1/18. Central nystagmus induced by deep-brain stimulation for epilepsy.PURPOSE: The goal of the present study was to describe the localization of central nystagmus induced as a side effect of electrical deep-brain stimulation for epilepsy. methods: Bilateral deep-brain stimulating electrodes were inserted in the centromedian nucleus of the thalamus to control seizures in a patient with intractable epilepsy. RESULTS: Cathodal high-frequency stimulation through the deepest contact of each electrode elicited cycles of slow ipsiversive conjugate eye deviations, each followed by rapid contralateral jerks. The involved electrode contacts were situated at the mesodiencephalic junction just inferior to the centromedian nucleus of the thalamus and rostral to the superior colliculus. Right-sided stimulation evoked left beating nystagmus and left-sided stimulation evoked right beating nystagmus. Stimulation through other electrode contacts did not induce nystagmus. electronystagmography showed the nystagmus to have constant velocity slow phases. CONCLUSIONS: A central nystagmogenic area exists in humans that appears to be homologous to the nucleus of the optic tract, a region described in nonhuman primates to play a role in the generation of optokinetic nystagmus.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
2/18. Hereditary motor and sensory neuropathy with absence of large myelinated fibers due to absence of large neurons in dorsal root ganglia and anterior horns, clinically associated with deafness, mental retardation, and epilepsy (HMSN-ADM).Hereditary motor and sensory neuropathy (HMSN) with autosomal recessive inheritance represents a genetically heterogeneous group of disorders with variable clinical, pathologic and electrophysiologic manifestations. A new variant of autosomal recessive HMSN, clinically defined by sensorimotor polyneuropathy associated with deafness and mental retardation, has recently been described. We report on the first autopsy case with this type of HMSN: a girl of non-consanguineous parents with a presumably autosomal recessive type of motor and sensory neuropathy clinically associated with deafness, mental retardation, and epilepsy. The autopsy showed complete absence of large myelinated fibers in peripheral motor and sensory nerves corresponding to a lack of large neurons in dorsal root ganglia and anterior horns of the spinal cord, moderate neurogenic muscle atrophy, and nearly complete absence of neurons in the dentate nucleus of the cerebellum. Molecular genetic analyses in our case revealed neither genetic alterations in the survival motor neuron gene nor in the PMP-22 gene.- - - - - - - - - - ranking = 0.33333333333333keywords = nucleus (Clic here for more details about this article) |
3/18. Antiepileptic effect of high-frequency stimulation of the subthalamic nucleus (corpus luysi) in a case of medically intractable epilepsy caused by focal dysplasia: a 30-month follow-up: technical case report.OBJECTIVE AND IMPORTANCE: Currently, some forms of epilepsy are resistant to both pharmacological and surgical interventions. As a result, there is a need for new therapeutic strategies. Because the nigral system modulates neuronal excitability in animal models of epilepsy, we considered therapeutic high-frequency stimulation of the subthalamic nucleus (STN). We were encouraged by the known relationship between the STN and the nigral system, as well as by our experience with high-frequency stimulation of the STN in Parkinsonian patients. CLINICAL PRESENTATION: A 5-year-old girl with pharmacologically resistant, inoperable epilepsy caused by focal centroparietal dysplasia underwent implantation with a permanent electrode in the left STN and was chronically stimulated. To date, we have followed up this patient for 30 months postoperatively. TECHNIQUE: High-frequency stimulation of the STN induced a significant voltage-dependent reduction (by 80%) in the number and severity of seizures. In addition, consistent improvement in both motor and cognitive functions was noted as a result of reduced postictal states. The effect was more prominent for seizures occurring in clusters (89% reduction) and during the day (88% reduction) than for those that occurred during sleep (53% reduction). CONCLUSION: This is the first report of epilepsy control using chronic high-frequency stimulation of the STN. Preliminary observations in three other operated patients (at 2, 12, and 18 mo) confirm these data. We think that high-frequency stimulation of the STN may hold significant future potential as a treatment for epilepsy, similar to its established role in the treatment of Parkinson's disease. This finding opens completely new experimental and therapeutic avenues for the treatment of surgically and medically intractable epilepsy.- - - - - - - - - - ranking = 1.6666666666667keywords = nucleus (Clic here for more details about this article) |
