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Phenobarbital-responsive ptyalism, dysphagia, and apparent esophageal spasm in a German shepherd puppy.

A 10-week-old, male German shepherd dog was presented with a primary complaint of episodic ptyalism, dysphagia, vomiting, and mandibular salivary gland enlargement. An esophagram with fluoroscopy showed normal pharyngeal and esophageal function; however, upper gastrointestinal endoscopy and cervical ultrasonography revealed a focal circumferential thickening of the midcervical esophageal muscular wall, consistent with esophageal spasm. The puppy responded dramatically and completely to phenobarbital treatment. An unusual syndrome of phenobarbital-responsive hypersialosis was consistent with this dog's clinical presentation and the finding of apparent esophageal spasm. The pathogenesis of this syndrome is unclear, but it may represent a form of limbic epilepsy or peripheral autonomic dysfunction.

Animals↗

Accommodative microfluctuation in asthenopia caused by accommodative spasm.

BACKGROUND: Although many patients complain of eye fatigue caused by accommodative spasm, there have been no reports of a good objective examination method to diagnose it. PURPOSE: The spectral power of the high frequency component of the accommodative microfluctuation (spectral power of HFC) differs according to the constrictive degree of the accommodation. In this paper, we expatiated upon our previously reported analyzing processes of the spectral power of HFC, and we investigated the relationship between normal subjects and subjects with asthenopia. METHOD: The accommodative microfluctuation were recorded when the subjects were looking at a stable target. The waves of the accommodative microfluctuation were analyzed by FFT. RESULTS: The spectral power of HFC for the distant target was 50-60 in the subjects with normal vision, but it was higher in the subjects with asthenopia. CONCLUSION: Our results suggested that the ciliary muscle was also actively working in asthenopia caused by accommodative spasm even if the patient was looking at a distant target.

Accommodation, Ocular↗

Coronary spasm management with verapamil (Isoptin).

Because of recurrent chest pain, 17 patients with documented spasm in coronary arteries without severe atherosclerotic obstructions were treated with verapamil in an open trial, using doses of 120 mg/day to 480 mg/day (mean, 240 mg/day). Thirteen patients received additional oral isosorbide dinitrate (30 mg/day). Eleven patients became asymptomatic, and four experienced marked reduction in frequency of attacks (one to four a month). While two patients did not respond, in most patients verapamil was effective in preventing attacks of angina due to coronary spasm.

Administration, Oral↗

Trigeminal neuralgia and hemifacial spasm: treatment by microvascular decompression.

On the basis of observation of 26 patients, we consider that the common cause in trigeminal neuralgia and hemifacial spasm is compression of the nerve-root entry zone at the brainstem by a blood vessel, usually an artery. By mobilising the vessel, and securing it away from the nerve, symptoms were relieved in 16 of 17 patients with trigeminal neuralgia and in seven of nine patients with hemifacial spasm for a follow-up period of up to four years. Trigeminal microvascular decompression had few complications, but three patients treated for facial hemispasm had postoperative deafness and one had a probable small brainstem infarct. The major advantage of this surgical approach is that is possible to relieve the symptoms without facial anaesthesia or paralysis.

Adult↗

[Coronary artery spasm induced by the somministration of ergonovine maleate in subjects with spontaneous angina (author's transl)].

In 6 patients affected by spontaneous angina with S-T elevation and coronariographic findings of obstruction, intravenous administration of ergonovine maleate determined the same clinical and ECG patterns of spontaneous episodes. The coronary arteriography during pain showed a marked spasm with occlusion of a large coronary vessel in four patients. In 2 patients with atypical chest pain and normal coronariogram, E.M. did not induce pain, ECG abnormality or coronariographic alterations. The role of spasm in spontaneous angina is discussed.

Adult↗

[Coronary spasm on exercise. Demonstration of a case by coronary angiography].

We have seen a case with spasm of the right coronary artery coming on during exercise ergometry in the course of a coronary arteriogram. The patient had angina pectoris spontaneously and on exercise. Bicycle ergometry was repeated four times by the same method, and was positive in three; in two of these there was angina and ST elevation in II, III, aVF, and ST depression in I, aVL and V2 to V5. On one occasion the test was negative, the patient having taken a trinitrin tablet one hour before the test. Repeating the exercise test during coronary arteriography showed spasm of the right coronary artery and elevation of the segment ST in II, III and aVF; this disappeared after trinitrin treatment.

Adult↗

[Use of botulin toxin in the treatment of diffuse esophageal spasm. A case report].

The diffuse esophageal spasm distinguishes from others esophageal disturbs by the symptoms severity and bad results to conservative treatment. This situation requires formal indication to surgical treatment or dilation. In high risk patients this situation becomes a big problem, due to the lack of another therapeutic option. In a patient with this problem we use an endoscopic injection of botulin toxin in cardia with good results in three months follow-up. Despite this short period of observation we believe that the infiltration with botulin toxin in cardia constitutes a valid therapeutic option in treatment of diffuse esophageal spasm.

Aged↗

X linked mental retardation and infantile spasms in a family: new clinical data and linkage to Xp11.4-Xp22.11.

