Trial of Globus Pallidus Focused Ultrasound Ablation in Parkinson's Disease.

BACKGROUND: Unilateral focused ultrasound ablation of the internal segment of globus pallidus has reduced motor symptoms of Parkinson's disease in open-label studies. METHODS: We randomly assigned, in a 3:1 ratio, patients with Parkinson's disease and dyskinesias or motor fluctuations and motor impairment in the off-medication state to undergo either focused ultrasound ablation opposite the most symptomatic side of the body or a sham procedure. The primary outcome was a response at 3 months, defined as a decrease of at least 3 points from baseline either in the score on the Movement Disorders Society-Unified Parkinson's Disease Rating Scale, part III (MDS-UPDRS III), for the treated side in the off-medication state or in the score on the Unified Dyskinesia Rating Scale (UDysRS) in the on-medication state. Secondary outcomes included changes from baseline to month 3 in the scores on various parts of the MDS-UPDRS. After the 3-month blinded phase, an open-label phase lasted until 12 months. RESULTS: Of 94 patients, 69 were assigned to undergo ultrasound ablation (active treatment) and 25 to undergo the sham procedure (control); 65 patients and 22 patients, respectively, completed the primary-outcome assessment. In the active-treatment group, 45 patients (69%) had a response, as compared with 7 (32%) in the control group (difference, 37 percentage points; 95% confidence interval, 15 to 60; P = 0.003). Of the patients in the active-treatment group who had a response, 19 met the MDS-UPDRS III criterion only, 8 met the UDysRS criterion only, and 18 met both criteria. Results for secondary outcomes were generally in the same direction as those for the primary outcome. Of the 39 patients in the active-treatment group who had had a response at 3 months and who were assessed at 12 months, 30 continued to have a response. Pallidotomy-related adverse events in the active-treatment group included dysarthria, gait disturbance, loss of taste, visual disturbance, and facial weakness. CONCLUSIONS: Unilateral pallidal ultrasound ablation resulted in a higher percentage of patients who had improved motor function or reduced dyskinesia than a sham procedure over a period of 3 months but was associated with adverse events. Longer and larger trials are required to determine the effect and safety of this technique in persons with Parkinson's disease. (Funded by Insightec; ClinicalTrials.gov number, NCT03319485.).

Arthroscopic release of the pectoralis minor tendon as an adjunct to acromioplasty in the treatment of subacromial syndrome associated with scapular dyskinesia.

INTRODUCTION: One of the causes of scapular dyskinesia is the retraction of the pectoralis minor muscle. It can be the cause of shoulder pain associated with increased anterior tilt and internal rotation of the shoulder. HYPOTHESIS: Arthroscopic release of the pectoralis minor tendon is effective for shoulder pain, linked to a correction of dyskinesia, resulting in the early disappearance of scapulalgia. PATIENTS AND METHODS: This was a retrospective, monocentric, single-surgeon study on a continuous series of patients with symptomatic shoulder pain, related to subacromial impingement associated with scapular dyskinesia. The series underwent arthroscopic tenotomies of the pectoralis minor between January 2015 and September 2018. Fifty-eight releases were performed to 57 patients (22 males, 35 females), with a mean age of 51.4 years (29-66 years). In all cases, they presented pain on palpation of the upper bundle of the trapezius muscle, and on palpation of the coracoid insertion of the pectoralis minor. RESULTS: Preoperatively, there were 30 cases of stage 1 scapular dyskinesia and 28 cases of stage 2, with a scapular index measured at 53.84 (41.66-65.78), while the constant score was 41.46 (19-59). Upper trapezius pain was present in 87.7% of cases. The 57 patients were clinically assessed, with a mean follow-up of 8.9 months (6-24 months). Post-operatively, 15 cases (25.8%) of scapulalgia persisted at 2 months of follow-up, while upper trapezius pain was present in only 22.4%. Scapular dyskinesia was no longer present at 2 months in patients without scapulalgia. The scapular index was measured at 60.42 (52.38-70.96), and the constant score at last follow-up was 76.36 (42-92). CONCLUSION: Tenotomy of the pectoralis minor improves painful symptomatology and scapular dyskinesia, in cases of subacromial syndrome with retraction of the pectoralis minor muscle. LEVEL OF EVIDENCE: IV, retrospective study.