4/18. Aplasia of the retinal vessels combined with optic nerve hypoplasia, neonatal epileptic seizures, and lactic acidosis due to mitochondrial complex I deficiency.A newborn male with mitochondrial complex I deficiency suffered from neonatal epileptic seizures, which later developed into infantile spasms. The infant was blind due to aplasia of the retinal vessels and hypoplasia of the optic nerve. There was congenital lactic acidosis, which persisted in later life. The boy was microcephalic and retarded. Muscular hypotonia later shifted to spasticity. succinic acid was increased in urine. We assume that the aplasia of the retinal vessels is due to damage of the retinal ganglion cells caused by the mitochondrial disease in the first 3 to 4 months of pregnancy.- - - - - - - - - - ranking = 3.2158042983618keywords = ganglion (Clic here for more details about this article) |
5/18. L-2-hydroxyglutaric aciduria: clinical, neuroimaging, and neuropathological findings.BACKGROUND: l-2-Hydroxyglutaric aciduria is a rare, infantile-onset, autosomal recessive organic aciduria affecting exclusively the central nervous system. To our knowledge, only 1 complete report of the neuropathological findings in an adult has been published. OBJECTIVE: To present the clinical, neuroimaging, and neuropathological findings of l-2-hydroxyglutaric aciduria. DESIGN: Case report. SETTING: Complexo Hospitalario de Pontevedra, Pontevedra, spain. PATIENT: A 15-year-old boy who had early infantile-onset progressive psychomotor regression, mild choreodystonia affecting the distal part of the upper limbs, pyramidal signs, and epilepsy. RESULTS: The diagnosis of l-2-hydroxyglutaric aciduria was confirmed by the finding of highly elevated levels of l-2-hydroxyglutaric acid in the serum, urine, and cerebrospinal fluid. The neuroimaging findings showed striking confluent subcortical white matter lesions and minimal basal ganglia (pallidum, thalamic, and putaminal) abnormalities. The patient died of a spontaneous mesenteric thrombosis. The postmortem neuropathological findings showed spongiosis and cystic cavitations in subcortical white matter, with minimal abnormalities of the basal ganglia. The dentate nucleus, a structure usually affected in neuroimaging studies, showed minimal neuronal loss but was surrounded by important spongiosis and microvacuolation with astrocytic proliferation. CONCLUSIONS: This case reaffirms that l-2-hydroxyglutaric aciduria is a spongiform type of leukoencephalopathy with cystic cavitations predominating in the subcortical areas. Although the neuroimaging findings are highly characteristic of the disease, in this patient cerebellar abnormalities were minimal and dentate signal abnormalities were not present.- - - - - - - - - - ranking = 0.33333333333333keywords = nucleus (Clic here for more details about this article) |
6/18. Depth electrode recorded cerebral responses with deep brain stimulation of the anterior thalamus for epilepsy.OBJECTIVE: We investigated the relation between anterior thalamic stimulation and the morphology of the evoked cerebral responses (CRs) using intracerebral depth electrodes in patients with intractable epilepsy undergoing deep brain stimulation (DBS) of the thalamus. methods: Monopolar cathodic and bipolar stimuli were delivered at a rate of 2 or 1 Hz to the anterior nucleus (AN) and the dorsomedian nucleus (DM) of two patients using the programmable stimulation device (Medtronic ITREL II) or a GRASS stimulation device (S12). CRs were recorded from depth or DBS electrodes, situated bilaterally in mesial temporal (hippocampus, both patients), lateral temporal (one patient), orbitofrontal (Brodmann area 11, one patient) and anterior thalamic sites (one patient). RESULTS: The distribution and morphology of the CRs depended primarily on the site of stimulation within the anterior thalamic region. overall, monopolar cathodic and bipolar stimulation