Recurrent strokes produced by bilateral high intraforaminal entering of vertebral arteries. Case report.
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Biomedical subjects
Publications and source records attributed to R Michelucci.
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1. The purpose of this study was to evaluate the efficacy of vigabatrin in relation to the seizure type. 2. A review of the literature concerning the efficacy of vigabatrin in clinical trials was performed. 3. To date, vigabatrin has been used in a small number of patients with generalized seizures: in this group no significant effect was noted. 4. Vigabatrin was also given to a large number of patients with complex partial seizures: in this group vigabatrin produced a greater than 50% decrease in seizure frequency in almost 50% of patients included. 5. This review provides evidence that vigabatrin is effective in the treatment of complex partial seizures: patients with only one seizure type, low seizure frequency and unifocal EEG abnormalities are particularly expected to respond. 6. This implies that patients with complex partial seizures do not constitute an homogeneous group and that various parameters should be considered in the evaluation of the response to treatment.
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Thirteen patients with dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome) had full clinical and neurophysiological study as well as muscle biopsy. The patients had action myoclonus, generalised epileptic seizures, and mild cerebellar syndrome. The disease was inherited in an autosomal recessive pattern in five patients, and occurred as isolated cases in the remaining eight patients. The age at onset of symptoms ranged from 6 to 15 years (mean, 10.4 years). The EEG and polygraphic findings included normal background activity in most patients, spontaneous fast generalised spike-and-wave discharges, photosensitivity, no activation during slow sleep, and vertex and rolandic spikes in REM sleep. Results of muscle biopsy, performed an average of 14 years after onset of the disease, were normal and showed no mitochondrial abnormalities. These findings suggest that Ramsay Hunt syndrome is a condition with distinctive clinical and neurophysiological features and unrelated to mitochondrial encephalomyopathies.
Six children with tactile-evoked spikes in the EEG, also defined as extreme somatosensory evoked potentials (ESEPs), underwent an SEPs study in order to define the characteristics of such evoked potentials. Short-latency SEPs showed normal mean latency and amplitude values. Mid- or long-latency SEPs of abnormally high amplitude were recorded after stimulation of one or more extremities. Such extreme responses which showed the same reactivity proper to normal long-latency SEPs could be considered to correspond to the evoked spikes in the EEG.
We studied four patients with a focal epilepsy and bilateral occipital corticosubcortical calcifications without any sign of phakomatosis. The clinical course of the disease was similar in all the patients and evolved from a benign onset to a severe encephalopathy with progressive mental impairment. The question of whether these patients have an incomplete and atypical form of Sturge-Weber syndrome or a previously undescribed disorder is addressed.
Thirty-one patients with severe drug-resistant epilepsy entered the study. Vigabatrin (2 to 3 g/d, stratified according to weight) and placebo were administered orally, as add-on therapy in random order under double-blind conditions, each for three months using a crossover design. Thirty patients completed both periods. Of these, ten patients (33%) showed a decrease in seizure frequency of 50% or more. In the 15 patients presenting with complex partial seizures, "temporal" electroencephalographic abnormalities, and relatively low seizure frequency, there was a significant reduction in seizure frequency during vigabatrin treatment. No significant treatment effect was found for the remaining 15 patients, who presented with mixed seizure types, multifocal electroencephalographic abnormalities, and high seizure frequencies. Tolerability to vigabatrin was good; the most frequently reported unwanted effect was drowsiness. Plasma concentrations of phenytoin showed a significant reduction during the vigabatrin period. The results demonstrate the efficacy and good tolerability of vigabatrin therapy in patients with severe complex partial epilepsy.
