Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions.

We report on two patients with unilateral hyperkinetic movement disorders associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. The first patient, a 47-year-old woman, had a low-grade astrocytoma located in the right basal ganglia extending into the subthalamic area and the cerebral peduncle. She presented with left hemiparesis, right hemichorea, and intermittent right-sided tremor at rest. The second patient, a 85-year-old woman, had hypertensive hemorrhage to the right posterior basal ganglia, the posterior limb of the internal capsule, the lateral thalamus, and the subthalamic region with accompanying intraventricular bleeding. She developed right-sided transient hemichorea-hemiballism. A videotape illustration of one of the patients is provided. The literature on the rare occurrence of ipsilateral hemichorea-hemiballism is discussed and possible pathomechanisms are reviewed. We postulate that hemiparesis contralateral to basal ganglia lesions might have a conditioning effect on the appearance of ipsilateral dyskinetic movement disorders.     

A magnetic resonance imaging study of putamen nuclei in major depression.

The basal ganglia are recognized as putative mediators of certain cognitive and behavioral symptoms of major depression. Moreover, patients with basal ganglia lesions have repeatedly exhibited significant affective symptomatology, including apathy, depressive mood, and psychosis. Using high resolution, axial T2 intermediate magnetic resonance images, and a systematic sampling stereologic method, we assessed putamen nuclei volumes in 41 patients with major depression (DSM-III) and 44 healthy volunteer controls of similar age. Depressed patients had significantly smaller putamen nuclei compared with controls. Age was negatively correlated with putamen size in both groups. These results are the first demonstration of diminished putamen volumes in depression and further support a role for basal ganglia structures in the etiopathogenesis of depression.     

Depression after stroke: the importance of cerebral hemisphere asymmetries.

The pattern of brain asymmetries was visualized on computed tomography (CT) scan in patients with a single acute cerebrovascular lesion. Patients were divided into those with typical or reversed frontal and/or occipital asymmetries. Among patients with a typical occipital asymmetry, those with left frontal or left basal ganglia lesions showed a significantly higher frequency of major depression and significantly higher depression scores than patients with similar lesion location but with reversed occipital asymmetry or those with a typical asymmetry and lesions in other (left or right) brain areas. Among patients with a reversed occipital asymmetry, there was no significant association between left frontal or left basal ganglia lesions and depression. This study demonstrates that the previously reported significant association between post-stroke major depression and lesion location is restricted to patients with a typical occipital asymmetry and is not present in patients with a reversed occipital asymmetry.     

A diffusion tensor MRI study of basal ganglia from patients with ADEM

Using diffusion tensor (DT) MRI and histogram analysis, we measured mean diffusivity ((-)D) of basal ganglia grey matter (GM) from eight patients with acute disseminated encephalomyelitis (ADEM), 10 patients with multiple sclerosis (MS), and 10 healthy controls. Patients with ADEM had higher average (-)D (p=0.02) and lower (-)D histogram peak height (p=0.008) of the basal ganglia GM than patients with MS. Microscopic tissue damage occurs in the basal ganglia of ADEM patients, but not in MS patients with a similar burden of MRI-visible brain lesions.     

Focal putamen lesions impair learning in rule-based, but not information-integration categorization tasks

Previous research on the role of the basal ganglia in category learning has focused on patients with Parkinson's and Huntington's disease, neurodegenerative diseases frequently accompanied by additional cortical pathology. The goal of the present study was to extend this work to patients with basal ganglia lesions due to stroke, asking if similar changes in performance would be observed in patients with more focal pathology. Patients with basal ganglia lesions centered in the putamen (6 left side, 1 right side) were tested on rule-based and information-integration visual categorization tasks. In rule-based tasks, it is assumed that participants can learn the category structures through an explicit reasoning process. In information-integration tasks, optimal performance requires the integration of information from two or more stimulus components, and participants are typically unaware of the category rules. Consistent with previous studies involving patients with degenerative disorders of the basal ganglia, the stroke patients were impaired on the rule-based task, and quantitative, model-based analyses indicate that this deficit was due to the inefficient application of decision strategies. In contrast, the patients were unimpaired on the information-integration task. This pattern of results provides converging evidence supporting a role of the basal ganglia and, in particular, the putamen in rule-based category learning.     

