计算机辅助设计和快速成型技术制造颜面萎缩衬垫物的可能性

背景：半侧颜面萎缩畸形的整复方法较多,以往常通过制备患者的石膏面模,在上面堆蜡来恢复患者的面形,蜡型用作手术中衬垫的参考。但由于畸形的变化多,矫治设计的难度大,矫治效果不甚理想。 目的：探讨应用计算机辅助设计和快速成型技术制造用于矫正半侧颜面萎缩等凹陷畸形衬垫物的可能性。 方法：对半侧颜面萎缩患者行螺旋CT扫描,利用工作站进行扫描图像的容积三维重建后,重新间隔分层,利用CuteFTP 4.0软件以BMP格式下载。应用课题组自主开发的CT图像处理软件对已下载的二维图像进行过滤、筛减、降噪、校正失真等处理,对图像的边缘轮廓进行提取,得到面颅骨皮质骨边缘轮廓的矢量化线图,将该线图数据输入Surfacer 9.0重建软件,对轮廓曲线进行矢量叠加,从而得到面颅骨的三维三角形面片线框模型及实体模型。将健侧面颅骨的点云数据按镜像对称变换到患侧,这样在患侧骨和健侧镜像之间就形成了充填物的三维模型,为补偿软组织的萎缩,将其外表面点云数据外移1.5 mm。对CAD后的三维Surfacer数据重新分层,在RpDataRepare中完成充填物的轮廓编辑和成型的支撑设置,形成RP项目文件,输出快速原型所需的加工文件.par,制造出衬垫物模板,作为实施手术过程中的参照。 结果与结论：获得了患者颅面骨表面轮廓的三维实体模型,并由计算机辅助设计,快速成型制造出衬垫物模板,并以此为参照完成手术,效果满意。说明应用快速成型技术可以完成半侧颜面萎缩等凹陷畸形的衬垫物的制造,精度高,快捷,在颅颌面外科假体的个体化制造方面有良好的应用前景。     

A case of progressive hemifacial atrophy with Pourfour de Petit syndrome which was successfully treated by stellate ganglion block]

We herein report a 31-year-old woman with progressive hemifacial atrophy. The atrophy at her left face began about ten years ago. She had been in a traffic accident one year before the onset of her facial atrophy. Neurological examination revealed anisocoria (right < left) and retraction of the left eyelid, which thus suggested the presence of Pourfour de Petit syndrome. The pupillary reaction to both cocaine and tyramine were reduced bilaterally. Thermography of the face showed slightly lower surface temperature on the left side. A facial thermal sweat test was normal. These findings indicated local hyperactivity of the sympathetic nervous system at the Th 1-Th2 levels on the left side. A left stellate ganglion block effectively induced an accumulation of the subcutaneus tissue of her face on the left side. This is a very rare case in which local sympathetic hyperactivity is present and has caused progressive hemifacial atrophy.     

Central motor pathways in patients with mirror movements.

Central motor pathways were investigated in three patients with congenital mirror movements using magnetic motor cortex stimulation. Response thresholds, amplitudes and latencies were normal. The projection of the corticomotoneuronal pathways was assessed by placing the coil over the vertex and comparing the size of responses in the first dorsal interosseous (FDI) muscles evoked by anticlockwise and clockwise [corrected] coil currents. In normal subjects, right FDI responses are larger with anticlockwise currents than with clockwise [corrected] currents at the same stimulation strength and vice versa. In two out of three patients with congenital mirror movements, this sensitivity of response amplitude to coil current direction was reversed. The third patient with congenital mirror movements and a fourth patient with acquired mirror movements had responses which were normally sensitive to current direction. These findings support the hypothesis that some cases of congenital mirror movements may be due to abnormal projection of corticomotoneuronal pathways.     

