Chronic electrical stimulation of the facial nerve causes signs of facial nucleus hyperactivity

The proximal segment of the facial nerve in rats was stimulated electrically daily for a duration of 2-10 min. After 4-8 weeks of such stimulation, 12 of 18 rats developed abnormal muscle responses that could be demonstrated by recording the electromyographic response from lower face muscles (the mentalis muscle) while the temporal branch of the facial nerve was being stimulated electrically. This abnormal electromyographic response consists of activity that appears in the latency range 6.5-15 ms. In addition, these chronically stimulated rats developed signs of facial synkinesis on the side that had been chronically stimulated. This could be demonstrated by recording electromyographic activity when the blink reflex was being elicited by electrical stimulation of the ophthalmic nerve. Rats in which electrodes had been implanted but which had not been stimulated did not develop any abnormal electromyographic activity. The abnormal electromyographic activity that could be recorded in rats that had been stimulated chronically could not be recorded 4-8 weeks after the stimulation had been terminated. We interpret these results to indicate that chronic electrical stimulation of the facial nerve can render the facial motonucleus hyperactive, and that the signs of this hyperactivity (abnormal muscle response and synkinesis) are similar to those typically seen in patients with hemifacial spasm. We thus presume that these results support the hypothesis that it is the irritation of the facial nerve from a compressing blood vessel that causes the facial nucleus to become hyperactive in patients with hemifacial spasm.     

Mucin-producing tumor of the pancreas: diagnostic value of diffusion-weighted echo-planar MR imaging

It is generally accepted that hemifacial spasm (HFS) and trigeminal neuralgia are caused by compression of the facial nerve (seventh cranial nerve) or the trigeminal nerve (fifth cranial nerve) at the nerve's root exit (or entry) zone (REZ); thus, neurosurgeons generally perform neurovascular decompression at the REZ. Neurosurgeons tend to ignore vascular compression at distal portions of the seventh cranial nerve, even when found incidentally while performing neurovascular decompression at the REZ of that nerve, because compression of distal portions of the seventh cranial nerve has not been regarded as a cause of HFS. Recently the authors treated seven cases of HFS in which compression of the distal portion of the seventh cranial nerve produced symptoms. The anterior inferior cerebellar artery (AICA) was the offending vessel in five of these cases. Great care must be taken not to stretch the internal auditory arteries during manipulation of the AICA because these small arteries are quite vulnerable to surgical manipulation and the patient may experience hearing loss postoperatively. It must be kept in mind that compression of distal portions of the seventh cranial nerve may be responsible for HFS in cases in which neurovascular compression at the REZ is not confirmed intraoperatively and in cases in which neurovascular decompression at the nerve's REZ does not cure HFS. Surgical procedures for decompression of the distal portion of the seventh cranial nerve as well as decompression at the REZ should be performed when a deep vascular groove is noticed at the distal site of compression of the nerve.     

Differentiating attention-deficit/hyperactivity disorder from pervasive developmental disorder not otherwise specified

To discriminate between the various compressing vessels of the facial nerves in patients with hemifacial spasm, pre-operative oblique sagittal gradient-echo MR imaging was performed. Forty-two patients underwent pre-operative MR imaging and microvascular decompression. The MR images were divided according to findings into three groups as follows: Group A, a thick and/or long high-intensity line along the root exit zone (REZ) of the facial nerve; Group B, a thin and/or short high-intensity line along the REZ; and Group C, an unreliable image around the REZ. Fifteen images were classified as Group A, 19 as Group B, and 8 as Group C. In Group A, vertebral artery (VA) compression was confirmed intra-operatively in 12 cases and posterior inferior cerebellar artery (PICA) or anterior inferior cerebellar artery (AICA) compression in 3. In Group B, PICA or AICA compression was confirmed intra-operatively in all cases. In Group C, PICA or AICA compression was confirmed intra-operatively in 7 cases and no compression in one. In all cases of VA compression of the facial nerve, the oblique sagittal gradient-echo images demonstrated a thick and/or long high intensity line along the REZ. Oblique sagittal gradient-echo MR imaging is a useful preoperative planning aid, which can predict the possibility of VA compression prior to microvascular decompression for hemifacial spasm.     