The impact of vascular burden on late-life depression

Small vessel pathology and microvascular lesions are no longer considered as minor players in the fields of cognitive impairment and mood regulation. Although frequently found in cognitively intact elders, both neuroimaging and neuropathological data revealed the negative impact on cognitive performances of their presence within neocortical association areas, thalamus and basal ganglia. Unlike cognition, the relationship between these lesions and mood dysregulation is still a matter of intense debate. Early studies focusing on the role of macroinfarct location in the occurrence of post-stroke depression (PSD) led to conflicting data. Later on, the concept of vascular depression proposed a deleterious effect of subcortical lacunes and deep white matter demyelination on mood regulation in elders who experienced the first depressive episode. More recently, the chronic accumulation of lacunes in thalamus, basal ganglia and deep white matter has been considered as a strong correlate of PSD. We provide here a critical overview of neuroimaging and neuropathological sets of evidence regarding the affective repercussions of vascular burden in the aging brain and discuss their conceptual and methodological limitations. Based on these observations, we propose that the accumulation of small vascular and microvascular lesions constitutes a common neuropathological platform for both cognitive decline and depressive episodes in old age.     

Organic psychoses. Delusional disorders and secondary mania

Organic delusional disorders and secondary mania are clinical syndromes produced by neurologic diseases and toxic-metabolic disorders. Delusional disorders are associated primarily with limbic system lesions and basal ganglia dysfunction. These two groups of structures are united into a single system involved in the assessment of ongoing perception and experience, and dysfunction in these structures may explain the presence of psychosis. Lesions producing delusional disorders involve dopaminergic projections considered important in idiopathic schizophrenia, and involvement of similar anatomic and biochemical systems may explain the identity of symptoms noted in both disorders. Secondary mania is associated with deep midline lesions and with pharmacologic agents affecting monoaminergic function. Midline lesions may involve reward-system nuclei and euphoriant enkephalins to produce an elevated mood and hypothalamic nuclei to produce alterations in sleep, appetite, and libido. Mania appears to be more common with right-sided than with left-sided lesions. Lesions, metabolic disturbances, and drugs causing mania may disturb serotonergic and noradrenergic transmitters implicated in idiopathic bipolar disease. Study of the organic psychoses is providing insight into the neurophysiologic basis of idiopathic psychotic disorders. Clinically, greater awareness of the organic psychoses may lead to the discovery of additional cases of secondary mania and organic delusional disorders with potentially treatable neuromedical causes.     

Tremor and dystonia after penetrating diencephalic-mesencephalic trauma

Isolated post-traumatic basal ganglia or brainstem lesions secondary to direct injury resulting in movement disorders, such as tremor or dystonia, are extremely rare. We report an unusual case with a 4-5 Hz rest and postural tremor and focal action-induced dystonia of the left arm. The movement disorder developed within 2 years of the patient sustaining a right-sided mesencephalic-diencephalic lesion due to a penetrating thrust injury with a screwdriver. Magnetic resonance imaging demonstrated involvement of the cerebral peduncle, the substantia nigra, the subthalamic region and the thalamus.     

The neurobiological substrates of behavioral manifestations during temporal lobe seizures: A neuroethological and ictal SPECT correlation study

Ictal behavior coupled with SPECT findings during 28 seizures in patients with temporal lobe epilepsy (TLE) with unilateral hippocampal sclerosis (13 left; 15 right) was displayed as flowcharts from right-sided (RTLE) plus left-sided (LTLE) seizures. Ictal SPECT was classified blind to neuroethology. Behaviors were categorized as ipsilateral to the epileptogenic zone (IL), contralateral to the epileptogenic zone (CL), or bilateral. SPECT intensity and region were categorized as IL or CL to the epileptogenic zone. All patients developed automatisms and had hyperperfusion in their temporal lobes. Patients’ verbal responses to questions had statistical interactions in RTLE but not in LTLE sum. Most CL dystonic posturing was correlated to IL basal ganglia hyperperfusion. Basal ganglia activation occurred in seizures without dystonic posturing and CL manual automatisms, and lack of IL dystonic posturing and the presence of CL cerebellar hemispheric hyperperfusion were also observed. Coupling of neuroethology and SPECT findings reliably evaluates ictal behavior and functionality of associated brain areas.     