Choreoathetosis in Moyamoya Disease.

Moyamoya disease is a cerebral angiopathy characterized by bilateral progressive narrowing of internal carotid arteries, developing collateral vessels with the aspect of a "puff of smoke." The presentation with movement disorders is extremely rare. We present the case of an 11-year-old girl with low academic performance who complained of involuntary movements starting in her right hand. Neurologic examination showed preserved muscle strength and right hemichoreoathetosis. Neuroimaging showed left hemisphere hypoperfusion and a hypertrophic distal lenticulostriate artery. The symptoms were controlled with medications, and cerebral revascularization was performed. Although movement disorders are usually related to cerebral lesions or hypoperfusion, cases without these etiologies were described. Thus the finding of asymmetric lenticulostriate arteries improving after asymmetry reduction suggests a possible role in the pathogenesis. Further studies are needed to fully elucidate the mechanisms between moyamoya disease and movement disorders.

Endoscopic Laser Fenestration to Treat a Bobble-Head Doll Syndrome Caused by Suprasellar Cyst.

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.

Suprasellar Cyst Presenting With Bobble-Head Doll Syndrome.

BACKGROUND: Bobble-head doll syndrome is a rare neurological syndrome presenting with repetitive anteroposterior head movements. It is usually associated with expansile cystic lesions in the third ventricular region. CASE DESCRIPTION: An 8-year-old boy presented with involuntary bobbling head movements. Magnetic resonance imaging of the brain revealed an extensive suprasellar cyst resulting in obstructive hydrocephalus. Endoscopic ventriculo-cysto-cisternostomy resulted in improved clinical outcome. CONCLUSIONS: Endoscopic ventriculo-cysto-cisternostomy is an effective, less-invasive technique in the treatment of suprasellar cysts that results in resolution of the bobbling head movements.

An unusual presentation of bobble-head doll syndrome in a patient with hydranencephaly and Chiari 3 malformation.

Bobble-head doll syndrome is a rare movement disorder that is usually associated with lesions involving the third ventricle. It is characterised by stereotypical rhythmic up-and-down or side-to-side head movements. The pathophysiology and anatomical basis for this unusual manifestation is still a subject of intense scrutiny. The syndrome has never been described in a patient with both hydranencephaly and Chiari type 3 malformation. We describe a 2-year-old female patient who presented with congenital hydrocephalus, an occipital encephalocele and rhythmic bobbling of the head. Imaging investigation revealed a Chiari type 3 malformation and hydranencephaly. The patient was taken to theatre for a ventriculoperitoneal shunt insertion, and at day 3 post operatively, the patient had a markedly decreased head circumference and a decrease in the frequency of the bobbling of the head. A further review at 2 weeks showed that the bobbling of the head had ceased. Although the pathophysiology of bobble-head doll syndrome is yet to be fully understood, there has been postulation of either a third ventricular enlargement or a cerebellar dysfunction to explain bobble-head doll syndrome. Our case illustrates that the pathophysiology is most likely multifactorial as illustrated by the fact that by just addressing the high intracranial pressure with a shunt was sufficient to treat the condition.

Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon.

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral ("no-no") involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

The Pioneering and Unknown Stereotactic Approach of Roeder and Orthner from Göttingen. Part II: Long-Term Outcome and Postmortem Analysis of Bilateral Pallidotomy in the Pre-Levodopa Era.

