1/10. Nonconvulsive status epilepticus in a child with congenital bilateral perisylvian syndrome.A 9-year-old male with congenital bilateral perisylvian syndrome is described. He had pseudobulbar palsy, mental retardation, and intractable epilepsy. Computed tomography and magnetic resonance images of the brain demonstrated bilateral perisylvian malformations and a diffuse pachygyric appearance. At 8 years of age, he had episodes of excessive drooling, fluctuating impairment of consciousness, unsteady sitting, and frequent head drop that lasted several days. The electroencephalogram demonstrated continuous diffuse slow spike and waves. These findings suggested atypical absence status epilepticus. Intravenous administration of diazepam resulted in transient improvement of clinical and electroencephalographic findings. status epilepticus recurred within several minutes after diazepam administration. Although no patient has been reported to have a history of status epilepticus among those affected by this syndrome, it seems that atypical absence status can occur more frequently than expected, as seen in Lennox-Gastaut syndrome. After recognition and confirmation of nonconvulsive status epilepticus, immediate treatment must be attempted.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
2/10. dysarthria as the leading symptom of hypothyroidism.Typical symptoms of hypothyroidism are lethargy, cold intolerance, slowing of intellectual and motor activity, declining appetite, increasing weight, and dry skin. A 43-year-old man with hypothyroidism presented with dysarthria as the leading symptom. Further symptoms were cramps in the legs after exercise, dizziness, and stunned feeling. He suffered from severe snoring for 4 years, and obstructive sleep apnea syndrome was diagnosed 2 years before. creatine phosphokinase was elevated. electromyography was myogenic. echocardiography showed a thickened myocardium. An otolaryngologic investigation revealed macroglossia and hypertrophy of the uvula. After administration of L-thyroxine, the symptoms rapidly improved. dysarthria may be the leading symptom of hypothyroidism and can be promptly resolved after hormone substitution.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
3/10. Reorganization of speech production at the motor cortex and cerebellum following capsular infarction: a follow-up functional magnetic resonance imaging study.Based on clinical data, Geschwind assumed left hemisphere dominance of speech production to extend to the cortical representation of articulatory and phonatory functions at the motor cortex. This author suggested, furthermore, that the clinical observation of rapid recovery from articulatory impairments after damage to the left-sided corticobulbar tracts reflects compensatory activation of an alternative pathway involving the contralateral pre-central gyrus and its efferent projections. In order to test this hypothesis, functional magnetic resonance imaging (fMRI) was performed 4 and 35 days after stroke in a 38-year-old man who had experienced sudden speech deterioration ('dysarthric speech') concomitant with weakness of the right upper limb and the right side of the face. Computerized tomography demonstrated an ischaemic infarction within the left internal capsule. The patient fully recovered from dysarthria within 9 days. Activation of the right hemisphere analogues of Broca and Wernicke areas has been assumed to contribute to recovery from aphasia. As a further aspect of the reorganization of speech function, the present case study demonstrates for the first time by means of fMRI a selective 'shift' of the cortical representation of speech motor control to the right Rolandic cortex and the left cerebellum during restitution of articulation in a case of transient dysarthria following infarction of the left internal capsule.- - - - - - - - - - ranking = 55.50222997685keywords = organization (Clic here for more details about this article) |
4/10. Transient loss of speech followed by dysarthria after removal of posterior fossa tumour.The authors report three children who suffered transient loss of speech during six to eight weeks following removal of a large midline cerebellar tumour. None manifested speech difficulties immediately after surgery, but all developed mutism within 24 to 48 hours. The speech of all children slowly but completely recovered, after a period of severe dysarthria. The re-organization of speech functions is discussed in relation to the functioning of musculature.- - - - - - - - - - ranking = 11.10044599537keywords = organization (Clic here for more details about this article) |
5/10. The human basis pontis: motor syndromes and topographic organization.Clinical-anatomic correlations were performed in 25 patients with focal infarcts in the basilar pons to determine whether pontine lacunar syndromes conform to discrete clinical entities, and whether there is topographic organization of the motor system within the human basis pontis. Twelve clinical signs were scored on a 6-point scale, neuroimaging lesions were mapped and defined with statistical certainty, and structure-function correlation was performed to develop a topographic map of motor function. Clinical findings ranged from major devastation following extensive lesions (pure motor hemiplegia) to incomplete basilar pontine syndrome and restricted deficits after small focal lesions (ataxic hemiparesis, dysarthria-clumsy hand syndrome, dysarthria-dysmetria and dysarthria-facial paresis). The syndromes are not absolutely discrete, and are distinguished from each other by the relative degree of involvement of each clinical feature. Structure-function correlations indicate that strength is conveyed by the corticofugal fibres destined for the spinal cord, whereas dysmetria results from lesions involving the neurons of the basilar pons that link the ipsilateral cerebral cortex with the contralateral cerebellar hemisphere. Facial movement and articulation are localized to rostral and medial basilar pons; hand coordination is medial and ventral in rostral and