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PubMed2000

Cortical visual impairment in children with infantile spasms.

BACKGROUND: Infantile spasms are often associated with cortical visual impairment. METHOD: We report a series of 10 patients with infantile spasms, hypsarrhythmic electroencephalograms, and developmental delay, (ie, West syndrome), who presented with severe visual inattention despite a normal ocular examination. RESULTS: At follow-up (14 months to 6 years), 5 patients (50%) had no improvement in their visual behaviour. Although some degree of improvement was observed in the others, their visual function remained abnormal. All patients had moderate or severe mental retardation. CONCLUSIONS: The clinical features of West syndrome are reviewed, and the cause of the associated cortical visual impairment is discussed. Cortical visual impairment with infantile spasms is an important association, which pediatric ophthalmologists should recognize.

Diagnosis, Differential↗
PubMed2000

[TENS in the treatment of muscle spasm].

This study deals with 60 patients with muscle spasm after the lesion of upper motor neurone. Thirty patients were treated by battery operated TENS unit, which produce biphasic impulses, with possibility of individual determination of impulse duration (0.05-0.25 msec), frequency (2-100 Hz) and intensity (0-80 mA). The electrodes were put on the motor points of m. tibialis anterior and m. extensor digitorum. The parameters of stimulation where determined individually, using the impulses that elicit dorsal flexion of the ankle joint and fingers. In the control group, consisted of 30 patients, passive exercises were performed (increasing range of motion, stretching of the agonistic and antagonistic muscles). At the end of the research, the authors report statistically significant decrease of muscles spasm in patients treated by TENS (p < 0.05), as well as improvement of the passive range of motion in ankle joint in both groups. Reducing of the muscle spasm is more pronounced if longer period of stimulation is applied.

Adult↗
PubMed2001

[A case of convergence spasm in hysteria improved with a brief psychiatric assessment].

We report a 16-year-old female who developed double vision. The diplopia was in the horizontal plane and persisted for 3 weeks. She also complained of headache and nausea. She kept her eyes closed unless she was told to open. When eyelids were passively open, both eyes deviated inward in an adducted position. The pupil size and the reaction to light were normal. No weakness was noted in the extraocular muscles when each eye was examined individually. The rest of the neurologic examinations were normal. We thought that she had a convergence spasm. Brain CT and MRI were normal. The CSF and blood chemistries were also normal. We treated her with supportive psychotherapy and her convergence spasm disappeared. We concluded that her convergence spasm was a manifestation of conversion hysteria.

Adolescent↗
PubMed2002

Vigabatrin therapy in infantile spasms.

RATIONALE: To determine the clinical outcome and side effects of vigabatrin (VGB) in the treatment of infantile spasms (IS) and its long-term outcome. METHOD: All children with IS treated with vigabatrin were studied. Clinical data regarding age of onset, duration of IS before therapy started, recurrence of IS, types of seizures that relapse, clinical outcome and side effects were monitored. RESULTS: 36 children (17 girls, 19 boys) with IS participated in the study. The mean age of onset of IS was 115.55 +/- 67.3 days old (range, 15 to 300 days). Six were cryptogenic IS and 30 were symptomatic IS. The etiologies of symptomatic IS in this study were tuberous sclerosis, hypoxic ischemic encephalopathy (HIE)/periventricular leukomalacia, porencephaly, partial agenesis of corpus callosum, hemimegalencephaly, cortical dysplasia, and microcephaly. 66.67 per cent (24 of 36) of patients responded to VGB within a mean 2.95 +/- 2.25 days (range, 1 to 7 days). In those who responded to VGB, 3 patients developed recurrent IS within 69.3 +/- 46.7 days (range, 30 to 121 days). Five patients developed epilepsy with different types of seizure during long-term follow-up. The mean duration of subsequent epilepsy after cessation of IS was 16.4 months (range, 5 months to 3 years 10 months). The mean duration of follow-up was 2.74 years (range, 1.09 years to 5.76 years). 10 patients were successfully weaned off VGB after a mean IS free period of 22.5 +/- 5.5 months (range, 12 to 27 months). Transient drowsiness was seen in 4 patients. Three patients had transient abnormal sleep patterns and irritability. Visual field abnormalities were not found but difficult to assess fully in this study. CONCLUSION: VGB therapy has a high response rate for the control of IS and is well tolerated in most children. All patients who responded to VGB and were spasm free for more than one year were successfully weaned off VGB therapy. Because serious side effects such as visual field abnormalities are difficult to monitor, the authors propose that VGB could be withdrawn or switched to another AED after a spasm-free period of more than one year.

