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We encountered a case of intractable radial artery graft spasm after stent implantation which was partially responsive to nominal nitroglycerin therapy. We report this case with intravascular ultrasound imaging at the radial artery spasm site.
Under the hypothesis that the surgical management of diffuse esophageal spasm requires the elimination or reduction of episodes of dysphagia and chest pain and prevention of postoperative gastroesophageal reflux, long esophageal myotomy and fundoplication had been performed. However, there have been some cases with unsatisfactory results. We describe herein a new surgical procedure of long myotomy of the esophagus and gastric cardia with a complete fundic patch operation for the patient with diffuse esophageal spasm. The advantages of this procedure are to preserve the separation of each myotomized edge and to reinforce the wall of the surface of the myotomized mucosa in order to avoid the postoperative problems. Postoperative course of the patient with this procedure was satisfactory.
West syndrome, an epileptic encephalopathy of infancy and early childhood with variable etiology, consist of the triad of infantile spasms, arrest or regress of psychomotor development and specific EEG pattern of hypsarrhythmia. Infantile spasms occur within the first year of life. West syndrome has proved to be resistant to treatment, although VPA and benzodiazepines have been shown to be helpful. Then is general agreement that ACTH or oral corticosteroids remain the treatment of choice, especially in idiopathic cases, which have a better prognosis.
BACKGROUND: Microvascular Decompression (MVD) operation is the most reliable treatment for hemifacial spasm (HFS), but it causes many complications. The aim of this retrospective study was to investigate the factors relavent to the effects and postoperative complications of microvascular decompression on hemifacial spasm. METHODS: A total of 1200 HFS patients treated with MVD were studied retrospectively. The root exit zone (REZ) of the facial nerve was exposed through the infraflocculus approach, the offending vessels were identified and separated from the REZ, and a Teflon graft was interposed between the offending vessels and the brain stem. Brain stem auditory evoked potential (AEP) was monitored intraoperatively. RESULTS: The offending vessels can be identified in all patients. The anteroinferior cerebellar artery was the main offending vessel (42.6%). Patients with vertebral artery compression had a multiple vascular compression fashion. Follow-up for 2 - 10 years (mean 4.2 years) showed that 88.7% patients were cured and 5.6% relieved, with an effective rate of 94.3%. Recurrence rate was 3.2%, and the ineffective rate was 2.6%. The most frequent complication was hearing dysfunction (2.8%). CONCLUSIONS: MVD is the most definitive treatment method of HFS. The key procedures of this operation include adequate exposure of the REZ, identification of the offending vessels, and proper positioning of Teflon grafts. Complications can be reduced effectively by utilizing a real-time AEP monitoring during the operation.
The present study was carried out on the effect of botulinum A toxin, made in Japan, in the management of 30 hemifacial spasm patients. In almost all cases, alleviation of hemifacial spasm reached its peak in a few days. Availability was 93.3% and its effect lasted about 4 months. Side effects were observed in 12 patients (40%), but no serious complications were observed.
Somatosensory blink response (SBR) is produced by electrical stimulation of peripheral nerves or skin areas remote from the face. We investigated the presence of SBR in cases with hemifacial spasm (HFS) and peripheral facial palsy (PFP). Fifty-seven cases of HFS, 54 cases of PFP and 39 normal subjects were included in the study. A routine blink reflex study was performed in all subjects. Supramaximal stimulation of the median nerve was given ipsilateral to the either spasm or paralytic side for SBR. Recordings were made at the orbicularis oculi (o.oc) bilaterally and ipsilaterally at the orbicularis oris (o.or) muscles. SBR was elicited in 12 of 39 control subject. Twenty four of 48 HFS cases were SBR positive. Twelve of them had o.or response. An SBR was elicited at the ipsilateral o.oc in 35 of 46 patients with PFP with synkinesia. Twenty-six patients had an SBR at the ipsilateral o.or. In the 13 patients with PFP without synkinesia only 3 people had an SBR. SBR positivity was seen more often in PFP with synkinesia than in cases with HFS. SBR if positive spreads to the lower part of the face in most of cases with HFS and PFP with synkinesia.
