Sonographic basal ganglia alterations are related to non-motor symptoms in multiple sclerosis

The anatomical basis of cognitive dysfunction and other non-motor symptoms in multiple sclerosis (MS) is poorly understood. In MS patients, transcranial sonography (TCS) shows neurodegenerative disease-like lesions of the substantia nigra (SN) and basal ganglia, thought to reflect iron accumulation. The present study deals with the question of whether sonographic changes of SN, brainstem raphe, lenticular nucleus (LN) or caudate nucleus are related to non-motor symptoms of MS. We used TCS to investigate 54 MS patients and 54 age- and sex-matched healthy subjects. Degree of cognitive (executive) dysfunction, fatigue, depression, and urinary urge incontinence in MS patients was assessed using the Paced Auditory Serial Addition Test, the Faces Symbol Test, the Modified Fatigue Impact Scale, the Beck Depression Inventory, and the Urinary Distress Inventory. Abnormal TCS findings of SN, brainstem raphe, LN, and caudate nucleus were found in 13, 7, 11, and 6% of the healthy subjects, but in 54, 43, 62, and 41% (each, p < 0.001) of the MS patients, with similar frequency in relapsing-remitting and primary or secondary progressive MS patients. Sonographic alteration of the LN correlated with cognitive dysfunction. Combined alteration of both, LN and SN, was clearly associated with cognitive dysfunction and cognitive fatigue. The combined sonographic alteration of SN and brainstem raphe indicated severe urinary urge incontinence irrespective of the presence of spinal MS lesions. No relation was found between depression and any of the TCS findings. These findings suggest that neurodegenerative processes affecting deep brain structures contribute to cognitive and autonomic dysfunction in MS.     

Organic schizophrenic syndrome associated with symmetrical basal ganglia sclerosis and XO/XY-mosaic.

Psychopathological alterations associated with symmetrical basal ganglia sclerosis have been well characterized. A preponderance of a so-called organic affective syndrome has been reported (K?nig 1989), but schizophrenic syndromes have also been described, in particular in young patients (Cummings et al 1983). Symmetrical basal ganglia sclerosis may be secondary to ischemia, hypoxia, trauma, intoxications, inflammations, or hyporesp. pseudohypoparathyroidism. Among idiopathic forms sporadic as well as familial ones with dominant and recessive inheritance have been observed (Billard et al 1989).     

Catecholamine alterations in basal ganglia after hippocampal lesions

Rats were given sham, cortical, or hippocampal lesions and sacrificed 7 or 28 days following surgery. Levels of norepinephrine, dopamine, and the major dopamine metabolites, 3, 4-dihydroxyphenylacetic acid and homovanillic acid (HVA), were assayed in 3 brain regions. At day 7 there was a decrease in dopamine utilization and a decrease in norepinephrine levels in the nucleus accumbens after hippocampal damage but both of these measures returned to normal levels by day 28. In the neostriatum HVA levels decreased at day 7 after hippocampal damage. The utilization of dopamine in the neostriatum was decreased at day 28 in animals that received neocortical lesions but this was not observed in animals with hippocampal destruction. No effects of any lesion at any day were found in the olfactory tubercle region, the third brain region analyzed. It is thought that the removal of hippocampal and neocortical input to the basal ganglia influences catecholamine function reflected in the loss and subsequent recovery of dopamine utilization.     

基底节生殖细胞瘤2例报告

颅内原发性生殖细胞瘤好发于松果体区,发生于基底节区者较少见,临床上早期诊断较为困难,常误诊为基底节胶质瘤.近年来我科收治2例基底节区生殖细胞瘤,经治疗后效果满意.     

Isolated symmetrical bilateral basal ganglia T2 hyperintensity in carbon monoxide poisoning

Carbon monoxide poisoning is not uncommon during the winter months. To make a diagnosis, strong clinical suspicion and acumen, and history of the exposure are necessary. Many a time, the presenting complaints may fail to help reach a diagnosis, in the absence of history. Imaging plays a role in the diagnosis of brain injury with the characteristic features, which are correlated with the clinical profile. Isolated bilateral basal ganglia injury revealing T2 hyperintensity in MRI may be observed in acute carbon monoxide poisoning.     

Signal intensity on MRI of basal ganglia in multiple sclerosis.