Anticonvulsants↗
PubMed2002

[Trigeminal neuralgia and hemifacial spasm. Vessel-nerve antagonism].

In the last 30 years, the concept of microvascular compression has achieved widespread acceptance. Still, some remain unconvinced. A review of our 182 operations, 104 trigeminal neuralgia and 78 hemifacial spasms, shows that microvascular decompression of the trigeminal and facial nerves is effective in alleviating trigeminal neuralgia and hemifacial spasm, because it removes the actual cause of the disorder rather than simply causing local injury to the nerve. Our neurophysiological investigations, the so-called abnormal muscle response, performed intraoperatively in twenty patients with hemifacial spasm, confirm that compression of the facial nerve at the root exit zone, by a blood vessel, causes pathological and clinical responses. Microvascular decompression is an extremely safe and effective treatment, even for the older patients without severe disabilities.

Adult↗
PubMed2003

Electrical stimulation in the treatment of pelvic pain due to levator ani spasm.

OBJECTIVE: To evaluate experience with intravaginal electrical stimulation for the relief of pain when used as adjunctive therapy in women with chronic pelvic pain and levator ani spasm. STUDY DESIGN: A retrospective cohort of consecutively treated patients from 1999 and 2000 was identified using billing records. Systematic chart review was completed using standardized data collection forms for all patients receiving electrical stimulation for pain from levator ani spasm. Data collected were objective for major variables and subjective for outcomes. Demographic data were reported as means and standard deviations. Stimulation characteristics were compared using ANOVA. Survival analysis was performed using life table methods. RESULTS: Medical records from 66 consecutive patients treated during an 18-month interval were reviewed. Demographic characteristics included mean age of 38.7 years, 13 years of education and parity of 2. Married women composed 75% of the study group, with 81% white, 10% Hispanic and 9% black. Of the 66 patients studied, 50 had follow-up documentation with an average duration of 14.5 weeks. Overall, 34 patients (52%) demonstrated improvement in pelvic pain following vaginal electrical stimulation. Using survival analysis, 51% of patients had persistent improvement 30 weeks after treatment. There were no differences in age, race, education or parity between patients reporting a sustained benefit of stimulation and those not reporting a benefit. CONCLUSION: Vaginal electrical stimulation may help a selected population of women with pelvic pain due to levator ani spasm.

Adolescent↗
PubMed1992

[Hemifacial spasm and basilar impression associated with Arnold-Chiari deformity. Report of a case].

The authors report a case of symptomatic basilar impression and Arnold-Chiari malformation being presented as the first symptom of hemifacial spasm. The surgical treatment of the malformation resulted in improvement of the clinical manifestation with reduction of the hemifacial spasm. The need for the aetiological therapy for the hemifacial spasm is emphasized, before symptomatic treatment with botulinum toxin is tried.

Arnold-Chiari Malformation↗
PubMed1992

Physiologic assessment and surgical management of diffuse esophageal spasm.

The physiologic abnormalities and management of patients with diffuse esophageal spasm are controversial. We evaluated the symptomatic and functional results of surgical therapy in 19 patients with diffuse esophageal spasm who were incapacitated with dysphagia and chest pain and unresponsive to conservative management. A long esophageal myotomy with an antireflux procedure was performed in 15 patients, and four patients with multiple previous esophageal procedures had an esophagectomy. Eleven patients had increased esophageal exposure to gastric juice on preoperative 24-hour esophageal pH monitoring. The severity of dysphagia, chest pain, regurgitation, and heartburn was scored on a scale of 0 to 3 before and a mean of 24 months (range 8 months to 13 years) after the operation. After myotomy, each of these symptoms and the overall symptom score improved significantly (p < 0.01). The improvement in the symptom scores in the patients who had esophagectomy were comparable with the improvement after myotomy. On self-assessment, 90% of the patients would have the operation again if again faced with the decision. Standard and ambulatory 24-hour manometry showed a significant reduction in the amplitude of the esophageal body contractions, a decrease in the frequency of simultaneous contractions, and the elimination of multi-peaked waves after the myotomy. Despite the addition of an antireflux procedure, lower esophageal sphincter pressure, overall length, and abdominal length were reduced markedly after the myotomy. This was associated with persistent or emerging heartburn or regurgitation in four patients. These data indicate that a long esophageal myotomy is a valid treatment alternative in appropriately selected patients with diffuse esophageal spasm. Esophagectomy and colon interposition is the procedure of choice in patients with multiple previously failed myotomies.

