Progressive Encephalomyelitis with Rigidity and Myoclonus Resolving after Thymectomy with Subsequent Anasarca: An Autopsy Case.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is an autoimmune disorder involving the brainstem and spinal cord and is sometimes associated with thymoma. We encountered a 75-year-old woman with typical PERM features, glycine receptor antibody, and thymoma. Her neurologic symptoms improved after thymectomy, but she unexpectedly developed anasarca with massive pleural effusions and hypoalbuminemia and finally succumbed to death. The autopsy showed edema and mononuclear infiltration in the pleura but no neuropathological findings typical of PERM. Effective treatment of PERM can reverse the neuropathological signs of encephalomyelitis. The autoimmune nature of anasarca is possible but not proven.

Magnetic Resonance Imaging-guided Focused Ultrasound Thalamotomy for Parkinson's Disease.

Thalamotomy is effective in treating refractory tremor in Parkinson's disease (PD). We herein report a PD patient who underwent left ventral intermediate nucleus and ventro oralis posterior nucleus thalamotomy using magnetic resonance imaging-guided focused ultrasound (MRgFUS). Right-side resting tremor and rigidity were abolished immediately following the ultrasound energy delivery. In addition, left-side resting tremor and rigidity also improved. No adverse events occurred during the procedure. We observed the exacerbation of bradykinesia, which might have been caused by edema around the target. This is the first report of thalamotomy using MRgFUS for PD patient from Japan. Further investigations concerning the efficacy and safety of this procedure are necessary.

Judet quadricepsplasty in the treatment of posttraumatic knee rigidity: long-term outcomes of 45 cases.

BACKGROUND: Posttraumatic knee stiffness is a very debilitating condition. Judet's quadricepsplasty technique has been used for more than 50 years. However, few reports of quadricepsplasty results exist in the literature. METHODS: We report the results of 45 cases of posttraumatic arthrofibrosis of the knee treated with Judet's quadricepsplasty. The results of the procedure were analyzed by measuring the degrees of flexion of the operated knees at different time points (before, immediately after, and late postoperatively). RESULTS: The degree of flexion increased from 33.6 degrees (range, 5­80 degrees) preoperatively to 105 degrees (range, 45­160 degrees)immediately after surgery, followed by a slight fall in the range of motion (ROM) in the late postoperative period, which reached an average of 84.8 degrees. There was no significant correlation between knee strength and the patient's gender, but there was a slight trend of lower strength with age. Although Judet's quadricepsplasty technique dates from more than 50 years ago, it still provides good outcomes in the treatment of rigid knees of various etiologies. In general, all cases showed the same pattern of a small decrease in the ROM in the late postoperative period. CONCLUSION: Judet's quadricepsplasty can increase the ROM of rigid knees. The ROM obtained with the surgery persists long term.

Restoration of opposition.

Opposition is not grasp but a preposition for grasp that involves 3 components of thumb movements: abduction, flexion, and pronation. Thumb opposition is usually lost with paralysis of the thenar muscles innervated by the median nerve. Many opposition transfers have been described that differ in the donor tendon, route of transfer, and method of attachment to the thumb. No one transfer is applicable for every clinical condition, and each transfer has its advantages and disadvantages. Many factors must be evaluated to decide if surgery is likely to be beneficial and then decide on the optimum treatment.

Brain penetration effects of microelectrodes and DBS leads in STN or GPi.

