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Microvascular decompression for hemifacial spasm.

The authors report the results of 782 microvascular decompression procedures for hemifacial spasm in 703 patients (705 sides), with follow-up study from 1 to 20 years (mean 8 years). Of 648 patients who had not undergone prior intracranial procedures for hemifacial spasm, 65% were women; their mean age was 52 years, and the mean preoperative duration of symptoms was 7 years. The onset of symptoms was typical in 92% and atypical in 8%. An additional 57 patients who had undergone prior microvascular decompression elsewhere were analyzed as a separate group. Patients were followed prospectively with annual questionnaires. Kaplan-Meier methods showed that among patients without prior microvascular decompression elsewhere, 84% had excellent results and 7% had partial success 10 years postoperatively. Subgroup analyses (Cox proportional hazards model) showed that men had better results than women, and patients with typical onset of symptoms had better results than those with atypical onset. Nearly all failures occurred within 24 months of operation; 9% of patients underwent reoperation for recurrent symptoms. Second microvascular decompression procedures were less successful, whether the first procedure was performed at Presbyterian-University Hospital or elsewhere, unless the procedure was performed within 30 days after the first microvascular decompression. Patient age, side and preoperative duration of symptoms, history of Bell's palsy, preoperative presence of facial weakness or synkinesis, and implant material used had no influence on postoperative results. Complications after the first microvascular decompression for hemifacial spasm included ipsilateral deaf ear in 2.6% and ipsilateral permanent, severe facial weakness in 0.9% of patients. Complications were more frequent in reoperated patients. In all, one operative death (0.1%) and two brainstem infarctions (0.3%) occurred. Microvascular decompression is a safe and definitive treatment for hemifacial spasm with proven long-term efficacy.

Adolescent↗

Clinical usefulness of magnetic resonance cisternography in patients having hemifacial spasm.

To evaluate the usefulness of MR cisternography fourteen patients that had hemifacial spasm and 20 control patients underwent MR cisternography. All the patients with hemifacial spasm had a confirmed vascular compression after surgery. MR cisternography was performed using a 1.5-tesla superconducting MR magnet in which a 3D (dimensional) heavily T2-weighted turbo spin-echo sequence was used. In 34 randomly selected individuals, we retrospectively determined whether MR cisternography images could be used to evaluate symptoms, and what the benefits of obtaining this image was. The results were correlated with the surgical findings. The sensitivity was 100% and the specificity was 94% in all patients having a hemifacial spasm. The offending vessels were the anterior inferior cerebellar artery (AICA) in six patients cases, the posterior inferior cerebellar artery (PICA) in six, both the vertebral artery and PICA in one, and the vertebral artery in one. All the images showed good resolution and contrast, and also showed the exact correlation between the facial nerve and intracranial vessels in the multiplaner image. The findings of neurovascular compression were well correlated with the surgical findings. We believe that high-resolution 3D MR cisternography is a very useful method for evaluating the neurovascular compression in patients that have hemifacial spasm.

Adult↗

Ependymoma of the fourth ventricle presenting with hemifacial spasm. Report of a case.

According to Gardner's hypothesis (1962) later confirmed by Jannetta (1982, 1985), hemifacial spasm can usually be related to a "vascular conflict" which takes place inside the cerebellopontine angle (CPA). Occasionally, the causative lesion can be identified as a mass encasing the facial nerve at its root exit zone (REZ) from the brain stem. The hemifacial spasm has been rarely reported in presence of a contralateral CPA mass ("false localising sign"). Hemifacial spasm in patients with masses in anatomical regions other than the CPA has to be considered exceptional. The case of an adult man harboring an ependymoma of the fourth ventricle whose only neurological sign was a left hemifacial spasm is reported. The rarity of such a condition prompted us to review the literature. Particular attention has been paid to the possible pathogenetic mechanisms and their therapeutic implications.

Brain Neoplasms↗

[Hemifacial spasm, quality of life and depression].

