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Hemifacial spasm: results of electrophysiologic recording during microvascular decompression operations.

Electrophysiologic recordings were made from patients with hemifacial spasm (HFS) during microvascular decompression (MVD) operations to see if spasm and synkinesis are caused by ephaptic transmission at the site of lesion (root entry zone [REZ] of the facial nerve). The response from the orbicularis oculi muscle to electrical stimulation of the marginal mandibular nerve had a 2.2-msec longer latency (average of 16 patients) than the sum of the conduction times of the parts of the facial nerve that would be involved if the response was the result of ephaptic transmission at the REZ of the seventh cranial nerve. Similar results were obtained when the zygomatic branch of the facial nerve was stimulated. These results indicate that the facial motonucleus is involved in HFS.

Action Potentials↗

Hemifacial spasm in sleep.

We conducted polygraphic studies during wakefulness and all-night sleep in 13 patients with cryptogenic and 3 with postparalytic hemifacial spasm. The movements decreased progressively with deepening sleep stages, reaching lowest values in REM sleep. The reduction was inversely related to the severity of movements during wakefulness. There was no relation between hemifacial spasm and mimic activity on the unaffected side. Central inhibitory processes may account for the partial decline in intensity of the movements in sleep.

Adult↗

Hemifacial spasm due to contralateral acoustic neuroma: case report.

A patient with a large acoustic neuroma had contralateral hemifacial spasm. On CT, the brainstem was markedly displaced and distorted by the tumor. After total removal of the tumor the hemifacial spasm was temporarily worse, but disappeared 14 days after the operation.

Facial Muscles↗

Infantile spasms: outcome and prognostic factors of cryptogenic and symptomatic groups.

We reviewed the outcome (developmental, neurologic, and seizure) and prognostic factors of 57 cases of infantile spasms (17 cryptogenic, 40 symptomatic). The mean developmental score of the cryptogenic group (71.2 +/- 24.2) was significantly higher than that of the symptomatic group (48.4 +/- 24.5), as assessed by the Griffith Mental Developmental Scale. A resultant neurologic deficit was present in 23.5% of the cryptogenic group and 75.0% of the symptomatic group. Coexistence of other forms of seizures was observed in 35.3% of the cryptogenic group and 57.5% of the symptomatic group. Outcome was affected by the coexistence of other seizures, presence of neurologic deficit, time lag in initiation of treatment (cognitive outcome only), poor response to ACTH treatment, and persistent EEG abnormality. Character of spasms and time lag in initiation of treatment (seizure outcome only) did not have any significant effect on outcome.

Adrenocorticotropic Hormone↗

Skeletal muscle-specific immunotoxin for the treatment of focal muscle spasm.

Intramuscular injection of botulinum toxin type A (BTX) is used to treat many disorders characterized by muscular spasms. The utility of BTX, however, is limited by its short duration of action, the development of resistance after repeated injections, and cross-reactivity with autonomic neurons. To overcome these limitations, we engineered an immunotoxin (ITX) to damage skeletal muscle fibers selectively by chemically linking a monoclonal antibody against the nicotinic acetylcholine receptor to the toxin ricin. In vitro, the ITX was 20,000-fold more toxic to myotubes than myoblasts, consistent with the degree of acetylcholine receptor expression. The gastrocnemius muscles of 30 rats were unilaterally injected with a series of protein toxins at various concentrations and examined histopathologically 7 and 30 days later. ITX produced destructive myopathic changes at a dose 300-fold less than the maximum tolerated dose. Assessment of rat muscle strength after unilateral gastrocnemius injections showed that ITX was more effective and had a longer duration of action than BTX. ITXs may have potential for the treatment of involuntary muscle spasms.

Animals↗

A case-controlled MRI/MRA study of neurovascular contact in hemifacial spasm.

