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The management of blepharospasm and hemifacial spasm.

The aetiology of blepharospasm and hemifacial spasm is different, but both produce involuntary eye closure and facial movements which do not respond to systemic drug treatment. The introduction of therapeutic focal muscle weakening with botulinum toxin injections in the early 1980s appeared to offer great promise in the management of these conditions. In this paper the results of botulinum toxin treatment of 234 patients with blepharospasm and 73 patients with hemifacial spasm over a 7-year period have been analysed. Most patients receive sustained benefit from repeated injections whilst side-effects become less frequent. A clinically recognisable subgroup of patients with blepharospasm respond poorly and may be better treated surgically.

Blepharoptosis↗

Biofeedback and self-regulation in the treatment of diffuse esophageal spasm: a single-case study.

Diffuse esophageal spasm is an uncommon esophageal motor disorder characterized clinically by chest pain and difficulty swallowing. A case is described in which failure of conventional medical treatment in the face of continued weight loss led to consideration for surgery. The need for surgery was obviated by the successful application of relaxation training, which resulted in a significant reduction in painful esophageal spasms, weight gain, and an improvement in psychological state; there was, however, no change in the underlying esophageal motor disorder. The use of a second coping strategy--double swallowing--had an additional beneficial effect, and clinical improvements were maintained and increased at 6-month follow-up. The rationale and the effects of this method at the physiological level are described. It is suggested that double swallowing is a promising new approach to helping patients cope with disordered esophageal motility and that its more general applicability warrants further study.

Adult↗

Spasm of gastric muscularis mucosae might play a key role in causing focal mucosal ischemia and ulceration. An experimental study in guinea pigs.

While transilluminating guinea pig gastric wall, we noticed foci of irregular compression of submucosal veins and arteries, featuring parallel bands across arteries, with complete cessation of blood flow, mucosal pallor, and shrinkage of the area. Other features demonstrated that this was not due to artifactual pressure. We attribute it to compression of vessels by spasm of the muscularis mucosae, because these vessels are embedded in the muscle. Arteries and veins returned to normal with normal mucosal perfusion, either spontaneously within 5 min, or within 5 sec following mucosal stimulation. However, in 20%, "ischemia" remained for 2-3 hr, resulting in full-thickness necrosis of the underlying mucosa but not in surrounding areas. Since in man mucosal arteries pass through the muscularis mucosae before reaching mucosa, and in view of evidence that they may be end arteries, we suggest that stress-induced spasm of muscularis mucosae may play an important role in human acute and chronic ulceration.

Animals↗

Pneumatic dilatation in patients with symptomatic diffuse esophageal spasm and lower esophageal sphincter dysfunction.

Nine patients with severe symptoms of diffuse esophageal spasm and lower esophageal sphincter dysfunction who were unresponsive to medical therapy and bougienage dilatation were treated by forceful pneumatic dilatation. Treatment with pneumatic dilatation in eight of the nine patients produced a marked improvement in dysphagia and regurgitation (average follow-up of 37.4 months). Esophageal motility performed up to three years (average 12.4 months) after clinically successful pneumatic dilatation revealed a decrease in lower esophageal sphincter pressure from 34.0 +/- 4.0 mm Hg (mean +/- standard error) to 19.2 +/- 2.7 mm Hg (P less than 0.01). There were no significant changes in either the percentage of lower esophageal sphincter relaxation or the type of esophageal motor pattern. We conclude from this study that pneumatic dilatation is an effective form of therapy for a select group of patients with severe symptomatic diffuse esophageal spasm with lower esophageal sphincter dysfunction who are unresponsive to conventional medical therapy.

Adult↗

Muscle spasm induced sympathetic reflex bursts on microneurography in a case with pontine demyelination.

Microneurography was performed in a 39-year-old woman with demyelination of the pontine white matter associated with muscle spasms in the lower extremities. Single bursts on the microneurogram were observed immediately after cessation of the spasm with no systemic changes in the blood pressure or heart rate. Voluntary tonic flexion of the lower extremities induced similar bursts with small amplitudes. These reflex bursts possessed a characteristic of muscle sympathetic nerve activity, because the latency between the peak of each burst and the prior R-wave on the electrocardiograph was constant. The occurrence of these bursts suggests that a segmental compensatory mechanism in the spinal cord may stabilize the muscle blood flow influenced by muscle contraction.

Adult↗

Management of diffuse esophageal spasm with balloon dilatation.

