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PubMed2000

Clinical and EEG video-polygraphic features of epileptic spasms in a child with dihydropteridine reductase deficiency. Efficiency of hydrocortisone.

West syndrome is an epileptic encephalopathy which includes psychomotor deterioration. In rare cases, it is due to an inherited, progressive metabolic disease. We report a 2 year-old child with dihydropteridine reductase deficiency who developed hypsarrhythmia and infantile spasms which were documented on video-polygraphic EEG. Despite dietary restriction of phenylalanine, and oral administration of amine precursors, the initial course was unfavorable. A beneficial effect from hydrocortisone was then observed, with control of spasms and improvement of psychomotor delay.

Anti-Inflammatory Agents↗
PubMed1998

[Blepharospasm and hemifacial spasm treated with botulinum A toxin injection].

OBJECTIVE: To study the efficacy of botulinum A toxin (BTA) injections for the treatment of blepharospasm and hemifacial spasm (HFS). METHODS: Twelve patients with blepharospasm and thirty eight patients with HFS were treated with BTA local injections (2.5 U to 5 U for each injection). RESULTS: Of the fifty patients, ten (86.7%) in blepharospasm and thirty two (84.2%) in HFS were completely relieved, two (13.3%) and five (13.2%) were remarkably relieved respectively, zero and one (2.6%) were partial relieved respectively. All patients experienced relief from spasm. The duration of the effect in blepharospasm were 10 to 24 weeks (mean, 18 weeks) and in HFS 14 to 32 weeks (mean, 22 weeks). The local side effects were transient and mild, including minor facial paralysis in eight patients, ptosis in four patients and tearing in four patients. No systemic adverse and allergic reactions were noted. CONCLUSION: Botulinum A toxin local injection is a safe, effective and simple means for the treatment of blepharospasm and HFS.

Adult↗
PubMed2001

[Microvascular decompression for hemifacial spasm: experience of 215 cases].

OBJECTIVE: To investigate the relationship between pre-operational etiological diagnosis of medically intractable hemifacial spasm (HFS) and patterns of offending vessels and the curative effect of microvascular decompression (MVD). METHODS: Two hundred and fifteen patients with medically intractable HFS underwent MVD under general anesthesia. Pre-operative MRI examination was made for all cases to exclude space occupying lesion and magnetic imaging tomographic angiography (MRTA) was conducted for 145 cases of them instead of routine MRI. RESULTS: Routine MRI failed to provide information about offending vessels except for ruling out mass lesions whereas MRTA revealed the offending vessels with a positive rate of 82.8%, thus helping make correct diagnosis and operation decision. Follow-up of more than two years showed that the surgical intervention achieved a high relief rate of 91.2% without major complication, and with a low recurrence rate of only 3.5% after averaging more than two years' follow-up. CONCLUSION: Pre-operative MRTA can replace conventional MRI as a best approach for pre-operational etiological diagnosis of facial spasm. It helps perceive the offending vessels before operation. Skilled microsurgical technique along with correct recognition and mobilization of offending vessels are a must to assure MVD a high efficacious and low risk treatment of choice for HFS patients.

Adult↗
PubMed1998

[Microvascular decompression of pons for idiopathic hemifacial spasm and exploration of its mechanism].

OBJECTIVE: To further explore the cause, mechanism and surgical treatment of idiopathic hemifacial spasm (IHFS). METHODS: Routine sagittal and coronal cryoslices of 8 (16 sides) pons were made. The sections were stained with Luxol fast blue and Nissl stains. The location of the facial nucleus and pathway of facial neurofibers in the pons were observed. Twenty-five patients with IHFS whose facial nerve root exit zone(REZ) was not compressed by vessels underwent microvascular decompression of pons. RESULTS: The distances from the facial nucleus to the ventral surface of pons, the median line, and the ventroexterior surface were 12.0-12.5 mm, 6.0 mm, and 7.5-9.0 mm respectively. The distance from the facial neurofibers in the pons to the surface of the pons was 1.0 to 2.0 mm, with an average of 1.65 mm. The vascular compression was at the surface of pons, within 4 mm anterosuperior to the REZ in 25 IHFS patients. The compressing vessels were the anterior inferior cerebellar arteries and their branches. The results of decompression in 25 patients with IHFS were as follows: spasm immediately disappeared in 8 patients, and within one week in 17 patients. No recurrence was found during a follow-up period from 6 months to 5 years. CONCLUSION: The main cause of IHFS was vascular compression, but the position of compression is not necessarily always at the REZ. The "short-circuiting" theory can only explain the cases with vascular compression at the REZ. But in cases who do not have vascular compression at the REZ, there might be dysfunction of the facial nucleus. In the present cases, vascular compression was at the surface of the brain stem anterosuperior to the REZ, pons microvascular decompression is an effective treatment.

Adult↗
PubMedDate not supplied

[Electroencephalographic discoveries in children with infantile massive spasms in Mexico].