of the AN elicited CRs mainly in ipsilateral mesial temporal cortical areas, whereas stimulation of the DM evoked high-amplitude CRs predominantly in ipsilateral orbitofrontal areas. The amplitude of the CR was positively related to the strength of the stimulus and generally higher with monopolar than with bipolar stimulation. The differences between CRs elicited during wakefulness or slow wave sleep were minimal. CONCLUSIONS: The distribution of the CRs corresponded with the intracerebral pathways of the involved structures and the findings are in good accordance with those of our previous study investigating the sources of CRs using statistical non-parametric mapping of low resolution electromagnetic tomography (LORETA) values. SIGNIFICANCE: Our findings indicate a certain degree of point-to-point specificity within the thalamocortical circuitry, which may make optimal localization of DBS electrodes important in patients with epilepsy.- - - - - - - - - - ranking = 0.66666666666667keywords = nucleus (Clic here for more details about this article) |
7/18. Roles of a subependymal nodule of tuberous sclerosis on pathophysiology of epilepsy.Polysomnographies (PSG) were performed on two cases with tuberous sclerosis (TS), both having subependymal nodules on the medial wall of the caudate nucleus adjacent to the thalamostriatal sulci. Clinically one had suffered from infantile spasm and which later turned out to be complex partial seizure with a rotation toward the right. The other had developed tonic seizure on the right with a rotation toward the left in early childhood. Clinico-pharmacological studies revealed the existence of synaptic supersensitivity of the dopamine (DA) receptor in the left caudate and PSG confirmed the synaptic supersensitivity of the former, while in the latter case, it suggested a decrease in DA activity on the left. Subependymal nodules in the caudate nucleus could cause a reduction in the DA transmission and develops synaptic supersensitivity after suffering from TS.- - - - - - - - - - ranking = 0.66666666666667keywords = nucleus (Clic here for more details about this article) |
8/18. Podiatric implications of psychogenic seizures.Psychogenic seizures can mimic epileptic conditions and are often difficult to diagnose. The present study reviews one such unusual occurrence in a patient having a complex psychiatric history who required surgery for the removal of a ganglionic cyst in her foot. The differential diagnosis and treatment of psychogenic seizures is discussed.- - - - - - - - - - ranking = 3.2158042983618keywords = ganglion (Clic here for more details about this article) |
9/18. epilepsy and agitated delirium caused by an astrocytoma of the amygdala.The clinical picture of a tumour involving the amygdaloid nucleus was characterized over a period of 6 years by generalised epileptic fits, then a state of agitated delirium with oneirism evolving during 2 months and leading finally to pulmonary embolism. The association of epilepsy and psychiatric disorders is the clinical picture usually observed in tumors of the limbic system but these symptoms are various and unspecific. The conventional neuroradiological procedures often fail to discover the lesion.- - - - - - - - - - ranking = 0.33333333333333keywords = nucleus (Clic here for more details about this article) |
10/18. Neuropathology of secondary generalized epilepsy--(Lennox-Gastaut syndrome)--a case report.Neuropathological considerations were performed on a case, who went into Lennox syndrome after an acute encephalopathy at the infantile period, and moreover who fell into an akinetic-mute state derived from brain damage by herniation caused by a head injury and subsequent status epilepticus. Neuropahtological background in the present case of Lennox syndrome is thought to be based on the widespread unilateral cerebral lesions and the basal ganglional, especially thalamic, degenerations derived secondarily from the diffuse cerebral damage. The patient revealed akinetic mutism with the disappearance of the epileptic seizures and the desynchronization of the EEG's, when the brain lesions formed at the adult period spread over the opposite hemispheric limbic system and the brain stem tectum.- - - - - - - - - - ranking = 9.8252231743336keywords = basal ganglion, ganglion (Clic here for more details about this article) |
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