1/136. Does the tuberous sclerosis complex include intracranial aneurysms? A case report with a review of the literature.BACKGROUND: tuberous sclerosis is a protean, genetically determined disease that may involve any organ or tissue and lead to a great number of symptoms and clinical features. OBJECTIVE: diagnosis can be very difficult in cases with incomplete manifestations (formes fruste) lacking the classic signs of the disease. MATERIALS AND methods: We report a case fulfilling the diagnostic criteria for tuberous sclerosis (shagreen patches, hypomelanotic macules, renal cysts and angiomyolipomas, and "migration tracts" in the cerebral white matter) in association with a giant intracranial aneurysm, but lacking mental retardation, epilepsy and facial angiofibroma. RESULTS: Fourteen other cases of tuberous sclerosis and intracranial aneurysms, all but one without any clear sign of polycystic kidney disease, were found in the literature. CONCLUSION: We suggest that vascular dysplasias in general and aneurysms (mainly intracranial) in particular can be added to the other non-primary diagnostic features for the clinical diagnosis of tuberous sclerosis.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/136. Ventricular asystole during vagus nerve stimulation for epilepsy in humans.Electrical stimulation of the vagus nerve, a recently available option for patients with refractory epilepsy, has demonstrated safety and efficacy. We report four patients with refractory epilepsy who experienced ventricular asystole intraoperatively during initial testing for implantation of the vagus nerve stimulator. Acute intraoperative vagus nerve stimulation may create ventricular asystole in humans. Extracorporeal cervical vagus nerve stimulation testing with continuous EKG monitoring intraoperatively before generator implantation is warranted.- - - - - - - - - - ranking = 248.36912338788keywords = nerve (Clic here for more details about this article) |
3/136. Convulsive syncope following placement of sphenoidal electrodes.Two cases of convulsive syncope following the insertion of sphenoidal electrodes are reported. The episodes occurred shortly after an uneventful insertion of the needle. Both patients exhibited behavioral arrest with loss of muscle tone, followed by flexor posturing, jerking of the extremities, then followed by what appeared to be a panic attack. Episodes were clinically distinct from the patients' typical spells and were initially interpreted as representing psychogenic events. EEGs during the episodes showed diffuse slowing followed by generalized suppression of rhythms. Simultaneous EKG showed bradycardia followed by brief asystole and then resumption of normal heart rhythms in both cases. Vagally mediated cardioinhibitory reactions induced by fear, pain and possibly stimulation of branches of the trigeminal nerve in the face represent an uncommon but potentially serious complication of placement of sphenoidal electrodes.- - - - - - - - - - ranking = 31.046140423485keywords = nerve (Clic here for more details about this article) |
4/136. Behavioral psychophysiological intervention in a mentally retarded epileptic patient with brain lesion.Behavioral psychophysiological treatment entailing Slow Cortical Potential (SCP) biofeedback training and behavioral self-control training was conducted with a 27-year-old male epileptic patient (seizures for 23 years) with Wechsler IQ 64 who underwent callosotomy. The patient had 12/week secondary generalized tonic-clonic seizures. The treatment, consisting of 43 SCP training sessions and 22 behavioral control sessions, yielded a highly significant reduction of seizure frequency to about 7.5/week; such a decrease had never been observed after administration of new anticonvulsant drugs, nor after the callosotomy. During SCP feedback training, the patient was able to produce highly-significant cortical differentiation of SCPs of about 4 microV. In addition, he developed several new behaviors indicating growing ability of self-perception and self-regulation. These findings suggest that a combination of SCP biofeedback with behavioral treatment of epilepsy can be used even in mentally retarded patients with organic brain disorders.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/136. epilepsy and polymicrogyria in Kabuki make-up (Niikawa-Kuroki) syndrome.Kabuki make-up syndrome is a rare dysmorphogenic disorder characterized by peculiar facial appearance (resembling the make-up of actors in Kabuki, the traditional Japanese theatre), skeletal anomalies, dermatoglyphic abnormalities, postnatal growth deficiency, and mental retardation. central nervous system dysfunctions, other than mental retardation, are rarely reported; they include microcephaly, brachycephaly, early hypotonia, feeding disorders, subatrophy of the optic nerves, subarachnoid cyst, cerebellar and brainstem atrophy, and epilepsy. These manifestations appear to be more common in non-Japanese patients. Reported is an Italian child with phenotypical appearance of Kabuki make-up syndrome and partial epilepsy who demonstrated polymicrogyria on neuroimaging. This article is the first report of a gyration disorder in Kabuki make-up syndrome. The relationship between epilepsy and polymicrogyria in this patient is discussed.- - - - - - - - - - ranking = 31.046140423485keywords = nerve (Clic here for more details about this article) |
6/136. Diphenylhydantoin teratogenicity: ocular manifestations and related deformities.This patient illustrates a classical case of what many pediatricians call the diphenylhydantoin teratogenic syndrome. It suggests the possibility of an additional ocular finding of retinoschisis and optic nerve abnormalities which could conceivably have a teratogenic basis. The effects of epilepsy and diphenylhydantoin on these formations is discussed.- - - - - - - - - - ranking = 31.046140423485keywords = nerve (Clic here for more details about this article) |