INTRODUCTION: Quality of life, a concept which has become increasingly important in recent years, refers to a patient's perception of himself in relation to his illness and its treatment. This is compared with the concept of quantity of life or survival. This approach is in keeping with the concepts of health of the World Health Organization. Hemifacial spasm is a disorder of movement with a prevalence of 7.4 and 14.5 per 100,000 men and women respectively. OBJECTIVE: To evaluate the quality of life of patients with hemifacial spasm, study the occurrence of depression and ascertain whether it correlates with the severity, response to treatment and muscle groups involved. PATIENTS AND METHODS: We present a cohort of 57 patients (average age 53.4 years) with hemifacial spasm, in whom the disorder had been present for an average of 3.7 years. RESULTS: It was found that in these patients the quality of life and index of depression were worse when the severity of the disease was greater, response to treatment poorer and there was generalized hemifacial paralysis, (p < 0.00000) for each parameter. The deterioration in quality of life was correlated with depression (correlation 0.6) for the criteria of severity and muscle groups involved, but not with the response to treatment (correlation 0.3). CONCLUSION: Hemifacial spasm significantly alters the quality of life of patients and is associated with depression. This should be taken into account when treating such patients.

Depression↗

[Detection of coronary artery spasm by the methylergometrin test. Technic. Results. Indications].

Methylergometrine (Methergin) was given intravenously (0.4 mg) to 118 patients undergoing coronary arteriography. The electrocardiogramme and intraaortic pressure was continuously monitored whilst coronary arteriography was performed, 1,3, and 5 minutes after the injection of the ergot alkaloid. The test was positive if: 1) coronary spasm was observed; 2) if ST segment elevation was recorded with or without pain. Positive tests were obtained in 13 out of 14 patients with Prinzmetal angina. The test was negative in the other patients. However in 3 patients with Prinzmetal angina, the test produced typical coronary spasm without electrocardiographic changes. In Prinzmetal angina the sensitivity of this test was 93 p. 100 with a high specificity: 96-100 p. 100 depending on whether or not electrocardiographical changes associated with spasm are considered. Taking into account current therapeutic methods of treating Prinzmetal angina the indications of this test of coronary spasm are: 1) patients presenting with resting angina whatever the state of their coronary arteries; 2) patients with documented Prinzmetal angina with "angiographically normal" coronary arteries.

Angina Pectoris, Variant↗

The use of vigabatrin in infantile spasms in Asian children.

BACKGROUND: The use of adrenocorticotrophic hormone (ACTH) and prednisolone in the management of infantile spasms has been well established, but is associated with significant morbidity and cannot be used as long-term medication. Since the introduction of vigabatrin, results have been promising with suggestions currently that it should be used as first-line management of infantile spasms. AIM: The aim of this study was to establish the efficacy, tolerability and problems associated with the use of vigabatrin, in Asian children with infantile spasms. MATERIALS AND METHODS: Eighteen local Asian patients with infantile spasms were given vigabatrin, in 8 as first-line monotherapy and in 10 patients as add-on therapy to pre-existing anti-convulsants. RESULTS: Thirty-nine per cent (7/18) showed 100% suppression of seizures at 2 weeks and 50% (9/18) showing complete suppression of seizures at 4 weeks after starting therapy. There was however, a high relapse rate (56%) in complete responders within the first 6 months of therapy. This was probably due to a lower maintenance dose in the patients, as those who relapsed were on an average dose of 59 mg/kg/day at the time of relapse and responded to a stepped up dose of 83 mg/kg/day. Vigabatrin was well tolerated and only 2 patients developed somnolence and irritability. CONCLUSION: A dose of at least 70 mg/kg/day may be necessary to achieve adequate control and yet avoid the common adverse affects of vigabatrin.

Anticonvulsants↗

Treatment of symptomatic diffuse esophageal spasm by endoscopic injections of botulinum toxin: a prospective study with long-term follow-up.

