Pallidal deep brain stimulation for patients with myoclonus-dystonia without SGCE mutations.

BACKGROUND: Pallidal deep brain stimulation (GPi-DBS) is effective for treating myoclonus and dystonia caused by SGCE mutations (DYT-SGCE, DYT11). However, it is unknown whether GPi-DBS is effective for the treatment of myoclonus-dystonia which is not associated with the SGCE gene mutations. In this study, we investigated the efficacy of GPi-DBS in treating myoclonus-dystonia in SGCE mutation-negative cases. METHODS: Three patients with myoclonus-dystonia without SGCE mutations who underwent GPi-DBS were evaluated preoperatively and 6 months postoperatively using the Unified Myoclonus Rating Scale (UMRS) and Fahn-Marsden Dystonia Rating Scale (FMDRS) for myoclonus and dystonia, respectively. In two of the three patients, myoclonus was more evident during action. Myoclonus was predominant at rest in the other patient, and he was unaware of his dystonia symptoms. The results were compared with those of the four DYT-SGCE cases. RESULTS: The mean UMRS score in patients with myoclonus-dystonia without SGCE mutations improved from 61.7 to 33.7 pre- and postoperatively, respectively, and the mean FMDRS score improved from 7.2 to 4.5. However, the degree of improvement in myoclonus-dystonia in patients without SGCE mutations was inferior to that in patients with DYT-SGCE (the UMRS score improved by 45% and 69%, respectively). CONCLUSIONS: GPi-DBS is effective for treating myoclonus-dystonia in patients with and without SGCE mutations. GPi-DBS should be considered as a treatment option for myoclonus-dystonia without SGCE mutations.

Symptomatic Change of an Intracranial Neuroepithelial Cyst 7 Years After Its Incidental Finding

A natural course of asymptomatic neuroepithelial cysts (NECs) is poorly understood due to its rarity.Herein we report a 23-year-old female patient of an asymptomatic NEC which grew in size from 1 cm to 5 cm and caused progressive symptoms seven years after its incidental finding. Partial resection of the cyst was performed for decompression and pathological examination and effectively achieved symptoms alleviation and regression of the cyst. Our case showed the importance of regular follow-up because NECs may show symptomatic change even in the late phase.

Intraoperative Anatomical Findings in Pediatric Clear Cell Meningioma of the Lumbar Spine: Case Report and Literature Review.

Clear cell meningioma (CCM) is a WHO classification Grade II meningioma. It is a very rare disease, of which only 41 cases of spinal cord CCM in children have been reported to date. CCMs sometimes do not have the "dural attachment" that is usually found in meningiomas, and our understanding of the origin of CCMs is therefore controversial. We hereby present a case of pediatric CCM of the lumbar spine, in which we examined intraoperatively, the detailed anatomical location of the tumor. The case is a 10-year-old boy, who presented to our hospital with a 2-month history of lower back and bilateral lower extremity pain upon waking, which gradually worsened. Lumbar spine CT and MRI revealed an intradural extramedullary tumor at the L3 vertebral level, and surgery was performed to remove it. The tumor was in close contact with the dura mater, and also in contact with the cauda equina via the arachnoid. The tumor was likely located primarily between the dura mater and arachnoid. The pathological diagnosis was CCM, with an MIB-1 index of less than 1%. His back pain and bilateral lower extremity pain improved after surgery, and he was discharged from our hospital. Postoperative radiation therapy was not performed. Based on this case, we suggest that intraoperative examination of the anatomical location of these tumors and accumulation of relevant experience are important to elucidate the embryological mechanisms of this rare disease.

Myelographic CT, A Check-Valve Mechanism, and Microsurgical Treatment of Sacral Perineural Tarlov Cysts.

OBJECTIVE: There is currently no consensus regarding surgical indications for symptomatic sacral perineural cysts. METHODS: Nine patients with symptomatic sacral perineural cysts underwent microsurgery. All patients fulfilled the following criteria: (1) cyst sizes larger than 15 mm; (2) cysts show the "delayed inflow" and/or "delayed outflow" of contrast on myelographic computed tomography (CT), and (3) neurological symptoms correlate with the primary cyst. RESULTS: On myelographic CT, all primary cysts showed the "delayed inflow" of contrast; the average cyst/thecal sac Hounsfield units (HU) ratio was 0.17. In 7 patients, the primary cyst showed "delayed outflow"; the average cyst/thecal sac HU ratio increased to 3.12 on images obtained 24 hours after contrast injection. Regarding the modified Rankin Scale, 67% of patients reported that their overall symptoms improved to normal activities after surgery. The most improved symptom was coccydynia (75% improvement, P = 0.017), followed by leg radiation pain (67% improvement, P = 0.027) and buttock pain (50% improvement, P = 0.068). Bowel/bladder dysfunction improved in 100% of patients, but newly developed in 1 patient (P = 0.32). Perineal pain only decreased in 33% (P = 0.41). CONCLUSIONS: To the best of our knowledge, this is the first study to have performed a quantitative analysis of the dynamics of cerebrospinal fluid in sacral perineural cysts using myelographic CT. Sixty-seven percent of patients benefited from surgery; however, our criteria may not be a necessary and sufficient condition for patient selection because 33% did not respond to surgery despite the successful elimination of the check-valve.

Frequency-Specific Synchronization in the Bilateral Subthalamic Nuclei Depending on Voluntary Muscle Contraction and Relaxation in Patients with Parkinson's Disease.

