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Acute hypercapnic respiratory failure due to thyrotoxic periodic paralysis.

Acute hypercapnic respiratory failure is a potentially life-threatening complication of profound hypokalemia usually seen in patients with large total-body potassium (K+) deficits. It has rarely been reported in thyrotoxic periodic paralysis (TPP), which is due to intra-cellular shifts of K+. The authors report on a 29-year-old man who presented with sudden onset of muscle paralysis in all extremities and acute progressive respiratory insufficiency requiring artificial respiratory support. He was treated with intravenous KCl. After six hours, muscular strength had returned to normal, with plasma K+ concentration of 3.0 mmol/L. At the eighth hour, rebound hyperkalemia (6.6 mmol/L) developed with high-tented T waves, even after the KCl was discontinued. Ventilatory support was uneventfully weaned at 14 hours. Elevated free thyroxine and undetectable thyroid-stimulating hormone confirmed the diagnosis of TPP. TPP should be kept in mind as a cause of acute respiratory failure in association with acute muscle weakness to avoid delayed diagnosis and improper management.

Acute Disease↗

An unusual cause of hypokalemic paralysis: aristolochic acid nephropathy with Fanconi syndrome.

Aristolochic acid nephropathy (AAN) with Fanconi syndrome presenting as hypokalemic paralysis is extraordinarily rare and may be unrecognized. We describe a 41-year-old man who presented with the inability to ambulate upon awakening in the morning. Physical examination revealed symmetric paralysis of bilateral lower limbs. Laboratory studies showed profound hypokalemia with renal potassium (K) wasting, hyperchloremic metabolic acidosis, hypophosphatemia with hyperphosphaturia, hypouricemia with hyperuricosuria, and glycosuria, consistent with Fanconi syndrome. Mild renal insufficiency was also observed. A meticulous search for underlying causes of Fanconi syndrome was unrevealing. However, a significant amount of aristolochic acid (AA) was detected in the consumed Chinese herb mixture (AA-I, 7 microg/g) for the treatment of his leg edema for the past 2 months. His hypokalemia, renal insufficiency, and Fanconi syndrome completely resolved 2 months after the withdrawal of Chinese herb mixture and the supplementation of potassium citrate and active vitamin D3. AAN with Fanconi syndrome should be considered as a cause of hypokalemia in any patient administered undefined Chinese herbs.

Adult↗

Position and motion of the human diaphragm during anesthesia-paralysis.

Regional motion of the human diaphragm was determined by high-speed, three-dimensional x-ray computed tomography. Six healthy volunteers were studied first while awake and breathing spontaneously and again while anesthetized-paralyzed and their lungs ventilated mechanically. Tidal volume (VT) and respiratory frequency were similar during both conditions. Three subjects were studied while they were supine and three while they were prone. During spontaneous breathing, movement of dependent diaphragm regions was greater than that of nondependent regions in four of six subjects. In five of the six subjects, dorsal diaphragm movement exceeded ventral movement regardless of body position. The volume displaced by the diaphragm (delta Vdi) was similar to VT in supine subjects but tended to be less than VT in prone subjects. After induction of anesthesia-paralysis, the end-expiratory position of the diaphragm did not change consistently in supine subjects, whereas a consistent cephalad volume shift occurred in prone subjects. During anesthesia-paralysis and mechanical ventilation, delta Vdi was reduced to approximately 50% of VT in both body positions. In the supine position, the pattern of diaphragm motion during mechanical inflation was nearly uniform. By contrast, in the prone position, the motion was nonuniform, with most motion occurring in the dorsal (nondependent) regions. It is concluded that the dominant influence on diaphragm motion may be some anatomical difference between the crural and costal diaphragm regions rather than the abdominal hydrostatic pressure gradient.

Adult↗

Edrophonium increases mivacurium concentrations during constant mivacurium infusion, and large doses minimally antagonize paralysis.