Therapeutic benefit of repetitive transcranial magnetic stimulation for severe mirror movements A case report

Congenital mirror movements retard typical hand functions,but no definite therapeutic modality is available to treat such movements.We report an 8-year-old boy with severe mirror movements of both hands.His mirror movements were assessed using the Woods and Teuber grading scale and his fine motor skills were also evaluated by the Purdue Pegboard Test.A 2-week regimen of repetitive transcranial magnetic stimulation produced markedly diminished mirror movement symptoms and increased the fine motor skills of both hands.Two weeks after the completion of the regimen,mirror movement grades had improved from grade 4 to 1 in both hands and the Purdue Pegboard Test results of the right and left hands also improved from 12 to 14 or 13.These improvements were maintained for 1 month after the 2-week repetitive transcranial magnetic stimulation regimen.After 18 months,the mirror movement grade was maintained and the Purdue Pegboard test score had improved to 15 for the right hand while the left hand score was maintained at 13.This occurred without any additional repetitive transcranial magnetic stimulation or other treatment.These findings suggest that repetitive transcranial magnetic stimulation for this patient had a therapeutic and long-term effect on mirror movements.     

Magnetic stimulation study in mirror movements

Summary A young man with congenital mirror movements was studied by non-invasive magnetic stimulation. Radiological examination showed no craniocervical or pituitary abnormality except for mild atrophy of the right hippocampus. Magnetic stimulation of the motor cortex caused large amplitudes in the ipsilateral hand muscles, indicating the possibility of functional disorder in the motor pathways from the motor cortex to muscles in the upper extremities.     

Mirror movements following cortical resection of polymicrogyria in a child with intractable epilepsy

Mirror movements may be congenital or acquired. There are few reports of acquired mirror movements in pediatric patients. Further, mirror movements in children with epilepsy have rarely been reported. A 9-year old male, with intractable partial epilepsy resulting from polymicrogyria of the right hemisphere, underwent cortical resection of the right frontotemporoparietal region for a malformation of cortical development. He developed left hemiplegia and mirror movements in the left hand in the postoperative period. Four months after surgery, he remained seizure-free with mild residual left-sided hemiplegia and persistent mirror movements. Mechanisms postulated for mirror movements include aberrant pyramidal tract development and transcallosal inhibitory pathways. The latter mechanism might have contributed to the mirror movements observed in this child. This study is the first report of mirror movements following focal cortical resection for intractable epilepsy due to polymicrogyria.     

Hemifacial spasm in sleep

We conducted polygraphic studies during wakefulness and all-night sleep in 13 patients with cryptogenic and 3 with postparalytic hemifacial spasm. The movements decreased progressively with deepening sleep stages, reaching lowest values in REM sleep. The reduction was inversely related to the severity of movements during wakefulness. There was no relation between hemifacial spasm and mimic activity on the unaffected side. Central inhibitory processes may account for the partial decline in intensity of the movements in sleep.     

Bilateral mirror writing movements (mirror dystonia) in a patient with writer's cramp: functional correlates.

A recent prospective analysis on writer's cramp showed that up to 44.6% of patients in a series of 65 presented mirror dystonia, defined as involuntary movements of the resting hand, abnormal posture, tremor, and jerks occurring while writing with the opposite hand. A clinical case is presented, with videotape evidence of right-handed writer's cramp, with mirror movements elicited while writing using either hand. Functional magnetic resonance imaging studies are compared both to those of a normal patient and to those from a patient with writer's cramp but lacking mirror dystonia. Widespread bilateral activation of cortical motor areas contralateral to the mirror movements in patients with writer's cramp and mirror movements suggests, that bilateral activation of the primary motor cortex may account for the appearance of these mirror movements. Further studies need to be conducted to determine whether mirror movements in dystonic patients appear as a result of loss of intra- and/or interhemispheric cortical inhibition or are simply a consequence of the sustained effort these patients need to exert while writing using a dystonic hand.     

Mirror movements after childhood hemiparesis

Mirror movements are normal in childhood and may persist to a later age following early brain lesions. We studied these movements in patients with childhood hemiparesis at different ages. The earlier the lesions, the more the mirror movements persisted. More mirror movement persisted in the nonparetic hand than in the paretic one. Complete paralysis of either hand tended to abolish all mirror movements in both hands. The task eliciting the most mirror movement was one that may come under ipsilateral control following contralateral damage. The greater persistence of mirror movements after earlier lesions appears to be an indicator of more extensive compensatory motor system reorganization that takes place after damage to a less mature nervous system.     