Before the advent of levodopa, pallidotomy was initially the most effective treatment for Parkinson disease, but it was soon superseded by thalamotomy. It is widely unknown that, similar to Leksell, 2 neurologists from Göttingen, Orthner and Roeder, perpetuated pallidotomy against the mainstream of their time. Postmortem studies demonstrated that true posterior and ventral pallidoansotomy sparing the overwhelming mass of the pallidum was accomplished. This was due to a unique and individually tailored stereotactic technique even allowing bilateral staged pallidotomies. In 1962, the long-term effects (3-year follow-up on average) of the first 18 out of 36 patients with staged bilateral pallidotomies were reported in great detail. Meticulous descriptions of each case indicate long-term improvements in parkinsonian rigidity and associated pain, as well as posture, gait, and akinesia (e.g., improved repetitive movements and arm swinging). Alleviation of tremor was found to require larger lesions than needed for suppression of rigidity. No improvement in speech, drooling, or seborrhea was observed. By 1962, the team had operated 13 patients with postencephalitic oculogyric crises with remarkable results (mean follow-up: 5 years). They also described alleviation of nonparkinsonian hyperkinetic disorders (e.g., hemiballism and chorea) with pallidotomy. The reported rates for surgical mortality and other complications had been remarkably low, even if compared to those reported after the revival of pallidotomy by Laitinen in the post-levodopa era. This applies also to bilateral pallidotomy performed with a positive risk-benefit ratio that has remained unparalleled to date. The intricate history of pallidotomy for movement disorders is incomplete without an appreciation of the achievements of the Göttingen group.

Bobble-head doll syndrome in an 80-year-old man, associated with a giant arachnoid cyst of the lamina quadrigemina, treated with endoscopic ventriculocystocisternotomy and cystoperitoneal shunt.

Bobble-head doll syndrome (BHDS) is a rare entity, characterized by antero-posterior head bobbing, which is of the type "yes-yes." Less frequently, having a head movement of the type "no-no" is described. We report an unusual case of an 80-year-old man with a cystic mass of the lamina quadrigemina, extending to the posterior fossa. We conclude that ventriculocystocisternotomy associated with a cystoperitoneal shunt is an effective treatment for a symptomatic giant arachnoid cyst in the lamina quadrigemina.

Surgical Release of the Pectoralis Minor Tendon for Scapular Dyskinesia and Shoulder Pain.

BACKGROUND: Pectoralis minor (PM) tightness has been linked to pain and dysfunction of the shoulder joint secondary to anterior tilt and internal rotation of the scapula, thus causing secondary impingement of the subacromial space. PURPOSE: To describe outcomes pertaining to nonoperative and operative treatment via surgical release of the PM tendon for pathologic PM tightness in an active population. STUDY DESIGN: Case series; Level of evidence, 4. METHODS: Over a 3-year period, a total of 46 patients were enrolled (mean age, 25.5 years; range, 18-33 years). Inclusion criteria consisted of symptomatic shoulder pain, limited range of overhead motion, inability to participate in overhead lifting activities, and examination findings consistent with scapular dysfunction secondary to a tight PM with tenderness to palpation of the PM tendon. All patients underwent a lengthy physical therapy and stretching program (mean, 11.4 months; range, 3-23 months), which was followed by serial examinations for resolution of symptoms and scapular tilt. Of the 46 patients, 6 (13%) were unable to adequately stretch the PM and underwent isolated mini-open PM release. Outcomes were assessed with scapula protraction measurements and pain scales as well as American Shoulder and Elbow Surgeons (ASES), Single Assessment Numeric Evaluation (SANE), and visual analog scale (VAS) scores. RESULTS: Forty of the 46 patients (87%) resolved the tight PM and scapular-mediated symptoms with a dedicated therapy program (pre- and posttreatment mean outcome scores: 58 and 91 [ASES], 50 and 90 [SANE], 4.9 and 0.8 [VAS]; P < .01 for all), but 6 patients were considered nonresponders (mean score, 48 [ASES], 40 [SANE], 5.9 [VAS]) and elected to have surgical PM release, with improved scores in all domains (mean score, 89 [ASES], 90.4 [SANE], 0.9 [VAS]; P < .01) at final follow-up of 26 months (range, 25-30 months). Additionally, protraction of the scapula improved from 1.2 to 0.3 cm in a mean midline measurement from the chest wall preoperatively to postoperatively ( P < .01), similar to results in nonoperative responders. No surgical complications were reported, and all patients returned to full activities. CONCLUSION: In most patients, PM tightness can be successfully treated with a nonoperative focused PM stretching program. However, in refractory and pathologically tight PM cases, this series demonstrates predictable return to function with notable improvement in shoulder symptoms after surgical release of the PM. Additional research is necessary to evaluate the long-term efficacy of isolated PM treatment.