Of 1200 patients referred to the esophageal laboratory at Guy's Hospital for investigation of suspected esophageal motility disorders, 61 (5.1%) were diagnosed as diffuse esophageal spasm. Twenty of these patients whose symptoms were severe did not respond to conservative treatment and were treated by balloon dilatation. Results were good in 14 and poor in six patients, which included one esophageal perforation. Diffuse esophageal spasm was diagnosed where more than 30% nonperistaltic activity was demonstrated by manometry. Lower esophageal sphincter pressure and relaxation were normal in all cases except one. Gastroesophageal reflux was present in four of five poor responders who were examined by 24-h ambulatory pH monitoring, and in only one of 10 good responders. Three of the six patients in whom balloon dilatation was successful proceeded to full-length myotomy, with relief of symptoms in two. The indications for, and results of, balloon dilatation in this condition are discussed, and a new radiological sign is described.

Adult↗

Computerized tomography of brain in infantile spasms (West syndrome).

Computerized tomographic scanning of the brain was performed in 26 infants with Infantile spasms. Majority of the patients, 18 (69%) had some abnormality. Changes noted were cerebral atrophy in 12, calcifications in 5 and dysgenesis of the corpus callosum in 3 patients. One infant each had porencephaly, hydrocephalus and cavum septum pellucidum. Five patients had more than one abnormality simultaneously. Three infants had progressively worsening atrophy on ACTH therapy. Patients with infantile spasms, without any physical or neurological abnormality, are unlikely to have any abnormalities on brain CT scanning (p = less than 0.005).

Atrophy↗

Recurrent pneumonia caused by diffuse oesophageal spasm.

A 14-month-old severely retarded girl with a history of regurgitation, aspiration, and recurrent pneumonia was found to suffer from diffuse oesophageal spasm. This diagnosis was made by oesophageal cineradiography. This case suggests that diffuse oesophageal spasm is an oesophageal motility disorder that causes respiratory disease in the retarded child.

Esophageal Spasm, Diffuse↗

Ampulla of Vater/duodenal wall spasm diagnosed by antroduodenal manometry.

Patients with chronic epigastric to right upper quadrant pain are often considered to have gallbladder of sphincter of Oddi dysfunction, but standard tests are nondiagnostic. In 62 consecutive patients with this compliant undergoing antroduodenal manometry, we correlated a change on duodenal motility with spasm of the ampulla of Vater/duodenal wall. This distinctive motility pattern occurred and was analyzed in 35% of patients. It is characterized by increased duodenal wall tone with phasic contractions of 19-22 or 41-44 contractions/min or by phasic activity alone. The subjects with spasm also underwent cholecystokinin cholescintigraphy, and 50% showed either significantly delayed gallbladder emptying of hilum to small intestine emptying, or both. The disorder appears to be secondary to a loss of neural inhibitory control and a dysfunctional small-bowel pacemaker. Antroduodenal manometry is an essential diagnostic procedure that complements sphincter of Oddi manometry in evaluation of unexplained right upper quadrant pain.

Abdominal Pain↗

Botulinum A toxin treatment for eyelid spasm, spasmodic torticollis and apraxia of eyelid opening.

Botulinum-A toxin (botAtox) was used in the treatment of blepharospasm (BS), idiopathic hemifacial spasm (HFS), idiopathic spasmodic torticollis (ST) and apraxia of eyelid opening (AEO). The injection of 7.5-30 U botAtox per eye spread over 3 or 4 sites in the palpebral part of orbicularis palpebrae (OP) reduced palpebral spasm in 12/13 cases of BS and in 7/8 cases of HFS. The effect lasted for 14.5 weeks on average (range 4-30 weeks). Palpebral ptosis (lasting 1-3 weeks) was the most frequent side effect (16/107 eyes treated) but was not related to dose of botAtox or number of inoculation sites. Injection of 60-160 U botAtox into the sternocleidomastoid, trapezius and splenius capitis muscles reduced ST objectively in 1/4 patients for about 4 weeks. In the other patients the reduction or abolition of the hypertrophy of the previous hyperactive muscles was accompanied by persistence or rearrangement of the dystonia pattern, suggesting a change in the pattern of activity of the neck muscles after botAtox. 5 U botAtox per eye spread over 4 sites in the OP significantly reduced the frequency of the episodes of involuntary eyelid closure in 2 patients with AEO but not BS. The therapeutic effect lasted for 7 months after the first treatment and for 8 months after the second in a 46 year old woman with a 6 month history while the second patient (72 year old parkinsonian) has now completed her 3rd month of treatment.