The volitional control of muscle contraction and relaxation is a fundamental component of human motor activity, but how the processing of the subcortical networks, including the subthalamic nucleus (STN), is involved in voluntary muscle contraction (VMC) and voluntary muscle relaxation (VMR) remains unclear. In this study, local field potentials (LFPs) of bilateral STNs were recorded in patients with Parkinson's disease (PD) while performing externally paced VMC and VMR tasks of the unilateral wrist extensor muscle. The VMC- or VMR-related oscillatory activities and their functional couplings were investigated over the theta (4-7 Hz), alpha (8-13 Hz), beta (14-35 Hz), and gamma (40-100 Hz) frequency bands. Alpha and beta desynchronizations were observed in bilateral STNs at the onset of both VMC and VMR tasks. On the other hand, theta and gamma synchronizations were prominent in bilateral STNs specifically at the onset of the VMC task. In particular, just after VMC, theta functional coupling between the bilateral STNs increased, and the theta phase became coupled to the gamma amplitude within the contralateral STN in a phase-amplitude cross-frequency coupled manner. On the other hand, the prominent beta-gamma cross-frequency couplings observed in the bilateral STNs at rest were reduced by the VMC and VMR tasks. These results suggest that STNs are bilaterally involved in the different performances of muscle contraction and relaxation through the theta-gamma and beta-gamma networks between bilateral STNs in patients with PD.

Hydrocephalus associated with cystic dilation of the foramina of Magendie and Luschka.

Cystic malformations in the posterior cranial fossa result from developmental failure in the paleocerebellum and meninges. The authors present the case of an infant with hydrocephalus associated with cystic dilation of the foramina of Magendie and Luschka. This 7-month-old female infant presented with sudden onset of tonic-clonic seizures. Computed tomography revealed tetraventricular hydrocephalus. Magnetic resonance imaging demonstrated a cyst communicating with the fourth ventricle and projecting to the cisterna magna and the cerebellopontine cisterns through the foramina of Magendie and Luschka. A suboccipital craniotomy was performed for removal of the cyst wall, and the transparent membrane covering the foramen of Magendie was removed under a microscope. After the surgery, the patient's hydrocephalus improved and a phase contrast cine MR imaging study showed evidence of normal CSF flow at the level of the third and fourth ventricles. Three weeks later, however, the hydrocephalus recurred. An endoscopic third ventriculocisternostomy was performed to address the possibility of stagnant CSF flow in the posterior cranial fossa, but the hydrocephalus continued. Finally the patient underwent placement of a ventriculoperitoneal shunt, resulting in improvement of her symptoms and resolution of the hydrocephalus. On the basis of this experience and previously published reports, the authors speculate that the cystic malformation in their patient could be classified in a continuum of persistent Blake pouch cysts. Hydrocephalus was caused by a combination of obstruction of CSF flow at the outlets of the fourth ventricle and disequilibrium between CSF production and absorption capacity.

Rheumatoid leptomeningitis: rare complication of rheumatoid arthritis.

Rheumatoid leptomeningitis is a rare complication of rheumatoid arthritis (RA). We describe a woman with rheumatoid leptomeningitis presenting with acute-onset behavioral changes and consciousness disturbance in the early stage of RA. On fluid-attenuated inversion recovery images or diffusion-weighted images, high-signal-intensity lesions in the subarachnoid spaces of the right frontal lobe were observed. Biopsies of brain tissues and the dura mater located in the right frontal lobe were obtained. On the basis of the findings of histopathological analysis, a diagnosis of necrotizing granulomas involving the leptomeninges consistent with rheumatoid leptomeningitis was made. An early diagnosis of rheumatoid leptomeningitis and immediate initiation of treatment may prevent neurological sequelae.

[The management of patients with thoracic outlet syndrome (TOS) and an assistant diagnosis to discriminate between TOS and cervical spondylosis].

Between 1984 and 2007, 20 patients, admitted to our hospital for the treatment of thoracic outlet syndrome (TOS), were analyzed. Of these 20 patients, 2 subsequently entered rehabilitation to strengthen the muscle power surrounding the shoulder, 5 were followed by a brachial plexus anesthesiological block for the purpose of both diagnosis and treatment of TOS, and the other 13 patients underwent a total of 18 surgeries. Surgical methods consisted of a supraclavicular first rib resection with anterior and middle scalenectomy (SCFR + AMS) in 16 surgeries, and a transaxillary first rib resection (TAFR) in 2 surgeries. Preoperative symptoms disappeared immediately after surgery in all cases except for 1 that was treated by a TAFR. The surgical result of SCFR + AMS was favorable and effective to prevent a symptomatic recurrence due to the postoperative re-adhesion. We measured the inclination of the clavicle in these TOS patients, using a chest X-ray, which determines indirectly the width of the costoclavicular space, and compared it with that of 18 patients with cervical diseases, including cervical spondylosis and cervical disc herniation, who were operated on at our institution. By comparison of the inclination angle of the clavicle between the injured and the non-injured side in each diseases, it was shown that the injured side had declined by 3.0 degrees but than the non-injured side in TOS, more by only -0.1 degrees in the cervical diseases. This method may be utilized as an assistant diagnosis to discriminate between TOS and cervical diseases.

[Treatment for the ruptured bilateral vertebral dissecting aneurysms].

We report a case of a bilateral vertebral dissecting aneurysm associated with subarachnoid hemorrhage. Proximal ligation of the vertebral artery on the ruptured side combined with wrapping of the contralateral dissection failed to prevent fatal rebleeding. Since enlargement of the contralateral dissection was observed by postoperative angiography, rupture of the growing contralateral dissecting aneurysm may have caused rebleeding. Hemodynamic changes following the occlusion of one vertebral artery might have led to enlargement and subsequent rupture of the contralateral dissection. Direct wrapping was unable to prevent enlargement of the dissection, so radical surgery including bilateral vertebral artery occlusion combined with vascular reconstruction may be the treatment of choice for this type of lesion.