Hemiballism after subthalamotomy in patients with Parkinson's disease: report of 2 cases.

The occurrence of persistent hemiballism after subthalamotomy for Parkinson's disease (PD) has not been described as frequently as mild or transient dyskinesia. We report on 2 patients with advanced PD who developed hemiballism and/or dyskinesia after subthalamotomy. One patient with a small lesion confined to the subthalamic nucleus (STN) developed persistent hemiballism; the other with a larger lesion involving the STN and also the zona incerta presented with a transient dyskinesia in a single limb. We conclude that a precise STN lesion might bear a potential risk of persistent hemiballism.     

Sarcoglycanopathies: a report of 25 cases

Twenty five patients with sarcoglycanopathies were studied prospectively. 21 of them had mild phenotype. Muscle involvement was more pronounced in adductor and flexor groups of muscles of the limbs, hip adductor muscles being the weakest. The selective and differential weakness between weak hip adductors and stronger hip abductors resulted in the hip abduction sign in 64% of cases. Distal muscle involvement in lower limbs was seen in 92% of cases, but was mild and late in the course of the disease. 44% patients had winging of scapulae. Immunocytochemistry showed multiple sarcoglycan deficiencies in 84% patients. Primary beta and delta sarcoglycanopathy was seen in the remaining 16% cases. Secondary dystrophin reduction was seen in 44% patients and correlated with beta sarcoglycan deficiency but not with functional disability.     

Hemiballism. Clinico-pathological conference

A case of left hemiballism is presented. Besides multiple lacunar infarcts an haemorrhage in the contralateral subthalamic nucleus is shown. A short review of the literature is given with special attention to the etiology and the therapy.     

Hemiballism with hyperglycemia and striatal T1-MRI hyperintensity: an autopsy report.

We report on an autopsy findings of a 92-year-old male with hemiballism-hemichorea associated with hyperglycemia and striatal hyperintensity on T1-weighed magnetic resonance imaging (MRI), a recently described clinicoradiological syndrome. Histologically, the putamen contralateral to the hemiballism consisted of multiple foci of recent infarcts associated with reactive astrocytic and interneuronal response. Substrate responsible for the MRI signal changes is still inconclusive. Copyright Movement Disorder Society.     

Hemiballism: revisiting a classic disorder

Hemiballism is a rare movement disorder that presents with unilateral flinging movements of the limbs. In traditional teaching, it has been characterised as almost pathognomonic of a lesion in the subthalamic nucleus (STN). The prognosis was described as grave, with severe disability and death in many cases. However, review of more recent reports shows that the STN is directly involved in only a minority of cases. The prognosis is benign in most cases, with almost all patients responding well to treatment and many having spontaneous remission, although long-term prognosis of cerebrovascular disease may not be so good. There have also been recent insights into the pathophysiology of hemiballism, which have emphasised the importance of altered firing patterns in basal-ganglia structures. Recent studies have pointed to previously unrecognised causes, particularly non-ketotic hyperosmolar hyperglycaemia and complications of HIV infection, that may account for a substantial proportion of cases of hemiballism.     

Hemiballism and tremor due to ependymal cyst.

A 21-year-old woman was admitted with right hemiballism and tremor. She had tremor since the age of 6 years. At age 12, an intracerebral, left paraventricular space-occupying lesion was found and treated with 4,500 rads. Increasing tremor was associated with mass enlargement. By age 20, there was insidious presentation of right hemiballism. At age 21, she had craniotomy and a large septate cyst was opened and drained. Biopsy of the cyst wall revealed that it was consistent with ependymal cyst. Postoperatively the hemiballism resolved and the tremor improved. This case is unusual due to the presentation of hemiballism caused by ependymal cyst.     

Chronic polyradiculoneuritis. 25 cases]

Twenty-five patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were studied in order to define the clinical, biological, electrophysiological and pathological features of this disease. There were 11 men and 14 women ranging in age from 15 to 82 years. The average follow-up was 43 months. Patients fulfilled the criteria laid down by Dyck et al. (1975), except that progression of weakness was at least 2 months and not 6. Fourteen patients had a progressive course and 11 a relapsing one. Weakness was almost constant (24/25), sensory impairment was present in 22/25 with deep sensation predominantly impaired. Areflexia was observed in all patients. A history of previous infection or other possible precipitating event was given by 7 patients. Cerebrospinal fluid examination showed a raised total protein count in 22 cases. Electrophysiological examination revealed a slowing down of nerve conduction velocities to an extent compatible with a demyelinating process in 23 cases; 2 patients only had prolongation of F-waves. Sural nerve biopsy was less informative: inflammatory process--the most specific finding--was observed in only 3 out of 20 biopsies. Six patients had benign forms or spontaneous remission, the others were treated with corticosteroids or with immunosuppressive drugs. Most patients (64%) recovered very well. Only one died during the time of study. Eight other patients were treated with high-dose intravenous immunoglobulins and 4 of them improved with administrations at regular intervals to maintain the benefits observed. The occurrence of CIDP in association with other conditions is reviewed and we discuss its nosological position among the acquired demyelinating neuropathies.     

