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Biomedical subjects

E Maida

Publications and source records attributed to E Maida.

35 records · Page 2Linked to original sources

Computerized tomography in multiple sclerosis.

Forty-three patients with multiple sclerosis (MS) were examined clinically and with computerized tomography (CT). A total of 80 focal cerebral lesions were predicted by clinical neurologic examination. The CT scans showed foci corresponding to 31 (39%) of the clinically predicted 80 lesions. In addition to these foci, CT scans revealed 17 plaques of atypical periventricular localization as well as 18 focal lesions that produced no clinical signs in the course of the disease. The relations between duration of the disease and number of CT foci was significant; however, no relationship could be established between number of foci and number of previous bouts. Cerebral atrophic changes without any significant relationship to the duration of the disease and the number of bouts were found in 15 of the 43 patients. Use of CT may furnish additional information in cases that present diagnostic problems or at the first manifestations of MS.

Adolescent↗

Symptomatology of the most severe form of tuberculous meningitis.

Seven cases of the most severe form of tuberculous meningitis, in which a midbrain syndrome developed, are reported. Three different types of progress were observed. Exudative inflammation and cerebral edema dominated in the first group, causing the rapid development of the acute midbrain syndrome, which may turn into a bulbar syndrome. In the second group the development of the midbrain was delayed and an apallic syndrome followed. The morphological examination disclosed local diencephalic and midbrain lesions caused by herniation and specific vasculitis and vascular compression. The third group showed disintegration of cortical function as a result of parenchymal lesions, apart from local midbrain symptoms which never fully intensified into the midbrain syndrome. Observation of the progress of the disease proved that late diagnosis and delayed therapy were decisive in cases of the most severe form of tuberculous meningitis.

Adolescent↗

[Cerebral manifestation in a case of Sjögren's syndrome (author's transl)].

A case of Sjögren's disease is described in which isolated cerebellar symptoms were prominent. Serological investigations gave no evidence of disseminated immune vasculitis, systemic lupus erythematosus or any other autoimmune disease. The cerebrospinal fluid protein changes, which were diagnostic of an autoimmune process in the CNS and suggestive of Sjögren's syndrome are discussed.

Aged↗

Serum cortisol levels of multiple sclerosis patients during ACTH treatment.

The adrenocortical function in 52 multiple sclerosis patients was studied and compared to that of ten healthy control subjects. The basic secretion rate of cortisol of the MS patients was within the range of normal. Reactivity of the adrenal cortex to stimulation by synthetic ACTH was markedly reduced independent of the duration of the disease with the exception of four patients who were in an early stage of the disease. No difference could be demonstrated between patients undergoing a bout and patients in a stage of remission. The pathogenetic and prognostic significance of the results of the present study are discussed.

Adrenocorticotropic Hormone↗

[Immunoreactions of the delayed type in patients with multiple sclerosis (author's transl)].

Skin tests were performed in 34 multiple sclerosis patients. The incidence of positive reactions was reduced in these patients compared with healthy controls, with regard to different recall antigens with the exception of varidase, as well as the PHA and DNCB. No definite differences in reaction between patients who had been suffering from multiple sclerosis for a long time or for a short time, could be established. However, there was a certain dependence on the stage of the disease in so far as positive reactions were less frequent during the acute episode--more pronounced during the subsiding attack than at the onset of the episode--, than during the interval between two attacks. These results suggest that multiple sclerosis is primarily characterised by a weakness of cell-mediated immunity and that this weakness becomes more pronounced during the acute episode. The differences between the skin test reactions performed during the individual phases of the disease are too slight to assist in defining the acute episodes. It may be possible to identify changes in the reaction level via long-term studies.

Humans↗

[The application of guanidine hydrochloride to the treatment of degenerative nervous and muscular diseases. I. Clinical results (author's transl)].

This paper reports the results obtained on using guanidine hydrochloride in the treatment of patients with amyotrophic lateral sclerosis, degenerative diseases of the spinocerebellar system or the peripheral nervous system and dystrophic muscle diseases. A long-term effect of the substance was a diminution in the rate of progression of the diseases, with the exception of the group with dystrophic muscle diseases. Initial clinical improvement occurred in certain patients of both groups. The substance seems to be more effective in less-advanced cases than on administration in the later stages of the disease. The therapeutic dosage was 20 to 40 mg/kg/day. The most frequent side-effect was paraesthesia and sometimes gastric disturbance was reported. Therapy had to be discontinued in 3 patients due to leucopenia. In these patients the symptoms rapidly increased in severity after discontinuation of treatment. This supports the assumption that guanidine hydrochloride treatment slows down the progress of the disease.

Adult↗

Further clinical experiences of a phase I study with the hypoxic cell radiosensitizer misonidazole.

Since April 1976 we have performed clinical investigations with multiple doses of the hypoxic cell radiosensitizer misonidazole in 21 patients. A significant side effect of the drug was the development of peripheral sensory neuropathies in 13 patients (8 mild, 5 severe) and of a transient acute organic psychosyndrome in 2 of the 5 patients with a severe polyneuropathy. The severity of the polyneuropathies is related to the total dose of misonidazole and the overall time of drug administration. Treatment schedules designed to obtain the desired sensitizing effect without neurological side effects are under investigation.

Adolescent↗

Electrophysiological studies on the "stiff-man" syndrome.