This paper is a summary of our observations on 105 cases of infantile spasms. The age of onset was around six months after birth, but the patients came for treatment mainly about one year after onset. Fever of unknown cause, asphyxia, birth injury, infection of the central nervous system, tuberous sclerosis, phenylketonuria and recent immunization etc. were complained. Clinically, it is characterized by head nodding, mental retardation, myoclonic jerks and various neurologic deficits. EEG findings showed classical or modified arrythmia or other epileptiform patterns. About one third of 22 cases examined had abnormal brain stem auditory evoked potentials. Among 42 patients who underwent CT scanning before ACTH treatment, 18 were normal and 7 abnormal; during ACTH treatment 3 normal and 4 abnormal; after completion of treatment, 4 normal and 6 abnormal, suggesting no further atrophy of the brain. Examination of trace elements of the hair by particle-induced X-ray emission (PIXE) method in 23 patients revealed a significant difference in lead, calcium and zinc contents between patients and 101 controls, but no statistical difference in iron and copper contents between the two groups. Sodium valproate, prednisone and ACTH appear to be effective in the treatment of infantile spasms. Eight patients fully recovered, and they can go to school without difficulty. Many patients derived various degrees of improvement to the satisfaction of their parents. Two patients were still amented and often attacked by myoclonus. The effects, side effects of these drugs, and the possible pathogenesis were discussed.
We experienced two cases of myocarditis occurring during ACTH therapy for infantile spasms. Myocarditis cases occurred during the reduction of ACTH doses, and viral infection was suspected. Steroid replacement therapy was effective in the both cases. It is important to consider that ACTH therapy for infantile spasms could sometimes lead to severe myocarditis.
Infantile spasms (IS) are characterised by neurodevelopmental regression, a unique type of seizures and a hypsarrhythmic EEG pattern. Studies recommend the medical treatment of IS as a positive short-term outcome with respect to the spasms and in the resolution of the hypsarrhythmia. However, the data are insufficient to recommend medical treatment with respect to improving the long-term outcome. The adrenocorticotropic hormone and prednisolone are recommended for treatment of IS, although side effects are common. Vigabatrin is also recommended; IS caused by tuberous sclerosis seems to respond especially well.
In 1976, Jannetta et al, suggested that microvascular compression caused by arteries and veins crossing the root entry zone of the facial nerve, was the major etiology of hemifacial spasm (HFS). Surgical treatment has become a major trend. Microvascular decompression (MVD) with teflon patches has a high cure rate. However, the possible occurrence of multiple vessel compression makes it difficult to decide which of these vessels are responsible for the symptom during operation. Recently, a method of abnormal EMG was elaborated upon by Møller and Jannetta in 1987, to ensure the quality of MVD intraoperatively. In this study, we report 6 cases who underwent the procedure successfully. After general anesthesia and muscle relaxation, the abnormal EMG was not suppressed. When the offending vessel was lifted up from the facial nerve, the abnormal EMG disappeared immediately. In each case, postoperative improvement of facial spasm correlated with intraoperative abnormal EMG changes. This method is not only able to guide the surgeon to the vessel that induces HFS but also guarantees the quality of MVD.
The paper reports on a very important aspect in a pediatric practice, referring to one of the most severe forms of epilepsy of the infant, known in the modern specialty literature as infantile spasms. The paper reviews the etiologic data of the disease in its secondary form, with emphasis on the form of the disease better studied in the last years, i.e. the so-called infantile benign epileptic spasms. The paper also analyzes the clinical and electroencephalographic aspects, discuss the most important aspects of the differential diagnosis that includes some entities recently individualized and appreciates, on the basis of the most topical literature data, the nature of the different treatment means used, and of the evolution and prognosis.
Vascular compression of the facial nerve is a well recognized cause of hemifacial spasm (HFS). Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) provide vascular and brain tissue diagnosis in a single non-invasive examination and should be recommended as primary neuroradiological procedure in HFS. We report a rare case of symptomatic HFS caused by a vertebrobasilar dolichoectasia. A 49-year-old women experienced left hemifacial spasm for 10 months. MRI showed an enlarged vertebrobasilar dolichoectasia of the left vertebral artery which compressed the seventh cranial nerve at its exit from the caude pons. MRI is essential in establishing the cause of HFS. Together with MR angiography it shows the correlation among the seventh cranial nerve, blood vessels and the structures of mid-brain. Vertebrobasilar delichoestasia is just one of the blood vessel anomalies which causes HFS and which can be shown by MRI. HFS caused by vertebrobasilar dolichoectasia is quite rare.