7/136. bradycardia and asystole with the use of vagus nerve stimulation for the treatment of epilepsy: a rare complication of intraoperative device testing.PURPOSES: A 56-year-old man with mild mental retardation, right congenital hemiparesis, and refractory partial seizures was referred for vagus nerve stimulation (VNS). methods: Routine lead diagnostic testing during the surgical procedure (1.0 mA, 20 Hz, and 500 micros, for approximately 17 s) resulted, during the initial two stimulations, in a bradycardia of approximately 30 beats/min. A third attempt led to transient asystole that required atropine and brief cardiopulmonary resuscitation. RESULTS: The procedure was immediately terminated, the device removed, and the patient recovered completely. A postoperative cardiologic evaluation, including an ECG, 24-h Holter monitor, echocardiogram, and a tilt-table test, was normal. CONCLUSIONS: Possible mechanisms for the bradycardia/asystole include stimulation of cervical cardiac branches of the vagus nerve either by collateral current spread or directly by inadvertent placement of the electrodes on one of these branches; improper plugging of the electrodes into the pulse generator, resulting in erratic varying intensity of stimulation; reverse polarity; and idiosyncratic-type reaction in a hypersusceptible individual. The manufacturer reports the occurrence rate in approximately 3,500 implants for this intraoperative event to be approximately one in 875 cases or 0.1%.- - - - - - - - - - ranking = 186.27684254091keywords = nerve (Clic here for more details about this article) |
8/136. motor cortex localization using functional MRI and transcranial magnetic stimulation.OBJECTIVE: Congenital brain lesions producing focal seizures may be accompanied by reorganization of the areas responsible for motor and sensory functions within the brain due to a phenomenon that has been termed "neuronal plasticity." This can be studied using functional MRI (fMRI) and transcranial magnetic stimulation (TMS). Using either method, the motor cortex can be localized noninvasively, but to date there have been few studies correlating the level of agreement between the two techniques. methods: We used fMRI and TMS to localize the motor cortex in a young woman with intractable focal seizures, congenital left arm weakness, and a dysplastic right hemisphere on MRI. RESULTS: There was excellent agreement in the localization of motor representation for each hand. Both were predominantly located in the left hemisphere. fMRI also showed an area of posterior activation in the right hemisphere, but there was no evidence of descending corticospinal projections from this site using TMS, direct cortical stimulation, and Wada testing. CONCLUSIONS: Functional MRI (fMRI) and transcranial magnetic stimulation (TMS) were successfully used to localize cortical motor function before epilepsy surgery. Each technique demonstrated migration of motor function for the left hand to the left motor cortex. After resection of the dysplastic right precentral gyrus there was no permanent increase in weakness or disability. The two techniques are complementary; fMRI indicates all cortical areas activated by the motor task, whereas TMS identifies only those areas giving rise to corticospinal projections.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/136. Somatosensory representation in patients who have undergone hemispherectomy: a functional magnetic resonance imaging study.OBJECT: Removal or disconnection of an entire cerebral hemisphere is occasionally used to treat refractory seizures. patients who have undergone a hemispherectomy provide useful models to study the reorganization of cortical somatosensory representation. This plasticity may be a consequence of the pathological lesion, the hemispherectomy itself, or both. methods: Three patients who had undergone hemispherectomy were studied with functional magnetic resonance (fMR) imaging. Responses to sensory stimulation in normal hands and hands opposite the lesioned hemisphere were studied. Multislice T2*-weighted gradient-echo echoplanar images were obtained using a 1.5-tesla MR imager. The activation condition consisted of somatosensory stimulation of the index finger. A T1-weighted anatomical MR image was acquired. The fMR and anatomical MR images were coregistered, and statistically significant activation foci (p < 0.01) were identified. Stimulation of the normal hand produced activation in the primary somatosensory cortex (SI) in all patients. Stimulation of the impaired hand resulted in activation of the ipsilateral parietal operculum (second somatosensory area [SII]) and posterior parietal lobe (Brodmann's Area 7) in all cases, but no activation was elicited in the SI in any patient. In addition, other areas within the ipsilateral frontal and parietal lobes were activated in some individuals. CONCLUSIONS: Residual somatosensory function in the hand opposite the lesioned hemisphere is mediated by the SII and other cortical regions in the intact hemisphere, without involvement of the SI.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/136. Bilateral focal polymicrogyria in ehlers-danlos syndrome.BACKGROUND: ehlers-danlos syndrome (EDS) is a heterogeneous group of generalized connective tissue disorders that has been described in association with epilepsy and cerebral cortical dysplasia, mostly gray matter heterotopias, in 3 reports. However, to our knowledge, association of EDS with another type of cortical cerebral dysplasia, bilateral focal polymicrogyria, has never previously been described. SETTING: Two research-oriented hospitals. patients: We describe 2 patients with EDS and bilateral polymicrogyria. The first, a 29-year-old black man, presented with EDS of unspecified type, seizures, and bilateral frontocentral and frontoposterior polymicrogyria with hypoplasia of the inferior part of the cerebellar vermis. The second, a 20-year-old woman, had type III EDS, seizures and congenital bilateral perisylvian syndrome with polymicrogyria. CONCLUSIONS: The association of bilateral focal polymicrogyria and EDS in these 2 patients suggests that extracellular matrix proteins implicated in the pathogenesis of EDS, such as collagen and tenascin, may play an important role in cerebral cortical formation and organization. In a clinical setting, the association of EDS with these cortical structural lesions has implications for diagnosis and management.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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