It has been reported that a relative reduction in signal intensity on T2 weighted MRI may be seen in the basal ganglia of patients with multiple sclerosis and furthermore that this is due to excessive iron deposition. The basal ganglia are, however, rarely involved clinically or pathologically in multiple sclerosis, casting some doubt on this finding. Therefore MRI was carried out in 46 patients with definite multiple sclerosis and 42 age matched controls. Contiguous, 5 mm thick axial dual echo spin-echo images of the brain were obtained on a 1.5T imager. Visual rating scales were used to measure the lesion load as well as the signal intensity of the globus pallidus, putamen, caudate nucleus, substantia nigra, red nucleus, and thalamus. There was a mild degree of low signal intensity in the patient group in the thalamus only. The signal intensity of the thalamus and putamen was never lower than that of the globus pallidus. Low signal in the basal ganglia is rarely, if ever, found in multiple sclerosis and is not a useful radiological sign.     

Distinct basal ganglia hyperechogenicity in idiopathic basal ganglia calcification

We report a 67‐year‐old patient with idiopathic basal ganglia calcification (IBGC). He presented with progressive cognitive impairment, frontal lobe dysfunction, mild leg spasticity, and levodopa (L ‐dopa)‐responsive parkinsonism. Transcranial sonography (TCS) revealed marked hyperechogenicity of the basal ganglia and periventricular spaces bilaterally. The detected signal alterations showed a fairly symmetric distribution and corresponded to the hyperintense calcifications depicted on the computer tomography brain scan. The combination of symmetric hyperechogenic areas adjacent to the lateral ventricles and of the basal ganglia may serve as an imaging marker characteristic of IBGC. Hyperechogenicity due to extended basal ganglia calcification as presented here is distinct from the pattern of hyperechogenicity caused by heavy metal accumulation, which is described to be less striking. In addition to atypical parkinsonian syndromes such as progressive supranuclear palsy and multiple system atrophy, IBGC is thus another differential diagnosis of parkinsonism with basal ganglia hyperechogenicity. © 2010 Movement Disorder Society.     

Morphine-induced alterations of local cerebral glucose utilization in the basal ganglia of rats

The actions of various doses of morphine on the local cerebral glucose utilization (LCGU) were studied by means of the autoradiographic [¹⁴C]2-deoxyglucose technique. Morphine (1–15 mg/kg i.p.) decreased LCGU in most areas of the basal ganglia (caudate nucleus, globus pallidus, nucleus accumbens), but not in the substantia nigra pars compacta. LCGU was also decreased in limbic nuclei, such as septum, hippocampus and amygdala, and in most thalamic areas. In most cortical regions, a decrease was found as well. Findings in some efferent nuclei seemed of particular interest, namely in the substantia nigra pars reticulata, anteroventral and lateral nucleus of the thalamus and the subthalamic nucleus, where decrease in LCGU were found after administration of 7.5 mg/kg or sometimes lower doses, but not after 15 mg/kg of morphine. The decreases seem to reflect a general depressory effect of morphine on neuronal activity which is known from electrophysiological studies. Part of these effects might be, in addition, due to an activation of dopaminergic neurons, since dopamine mainly acts as an inhibitory neurotransmitter. This dopaminergic activation leads to characteristic behavioral effects after lower doses of morphine. The largest dose used (15 mg/kg) produces muscular rigidity, probably by a direct action on the striatum. This effect antagonizes and masks the dopaminomimetic effects. The results suggest that it also antagonizes the functional alterations in some efferent nuclei of the basal ganglia manifest after lower doses of morphine. Local injections of morphine (15 μg) led to decreases of LCGU in the various parts of the striatum, but to increases in lateral and anteroventral thalamus. These increases in LCGU in two thalamic areas seem to support the above hypothesis that the rigidity is accompanied, at least in part of the efferent nuclei of the basal ganglia, by increases in LCGU antagonizing the inhibitory effects described above.     

