1/116. Intracranial Castleman's disease of solitary form. Case report.This 62-year-old woman presented with clumsiness in her right hand. magnetic resonance imaging demonstrated a small lesion mimicking a meningioma, which had arisen from the tentorium and contained notable edema. Full recovery was achieved by total removal of the lesion, which was diagnosed as a lymphoid mass resembling giant lymph node hyperplasia on histological examination. The lack of notable findings on whole-body and laboratory studies was compatible with a rare case of intracranial Castleman's disease of solitary form. The authors document clinical, neuroradiological, and pathological features of this rare disease.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
2/116. Chronic toxic encephalopathy in a painter exposed to mixed solvents.This paper describes symptoms and findings in a 57-year-old painter who had been exposed to various organic solvents for over 30 years. He began to work as a painter at 16 years of age, frequently working in poorly ventilated areas; he used solvents to remove paint from the skin of his arms and hands at the end of each work shift. The patient and his family noticed impaired short-term memory function and changes in affect in his early forties, which progressed until after he stopped working and was thus no longer exposed to paints and solvents. After the patient's exposures had ended, serial neuropsychological testing revealed persistent cognitive deficits without evidence of further progression, and improvement in some domains. magnetic resonance imaging revealed global and symmetrical volume loss, involving more white than gray matter. The findings in this patient are consistent with chronic toxic encephalopathy and are differentiated from other dementing processes such as Alzheimer's disease, multi-infarct (vascular) dementia, and alcoholic dementia. Previous descriptions in the literature of persistent neurobehavioral effects associated with chronic exposure to organic solvents corroborate the findings in this case.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
3/116. status epilepticus-induced brain damage and opercular syndrome in childhood.This study reports on a girl with a permanent cerebral lesion and opercular syndrome after status epilepticus (SE). She had previously been healthy and had her first focal motor seizure at 5 years of age, which was controlled with intravenous phenytoin and rectal diazepam. Twenty-four hours later, she developed partial SE consisting of right facial twitching and right-hand clonic movements. These uncontrollable seizures lasted for 5 days, after which the partial SE changed to generalized SE, and the seizures continued for another 5 days. CT performed the day before onset of SE revealed no brain abnormality. Another CT performed a year later disclosed bilateral brain lesions, more severe in the left hemisphere. Follow up at 16 years of age revealed moderate motor sequelae of the right-hand side of the body, anarthria, difficulty chewing, dysphagia, bilateral facial weakness, and drooling, all of which clinically characterize opercular syndrome. An MRI study performed at 14 years of age showed a cerebral parenchymatous lesion which extended between the parietal cortices of both hemispheres, more severe on the left side, and which crossed the corpus callosum, destroying the posterior-middle zone. Evidence from the CT indicates that the lesion was not present before onset of SE. It seems likely that the focal SE caused the focal brain damage, but the possibility that the subsequent generalized SE played a role cannot be excluded.- - - - - - - - - - ranking = 2keywords = hand (Clic here for more details about this article) |
4/116. Leukoencephalopathy and raised brain lactate from heroin vapor inhalation ("chasing the dragon")BACKGROUND: inhalation of heated heroin vapor ("chasing the dragon"), which is gaining popularity among drug users seeking to avoid the risks of parenteral drug administration, can produce progressive spongiform leukoencephalopathy. methods: We studied the clinical phenotype and course, MRI, MRS, and brain pathology in the first American patients described with this syndrome. RESULTS: Two of the three heroin users studied inhaled heroin pyrolysate together daily over the course of 2 weeks. They developed ataxia, dysmetria, and dysarthria. Patient 1 progressed to an akinetic mute state with decorticate posture and subsequent spastic quadriparesis. Patient 2 developed a mild spastic quadriparesis and gait freezing. Patient 3 was asymptomatic following less heroin exposure. brain MRI showed diffuse, symmetrical white matter hyperintensities in the cerebellum, posterior cerebrum, posterior limbs of the internal capsule, splenium of the corpus callosum, medial lemniscus, and lateral brainstem. MRS showed elevated lactate. brain biopsy (Patient 1) showed white matter spongiform degeneration with relative sparing of U-fibers; electron microscopy revealed intramyelinic vacuolation with splitting of intraperiod lines. Progressive deterioration occurred in patients 1 and 2 over 4 weeks. Both were treated with antioxidants including oral coenzyme Q, and clinical improvement occurred. Patient 1 recovered nearly completely over 24 months. Patient 2 improved, but developed a delayed-onset cerebellar hand tremor. Both still have white matter abnormalities on MRI and MRS. CONCLUSIONS: Elevated lactate in white matter and the possible response to antioxidants suggests mitochondrial dysfunction in progressive spongiform leukoencephalopathy following inhalation of heated heroin vapor.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