Esophageal Spasm, Diffuse↗
PubMed1992

[Benign familial neonatal convulsions in a family with one member with infantile spasms].

A pedigree of benign familial neonatal convulsions (BFNC) was reported. Seven members of two generations experienced convulsions in the neonatal period and/or in early infancy. All of these members except one had a good prognosis. One member who had infantile spasms was uneventfully delivered at 37 weeks of gestation, with a birth weight of 2,562 g and, without trouble during pregnancy. At the age of 20 days, she began to have adversive seizures. Later, she developed complex partial seizures and infantile spasms at 1 month and 10 days of age. Interictal EEG showed hypsarrhythmia. Biochemical investigations and MRI of the head revealed no abnormality. Treatment with sodium valproate and carbamazepine succeeded in stopping the seizures and she had no seizures after 3 months. But her psychomotor development was moderately delayed at 8 months. No case with severe epilepsy such as infantile spasms has been reported in the previous literature on BFNC. From our experience, early treatment and careful follow-up are considered to be important for BFNC.

Child↗
PubMed1992

Treatment of blepharospasm, hemifacial spasm and strabismus with botulinum a toxin.

Thirty patients with blepharospasm, hemifacial spasm, strabismus and entropion were treated with botulinum A toxin giving satisfactory results. Rapid spasm relief, correction of strabismus and entropion were obtained. Only mild, transient and local side-effects occurred. The patients were followed up for 4-12 weeks with no recurrence. The clinical results show that local injection of a minute dose of botulinum A toxin in treating blepharospasm, hemifacial spasm, strabismus and entropion is a safe, effective and simple method of nonsurgical therapy.

Adult↗
PubMed2003

[Preoperative etiological diagnosis of hemifacial spasm: a comparative study between magnetic resonance tomographic angiography and intra-operative findings].

OBJECTIVE: To summarize retrospectively the value of magnetic resonance tomographic angiography (MRTA) in the preoperative etiological diagnosis for hemifacial spasms (HFS). METHODS: 336 cases of HFS, 127 male and 209 female, aged 46.2 (16-75), with a course of 1-29 years (6.2 years on average) and with the unilateral symptoms except one case were subjected to routine MRTA check up preoperatively. The MRTA findings were compared with the operative findings. RESULTS: Pre-operative evaluation by MRTA discerned 273 offending vessels with a positive rate of 81.3%, which were completely consistent with the intra-operative findings. The false positive and false negative rates were 8.0% and 9.8% respectively. Three cases of HFS due to secondary cause were found: one of CPA meningioma at the cerebellopontine angle and two cases of cholesteatoma. CONCLUSION: MRTA imaging provides more useful information concerning the etiology of hemifacial spasm than any other available diagnostic modalities. It is an effective and reliable means for pre-operative evaluation of the existence and sources of offending vessels in hemifacial spasm patients, and thus benefits the planning and guiding of intra-operative manipulation.

Adolescent↗
PubMed2004

[A case of intractable epilepsy presenting epileptic spasm treated with callosotomy in childhood].