In addition to anamnestic and clinical data electrophysiological and pharmacokinetic investigations may be necessary for the diagnosis of stiff man syndrome. Continuous activity of motor units with superimposed bursts during muscular spasms was found by electromyography in the two patients reported. Rigidity and continuous activity disappears during sleep, after i.v. application of Tubocurarine and Diazepam, during Thiopenal anesthesia and after neural block with Procaine. Dipropylacetate and Baclofen improved the condition but did not lead to complete relaxation. Biperidenlactat and Magnesiumlaevulinat have only a temporary effect on rigidity. Neostigmine, Phenytoine, Glycine, Dopa and 5-Hydroxy-Tryptophan had no effect. Passive shortening or stretching of the m. biceps brachii as well as touching the skin increased motor activity which spread to other segments and to the contralateral side. The H/M ratio was increased but the silent period was normal. A combination of Diazepam and Dipropylacetate or Clonazepam was therapeutically effective in the cases reported. A central genesis, of the pathogenetic mechanisms discussed, is the most probable in our cases.

Brain↗

[The efficacy of guanidine hydrochloride in the treatment of Werdnig-Hoffmann disease (author's transl)].

The use of guanidine hydrochloride is reported in the treatment of 4 cases of Werdnig-Hoffmann disease. Improvement was achieved in two cases. Amelioration was initially observed in the third patient, but a relapse occurred during temporary discontinuation of therapy and the disease progressed, albeit at a slower rate, after resumption of therapy. The final case was a terminal one in which, however, slight temporary improvement occurred in response to therapy. A maximum dosage of 0.4 g (based on the assumption of an average body weight of 15 kg in these children) was reached by gradual increments. Side effects like shortlasting vomiting were observed only in case 1. The pharmacological action of guanidine hydrochloride is discussed. According to Otsuka and Endon the mechanism of action may be based on an increase in the end-plate potential amplitude. However, an augmented supply of spinal transmitters may also play a role in the mediation to the pharmacological action.

Child, Preschool↗

Initial clinical experience with the radiosensitizing nitroimidazole Ro 07-0582.

The 2-nitroimidazole derivate Ro 07-0582 is known from experimental studies to be a very efficient radiosensitizer of hypoxic cells. Experiences with its clinical use are very limited so far. This study reports the side effects observed in the use of this drug in 12 patients. Nine of these patients were suffering from brain tumors, 3 from extracerebral tumors. In 8 of our 12 patients a sensoric polyneuropathy occurred which was strongly related to the total dose of the drug administered. Polyneuropathy was observed on the average after a total dose of 26 grams, but was already noticed at doses as low as 22 grams. It showed a good tendency for remission after termination of the drug treatment and was reversible in all patients within 4 weeks. In one patient with a brain tumor a severe organic psychosyndrome occurred which is possibly related to the drug. This side effect was also reversible. In this small group of patients the critical dose limit seems to be 29 grams. This dose can be divided into 6 fractions of 60-80 mg/kg. Up to the present knowledge this single dose is likely to give an enhancement ratio of 1,5, which would be extremly valuable for the treatment of hypoxic tumor cells.

Adolescent↗

[Organic dementia as a first symptom of infantile Huntington's chorea (author's transl)].

A loss of intellectual capacity at the age of 10 was the first symptom in a case of infantile Huntington's chorea. Subsequently a rigid hypokinetic syndrome occurred only at the age of 20. The dementia syndrome was confirmed in psychological tests. Auxiliary findings such as a diffuse abnornal EEG, as well as a dilatation of the lateral ventricles and an extension of the subarachnoid spaces in the PEG corresponded to a positive diagnosis of Huntington's chorea.

Adolescent↗

[Cerebral involvement in the Sézary syndrome (author's transl)].

Two foci of histologically verified mycosis fungoides were found in the left parietal region of a 45-year-old man with involvement of the brain during an exacerbation of leukemia. There was perivascular and diffuse infiltration with lymphoma-like cells and occasional giant (Lutzner) cells but no involvement of the meninges. Both before and after operation the CSF contained a few atypical lymphocytes and mononuclear cells, one of which showed ultrastructurally the deeply clefted nucleus of Sézary cells. Atypical cells were confirmed morphologically in the peripheral blood, as well as by cytochemical (beta-glucuronidase) and immunological (rosettes with SRBC) markers, after the postoperative leucocytosis had subsided. It is suggested that this CNS involvement during a leukemic conversion phase of mycosis fungoides is consistent with the Sézary syndrome.

Brain Neoplasms↗

[Hypokinetic appearance of huntington's chorea during the childhood (author's transl)].

During the last two years two patients with hypokinetic appearance of Huntington's chorea--beginning in childhood--were investigated in the Neurological Univ.-Clinic of Vienna. Both of the patients descend from families with primary hyperkinetic Huntington's chorea in adult life. The problem of the early manifest hypokinetic form--when the family case history is not attended--are of special interest for the first symptoms can lead into false diagnosis. Therefore oftenly systematic treatment will be done at first just during the adult life, when already the complete picture of Huntington's chorea has appeared. From the point of eugenic deliberations it is very interesting for relatives of families with Huntington's chorea to be informed about the heredity, the most lately appearance of the disease and by the possiblity of anteposition.

Adult↗