BACKGROUND: Mivacurium, a nondepolarizing muscle relaxant, is metabolized by plasma cholinesterase. Although edrophonium does not alter plasma cholinesterase activity, we have observed that doses of edrophonium that antagonize paralysis from other nondepolarizing muscle relaxants are less effective with mivacurium. We speculated that edrophonium might after metabolism of mivacurium, thereby hindering antagonism of paralysis. Accordingly, we determined the effect of edrophonium on neuromuscular function and plasma mivacurium concentrations during constant mivacurium infusion. METHODS: We infused mivacurium to maintain 90% depression of adductor pollicis twitch tension and then gave edrophonium in doses ranging from 125-2,000 micrograms/kg without altering the mivacurium infusion. Peak twitch tension after edrophonium was determined to estimate the dose of edrophonium antagonizing 50% of twitch depression for antagonism of mivacurium; plasma cholinesterase activity and mivacurium concentrations before and after edrophonium were measured. Additional subjects were given 500 micrograms/kg edrophonium to antagonize continuous infusions of d-tubocurarine and vecuronium. RESULTS: With mivacurium, edrophonium increased twitch tension in a dose-dependent manner: the dose of edrophonium antagonizing 50% of twitch depression was 2,810 micrograms/kg. The largest dose of edrophonium (2,000 micrograms/kg) produced only 45 +/- 7% antagonism. Edrophonium, 500 micrograms/kg, antagonized mivacurium markedly less than it antagonized d-tubocurarine and vecuronium. Edrophonium increased plasma concentrations of the two potent stereoisomers of mivacurium 48% and 79%, these peaking at 1-2 min; plasma cholinesterase activity was unchanged. CONCLUSIONS: Edrophonium doses that antagonize d-tubocurarine and vecuronium are less effective in antagonizing the neuromuscular effects of mivacurium during constant infusion. Edrophonium increases plasma mivacurium concentrations, partly or completely explaining its limited efficacy; the mechanism by which edrophonium increases mivacurium concentrations remains unexplained. Our results demonstrate that antagonism of mivacurium by edrophonium is impaired, and therefore we question whether edrophonium should be used to antagonize mivacurium.

Adolescent↗

Long-term follow-up study of browlift for treatment of facial paralysis.

Browlift for patients with irreversible paralysis of the frontalis muscle was evaluated in a review of 40 patients who had undergone follow-up studies for durations of more than 3 years. The procedure consists of a lazy S-shaped excision above the upper border of the eyebrow and upward fixation of the lower skin margin. The results were as follows: (1) The percentage of patients for whom symmetrical positioning of the eyebrow was achieved was 65%. (2) The greater the preoperative severity of ptosis, the more often did recurrence occur. (3) Recurrence progressed rapidly during the first postoperative year, but thereafter recurrence was seen only in those patients in whom the affected eyebrow was still in a position higher than its counterpart. (4) No relationship could be seen between age at the time of surgery and postoperative position of eyebrow. (5) Improvement in contraction of the visual field was seen in 85% of patients postoperatively, and complaints were eliminated in 50%. (6) Careful preservation of the sensory nerve is required. (7) The postoperative scar was acceptable. This procedure is a simple and effective method of correction of paralytic ptosis. Although a degree of skill is required to obtain satisfactory results and a surgical scar remains at the upper border of the eyebrow, the advantages of the procedure surpass its disadvantages. It is believed that this method should be used more often in the treatment of facial paralysis.

Adolescent↗

Infectious causes of acute flaccid paralysis.

PURPOSE OF REVIEW: Although acute flaccid paralysis (AFP) is more frequently seen in the tropics than in temperate regions, recent outbreaks of West Nile virus (WNV) in North America have drawn attention to this important presentation. Starting with anatomical and neurophysiological considerations, this article examines data on AFP caused by WNV, and considers recent data on paralysis caused by enteroviruses, and Guillain-Barré syndrome (GBS). RECENT FINDINGS: Neurophysiological, radiological and pathological studies suggest WNV causes AFP by damaging anterior horn cells in the spinal cord. The clinical presentation is probably best described as a 'poliomyelitis-like illness', and the disease as 'WNV myelitis'. Other findings during the recent outbreaks include increasing recognition of a Parkinson's-disease like presentation, and descriptions of virus transmission in blood transfusions and transplanted organs. GBS is now recognized as several disorders characterized by immune-mediated attack on peripheral nerves: in acute inflammatory demyelinating polyneuropathy the myelin sheath and Schwann cell of sensory and motor nerves are targeted; acute motor axonal and acute motor and sensory axonal neuropathy often follow Campylobacter jejuni enteritis and are associated with antibodies against the ganglioside component of the nerve axolemmal membrane. In Asia-Pacific, enterovirus 71 has caused outbreaks of neurological diseases with AFP and encephalitis, but no single genogroup of virus appears responsible for severe disease. SUMMARY: Despite the near eradication of poliomyelitis, AFP caused by viruses remains an important clinical presentation. Distinguishing direct viral causes from GBS is important for public health reasons, and to avoid inappropriate therapies.