mid-pons; and arm function is represented ventral and lateral to the hand. Leg coordination is in the caudal half of the pons, with lateral predominance. Swallowing is dependent upon the integrity of a number of regions in the rostral pons. gait is in medial and lateral locations throughout the rostral- caudal extent of the pons. Dysmetria ipsilateral to the lesion constitutes a disconnection syndrome, as it occurs when the hemipontine lesion is extensive and interrupts pontocerebellar fibres traversing from the opposite, intact side of the pons. The heterogeneity of manifestations reflects the well-organized topography of motor function in the human basis pontis, in agreement with the anatomic organization of the motor corticopontine projections in the monkey. Higher order impairments including motor neglect, paraphasic errors and pathological laughter result from rostral and medial pontine lesions, and may result from disruption of the pontine component of associative corticopontocerebellar circuits.- - - - - - - - - - ranking = 66.60267597222keywords = organization (Clic here for more details about this article) |
6/10. Nonsurgical treatment of drooling in a patient with closed head injury and severe dysarthria.The purpose of this investigation was to measure the effectiveness of the antimuscarinic drug atropine sulfate in the treatment of chronic drooling in a patient with a history of severe closed head injury and resultant widespread oral neuromuscular and higher cortical disturbances. Results of the A-B-A-B-A-B withdrawal paradigm, chosen to demonstrate the functional relationship between drug therapy and the degree of drooling, revealed that administration of atropine sulfate reduced by more than 50% of baseline levels the amount of resting secretion, intraoral accumulation, and pharyngeal-laryngeal pooling of saliva, with negligible side effects. These results are discussed and compared to the alternative drug and surgical approaches to treatment that have been the primary focus of recent research on drooling.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
7/10. Hemiparesis and ischemic changes of the white matter after intrathecal therapy for children with acute lymphocytic leukemia.Three children with acute lymphocytic leukemia (ALL) developed delayed-onset transient hemiparesis and facial palsy after intrathecal (IT) administration of methotrexate (MTX) alone or as part of triple intrathecal chemotherapy for central nervous system (CNS) prophylaxis. The hemiparesis developed 10 to 14 days after IT therapy. Two of three children also experienced transient, profound expressive dysarthria. These episodes occurred during maintenance treatment after multiple IT administrations and without previous CNS toxicity. Two of three children received intermediate-dose MTX, 1 g/m2, not less than 5 weeks before events. These patients had not received cranial irradiation and had no evidence of CNS leukemia before or after these episodes. Ischemic changes on computerized tomographic scan or magnetic resonance imaging studies were documented in all three cases. Such changes are unusual manifestations of neurotoxicity in children after intrathecal therapy.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
8/10. Disturbances of the temporal organization of speech following bilateral thalamic surgery in a patient with Parkinson's disease.This report summarizes a detailed analysis of the speech of a 45-yr-old man who had become dysarthric following bilateral thalamic surgery for the relief of symptoms of Parkinson's disease. His speech was characterized by a rapid rate and a mild-to-moderate articulatory deficit. Intelligibility was markedly reduced. The rapid rate was found to be the result of decreased syllable durations rather than to changes in pause or phrase patterns. Decreased syllable durations resulted from abnormal shortening of vowels. Consonant releases were found to be prolonged. This distorted temporal relationship among speech segments was considered to be an important factor in the patient's poor intelligibility and partially explained why uniform electronic expansion of his speech resulted in only negligible increase in intelligibility. It is hypothesized that this speech disturbance results from the interaction of central "metronomic" abnormality with a peripheral neuromotor articulatory impairment.- - - - - - - - - - ranking = 44.40178398148keywords = organization (Clic here for more details about this article) |
9/10. Periodic ataxia: an unusual non-familial variation with paroxysmal EEG features.A 19-year-old youth suffered from periodic attacks of ataxia and dysarthria. Abnormally high IgG and IgA levels were found in the CSF. The length of the episodes of ataxia, absence of family history and the presence of generalised paroxysmal features in the EEG constitute a combination which is not believed to have been recorded previously. carbamazepine was ineffective but temporary clinical and electroencephalographic improvement followed the administration of ACTH. acetazolamide therapy has resulted in prolonged remissions from attacks.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
10/10. dysarthria and apraxia of speech associated with FK-506 (tacrolimus).The immunosuppressive agent FK-506 (tacrolimus) is one of the agents most commonly used to prevent rejection after liver transplantation. Neurologic toxicity related to FK-506 has been reported, including speech disorders; however, a detailed analysis of the speech disorder associated with use of FK-506 has not been presented. Herein we describe a patient who exhibited mutism, then severe apraxia of speech with a concomitant hypokinetic, spastic, and ataxic dysarthria after administration of FK-506. His residual mixed dysarthria, without radiographic evidence of a structural lesion, suggests dysfunction of one or more neurochemical systems. The pathophysiologic mechanisms underlying this intriguing entity remain obscure.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
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