Electrical stimulation of the brain in Wilson-Konovalov hepatocerebral dystrophy (a neuromorphological and neurophysiological analysis).

Stereotaxic surgery was performed in 27 patients. Complete elimination of or significant reductions in hyperkinesia were obtained in 17 cases; five patients died. There was no correlation between the severity of clinical manifestations of hepatocellular dystrophy and the relatively normal quantitative measures of cortical and subcortical biopotentials, which were produced on a background of microstructural changes affecting neurons in these regions. It is suggested that qualitative significance of these biopotentials is that they carry an excess pathological spike activity resulting in hyperkinesia. This is supported by the fact that hyperkinesia was suppressed after surgical destruction of the ventrolateral nucleus of the thalamus and subthalamic structures.

[Electric stimulation of the brain in patients with hepatocerebral Wilson-Konovalov's degeneration (neuromorphological and neurophysiological analysis)]. / Elektricheskaia stimuliatsiia mozga pri gepatotserebral'noi distrofii Vil'sona-Konovalova (neiromorfologicheskii i neirofiziologicheskii analiz).

36 stereotactic operations were carried out in 27 patients. Either the total lack of hyperkinesis or its significant decrease was found in 17 cases; 5 patients died. There was no correlation between manifestations of hepatocerebral degeneration and relatively normal quantitative characteristics of both cortical and subcortical biopotentials. These potentials are produced on the background of the microstructural changes in the neurons of the same regions. It is suggested that qualitative importance of such biopotentials lies in their exceeding pathologic impulsation, arising hyperkinesis. That was confirmed by the fact, that hyperkinesis eliminates after surgical destruction of the ventrolateral nucleus of the thalamus and the subthalamic structures.

[Hyperactivity of the muscles of the lip and chin and indications for surgery to reduce it]. / Dysfonctionnement des muscles labio-mentonniers et indications de la chirurgie d'affaiblissement.

Hyperactivity of the muscles controlling the lower lip and chin (muscles of the chin, buccinator, orbicularis, etc.) can be demonstrated at different levels of the muscular complex and may act as an elastic force against the mandibular alveolar process. Dysfunction in this region may lead to reduction of the alveolar bone quantity, reducing the tooth-bone equilibrium. If this balance is disturbed, periodontal lesions, a lower incisal crowding and a retrognathic mandibular process may result. Surgery to correct overactivity of the chin muscles is described here step by step. Resection of the muscles may be undertaken on a greater or lesser scale and striation of the muscle may also allow reduction of muscular strength. Reducing the muscular activity creates a better environment for the development of the mandible and its alveolar process. Depending on the clinical situation, these techniques can be associated with genioplasty, bone graft and/or mandibular orthognathic surgery.

Functional stereotactic surgery for hemiballism.

The symptomatic and functional outcomes of a series of 14 patients with disabling and medically refractory hemiballism who were treated with functional stereotactic surgery are reported. Seven (50%) of the 14 patients had concomitant hemichorea. To relieve the hyperkinesia, the 14 patients underwent a total of 15 stereotactic operations (one patient had a second stereotactic procedure). Combined lesions in the contralateral zona incerta and the base of the ventrolateral (oroventral) thalamus were applied in 13 instances. The zona incerta was reached by means of a movable chord electrode to obviate the need for repeated puncture. In two instances the medial pallidum was used as the stereotactic target. Hemiballism was abolished or considerably improved in 13 (93%) of 14 patients in the immediate postoperative phase. Residual dyskinesia was evaluated using the hemiballism/hemichorea outcome rating scale. Long-term follow-up review was available for 13 of the 14 patients (mean follow-up period 11 years). Persistent improvement in the hemiballism was found in 12 of these 13 patients: seven patients (54%) were free of any hyperkinesia and five patients (39%) had minor residual and predominantly hemichoreic hyperkinesia. One of the 13 patients presented with a probable psychogenic movement disorder at long-term follow-up examination. Persistent morbidity, most likely related to the operative intervention, was detected in three of the 13 patients; this included mild hemiparesis and dystonia. Functional disability was assessed using the Huntington's Disease Activities of Daily Living scale. The patients' preoperative mean value of 83% of maximum disability was reduced to a mean of 30% observed at long-term follow-up review (p < 0.001). The residual disability exhibited in most older patients was associated with cardiovascular disease. The authors compare their findings with the results of 44 cases reported previously. The authors contend that functional stereotactic surgery should be considered in patients with persistent, medically refractory hemiballism.

