Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To evaluate the role of speech manifestations in lateralization of temporal lobe seizures, we reviewed videotapes of 100 complex partial seizures in 35 patients who underwent temporal lobectomy for intractable epilepsy. All patients had prolonged electroencephalographic video monitoring with scalp and subdural electrodes, and their speech dominance was determined with an intracarotid amobarbital test. Speech manifestations were observed in 79 seizures and were classified as vocalization, normal speech, or abnormal speech. Vocalization of sounds without speech quality occurred ictally in 48.5% of patients. Normal speech (identifiable speech) occurred ictally in 34.2% of patients. Abnormal speech (speech arrest, dysphasia, dysarthria, and nonidentifiable speech) occurred in 51.4% of patients, either ictally or postictally. Of all the above speech manifestations, only postictal dysphasia and ictal identifiable speech had significant lateralizing value: 92% of patients with postictal dysphasia had their seizures originating from the dominant temporal lobe (p less than 0.001), and 83% of those with ictal identifiable speech had their seizures from the nondominant side (p = 0.013). This study shows that speech manifestations are common in complex partial seizures of temporal lobe origin and can provide an excellent clinical tool for lateralization of seizure onset.
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PMID:Speech manifestations in lateralization of temporal lobe seizures. 291 32

The amino acids L-glutamate and L-aspartate have been shown to be excitatory neurotransmitters in mammalian central nervous systems. Antagonists acting selectively at excitatory amino acid receptors have shown antiepileptic properties in several animal models. We report the results of the first therapeutic trial of the competitive NMDA antagonist, D-CPP-ene (SDZ EAA-494), in eight patients with intractable complex partial seizures. All patients withdrew prematurely because of side-effects, including poor concentration (8), sedation (7), ataxia (6), depression (3), dysarthria (2), amnesia (2) and unilateral choreo-athetosis in a patient with contralateral Sturge-Weber syndrome. Seizures were unchanged in four patients and worse in three. A further patient with apparent improvement in seizures in the first week developed complex partial status epilepticus on withdrawal of DCPP-ene. EEG on treatment (5) or in the immediate post-treatment period (2) showed slowing of background activity and, in five cases, an increase in epileptiform activity. Serum concentrations of DCPP-ene were found to be unpredictable and higher than expected from pharmacokinetic data on normal subjects. There was no clear relationship between serum concentrations and the severity of side-effects. Preliminary experience with DCPP-ene in patients with refractory partial seizures is not promising. Evaluation of related compounds is warranted.
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PMID:The excitatory amino acid antagonist D-CPP-ene (SDZ EAA-494) in patients with epilepsy. 826 15

We report the results of 34 patients who underwent corpus callosotomy between 1986 and 1989 with 28-65 months of postoperative follow-up (mean 42 months). Thirty-two patients had mental retardation and 26 had significant behavioral problems. Thirteen patients had total section, 8 had subtotal section with preservation of the posterior half of the splenium, and 13 had section of the anterior two thirds of the callosum. Satisfactory seizure control was achieved in 25 patients (73.5%) Atonic seizures, followed by tonic seizures, generalized tonic-clonic seizures (GTCs), and atypical absence seizures were most improved. Myoclonic and complex partial seizures (CPS) did not improve significantly. No deterioration in seizure status was observed postoperatively. Two patients developed previously unobserved simple seizures and CPS postoperatively, but they were not as disabling as the preoperative seizures. Among the patients with behavioral problems, 81% had significant decrease in aggressiveness, hyperactivity, and/or attention deficit. Patients who underwent total section had interhemispheric disconnection symptoms that improved progressively and did not interfere with daily life. Decreased speech output, dysarthria, and gait dyspraxia occurred after total callosal section and persisted in 5 of the 13 patients. Patients who underwent anterior two thirds or subtotal sections did not have such symptoms. Early postoperative complications consisted of aseptic ventriculitis (5), subdural hematoma (1), and wound infection (4) and resolved without sequelae.
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PMID:Corpus callosotomy in treatment of medically resistant epilepsy: preliminary results in a pediatric population. 840 46

