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Infantile spasms--seen in West's Syndrome--are often associated with cortical abnormalities. The spasms themselves, however, appear to be generated subcortically. Dr. Chugani reviews the clinical data related to the pathophysiology of infantile spasms and proposes a hypothesis which involves both cortical and subcortical mechanisms.
The authors report two patients with neurofibromatosis type 1 who were affected by infantile spasms. The infantile spasms were severe and unresponsive to anticonvulsant treatment. The authors maintain that infantile spasms may belong to the clinical features of neurofibromatosis type 1.
From a series of 230 subjects with infantile spasms (I.S.) observed from 1955 to 1982, 191 cases with sufficient data on follow-up and evaluation were selected. The period of follow-up in 100 subjects ranged from 3 months to 3 years and was more than 3 years in the remaining 91 patients. Disappearance of spasms and normal psychomotor development was observed in 34 of the 61 idiopathic and post-infectious cases. In 130 subjects with secondary forms normal psychomotor development was observed in only two patients; disappearance of spasms was noted in 54 and normalisation of the EEG in 13 subjects. Satisfactory long term results can be obtained with a relatively low dose of corticotropin in a considerable number of patients having idiopathic and post-infectious forms of I.S. To obtain the best results treatment should be started as soon as possible.
Two cases of unilateral tonic spasm are described in which high signal lesions typical of demyelination were identified on magnetic resonance imaging in the contralateral cerebral peduncle. Both cases exhibited subtle upper motoneuron signs on the side of the tonic spasms that were exacerbated immediately following an attack. In one case, functional impairment of contralateral corticospinal pathways was demonstrated by delayed central motor conduction. These cases support the view that tonic spasms in multiple sclerosis are due to lesions within the corticospinal tract.
We show evidence that the motonucleus of the facial nerve is involved in producing the synkinesis in patients with hemifacial spasm. These results were obtained by recording from the intracranial portion of the facial nerve and from the orbicularis oculi muscle in patients operated upon for hemifacial spasm during electrical stimulation of the mandibular branch of the facial nerve. Also, the electromyographic response from the same muscle was recorded when the facial nerve was electrically stimulated at a location near the brainstem. The results show that it is unlikely that the symptoms of patients with hemifacial spasm can be explained on the basis of ephaptic transmission at the site of lesion of the facial nerve.
There are no requirements concerning the amplitude of simultaneous contractions among the present criteria for the manometric diagnosis of diffuse esophageal spasm. The purpose of this investigation was to determine whether the current criteria effectively identify an appropriately homogenous patient population. Sixty consecutive motility tracings that met the criteria for diffuse esophageal spasm were evaluated. A bimodal distribution of the highest simultaneous esophageal contraction for each patient was observed. One group's (N = 29) highest simultaneous esophageal contractile amplitude was < or = 74 mm Hg, the other's (N = 31) highest simultaneous esophageal contractile amplitude was > or = 100 mm Hg. Group 1 had significantly decreased lower esophageal sphincter pressure, lower peristaltic amplitude, more aperistalsis, fewer simultaneous contractions, and fewer complaints of chest pain. These comparisons suggest that consideration be given to the amplitude of simultaneous esophageal contractions in the manometric diagnosis of diffuse esophageal spasm.
Botulinum A toxin was injected into the affected muscles in 20 patients with blepharospasm, 8 with torticollis and 12 with hemifacial spasm. In all cases blepharospasm and hemifacial spasm was abolished or markedly reduced. The only side effect was transient ptosis and diplopia. Patients with torticollis had a mild to moderate improvement of the dystonic posture and pain; dysphagia was the most troublesome side effect. Botulinum A toxin is an effective therapy in patients with focal dystonia and spasms.
In six Centers belonging to the Italian Movement Disorder Study Group, the efficacy of botulinum toxin treatment was evaluated in an open collaborative study in 251 patients with focal dystonia and hemifacial spasm. The percentage of functional improvement ranged from 66% to 81% in patients with blepharospasm, from 40% to 51% in patients with spasmodic torticollis and from 73% to 81% in those with hemifacial spasm. Good results were also obtained in patients with oromandibular dystonia, laryngeal dystonia and writer's cramp. Side effects were mild and transient. Local botulinum toxin injection is the first choice symptomatic treatment in focal dystonia and hemifacial spasm.
Five patients developed coronary artery spasm during open heart surgery in our institute between 1984 and 1988. One patient was undergoing coronary artery bypass grafting and the other four valvular surgery or surgery for congenital heart disease. In one of the patients undergoing non-coronary surgery, the preoperative induction of right coronary artery spasm by ergonovine had been documented angiographically while the remaining three patients did not possess organic or functional coronary disease. All five patients exhibited a sudden onset of hemodynamic collapse with ventricular tachyarrhythmias or ST elevation during the early period of reperfusion, the time to onset being 89.2 +/- 84.8 minutes after unclamping of the aorta. In addition, contraction of the right ventricular free wall was severely impaired. Although one patient died due to left ventricular rupture caused by direct cardiac massage, the early mortality thus being 20 per cent, the other four were successfully treated with the intravenous administration of nitroglycerin and diltiazem. Three patients required the assistance of intraaortic balloon pumping for severe cardiac failure. Thus, during open heart surgery, coronary artery spasm can occur even in patients without organic coronary lesions and the possible mechanisms of this condition are discussed herein.
