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We describe two children with Williams syndrome and infantile spasms. The diagnosis of Williams syndrome was confirmed by documentation of a deletion of the elastin gene/Williams syndrome region at 7q11.23. The diagnosis of infantile spasms was confirmed through the presence of interictal hypsarrhythmia. This represents one of the first reports of infantile spasms in the Williams syndrome.
Hemifacial spasm features myoclonic-like, paroxysmal, unilateral muscle twitching, attributable to vascular compression at the facial pontine root entry zone. We present the case of an 85-year-old man who presented with idiopathic hemifacial spasm with onset 23 years before. For the last 5 years, he was successfully treated with botulinum toxin injections. However, occasional nitrate intake for precordial pain promptly triggered muscle twitching. Vasodilation may exacerbate not only cases of hemifacial spasm, but even of trigeminal neuralgia, both recognized as neurovascular compressive syndromes.
A simple, safe and reliable method of differentiating between organic obstruction spasm at the uterocornual junction of the oviduct during routine hysterosalpingography is described. Seventeen patients were studied. In all patients uterocornual obstruction was found during hysterosalpingography, which was performed without general anaesthesia. In 8 patients the apparent obstruction was alleviated within 30 seconds of the intravenous administration of 0.25 mg orciprenaline. Surgical and endoscopic findings confirmed the presence of the obstruction in all the other 9 patients. These findings are discussed on the basis of the neuro-anatomy of the Fallopian tube. It is suggested that this form of treatment, in many cases, eliminates the need to perform the procedure under general anaesthesia, which is the only other method of consistently alleviating such a 'spasm'. The use of orciprenaline may facilitate a rapid turnover of patients in units where hysterosalpingography is performed as a screening investigation for infertility. Furthermore, it is suggested that the oral administration of beta-adrenergic agents during the peri-ovulatory period to patients in whom 'tubal spasm' has been diagnosed might offer a rational approach to treatment.
BACKGROUND AND PURPOSE: We applied a 3D fast spin-echo (3D-FSE) MR imaging technique to the preoperative and postoperative evaluation of patients with hemifacial spasm. METHODS: The study group comprised 20 patients. All images were acquired on a 1.5-T MR system with a 3D-FSE sequence. RESULTS: In all 20 patients, the courses of the seventh and eighth cranial nerves were depicted separately, and the arteries presumed to be responsible for the hemifacial spasm were seen to be in contact with the facial nerves at the root exit zone (REZ). Eight patients underwent neurovascular decompression. In all patients, the presumed responsible blood vessels depicted by 3D-FSE MR imaging corresponded to intraoperative findings. In addition, postoperative 3D-FSE images confirmed the separation of the facial nerve from a contiguous vessel at the REZ. DISCUSSION: The 3D-FSE technique makes it possible to obtain extremely high-quality images of microstructures in the cerebellopontine cistern, and it has several advantages over conventional angiography: it is noninvasive and able to depict the cranial nerves and surrounding vessels in the same image without contrast material, and it may be useful for postoperative evaluation of the decompression procedure. This imaging technique is expected to prove useful for the clinical evaluation of hemifacial spasm.
A review of the literature as well as recent surgical observations indicate that hemifacial spasm may be caused by normal or pathological vascular structures that cross-compress the facial nerve. The critical area of compression is invariably found at the brain stem exit zone of the seventh cranial nerve. In this area the central glial investment of the facial nerve changes to peripheral or schwannian myelin. It is suspected that this anatomical junction zone may be of pathophysiologic significance when directly compressed or irritated. A retromastoid craniectomy and vascular decompression operation is highly successful in relieving hemifacial spasm while at the same time preserving facial nerve function. This is in contrast to most commonly used destructive operations for hemifacial spasm. Microsurgical techniques, however, must be employed or high morbidity and mortality may occur from the retromastoid approach.
OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.
A 47-year-old black male who presented with classical angina pectoris and positive exercise test developed spasm of the right coronary artery in the vicinity of an atherosclerotic lesion resulting in transient total occlusion of the vessel. It seemed unlikely that spasm was catheter induced in this case, and the vessel relaxed promptly following nitroglycerin administration. The occurrence of severe spasm in proximity to atherosclerotic narrowing of coronary artery may contribute to stable and unstable angina, myocardial infaction, and sudden death in patients with these conditions coexisting. The awareness of such a phenomenon is necessary to avoid serious errors in the interpretation of coronary angiograms.
