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Linear scleroderma en coup de sabre with associated neurologic abnormalities.

Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. Our patients' cases highlight the underrecognized relationship between neurologic complications and linear scleroderma en coup de sabre and illustrate the importance of a thorough skin examination in patients with unexplained neurologic disease.

Bell Palsy↗

Magnetic resonance imaging in kainic acid-induced limbic seizure status in cats.

Magnetic resonance imaging before, during, and after kainic acid (KA)-induced limbic seizure status in cats demonstrated the bilateral hippocampi as slightly high-intensity areas on the T2-weighted images during the limbic seizure status, and isointensity areas 1-2 weeks after KA injection when the limbic seizure status subsided. However, the hippocampi again became high-intense 1-3 months after KA injection. Histological study suggested that the high-intensity area during the limbic seizure status resulted from regional edema, and in the chronic period from marked gliosis and/or atrophic change as a consequence of tissue damage in the hippocampus.

Amygdala↗

Visual activation positron emission tomography for presurgical evaluation of occipital lobe epilepsy--case report.

A 14-year-old boy suffered from daily epileptic seizures originating from the left polymicrogyric occipital cortex. Visual activation positron emission tomography (PET) was used to map the function of the occipital cortex presurgically. Loss of visual function in the left occipital cortex was suggested by both visual activation PET and electrical cortical stimulation. Left occipital lobectomy resulted in a completely seizure-free status without deterioration in the visual function. Preoperative evaluation of the visual function in the epileptogenic occipital cortex by activation studies using PET or functional magnetic resonance imaging is the key to the successful surgical treatment of occipital lobe epilepsy.

Adolescent↗

Propensity to report paranormal experiences is correlated with temporal lobe signs.

Bivariate correlation analyses indicated that people who reported greater numbers of different types of paranormal experiences also reported greater numbers of temporal lobe signs. Whereas responses of one group (n = 108) of male and female university students gave a correlation of 0.60 between the two measures, for another group (n = 41) the correlation was 0.72. Partial correlation analyses, which involved holding the shared variance with affirmative responses to mundane psychological statements or odd sensations constant, did not alter the strength of the relationship. These results support the hypothesis that spontaneous paranormal experiences and the psychological components of complex partial (psychomotor) epilepsy may exist along the same continuum of temporal lobe sensitivity.

Electroencephalography↗

Influence of premorbid personality and location of lesion on emotional expression.

Both premorbid personality characteristics and location of brain lesion have been proposed to account for emotional changes after cerebral damage. To explore the relative merits of each hypothesis, we compared the MMPI profiles of 30 epileptic patients, who showed either a euphoric or depressive reaction after injection of amobarbital into the right or left hemisphere, with the MMPI profiles of 30 matched controls. There were no significant T-score differences between euphoric, depressed, or control patients on any of the MMPI scales. However, more patients with euphoric (42%) than depressed (0%) reactions had hypomania (scale 9) scores greater than 70 T. With regard to location of lesion, there was a statistically significant association between emotional reaction and side of amobarbital brain inactivation. Euphoria was more frequent following right, and depression was more common after left, hemisphere inactivation. Although support was found for both premorbid personality and lesion location in the genesis of emotional reactions after brain damage, results were more robust for location of lesion.

Adult↗

Posterior fossa lesions associated with neuropsychiatric symptomatology.

We reviewed 7 cases with posterior fossa structural abnormalities (3 tumors, 2 megacisterna magna and 2 Dandy-Walker syndrome) presenting with neuropsychiatric symptomatology. Derangement in the balance of dopamine, serotonin and noradrenergic networks has been implicated in the pathogenesis of schizophrenia, affective and even personality disorders. Disruption of the cerebellar output to mesial dopaminergic areas, locus coeruleus and raphe nuclei, or deafferentation of the thalamolimbic circuits by a cerebellar lesion may lead to behavioral changes. Seven patients (pts) (comprising 4 men and 3 women with mean age 22 years) were diagnosed as suffering from psychosis (2 pts), major depression (1 pt), personality disorders (2 pts) and somatoform disorders (2 pts) (DSM-IV criteria). Brain CT scan (7 pts) and MRI (4 pts) revealed tumors of the posterior fossa (2 pts), megacisterna magna (2 pts) and Dandy-Walker variant (2 pts). In one patient a IVth ventricle tumor was removed in childhood.

Adult↗

A case of experiential hallucinations of unknown origins, or the mystery of the mime.

A case is described of repetitive familiar activities apparently accompanied by a hallucinated physical environment in an older brain-injured man with an amputation of the left temporal lobe. A parallel is drawn to the 'experiential hallucinations' described in psychomotor seizures and direct stimulation of the cortex.

Activities of Daily Living↗

Supratentorial-infraoccipital approach for posteromedial temporal lobe lesions.

The use of the supratentorial-infraoccipital approach is reported in seven patients with posteromedial temporal lobe lesions. No patient had permanent morbidity. Gross total resection of three low-grade gliomas and two gangliogliomas was achieved in five patients; one patient had subtotal resection of a low-grade glioma with adjacent gliosis, and one was initially thought to have a glioma but proved to have encephalomalacia on final pathological analysis. The patients ranged in age from 5 to 34 years. All seven patients presented with seizures, and four had uncontrolled seizures preoperatively. Six have been seizure-free since surgery (mean follow-up period 15 months), and one is well controlled on anticonvulsant medication. An anatomical study was performed to delineate the microsurgical anatomy relevant to operating on the medial temporal lobe through this posterior approach. A viewing wand intraoperative navigational system was utilized with this approach and proved helpful in gaining access as far anterior as the uncus through this occipital craniotomy. This approach is favorable in selected patients with posterior, medial, temporal lobe tumors because resection of otherwise difficult lesions may be accomplished without sacrificing lateral temporal lobe cortex or transecting the optic radiations.