Although the beneficial effect of subcutaneous injections of botulinum toxin type A (BTX-A) is well known in both blepharospasm and hemifacial spasm, the position of the injection sites around the orbicularis oculi may influence the effectiveness and side effects. Here we report results of preseptal and pretarsal BTX-A injections in 53 patients (25 blepharospasm and 28 hemifacial spasm) in whom we used both injection techniques successively. Pretarsal injections were used in 102 out of 186 treatments in blepharospasm group and in 84 out of 202 treatments in hemifacial spasm group. Pretarsal BTX-A treatment produced significantly higher response rate and longer duration of maximum response in both patient groups. This technique was also associated with a lower frequency of major side effects such asptosis. We concluded that injections of BTX-A into the pretarsal, rather than the preseptal portion of the orbicularis oculiis more effective for treatment of involuntary eyelid closure due to contractions of this muscle.
MRI was used to investigate 100 patients with hemifacial spasm, using 3D-FT T2-weighted (CISS) and contrast-enhanced 3D-FT T1-weighted (turbo-FLASH) sequences in all cases. MR angiography was performed in 54 patients, using 3D-MT FISP images. Decompression of the facial nerve through a retromastoid craniotomy was performed in all patients. Hemifacial spasm caused by tumours in the cerebellopontine angle was not included. Vascular contact with the facial nerve root-exit zone or at the internal auditory canal was present in 96 of 100 patients with hemifacial spasm. The vessel responsible was the vertebral artery (VA) in 18 cases, the posterior inferior cerebellar artery (PICA) in 23, the anterior inferior cerebellar artery (AICA) in 22, the VA and PICA in 24, VA and AICA in 3, PICA and AICA in 1, VA, PICA and AICA in 4, and a vein in 1 case. CISS images showed compressive vascular loops better than contrast-enhanced turbo-FLASH images alone. The sensitivity of MRI was high, since only one false-negative case was found among the 100 patients who underwent surgery.
We report a rare case of symptomatic hemifacial spasm caused by a fusiform vertebral artery aneurysm. A 59-year-old woman presented with left hemifacial spasm of 18 months duration. Magnetic resonance imaging showed an enlarged a fusiform aneurysm of the left vertebral artery which compressed the seventh cranial nerve at its exit from the caudal pons. Microvascular decompression of the facial nerve with moving of the aneurysm resulted in complete relief of the hemifacial spasm. No enlargement of the aneurysm was shown on follow-up for a period of 6 years.
BACKGROUND: Microvascular decompression (MVD) for hemifacial spasm (HFS) provides a long-term cure rate. Delayed facial palsy (DFP) is not an unusual complication, but it has only been sporadically described in the literature. The purpose of this report is to evaluate the incidence of delayed facial palsy after MVD and its clinical course and final results. METHODS: From January, 1998 to April, 2004, 410 patients underwent microvascular decompression for hemifacial spasm at our Institute. During this time, 21 patients (5.4%) developed delayed facial weakness; eighteen of them were given steroid medication and they were followed up in the out-patient clinic. FINDINGS: Twenty-one patients developed DFP after microvascular decompression an incidence of 5.4%. There were seventeen women (81.0%) among the 21 patients with DFP who were included in this study. In twenty of them, the symptoms of HFS improved completely after the operation, but the spasm remained with one of them. The onset of palsy occurred between postoperative day 7 and 23 (average: 12.1 days). The palsy was at least Grade II or worse on the House-Brackmann (HB) scale. The time to recovery averaged 5.7 weeks (range: 25 days-17 weeks); 20 patients improved to complete recovery and 1 patient remained with minimal weakness, as Grade II on the HB scale, at the follow-up examination. CONCLUSION: Our findings demonstrated that the incidence of DFP was not so low as has been reported the literature, and it did not have any striking predisposing factors. Even though the degree of facial palsy was variable, almost all patients exhibited a complete recovery without any further special treatment. The etiology of DFP and its association with herpes infection should be further clarified.
Pre-operative and postoperative oblique sagittal gradient-echo magnetic resonance (MR) imaging was used to evaluate micro-vascular decompression of the facial nerves in 26 patients with hemifacial spasm. The pre-operative MR images were divided into two groups as follows: 22 images in Group I, clear imaging of a high-intensity line and/or spot at the root exit zone (REZ) of the facial nerve; and 4 in Group II, and unreliable image around the REZ. Surgery found that the causative vessel was the vertebral artery (VA) in 9 cases and the anterior inferior cerebellar artery (AICA) or the posterior inferior cerebellar artery (PICA) in 13 cases in Group I, and the AICA or the PICA in the 4 cases in Group II. Postoperative MR imaging showed clear decompression as the high-intensity line and/or spot completely separated from the REZ by a low- and/or iso- intensity area in 9 cases of VA compression repositioned to the petrous dura matter, in 11 cases of PICA or AICA compression treated by shredded Teflon pledgets in Group I and in 3 cases in Group II. Postoperative MR imaging showed an incomplete separation of any high-intensity line and/or spot in the REZ in 2 cases of PICA or AICA compression in Group I and in one in Group II. The outcome was excellent in 22 of 23 cases with clear decompression, and in 1 of 3 cases of unclear decompression. Hemifacial spasm persisted in 3 cases. Oblique sagittal gradient-echo MR imaging is a useful method for postoperative follow-up which can demonstrate changes around the REZ of the facial nerve if hemifacial spasm recurs.