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EEG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental retardation and particularly congenital pseudobulbar palsy suggest this congenital entity. Those patients who exhibit these typically clinical features, must have MRI.
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PMID:A clinically recognizable neuronal migration disorder: congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up. 953 Sep 47

We describe two patients with juvenile-onset Niemann-Pick disease type C (NPC) to illustrate the variable neurologic features of this condition. One presented with hypersplenism at age 10 and was misdiagnosed with Gaucher disease. He developed complex partial seizures in his teens but remained otherwise neurologically asymptomatic until his mid 30s. At age 45, he had mild dementia and dysarthria, vertical supranuclear ophthalmoplegia, axonal sensorimotor polyneuropathy, and cerebellar ataxia. The second patient presented with rapidly progressive dystonia at age 8, and mild hepatosplenomegaly, vertical supranuclear ophthalmoplegia, severe behavioral disorder, and dementia by age 14. The diagnosis of NPC was based on deficient cholesterol esterification and excessive lysosomal filipin staining in cultured skin fibroblasts. Current notions about diagnosis and pathogenesis of NPC are reviewed.
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PMID:Niemann-Pick disease type C: two cases and an update. 1110 5

Primary malignant melanoma of the leptomeninges is a rare and aggressive tumor in children and accounts for less than 1% of all pediatric malignancies. Usually its symptoms include raised intracraneal pressure resulting from hydrocephalus secondary to tumoral obliteration of basal cisterns, but the passage of time from the initial symptomatology to diagnosis is frequently delayed. A 7-year-old male with primary leptomeningeal melanoma is reported. At the beginning, he presented ataxia and dysarthria followed by symptoms of raised intracranial pressure, complex partial seizures, progressive loss of consciousness, and coma. Cerebrospinal fluid analysis demonstrated raised opening pressure, normal glucose, and increased protein concentration, but malignant melanoma cells were not found. Magnetic resonance imaging scans depicted bright signals in the subarachnoid spaces on T(1) images and gadolinium-enhanced focal lesions. Cerebral biopsy was proposed, but it was not authorized. Definitive diagnosis was thus made by pathologic postmortem examination.
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PMID:Primary leptomeningeal melanoma in a child. 1151 18

Carbamazepine is a well-established, effective treatment of complex partial seizures and is well tolerated in most patients. The adverse effects of carbamazepine include aplastic anemia, agranulocytosis, pancytopenia, bone marrow depression, thrombocytopenia, cardiac conduction abnormalities, congestive heart failure, and peripheral edema. Hypertension or hypotension has also rarely been documented in patients with either therapeutic or toxic blood levels of carbamazepine. It is possible that carbamazepine-induced hypertension in those with therapeutic blood levels is rarely seen because most of the patients who begin treatment are young and do not have baseline hypertension. The authors describe a patient of African-American descent with a history of controlled essential hypertension who developed severe uncontrolled hypertension when started on carbamazepine. Treatment with additional antihypertensive medications did not reduce his blood pressure. In addition, he developed two episodes of transient neurologic deficits, the symptoms of which consisted of dysarthria, vertigo, and unstable gait. A substantial reduction of his carbamazepine dose resulted in the control of his blood pressure and no recurrence of his symptoms.
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PMID:Transient neurologic deficits associated with carbamazepine-induced hypertension. 1289 34