A case of overlapping adult-onset linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic involvement

Linear scleroderma is a variant of localized scleroderma. We report a 43-year-old woman who had developed left arm weakness and linear scleroderma on her back during pregnancy at 25 years of age, followed by left hemifacial atrophy and left leg weakness. She had multiple linear scleroderma lesions on her trunk and left limbs, left eyelid ptosis, impairment of vertical movement and abduction of the left eye, left hemifacial atrophy, and weakness and atrophy of the sternocleidomastoid, trapezius, and proximal limb muscles on the left side. On serology, antibodies to U1-ribonucleoprotein and Jo-1 were positive; anti-scleroderma-70 antibody was negative. Skin biopsy demonstrated increased hypertrophic collagen fibers without inflammatory infiltrates. Needle electromyography of left limb muscles revealed mild neurogenic patterns; left quadriceps muscle biopsy showed chronic neurogenic changes. Brain magnetic resonance imaging revealed mild left hemispheric atrophy. This is a rare case of linear scleroderma and Parry–Romberg syndrome presenting with widespread ipsilateral neurogenic manifestations.     

Bilateral primary sensori-motor cortex activation of post-stroke mirror movements: an fMRI study

This fMRI study was undertaken to test whether the pathophysiological mechanism of mirror movements in hemiparetic stroke patients involves activation of the unaffected motor cortex. We studied 16 control subjects and 51 stroke patients. fMRI was performed at 1.5 T using a finger flexion-extension movement paradigm. The incidence of bilateral primary sensorimotor cortex activation was significantly increased during movements of the affected hand of stroke patients who showed mirror movements. Moreover, the incidence of bilateral primary sensorimotor cortex activation increased with the severity of mirror movements and primary sensorimotor cortex was activated bilaterally in all patients who showed sustained mirror movements. We conclude that the motor cortex activation on the non-stroke side is associated with mirror movements and is correlated with the severity of mirror movements. It seems that the pathophysiological mechanism of sustained mirror movements in stroke patients involves the unaffected motor cortex.     

Progressive hemifacial atrophy with sympathetic nerve dysfunction of central origin

A 37-year-old unmarried man was admitted because of gait disturbance and right hemifacial atrophy. Family history was unremarkable. He had an unconscious attack at age 13 and had writer's cramp since age 15. He was thin and lipodystrophic. In reviewing his portraits, hemifacial atrophy was considered to develop in his early teens and to be progressive since then. Pigmented gum, high arched palate, mild mental retardation, pseudo-Argyll Robertson's pupil, sexual impotence, amyotrophy of the left thigh and the right calf, and a limp due to bony abnormalities was detected. Serological tests for syphilis were negative. Bone X-rays disclosed coxa-deformance. Cerebrospinal fluid. EMG, EEG, muscle biopsy and brain CT were normal. Hearing was decreased to 20-35 dB bilaterally. Plasma norepinephrine levels were 450 pg/ml in the supine position and 539 pg/ml in standing. Plasma renin activity was 5.1-5.4 ng/ml/hr. Microneurography revealed highly accentuated muscle and skin sympathetic nerve activities. Hypothermia on the feet, reduced CVR-R and decreased mydriatic response to 5% cocaine instillation were present. Intravenous infusion of norepinephrine and intradermal injection of either acetylcholine or histamine revealed normal results. In the case, sympathicotonia due to dysfunction in the central nervous system is considered to be related to the pathogenesis of hemifacial atrophy.     