We present a case of intractable epilepsy presenting epileptic spasm successfully treated with anterior callosotomy in childhood. A 12-year-old boy had seizures frequently since the age of 11 months which were diagnosed as an infantile spasm. He underwent various treatments including ACTH therapy, but the seizures were intractable. Characteristics of his seizures were an abrupt axial movement leading to sustained posturing, most often truncal, and neck flexion with pronounced arm extension and abduction. He lost consciousness transiently and these seizures frequently occurred in clusters and the duration of seizure was 1 to 3 seconds. Neurologically he had no deficit, but his intelligence was below the standard level. Magnetic resonance image (MRI) showed no abnormalities in the brain. Electroencephalogram (EEG) demonstrated secondary bilateral synchrony (SBS) with left frontal dominance in amplitude. Subdural strip electrodes were implanted bilaterally over the frontal lobes to detect an epileptogenic region. However intracranial ictal-EEG demonstrated an abrupt bilateral desynchronization for 1-3 seconds. As the epileptogenic zone was not clearly identified and an abrupt bilateral desynchronization was identified, anterior callosotomy was performed. Since surgery, he has remained seizure-free for two years while taking anticonvulsants and the SBS has disappeared. This case may indicate that the corpus callosum influences the occurrence of epileptic spasm and SBS on EEG.

Child↗
PubMed2004

Successful treatment of hemifacial spasm with selective facial nerve block using doxorubicin (adriamycin) under local anesthesia.

OBJECTIVE: To describe our experience with selective chemical rhizotomy of facial nerves using adriamycin (ADM) in a patient with hemifacial spasm (HFS). This unique technique is less invasive than intracranial neurosurgery and enables one to perform a permanent nerve block under local anesthesia. PATIENT: The patient, a 73-year-old female, had difficulty opening her left eye. Following unsuccessful treatment with anti-epileptic medicine, she received selective intraneural injections of ADM under local anesthesia. One week after the surgery the spasms had disappeared completely. No major complications were caused by this procedure and there had been no recurrence of spasms 3 years after the surgery. RESULTS: It is thought that recurrence of HFS should be observed after simple neurotomy due to regrowth of nerve fibers. However, this did not occur after chemical rhizotomy with ADM. This method clearly differs from previously used varieties of simple neurotomy because the latter technique does not cause severe destructive changes in the facial motor nucleus. CONCLUSION: Selective facial nerve chemical rhizotomy with ADM under local anesthesia may be effective in treating a subgroup of patients with HFS, especially elderly patients and those in the high-risk group for general anesthesia and intracranial neurosurgery.

Aged↗
PubMed2004

[The pathogeny and treatment of hemifacial spasm].

OBJECTIVE: To find out the etiopathogenesis of hemifacial spasm, and to research the principles and methods of surgical treatment. METHOD: The micro-surgical relationships between facial nerve and adjacent vessels were examined in 30 adults cadavers. One hundred and twenty cases of hemifacial spasm were treated through posterior fossa microsurgery, twenty of which received microvascular decompression, sixty-eight by combing of facial nerve, and thirty-two by both microvascular decompression and combing. RESULT: Forty-one out of the 60 facial nerves in 30 adults cadavers were observed to have been in touch with arteries for 42 times,accounting for 68.3% of the cases. In 120 operations, 56 facial nerves were found to be compressed or distorted by the arteries. One hundred and twenty cases were followed up for 3 years. Five recurrent cases were found in 20 cases operated by microvascular decompression, 10 recurrent cases in 68 cases operated by combing of facial nerve and 2 recurrent cases in 32 cases operated by both microvascular decompression and combing. CONCLUSION: Vascular compression is a cause of the hemifacial spasm, but not the only one. This paper pointed out the importance of selecting suitable treatment for the patient individually.

Adult↗
PubMed2004

Mind-refreshing acupuncture therapy for facial spasm, trigeminal neuralgia and stubborn facial paralysis.

It has been proved by clinical experiment that needling at Fengchi (GB 20), Wangu (GB 12) and Tianzhu (BL 10) can markedly improve the blood supply to the vertebral basilar artery, increase the cerebral blood flow, and relax the spasm of the vascular smooth muscles. The combined use of Shangxing (GV 23) and Yintang (EX-HN3) can give the effects of resuscitating and tranquilizing the mind, dispelling wind, dredging the channels, and relieving spasm and pain. In short, the above therapy may turn the pathological state into a normal physiological state, and bring a quicker recovery for patients with facial spasm, trigeminal neuralgia and stubborn facial paralysis.

Acupuncture Therapy↗
PubMed2004

Hemifacial spasm: results of treatment with low dose botulinum toxin injection.

