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1/118. Integration of preoperative and intraoperative functional brain mapping in a frameless stereotactic environment for lesions near eloquent cortex. Technical note.

    The authors present a method of incorporating preoperative noninvasive functional brain mapping data into the frameless stereotactic magnetic resonance (MR) imaging dataset used for image-guided resection of brain lesions located near eloquent cortex. They report the use of functional (f)MR imaging and magnetic source (MS) imaging for preoperative mapping of eloquent cortex in difficult cases of brain tumor resection such as those in which there are large expansive masses or in which reoperations are required and the anatomy is distorted from prior treatments. To correlate methods of preoperative and intraoperative mapping localization directly, the authors have developed techniques of importing preoperative MS and fMR imaging data into an image-guided frameless stereotactic computer workstation. The data appear as a seamless overlay on the same preoperative volumetric MR imaging dataset used for stereotactic guidance during the operation. Intraoperatively identified functional locations mapped by cortical stimulation are recorded as digitally registered points. This approach should prove useful in assessing the accuracy and reliability of various preoperative functional brain mapping techniques.
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2/118. Leukoencephalopathy with a mild clinical course: a case report.

    Infantile-onset leukoencephalopathy of van der Knaap type is manifested by initially normal or near normal neurological findings despite infantile-onset megalencephaly and magnetic resonance imaging evidence of severe white matter affection. Until this entity was recently described, these cases were usually presented under the heading of atypical variants of alexander disease. To date 63 individuals have been reported in English literature. We report a four-year-old boy presented in the first months of life with progressive megalencephaly, delay in walking, clumsiness, convulsions and magnetic resonance imaging evidence of diffuse swelling of white matter, cystic cavitations in frontal, temporal and parietal lobes.
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3/118. consciousness in congenitally decorticate children: developmental vegetative state as self-fulfilling prophecy.

    According to traditional neurophysiological theory, consciousness requires neocortical functioning, and children born without cerebral hemispheres necessarily remain indefinitely in a developmental vegetative state. Four children between 5 and 17 years old are reported with congenital brain malformations involving total or near-total absence of cerebral cortex but who, nevertheless, possessed discriminative awareness: for example, distinguishing familiar from unfamiliar people and environments, social interaction, functional vision, orienting, musical preferences, appropriate affective responses, and associative learning. These abilities may reflect 'vertical' plasticity of brainstem and diencephalic structures. The relative rarity of manifest consciousness in congenitally decorticate children could be due largely to an inherent tendency of the label 'developmental vegetative state' to become a self-fulfilling prophecy.
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4/118. Postencephalitic focal retrograde amnesia after bilateral anterior temporal lobe damage.

    BACKGROUND: Marked retrograde amnesia with no or almost no anterograde amnesia is rare. Recently, a combination of ventrolateral prefrontal and temporopolar cortical lesions has been suggested as the cause of such isolated or focal retrograde amnesia. It is also assumed that when the right-sided cortical structures are damaged, autobiographical episodic memories are affected. OBJECTIVE: To search for new anatomic substrates for focal retrograde amnesia. methods: We performed extensive neuropsychological tests and obtained detailed neuroimages on a 43-year-old woman who showed a severe, persistent retrograde amnesia but only a limited anterograde amnesia after probable herpes simplex encephalitis. RESULTS: Tests of autobiographical memory revealed that she had a memory loss extending back to her childhood for both semantics and incidents; however, the ability to recall specific episodes appeared much more severely impaired than the ability to recall factual information about her past. The patient also showed profound impairments in recalling public memories; however, her scores improved nearly to a control level on forced-choice recognition memory tasks, although the recall of memories for a decade just before her illness remained mildly impaired. MRI revealed focal pathologies in the temporal poles and the anterior parts of the inferotemporal lobes on both sides, predominantly on the left, with some extension to the anterior parts of the medial temporal lobes. There was additional damage to the left insular cortex and its surrounding structures but no evidence of frontal lobe damage on MRIs or cognitive tests. CONCLUSIONS: A profound retrograde amnesia may be produced by damage to the bilateral temporal poles and anterior inferotemporal lobes in the absence of frontal lobe pathologies, and a dense and persistent episodic old memory loss can arise even with a relatively small lesion in the right anterior temporal lobe if it is combined with extensive damage to the left.
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5/118. Familial fatal and near-fatal third ventricle colloid cysts.