The arteriographic distinction between a fixed atheromatous obstruction and localized vasospasm in the coronary artery is often decided by the response of the lesion to nitroglycerin. We studied the time course of nitroglycerin in four patients with coronary artery spasm as revealed by selective angiography. Following complete dissolution of a 0.6 mg tablet of nitroglycerin sublingually a slight increase in heart rate occurred as early as two minutes, variable changes in overall vessel diameter were observed within four minutes, but the localized spasm remained fixed. It was not until six minutes had elasped that reinjection showed disappearance of spasm and uniform patency of the vessel in all cases. These observations stress the importance of waiting an appropriate period of time (at least six minutes) following complete absorption of sublingual nitroglycerin before any conclusion can be rationally drawn regarding the nature of a stenotic lesion as seen angiographically.
We administered ergonovine and used both electrocardiographic monitoring and thallium-201 [201Tl] imaging to detect reversible ischemia in 100 patients. Patients already established as having coronary artery spasm and those with nonbypassed, proximal, high-grade coronary artery stenosis were excluded. No complication occurred in any patient. The use of thallium imaging in addition to electrocardiographic monitoring resulted in a higher degree of sensitivity than did ECG monitoring alone. Fourteen patients demonstrated evidence of coronary artery spasm as documented by 201Tl imaging but of the 14, significant ECG changes occurred in only 50%, and classic ST segment elevation in 21%. Thus, in carefully selected patients the noninvasive provocation of coronary spasm can be accomplished safely, but ECG monitoring must be combined with thallium-201 imaging to achieve an acceptable degree of sensitivity.
Recent investigations suggest that the vasoactive substances, histamine, prostaglandin E2, and thromboxane A2 are mediators of coronary artery spasm. These substances may be released during a episode of urticaria. We report here a case of coronary artery spasm associated with an episode of acute urticaria. The coronary spasm may have been mediated by the vasoactive substances released from basophils and secondary platelet aggregation, known to occur with urticaria.
Unexpected occurrence of coronary artery spasm is sometimes observed during cardiac catheterization. We report here two cases of coronary artery spasm with hypotension and urticaria subsequent to administration of contrast material. The etiology of coronary artery spasm is discussed.
Diagnosis of coronary microvascular spasm remains largely speculative because it has been mostly based on chest pain and electrocardiographic ST-segment shift with slow filling of contrast medium into the coronary artery. A patient with resting chest pain and normal coronary angiograms underwent provocative tests with intracoronary acetylcholine (ACh) and ergonovine. During the tests, coronary diameter and flow velocity in the left anterior descending (LAD) coronary artery were measured with quantitative coronary angiography and intracoronary Doppler guide wire, respectively. Vascular resistance of the LAD and lactate production were determined separately. With injections of 100 microg of ACh and 20 microg of ergonovine, chest pain occurred with ST-segment elevation in the precordial leads in the absence of epicardial coronary spasm. Coronary vascular resistance increased by 2.2- and 1.6-fold of the baseline value with ACh and ergonovine, respectively. Myocardial lactate production was noted during the ST-segment elevation. Coronary microvascular spasm was verified by the increment in coronary vascular resistance and myocardial lactate production with concomitant ST-segment elevation in the presence of normal coronary angiograms.
Pathogenesis of the so-called "heart attack" still remains to be elucidated. The links between stable effort angina and unstable or acute myocardial infarction, and between asymptomatic and spontaneous angina are all missing. In medicine presently, pathophysiology of ischemic heart disease is considered a consequence of i) the progression of atherosclerotic narrowing of the coronary artery, and ii) dynamic and transient obstruction (coronary spasm), but these mechanisms are traditionally believed to be unrelated. This article demonstrates various experimental evidence indicating that these two mechanisms are related. And, this review article describes how to produce experimental coronary spasm in the presence of atherosclerosis, similar to that seen in patients with variant angina, and that coronary spasm can produce sudden progression of coronary atherosclerotic obstruction due to intramural hemorrhage. Establishment of various animal models to elucidate mechanisms related to various stages of ischemic heart disease are needed.