BACKGROUND: Hemifacial Sapsm (HFS) is a common movement disorder in Thailand. Botulinum toxin type A (BTA) is an effective and safe treatment for this condition. The success of BTA treatment depends on the experience of the clinician. OBJECTIVE: To study the demographic data, efficacy and safety of low dose BTA injection in HFS patients. SETTING: The Spastic and Dystonia Clinic, Department of Rehabilitation Medicine, King Chulalongkorn Memorial Hospital. DESIGN: Open-label, prospective case-series study. PATIENTS: All patients with HFS referred for BTA injection from December 1st, 1995 to November 30th, 2003. METHOD: Sex, age, side of spasm, onset of symptoms before BTA injection, underlying diseases, sites of BTA injection, dose of each BTA treatment, duration of response, efficacy, and side-effects were analyzed. 3-5 units of BOTOX were intramuscularly injected per site to all muscles that had spasm. After injection, a 20-minute cold compression on the first day was followed by 20-minute warm compression with massage at each injection site per day for 14 days. RESULTS: A total of 112 patients with HFS were treated with 874 BTA treatments. There were 71 females (63.4%) and 41 males (36.6%). The mean age was 45 years. 75 patients (67%) were affected on the left side. Mean duration of symptoms was 3.4 years. The sites of injection were orbicularis occuli and orbicularis oris muscles in all 874 treatments (100%). The mean dose of all treatments was 25 units. The mean initial dose was 30.5 units. The mean dose for subsequent injection was 23 units. The mean duration between treatments was 4.7 months. The mean initial duration was 3.5 months. The mean duration for subsequent injection was 4.8 months. The outcomes of treatment assessed at 4 weeks after injection classified as excellent (>80% improvement) were found in 845 treatments (96.7%). Most treatments had no complication (91.9%). Ptosis, facial paresis and double vision were mild and transient, lasting 1-4 weeks. There were no long-term complications of BTA treatment in the present series. CONCLUSION: Low dose BTA injection is an effective treatment for hemifacial spasm patients. There was a longer duration of response in subsequent injections and a lower complication rate in the present study when compared to others.

Adult↗
PubMed1992

Manometry during food ingestion aids in the diagnosis of diffuse esophageal spasm.

It has been shown that food ingestion can provoke esophageal motor abnormalities in patients with otherwise normal manometry. Such motor abnormalities are usually nonspecific in character. We now report water swallow and food ingestion data on 12 patients with a history of dysphagia and/or chest pain who satisfied strict manometric diagnostic requirements for diffuse esophageal spasm. Three of these patients had normal water swallow manometry, yet, during food ingestion, showed manometric evidence of diffuse esophageal spasm. In the other nine patients, the occurrence of nonperistaltic contractions was greater, and there was a greater incidence of nonperistaltic contractions of 100 mm Hg or more after ingestion of food. We conclude that food ingestion increases the diagnostic yield of manometric testing for diffuse esophageal spasm and, not infrequently, magnifies an abnormality seen during standard water-swallow testing.

Adult↗
PubMed2005

Quantitative assessment of efficacy of dysport (botulinum toxin type A) in the treatment of idiopathic blepharospasm and hemifacial spasm.

This study was a Phase IV, prospective, one arm, non-comparative open trial, to investigate the efficacy and safety of Dysport (Botulinum toxin type A) in patients with idiopathic blepharospasm or hemifacial spasm. During the treatment period, patients were evaluated at baseline (week 0), week 6, and week 8, 10, or 12. Thirty two women and 16 men completed the whole course of the study. The therapeutic efficacy of Dysport became evident from 1.5 to 15 days (mean+/- SD, 6.1 +/- 2.9 days). The maximal effect appeared 12.2+/-5.0 days later. Injection of Dysport achieved 72.9 (13.0% amelioration in the spasm symptom. Dysport significantly improved the following functions, such as reading, watching TV, house work, working, driving and outing alone. At the twelfth week after Dysport injection, it was still effective in relieving blepharospasm or hemifacial spasm. The most frequent adverse event was ptosis, which was noted in 9 cases and represented 18.7% of total patients. Other adverse events were very mild, although lagophthalmos and dry eyes occurred in some patients, but none manifested any corneal complications. In conclusion, Dysport injection appears to be a safe, and effective procedure - accompanied only by minor, and transit adverse events.

Adult↗