BACKGROUND: Diffuse esophageal spasm is a rare esophageal motility disorder for which there are no satisfactory pharmacologic alternatives for treatment. The aim of this study was to investigate whether botulinum toxin (BTX) injection is an effective short- and long-term treatment for patients with symptoms caused by diffuse esophageal spasm. Whether recurrence of clinical symptoms can be successfully retreated by BTX injection was also studied. METHODS: Nine symptomatic patients (6 women, 3 men; 57-86 years) with manometrically proven diffuse esophageal spasm underwent BTX injection. One hundred IU BTX were diluted in l0 mL of saline solution and injected endoscopically at multiple sites along the esophageal wall beginning in the region of the lower esophageal sphincter and moving proximally in 1- to 1.5-cm intervals, and into endoscopically visible contraction rings. Symptom scores based on an analogue scale for dysphagia, regurgitation, and noncardiac chest pain were assessed before and after therapy, 1 day thereafter, and at 1 and 6 months. RESULTS: Symptoms improved immediately in 7 (78%) patients after 1 injection session. After 4 weeks 8 (89%) patients were in remission with a decrease in total symptom score. The total symptom score decreased from a median 8.0 (interquartile range: 6.75; 9.0) before treatment to 2.0 (1.5; 3.75) after 1 day (p < 0.01) and to 2.0 (interquartile range: 0.75; 3.0) after 1 month (p < 0.01). After 6 months all 8 patients with a response at 1 month still had a symptom score of 3 or less without further treatment. Subsequently 4 patients required reinjection 8, 12, 15, or 24 months after the initial treatment with similarly good results. No serious adverse effects were observed. CONCLUSIONS: BTX injection at several levels of the tubular esophagus is an effective treatment for patients with symptoms caused by diffuse esophageal spasm. Symptom relapse can be effectively treated by repeated BTX injection.

Aged↗

[Efficacy and tolerance of gabapentin in dystonia and hemifacial spasm: a pilot study].

INTRODUCTION: The pharmacological management of dystonia is suboptimal. Focal dystonia and hemifacial spasm can be successfully managed with botulinum toxin injections. The physiopathology of dystonia remains a mystery. The gabaergic transmission may be involved in the origin of dystonia. Gabapentin is a gabaergic agent and therefore it can be useful in dystonia. PATIENTS AND METHODS: 28 patients with different forms of dystonia and hemifacial spasm were included in this open study. They received gabapentin (mean dose 1885 mg/d). RESULTS: Gabapentin was not efficacious but a subgroup of 6 patients (3 with hemifacial spasm, 2 with posttraumatic dystonia and 1 with spasmodic torticollis) significantly improved. Gabapentin was well tolerated. Somnolence was the most commonly reported side effect. CONCLUSION: Gabapentin may be useful in some patients with dystonia and hemifacial spasm. Double blind studies are clearly needed.

Acetates↗

[Hemifacial spasms caused by neurovascular compression].

Three patients, one woman aged 52 years and two men aged 63 and 71 years, respectively, had involuntary movement on one side of their face due to hemifacial spasms. The first patient's spasms were misdiagnosed as a tic, the second patient had received injections of botulinum A toxin which gave no improvement and the third patient suffered from persistent symptoms after a first neurovascular decompression without intraoperative EMG monitoring. All three patients underwent microvascular decompression of the facial nerve with intraoperative EMG monitoring. All three patients were cured. Hemifacial spasm is a curable illness if appropriately diagnosed and treated. Oral medications have no effect on the disease. Local injection of botulinum A toxin is indicated when the spasm is mild or when surgery is contraindicated. The primary causative factor is vascular compression of the facial nerve at its exit zone. Therefore, decompressive surgery is the logical treatment, and the best results are obtained with intraoperative EMG monitoring.

Aged↗

[A surgical case of hemifacial spasm caused by a tortuous, enlarged, and calcified vertebral artery].

Microvascular decompression (MVD) is an established procedure to treat hemifacial spasm and trigeminal neuralgia. However, this cannot be done in some cases in which ecstatic vertebrobasilar arteries are involved. In these instances, alternative techniques must be used. We encountered a patient with hemifacial spasm caused by a calcified, enlarged, and tortuous vertebral artery. To obtain safe and certain neurovascular decompression of the facial nerve, the tortuous and calcified vertebral artery was surgically repositioned by placing layers of Teflon felt at two regions between the brain stem and the artery. After surgery hemifacial spasm completely disappeared. This method is thought to be a useful option for the treatment of hemifacial spasm caused by a tortuous, enlarged, or calcified vertebral artery.

Calcinosis↗

[Microsurgical vascular decompression for hemifacial spasm. Follow-up over one year, clinical results and prognostic factors. Study of a series of 100 cases].