Medical imaging research on idiopathic hemifacial spasm

In order to have a medical imaging examination for idiopathic hemifacial spasm before surgery, we designed a new method by using vertebroarterial DSA and vertebroarterial CTA based on an aired cisternapontis, 36 patients were examined since 1989 and the compression of the facial nerve from brain stem to porus acousticus internus was showed clearly in all the cases. The arteries responsible for the compression were cerebellar inferior posterior (55.6%), cerebellar inferior anterior (44.4%), auditory internus (25.0%) and vertebroarterial (11.1%), 36% of all the cases had more than one responsible arteries, 22.2% cases had only one compression point, 38.9% had two points, 25.0% had 3 and 13.9% had more. 85.7% of the compression points located in the root zone and 14.3% near the porus acousticus internus. 24 cases were treated with decompression surgery, it indicated that the compression conditions just met the medical imaging examinationg results. After surgery the symptom disappeared in 23 cases and remarkably released in one case with no death case. We believed that this method might be helpful to idiopathic hemifacial spasm decompression surgery and other surgeries in the CP angle.     

Hemifacial spasm in childhood and adolescence

Hemifacial spasm (HFS), a hyperactive dysfunction of the facial nerve, is rarely seen in young people. Between 1984 and 1994, we treated 924 patients with HFS by microvascular decompression at our institution. Of these, 8 (0.9%) were younger than 30 years. In most of the older patients with HFS, the offending artery which compresses the root exit zone was elongated, redundant, and focally arteriosclerotic as a result of hemodynamic effects due to aging or hypertension. On the other hand, the offending artery did not exhibit such characteristic changes of the vasculature in children and adolescents with HFS. In all of the young patients who underwent initial microvascular decompression at our clinic, the arachnoid membrane around the facial nerve was thickened and encased the artery, resulting in compression of the root exit zone of the facial nerve. Such thickening of the arachnoid surrounding the offending vessel may play an important role in the pathogenesis of HFS by trapping and encasing the artery to compress the root exit zone, particularly in the young patients.     

Hemifacial spasm caused by a hemangioma at the geniculate ganglion: case report

OBJECTIVE AND IMPORTANCE: Hemifacial spasm is rarely caused by facial nerve lesions in the temporal bone. Intratemporal facial nerve hemangiomas may initially present as facial spasm. CLINICAL PRESENTATION: A 30-year-old woman developed right hemifacial spasm. Physicians observed slight weakness on the right side of her face, in addition to the hemifacial spasm, but routine radiological examinations did not detect any abnormal findings along the course of the facial nerve. Although the patient underwent neurovascular decompression, the spasm persisted postoperatively. Two years after surgery, the right facial palsy progressed. Concurrently, the hemifacial spasm diminished. High-resolution computed tomography demonstrated a small mass lesion expanding the cortex of the right petrosal bone involving the geniculate ganglion of the facial nerve. INTERVENTION: The patient underwent a second craniotomy through a subtemporal extradural route, and the tumor was completely removed. A pathological examination demonstrated a cavernous hemangioma. CONCLUSION: Routine radiological examinations may fail to detect small intratemporal facial nerve hemangiomas, particularly at the geniculate ganglion. Therefore, when physicians encounter atypical facial spasm, the intratemporal portion of the facial nerve should be carefully examined using high-resolution computed tomography.     