BACKGROUND: Neurovascular contact (NVC) with the root exit zone (REZ) of the ipsilateral facial nerve is associated with hemifacial spasm (HFS), but unresolved issues remain. OBJECTIVES: To 1) determine the frequency of symptomatic and nonsymptomatic NVC, 2) determine the features of NVC associated with HFS, and 3) correlate severity of HFS to these features. METHODS: Two independent, blinded, prospective assessments of high-resolution MR and MR angiography (MRA) images were performed on Chinese cases (HFS: n = 44; age-matched control subjects: n = 20). RESULTS: Over 88% of 44 symptomatic sides in patients with HFS had NVC of the ipsilateral facial nerve. At least 80% of symptomatic sides involved NVC at the anterior aspect of the REZ [REZ(ant.)]. Although NVC was observed in approximately half of nonsymptomatic sides, at least 70% of them were not at REZ(ant.). NVC at the cisternal and intracanalicular portions of the facial nerve were not associated with HFS. Half of our patients with HFS had bilateral NVC, but none had bilateral symptoms. Most of our MR/MRA images showed that the size and position of the arterial branches of the vertebrobasilar system were markedly asymmetric. Of patients with bilateral NVC, over 83% had asymmetric NVC sites. The anterior inferior cerebellar artery was the most common vessel involved in NVC, but was not significantly associated with HFS. Most of the NVC involved one vessel at one contact point with no indentation. The development of HFS was significantly associated with nerve indentation in NVC. The development and severity of HFS were not associated with multiple contact points in NVC. No significant interobserver variability existed between the blinded assessments. CONCLUSIONS: MRI/MR angiography are accurate, fast, and safe in characterizing neurovascular contact (NVC) at the brainstem. The site of NVC and ipsilateral facial nerve indentation in NVC are significant determinants for the development of hemifacial spasm (HFS). The lack of bilateral NVC at the anterior aspect of the root exit zone of the facial nerve could explain in part the lack of bilateral symptoms. The development and severity of HFS are not associated with a specific blood vessel or multiple contact points in NVC.

Adult↗

Masseter muscle spasm in children: implications of continuing the triggering anesthetic.

This retrospective study was undertaken to examine the management and outcome of children who developed isolated masseter muscle spasm (MMS) after the administration of intravenous succinylcholine during anesthetic induction. The inhalation anesthetics used for induction were continued in all of these cases. The medical records of 68 patients (male/female ratio, 1.7:1), identified from approximately 42,000 anesthetics given during the period 1980-1989, were reviewed. Fifty-seven children (2.3-12 yr old) were diagnosed as having isolated MMS, i.e., MMS without spasm of other muscles; 11 experienced generalized rigidity in combination with MMS. Anesthetic and postoperative management of these two groups differed. The overall incidence of MMS was 0.3% of inhalation anesthetics during which succinylcholine was given. Intraoperative arrhythmias occurred in 33% of the patients who developed isolated MMS and more frequently in older children. Most children experienced some degree of hypercarbia and/or metabolic acidosis, but the significance of these abnormalities in the spontaneously ventilating, fasting child is unknown. Serum creatine kinase levels when measured 18-24 h postoperatively were elevated in all but one child (n = 45). There was no long-term morbidity and no mortality. We conclude that failure of the masseter muscles to relax after succinylcholine is not uncommon in children. Based on our experience, and accepting that MMS may be part of the clinical spectrum of malignant hyperthermia, we believe that anesthesia can be continued safely in cases of isolated MMS when careful monitoring accompanies diagnostic evaluation. This differs from the current practice of discontinuing the anesthetic or switching to a nontriggering anesthetic technique.

Anesthesia, Inhalation↗

Hemifacial spasm: treatment by microsurgical facial nerve decompression.

The literature on hemifacial spasm and its surgical therapy is reviewed, and the authors' experiences with 20 patients are described. Vascular cross compression of the facial nerve adjacent to the brain stem is seen in 90% of the surgical patients. Mobilizing the offending vessel will cure or significantly improve approximately 80% of the patients. Complications occur in 25% of the patients and usually involve decreased hearing or facial weakness. Mortality is virtually zero, and this operation is vastly superior to any other medical or surgical therapy for hemifacial spasm. The pathophysiology is not yet understood.

Adult↗

Microvascular decompression for hemifacial spasm: analyses of operative findings and results in 310 patients.