OBJECTIVE: To determine how intraoperative microelectrode recordings (MER) and intraoperative lead placement acutely influence tremor, rigidity, and bradykinesia. Secondarily, to evaluate whether the longevity of the MER and lead placement effects were influenced by target location (subthalamic nucleus (STN) or globus pallidus interna (GPi)). BACKGROUND: Currently most groups who perform deep brain stimulation (DBS) for Parkinson disease (PD) use MER, as well as macrostimulation (test stimulation), to refine DBS lead position. Following MER and/or test stimulation, however, there may be a resultant "collision/implantation" or "microlesion" effect, thought to result from disruption of cells and/or fibres within the penetrated region. These effects have not been carefully quantified. METHODS: 47 consecutive patients with PD undergoing unilateral DBS for PD (STN or GPi DBS) were evaluated. Motor function was measured at six time points with a modified motor Unified Parkinson Disease Rating Scale (UPDRS): (1) preoperatively, (2) immediately after MER, (3) immediately after lead implantation/collision, (4) 4 months following surgery-off medications, on DBS (12 h medication washout), (5) 6 months postoperatively-off medication and off DBS (12 h washout) and (6) 6 months-on medication and off DBS (12 h washout). RESULTS: Significant improvements in motor scores (p<0.05) (tremor, rigidity, bradykinesia) were observed as a result of MER and lead placement. The improvements were similar in magnitude to what was observed at 4 and 6 months post-DBS following programming and medication optimisation. When washed out (medications and DBS) for 12 h, UPDRS motor scores were still improved compared with preoperative testing. There was a larger improvement in STN compared with GPi following MER (p<0.05) and a trend for significance following lead placement (p<0.08) but long term outcome was similar. CONCLUSION: This study demonstrated significant acute intraoperative penetration effects resulting from MER and lead placement/collision in PD. Clinicians rating patients in the operating suite should be aware of these effects, and should consider pre- and post-lead placement rating scales prior to activating DBS. The collision/implantation effects were greater intraoperatively with STN compared with GPi, and with greater disease duration there was a larger effect.

Bilateral subthalamotomy for advanced Parkinson disease.

BACKGROUND: Unilateral subthalamotomy has been reported to be effective in the treatment of rigidity, bradykinesia, and tremor of the contralateral limb. However, gait, clinical fluctuation, and postural stability are not significantly improved by unilateral lesioning of the STN in the long term. We sought to determine if bilateral surgery of the STN offers more benefits in the treatment of advanced PD. METHODS: Radiofrequency thermal coagulation was performed bilaterally in the STN in 10 patients. Under microelectrode and stereotactic guidance, surgery was directed at the dorsolateral portion of the STN in stages and followed by MRI in each patient to confirm lesion location. Patients have been followed for a median duration of 26 months as measured from the date of first surgery (range, 6-48 months) with UPDRS before and after surgery. RESULTS: Bilateral subthalamotomy demonstrated persistent benefits in bradykinesia, rigidity of the limbs, and consequently the improvement in activities of daily living, motor function, Schwab and England scales. In addition, significant improvement in axial motor features, gait, postural stability, and clinical fluctuation were present with bilateral STN surgeries. The benefits were sustained at the last evaluation period of 36 months. Tremor and drug-induced dyskinesia improved in early postoperative period, but the benefits declined over time. The reduction of daily l-dopa equivalent was 34%. No speech impairment was observed. Mild choreic movement occurred in 2 of 20 procedures that resolved spontaneously in 4 to 8 weeks. CONCLUSION: For advanced PD present with bilateral symptoms, axial motor impairment, or clinical fluctuation, staged bilateral subthalamotomy appears as a safe and effective treatment in the long term.

[Severe rigid kyphotic scoliosis treated with posterior spinal osteotomy].

OBJECTIVE: To evaluate the effect of severe rigid kyphotic scoliosis treated with posterior spinal osteotomy METHODS: A total of 11 cases(average 11.2 years) of severe rigid kyphotic scoliosis treated with posterior spinal osteotomy from Mar. 2005 to Sept. 2006 were retrospective. Of those cases, 3 were of neurofibromatosis, 5 of congenital scoliosis, 1 of poliomyelitis and 2 of idiopathic scoliosis. The flexibility of all patients was less than 25%. They had an average follow-up period of 1.3 years (0.5-2 years). RESULTS: There were 3 cases of one segment posterior wedge osteotomy, 2 of two segment posterior wedge osteotomy and 2 of three segment wedge osteotomy. Transpedicular osteotomy was performed in 2 cases, and the last 2 cases encountered vertebral resection and posterior column removed. The average kyphotic angle was 108 degrees (ranging from 87 to 135 degrees) and the average scoliosis angle was 97 degrees (ranging from 65 to 135 degrees) before operation. After operation the average kyphotic angle was corrected to 49 degrees (the correction rate was 55%) and the average scoliosis angle was corrected to 37 degrees(the correction rate was 66%) . The height rose up by an average of 4.7 cm. No neurological complication happened in all the 11 cases. CONCLUSION: It is an effective method that severe rigid kyphotic spinal scoliosis is treated with posterior osteotomy.