Mimetic modulation for problem creases of the face.

Problem creases of the face such as frontal lines and frown lines, crow's feet, deep nasolabial and perioral folds, and cervical bands may be caused by the aging process, excessive exposure to the sun, disease, or genetic disposition. The condition may become aggravated by habitual hyperkinesia of certain mimetic muscles like the frontalis, corrugators, orbicularis oculi, levatores labii superioris, zygomatici, and the platysma. The diagnosis is established clinically by electromyography and selective muscle and nerve blocks. In these cases we advocate regulation of the mimetic hyperkinesia through selective myotomy, myectomy, and neurotomy of the responsible mimetic muscles (mimetic modulation). These procedures may be performed exclusively or in combination with a blepharoplasty, rhytidectomy, or other procedure. Our experience with 60 patients over the past five years (medium = 3.5 years) is presented. Problems and complications such as paresthesia and hypesthesia, partial paresis and asymmetry, incomplete correction, and recurrences are discussed. We believe that mimetic modulation is a valuable concept in treating problem creases and thereby improves the results of the aesthetic surgery of the face.

[The surgical treatment of muscle tonus disorders by the destruction of the input areas of the posterior spinal nerve roots]. / Khirurgicheskoe lechenie narushenii myshechnogo tonusa destruktsiei vkhodnykh zon zadnikh koreshkov spinnogo mozga.

Stereotactic cryodestruction of the subcortical ganglions for microdestruction of the inlets of the radices spinales was conducted in resistant to drug and surgical treatment patients with impaired muscular tension and spastic pain syndrome (infantile cerebral paralysis, dystonia musculorum deformans, postencephalitic spastic-hyperkinetic syndrome). A total of 8 patients aged 19-46 were treated. The destruction of the segments depended on the site of the tension impairment. In cervical, upper extremity, lumbar, lower extremity involvement the segments to be destroyed were, respectively: SV--SVII, SVIII, LII--LIII, LIV, LV and SI. The surgery aimed at breaking the centripetal nociceptive and myotactic tracts. The treatment reduced muscular hypertonicity and hyperkinesis, relieved spastic pain. The paper presents anatomic and neurophysiological grounds for the procedure application.

[Tremulous hyperkinesia in the parkinsonism syndrome and its electromyographic characteristics]. / Drozhatel'nyi giperkinez v sindrome parkinsonizma i ego élektromiograficheskaia kharakteristika.

Basing on the results of global electromyography performed for 51 patients with parkinsonism, it is concluded that volley muscular activity may be related with neurophysiological segmentary reflexes: discharge of alpha-motoneurons from primary endings of the muscle spindle; autogenic, reciprocal and feedback inhibition of alpha-motoneurons.

[The biopsy of brain structures in patients with extrapyramidal hyperkinesia]. / Biopsiia mozgovykh struktur u bol'nykh s ékstrapiramidnymi giperkinezami.

A new device for brain biopsy is presented, the techniques and method of biopsy of brain structures are described which allow full value biopsy material to be obtained from any area of the brain with minimal risk for the patient and high accuracy of hitting the aim. Biopsy of the cerebral and cerebellar cortex, and thalamic and cerebellar nuclei was conducted in 82 patients during stereotaxic operations (34 patients with infantile cerebral paralysis, 8 with torsion dystonia, 8 with spasmodic torticollis). Stereotaxic biopsy of brain tumors of various localization was also performed in 20 patients.

[The electromyographic analysis of trembling hyperkinesis in Wilson-Konovalov's hepatocerebral dystrophy]. / Elektromiograficheskii analiz drozhatel'nogo giperkineza pri gepatotserebal'noi distrofii Vil'sona--Konovalova.

Global electromyography was applied in the examination of 20 patients with Wilson-Konovalov's hepatocerebral dystrophy before and after stereotaxic operations. The origin of volley muscular activity is explained on the basis of segmental reflexes known in neurophysiology: indirect relief of alpha-motoneurons from the primary terminals of the spindle, autogenous, recurrent and reciprocal inhibition of alpha-motoneurons.

[Stereotaxic surgery in infantile cerebral palsy]. / Stereotaksicheskie operatsii pri detskom tserebal'nom paraliche.