BACKGROUND: In view of the fact that a basal craniectomy in microvascular decompression (MVD) for hemifacial spasm (HFS) can minimize cerebellar retraction and expose the facial nerve root exit zone (FNREZ) directly from below without placement of tension on the seventh-eighth cranial nerve complex, we used a more basal approach in 32 patients with typical HFS. METHOD: A slightly curved skin incision 5 cm in length and 2 cm posterior and parallel to the mastoid notch was made. The basal lateral occipital plate including the lateral one-fourth of the condylar fossa and the posterior one-fourth of the jugular process were removed. For the early drainage of cerebrospinal fluid through a small dural hole, the basal occipital plate posteromedial to the condylar fossa was removed. With this basal craniectomy, minimum elevation of the cerebellar tonsil and flocculus could expose FNREZ safely. FINDINGS: Thirty one of 32 patients displayed complete disappearance of spasm following surgery. One patient showed 70% decrease of spasm. Delayed transient facial weakness occurred in one patient. Audiometries showed no postoperative hearing decrease in any patient, even though no intra-operative monitoring of the cochlear function was undertaken. INTERPRETATION: Although this basal approach, the para-condylar fossa approach, is a slightly basal modification of the conventional procedure, it may minimize complications.
Interstitial deletion of 7q11.23-q21.11 was identified by cytogenetic methods in a 4-year-old boy with Williams syndrome (WS) and infantile spasms. Deletion of the elastin (ELN) gene and the DNA polymorphic markers, D7S1870, D7S2490, D7S2518, and D7S2421, were identified in the patient, but the loci for D7S653 and D7S675 were not involved. Zackowski et al. (1990) reported that 6 of 16 patients with the interstitial deletion of 7q11.2-q22 had abnormal electro encephalograms, or seizures, or both, and that infantile spasms were present in 2 of the 6 patients. WS is a well defined developmental disorder characterized by distinct facial features, gregarious personality, and congenital heart defects. Seizures are not generally associated with this syndrome. WS commonly is characterized by deletion of the loci for ELN and D7S1870, but not those for D7S2490, D7S2518, or D7S2421. This suggests that a gene responsible for infantile spasms is located in the 2.7-cM interval between loci D7S1870 and D7S675.
In most congested, dilated cardiomyopathies, pathologic analysis reveals focal myocardial necrosis, microscopic scarring, interstitial fibrosis, and myocellular hypertrophy. The focal nature of these processes indicates that the cardiomyopathies may not be due to diffuse abnormalities of cardiac muscle cells, at least in the early stages, but that the changes may represent focal events affecting discrete, small volumes of tissue. Experimental evidence of microvascular hyperreactivity (spasm), both in the hereditary cardiomyopathy of the Syrian hamster and in the acquired cardiomyopathy of the hypertensive-diabetic rat, suggests that transient spasm of the myocardial microcirculation may lead to focal myocellular necrosis and scarring, which then causes the unaffected myocardial cells to undergo compensatory hypertrophy in order to normalize the increased load per unit of myocardium. When enough myocardium is lost and the compensatory hypertrophy results in decreased contractility, congestive heart failure ensues as a systemic response to altered ventricular function. The efficacy of verapamil therapy in the Syrian hamster in preventing microvascular spasm, cell necrosis, and the subsequent development of a cardiomyopathy has provided hope that similar preventive treatment can be applied to patients with early stages of cardiomyopathy.
Myocardial infarction and sudden cardiac death may be initiated by a sudden intense localized contraction of coronary artery smooth muscle. When this event occurs around a vulnerable eccentric lipid-filled plaque, rupture and extrusion of plaque contents and exposure of collagen occur. This may sometimes be a silent and self-limiting event; other times it leads to thrombus formation. A second wave of spasm due to accumulated platelet and inflammatory mediators may compound the contractile consequences of the initiating event. Spasm involves intrinsic smooth muscle cell electrical mechanisms, hyper-responsive cells, and multiple agonists that synergize their actions, and the involvement of each mechanism varies at different times in the sequence of vascular occlusion. Study of spasm requires vascular systems that adequately model coronary artery responses of the ageing human heart. As previously emphasized, tissues obtained postmortem, and when possible from recipients during heart transplants, must be integral to theory building, alongside animal models, despite the experimental limitations such tissues impose. A multidisciplinary approach, at all levels of vascular physiology and pharmacology, will be necessary to understand coronary motor activity and human heart disease.