Cysticercosis lesions in basal ganglia are common but clinically silent.

Movement disorders due to basal ganglia involvement by neurocysticercosis are rarely seen. To evaluate the frequency of basal ganglia location of cysticercotic cysts and its clinical manifestations, baseline MRI scans of 120 consecutive patients with active neurocysticercosis were reviewed and the presence and number of active cysticercosis lesions (viable cysts or enhancing lesions) in the basal ganglia were recorded and correlated with demographic and clinical data. Basal ganglia involvement was found in 32 cases (26.7%). The frequency of lesions in basal ganglia was related to the total number of lesions, ranging from 5% of patients with a single cysticerci, to 60% in patients with more than five parasites. Putamen and caudate nuclei were the most frequent sites of lesions. No significant difference between both hemispheres was observed. Basal ganglia localization is common in neurocysticercosis but it is rarely associated with clinical manifestations.     

Primary CNS lymphoma presenting as a choreic movement disorder followed by segmental dystonia

Clinical presentation of primary CNS lymphoma with an extrapyramidal movement disorder has not been recorded. A 66-year-old woman presented with chorea involving her left arm and subsequently developed right-sided segmental dystonia with prominent hemifacial dystonic spasms, milder torticollis and dystonia of the right arm. Investigations revealed primary CNS lymphoma with extensive involvement of the right-sided basal ganglia as well as lesions confined to the head of the left caudate nucleus and the corpus callosum. Chorea of her left arm subsided with progressing disease while remission of right-sided segmental dystonia was observed following radiotherapy of the brain. This patient's findings and a review of the literature suggest a possible relation between cranio-cervical dystonia and pathology affecting the head of the caudate nucleus.     

非酮症性高血糖合并舞蹈症6例临床分析

目的探讨非酮症性高血糖合并舞蹈症患者的临床、影像学特点及随访结果。方法对6例非酮症性高血糖合并舞蹈症患者的临床资料、出院后随访结果进行回顾性分析。结果患者的平均起病年龄为(65.83±13.93)岁,其中女性患者5例,男性1例;急性-亚急性病程,患者表现为单侧(n=5例)或双侧肢体(n=1)舞蹈样动作,面部肌肉均有受累(n=6)。入院时静脉血糖平均值为:(9.21±5.15)mmol/L;所有患者的糖化血红蛋白显著升高:(14.1%±3.5%)。所有患者的MRI均提示基底节区T1序列高信号病灶,均累及壳核,可同时合并豆状核、尾状核受累;3例患者磁敏感成像(SWI)序列发现,对应的基底节区病灶为低信号病灶。2例患者发现大脑中动脉重度狭窄。5例患者平均随访(16.2±7.2)个月,3例患者死亡。结论颅脑MRI示基底节区T1加权序列高信号病灶是C-H-BG主要特点;患者可合并对应的SWI序列的低信号病灶。     

Asymmetric dystonia with frontal white matter lesions in Wilson's disease

Asymmetrical manifestation of dystonia was investigated in 26 patients with Wilson's disease with modified dystonia rating scale and correlated with the asymmetricity of CT and/or MRI findings. Ten patients (eight men and two women) had dystonia. The age of disease onset ranged from 11 to 30 years with a mean of 18.6 ± 5.2 years. The duration of the illness was from 2 to 15 years (mean 7.0; S.D. 3.6). Six patients had subcortical white matter lesions in the frontal and/or parietal lobes, as well as lesions in the basal ganglia. Five patients, who had asymmetrical white matter lesions in the frontal lobes and symmetrical lesions in the basal ganglia and the thalamus, developed more severe contralateral dystonia. The other four patients with symmetric lesions in the basal ganglia and the thalamus had nearly-symmetrical dystonia. One patient with symmetrical lesions in the frontal lobes and the basal ganglia also had symmetrical manifestation of dystonia. We suggest that the interruption of the loop between the thalamus and premotor cortex by subcortical white matter lesions may enhance contralateral dystonia.     

