Chorea in Behçet's syndrome.
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Biomedical subjects
Publications and source records attributed to A Boiardi.
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The clinical effects of the protracted treatment with 5-OH Tryptophane (5-HTP) of some patients with chronic migraine are compared with other patients with acetylsalicylic acid. The pain threshold was neurophysiologically determined in migraneous on patients whose response to 5-HTP therapy was specially good. The results with 5-HTP can be accounted for by an action of the substance on the serotonin turnover with activation of the serotoninergic antinociceptive system.
A typical case of stiff-man syndrome was studied neurophysiologically and pharmacologically focusing the attention on a central disorder, either spinal or suprasegmental. The results support the hypothesis that a gamma type of hypertonia is one of the causes of the stiff-man syndrome, ruling out the participation of the Renshaw circuits. The syndrome should be considered a central disorder of the descending pathways that control and modify gamma activity and the startle reaction mechanisms, especially affecting the circuits that use Gabaergic mediators.
A man aged 56 years, previously healthy, developed asthenia, hypersonnia, apathy, later polydipsia and bulimia, headache and episodes of unconsciousness. There was temporary improvement with steriod therapy, but ever-deepening stupor appeared till death due to bronchopneumonia. All blood chemistry examinations were normal. The CSF IgG was elevated but no neoplastic cells were seen. At autopsy areas of grayish color in the basal gagnlia and granulomatous tissue in the floor of the third ventricle and in the mamillary bodies were seen, and the gonads were attrophied. Microscopically, in the floor of third ventricle, mamillary bodies and adjacent leptomeninges there was granulomatous tissue made up of more-or-less typical cells of the reticulum, polymorphonuclear cells, plasma cells and some phagocytes along with proliferation of small blood vessels and reticulin fibers. In addition, the white matter of the frontal lobes, pons, middle cerebellar peduncle and cerebellar white matter contained diffuse proliferation of pleomorphic histiocytic elements with questionable atypical mitoses. Notwithstanding, the morphology of our case suggests that it is a peculiar form of malignant reticulosis (or malignant histocytosis) related to histiocytosis X. The duplicity of the features of our case suggests it to be neoplastic, where the proliferative phase is followed by a granulomatous and sclerotic one.
A man of 23 years was affected by myasthenia with amyotrophic patterns. From the neurophysiological viewpoint, there were typical electrophysiological aspects of myasthenia gravis and the Eaton-Lambert syndrome simultaneously. In some instances the various electrophysiological tests were preceeded by the administration of Tensilon, calcium gluconate and sodium benzoate caffeine. It is hypothesized that there are two types of endplates present, or there is a functional variability by which at different moments a block results from a defect in the formation or liberation of acetylcholine.
A clinical trial of lithium carbonate was undertaken in a group of 20 patients suffering from chronic cluster headache. Blood levels of lithium were determined at regular intervals to monitor the therapeutic dosage. Lithium was found to be an effective prophylactic agent for chronic cluster headache patients. The effectiveness of lithium was evident in less than a week after the beginning of treatment. It is not clear in what way lithium may act on cluster headache.
A case of multicentric glioblastoma with the clinical and histopathological findings is presented in which three lesions are located above and below the tnetorium. Multicentric glioblastomas are those which have no macroscopic or microscopic connection.
The results of the study of the activity of platelet monoamine oxidase (MAO) in patients with migraine headaches or with 'cluster headaches' are presented. In both types of headaches, MAO activity is lower than in normal subjects. We suggest that the low MAO activity is due to a primary constitutional defect.
In a group of 60 patients of which 30 were affected by myasthenia, we used the regional curare test. The results were evaluated from a critical point of view and were then compared with the results obtained by the repetitive stimulation of the circumflex nerve, deriving from deltoid muscle. We considered too the specificity of the regional curare test for the evaluation of ocular myasthenia.