    BACKGROUND: Despite having a presumed congenital origin, familial cases of colloid cysts have been reported only rarely. The first case of a brother and sister with colloid cysts is reported here, and the relevant literature is reviewed. methods: A 25-year-old man presented with a 24-h history of headache and vomiting. He rapidly became unconscious and fulfilled the criteria for brain death on arrival at hospital. No surgical intervention was performed. RESULTS: The patient's sister presented at the age of 41 with headaches and rapidly became unconscious. The sister had urgent bilateral ventriculostomies. followed by transcallosal removal of a colloid cyst. CONCLUSIONS: These cases support the hypothesis that colloid cysts are congenital lesions and provide some evidence of a possible genetic predisposition to their formation. Sudden death remains a real risk for patients harbouring a colloid cyst.
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6/118. 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.
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7/118. apoptosis in leukoaraiosis.

    We report a case of leukoaraiosis that was studied for apoptosis. In the neuropil, the number of cells that showed dna fragmentation was 2.5 times as great in the area of leukoaraiosis as in the adjacent white matter (P = .004) and 25 times as great as in the nearby cortex (P < .001). Our findings suggest that apoptosis, predominantly of oligodendrocytes, is involved in the pathogenesis of leukoaraiosis. Within the area of leukoaraiosis, we also found numerous small veins that were partially occluded by severe collagenous thickening of the vessel walls. This collagenosis may have contributed to or resulted from chronic ischemia in that area.
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8/118. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster-Drought syndrome.

    Foix-Chavany-Marie syndrome (FCMS) is a distinct clinical picture of suprabulbar (pseudobulbar) palsy due to bilateral anterior opercular lesions. Symptoms include anarthria/severe dysarthria and loss of voluntary muscular functions of the face and tongue, and problems with mastication and swallowing with preservation of reflex and autonomic functions. FCMS may be congenital or acquired as well as persistent or intermittent. The aetiology is heterogeneous; vascular events in adulthood, nearly exclusively affecting adults who experience multiple subsequent strokes; CNS infections; bilateral dysgenesis of the perisylvian region; and epileptic disorders. Of the six cases reported here, three children had FCMS as the result of meningoencephalitis, two children had FCMS due to a congenital bilateral perisylvian syndrome, and one child had intermittent FCMS due to an atypical benign partial epilepsy with partial status epilepticus. The congenital dysgenetic type of FCMS and its functional epileptogenic variant share clinical and EEG features suggesting a common pathogenesis. Consequently, an increased vulnerability of the perisylvian region to adverse events in utero is discussed. In honour of Worster-Drought, who described the clinical entity in children 40 years ago, the term Worster-Drought syndrome is proposed for this unique disorder in children.
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9/118. Anaesthetic management of a parturient with a colloid cyst of the third ventricle.

    A colloid cyst in the third ventricle near the foramen of Monroe can obstruct cerebrospinal fluid (CSF) flow from the lateral ventricles. Any change in the CSF pressure on either side of the cyst can lead to displacement and thus precipitate acute hydrocephalus. Management of the confinement of a patient with a colloid cyst must therefore aim to minimize changes in CSF pressure. We describe our management of a patient with a small colloid cyst who was permitted to labour with the assistance of patient-controlled epidural analgesia. The available alternatives are discussed.
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10/118. Unusual signs for dural arteriovenous fistulas with diffuse basal ganglia and cerebral calcification.

    We present a case of multiple dural arteriovenous fistulas (AVFs) in a 60-year-old man with the chief complaint of worsening headache, altered mental status and progressively unsteady gait over the course of one year. Computerized tomography revealed diffuse, symmetric calcification in the bilateral basal ganglia and bilateral periventricular and subcortical white matter. magnetic resonance imaging revealed multiple, enhanced, punctate and linear vessels. These images were due to reflux into the parenchymal veins in the dural AVF of the superior sagittal sinus within the basal ganglia and deep white matter of both cerebral hemispheres. cerebral angiography disclosed multiple dural AVFs. The exact mechanism of basal ganglia and subcortical calcification is proposed to be an arterial steal phenomenon or persistent venous congestion, with calcification occurring in a chronic hypoperfused state or with dystrophic changes in the walls of congested veins.
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