Adult↗

Intracranial microvascular decompression for "cryptogenic" hemifacial spasm, trigeminal and glossopharyngeal neuralgia, paroxysmal vertigo and tinnitus: II. Clinical study and long-term follow up.

20 patients who had undergone microvascular decompression for the treatment of "idiopathic" trigeminal neuralgia (9 cases), hemifacial spasm (7 cases), glossopharyngeal neuralgia (3 cases) and paroxysmal vertigo and tinnitus (1 case) were followed up for 25 months on average. Permanent relief of symptoms was observed in 19 (95%), with sparing of cranial nerve function. Analysis of the clinical data shows that the patients described in the present series did not differ from those considered to suffer from "idiopathic" cranial nerve dysfunction syndromes. The importance of vascular cross compression as etiological factor in such conditions is stressed and the pathophysiology discussed. The term "cryptogenic" applied to trigeminal neuralgia or hemifacial spasm thus needs revising. Lastly, the indications of microvascular decompression in the treatment of "cryptogenic" cranial nerve dysfunction syndromes are defined.

Adult↗

Hemifacial spasm associated with Marfan's syndrome: a case report.

The authors describe a case of hemifacial spasm associated with Marfan's syndrome. A 24-year-old-woman suffered from left hemifacial spasm for 6 years. She had undergone surgical treatments for bilateral ectopia lentis and kyphoscoliosis in the second decade of life. She also had unusually long and slim limbs with arachnodactyly. Three-dimensional CT angiography revealed bilateral tortuous and elongated vertebral arteries. Microvascular decompression was performed following a left lateral suboccipital craniotomy. The root exit zone of the left seventh nerve was directly compressed by the proximal segment of the anterior inferior cerebellar artery (AICA) and the left vertebral artery. These arteries were decompressed with pieces of Teflon cotton. The patient's symptoms completely resolved following surgery. We review cerebrovascular disorders in Marfan's syndrome and discuss the pathogenesis and possible mechanisms of vascular compression in these patients.

Adult↗

Transient hemifacial spasm associated with subarachnoid brainstem cysticercosis: a case report.

The authors present the case of a 69 year old woman who developed bladder incontinence and confusion. A CT scan showed severe hydrocephalus and calcifications, prompting a ventriculoperitoneal shunt placement. On the day after operation the patient presented left hemifacial spasm. MR revealed a subarachnoid cysticercus rostral to the pons, and surgical excision was proposed. She refused surgery and was placed on prednisone. Brainstem auditory responses were absent on the left side 2 months after shunting and were still abnormal 53 months later. The hemifacial spasm disappeared 3 months after shunt placement and has not recurred in 5 years of follow-up.

Aged↗

The effect of intravesically administered Cetiprin on bladder spasms after transurethral resection of the prostate. A double-blind randomized study.

A double-blind study of the short-term effects of intravesically administered emepronium bromide (Cetiprin) versus placebo on bladder spasms after transurethral prostatectomy was done in 40 male patients. In the Cetiprin group there was a significant decline in the need for analgesia, and significantly fewer catheter problems. The use of intravesically instilled Cetiprin is recommended only in cases of severe postoperative bladder spasms.

Administration, Intravesical↗

Previous cholecystectomy and choledochal sphincter spasm after morphine sedation.

PURPOSE: To describe a patient with probable choledochal sphincter spasm after preoperative morphine, and to suggest a history of cholecystectomy as a predisposing factor. CLINICAL FEATURES: A 60 yr old man for femoral-popliteal artery bypass grafting developed right upper quadrant abdominal pain after preoperative morphine and scopolamine. He had a previous cholecystectomy. His pain was relieved with naloxone. CONCLUSION: Choledochal sphincter spasm should be part of a differential diagnosis in right upper quadrant pain after sedative doses of morphine, especially if there is a previous cholecystectomy.

Cholecystectomy↗

Bladder spasm in children after surgery for ureteric reimplantation.

The primary aim of this study was to prospectively investigate whether there was an association between the duration of post-operative catheterisation and the incidence of bladder spasm in children following ureteric reimplantation surgery. A secondary aim was to investigate whether other surgical and post-operative factors had an effect on the incidence of bladder spasm.

Adolescent↗