BACKGROUND AND PURPOSE: The results of a series of 100 patients operated on for hemifacial spasm (HFS), using microsurgical vascular decompression (MVD), are reported. METHOD: MVD was performed through a retromastoid keyhole approach, under monitoring of brainstem auditory evoked potentials (BAEP) and facial EMG, and consisted in dissection of VII nerve from conflicting vessel(s), and interposition of Teflon fibers and/or screen(s). RESULTS: The offending vessels found were: the antero-inferior cerebellar artery in 57 cases, the postero-inferior cerebellar artery in 56 cases, the vertebrobasilar artery in 22 cases. A multiple conflict was found in 32 cases (32%). The result was considered excellent if there was no residual spasm, good if only "minimal twitching" remained with relief>80%, poor for spasm relief 20 to 80%, and as a failure if relief<20%. The effect of MVD was satisfying (excellent or good) in 75 patients (75%) at discharge (10th day) and in 85 (85%) after 1 to 18 years follow-up (mean: 5 years). Amongst the latter patients, 29 (34%) experienced a delayed (up to 3(1/2) years in one) cure. Spasm recurrence was noted in 9 cases after satisfying effect on discharge. We encountered following permanent neurological complications: 1 facial palsy, 7 cases of hearing deficit (5 of them complete), and 1 case of IX-X deficit. Neither death nor ischaemic complication at brainstem or cerebellum. Most of our hearing complications occurred before using intraoperative BAEP monitoring (3 cases of cophosis among our first 7 patients vs 2 out of our last 93). Local complications were: 1 meningitis, 8 cases of CSF leakage requiring either a series of lumbar punctures or a lumbar external drain, and 3 cases of wound infection and/or delayed woundhealing requiring surgical treatment. CONCLUSIONS: Our data are consistent with those of the literature, especially concerning high rate of long-term success and low complication rate of MVD for HFS. We do not recommend early re-operation in case of initial poor result. Again, the necessity of intraoperative BAEP monitoring to prevent hearing morbidity is highlighted.

Adult↗

[Recording muscle spasm in the musculus erector spinae using in vivo 31P magnetic resonance spectroscopy in patients with chronic lumbalgia and generalized tendomyopathies].

Using 31P nuclear magnetic resonance, the following parameters were determined in the resting musculus erector spinae of five patients suffering from chronic low back pain, five patients with fibromyalgia, and five healthy controls: Inorganic phosphate (Pi), phosphocreatine (PCr), ATP gamma, ATP alpha, ATP beta. The intracellular pH was derived from the chemical shift of Pi referenced to the PCr resonance. In addition, the Pi-Index was calculated according to the formula: Pi/(Pi + PCr). We discovered a tendency towards a shift of the Pi resonance in the alcalic direction, which was the larger, the stronger muscle spasm was found on palpation. The pH showed the most reliable relationship to the clinical status of muscle spasm. The surprising finding that there is no acidification within the spasmed muscle indicates that generalized hypoxia does not exist in this tissue. This has already been shown with PO2 measurements. An intracellular acidification is only recorded during maximal isometric contraction. Thus, ischemia cannot be responsible for pain experienced during muscle spasm.

Acid-Base Equilibrium↗

Vascular loop compressing facial nerve in hemifacial spasm: demonstrated by 3D-phase contrast magnetic resonance angiography in 101 patients.

BACKGROUND: Vascular compression of the facial nerve is deemed to be the common cause of hemifacial spasm producing emphatic transmission. Although facial nucleus supersensitivity is more accepted as the main cause of hemifacial spasm. PURPOSE: To determine the vascular loop compression of the facial nerve in patients with hemifacial spasm by 3D-phase contrast (PC) magnatic resonance imaging (MRI). MATERIAL AND METHOD: A retrospective study of 101 patients with hemifacial spasm who went MRI and magnetic resonance angiography (MRA) of the brain was done. The magnitude images of the 3D-PC MRA was evaluated in axial and oblique coronal reconstruction planes blindly from symptomatic information. RESULTS: Among 101 patients, 53 affected the left side, 48 patients were right sided and none had bilateral involvement. Vascular loop compressing on the symptomatic side was found in 61 (60.4%) patients. For the asymptomatic side, there were 14 (13.86%) with vascular loop contact. Five patients (4.9%) had bilateral vascular compression. The proportion of vascular contact of the symptomatic and asymptomatic side was significantly different (with p < 0.001). The offending vessels were vertebral artery (32, 52.46%), posterior inferior cerebellar artery (7, 6.93%), anterior inferior cerebellar artery (6, 5.94%) and artery of uncertain origin (16, 26.23%). CONCLUSION: The study implied the usefulness of this simple technique to demonstrate the neurovascular contact of the facial nerve.

