disuse atrophy after unilateral hindlimb immobilization in a cast for 25 days. Dose-response curves to dimethyl tubocurarine (MTC) were determined during anesthesia with pentobarbital sodium-N2O. Bolus and continuous infusion increments of MTC every 30 min provided steady-state blood levels at each stage of paralysis. Both gastrocnemius tendons were sectioned and attached to transducers. Both sciatic nerves were stimulated every 30 min: 2 Hz for 2 s, a 15-s pause, 50 Hz for 2 s. Dose-response curves, computer calculated by nonlinear regression using a sigmoid maximal effect model of the Hill equation, were parallel for the data relating blocking of tetanus to dose of MTC. The 50% paralyzing dose (tetanus) for control vs. casted gastrocnemius muscle was 64 vs. 813 mg/kg; corresponding plasma concentrations were 0.12 vs. 2.0 micrograms/ml. Thus in vivo simultaneous tension measurements of both gastrocnemius muscles, one casted and one uncasted, demonstrated resistance to paralysis by MTC in muscle with disuse atrophy.

Animals↗
PubMed2006

[Laboratory Network within the Polio Eradication Initiative (1998-2003): six years of surveillance for acute flaccid paralysis in Spain].

INTRODUCTION: Worldwide poliomyelitis eradication was proposed in 1988 by the World Health Organization (WHO), based on reaching and maintaining high vaccination coverage and on implementing effective poliovirus infection surveillance systems. METHODS: In Spain the surveillance system focuses on active searching for acute flaccid paralysis cases through a nine-laboratory network, coordinated by the National Poliovirus Laboratory (NPL) in the National Center of Microbiology, and supported by Autonomous Community epidemiologists. Additionally, the Network sends enterovirus isolation data from other syndromes. The Laboratory Network is responsible for the primary virological study, while the NPL characterizes all polioviruses and the most epidemiologically relevant non-polio enteroviruses. RESULTS: A total of 54,533 samples were studied during the six-year period, and enteroviruses were detected in 9%. All the polioviruses isolated (n = 196), were characterized as Sabin-like (poliovirus vaccine), and among the non-polio enteroviruses, the most frequent was Echovirus 30. A total of 3% of the samples studied corresponded to the 268 acute flaccid paralysis cases or their contacts. DISCUSSION: According to the results and the WHO virological classification, Spain can be considered polio-free. However, the geographic situation of our country may facilitate the introduction of wild polioviruses that can give rise to imported poliomyelitis. Hence, the laboratory network should actively continue to participate in all the proposed WHO strategies, particularly maintenance of the poliomyelitis eradication infrastructures, and continuing monitoring and vaccination.

Humans↗
PubMed2001

Transient paralysis of the bladder due to wound botulism.

In the last 10 years, wound botulism has increasingly been reported and nearly all of these new cases have occurred in injecting-drug abusers. After absorption into the bloodstream, botulinum toxin binds irreversibly to the presynaptic nerve endings, where it inhibits the release of acetylcholine. Diplopia, blurred vision, dysarthria, dysphagia, respiratory failure and paresis of the limbs are common symptoms of this intoxication. Surprisingly and despite the well-known blocking action of the botulinum toxin on the autonomic nerve system, little attention has been paid to changes in the lower urinary tract following acute botulinum toxin poisoning. Here we report a case of bladder paralysis following wound botulism. Early diagnosis and adequate management of bladder paralysis following botulism is mandatory to avoid urologic complications. Accordingly, the prognosis is usually favorable and the bladder recovery complete.

Botulism↗
PubMed2003

Clinical and molecular analysis of chinese patients with thyrotoxic periodic paralysis.

Although sporadic thyrotoxic periodic paralysis (TPP) has a much higher prevalence in Asian than in all the other populations studied so far, it is also increasingly being seen at the emergency departments of the West, hence, it is vital to stress the importance of recognizing it. TPP shares some similarities with hypokalemic periodic paralysis (HOKPP). However, the pathophysiology of TPP and the reasons for this higher incidence are not known. We hypothesized that some mutations in the CACNA1S gene, which has been implicated in familial HOKPP, might play a role in TPP. We present 5 Chinese patients who suffer from TPP and demonstrate typical clinical features. No mutation was found on the whole CACNA1S gene. Therefore other molecular mechanisms will have to be examined in order to explain the different TPP incidences.

Adult↗
PubMed2003

Manifestation of thyrotoxic periodic paralysis in two patients with adrenal adenomas and hyperandrogenaemia.

Hypokalaemic periodic paralysis is a fairly common complication of hyperthyroidism in Asian populations, but a rare event in Caucasians. In the present work we describe 2 male Caucasian patients with thyrotoxic periodic paralysis (TPP) as initial clinical manifestation of Graves' disease. Further diagnostic procedures demonstrated unilateral adrenal adenoma and hyperandrogenaemia in both patients. To date, only few data are available concerning the hormonal status of Caucasian patients with TPP. The constellation of TPP and adrenal adenomas with increased levels of androgens has not been described previously. Since androgens are capable of inducing sodium-potassium ATPase, which is thought to be centrally involved in the pathogenesis of TPP, hyperandrogenaemia may have triggered the manifestation of paralytic attacks in our patients. It may be of interest to focus not only on thyroid dysbalances in patients with TTP but also to investigate other hormonal disturbances.

Adenoma↗
PubMed2003

Motor evoked potentials in predicting recovery from upper extremity paralysis after acute stroke.

OBJECTIVE: The use of motor evoked potentials (MEPs) in predicting recovery after stroke still appears to be somehow equivocal. We assessed the prognostic value of MEPs with respect to arm and hand motor recovery in acute stroke patients. METHODS: This cohort study included 43 consecutive acute stroke patients with complete paralysis of the upper extremity. MEPs of the abductor digiti minimi muscle (ADM) and the biceps brachii muscle (BB) were obtained within 10 days after stroke onset. The upper limb subset of the Fugl-Meyer Motor Assessment was used to evaluate the motor performance at regular intervals until 6 months after stroke. RESULTS: The follow-up was complete in 40 patients (2 patients died and 1 patient had a recurrent stroke); 14 patients showed motor recovery of the arm and their mean 26-week arm motor score was 17.93 (range 3-30, SD 11.68); hand motor recovery occurred in 11 patients and their mean 26-week hand motor score was 11.09 (range 4-14, SD 4.10). Stepwise logistic regression revealed prognostic models for both arm and hand motor recovery based on BB MEPs (odds ratio 7.69, confidence interval, CI, 1.16-50.95) and ADM MEPs (odds ratio 16.20, CI 2.51-104.40), respectively. CONCLUSIONS: The predictive significance of MEPs with respect to motor recovery of the upper extremity was obvious in our homogeneous sample of patients. This agrees with the paradigm that motor recovery after infarction is strongly dependent on a critical residual sparing of the corticospinal function. In this context, the test properties of MEPs in predicting motor recovery are discussed. The added value of MEPs with respect to motor recovery of the upper extremity should be regarded as established for patients with initial paralysis, especially since clinical examination alone lacks the possibility to detect the potential for motor recovery in these cases.

Adolescent↗
PubMed1984

Triiodothyronine (T3) toxicosis with hypokalemic periodic paralysis.

A case of triiodothyronine (T3) toxicosis associated with hypokalemic periodic paralysis is reported. Thyrotoxic manifestation was minimal except for mild struma. Serum T3 levels were moderately elevated, though other thyroid function tests were within the normal range. The patient was treated with methimazole, and paralytic attacks ceased. This is the first report of this combination and the necessity of careful thyroid function tests in sporadic periodic paralysis is emphasized.

Adult↗