5/116. Subjective experience, involuntary movement, and posterior alien hand syndrome.The alien hand syndrome, as originally defined, was used to describe cases involving anterior corpus callosal lesions producing involuntary movement and a concomitant inability to distinguish the affected hand from an examiner's hand when these were placed in the patient's unaffected hand. In recent years, acceptable usage of the term has broadened considerably, and has been defined as involuntary movement occurring in the context of feelings of estrangement from or personification of the affected limb or its movements. Three varieties of alien hand syndrome have been reported, involving lesions of the corpus callosum alone, the corpus callosum plus dominant medial frontal cortex, and posterior cortical/subcortical areas. A patient with posterior alien hand syndrome of vascular aetiology is reported and the findings are discussed in the light of a conceptualization of posterior alien hand syndrome as a disorder which may be less associated with specific focal neuropathology than are its callosal and callosal-frontal counterparts.- - - - - - - - - - ranking = 34634.615447753keywords = alien hand syndrome, hand syndrome, alien hand, alien, hand (Clic here for more details about this article) |
6/116. Planning and simulation of neurosurgery in a virtual reality environment.OBJECTIVE: To report our experience with preoperative neurosurgical planning in our stereoscopic virtual reality environment for 21 patients with intra- and extra-axial brain tumors and vascular malformations. methods: A neurosurgical planning system called VIVIAN (Virtual Intracranial Visualization and Navigation) was developed for the Dextroscope, a virtual reality environment in which the operator reaches with both hands behind a mirror into a computer-generated stereoscopic three-dimensional (3-D) object and moves and manipulates the object in real time with natural 3-D hand movements. Patient-specific data sets from multiple imaging techniques (magnetic resonance imaging, magnetic resonance angiography, magnetic resonance venography, and computed tomography) were coregistered, fused, and displayed as a stereoscopic 3-D object. A suite of 3-D tools accessible inside the VIVIAN workspace enabled users to coregister data, perform segmentation, obtain measurements, and simulate intraoperative viewpoints and the removal of bone and soft tissue. RESULTS: VIVIAN was used to plan neurosurgical procedures primarily in difficult-to-access areas, such as the cranial base and the deep brain. The intraoperative and virtual reality 3-D scenarios correlated well. The VIVIAN system substantially contributed to surgical planning by 1) providing a quick and better understanding of intracranial anatomic and abnormal spatial relationships, 2) simulating the craniotomy and the required cranial base bone work, and 3) simulating intraoperative views. CONCLUSION: The VIVIAN system allows users to work with complex imaging data in a fast, comprehensive, and intuitive manner. The 3-D interaction of this virtual reality environment is essential to the efficient assembly of surgically relevant spatial information from the data derived from multiple imaging techniques. The usefulness of the system is highly dependent on the accurate coregistration of the data and the real-time speed of the interaction.- - - - - - - - - - ranking = 2keywords = hand (Clic here for more details about this article) |
7/116. Familial systemic lupus erythematosus and congenital infection-like syndrome.We present two siblings with congenital and progressive encephalopathy associated with systemic lupus erythematosus. The two brothers presented soon after birth with an encephalopathy associated with intracranial calcification (=2), intrauterine growth retardation (= 2), hepatitis (= 1) and thrombocytopenia (= 1), mimicking a congenital virus infection. Within the first year of life both children developed hypocomplementaemia and systemic lupus erythematosus (SLE), the main features of which were a discoid lupus-like rash on the hands and feet and the progressive production of high levels of autoantibodies. Both children were severely handicapped and died in early childhood from streptococcal infections. There are many causes of congenital encephalopathy with intracranial calcification. The early development of systemic lupus in these children suggested that their cerebral disease formed part of an autoimmune process. Complement levels and autoantibody profiles should be considered part of the investigation of a child with congenital infection-like syndrome, particularly when there are progressive dermatological complications.- - - - - - - - - - ranking = 2keywords = hand (Clic here for more details about this article) |