The operative findings and results of microvascular decompression (MVD) on 310 Chinese patients with hemifacial spasm are analyzed in this report. The operations were performed at the Neurological Institute of the Veterans General Hospital-Taipei between January 1983 and June 1990. The length of follow-up ranged from 6 months to 8 years (mean, 4.3 years); 273 patients (88%) had complete relief of spasm within 3 days after one MVD, and the remaining 37 patients (12%) showed no immediate postoperative improvement. Sixteen (5.2%) of these 37 initially unresponsive patients subsequently experienced complete relief, which occurred from 4 days to 22 months (median, 21 days) after one MVD; 13 others (4.2%) had complete relief immediately after the second MVD; another 3 (1%) had delayed complete relief 6, 9, and 11 months after the second MVD, respectively; and the remaining 5 (1.6%) only had delayed partial relief, which occurred 2 to 9 weeks after one MVD. Late recurrence occurred in three patients (1%). These immediate and long-term results lend support to the conclusion that the timing of reoperation can be postponed for a period of 3 to 4 weeks in the event of an initial failure to get improvement, and that a second MVD may be of value.

Adult↗

Hemifacial spasm resulting from cerebellopontine angle lipoma: case report.

A case of hemifacial spasm associated with a cerebellopontine angle lipoma is described. Both the seventh and the eighth cranial nerves were incorporated and distorted within this tumor, which seemed to be the cause of hemifacial spasm and other cranial nerve dysfunctions, but obvious vascular elements were not included. To identify a cerebellopontine angle lesion as a lipoma is very important in surgical management. Magnetic resonance imaging is essential to the differential diagnosis of the cerebellopontine angle lesion.

Aged↗

Brainstem auditory evoked potential monitoring during microvascular decompression for hemifacial spasm: intraoperative brainstem auditory evoked potential changes and warning values to prevent hearing loss--prospective study in a consecutive series of 84 patients.

OBJECTIVE: The nerve function of Cranial Nerve VIII is at risk during microvascular decompression for hemifacial spasm. Intraoperative monitoring of brainstem auditory evoked potentials (BAEPs) can be a useful tool to decrease the danger of hearing loss. The aim of this study was 1) to assess the side effects of surgery on hearing and describe the main intraoperative BAEP changes observed in the authors' series, and 2) to define warning values beyond which the probability of hearing impairment rises significantly. These values were calculated by correlating the (possible) postoperative hearing disturbances evaluated in terms of pure tone average with intraoperative BAEP changes (especially delay in Wave V latency). METHODS: This series included 84 consecutive patients affected with hemifacial spasm who underwent microvascular decompression during which BAEPs were monitored. During surgery, Wave I, I to V interpeak interval, latency, and amplitude of Wave V were recorded and measured. Auditory function was studied before and after surgery and expressed as a pure tone average in all patients. Then, correlations were made between hearing impairment after surgery and intraoperative BAEP changes in an attempt to define warning values. RESULTS: Seventy-four patients (88%) had no hearing loss after surgery (Group 1). Eight patients (9.5%) had hearing impairment with a decrease in pure tone average of more than 20 dB (Group 2). Two patients (2.3%) experienced a definitive and complete hearing loss on the side operated on (Group 3). Among intraoperative BAEP changes, latency of Peak V was the most frequently observed and the most significant phenomenon, especially during cerebellar retraction and the decompression step of the microvascular decompression procedure. In the group of patients without hearing loss (Group 1), the mean delay in latency of Peak V was 0.61 millisecond (standard deviation, +/-0.36 ms); in the group with hearing decrease (Group 2), the mean delay was 1.05 milliseconds (standard deviation, +/-0.64 ms); and in the group with deafness (Group 3), Wave V was abolished. CONCLUSION: From a practical standpoint, three warning values, based on delay in latency of Peak V, were established for use during surgery: an initial one at 0.4 millisecond ("watching" signal) at the safety limit; a second one at 0.6 millisecond (risk "warning" signal), which is the mean value corresponding to the group of patients without postoperative hearing loss; and an ultimate one at 1 millisecond ("critical" warning), before irreversibility. These warnings should help the surgeon to avoid or correct maneuvers that are dangerous for hearing function, which is mandatory in functional surgery.