Neurological manifestations of Ehlers-Danlos Syndrome

Ehlers-Danlos Syndrome (EDS) is more identified for its cutaneous features but its neurological manifestations have not received the focused attention. Four patients of Ehlers-Danlos Syndrome (EDS) with neurological manifestations were evaluated for phenotypic data. These four men were from three families and two had consanguineous parentage. The mean age at onset and presentation of neurological symptoms were 10.5 years and 19 years respectively. Patient 1 presented with bilateral optic atrophy, sensorineural deafness, cerebellar ataxia and neuropathy. Patient 2 had marfanoid habitus, chorea and cerebellar ataxia. Patient 3 had action and percussion myotonia, wasting and weakness of sternocleidomastoid and distal limb muscles. Patient 4 had action myotonia, mirror movements of both hands and neuropathy. MRI of brain showed right parietal polymicrogyria. Neuroaxis involvement at multiple levels in EDS may have prognostic significance.     

Chronic isolated hemifacial spasm as a manifestation of epilepsia partialis continua

The objective of this case study was to describe the clinical and electroencephalography (EEG)/functional magnetic resonance imaging (fMRI) data of a case of isolated hemifacial spasm due to epilepsia partialis continua in a 59-year-old man with abnormal hemifacial movements that disappeared during voluntary tasks, were absent during sleep, and responded to carbamazepine. His neurological examination was normal; EEG revealed right inferior frontal epileptiform discharges. EEG/fMRI demonstrated increased blood oxygenation level-dependent contrast in the right inferior and middle frontal gyri corresponding to the contralateral motor and premotor cortex responsible for facial movements (BA 44, 45, 45, 9), with widespread BOLD signal deactivations suggestive of epileptic network involvement despite a very focal epileptogenic process. We hypothesize that the response of some cases of hemifacial spasm to carbamazepine, a first-line treatment in the pre-botulinum toxin era, may have been due to its antiepileptic effects, rather than to modulation of facial nerve hyperexcitability.     

Multiple sclerosis and canine pets

Spontaneous and associated hyperkinetic facial movements and contracture which follow injury to the seventh cranial nerve (postparalytic hemifacial spasm) or arise without known previous injury (cryptogenic hemifacial spasm) are pathological motor phenomena not found in the distribution of other cranial or somatic motor nerves. The commonly expressed hypotheses of pathogenesis—aberrant regeneration and fiber excitation by false synapse formation (ephapses) at the site of injury—cannot account for all aspects of these phenomena or for the uniqueness of such movements to the distribution of the seventh nerve. The suggestion is made that the existing diversity of facial motor behavior, which encompasses voluntary, emotional, and especially automatic, associated, and reflexive movements, is based on a unique central organization that sets it apart from other motor groups. I hypothesize that because of this organization, the changes following axonal injury—which include selective deafferentation, glial response, axonal sprouting, functional reconnection, and hyperexcitability from dendritic spike generation—can unmask and augment automatic, associated, and reflexive movements already present in the facial neuronal network to result in facial hyperkinesia.     

The many faces of hemifacial spasm: Differential diagnosis of unilateral facial spasms

Hemifacial spasm is defined as unilateral, involuntary, irregular clonic or tonic movement of muscles innervated by the seventh cranial nerve. Most frequently attributed to vascular loop compression at the root exit zone of the facial nerve, there are many other etiologies of unilateral facial movements that must be considered in the differential diagnosis of hemifacial spasm. The primary purpose of this review is to draw attention to the marked heterogeneity of unilateral facial spasms and to focus on clinical characteristics of mimickers of hemifacial spasm and on atypical presentations of nonvascular cases. In addition to a comprehensive review of the literature on hemifacial spasm, medical records and videos of consecutive patients referred to the Movement Disorders Clinic at Baylor College of Medicine for hemifacial spasm between 2000 and 2010 were reviewed, and videos of illustrative cases were edited. Among 215 patients referred for evaluation of hemifacial spasm, 133 (62%) were classified as primary or idiopathic hemifacial spasm (presumably caused by vascular compression of the ipsilateral facial nerve), and 4 (2%) had hereditary hemifacial spasm. Secondary causes were found in 40 patients (19%) and included Bell's palsy (n = 23, 11%), facial nerve injury (n = 13, 6%), demyelination (n = 2), and brain vascular insults (n = 2). There were an additional 38 patients (18%) with hemifacial spasm mimickers classified as psychogenic, tics, dystonia, myoclonus, and hemimasticatory spasm. We concluded that although most cases of hemifacial spasm are idiopathic and probably caused by vascular compression of the facial nerve, other etiologies should be considered in the differential diagnosis, particularly if there are atypical features. © 2011 Movement Disorder Society     