8/116. Loss of left-sided volitional movements caused by a combined lesion of the corpus callosum and right hemisphere:'initiation pseudohemiakinesia'.We report a right-handed patient who showed a marked loss of unilateral volitional movements of the left limbs after the onset of a cerebral infarction in the combined territories of the right anterior and middle cerebral arteries. The same limbs retained their mobility in acts requiring bilateral sides of the body. This left-sided abnormal behavior resembled motor neglect resulting from lateralized brain damage. Behavioral and neuroradiological findings presented by this patient, however, suggested that callosal disconnection was definitely involved in this symptomatology. We postulate that in this patient, the diseased right hemisphere could no longer initiate movements of the left limbs despite its potential ability to realize them, and that the injured callosum prevented the intact left hemisphere from initiating unilateral voluntary movements of the left limbs. We suggest that this so far undescribed symptomatology be called 'initiation pseudohemiakinesia' in order to be distinguished from other rare forms of unilateral voluntary movements like motor neglect, extinction or directional hypokinesia.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
9/116. Developmental lesions of visual cortex influence control of reaching.We examined visually guided reaching movements in a young adult (EW) who had extensive bilateral lesions in the visual cortex since birth. EW lacked a right occipital lobe and ventral portions of the left and had poor visual acuity (3/400), yet could point to visual targets as quickly as 9 controls with visual cortex lesions acquired in adulthood and 4 adults without neurological disease. However, EW's endpoint variability and hand movement path curvature were much greater, especially for left hand movements, in concert with large sensorimotor transformation errors. Experimental reduction of acuity (to 3/240 or worse) in the normal controls produced symmetric increases in endpoint variability but did not change hand path curvature, indicating that EW's impaired movements were not due to poor vision alone. Results suggest that visual cortex in early life supports the development of lifelong neural mechanisms for the planning and control of reaching movements.- - - - - - - - - - ranking = 3keywords = hand (Clic here for more details about this article) |
10/116. Functional neuroradiologic investigations in band heterotopia.Band heterotopias are an example of genetic generalized neuronal migration disorders that may be present in patients with mild epilepsy and normal or slightly impaired intellect, as well as in patients with intractable epilepsy and mental retardation. The case of a 17-year-old left-handed female patient with epilepsy and normal cognitive development is reported in whom single-photon emission computed tomography (SPECT), proton magnetic resonance spectroscopy, and functional magnetic resonance imaging (fMRI) were performed. MRI revealed the presence of bilateral asymmetric band heterotopia. SPECT revealed a left frontoparietal and occipital hypoperfusion, demonstrating a good correlation with the electroencephalogram abnormalities. Because of the appearance of new types of seizures, the patient underwent a second MRI investigation together with a proton magnetic resonance spectroscopy (MRS) study. MRI confirmed bilateral band heterotopia characterized by greater thickness in the left hemisphere at the frontal and occipital level. MRI and SPECT findings were in agreement with left occipital electroencephalogram abnormalities and with occipital seizure type. Qualitative results of proton MRS revealed normal spectra profiles in the examined left frontal and occipital heterotopic area and in the normal overlying cortex. Later, fMRI was performed. The finger-tapping test of the right hand yielded the activation of both normal left sensory-motor cortex and the facing band heterotopia. In the right hemisphere, only the activation of the sensory-motor neocortex was observed; no involvement of the right misplaced brain tissue was present. This functional behavior could be considered the consequence of poor neuronal representation. On the contrary, the involvement of both band heterotopia and normal cortex observed in the left hemisphere could be the result of many synaptic interconnections. Functional investigations may have an important role in defining the activity of band heterotopia per se and in relation to the overlying neocortex.- - - - - - - - - - ranking = 2keywords = hand (Clic here for more details about this article) |
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