Adolescent↗

Localization of language-specific cortex by using magnetic source imaging and electrical stimulation mapping.

OBJECT: In this paper the authors demonstrate the concordance between magnetic source (MS) imaging and direct cortical stimulation for mapping receptive language cortex. METHODS: In 13 consecutive surgical patients, cortex specialized for receptive language functions was identified noninvasively by obtaining activation maps aided by MS imaging in the context of visual and auditory word-recognition tasks. Surgery was then performed for treatment of medically intractable seizure disorder (eight patients), and for resection of tumor (four), or angioma (one). Mapping of language areas with cortical stimulation was performed intraoperatively in 10 patients and extraoperatively in three. Cortical stimulation mapping verified the accuracy of the MS imaging-based localization in all cases. CONCLUSIONS: Information provided by MS imaging can be especially helpful in cases of atypical language representation, including bihemispheric representation, and location of language in areas other than those expected within the dominant hemisphere, such as the anterior portion of the superior temporal gyrus, the posteroinferior portion of the middle temporal gyrus, the basal temporal cortex, and the lateral temporooccipital cortex.

Acoustic Stimulation↗

Supracerebellar transtentorial approach to posterior temporomedial structures.

The supracerebellar transtentorial (SCTT) approach, a modification of the infratentorial supracerebellar approach, facilitates simple and minimally invasive access to posterior temporomedial structures without requiring retraction of the temporal or occipital lobe. The SCTT approach was used in 16 patients over a 3-year period. Eleven patients harbored tumors confined to, or located mainly within, the posterior hippocampal formation, three patients harbored aneurysms (one ruptured posterior cerebral artery [PCA] aneurysm at the P2-P3 junction, one ruptured giant PCA [P2] aneurysm, and one giant basilar artery-superior cerebellar artery aneurysm), one patient had juvenile-type moyamoya disease, and one patient suffered from medically intractable epilepsy. In these patients, the SCTT approach enabled tumor removal, aneurysm clipping, and vascular bypass procedures. The authors' experience suggests that this approach can be used routinely in treating lesions in the posterior temporomedial region.

Adolescent↗

Rhabdoid meningioma occurring in an unrelated resection cavity with leptomeningeal carcinomatosis. Case report.

Rhabdoid meningioma (RM) is a recently described, aggressive variant of meningioma. The authors report a case of RM occurring in the resection cavity of an unrelated neurosurgical procedure, temporal lobectomy for intractable seizures. The patient presented with intractable headache 10 years after the temporal lobectomy. Imaging revealed a dura-based, uniformly enhancing lesion within the resection cavity. She underwent gross-total resection and the findings of the surgical pathological report were consistent with an RM, with a dramatically elevated MIB-1 index of approximately 50%. The patient's clinical course was complicated by severe pain and communicating hydrocephalus secondary to rapid dissemination of malignant cells throughout the CSF pathways. Despite aggressive measures, including tumor resection, ventriculoperitoneal shunt placement, and the initiation of conventional radiation therapy, the ensuing leptomeningeal carcinomatosis proved to be rapidly fatal.

Adult↗

Conduction aphasia as a function of the dominant posterior perisylvian cortex. Report of two cases.

Assessment of eloquent functions during brain mapping usually relies on testing reading, speech, and comprehension to uncover transient deficits during electrical stimulation. These tests stem from findings predicted by the Geschwind-Wernicke hypothesis of receptive and expressive cortices connected by white matter tracts. Later work, however, has emphasized cortical mechanisms of language function. The authors report two cases that demonstrate that conduction aphasia is cortically mediated and can be inadequately assessed if not specifically evaluated during brain mapping. To determine the distribution of language on the dominant cortex, electrical cortical stimulation was performed in two cases by using implanted subdural electrodes during brain mapping before epilepsy surgery. A transient isolated deficit in repetition of language was reported during stimulation of the posterior portion of the dominant superior temporal gyrus in one patient and during stimulation of the supramarginal gyrus in the other patient. These cases demonstrate a localization of language repetition to the posterior perisylvian cortex. Brain mapping of this region should include assessment of verbal repetition to avoid potential deficits resembling conduction aphasia.

Adult↗

Hypothalamic ganglioglioma treated by temporal lobectomy. Case report and review of the literature.

The authors report the case of a hypothalamic ganglioglioma with left-sided temporal lobe extension in an 8-year-old girl who presented with seizures. Other cases of ganglioglioma involving the hypothalamus have been reported in the literature; however, this site of origin is exceedingly rare and worthy of report. Treatment involved medial temporal lobectomy with the hypothalamic component of the tumor remaining untouched. The patient recovered postoperatively with no neurological deficits and was seizure free at 12 months. Neither radio- nor chemotherapy was recommended because of the tumor histology, location, and the patient's age. The authors recommend follow up and surgical treatment for possible tumor recurrence. The prognosis for hypothalamic ganglioglioma is unknown.

Anterior Temporal Lobectomy↗

Phenytoin toxicity with mandibular tremor secondary to intravenous administration.

The parenteral form of phenytoin is the most commonly used antiepileptic agent during the perioperative period in neurosurgery clinics. We report observations in a 52-year-old male patient with hypoalbuminemia and phenytoin intoxicity following 1 day preoperative administration and a 7-day postoperative intravenous administration of the drug with no modification of the oral dose. This report emphasizes the need for careful surveillance of phenytoin-induced toxicity during parenteral therapy, especially in debilitated patients.

Anticonvulsants↗