The article analyses the results of 439 stereotaxic operations performed on 326 patients with the spastic-hyperkinetic form of infantile cerebral paralysis (ICP). Differentiated destructions of the brain structures were conducted. Extended thalamotomy, sagittal thalamotomy, and combined (cross) thalamodentatotomy were the most frequent complexes of destructions. Essential diminution of hyperkineses and hypertonia was noted in 86 and 81% of patients, respectively, in the immediate postoperative periods; the condition of 70% of patients improved in the late-term periods as compared to that in the preoperative period. Combined (cross) thalamodentatomy was found to be the most effective operative intervention.

Bell's palsy: management of sequelae using EMG rehabilitation, botulinum toxin, and surgery.

Fifteen percent of patients who have had an acute episode of Bell's palsy will be left with debilitating facial dysfunction. This chapter describes our approach to managing a variety of hypo- and hyperkinetic disorders caused by injury and faulty regeneration of the facial nerve, using electromyographic rehabilitation (EMGR) (13 patients), Oculinum toxin injection (14 patients), or surgical reanimation (72 patients). Improvement was noted after EMGR in 12 of 13 patients (92%), all 14 patients treated with Oculinum experienced temporary improvement, and improvement was noted in 66 of 72 patients who underwent surgery (92%). The indications, techniques, and results of these three rehabilitative methods are discussed.

[Ballism syndromes treated by cryothalamotomy]. / Zespoly balizmu leczone kriotalamotomia.

Two cases of ballism of different aetiology and clinical manifestations are reported. One of them was a typical case of hemiballism with exceptionally violent hyperkineses leading to injuries to extremities. The syndrome developed after stroke. Cryothalamotomy caused regression of violent hyperkineses and made possible resuming of independent life. In another case, less certain with respect to aetiology, the patient had bilateral ballism with preponderance of the right side and with falling. Unilateral (left sided cryothalamotomy caused a significant improvement of contralateral hyperkineses and even of ipsilateral hyperkineses so that the patient could walk and eat. In most cases ballism requires surgical treatment with thalamotomy and in case of hemiballism this treatment makes possible complete regression of violent hyperkineses.

[Somatosensory evoked potentials before and after stereotaxic operations in patients with childhood cerebral palsy]. / Somatosensornye vyzvannye potentsialy do i posle stereotaksicheskikh operatisii u bol'nykh s detskim tserebral'nym paralichom.

The somatosensory function in patients with the hyperkinetic form of infantile cerebral paralysis (ICP) was evaluated by means of short-latent somatosensory evoked potentials (SSEP) in the preoperative period and after stereotaxic operations on the thalamic nuclei and structures of the subthalamic region; the evaluation was conducted by comparison with the dynamics of the main manifestations of the disease. The SSEP characteristics in patients in the preoperative period bear evidence of bilateral disorders in the somatosensory system. Stereotaxic operations, leading to certain normalization of the muscular tone and inhibition of hyperkinesis, do not cause positive changes of the SSEP characteristics. The peculiarities of the SSEP characteristics in the preoperative period in patients with ICP may serve as a definite prognostic sign of the efficacy of stereotaxic operations in these patients.

Primary and revision surgery (selective neurectomy) for facial hyperkinesia.

Symptomatic relief for hyperkinetic movements of facial musculature can be obtained by selective neurectomy of the peripheral divisions of the facial nerve with preservation of the frontal branch. Hemifacial spasm and blepharospasm represent the most common forms of facial hyperkinesia. Satisfactory results using the described technique on 191 patients were achieved in 74% of the patients with hemifacial spasm and blepharospasm in the follow-up period of one to 12 years. In hemifacial spasm, 80% were relieved after a single operation, and 95% were relieved after revision surgery. There were no major postoperative complications. Minor complications included insufficient lid closures in 1.5% of the patients and delayed wound healing with minor salivary fistulas that closed spontaneously in 7% of the patients. Resection of all fibers innervating the orbicularis oculi muscle is essential in preventing recurrence.

[Musculotendinous alloplasty in patients with infantile cerebral palsy]. / Sukhozhil'no-myshechnaia alloplastika u bol'nykh detskim tserebral'nymparalichom.

A special alloplastic material was used in 20 patients aged from 7 to 16 for substituting or making up a deficient function of muscles, ligaments and tendons after preliminary elimination of all fixed contractures and deformities. A conclusion is made of the application of the special alloplastic material not only for patients with infantile cerebral paralysis but also in other aspects of surgery especially for children.