Microvascular decompression in glossopharyngeal neuralgia

Glossopharyngeal neuralgia is a rare and often controversial cause for odynophagia and otalgia. The otolaryngologist, head and neck surgeon may be the primary physician called upon to diagnose and treat this entity. In this study, vascular decompression, or more specifically, elimination of contact between the ninth cranial nerve and the posterior inferior cerebellar artery, was employed as treatment in three patients. All achieved relief of their symptoms with this intervention. A review of the neurosurgical literature and the experience with vascular decompression in trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia is presented. The authors conclude that vascular decompression is effective in carefully selected patients, and the role of the skull base surgeon in managing this problem is expanding.     

The treatment of hemifacial spasm using botulinum toxin]

Hemifacial spasm is a disagreeable disturbance with involuntary unilateral twitching of the facial muscles. Its most common form is supposedly due to an irritation of the facial nerve at its proximal intracranial segment by vascular structures. Various forms of treatment including surgical procedures are employed, very often without satisfactory results but frequently involving the risk for severe complications. For a few years a new method has been using injection of botulinum toxin into the affected muscles, which in the majority of patients relieves the abnormal movements for about half a year; therefore, this very effective and secure procedure is recommended as first-line treatment of hemifacial spasm.     

Thermodilution cardiac output monitor

The authors describe the case of a 53-year-old woman who suffered from a hemifacial spasm caused by a venous angioma in the posterior fossa. At operation the parenchymal segment of the angioma was preserved and vascular decompression was performed by placing pieces of shredded Teflon between the vessel and nerve. After decompression the patient was completely free from spasm.     

Delineation of small nerves and blood vessels with three-dimensional fast spin-echo MR imaging: comparison of presurgical and surgical findings in patients with hemifacial spasm

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.     

Effect of repetitive stimulation on lateral spreads and F-waves in hemifacial spasm

The lateral spread (LS) response, which can be elicited in muscles innervated by other branches of the facial nerve, is electromyographycally specific for patients with hemifacial spasm (HFS), occurring about 10 ms after stimulus. The F-wave in facial muscles, which is a late response that antidromicaly propagates to the facial motonucleus and returns orthodromicaly down the same axon, revealed a trend toward enhancement in patients with HFS. The LSs were facilitated by repetitive stimulation during the microvascular decompression (MVD) operation, which has proved to be a successful treatment, and the F-waves were also facilitated by repetitive stimulation on the spasm side more than on the normal side. Greater facilitation of these responses was in direct proportion to higher stimulation rates and greater numbers of stimulations. The repetitive stimulation of the facial nerve may result in activation of the motoneuron pool and in the lowering of the threshold of somatic membranes. These results support the hypothesis that hemifacial spasm is caused by hyperexcitability of the facial motonucleus, which is increased by antidromic repetitive stimulation.     

Posterior inferior cerebellar artery aneurysm in the fourth ventricle

We report a patient with a ruptured aneurysm of the choroidal branch of the right posterior inferior cerebellar artery (PICA), lying in and causing an isolated haemorrhage in the fourth ventricle. MRI on the first day after bleeding revealed an abnormal vessel in the fourth ventricle, which was surrounded by a mass of intermediate signal on T1- and T2-weighted images. The aneurysm was clipped via partial splitting of the lower vermis and opening the inferior medullary velum. A postoperative angiogram confirmed complete obliteration of the aneurysm. With PICA aneurysms the rate of intraventricular haemorrhage is high and in most cases due to reflux of blood. If there is an isolated intraventricular haemorrhage, a peripheral PICA aneurysm, lying in or near the fourth ventricle, may be suspected.     