Adult↗

[Selective facial neurectomy for hemifacial spasm].

Twenty-six patients were treated for hemifacial spasm by selective facial neurectomy. The symptoms of hemifacial spasm can be relieved by performing extracranial selective facial neurectomy with preservation of the temporal and cervical ramus. The facial nerve branches have been identified by selective electrical stimulation over the nerve, which evokes responses from the corresponding facial muscles. Selective facial neurectomy were completed when stimulation of the nerve produces minimal movement in the orbicularis oculi muscle while normal contractions appear in the frontal region and lip portion. The results of surgery 2 years postoperatively indicated that the complete disappearance of hemifacial spasm was achieved in 65.6 percent of the patients. A slight relief was achieved in 15.5 percent of the patients. Unsatisfactory results were obtained in 11.5 percent. No spasm recurred in 7.5 percent of patients, but paralysis of frontal muscle persisted.

Adult↗

[Infantile spasms].

BACKGROUND: Infantile spasms are an epileptic manifestation typical of infancy. The spasms may be associated with a wide spectrum of brain abnormalities and diseases, but coexisting pathology is not always found. Prognosis depends to a substantial extent on the underlying condition. Most of the traditional antiepileptics are not effective. Adrenocorticotropic hormone (ACTH) and more recently vigabatrin are the most common first-line medications. MATERIAL AND METHODS: We have performed a literature search mainly in the PubMed database on the treatment of infantile spasms. The results are discussed in relation to Norwegian practice, for which the Norwegian Paediatric Society's recommendations are the main guidelines. RESULTS/INTERPRETATION: Vigabatrin has emerged as an accepted first-line medication for infantile spasms in Norway and many other countries, although it has not been shown that this therapy in general is as effective as ACTH/steroids. Well-designed treatment studies have been difficult to carry out. Until the final results of such ongoing studies should give different signals, it seems reasonable to continue to give vigabatrin before hormones. Oral steroids can probably be tried before ACTH, although a firm conclusion about this has yet to be drawn.

Adrenocorticotropic Hormone↗

Hemifacial spasm resulting from vertebral artery dolichoectasia.

A 32-year-old man presented with left hemifacial spasm. Neurophysiological findings revealed an absent ipsilateral R1 on blink reflex. An MRI showed a dolichoectatic left vertebral artery impinging on the root exit zone of the left facial nerve. Botulinum toxin infections relieved the manifestations of hemifacial spasm. This case demonstrates that MRI/MRA is an essential part of the work-up for hemifacial spasm, and shows that in accordance with the literature, vertebral dolichoectasia is an uncommon cause of hemifacial spasm.

Adult↗

Partial seizures triggering infantile spasms in the presence of a basal ganglia glioma.

Infantile spasms associated with brain tumors have been reported. A focal cortical lesion can induce infantile spasms by triggering the brainstem and basal ganglia in this vulnerable age group. We report the case of a female infant with a low-grade glioma in the right basal ganglia, spreading to the cortical area. She presented at the age of five months with left hemiparesis and partial seizures. She developed infantile spasms at the age of 12 months. This is the first video clip report of partial seizures triggering symmetrical spasms in series, secondary to a basal ganglia glioma extending to the cortex. [Published with video sequences].

Anticonvulsants↗

[Delayed hearing loss after microvascular decompression for hemifacial spasm: report of two cases].

We report two unusual cases of delayed hearing loss after microvascular decompression (MVD) for hemifacial spasm. In the first case, A 59-year-old female noted left hearing loss one week after receiving MVD for left hemifacial spasm. In the second case, A 39-year-old male also noticed ipsilateral hearing loss on the 7th day after MVD for right hemifacial spasm. Both cases were treated by steroid. Two months after the onset, their hearing function improved dramatically. These cases indicated that the delayed hearing loss after MVD for hemifacial spasm can occur, even when gentle microsurgical technique is used, but the prognosis for this condition is fairly good.

Adult↗