In order to describe the neurological abnormalities and to identify the gene localisation, we re-evaluated a previously reported family with X linked mental retardation (XLMR). Reliable data were obtained for six of the seven affected males, of whom two had had infantile spasms. Profound MR (IQ<20) was found in one and mild MR (IQ 50-70) in five males. No dysmorphic features, except for macrocephaly in one male, were found. Neurological abnormalities included varying degrees of spinocerebellar involvement. Neuroimaging studies showed abnormalities, such as cerebellar atrophy or corpus callosum hypoplasia or both, in three of the six males. Several affected and unaffected subjects suffered from hyperhidrosis, which appeared to segregate independently as an autosomal dominant trait. Genetic linkage analysis localised the XLMR disease gene to Xp11.4-Xp22.11 with a maximum multipoint lod score of 3.57, overlapping the candidate region recently found in two Belgian XLMR-infantile spasm families. Compared to the Belgian patients, the majority of the affected males in this report had a considerably milder phenotype.

Adolescent↗

[The eye-lid reflex in hemifacial spasms. Study of 57 patients].

FUNDAMENTALS: [corrected] The blink reflex is the neurophysiological method for the study of hemifacial spasm. It is a polysynaptic reflex with a primary component or R1, which is of pontine origin, and a secondary component or R2 probably originating on the pons and lateral bulb. OBJECTIVE: To study hemifacial spasm, its clinical, and demographic characteristics and the possible neurophysiological (blink reflex) differences between symptomatic and idiopathic cases. METHODS: The study population included 57 patients (35 women and 22 men) with no axonal damage on the seventh cranial nerve; the control group included 57 healthy people paired up according to age and sex. Patients underwent a CT scan or MRI. Blink reflex was studied on the healthy and damaged sides of the patient group and left side was studied in the control group. After hyperventilation the study was repeated on the affected patient group only. RESULTS: The patients' average age was 53.4 years and the average duration of the disease was 3.7 years. Thirty-four patients had proven structural anomalies and the left side was affected in the same number. When comparing the results between the damaged side, the normal one and the control side it was only significant for the R1 component (p < 0.00000). No differences were found between the symptomatic and the idiopathic cases before or after hyperventilation, that lengthened R1, R2 and R2c latency periods (p < 0.00000; 0.0007 and 0.00000, respectively). CONCLUSIONS: Blink reflex does not enable the establishment of differences between the idiopathic and the symptomatic cases. The rise of the latency periods after hyperventilation signals the beginning of hyperexcitability of the secondary facial motor nucleus due to a peripheral lesion, to loss of supranuclear inhibitory control or both.

Blinking↗

The Smith-Magenis syndrome: a new case with infant spasms.

The Smith-Magenis syndrome (SMS) is characterized by congenital anomalies, mental retardation and the interstitial deletion of the 17p. 11.2 chromosome. The subjects affected by this syndrome show cranio-facial dysmorphias, brachycephalia, skeletal, ocular, cardiac, genitourinary and otolaryngological anomalies. The central nervous system is affected and this may be shown by psychomotor retardation, intellective deficit, electroencephalographic alterations (reduced/missing REM phase); the neuroradiological tests detect megacisterna magna, cerebellar hypoplasia, cortical dysplasia, ventricular asymmetry. Behavioural troubles are frequent and, among them, self-aggressive conducts (tearing out the nails). The syndrome is associated with the interstitial deletion of the 17p. 11.2 chromosome. The diagnosis can be made in the pre-natal period and a mosaic situation is possible. Even though the cases of SMS reported in the literature allow defining a characteristic phenotype, studies have been carried out to quantify the deletion of the chromosome 17 in order to identify the chromosomic tract which is responsible for the phenotypical induction. The deletion can either appear de novo or come from one of the parents. In addition, these subjects can show peripheral neuropathy, missing or reduced deep tendon reflexes and (rarely) epileptic crises. However, by reviewing the literature, no descriptions of patients affected by infant spasms are pointed out. This report refers to a new case of Smith-Magenis syndrome in a nine-month-old girl with spasms in extension.

Brain↗

[Treatment of hemifacial spasm with type A botulinum toxin (AGN 191622): a dose finding study and the evaluation of clinical effect with electromyography].

Forty-one patients with hemifacial spasm had an injection of type A botulinum toxin (AGN 191622; Allergan Co. Ltd., Irvine, CA). Patients were randomly divided into 3 groups by the injection dose: group L (1 unit; 14 patients), group M (5 units; 14 patients), and group H (10 units; 13 patients). Half of the dose was injected into the orbicularis oculi and the rest into the zygomaticus major muscles on the affected side. The clinical effect and electromyogram were evaluated at 2 weeks after the injection. The clinical benefit was dependent on the injection dose, and group H showed the highest rate of improvement (84.6%). No adverse effect related to the toxin was demonstrated except one patient in group H who showed mild and transient lagophthalmos. For 81.8% of group H patients, the final judgement was "useful" or "very useful", which was 9.1% for group L and 50.0% for group M. On the other hand, electromyography disclosed no consistent dose-finding relationship. We conclude that at least 10 (preferably more) units of botulinum toxin are necessary for effectively treating hemifacial spasm. Electromyography has only limited value for the evaluation of clinical effect.