Stereotactic radiosurgery for deep intracranial arteriovenous malformations,part 2: Basal ganglia and thalamus arteriovenous malformations

The aim of this review is to critically analyze the outcomes following stereotactic radiosurgery (SRS) for arteriovenous malformations (AVM) of the basal ganglia and thalamus. The management of these deep-seated lesions continues to challenge neurosurgeons. Basal ganglia and thalamic AVM show a higher risk of hemorrhage, and an associated devastating morbidity and mortality, as compared to AVM in more superficial locations. Any of the currently available treatment modalities may fail or result in iatrogenic neurologic deterioration. Recent evidence from A Randomized Trial of Unruptured Brain AVM (ARUBA) further deters aggressive approaches that carry a significant risk of treatment-related adverse events. Microsurgical resection, endovascular embolization and SRS all play a role in the treatment of AVM. SRS is an effective therapeutic option for AVM of the thalamus and basal ganglia that are deemed high risk for resection. SRS offers acceptable obliteration rates, with generally lower risks of hemorrhage occurring during the latency period compared to the AVM natural history. Considering that incompletely obliterated lesions still harbor the potential for rupture, additional treatments such as repeat SRS and microsurgical resection should be considered when complete obliteration is not achieved by an initial SRS procedure. Patients with AVM of the basal ganglia and thalamus require continued clinical and radiologic observation and follow-up after SRS, even after angiographic obliteration has been confirmed.     

Olfactory event-related potentials in patients with brain tumors.

OBJECTIVE: The aim of the study was to determine how odor processing is altered in patients with unilateral supratentorial brain tumors. METHODS: Olfactory event-related potentials (OERPs) were evaluated in 10 patients with unilateral brain tumors of the frontal or temporal lobe in response to linalool and allylcaproate. Both odors were presented monorhinally by a constant-flow olfactometer. In addition, 20 healthy subjects were examined. While sniffing, the subjects were asked to discriminate the two odors. EEG was recorded from 7 electrode positions (Fz, Cz, Pz, F3/4, P3/4). Amplitudes and latencies of 3 peaks (N1, P2, P3) were measured. To control for effects of modality-non-specific alterations on the olfactory components acoustic event-related potentials (AERPs) were registered by use of an oddball paradigm. RESULTS: Patients with right-sided lesions showed distinct deficits in the discrimination task after stimulation of the right and left nostril. In contrast, patients with left-sided lesions only had an attenuation of correct reactions after left-sided stimulation. In the OERPs, patients with right-sided lesions showed P2- and P3-components with decreased amplitudes at parietal electrode positions. These alterations appeared after ipsi- and contralateral stimulation. Patients with left-sided lesions showed a significant effect of the side of stimulation. Their OERP-amplitudes were decreased after left-sided stimulation but not after right-sided stimulation. After right-sided olfactory stimulation a correlation between the olfactory and the acoustic ERP was seen in patients with right-sided lesions. CONCLUSIONS: Olfactory performance of the participating patients was markedly reduced. Patients with right-sided lesions showed bilateral impairment, which would support the importance of the right hemisphere in olfaction. The alteration of the topographic distribution of P2- and P3-amplitudes in patients with right-sided lesions might reflect an impairment of early and late olfactory processing steps.     

Two sisters with generalized dystonia associated with homocystinuria

Two sisters with progressive dystonic syndromes and homocystinuria are presented. The biochemical defect was not accompanied by the typical clinical features of homocystinuria. Magnetic resonance imaging (MRI) revealed bilateral lesions of the basal ganglia. Homocystinuria should be considered among the causes of symptomatic or secondary dystonias associated with basal ganglia lesions.     

An fMRI study of affective state and medication on cortical and subcortical brain regions during motor performance in bipolar disorder

Structural neuroimaging studies have identified abnormalities in the basal ganglia in patients with bipolar disorder. Findings have been mixed with regard to affective state and have not elaborated on the role of medication on functional brain activity. The aims of the present study were to use functional magnetic resonance imaging (fMRI) to test whether depressed and manic bipolar disorder patients differ in terms of activity in cortical and subcortical brain areas and to examine the effects of psychotropic medication. Twenty-four bipolar disorder subjects and 13 healthy comparison subjects participated in an fMRI study of manual reaction time. Both manic and depressed subjects exhibited abnormally elevated blood oxygen level dependent BOLD responses in cortical and subcortical areas. Manic bipolar subjects had significantly higher BOLD responses in the left globus pallidus and significantly lower BOLD responses in the right globus pallidus compared with depressed bipolar patients. Correlational analyses revealed significant relationships between the severity of mania and activity within the globus pallidus and caudate. Patients off antipsychotic or mood-stabilizing medication exhibited significantly higher BOLD responses throughout the motor cortex, basal ganglia and thalamus compared with patients on these medications. These results suggest that affective state in bipolar disorder may be related to a disturbance of inhibitory regulation within the basal ganglia and that antipsychotics and/or mood stabilizers normalize cortical and subcortical hyperactivity.     