Atypical measles is characterized by high fever, headache, myalgia and atypical rash, in patients who were vaccinated against measles. In this report a 22 years old male patient presenting with severe cervical and shoulder muscles spasms, purpuric and petechial lesions on palm, sole and whole body, has been presented. The patient had not an underlying disease or immunosuppression, and he had experienced single dose of measles vaccine when he was nine months old. Diagnosis of atypical measles was based on the clinical and serological findings (by measles IgM and IgG positivity). The aim of the presentation of this case was to emphasize that atypical measles should be considered for differential diagnosis in the cases complaining of muscle spasms and atypical skin rashes even if vaccinated against measles.
Electrically elicited blink reflex (BR) were analyzed in seven patients with age dependent epileptic encephalopathies (5 patients with infantile spasms and 2 with EIEE). Four patients with infantile spasms showed prolonged latency of the late BR responses (R2). In 2 patients with EIEE showing suppression burst pattern on EEG, R2 was not detectable. R2 abnormality in BR might reflect the dysfunction of the brainstem reticular formation in age dependent epileptic encephalopathies.
Ten facial nerve root specimens obtained from patients with hemifacial spasm were observed under the electronmicroscopy. The axons showed a particular degeneration. Axonal mitochondria and smooth endoplasm showed irregular vacuolization. In severe cases, the mitochondria and smooth endoplasm disappeared. The myelin sheath showed irregular vacuolization, replication, convolution and extensive segmental demyelination. Schwann cells were apparently in a state of degeneration. These changes were not seen in the normal subject. The causes and mechanism of the hemifacial spasm were discussed.
Movement disorders, or dyskinesias, in the facial region may be categorized in several ways. Dystonic movement disorders in the cranial-cervical region, including essential blepharospasm, Meige syndrome and spasmodic torticollis, are characterized by uncontrollable squeezing movements in the face and neck. These disorders typically present in the fifth and sixth decades of life. Essential blepharospasm is particularly debilitating, as the involuntary eyelid closure that accompanies this condition may result in functional blindness with an otherwise normal visual pathway. Hemifacial spasm is an intermittent, unilateral, spasmodic contraction of the muscles innervated by the facial nerve. This disorder usually presents in the third or fourth decade and has a different underlying pathophysiology than the dystonias. Botulinum A toxin therapy has largely supplanted surgical intervention in the treatment of essential blepharospasm and hemifacial spasm.
Botulinum A exotoxin was recently approved for use in Canada. We describe the efficacy of botulinum toxin in the management of 235 patients with blepharospasm (mean age 64.3 years) and 130 patients with hemifacial spasm (mean age 60.4 years) treated at three Canadian ophthalmologic centres between 1984 and 1989. A total of 98% of the patients with blepharospasm and 100% of the patients with hemifacial spasm had significant relief of their symptoms; however, 11% of the former and 2% of the latter did not respond to the usual starting concentrations of the drug and needed stronger dosages for relief. The duration of relief varied widely in both groups. Up to 7% of patients had ineffective treatments but responded to subsequent injections. Analysis of variance and linear trend statistics showed that there were no changes in the mean duration of relief over the first several treatments for individual patients in either group. Side effects were transient and included ptosis, exposure keratitis, epiphora and strabismus.
A prospective investigation of 52 children with infantile spasms was carried out employing computed tomography. Normal conditions were demonstrated in 21 (40%). Among the 31 children with pathological computed tomografic findings, dilatation of the ventricular system and/or subarachnoid space was found in nine cases (17%), congenital malformations in ten (19%) and cerebrovascular changes in a similar number (19%). One patient had a tumour and one had congenital toxoplasmosis. No significant differences were found in the frequencies of normal psychomotor development between children with normal and abnormal computed tomographic findings. The reason for this is probably that a good prognosis may be anticipated in the idiopathic forms provided that ACTH treatment is initiated early in the course of the disease. Computed tomography is useful to differentiate between symptomatic and idiopathic cases of infantile spasms. It may, however, be assumed that neuronal heterotopy without other malformations in the central nervous system may be difficult to diagnose with computed tomography. Magnetic resonance scanning will probably be able to demonstrate these cases.