Botulinum Toxins, Type A↗

Role of magnetic resonance imaging and magnetic resonance angiography in patients with hemifacial spasm.

Vascular compression of the facial nerve is a well recognized cause of hemifacial spasm (HFS). In this study, we described the magnetic resonance imaging (MRI) and three-dimensional magnetic resonance angiography (MRA) techniques used and findings in 34 patients with hemifacial spasm. A vascular abnormality, defined as a vessel seen in close proximity, touching or compressing the facial nerve, was identified in 22 of the 25 patients (88%) who had both MRI and MRA studies. Vessels identified were anterior inferior cerebellar artery (59.2%), posterior inferior cerebellar artery (13.6%), vertebral artery (18.2%) and basilar artery (4.5%). All vascular abnormalities were ipsilateral to the side of the HFS. Only 3 of the 12 controls (25%) had a vascular abnormality in both MRI and MRA studies. One of the 9 HFS patients (11.1%) who had MRI only had an ipsilateral vascular abnormality. There is a role for combined MRI and MRA studies in the non-invasive evaluation of patients with HFS.

Adult↗

Botulinum toxin treatment of hemifacial spasm and blepharospasm: objective response evaluation.

Twenty seven patients with hemifacial spasm (HFS) and sixteen patients with blepharospasm (BS) having mean Jankovic disability rating scale score of 2.56+0.58 SD and 2.81+0.54 SD, respectively, were treated with botulinum toxin A (BTX-A) injections. The total number of injection sessions were ninety one with relief response in 98.91%. The mean improvement in function scale score was 3.78+0.64 SD and 3.29+1.07 SD respectively, in HFS and BS groups. The clinical benefit induced by botulinum toxin lasted for a mean of 4.46+3.11 SD (range 2 to 13) months in HFS group and 2.66+1.37 SD (range 1 to 6) months, in BS groups. Transient ptosis was seen in 4.39% of total ninety one injection sessions. These findings show that local botulinum toxin treatment provides effective, safe and long lasting relief of spasms.

Adult↗

[Coronary artery bypass grafting for a patient with bronchial asthma seceeded from cardiopulmonary bypass by additional bypass for spasm of radial artery graft: a case report].

A 78-year-old male who had a bronchial asthma underwent coronary artery bypass grafting (CABG) using the left internal thoracic artery and the radial artery. The patient could not be weaned from the cardiopulmonary bypass because the radial artery which anastomosed to the obtuse marginal artery (OM) had a spasm after CABG. An additional bypass using a long saphenous vein to OM was carried out immediately. It brought a weaning from cardiopulmonary bypass. If the cardiac function after CABG is insufficient in patients with bronchial asthma, CABG must be re-done immediately, considering that they cause the arterial spasm more than patients without bronchial asthma.

Aged↗

[Infantile spasms: clinical course].

The authors report on the factor which interferes on follow-up of children affected by infantile spasm. On the basis of their series and on the data of literature the factor more indicative for the clinical evolution are: type and dosage of treatment, onset early or delayed, and the aetiology. While for the first two factors the opinion of the expert are remarkable discordant, there is a general agreement that the evolution of infantile spasm is mainly based on the causes determining such kind of epilepsy.

Humans↗

Transurethral surgery and the adductor spasm.

INTRODUCTION: Transurethral surgery has become the dominant treatment for bladder and prostatic tumours. Regional anaesthesia is the favoured anaesthetic technique but adductor spasm leading to bladder wall perforation and increased morbidity is not uncommon. This review seeks to outline the cause, risk factors predisposing to this complication and the various techniques that have been adopted to eradicate it. METHODS: Literature search was performed from PubMed (1965 onwards). All studies related to obturator nerve stimulation or adductor spasm in transurethral surgery were short-listed. RESULTS: Various methods have been attempted. These ranged from local blockade of the obturator nerve, periprostatic and subvesical lignocaine infiltration, changing the site of the inactive electrode, reduction of the electrocoagulation voltage and general anaesthesia with muscle relaxants. CONCLUSIONS: Hitherto, local blockade of the obturator nerve, either by the direct method or "3-in-1" method is the most effective in preventing this complication. With the aid of a nerve stimulator, greater accuracy and use of smaller volumes of local anaesthetic will improve the safety of this block.

Female↗

Hemifacial spasm or subcortical epilepsy?

A child with Goldenhar's syndrome presented at about one week of age with stereotyped, repetitive paroxysmal episodes of hemifacial contraction, suggestive of partial seizures or hemifacial spasm. Later in life he also developed independent, permanent abnormal eye and chin movements identical in rhythm to those seen in myorhythmias, suggesting involvement of the dentato-olivary pathway. MRI demonstrated a hamartomatous lesion at the level of the pontomedullary junction. We speculate that the nature of the lesion could be responsible for the partial seizures mimicking hemifacial spasm and that because of its location, this same lesion could also be implicated in the genesis of myorhythmias. The presence of a hamartomatous lesion in a region affected by the abnormal development of the first and second branchial arches is not fortuitous.

Adolescent↗