亚急性坏死性基底节脑病(附50例临床分析)

目的探讨亚急性坏死性基底节脑病(SNBGE)的病因学、临床特征及其与Leigh's病的关系。方法对50例SNBGE的病因、临床特征、实验室检查及CT/MRI进行分析。结果50例SNBGE均因各种不同病因引起,呈亚临床急性发病,表现锥体外系及锥体系损害等多种多样的症状、体征,头部CT、MRI显示非梗死性双侧基底节对称性坏死、软化,治疗部分有效。结论头部CT、MRI可对SNBGE生前作出诊断,分为原发性(即Leigh's病)和继发性(病因有中毒、缺氧及感染等),临床需与肝豆状核变性、Hallervorden-spatz病(HSD)等鉴别。     

Bilateral, symmetrical hemorrhagic infarction of the basal ganglia and thalamus following neonatal asphyxia

With the advancement of perinatal intensive care, the occurrence of subependymal germinal matrix hemorrhage (= GMH) in low-birth-weight (premature) infants has became a major concern in perinatal medicine. The pathophysiology of the GMH has long been controversial. The introduction of computed tomographic (= CT) scanning to perinatal medicine has revealed various pathological events heretofore unknown in newborn infants having respiratory and circulatory distress. At our serving the entire Osaka Prefecture, infants suffering from birth asphyxia with severe perinatal brain damage were found to have CT findings distinguishable from those of GMH. We report three asphyxiated newborn infant who had hemorrhagic infarction in bilateral caudate nucleus, striatum and thalamus on the CT scan. Reports of similar findings are rare, and ours is the first serial observation of such CT scan image in newborn infants. The mechanism of development and pathology of this pathological condition have been variously argued as pathophysiology of GMH. The present study lacks postmortem examination, however, the findings in serial CT scans in three infants and review of the literatures related to the pathology of neonatal asphyxia indicate the following course. The thrombosis in the internal cerebral veins led to severe swelling of the brain, and hemorrhage occurred with the reduction in the swelling, eventually resulting in diffuse leukomalacia. Etat marbré (status marmoratus), mentioned earlier, is considered to represent a milder stage of this pathologic course.(ABSTRACT TRUNCATED AT 250 WORDS)     

外伤性基底节区血肿(附20例报告)

本文回顾性分析 2 0例外伤性基底节血肿 (traumaticbasalgangliahematomas,TBGHs) ,病情凶险 ,预后差。一、对象与方法1.临床资料 :1980年 1月至 2 0 0 0年 1月的 2 0年间 ,共收治 2 0例外伤性基底节血肿患者入院 ,占同期颅脑损伤 2 4 0 0例中的 1.2 %。其中男性 18例 ,女性 2例 ,年龄 14～ 4 5岁 ,平均年龄 2 8岁。外伤原因 :汽车事故 2例 ,摩托车事故 18例。所有病例格拉斯哥昏迷评分 (Glosgowcomascale ,GCS)≤ 6分 ,昏迷 2 0例 (10 0 % ) ,呼吸急促 9例 (45 % ) ,呕吐 10例(5 0 % ) ,肢体异常屈曲 11例 (5 5 % ) ,定位反应消失 19例…     

Traumatic basal ganglia haemorrhage: report of 7 cases.

We report 7 cases of traumatic basal ganglia haemorrhage (TBGH), which is closely related to diffuse axonal injury (DAI). Although most of our patients were in coma for more than 24 hours, the outcome was relatively favourable. All the patients regained consciousness, and 2 of 4 patients with contralateral hemiparesis showed improvement. Although expressive and nonfluent aphasia occurred in 5 patients, all of them recovered completely. The outcome in TBGH may be better than previously believed.     

Primate model of Parkinson''s disease: alterations in multiple opioid systems in the basal ganglia

A motor disorder similar to idiopathic Parkinson's Disease develops in rhesus monkeys after several daily repeated doses of N-methyl-4-phenyl, 1,2,3,6-tetrahydropyridine (MPTP). The concentrations of peptides derived from proenkephalin A, proenkephalin B, substance P and somatostatin were measured by specific radioimmunoassays in the basal ganglia of MPTP-treated monkeys. In MPTP-treated monkeys, dynorphin B concentration was reduced in the caudate. In the putamen, the concentrations of peptides derived from both proenkephalin A and proenkephalin B were decreased. In the globus pallidus, the concentrations of all opioid peptides tend to be increased, reaching significance only for alpha-neo-endorphin. In the substantia nigra, only Met-enkephalin concentration was reduced, while other peptides derived from either proenkephalin A or proenkephalin B were not changed. Substance P and somatostatin were not changed in any brain area examined. Some of the symptoms associated with Parkinson's Disease may be related to altered activity of endogenous opiates in basal ganglia.     