Hemifacial motor and crying seizures of temporal lobe onset: case report and review of electro-clinical localisation

OBJECTIVE: To report a case of temporal lobe epilepsy with clinical presentation of paroxysmal episodes of "tightness" over the right hemiface, and ictal crying, and review electroclinical localisation of this phenomenon. METHODS: Clinical semiology, neurophysiological localising tests, and epilepsy surgery outcome are reported in a subject presenting with paroxysmal right hemifacial movements and ictal crying. Pertinent past reports of somato-motor signs and ictal crying in temporal lobe epilepsy are reviewed and the findings correlated with proposed human facial cortical representation. RESULTS: Simple partial seizures caused by temporal lobe epilepsy presented with right sided tonic facial movements and ictal crying. Intracranial EEG monitoring documented a left medial temporal onset of seizures that remained asymptomatic until they propagated to the left cingulate region. Anterior temporal lobectomy with resection of the amygdala and anterior hippocampus resulted in cessation of seizures. CONCLUSIONS: This is a rare example of epileptic seizures of medial temporal onset presenting with isolated somato-motor manifestations and ictal crying. Anatomical-electrical-clinical correlations with cortical regions controlling facial movements were highly suggestive that this case represents secondary activation of "emotional" motor cortex M3 and M4 (rostral and caudal cingulate motor cortex), giving rise to focal hemifacial movements and ictal crying.     

Functional MR imaging of hand motor cortex in a case of persistent mirror movement

Persistent mirror movements are unwanted movements restricted to muscles homologous to those moved intentionally on the opposite body half. It is rarely observed and the functional MRI findings in a case of persistent mirror movement are described.     

Mirror movements in healthy humans across the lifespan: effects of development and ageing

Aim Mirror movements are a transient phenomenon during childhood, which decrease in intensity with motor development. An increasing inhibitory competence resulting in the ability of movement lateralization is thought to be the underlying mechanism. We aimed to quantify unintended mirror movements systematically across the lifespan and to investigate the influences of age, sex, handedness, and task frequency. Method A total of 236 participants (127 females, 109 males; 216 right‐handed, 20 left‐handed; age range 3–96y, median 25y 8mo) first performed four clinical routine tests while mirror movements were rated by the observer. They were then asked to hold a force transducer in each hand between the thumb and index finger and to perform oscillatory grip force changes in one hand, while the other hand had to prevent the force transducer from dropping. Results Age showed a strong nonlinear effect on the mirror‐movement ratio (the amplitude ratio of the mirror and active hand, adjusted by the respective maximum grip force). Initially, there was a steep decline in the mirror‐movement ratio during childhood and adolescence, followed by a gradual rise during adulthood. Males had lower mirror‐movement ratios than females. The high‐frequency condition triggered lower mirror‐movement ratios. No significant differences of mirror movements between dominant and non‐dominant hand, or left‐ and right‐handed participants, were found. Interpretation This study provides, for the first time to our knowledge, normative values of mirror movements across the lifespan that can aid differentiation between physiological and pathological mirror movements.     

Stronger reactivity of the human primary motor cortex during observation of live rather than video motor acts.

The monkey premotor cortex contains neurons that are activated both when the monkey performs motor acts and when he observes actions made by others. A similar mirror neuron system, involving several brain areas, has been found in humans. We recorded neuromagnetic oscillatory activity from the primary motor cortex of 10 healthy subjects when they observed live and videotaped finger movements. The left and right median nerves were stimulated alternatingly and the poststimulus level of the approximately 20 Hz rhythm was quantified. Compared with the rest condition, the approximately 20 Hz rhythm was dampened 15-19% more when the subjects observed live rather than videotaped hand movements, indicating stronger activation of the primary motor cortex. These results suggest that the human mirror neuron system differentiates natural and artificially presented movements.