外科治疗中央区顽固性癫痫

目的探讨累及中央区顽固性癫痫的外科治疗方法,并分析影响预后结果的相关因素。方法回顾性分析2008～2011年手术治疗的25例累及中央区的顽固性癫痫患者的临床资料。所有病例依情况分别结合颅内电极、皮层电刺激功能区描记、神经导航、术中唤醒和术中电生理监测等手段进行病灶、致痫区和功能区定位。结果3例术后出现短暂的对侧肢体肌力下降,2例术后对侧肢体肌力有回升,活动更灵活。术后随访12—24个月：EngelⅠ级16例,Ⅱ级5例,Ⅲ级1例,Ⅳ级3例。结论对累及中央区的顽固性癫痫患者,应在保障安全的前提下尽可能切除致痫灶和病变。颅内电极记录和术中唤醒等技术可用于颅内致痫灶的定位,在术中电生理监测下充分切除病灶及癫痫样放电区对控制癫痫发作效果良好。     

外伤性硬膜下积液的治疗(附25例报告)

外伤性硬膜下积液是颅脑损伤中一种特殊的类型,我院从1998年10月至2002年12月共收治25例,分析如下:     

腹腔分流术治疗儿童外伤性硬膜下积液25例报告

颅内外伤性硬脑膜下腔积液是儿童颅脑外伤的常见疾病,通常出现颅高压症状、癫痫和部分神经功能障碍时需要手术治疗。以往的外科治疗方法以钻孔引流术为主,但是相当一部分患者引流效果不佳,复发率很高,临床症状不易消失。自2002年8月至2005年5月我科共实施硬脑膜下腔积液-腹腔分     

Os odontoideum: report of three cases.

Os odontoideum is a condition in which a smoothly corticated ossicle exists dorsal to the anterior arch of C1, taking the place of the rostral dens, but with no bony connection to the body of the axis. Three patients presented with this condition: the first with Lhermitte's phenomenon 10 years after significant trauma, the second as an incidental finding during routine cervical spine imaging following a road traffic accident, and the third with recurrent transient quadriparesis precipitated by falls from a surfboard. Patients had at least 10 mm of sagittal instability on dynamic imaging and the second patient had a minimum sagittal canal diameter of only 11.5 mm. Posterior atlanto-axial fixation was successfully achieved in all cases using polyaxial screws and rods with the assistance of computed tomography-based image guidance. Image guidance provided an invaluable aid to preoperative planning and intraoperative placement of the posterior spinal instrumentation.     

Brain hydatidosis: report of two cases.

Two children affected by cerebral hydatidosis are described. For preoperative diagnostic purposes computerised axial tomography appears currently to be the most suitable procedure. Complete surgical removal of the cyst, with methods preserving its integrity, seems to be the main factor for definitive recovery as the different outcomes in the two cases show.     

迷走神经刺激术治疗难治性癫痫(附25例报告)

目的研究迷走神经刺激术治疗难治性癫痫的适应证选择、手术方法、术后程控及疗效。方法 2009年11月~2016年7月清华大学玉泉医院癫痫中心对25例难治性癫痫患者行迷走神经刺激术,术后给予程控管理控制癫痫发作;并通过随访回顾性分析术后疗效。结果术后随访患者4个月~7年,其中癫痫发作频率减少达McHughⅠ级6例、Ⅱ级12例、Ⅲ级4例、Ⅳ级1例、Ⅴ级2例。手术前后癫痫发作频率对比,差异有统计学意义(P0.05)。结论迷走神经刺激术作为一种安全有效的辅助治疗难治性癫痫的方法,其治疗效果可以随着参数的调整和时间延长而增强,并可以一定程度改善患者的认知行为和生活质量。     

Cockayne syndrome: Review of 25 cases

Clinical and laboratory findings of 25 patients with classical Cockayne syndrome (CS) are reviewed. A history of consanguinity was present in 21 patients, and 15 patients had at least 1 affected sibling. Apart from the cardinal features of dwarfism, microcephaly, and mental retardation, the most consistent clinical features included photosensitivity (84%), gait disturbances (84%), progeroid appearance (84%), and ocular abnormalities (88%). The most consistent laboratory findings comprised abnormal nerve conduction (slowed conduction in 13 of the 16 cases with an ENMG), and an abnormal brainstem auditory evoked response (BAER) and/or audiometry (abnormal in 13 of the 17 cases in whom either one of them were available). Cerebral atrophy and calcification of the basal ganglia were the next more common laboratory findings. Clinical criteria are useful in most instances in the diagnosis of CS. In patients in whom the clinical features are controversial for a diagnosis of Cockayne syndrome, studies directed to disorders of myelination involving both peripheral and central nervous systems in conjunction with audiometry may aid in the diagnosis.     

Stupor: a review of 25 cases

ABSTRACT– Twenty-five cases of stupor referred to a psychiatric teaching hospital over 15 years are reviewed. Ten cases presented with a depressive syndrome, four cases with catatonic phenomena and a further 10 cases with established organic aetiology; one case was considered to be psychogenic in origin. The overall mortality was high (16%), attributable to the basic pathology. Compared with earlier series there was a reduction in cases with catatonic features with a corresponding increase in the proportion of organic cases.     

Multicystic acoustic neurinoma: report of two cases.

A report of two unusual and remarkably similar cases of multicystic acoustic neurinoma in 46 and 60 year old patients is presented. The clinical presentation mimicked a regular acoustic neurinoma. The tumours in each case were similar in their radiological appearance. The entire tumour had a honeycomb appearance with well defined and multiple cysts. Each cyst contained xanthochromic fluid. Like in any other cystic acoustic neurinoma, the presence of multiple cysts made internal decompression of the tumour relatively easy but the dissection of the tumour from the adjoining cranial nerves was difficult. The pathogenesis of multiple cysts is analyzed and the literature on this subject is briefly reviewed.