Los principios generales de la osteosíntesis / General Principles of Osteosynthesis

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Long-term effects of bilateral subthalamic nucleus stimulation in advanced Parkinson disease: a four year follow-up study.

In this study we aimed to investigate the effects of bilateral STN HFS in patients with advanced Parkinson disease (PD) at long-term, with a minimum follow-up of 4 years. Twenty patients (15 men, five women) were included, with a mean age of 60.9+/-8.1 years. Surgery was performed under local anesthesia. The target was defined on computerized tomography (CT). At 3 months, significant improvements were found on the total Unified Parkinson disease rating scale (UPDRS) III (motor) score, in the medication. off (from 42.3+/-9.3 to 19.5+/-6.4), as well as the medication on (from 18.6+/-12.1 to 10.1+/-5.9) phase. The UPDRS IVa (dyskinesias) and IVb (motor fluctuations) scores decreased significantly. At long-term follow-up, there were still significant improvements on the total UPDRS III motor score (from 42.3+/-9.3 to 24.2+/-13.2), as well as in all motor subscores, in the off phase, during stimulation. In the on phase, the only significant improvement was seen for rigidity. Complications included hypomania to mania in four patients. Our results indicate that HFS STN results in long-lasting improvement of the motor symptoms, ADL activities and functional performance in patients suffering from advanced PD. The stimulation induced behavioural changes need special consideration.

Single-staged double anterior and posterior spinal reconstruction for rigid adult spinal deformity: a report of four cases.

BACKGROUND CONTEXT: Sequential anterior/posterior spinal reconstruction for rigid adult spinal deformity has become a standard operative option. Single-staged double anterior/posterior spinal reconstruction for rigid double major curvature has not been reported in the literature to date. PURPOSE: To report a previously unreported approach for rigid double major curvature of the thoracic and thoracolumbar spine with emphasis on indications and avoiding complications. STUDY DESIGN: Four cases of sequential double anterior/posterior spinal reconstruction are reported. METHODS: Single-staged double anterior spinal reconstruction was performed on four adult patients with rigid thoracic and thoracolumbar scoliosis. Osteotomies were performed by the anterior and posterior approach and followed by posterior instrumentation. A right thoracotomy and left retropleural/retroperitoneal approach was performed for each patient followed by the posterior approach in a single stage. RESULTS: Only one complication occurred, a posterior dural tear, treated without incident. A high level of patient satisfaction and return to activity was noted. Solid arthrodesis with good coronal and sagittal balance occurred in all patients. CONCLUSIONS: Single-staged double anterior/posterior spinal reconstruction for rigid adult deformity can be performed safely and effectively with good patient outcome. The procedure should be reserved only for those patients with severe double major curvature of similar magnitude and rigidity.

Unilateral stereotactic posteroventral globus pallidus internus pallidotomy for Parkinson's disease: surgical techniques and 2-year follow-up.

With the advent of levodopa (L-dopa) and the recognition of its striking effect on Parkinson's disease (PD), virtually all surgical procedures for PD ceased from the mid 1960s. However, there has been a resurgence of pallidotomy and other stereotactic procedures in the last two decades as physicians realized that most PD patients eventually face medical failure after long-term treatment with L-dopa. Nine PD patients, three men and six women, with an average age of 62 years and disease duration of 13 years underwent unilateral globus pallidus internus (GPi) pallidotomy contralateral to the side with marked akinetic symptoms and drug-induced dyskinesia. All patients were evaluated using the Unified Parkinson's disease Rating Scale (UPDRS) after drug withdrawal and while taking their optimal medical regimen, preoperatively and 6, 12, and 24 months after surgery. There was significant improvement in activities of daily living and motor subscores as well as total UPDRS score in the "off" state at the 2-year follow-up, which mainly resulted from improvement in contralateral bradykinesia and rigidity. Significant improvements in contralateral akinetic symptoms and drug-induced dyskinesia were also observed in the "on" state and were sustained for at least 2 years. Ipsilateral and axial symptoms were not altered by unilateral GPi pallidotomy. The complications of surgery were generally well tolerated. One patient had a small postoperative asymptomatic hemorrhage identified by routine follow-up magnetic resonance imaging. Another two patients developed temporary sexual disinhibition and auditory hallucination, respectively, which resolved spontaneously 2 weeks after surgery. The effect of pallidotomy for alleviation of akinetic parkinsonism is modest but significant, and continues to be effective for at least 2 years. Further analytical studies, especially the correlation of clinical effects and lesion locations, are important not only to provide direct feedback for surgeons to examine the technical accuracy and but also to facilitate understanding of the pathophysiology of PD.