Cognitive functioning in neurologically symptomatic and asymptomatic forms of Wilson's disease.

We sought to determine the pattern of cognitive deficits in patients with Wilson's disease (WD) with different type and degree of neurological involvement, and to interpret the findings in relation to the underlying pathology. A total of 67 WD patients were examined with a neuropsychological test battery assessing different aspects of cognitive processing. The patients were subdivided into three groups: neurologically asymptomatic, neurological with pure basal ganglia lesions, and neurological with more extensive pathology. The results were compared with 50 matched healthy controls. Patients with a neurological form of WD showed a mild but definitive impairment in all cognitive functions. In contrast, the neurologically asymptomatic patients showed no deficits when compared with normal controls. Multifocal pathology was associated with more severe cognitive deficits than selective basal ganglia lesions but did not contribute significantly to memory impairment. A range of cognitive functions, including frontal-executive ability, aspects of memory and visuospatial processing, are affected in the neurologically symptomatic WD patients. In contrast, no subliminal deficits were observed in the asymptomatic patients. The lesions of the basal ganglia seem to be of central importance in explaining the symptomatology.     

Neuroanatomic correlates of psychopathologic components of major depressive disorder

BACKGROUND: The Hamilton Depression Rating Scale (HDRS) is widely used to measure the severity of depression in mood disorders. Total HDRS score correlates with brain metabolism as measured by fludeoxyglucose F 18 ([(18)F]-FDG) positron emission tomography. The HDRS comprises distinct symptom clusters that may be associated with different patterns of regional brain glucose metabolism. OBJECTIVE: To examine associations between HDRS component psychopathologic clusters and resting glucose cerebral metabolism assessed by [(18)F]-FDG positron emission tomography.Patients We evaluated 298 drug-free patients who met the DSM-III-R criteria for major depressive disorder. MAIN OUTCOME MEASURES: Five principal components were extracted from the 24-item HDRS for all subjects and ProMax rotated: psychic depression, loss of motivated behavior, psychosis, anxiety, and sleep disturbance. The [(18)F]-FDG scans were acquired in a subgroup of 43 drug-free patients in twelve 5-minute frames. Voxel-level correlation maps were generated with HDRS total and factor scores. RESULTS: Total HDRS score correlated positively with activity in a large bilateral ventral cortical and subcortical region that included limbic, thalamic, and basal ganglia structures. Distinct correlation patterns were found with the 3 individual HDRS factors. Psychic depression correlated positively with metabolism in the cingulate gyrus, thalamus, and basal ganglia. Sleep disturbance correlated positively with metabolism in limbic structures and basal ganglia. Loss of motivated behavior was negatively associated with parietal and superior frontal cortical areas. CONCLUSIONS: Different brain regions correlate with discrete symptom components that compose the overall syndrome of major depression. Future studies should extend knowledge about specific regional networks by identifying responsible neurotransmitters related to specific psychopathologic components of mood disorders.     

Mood state and cerebral metabolism in persons with age-associated memory impairment

People undergoing medical procedures sometimes experience feelings that may influence the results. In this study, we explore the relationship between changes in mood state self-ratings and cerebral glucose metabolism during positron emission tomography (PET) in persons with age-associated memory impairment (mean age 59.4±9.8 years). Brain regions of interest involved in both mood and memory were examined. Mood ratings of increased boredom correlated significantly with mesial temporal and parietal asymmetry and decreased parietal metabolism. Mood ratings of increased fatigue correlated with basal ganglia asymmetry and the right basal ganglia and left mesial temporal metabolism. These findings suggest that subjective mood state changes during PET may influence metabolism in brain regions implicated in emotion and memory function in people with age-related memory complaints.