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At least 865 records · Page 48Linked to original sources
PubMed1996

Etiologic classification of infantile spasms in 140 cases: role of positron emission tomography.

The classification of infantile spasms into symptomatic, cryptogenic, and idiopathic subgroups depends on clinical examination and available diagnostic technology. Positron emission tomography (PET) of glucose utilization is a powerful tool in detecting brain malformations (particularly cortical dysplasia) in infants with spasms. We analyzed etiologic data from 140 such infants, 78 girls and 62 boys, ages 2 months to 4 years 10 months (mean, 17 months). All had been evaluated extensively in one of two major medical centers. It should be emphasized that our referral population is biased toward infants with intractable spasms who fail to show a structural lesion. Seven patients had neurocutaneous syndromes, two had chromosomal abnormalities, two had inborn errors of metabolism, and one each had craniosynostosis or Menkes syndrome. Computed tomography and/or magnetic resonance imaging detected lesions in another 29 infants (20.7%) who did not have a specific disease or syndrome. Without the benefit of PET, the total number of symptomatic cases was 42 (30.0%). One infant, classified as idiopathic, had normal development and PET. In 97 cryptogenic cases, PET uncovered unifocal abnormalities in 30 and multifocal abnormalities in 62. Diffuse PET abnormalities, which did not provide specific etiologic information, were seen in three infants. Another two infants had normal PET scans. Thus, with the benefit of PET, the number of symptomatic cases rose dramatically from 42 (30.0%) to 134 (95.7%). The majority of unifocal and multifocal abnormalities on PET are believed to represent dysplastic lesions.

Brain↗
PubMed1996

How do cryptogenic and symptomatic infantile spasms differ? Review of biochemical studies in Finnish patients.

Infants with cryptogenic infantile spasms seem to differ from those with symptomatic spasms in having a higher cerebrospinal fluid corticotropin content, different levels of corticotropin release after exogenous vasopressin, higher serum levels of progesterone, higher dehydroepiandrosterone: androstenedione ratio (during corticotropin therapy), a higher cerebrospinal fluid gamma-aminobutyric acid content, and higher cerebrospinal fluid nerve growth factor concentrations. It remains to be seen whether the biochemical differences between the two groups are specific or only happen to correlate with the early brain damage. However, these differences would explain many pathophysiologic features of infantile spasms.

Diagnosis, Differential↗
PubMed1999

Vein of Galen malformation and infantile spasms.

Recently, there has been increasing interest in the study, characterization, and management of infantile spasms. With technological advances, it is now possible to determine a specific diagnosis on many patients with infantile spasms, thus broadening the spectrum of conditions associated with this electroclinical syndrome. We report a child with infantile spasms who also had a vascular malformation of the vein of Galen. Both clinical and electroencephalographic findings improved rapidly after endovascular treatment of the vascular anomaly, suggesting a possible association.

Cerebral Veins↗
PubMed1975

Infantile spasms associated with histidinemia.

A case of infantile spasms associated with histidinemia is presented. Histidinemia was well-documented through biochemical assays. The patient was treated with the standard anticonvulsant regimen for infantile spasms, as well as an elimination diet for histidinemia. Despite low levels of histidine and adequate anticonvulsant therapy, the child continues to have seizures and is markedly retarded. The natural history of infantile spasms and its possible association with histidinemia is discussed.

Amino Acid Metabolism, Inborn Errors↗
PubMed1979

Hemifacial spasm: clinical and electrophysiologic observations.

Twenty-three patients with hemifacial spasm were studied clinically and electrodiagnostically. Seven patients had mild facial weakness. All patients had clinical evidence of synkinesis, which often varied considerably. Facial nerve conduction and blink reflex latencies were normal. Facial synkinesis could be measured objectively on the involved side in all patients by simultaneously recording from the orbicularis oculi and orbicularis oris muscles at the time of supraorbital nerve stimulation. Using this procedure, synkinesis was also observed in association with aberrant regeneration after Bell palsy but was not seen in other movement disorders involving the face. The demonstration of synkinesis and its variability in hemifacial spasm can be of value in differentiating hemifacial spasm from other movement disorders affecting the face and provides further insight into its pathogenesis.

Electric Stimulation↗
PubMed1981

Microvascular decompression of the facial nerve for hemifacial spasm: clinical and electrophysiologic observations.

Eight patients with idiopathic hemifacial spasm were studied before and after decompression of the facial nerve. Seven patients had an excellent clinical response to surgery, with total resolution of the spasm. One patient had a complete facial palsy and sensorineural deafness on the involved side after surgery, with recurrence of the spasm 6 months later. In all patients, synkinetic activity was present on the involved side before surgery and disappeared after surgery. These findings suggest that the disorder involves the extra-axial portion of the facial nerve. The findings do not require an etiologic role of vascular compression because the response to surgery could be related either to mild trauma of the nerve during the surgical procedure or to subsequent fibrosis.