Structural abnormalities related with pseudobulbar palsy have been gaining attention because of their characteristic symptoms and unique pathogenesis. We present five cases of bilateral perisylvian ulegyria (BPU) presenting epilepsy and pseudobulbar palsy with pathogenesis different from previously reported syndromes. All patients showed medically intractable seizures, complex partial seizures with secondary generalization and clinical symptoms of pseudobulbar palsy, including dysarthria, limitation of tongue movement and drooling. MRI revealed BPU in all patients, and BPU associated with hippocampal sclerosis in four patients. Intracranial EEG recording with subdural grip and stripe was helpful for localizing the area of ictal generation. Resective surgeries, including the temporal lobe, central area and parietal lobe, were performed depending on the localizing information. The surgical outcome was favorable after 9.8 years of follow-up. Characteristic features of ulegyria were confirmed on pathological examination. Ulegyria is considered to be another important perinatal or postnatal structural abnormality which can explain the etiological heterogeneity for pseudobulbar palsy, which results from bilateral perisylvian lesions. Awareness of this disorder can provide a useful strategy for evaluation and treatment which differs from that in perisylvian polymicrogyria.
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PMID:Bilateral perisylvian ulegyria: clinicopathological study of patients presenting with pseudobulbar palsy and epilepsy. 1677 Nov 81

Few studies focused on the long-term outcome of Dravet syndrome in adulthood are available in the literature, but all are concordant. In this article, we consider the outcomes of 24 patients followed at the Centre Saint-Paul, Marseille, up to the age of 50, and compare them to the patients reported in the literature. Five patients (20.8%) died, at a mean age of 24.8 years, one by status epilepticus, three by sudden unexpected death in epilepsy (SUDEP), and one of unknown cause. Epileptic seizures tend to become less frequent and less severe after childhood. Fever sensitivity (temperature variations) persists throughout the clinical course of DS, but its impact on seizure frequency and severity is milder than in infancy. Generalized convulsive seizures, mostly reported as generalized tonic-clonic seizures (GTCS), were the only seizure type observed in almost all of the patients, often with a focal onset. They are less frequent than in childhood and mostly nocturnal. Some of these major convulsive seizures have less typical aspects, for example, bilateral or asymmetric tonic posturing, followed in some cases by a tonic vibratory state or clonic movements (Oguni et al., Brain Dev 2001;23:736-748; Akiyama et al., Epilepsia 2010;51:1043-1052). Other seizures like myoclonic seizures, atypical absences, and complex partial seizures (CPS) are less common in adulthood: Among our 24 patients, only 6 had atypical absences, and one myoclonic and one complex focal seizures. Electroencephalography (EEG) also changes with age but is still multiple and heterogenous, interictally and ictally. Photosensitivity and pattern sensitivity also showed a tendency to disappear before the age of 20. Motor abnormalities are common. Cerebellar features, including ataxia, dysarthria, intention tremor, and eye movement disorder, become more prominent. Walking is markedly impaired, often due to orthopedic signs such as kyphosis, kyphoscoliosis, flat feet, or claw feet. This symptomatology was minor during childhood and worsened during and after adolescence, despite physiotherapy. Mental retardation ranged from moderate to severe, with predominance of language impairment, and some patients had a major personality disorder, labeled autistic or psychotic. Dependency in adulthood is nearly constant: Only 3 of our 24 adult patients lived independently.
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PMID:Dravet syndrome: the long-term outcome. 2146 79

We present a 9-year-old boy with history of perinatal asphyxia and neonatal seizures; who presented with delayed development of speech, with predominant dysarthria, dysphagia, and drooling of saliva and unable to protrude tongue along with delayed motor and mental milestones. He had complex partial seizures since last 3 years requiring multiple anti-epileptic drugs. He had dysarthria, nasal twang, and drooling of saliva with difficulty in chewing and swallowing. Hearing and understanding were normal. Bilateral trigemino-facio-linguo-pharyngeal palsy was noticed on voluntary movements with normal jaw jerk with preserved automatic and emotional motor movements. Electroencephalography revealed focal left fronto-temporal epileptiform discharges and brain imaging was suggestive of bilateral cortical and subcortical region encephalomalacia, predominantly involving bilateral opercular region. The clinical and neuroimaging features correspond to bilateral opercular syndrome which could have resulted from the perinatal insult in this case.
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PMID:Opercular syndrome: A case report and review. 2408 30


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