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.

Surgery of the motor thalamus: problems with the present nomenclatures.

The literature on thalamic surgery is difficult to read because different nomenclatures are in use. Neurosurgeons mostly use the stereotactic atlas of Schaltenbrand with Hassler's nomenclature of the thalamus. Neuroanatomists use different nomenclatures for the primate thalamus. The cytoarchitectonic definition of nuclei is difficult in the motor thalamus, and it would be best to define the nuclei based on their subcortical afferents. However, tracing studies are not available in humans. Thus, human thalamic nomenclature is based entirely on cytoarchitectonic subdivisions and transfer of knowledge by analogy from monkey to man. Problems arise when trying to transfer the detailed knowledge from monkey to the human brain. By doing so, different authors have come to different conclusions concerning the subcortical afferents of Hassler's motor nuclei, which inevitably leads to confusion when attempting neurophysiological interpretations of the surgical data. The present review draws attention to the discrepancies and open questions in the literature. There is a need to better define the limits of the sensory and cerebellar afferent receiving thalamic nuclei as well as those of the cerebellar and pallidal afferent receiving territories in humans.

Lesions as therapy: rigidity and Parkinson's disease.

Since James Parkinson (1817) first characterized the shaking palsy as a unique condition, significant confusion has remained concerning the causes and treatments of Parkinson's disease (PD). Through the 19th century, a wide variety of approaches were attempted in an effort to reduce its cardinal signs--rigidity, tremor, and bradykinesia--but to little effect. Today, approaching 200 years after Parkinson's seminal work, this disorder is commonly treated by surgical means, inducing a lesion in one specific portion of a small nucleus in the central nervous system (Desaloms et al., 1998, Lang et al., 1999). The notion of providing a lesion to the nervous system as a therapy for PD, however, began in earnest at the beginning of the 20th century. The first attempt to alleviate the symptoms of PD through surgical means involved a section of the dorsal roots of the spinal cord supplying the affected limb (also known as dorsal rhizotomy). Although ultimately resulting in disastrous effects, these early surgical attempts proceeded from a firm body of clinical and experimental research on both the central and peripheral nervous systems. After briefly reviewing the use and failure of dorsal rhizotomy as a treatment for parkinsonian rigidity, this paper will examine the manner in which clinicians and scientists justified the procedure.

[Details and indications of pallidotomy and thalamotomy for Parkinson's disease].

Pallidotomy has recently regained acceptance as an effective treatment for Parkinson's disease. From our 50 cases of unilateral pallidotomy and 10 cases of staged bilateral pallidotomy, details and indications of the procedure is described. The unilateral pallidotomy is quite effective for L-dopa induced dyskinesia, which usually completely disappears soon after the operation. The effect is long-lasting. When on-off phenomenon exists, unilateral pallidotomy improves off-stage rigidity or akinesia. Symptoms during on-stage are not changed. Indications of pallidotomy is that(1) L-dopa induced dyskinesia, and(2) on-off phenomenon. Bilateral pallidotomy, even by staged one, causes severe drooling, or speech disturbance(the volume of the voice decreases and the articulation worsens), and is not recommended. Vim thalamotomy is, on the other hand, the established treatment for tremor of Parkinson's disease or of essential tremor. The effect is long-lasting. Rigidity or akinesia is not expected to be improved so much.

Forty-five years of stereotactic surgery for Parkinson's disease: a review.

An overview of a large experience of the surgical management of parkinsonism from 1954 to 1999 is outlined--from the original open surgery of Fenelon to the sophisticated, motor-driven electrodes of stereotactic surgery using depth microelectrode recording. The definition of the best target sites for the relief of tremor, rigidity and bradykinesia evolved progressively as accuracy in siting of lesions developed. The significance of these targets in understanding the pathophysiology of the disease and the neurotransmitters involved gradually became clearer and a way forward in future management was suggested.