Adult↗
PubMed1984

Cerebrospinal fluid monoamine metabolites in patients with infantile spasms.

We measured CSF levels of homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) by high-performance liquid chromatography in seven children with infantile spasms and in a group of age- and sex-matched controls. The mean concentration of the serotonin metabolite 5-HIAA was 40% (p less than 0.01) lower in the infantile spasms group as compared with controls; HVA levels were similar in both groups. The data provide additional evidence that serotonin metabolism is abnormal in patients with infantile spasms.

Adolescent↗
PubMed1984

Cerebrospinal fluid GABA levels in children with infantile spasms.

The mean CSF GABA level in 22 children with infantile spasms was significantly lower than in 35 controls. There was no significant difference between the CSF GABA levels in untreated children and those treated with anticonvulsants, or between uncontrolled and temporarily controlled patients. After treatment with ACTH, CSF GABA levels were lower than before treatment, and seizures disappeared. Brain GABAergic neuronal function may be disturbed in children with infantile spasms, but the decreased CSF GABA level does not directly correlate with the seizures of infantile spasms.

Adrenocorticotropic Hormone↗
PubMed1989

Blink reflex excitability cycle in hemifacial spasm.

We studied electrically elicited blink reflex responses in patients with hemifacial spasm (HFS) by applying single isolated, as well as paired (conditioning and test), stimuli at both sides of the face. Responses after single stimuli were of larger size on the side of the spasm compared with the uninvolved side and controls. With paired stimuli, the inhibitory effect of the conditioning stimuli upon the test stimuli late response (R2), which was always observed in normals, was significantly less pronounced at short interstimuli intervals. This resulted in an enhanced recovery curve of R2, which was observed on the side of the spasm and the contralateral, clinically normal side. Patients with longer disease duration showed more striking abnormalities of the recovery curve. We suggest that there is enhanced excitability of facial motoneurons and of those brainstem interneurons that mediate the blink reflex pathway in patients with HFS.

Action Potentials↗
PubMed2000

Primary hemifacial spasm and arterial hypertension: a multicenter case-control study.

In a case-control study, the authors found that arterial hypertension occurred more frequently among 115 patients with primary hemifacial spasm than among 115 neurologic controls matched for age (+/-5 years), sex, and referral center. The association was not confounded by education level, smoking history, diabetes, or other diseases (adjusted OR 2.64; 95% CI 1.3 to 5.33, p = 0.007). Hypertension was significantly associated with the outcome in the left-sided group (OR 4.0; 95% CI 1.4 to 11.5), but data concerning patients with right-sided spasm were inconclusive (OR 1.05; 95% CI 0.36 to 3.1). In our sample, hypertension either preceded or followed the onset of hemifacial spasm.

Aged↗
PubMed1984

Treatment of tic douloureux and hemifacial spasm by posterior fossa exploration: therapeutic implications of various neurovascular relationships.

A series of 152 posterior fossa explorations for tic douloureux and hemifacial spasm has been reviewed with assessment of outcome at the last follow-up examination. Among 103 cases of tic followed for an average of 48.3 months, 79 patients (77%) obtained good or excellent symptomatic relief, and there were 24 failures or recurrences (23%). Of 48 cases of hemifacial spasm followed for an average of 42.1 months, there were good or excellent results in 42 cases (87.5%); only 6 patients (12.5%) experienced failure or recurrence. Patients noted to have arterial contact at the 5th nerve entry zone responded significantly better to microvascular decompression than did patients with no arterial contact. Further, patients noted to have anatomical distortion of the 5th nerve by an artery or wedging of an artery into the crevice between the nerve and the pons had significantly better outcomes after microvascular decompression than did patients with other kinds of arterial contact. Partial sensory rhizotomy proved to be a highly effective alternative to microvascular decompression in cases of doubtful neurovascular compression. It was not possible to define similar neuroanatomical criteria predictive of response to microvascular decompression in patients with hemifacial spasm.

Adult↗
PubMed1986

Resolution of hemifacial spasm after posterior fossa exploration without vascular decompression.

A 57-year-old woman who had hemifacial spasm on the left side for 5 years underwent exploration of the cerebellopontine angle region for the purpose of microvascular decompression. At operation, however, no artery, vein, or other abnormality was noted at the facial nerve root exit zone. Consequently, the exploration was completed without additional manipulation or the placement of a protective barrier, but the patient awoke from the operation completely free of the spasm. The cause of the hemifacial spasm in this patient could not be explained by the currently predominating vascular compression theory.