Effect of scapular dyskinesis on supraspinatus repair healing in a rat model.

BACKGROUND: Rotator cuff tears are common conditions that often require surgical repair to improve function and to relieve pain. Unfortunately, repair failure remains a common problem after rotator cuff repair surgery. Several factors may contribute to repair failure, including age, tear size, and time from injury. However, the mechanical mechanisms resulting in repair failure are not well understood, making clinical management difficult. Specifically, altered scapular motion (termed scapular dyskinesis) may be one important and modifiable factor contributing to the risk of repair failure. Therefore, the objective of this study was to determine the effect of scapular dyskinesis on supraspinatus tendon healing after repair. METHODS: A rat model of scapular dyskinesis was used. Seventy adult male Sprague-Dawley rats (400-450 g) were randomized into 2 groups: nerve transection of the accessory and long thoracic nerves (SD) or sham nerve transection (Sham control). After this procedure, all rats underwent unilateral detachment and repair of the supraspinatus tendon. All rats were sacrificed at 2, 4, and 8 weeks after surgery. Shoulder function, passive joint mechanics, and tendon properties (mechanical, histologic, organizational, and compositional) were evaluated. RESULTS: Scapular dyskinesis alters joint function and may lead to compromised supraspinatus tendon properties. Specifically, diminished mechanical properties, altered histology, and decreased tendon organization were observed for some parameters. CONCLUSION: This study identifies scapular dyskinesis as one underlying mechanism leading to compromise of supraspinatus healing after repair. Identifying modifiable factors that lead to compromised tendon healing will help improve clinical outcomes after repair.

Bilateral asymmetrical asterixis as limb-shaking transient ischemic attack in bilateral carotid stenosis.

Asterixis as limb-shaking transient ischemic attack (TIA) is rare and poorly understood. Bilateral asymmetrical asterixis as limb-shaking TIA has not been reported in carotid stenosis. A 69-year-old gentleman presented with a TIA episode (dysarthria, right-arm weakness, and numbness). Bilateral asterixis was observed and was more severe on the right side. No prior infarcts were noted in the thalamus. Liver function was normal. A computerized tomography angiogram revealed 85%stenosis of the right internal carotid artery (ICA) and 65% stenosis of the left ICA. Three days after left ICA endarterectomy, the patient had complete disappearance of bilateral asterixis, with the right side showing initial improvement. The bilateral asterixis observed is proposed to be secondary to hemodynamic impairment and hypoperfusion of certain brain territory with resolution on revascularization.

"No-no" type bobble-head doll syndrome in an infant with an arachnoid cyst of the posterior fossa: a case report.

BACKGROUND: Bobble-head doll syndrome is a rare and surgically treatable movement disorder characterized by up-and-down (yes-yes) head bobbing occurring at a rate of 2-3 Hz. Side-to-side (no-no) head bobbing is less frequently described. Bobble-head doll syndrome is usually associated with dilation of the third ventricle, but is rarely associated with posterior fossa disease. PATIENT: We describe an infant with fetal hydrocephalus and an arachnoid cyst of the posterior fossa. Endoscopic fenestration of the arachnoid cyst was performed on postnatal day 12. A routine examination at 4 months indicated the infant showed "no-no" type head bobbing, but no other neurological disorder was observed. The third ventricle was dilated during the perioperative period, but not at 2-4 months. In contrast, cerebellar compression decreased gradually and persisted at 4 months. CONCLUSION: Although few patients with bobble-head doll syndrome do not have third ventricle dilation, these patients typically show cerebellar dysfunction. Our findings support the hypothesis that cerebellar dysfunction is present in bobble-head doll syndrome when third ventricle dilation is absent.