INTRODUCTION: Infantile massive spasms (IMS) is an age dependent epileptic syndrome that appears before the first year, and is characterised by delayed psychomotor development, massive spasms and hypsarrhythmia. There are classifications that only take the electroencephalogram (EEG) into account, without linking it with the IMS associated crisis pattern which can establish a treatment to improve the recurrence of the crises in this population, according to EEG discoveries and patterns of epileptic fits. PATIENTS AND METHODS: We include 100 EEGs of patients diagnosed with IMS, between 2 and 12 months old, selected by using the classification of Hrachovy to identify classic and modified hypsarrhythmia, and to correlate it with the pattern of epileptic seizures associated with IMS. RESULTS: The hypsarrhythmia found was mainly of the modified variety, and classic hypsarrhythmia only accounted for 9% of cases. The first case presented flexion IMS with generalised tonic seizures, and in the second, generalised tonic seizures and mixed IMS. Start age was between 2 and 4 months old. CONCLUSIONS: In comparison with results in other publications, in the EEG it was found that modified hypsarrhythmia with IMS associated partial and generalised tonic seizures with was the most common, while in the classic variety mixed IMS predominated. It must be taken into account that the absence of this variety does not rule out a diagnosis of IMS, but its presence makes the prognosis worse.

Electroencephalography↗
PubMedDate not supplied

[Bilateral hemifacial spasm: eight personal case reports].

INTRODUCTION: Hemifacial spasms consist in tonic clonic, involuntary, asymmetrical and asynchronous contractions in the territory innerved by the facial nerve. Several different causes may give rise to this disorder, the most frequent of which are vascular abnormalities in the cerebellopontine angle. Its clinical features and electrophysiological studies are commonly used in diagnosis and its etiological diagnosis is most frequently performed by means of magnetic resonance imaging. Symptoms are treated using local injections of Botulinum toxin Type A in the affected muscles. AIMS: To review our experience in the handling of this pathological condition and to determine the results of employing Botulinum toxin. PATIENTS AND METHODS: We describe the cases of bilateral hemifacial spasms that have been diagnosed in the Virgen Macarena Hospital in Seville and La Fe in Valencia since 1980, as well as the follow up after treatment with Botulinum toxin. RESULTS: We describe eight cases of this pathological condition in which patients were treated with Botulinum toxin, and in all cases there was an improvement in the symptoms. CONCLUSIONS: Treatment with Botulinum toxin is considered to be satisfactory and provides a marked improvement in the patients quality of life.

Adult↗
PubMedDate not supplied

[Comparative analysis of the efficacy of some methods of conservative treatment of accomodation spasms and myopia in children].

A study was carried out of short- and long-term results in treatment of accommodation spasms and myopia of mild and moderate degrees in children with equipment having different operating mechanism. 171 patients aged 6-15 years were examined (30 persons made up a control group). Electrostimulation, laser stimulation and treatment on "Ambliokor" equipment were used. It was found out that these procedures give a good immediate effect in treatment of accommodation spasm and myopia but 5-6 months later the obtained results deteriorated in 2/3 of children and therefore it is recommended to repeat the treatment in the above period. Using "Ambliokor" equipment provides the best short-term effect. The highest effectiveness of treatment was obtained in combined method comprising all three procedures which made it possible to reduce the rate of myopia progression 2-fold and more compared with the similar index in the control group.

Accommodation, Ocular↗
PubMed2003

[Delayed facial palsy after microvascular decompression for hemifacial spasm due to reactivation of varicella-zoster virus].

We report a patient suffering from delayed facial palsy after microvascular decompression (MVD) for hemifacial spasm, in whom the pathogenesis was proved. A 56-year-old man with a left hemifacial spasm was admitted to our hospital. Preoperative MR imaging showed that the left anterior inferior cerebellar artery (AICA) was compressing the left facial nerve. The causative vessel was defined as AICA during surgery, and MVD was performed successfully. Seven days later, the patient showed severe left facial palsy. Serum antibody of varicella-zoster virus (VZV) was increased, and Gd enhanced MR imaging demonstrated an enhancement of a geniculate ganglion of the left facial nerve, indicating inflammation. These findings suggested that delayed facial palsy after MVD was caused by a re-activation of VZV. The facial palsy disappeared completely over a period of nine months.

Antibodies, Viral↗
PubMed1992

[Neurovascular decompression for hemifacial spasm].

Thirty-six cases of hemifacial spasm were treated by neurovascular decompression. Via transposterior cranial fossa approach, a small incision was made behind the ear. After careful dissection, a piece of teflon was placed between the vessel and initial segment of the facial nerve root and fixed with silver clip after wrapping of the nerve root. The cases were free from tic immediately after operation. Follow-up of 34 cases for 1 month to 3.5 years showed no recurrence and less and mild operative complications. In this series physiological function of the facial nerve was kept well and there was no mortality. This operation is regarded as the first choice in the treatment of hemifacial spasm.

Adult↗
PubMed1992

[Infantile spasms. A retrospective study of 105 cases].