Acute Disease↗

Botulinum A toxin injection. Failures in clinical practice and a biomechanical system for the study of toxin-induced paralysis.

Botulinum A toxin injection has great utility in the treatment of essential blepharospasm and other facial spasm disorders. Several investigators have noted the failure of botulinum toxin injections to relieve lid spasm in occasional patients and a decrease in effectiveness or duration of effect following multiple injections in other patients. We reviewed the charts of 30 consecutive patients presenting for the evaluation or treatment of facial dystonia. Of 20 patients who had received multiple injections of botulinum toxin, 10 patients were felt to be treatment failures. A new biomechanical system was developed to investigate the duration and degree of paralysis induced in the gastrocnemius muscle of the rat. Animals were treated with four sequential injections at 6-week intervals to the same muscle, resulting in muscle atrophy and an increase in the duration and degree of muscle paralysis, contrary to clinical findings in humans. The review of patient data confirms that, for many patients, repeated injection of botulinum toxin results in a decrease in duration and degree of effect despite an increased toxin dose. An opposite effect was noted in our experimental model because of progressive muscle atrophy.

Adult↗

Diaphragmatic paralysis after pediatric cardiac surgery: a retrospective analysis of 34 cases.

Thirty-four cases of diaphragmatic paralysis after pediatric cardiac surgery are reviewed. Differences between pediatric and adult pulmonary physiology account for the increased severity of respiratory distress seen in children with this condition. The efficacy of treatment with endotracheal intubation and continuous positive airway pressure (CPAP) is confirmed. No patient over 3 yr of age required intubation for longer than 2 wk. This finding is consistent with the development of sufficient chest wall stability to compensate for paralysis of the hemidiaphragm. Patients under 3 yr of age, without complicating heart failure, who still required intubation and CPAP 3-4 wk after injury to the phrenic nerve should consider operative plication.

Adolescent↗

Long-term management of diaphragmatic paralysis complicating prosthetic valve replacement.

The exact incidence of diaphragmatic paralysis complicating secondary heart procedures is not known. The postoperative period can be complicated by difficulty in weaning from mechanical ventilation and misinterpretation of the clinical signs of respiratory muscle fatigue for congestive heart failure and acute bronchospasm. We present 3 patients, all of whom had right diaphragmatic paralysis and recurrent respiratory failure after a second mitral valve replacement. Long-term management with night-time ventilation in 2 patients resulted in no further episodes of respiratory failure and physical rehabilitation with exercise tolerance significantly greater than the preoperative state.

Aged↗

Posterior dislocation of the humeral head in association with obstetric paralysis.

Twelve children with obstetric paralysis were diagnosed as having a posterior dislocation of the humeral head. The diagnosis was suspected on clinical grounds and confirmed by computed tomography (CT) scans in all cases. All 12 patients were treated with open reduction via an anterior approach. The age range at the time of surgery was from 7 months to 7 years (average, 2 years and 3 months). All patients were immobilized in a shoulder spica for 6 weeks and a further 6 weeks in an orthosis. All patients were examined by CT scans in the postoperative period, which confirmed a satisfactory reduction in all cases. With a minimal follow-up of 12 months, there have been no redislocations. This article demonstrates that dislocation of the shoulder in association with obstetric paralysis is not rare, as previously described, and shows that once diagnosed, the dislocation can be satisfactorily treated by a single anterior open reduction of the shoulder.

Child↗

Cystic hygroma and facial nerve paralysis: a rare association.

Cystic hygromas most often occur in the neck in children and are benign lesions not associated with cranial nerve palsies. We present two cases in which a facial nerve paralysis was one of the presenting symptoms. The computed tomographic (CT) scans localized the lesions, suggested a diagnosis, and obviated angiography. Secondary otitis media and intraparotid invasion and hemorrhage were responsible for the reversible facial nerve palsies. We suggest that CT of the temporal bone, parotid bed, and neck is the diagnostic procedure of choice in children clinically suspected of having cystic hygroma as the cause of a facial nerve paralysis.

Child↗

CT mediastinum examination in recurrent nerve paralysis.