Use of selective thalamotomy for various kinds of movement disorder, based on basic studies.

Methods of selective thalamotomy with microrecording for several kinds of movement disorder are described. Precise thalamotomy (Vim, VO or Vim+VO) depends on the understanding of the functional organization of the human thalamus on the one hand, and the patient's specific feature of movement disorders on the other. To realize selective thalamotomy, microrecording and a computerized planning system are essential. Thus, a properly selected thalamotomy for movement disorders is quite useful.

The treatment of movement disorders using Gamma Knife stereotactic radiosurgery.

In this era of modern neurosurgery, we are able to provide adequate amelioration of disabling symptoms for the small subset of patients who have conditions that may make them unacceptable candidates for invasive stereotactic neurosurgical intervention. Gamma Knife radiosurgical thalamotomy is an effective and useful alternative to invasive radiofrequency techniques for patients at high surgical risk. The mechanical accuracy of the gamma unit combined with the anatomical accuracy of high-resolution magnetic resonance imaging makes radiosurgical lesioning safe and precise.

Gamma knife radiosurgery as a lesioning technique in movement disorder surgery.

OBJECT: To increase knowledge of the safety and efficacy of the use of gamma knife radiosurgery in patients with movement disorders, the authors describe their own experience in this field and include blinded independent assessments of their results. METHODS: Fifty-five patients underwent radiosurgical placement of lesions either in the thalamus (27 patients) or globus pallidus (28 patients) for treatment of movement disorders. Patients were evaluated pre- and postoperatively by a team of observers skilled in the assessment of gait and movement disorders who were blinded to the procedure performed. The observers were not associated with the surgical team and concomitantly and blindly also assessed a group of 11 control patients with Parkinson's disease who did not undergo any surgical procedures. All stereotactic lesions were made with the Leksell gamma unit using the 4-mm secondary collimator helmet and a single isocenter with maximum doses from 120 to 160 Gy. Clinical follow-up evaluation indicated that 88% of patients who underwent thalamotomy became tremor free or nearly tremor free. Statistically significant improvements in performance were noted in the independent assessments of Unified Parkinson's Disease Rating Scale (UPDRS) scores in the patients undergoing thalamotomy. Of patients undergoing pallidotomy who had exhibited levodopainduced dyskinesias, 85.7% had total or near-total relief of that symptom. Clinical assessment indicated improvements in bradykinesia and rigidity in 64.3% of patients who underwent pallidotomy. Independent blinded assessments did not reveal statistically significant improvements in Hoehn and Yahr scores or UPDRS scores. On the other hand, 64.7% of patients showed improvements in subscores of the UPDRS, including activities of daily living (58%), total contralateral score (58%), and contralateral motor scores (47%). Total ipsilateral score and ipsilateral motor scores were both improved in 59% of patients. One (1.8%) of 55 patients experienced a homonymous hemianopsia 9 months after pallidotomy due to an unexpectedly large lesion. No other complications of any kind were seen. Neuropsychological test scores that were obtained for the combined pallidotomy and thalamotomy treatment groups preoperatively and at 6 months postoperatively demonstrated an absence of cognitive morbidity. Follow-up neuroimaging confirmed correct lesion location in all patients, with a mean maximum deviation from the planned target of 1 mm in the vertical axis. Measurements of lesions at regular intervals on postoperative magnetic resonance images demonstrated considerable variability in lesion volumes. The safety and efficacy of functional lesions made with the gamma knife appear to be similar to those made with the assistance of electrophysiological guidance with open functional stereotactic procedures. CONCLUSIONS: Functional lesions may be made safely and accurately using gamma knife radiosurgical techniques. The efficacy is equivalent to that reported for open techniques that use radiofrequency lesioning methods with electrophysiological guidance. Complications are very infrequent with the radiosurgical method. The use of functional radiosurgical lesioning to treat movement disorders is particularly attractive in older patients and in those with major systemic diseases or coagulopathies; its use in the general movement disorder population seems reasonable as well.