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 possible etiology. Clinically, it is characterized by head nodding, mental retardation, myoclonic jerks and various neurologic deficits. EEG findings showed classical or modified arrhythmia 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 proton-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 of the satisfaction of their parents. Two patients were still amended and often attacked by myoclonus. The effects, side effects of these drugs, and the possible pathogenesis were discussed.

Adrenocorticotropic Hormone↗
PubMed1992

[Treatment of infantile spasms with long-term low dose ACTH].

We investigated the effect of long-term, low-dose ACTH in 13 patients (10 boys and 3 girls) with infantile spasms who were treated with low-dose ACTH (mean: 0.0081 mg/kg/day). Two patients (one boy and one girl) received this therapy twice because of relapse of tonic spasms. ACTH was injected intramuscularly every morning for 30 days, after which dosage was tapered. The mean observation period was 53.9 months. Complete cessation of seizures was attained in 13 of 15 treatment trials. In one trial, complete cessation was not attained but the number of attacks decreased to less than one-third of that before treatment. In only one trial was treatment not effective. EEG showed good response to this treatment. The side-effects of this therapy were hypertension in 6 patients, hypokalemia in 7, and emotional outburst in 7. Emotional outburst appeared during the early phase of therapy, while the other two side-effects appeared in the later phase and disappeared when ACTH-tapering was begun. Brain shrinkage observed on CT scan was mild in all trials. Five patients have had no relapse. The total dose of ACTH was significantly larger in the group with good outcome than in the group with poor outcome.

Adrenocorticotropic Hormone↗
PubMed1992

Two hundred and fifty patients with hemifacial spasm treated with botulinum toxin injection.

Two hundred and fifty patients with hemifacial spasm from the Movement Disorder Clinic, at Siriraj Hospital have been treated with botulinum toxin injection since January 1989 as a collaborating research project with Smith-Kettlewell Eye Research Institute in San Francisco. Each patient received 30 units in four injection sites over the hyperkinetic facial muscles. There were 169 female and 81 male patients, the sex ratio of female to male was 2.1:1. The mean age of all patients was 50.2 +/- 12.6 years with the range of 22 to 78 years. The majority of patients had been suffering for 3-10 years. The results of botulinum toxin injection were classified as excellent in 81.2 per cent, moderate improvement 10.0 per cent, mild improvement 6.8 per cent and no improvement or worse in 2.0 per cent. There were complications of mild transient facial weakness in 44 patients (17.6%) mild ptosis in 7 patients (2.8%) and excessive lacrimation in 1 patient (0.4%). The effect of botulinum toxin treatment lasted for 3-6 months duration. Botulinum toxin injection is a simple and effective out-patient treatment for patients with hemifacial spasm with no systemic side effects and minor transient local complications.

Adult↗
PubMed1992

[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.

Adult↗
PubMed1992

Contralateral injections of botulinum A toxin for the treatment of hemifacial spasm to achieve increased facial symmetry.

Six patients noted facial asymmetry after botulinum toxin injection for hemifacial spasm. Each patient was injected on the side contralateral to the spasms with 10 to 15 IU over the zygomatic major and minor muscles. Each patient noted improvement in facial symmetry in the resting position and dynamic facial movements. Five of the six patients desired this approach with subsequent injections. This injection method variation proved helpful in the managing of hemifacial weakness created by botulinum A toxin for this condition.

Adult↗
PubMedDate not supplied

[Hemifacial spasm as an early symptom of posterior fossa meningioma].

A rare case of a 47-year old female with hemifacial spasm of the one-year duration as the initial and only symptom of posterior fossa meningioma is reported. At first the patient did not consent to surgery and was unsuccessfully treated with anticonvulsants. A month prior to her admission she started to complain of headache and balance disorder, and developed sensory loss to light touch in II and III branches of the right V nerve. CT revealed a huge posterior fossa meningioma. After a total removal of meningioma, the patient's hemifacial spasm disappeared.

Cranial Fossa, Posterior↗
PubMed1992

The aid of computerized tomography in hemifacial spasm.

Although the physiopathological mechanisms causing hemifacial spasm are not clearly understood, it is generally accepted that the causative factor is compression of the facial nerve at its root exit zone. In this paper we present seven cases of hemifacial spasm which were studied by dynamic CT scanning in the preoperative period, and treated with microvascular decompression. We suggest that CT scanning has considerable diagnostic value in disclosing the asymmetrical vascular formation causing root exit zone compression and that microvascular decompression should be the treatment of choice in this condition.

Adult↗
PubMed2004

[Hemimasticatory muscle spasm: an electromyogram analysis].

OBJECTIVE: To investigate the usefulness of electromyogram (EMG) in the diagnosis of the patients with hemimasticatory spasm (HMS). METHODS: Four cases with HMS were reported. All the 4 patients were undertaken needle and surface electrode EMG examination. RESULTS: Needle electrode EMG of the 4 patients with HMS showed grouped potentials synchronously with the onset of the spasm, which indicated abnormal excitatory electrical activities of the trigeminal nerve resulting in involuntary masticatory muscle movements. CONCLUSION: It is very important to use EMG for the diagnosis of HMS.

Adult↗