Twenty-two patients with recurrent nerve paralysis without a cervical cause were studied by CT of the mediastinum. Positive findings were present in 22 cases, including one false-positive case. The CT findings were not apparent on chest X-ray film in five of nine cases with mediastinal lymph node involvement, five of nine mediastinal tumors, and two of three cases of vascular etiology. We suggest that CT of the mediastinum is a good examination to search for the etiology of recurrent nerve paralysis without a known cervical cause.

Aged↗

Hemorrhaged cystic hygroma and facial nerve paralysis: CT and MR findings.

The association of peripheral facial nerve paralysis and cystic hygroma is rare, this being only the third reported case. In this patient paralysis was secondary to hemorrhage within the cyst and nerve compression. Although the magnetic resonance studies allowed the diagnosis to be made eventually, the complex signal intensities due to excessive bleeding within the cyst confused the issue. In this case, CT allowed an immediate diagnosis to be established.

Adult↗

Changes of the neuronal endoplasmic reticulum in the peripheral nervous system in mutant hamsters with hind leg paralysis and normal controls.

The fine structure of the spinal root and gasserian ganglia of the mutant hamster with hind leg paralysis and of normal controls was examined. Three types of whorl-like or lamellar alteration of the endoplasmic reticulum were encountered but these were also found in the normal control animals and are therefore probably not related to the paralysis of the mutants. These alterations of the endoplasmic reticulum were similar to or identical with those seen in a variety of other conditions and in other types of cells. In the hamster, however the three configurations could sometimes be seen within the same membranous accumulations and are probably related to one another in a developmental fashion.

Animals↗

Solitary paralysis of the triceps muscle due to trauma.

We report a rare solitary paralysis of the triceps muscle. The patient, an 18-year-old man who had had a work-related accident, sustained a severe contusion of the proximal posterolateral arm after being struck by a piece of lumber. This injury produced an extraordinary amount of soft-tissue swelling in the region of the trauma. After the accident it was noted that active flexion of the elbow was preserved, but active extension was absent. Three months after injury, surgical exploration of the radial nerve and its rami musculares to the three heads of the triceps muscle was undertaken. Dense adhesions were found fixing the rami musculares to the surfaces of the triceps muscle. Neurolysis of the rami musculares was performed, leading to complete recovery of the triceps muscle. The etiology of this isolated paralysis of the triceps muscle was determined to be dense adhesions compressing and compromising the function of the rami musculares to the three heads of the triceps muscle. These adhesions formed because of severe posttraumatic bleeding into the potential posterior humeral space, where the rami musculares are found.

Accidents, Occupational↗

Longitudinal evaluation of vocal function after thyroplasty type I in the treatment of unilateral vocal paralysis.

This study investigated longitudinal changes of vocal efficiency and stability after primary thyroplasty type 1. Fifty-three patients with unilateral vocal-fold paralysis underwent vocal-function evaluation preoperatively and at periodic intervals of 1, 3, and 6 months postoperatively. Vocal-function assessment included videostrobolaryngoscopic examination, acoustical and aerodynamic analysis, and perceptual judgment of voice characteristics. Parameters that included glottic-gap size, maximum phonation time, glottic-flow rate, jitter, harmonic/noise ratio, breathiness, hoarseness, loudness, and phrasing showed significant improvement after thyroplasty and remained stable as early as 1 month postoperatively, with only slight fluctuations over a 6-month period. Postoperative voice outcome was not affected by age, sex, duration of vocal symptoms, cause of paralysis, or preoperative pulmonary function.

Adolescent↗

Early eyelid rehabilitation in facial nerve paralysis.

Upper-lid gold-weight insertions and lower-lid-shortening procedures are standard surgical techniques used to restore eyelid function and protect the cornea in patients with facial nerve paralysis. Different opinions exist in the literature regarding the correct timing and the morbidity of these interventions. The retrospective analysis of 45 patients over a 5-year period revealed extrusion of the gold weight in one (2.2%) patient and delayed infections in three (6.6%). Sixty percent of all gold-weight insertions were performed within 4 weeks after the onset of facial nerve paralysis. We strongly favor gold-weight insertion, often combined with lower-lid-shortening procedures, as a simple, reliable, reversible, and successful technique for early rehabilitation of the paralyzed eyelid. Using these guidelines, we have markedly reduced the need for tarsorrhaphies.

Adolescent↗