Biomechanical Phenomena↗
PubMed2006

Resolution of hemifacial spasm after surgical obliteration of a tentorial arteriovenous fistula: case report.

OBJECTIVE AND IMPORTANCE: We describe a patient with a tentorial dural arteriovenous fistula who presented with ipsilateral hemifacial spasm. CLINICAL PRESENTATION: A 50-year-old man sought treatment for left facial twitching that worsened over 6 months. Magnetic resonance imaging and catheter angiography demonstrated a left tentorial dural arteriovenous fistula. INTERVENTION: The patient underwent a retrosigmoid craniotomy and ligation of the draining vein at the site of the fistula. Intraoperative angiography showed complete obliteration of the fistula. The patient's hemifacial spasm improved significantly after the fistula was obliterated. CONCLUSION: Posterior fossa arteriovenous fistulas can present with a hemifacial spasm related to compression of the facial nerve by arterialized leptomeningeal veins. Microsurgical obliteration of the fistula can resolve the related symptoms.

Arteriovenous Fistula↗
PubMed2006

Reoperation for persistent or recurrent hemifacial spasm after microvascular decompression.

OBJECTIVE: The objective of this study was to investigate the outcome of reoperation for persistent or recurrent hemifacial spasm (HFS) after microvascular decompression (MVD). METHODS: Repeat MVD was performed on 13 patients with an HFS between June 1994 and May 2004. Patients who had compressing offending vessels identified on postoperative (prerevision) three-dimensional short-range magnetic resonance angiography were selected for repeat MVD. RESULTS: Six patients were found to have no improvement in HFS with the first MVD. All of these patients exhibited excellent improvement after the second MVD. In one patient who had mild improvement with the first MVD, but with more than 50% of remaining spasm, complete abolition of spasm occurred immediately after the second MVD. Six patients showed initial relief and subsequent aggravation of HFS after the first MVD. Of these patients, four had excellent results with the second MVD, one had a good result, and one had a fair outcome. Adverse effects after the second MVD were found in two patients (one patient with permanent mild facial weakness and one patient with hearing impairment). There was no serious morbidity associated with the second MVD. CONCLUSION: Our data suggest that repeat MVD of the facial nerve may be sufficient to resolve symptoms in selected patients with persistent or recurrent HFS. Additionally, three-dimensional short-range magnetic resonance angiography may help to identify the offending vessels and to select the patients with persistent or recurrent HFS.

Adult↗
PubMed2005

Outcome following retrosigmoid microvascular decompression of the facial nerve for hemifacial spasm.

This paper evaluates the outcome of retrosigmoid microvascular decompression of the facial nerve in a series of patients suffering from hemifacial spasm who had been referred to the skull-base team (comprising senior authors DAM and DGH). The paper is a retrospective review of 15 patients who underwent retrosigmoid microvascular decompression of the facial nerve at Addenbrooke's Hospital between 1985 and 1995. In this series it was possible to obtain complete resolution of hemifacial spasm in 93.3 per cent of cases in the short term and in 80 per cent in the long term. Twelve patients (80 per cent) were symptom-free post-operatively. Two patients had minor recurrence of symptoms occurring within six months of the procedure. One patient with no identifiable vascular impingement of the facial nerve had no improvement following surgery. Three patients suffered sensorineural hearing loss. Two patients complained of post-operative tinnitus, and transient facial palsy was noted in one patient. Retrosigmoid microvascular decompression of the facial nerve provides excellent long-term symptom control in a high percentage of patients with hemifacial spasm.

Adult↗
PubMed1995

Does peppermint oil relieve spasm during barium enema?

The effectiveness of topical peppermint oil added to barium sulphate suspension in relieving colonic muscle spasm during double contrast barium enema examination was assessed in a double blind study. 141 patients were randomized either to a control group (71 patients) examined with standard barium suspension or to the treatment group which received peppermint oil mixed with the barium preparation. No residual spasm was evident in a significant proportion of patients in the treated group (60%) compared with the control group (35%) (p < 0.001). The patients' acceptability of the procedure was good and there were no adverse effects on the overall quality of the examination. In conclusion, the addition of peppermint oil to the barium suspension seems to reduce the incidence of colonic spasm during the examination. The technique is simple, safe, cheap and it may lessen the need for intravenous administration of spasmolytic agents.

Adult↗
PubMed1987

Treatment of hemifacial spasm with transcutaneous electrical stimulation.

A transcutaneous electrical stimulation method was used to treat hemifacial spasms. This treatment was applied concomitantly with drug therapy to 21 patients, and was found effective in 17 patients (81%). Electrotherapy is considered to be useful in patients with mild spasms and in those with severe spasms for whom surgical treatment is not possible.

Adult↗