Intracranial microvascular decompression was performed in 21 out of 24 patients with hyperactive dysfunction of cranial nerves: 8 cases of hemifacial spasm, 12 of trigeminal neuralgia, 3 of glossopharyngeal neuralgia and 1 case of paroxysmal vertigo and tinnitus. In 21 cases an abnormal vascular loop was found to impinge on the root entry zone of the nerve in the brainstem. Dissection of this loop with decompression of the nerve resulted in long-lasting relief of symptoms in all but two patients who presented early recurrence; in one of these a second procedure was eventually successful. In two patients with trigeminal neuralgia a benign tumor of the cerebellopontine angle that had escaped preoperative diagnosis was present. Finally, in one case no compressive lesions were found. From the data of the literature and from our present experience microvascular decompression can be considered a safe as well as an effective procedure, affording a high success rate in conditions often or usually resistant to medical treatment and erroneously considered "idiopathic".
20 patients who had undergone microvascular decompression for the treatment of "idiopathic" trigeminal neuralgia (9 cases), hemifacial spasm (7 cases), glossopharyngeal neuralgia (3 cases) and paroxysmal vertigo and tinnitus (1 case) were followed up for 25 months on average. Permanent relief of symptoms was observed in 19 (95%), with sparing of cranial nerve function. Analysis of the clinical data shows that the patients described in the present series did not differ from those considered to suffer from "idiopathic" cranial nerve dysfunction syndromes. The importance of vascular cross compression as etiological factor in such conditions is stressed and the pathophysiology discussed. The term "cryptogenic" applied to trigeminal neuralgia or hemifacial spasm thus needs revising. Lastly, the indications of microvascular decompression in the treatment of "cryptogenic" cranial nerve dysfunction syndromes are defined.
This chapter reviews the literature about intensive monitoring (IM) in status epilepticus (SE). The description of the newly developed and suitable monitoring techniques is followed by a few sections devoted to the clinical application of IM in SE. IM is mostly required to establish the correct diagnosis in SE, particularly in the nonconvulsive type. Similarly, the classification and the medical treatment of SE are greatly improved by IM. Although no definitive data exist, the practical relevance of IM in the prognosis of SE is discussed in detail. Furthermore, special emphasis is given to the value of neuroradiological monitoring in SE, as evidenced by a proper case report. It is concluded that a further development and a more widespread employment of monitoring techniques are needed to assist and improve the management of SE.
Computed tomographic (CT) findings of three patients affected by progressive supranuclear palsy (PSP) are reported. The radiological picture consisted of atrophy of the midbrain and quadrigeminal plate, with prominent interpeduncular, crural, ambient, and quadrigeminal plate cisterns, and dilatation of the aqueduct and third ventricle. These features were not related to the duration of the illness and degree of neurological deficits. In the authors' opinion CT is useful in the differential diagnosis of PSP and other extrapyramidal disorders.
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2 cases of Sturge-Weber syndrome without facial nevus are reported. The patients presented different forms of epilepsy. The diagnosis was made by computed tomography (CT) which showed typical intracranial calcifications in both occipital regions. The problems concerning the atypical and incomplete forms of the syndrome are briefly discussed. The importance of CT as a diagnostic procedure for this disease is emphasized.
Both our personal experience and the findings of others indicate that the benzodiazepines (a) are the drugs of first choice for control of status epilepticus occurring in patients with primary generalized epilepsy (90%-100% effective) or control of hemiclonic convulsions in children without brain lesions, (b) are effective in approximately 60% of cases of status epilepticus occurring in partial epilepsy, (c) are effective in only 15% to 59% of cases of tonic status or various types of absence status occurring in secondary generalized epilepsy (but no other drug is more effective), (d) are helpful in status epilepticus occurring in nonepileptic patients if there is no overt brain lesion (but give only temporary relief when status is the result of a severe organic brain lesion), and (e) are generally safe drugs.
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A 60-year-old patient had a 3-year history of recurrent memory disturbances. The video electroencephalographic (EEG) recording of one attack showed that the amnesic state, which clinically resembled transient global amnesia, was characterized by a normal EEG tracing followed by electroclinical complex partial seizures. We suggest that "epileptic" transient global amnesia is postictal in nature.
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