Defect in succinate oxidation by isolated muscle mitochondria in a patient with symmetrical lesions in the basal ganglia

A 3-year-old boy was referred for evaluation of psychomotor retardation. He had a waddling gait with proximal hypotonia and paresis. Computed tomography (CT scan) and magnetic resonance imaging (MRI) of the brain demonstrated symmetrical lesions in the basal ganglia suggesting bilateral necrosis. Lactate and pyruvate levels in blood and cerebrospinal fluid were persistently elevated. A biopsy of the quadriceps muscle showed normal light microscopic findings except for a slightly raised number of lipid droplets. Electron microscopy confirmed this and also showed a rather large number of subsarcolemmal mitochondria without crystalline inclusions. Biochemical studies showed a normal carnitine level and normal mitochondrial enzyme activities in muscle homogenate, including succinate-cytochrome c reductase. However, intact isolated mitochondria failed to oxidize succinate. An explanation for this paradoxical finding is a deficiency in that part of the coenzyme Q (CoQ) that is reduced by the succinate dehydrogenase complex. The differential diagnosis between Leigh's syndrome and infantile bilateral striatal necrosis (IBSN) is discussed. The role of neuroradiology in prompting complementary investigations is stressed.     

Electrical synapses in basal ganglia

The basal ganglia (BG) provide a major integrative system of the forebrain involved in the organization of goal-directed behaviour. Pathological alteration of BG function leads to major motor and cognitive impairments such as observed in Parkinson's disease. Recent advances in BG research stress the role of neural oscillations and synchronization in the normal and pathological function of BG. As demonstrated in several brain structures, these patterns of neural activity can emerge from electrically coupled neuronal networks. This review aims at addressing the presence, functionality and putative role of electrical synapses in BG, with a particular emphasis on the striatum and the substantia nigra pars compacta (SNc), two main BG nuclei in which the existence and functional properties of neuronal coupling are best documented.     

Structural alterations in brainstem,basal ganglia and thalamus associated with parkinsonism in schizophrenia spectrum disorders

European Archives of Psychiatry and Clinical Neuroscience - The relative roles of brainstem, thalamus and striatum in parkinsonism in schizophrenia spectrum disorder (SSD) patients are largely...     

手术全切岛叶基底节区胶质瘤21例报告

目的对岛叶基底节区胶质瘤的临床特征、手术切除效果进行分析。方法对近年来采用翼点入路手术切除的21例岛叶基底节区胶质瘤病人的临床表现、病理类型、影像学特征、手术及预后进行回顾性分析。结果本组均进行了肿瘤全切除,术后除2例病人出现暂时运动性失语,2例对侧肢体偏瘫外,均恢复良好,无严重的手术并发症及手术死亡。结论对岛叶基底节区胶质瘤可以进行全切除而没有或仅遗留轻微的神经功能缺失。     

Role of basal ganglia in saccades

The basal ganglia, substantia nigra pars reticulata (SNr) and caudate nucleus, contribute to the suppression and initiation of saccadic eye movements by imposing a tonic inhibition on the superior colliculus (SC) and by removing it. The tonic inhibition originates from the SNr where neurons show continuous, high frequency discharges. With its inhibitory connection to the SNr, the caudate nucleus transiently suppresses the SNr activity thereby removing the tonic inhibition on the SC. Signals carried by neurons in the SNr and the caudate nucleus are both heavily dependent on or selective for different aspects of learned paradigms. Such task-specific, learned information originating from the basal ganglia may open or close the gate for a variety of excitatory inputs to the SC.     

神经内镜治疗高血压基底核区出血（附32例分析）

目的总结神经内镜治疗高血压基底核区出血的手术经验。方法回顾性分析32例高血压脑基底核区出血病人的内镜手术临床资料。均采用自制透明内镜导管、固定装置,行单纯神经内镜下微创颅内血肿清除术。结果术后24h行头颅CT复查．显示血肿清除率为92％-96％,平均93．6％;术后无颅内感染、脑积水及死亡发生。术后随访3个月GOS评分：5分12例,4分16例,3分3例,2分1例。结论神经内镜手术清除基底核区出血具有微创、直视等优点;采用自制透明内镜导管及固定装置,使内镜清除血肿更具优越性。