The authors report the case of a 67-year-old man, affected by diffuse spasms evoked by ischemia and voluntary and reflex movements electromyographically characterized by an intense repetitive activity. There was also a modest and diffuse muscle wasting with lower limbs areflexia; no myokymia. The EMG showed severe neurogenic changes and a considerable reduction of the MMCV in the lateral popliteal nerve area.
The authors studied the recruitment of motor units of EDB muscle by long lasting threshold intensity stimulation at 1 c/sec of external popliteal nerve at the ankle in healthy subjects and in myasthenic patients. In the healthy volunteers, at steady intensity of threshold stimuli, maximum number of motor units never exceeded 5--6 electrical increments with a final incremental response of constant amplitude during 60 min of stimulation. In myasthenic patients, both an early exhaustion of threshold motor units and a marked facilitation phenomenon occurred with massive recruitment of incremental responses, while in a small number of cases no changes were observed. These data are discussed in the light of various morphofunctional hypotheses regarding the localization of the myasthenic impairment.
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20, 25-Diazacholesterol does not induce myotonia in denervated mammalian skeletal muscle, and cordotomy also renders the muscles resistant to the induction of myotonia. Denervation of muscle already rendered myotonic, has no effect on the intensity of the phenomenon. Finally, tenotomy and posterior rhizotomy leave the myotonic activity in the "deafferentated" muscles unchanged. These findings are discussed in relation to "trophic" influences of the motor pathways on the postsynaptic structures of skeletal muscle.
In 19 normal adults reserpine administration induced significant changes in the parameters of the second glabellar response (R2): shortening of the latency and duration; decrease of the excitability threshold and complete blockade of the physiological habituation of R2 to the electrical and mechanical stimulation. No changes in the first response (R1) were observed. All the R2 changes disappeared within about 3 days of drug administration. The Parkinson-like effect of reserpine on the glabellar reflex is discussed in the light of a neurohormonal hypothesis in the control of the polysynaptic pathways biasing R2.
There are limited data in the literature concerning chemotherapy trials for the treatment of anaplastic astrocytomas. Forty-one anaplastic astrocytoma patients, operated on during the period 1988 to 1993 at the Neurological institute of Milan, received 4-5 cycles of chemotherapy (BCNU + cisplatin), subsequently radiotherapy (median dose 56.5 Gy), and finally a second-line chemotherapy protocol at recurrence (procarbazine, vincristine, lomustine). The aim of the study was to evaluate the effectiveness of the planned protocol, considering the time to tumor progression and the survival time. The group of anaplastic astrocytoma patients was compared with a homogeneous group of 39 anaplastic astrocytoma patients treated only with radiotherapy after surgery. The median time to tumor progression of patients on the protocol was 24.5 months. The median survival time for anaplastic astrocytoma patients treated with our scheduled protocol or only with radiotherapy was 38.8 and 21 months, respectively. However, our data need to be confirmed by large randomized clinical studies.
We describe a case of a patient with neuroblastoma and opsoclonus- myoclonus ataxia displaying serum and CSF anti-Hu antibodies that were able to recognize antigens of the patient's own tumor.
We evaluated the frequency of serum antineuronal antibodies in a cohort of 39 neuroblastoma patients and related their presence to clinical features. Twelve patients displayed antineuronal antibodies at immunocytochemistry. Only one of these 12 patients suffered from a clinically overt paraneoplastic syndrome. No significant differences emerged between autoantibody-positive and autoantibody-negative patients in terms of progression-free and overall survival, although when only patients evolving to disease progression were considered, the time interval between diagnosis and progression was slightly longer in autoantibody-positive patients.
Doses and schedules for treatment of malignant glial tumors, using IFN are still uncertain and controversial. In this study preliminary results of treatment of 10 glioblastoma multiforme patients are shown. Six patients were treated with local injection of beta-IFN through an Ommaya reservoir: 4 with beta-IFN followed by systemic chemotherapy (CDDP-VP16); we found that IFN alone was ineffective. Results were improved when local immunotherapy was associated with systemic chemotherapy. New drugs and investigation of possible pharmacological synergism are needed.