Adult↗

A case of rest angina due to microvascular spasm.

A 66-year-old woman underwent elective cardiac catheterization for investigation of periodic attacks of chest pain at rest. During the examination, a chest pain attack occurred unexpectedly, resulting in ST elevation in the precordial leads on electrocardiography. Immediate coronary arteriography demonstrated no organic stenosis but markedly delayed contrast medium perfusion in the mild to distal portion of the left anterior descending artery. These phenomena spontaneously disappeared about 3 min later, and the patient was diagnosed as having angina pectoris due to microvascular spasm. The demonstration of angina pectoris due to microvascular spasm by coronary arteriography during a spontaneous attack is very rare.

Aged↗

Pseudoxanthoma elasticum with dipyridamole-induced coronary artery spasm: a case report.

In patients with pseudoxanthoma elasticum, severe organic coronary artery stenosis often occurs without coronary risk factors. However, this report presents the case of a 49-year-old woman with pseudoxanthoma elasticum who had coronary artery spasm with an angiographically normal coronary artery. In addition, coronary artery spasm was provoked with dipyridamole thallium-201 cardiac imaging.

Coronary Angiography↗

Spasm of a saphenous vein bypass graft. A possible mechanism for occlusion of the venous graft.

The phenomenon of spasm in a venous graft was documented by angiographic study of the graft. This phenomenon has not been reported previously, and, therefore, its frequency of occurrence is unknown. Spasm of a venous graft may prove to play a significant role in the early development of myocardial infarction, closure of the graft, or recurrence of angina after initially successful surgery for aortocoronary bypass when venous grafts remain patent.

Angina Pectoris↗

[Familial hemifacial spasm: report of 2 cases].

The authors report the clinical and angiographical findings of two cases of familial hemifacial spasm. This is the fifth description in the literature and presents mother and daughter at the ages of 76 and 51 respectively, in whom the left side was affected. They underwent exams of angioresonance that showed dolichobasilar with left side origin in both patients. The exams also demonstrated postero-inferior cerebellar artery very developed and irregularities in the walls of the vertebral and basilar arteries suggestive of arteriosclerosis in the mother and slightly elongated intracranial vessels in the daughter. Literature review and etiology data of the hemifacial spasm are focused.

Aged↗

A case of coronary spasm induced by 5-fluorouracil.

Cardiotoxicity is an uncommon adverse effect of 5-fluorouracil (5-FU). Coronary artery spasm has been postulated to be involved in the mechanism of this incident Patients may present with angina, myocardial infarction, arrhythmias and/or even sudden death. When the drug is readministered, there is a high risk of relapse. The underlying mechanisms of cardiotoxicity are not yet fully understood, although coronary vasospasm may be responsible. We report one woman receiving 5-fluorouracil therapy with typical chest pain and electrocardiographic changes consistent with acute coronary syndrome. A resolving pain and normalisation of ECG changes with nitrate therapy and normal coronary arteries indicate that this incident was about a coronary spasm caused by 5-FU.

Acute Disease↗

An overview of the role of calcium antagonists in the treatment of achalasia and diffuse oesophageal spasm.

Early studies confirmed the beneficial effects of calcium channel blockers on the normal oesophagus, which included a decrease in lower oesophageal sphincter tone in achalasia and a decrease in oesophageal contractions and amplitude in diffuse oesophageal spasm. This resulted in the enthusiastic use of the drugs in both disorders. With further experience, and with increased recognition of side effects, the role of these drugs in the 2 disorders has been better clarified. Clinical trials in general have not reflected the improvement observed in the manometric parameters. Only a minority of patients appear to derive sustained symptomatic benefit. Calcium channel blockers may be the initial choice for high or moderate risk patients with achalasia prior to proceeding with pneumatic dilatation or surgical myotomy. In diffuse oesophageal spasm, they are a reasonable first choice for all risk categories.

Calcium Channel Blockers↗