Disappearance of the co-existing aneurysm and arteriovenous malformation after wrapping of aneurysm (author's transl)

The authors reported a case of an arteriovenous malformation associated with an aneurysm on its feeding vessel that is the left anterior inferior cerebellar artery. A housewife, aged 56-years, was admitted to our hospital with severe headache, nausea and vomiting. On admission, lumbar puncture revealed grossly bloody CSF. Neurological examination revealed meningeal irritation sign, horizontal nystgmus and disturbance of left auditory acuity. Bilateral carotid and retrograde brachial arteryography revealed an AVM near the left cerebellopontine angle and a small aneurysm of the left anterior inferior cerebellar artery at the left internal auditory meatus. At operation, the aneurysm was wrapped with a muscle piece and no surgical intervention for AVM. Post-operative course was uneventful except for disterbance of the left auditory acuity. Follow-up angiographies revealed a change of size the aneurysm and AVM and finaly failed to demonstrate the aneurysm and AVM demonstrated preoperatively. The spontaneous regression of the AVM might be due to the post-operative brain swelling and adhesion. We also speculated that the spontaneous disappearance of the aneurysm might be due to the decreased blood flow of parent artery by the spontaneous regression of the AVM. It is very rare that an aneurysm of anterior inferior cerebellar artery co-existed with AVM, and resulted in thrombosis of the aneurysm and regression of the AVM after wrapping alone.     

Computerized nuclear morphometry of hepatocellular carcinoma and its relation to proliferative activity

Acute infarcts of the anterior inferior cerebellar artery (AICA) are unusual. We report 15 cases of AICA infarcts and their correlation with the topography of the lesion by brain MRI. During 2 years we prospectively identified 7 cases of AICA infarcts among 770 acute strokes (0.9% of the acute strokes seen in our department). We studied these cases and also another 8 that we found retrospectively. Most patients (8/15) had a unilateral affectation of both middle cerebellar peduncle (MCP) and inferior lateral pontine area (ILP), in these cases the main symptoms were vertigo, ataxia, peripheral facial palsy and hypoacusia. Two other patients had isolated MCP infarcts and were characterized by peripheral vertigo and ataxia, without hypoacusia or facial palsy. Another 2 patients had isolated ILP territory infarct characterized by vertigo, left peripheral facial palsy without hypoacusia and mild or no ataxia. One patient had a Gasperini syndrome. Finally 3 patients had bilateral AICA infarcts due to basilar thrombosis. The etiology was atherosclerosis in 9 patients, lacunar due to hypertension in 1, cardiac embolism in 1, migraine in 1 and unknown in 3. Among the 15 patients only 2 died, both with AICA plus infarcts. In the remaining patients a follow-up during a mean of 31 months (3 months to 12 years) showed no recurrences.     

Pediatric hemifacial spasm: the efficacy of microvascular decompression

Hemifacial spasm (HFS), generally a disease of the elderly, is caused by vascular compression of the seventh nerve. Vascular compression is thought to result from atherosclerotic changes within the vessels of the posterior fossa, and therefore rarely presents in childhood. Here we describe our experience with 12 patients with onset of HFS during childhood (age 18 or less) and who had surgical exploration of the cerebellopontine angle. These patients represent less than 1.2% of the patient population with HFS operated upon at this institution during the study period. Nine patients had follow-up data extending over 83 months. All 12 patients were found to have microvascular compression of the seventh nerve at the time of surgery. The most common operative finding was compression of the seventh nerve by a vein, alone or in combination with a branch of the anterior inferior cerebellar artery. At the time of discharge and after a mean follow-up period of 125 months, microvascular decompression resulted in complete relief of spasm in 67% of the patients.     

Mechanism of action, clinical indication and results of treatment of botulinum toxin

Botulinum toxin, the most potent of the neurotoxins, produces paralysis by blocking presynaptic release of the neurotransmitter (acetylcholine) at the neuromuscular junction, with reversible chemical denervation of the muscle fibre, thereby inducing partial paralysis and atrophy. Because chemical denervation is reversible, botulinum toxin has temporary effects, the muscle being progressively reinnervated by nerve sproutings. Type A botulinum toxin (Bix-A) is available under two dosage forms: Botox and Dysport. Although the initial clinical indication was strabismus, subsequent studies have demonstrated the efficacy of Btx-A, mainly in dystonia, hemifacial spasm and spasticity. However, botulinum toxin has been successfully used in various other clinical indications. In regard to spasticity associated with cerebral palsy, Btx-A is a promising treatment requiring a multidisciplinary approach. Btx-A injections lead to effective reduction of muscle hyperactivity with minor side-effects. They are painless, even though electromyographic guidance may be required for the injection of deep muscles. However, the production of antibodies to Btx-A may compromise the effect of long-term treatment.     