Bobble-head doll syndrome: therapeutic outcome and long-term follow-up in four children.

Bobble-head doll syndrome (BHDS) is a complex syndrome with the dominant symptom of repetitive anteroposterior head movement. Only 57 patients are quoted in the literature. The etiology of this syndrome remains unknown and no standard treatment has yet been established. We hereby report four cases treated at our department. All the patients presented a psychomotor retardation due to an obstructive hydrocephalus. All the patients were treated using neuroendoscopic techniques: two with ventriculocystostomy, and two with ventriculocystocisternostomy. Cyst decompression was achieved in all four cases and clinical recovery was evident in three of the four patients observed. After surgery, BHDS persisted longer the more the subsequent treatment was delayed. In this article, we provide a concise overview of the theories of pathogenesis, presentation, and management of this syndrome. Based on our own experience, we state that the method of choice should be the neuroendoscopy and this must be performed promptly after diagnosis is made.

Pseudo-piano playing motions and nocturnal hypoventilation in anti-NMDA receptor encephalitis: response to prompt tumor removal and immunotherapy.

Tumor resection is recommended in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, however it is often difficult during an early stage of the disease. We report here the efficacy of early tumor removal in a patient with anti-NMDAR encephalitis. This 21-year-old woman was admitted to another hospital with rapidly progressive psychiatric symptoms, a decreased level of consciousness, and seizures. Abdominal CT showed a pelvic mass. On day 1 of admission to our center, she developed hypoventilation requiring mechanical support. She had orofacial dyskinesias with well-coordinated, pseudo-piano playing involuntary finger movements. Based on these clinical features, she was immediately scheduled for tumor resection on day 3. While awaiting surgery, she began to receive high-dose intravenous methylprednisolone. After tumor removal, she received plasma exchange, followed by intravenous immunoglobulin and additional high-dose methylprednisolone. Two weeks after tumor removal, she started following simple commands and progressive improvement, although she remained on mechanical ventilation for 10 weeks due to nocturnal central hypoventilation. Anti-NMDAR antibodies in serum/CSF were detected. Pathological examination showed immature teratoma with foci of infiltrates of B- and T-cells. Early tumor resection with immunotherapy facilitates recovery from this disease, but central hypoventilation may require long mechanical support. Non-jerky elaborate finger movements suggest antibody-mediated disinhibition of the cortico-striatal systems.

Patients with moyamoya disease presenting with movement disorder.

Moyamoya disease is a rare cerebrovascular disease characterized by idiopathic bilateral stenosis or occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels at the base of the brain. Typical presentations include transient ischemic attacks or stroke, and hemorrhage. Presentation with movement disorders is extremely rare, especially in the pediatric population. The authors describe the cases of 4 children with moyamoya disease who presented with movement disorders. Among 446 patients (118 pediatric) with moyamoya disease surgically treated by the senior author, 4 pediatric patients had presented with movement disorders. The clinical records, imaging studies, surgical details, and postoperative clinical and imaging data were retrospectively reviewed. The initial presenting symptom was movement disorder in all 4 patients: chorea in 2, hemiballismus in 1, and involuntary limb shaking in 1. All the patients had watershed infarcts involving the frontal subcortical region on MR imaging. Additionally, 1 patient had a ganglionic infarct. Single-photon emission computed tomography studies showed frontoparietal cortical and subcortical hypoperfusion in all patients. Three patients had bilateral disease, whereas 1 had unilateral disease. All the patients underwent superficial temporal artery-middle cerebral artery bypass. Postoperatively, all 4 patients had complete improvement in their symptoms. The SPECT scans revealed normal perfusion in 3 patients and a small residual perfusion deficit in 1. Movement disorders are a rare presenting feature of moyamoya disease. Hypoperfusion of the frontal cortical and subcortical region was seen in all patients, and the symptomatology was attributed to ischemic dysfunction and imbalance in the cortical-subcortical-ganglionic-thalamic-cortical circuitry. Combined revascularization with superficial temporal artery-middle cerebral artery bypass and encephaloduroarteriosynangiosis leads to excellent results.