An understanding of the hemodynamics of cerebrovascular spasm following subarachnoid hemorrhage is important for the diagnosis and treatment of this potentially dangerous condition. An overview model is presented which includes the main elements determining the overall effect of vasospasm. The model included realistic pressure-flow-velocity-diameter relationship encountered in a geometry resembling that of vasospasm of the middle cerebral artery. Viscosity was adjusted to that expected of human blood. Furthermore, a realistic model the cerebral autoregulation was included. The effects of induced hypertension as well as hypotension were studied. It was found that the friction pressure loss in the spastic segment was 3.5 times as high as that predicted by using the Hagen-Poiseuille formula. The reason for this discrepancy was probably the 'inlet length effect' considerably increasing the friction. Furthermore, including the Bernoulli kinetic pressure energy, a formula was proposed that accurately described the experimental data. From this hemodynamic perspective, strong support was found for the present trend to use aggressive hypertensive therapy in patients with vasospasm. The results also confirmed that TCD velocity measurements in the spastic segment when taken alone may not be a good index of the degree and effect of the spasm. These measurements must be combined with other techniques such as extracranial Doppler or CBF to assess the degree of spasm.
Three patients who, following partial brachial plexus avulsion, experienced pain, involuntary finger twitching and muscular spasms are reported. Two exhibited cutaneous trigger zones, stimulation of which exacerbated their pain; changes in emotional tone aggravated both the pain and the spasms. Pain would appear to be due not only to deafferentation and scarring of the dorsal horn, but also to an afferent pathological barrage from partially damaged dorsal roots. The pathological sensory barrage may activate metameric interneuronal circuits and produce involuntary movements. Exacerbation of both the pain and the spasms can be explained on the basis of intrinsic properties of these ectopic pacemakers.
Two cases of cystic brainstem schwannomas protruding into the fourth ventricle are described. Both patients presented with hemifacial spasm. While there is only one previous case report of an intraventricular brainstem schwannoma, there has been no prior description in the literature of hemifacial spasm associated with such a tumor. The clinical, radiographic, surgical, and histopathological features of these tumors are elaborated. The relationship of hemifacial spasm to the various putative theories of origin proposed for intraparenchymal schwannomas is discussed.
Intracranial pressure (ICP) was recorded continuously in 12 pre-operative patients with angiographic evidence of diffuse cerebral arterial spasm due to a ruptured intracranial aneurysm. Recordings were made for 1 to 7 days, starting within 13 days after the haemorrhage. 1. An increased ICP was observed in the first week after subarachnoid haemorrhage (SAH) in 4 of the patients without any signs of angiographic arterial spasm. 2. This initial increase was regularly followed by a depression of ICP in between 7--12 days after SAH. In 11 out of 12 patients such a depression was concomitant with the beginning of arterial spasm. During the period of depressed ICP pattern, 6 patients showed little or no neurological deterioration, whereas 5 patients showed impaired consciousness or neurological deficits. 3. A secondary rise of ICP thereafter always followed due to ischaemic brain swelling or infarction, and was usually associated with a serious neurological deterioration. 4. Continuous ventricular drainage was performed to control the secondary increased ICP in 7 patients who survived, 4 of them with good clinical improvement and 3 with severe neurological deficits. 5. In the stage of depressed ICP, the administration of isoproterenol and steroids is recommended in order to try to alleviate the secondary rise of ICP.
Specific antagonists to the influx of calcium, necessary for the excitation-contraction coupling process in arterial smooth muscle, are potentially useful in the treatment of cerebral vasospasm but systemic hypotension might limit their clinical applicability. We studied the effect of the calcium antagonist nimodipine (BAY e 9736) on cerebral arterial spasm, intraventricular pressure and blood pressure (BP), when administered into the cerebral ventricles of the dog. Cerebral vasospasm was produced by the injection of autologous blood into the cisterna magna. In a group of 8 dogs, 100 micrograms of nimodipine was injected into the lateral ventricle. The effect of the drug on the basilar artery was monitored angiographically. Nimodipine always relieved spasm, and often the relaxation surpassed the resting vessel diameter. In a control group, the injection of placebo did not relax the spastic arteries. Determinations using gas chromatography of nimodipine in CSF and blood demonstrated that a concentration of 1 microgram/ml in cisternal CSF was sufficient to reduce spasm while concomitant plasma concentrations of 0.004 micrograms/ml did not result in significant BP reduction.