Seminal vesicle cysts in the patients with adult polycystic kidney disease

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.     

Ruptured de novo aneurysm induced by ethyl 2-cyanoacrylate: case report

OBJECTIVE AND IMPORTANCE: We report a rare case of a ruptured de novo aneurysm induced by ethyl 2-cyanoacrylate. CLINICAL PRESENTATION: A 44-year-old woman had undergone microvascular decompression for a right-sided facial spasm. The preoperative vertebral angiogram did not show any aneurysmal dilation. The right anteroinferior cerebellar artery, which was compressing the exit zone of the facial nerve, was detached and fixed to the dura mater with ethyl 2-cyanoacrylate. Nine years later, the patient suffered a subarachnoid hemorrhage caused by the rupture of a newly developed aneurysm of the right anteroinferior cerebellar artery. INTERVENTION: The aneurysm was clipped 2 days after onset of the subarachnoid hemorrhage. It consisted of two bulges in the arterial wall on the proximal side of the meatal loop. One bulge was stuck to the dura mater of the pyramis by ethyl 2-cyanoacrylate, which had been used in the microvascular decompression 9 years previously. CONCLUSION: This is the first reported clinical case of a de novo aneurysm induced by a cyanoacrylate adhesive. Ethyl 2-cyanoacrylate can damage the arterial wall and induce a de novo aneurysm.     

Vascular compression and cranial nerve diseases

This paper offers a review of cranial nerve rhizopathies caused by vascular compression of cranial nerves in the posterior cranial fossa. We present our results of microvascular decompression for trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia caused by compression of the 5th, 7th and 9th cranial nerves, respectively. After a median observation time of 38 months, 20 of 25 patients with trigeminal neuralgia were completely free of pain, and one patient reported more than 50% pain relief. Four out of five patients treated for hemifacial spasms were completely free of spasms. Of two patients treated for glossopharyngeal neuralgia, one reported complete pain relief, whereas the other reported less than 50% pain relief. No serious complications occurred. The results of microvascular decompression reported in the literature reviewed, including results of the treatment of tinnitus and positional vertigo due to compression of the 8th cranial nerve, hypertension due to compression of the 10th cranial nerve and spastic torticollis due to compression of the 11th cranial nerve. It is concluded that the rationale behind microvascular decompression is supported by an extensive amount of data.     

Neuronal nicotinic receptor operates slow Ca2+ mobilization at mouse muscle endplate

The authors describe the anatomical characteristics of the levator labii superioris muscle by dissection in cadavers. PURPOSE: We describe the characteristics of these muscle, the details and relations, hopefully contributing to the study of muscle of the face. METHODS: Twenty faces of cadavers were dissected. The following features were studied: origin, insertion, length, width, thickness, relations, innervation and blood supply. RESULTS: In all cases the muscle originated from the inferior orbital margin. Two insertions were observed: via lateral fibers, superficial to the orbicularis oris muscle and via deep fibers than form part of the raphe at the corner of the mouth (70%); via superficial fibers to the orbicularis oris muscle (30%). The average of the length was 24.66 mm and the average of the thickness was 3.57mm. The width at its insertion was 11.2mm, and at the origin was 15.96mm. The levator labii superioris muscle was found to be anterior to the levator anguli oris; it was posterior to the distal portion of the zygomaticus minor (90%) and posterior to the mid portion of the zygomaticus minor (10%). The innervation was from the inferior branch of the zygomatic nerve (facial nerve) and from the infraorbital nerve (trigeminal nerve). The inferior portion of the muscle is supplied by branches of the angular artery and the superior part from branches of the infraorbital artery.