Rivista di neurologia最新文献

We report three young patients, two male and one female, with B.F.A., followed up clinically and neurophysiologically (two after a muscle biopsy) for 13-15 years. We confirm also in Italy the existence of this monomelic type of muscle atrophy (Hirayama type), already described in other countries, which shows a mild progression of the amyotrophy during the first two years and thereafter remains stationary. In our opinion the disorder represents a segmental transitional form of A.L.S. in which some "delaying" factor, unfortunately not yet demonstrated, is operating. In fact the neurophysiological studies, apart from its attribute of remaining segmentary, do not show differences between B.F.A. and the other forms of motoneuron disease.

During a viral meningoencephalitis, a 60-year-old woman developed a full parkinsonian syndrome, unchanged four months after the onset. The viral researches demonstrated an increase of the anti-CMV antibodies, especially in the cerebrospinal fluid. CSF's and neuroimaging data are discussed.

Most of Parkinson's disease patients treated with Levodopa develop the Long Treatment Levodopa Syndrome. Many authors showed a correlation between clinical features and plasma level of Levodopa. In our study, five parkinsonian patients with severe clinical response fluctuations, oral levodopa treatment was replaced by repeated continuous infusions of Levodopa (with oral carbidopa). Our results confirm that repeated intravenous infusion are very effective in PD patients with LTS.

The present study describes a case of subacute thyroiditis characterized by involvement of ocular motility that appeared many months before bioumoral findings of the disease. The authors believe that when the etiological diagnosis of an ophthalmoparesis, especially when isolated, is uncertain, one must carefully evaluate the function of the thyroid and levels of the specific antibodies, in order to start the appropriate treatment.

Seventy five adult patients suffering from partial epilepsy were investigated by MRI. Results were then compared with those obtained with CT scan and EEG analysis. The interval between the two neuroradiological studies did not exceed five years. MRI and CT showed abnormalities respectively in 45 and 55% of patients, MRI showed a better sensitivity in detecting ischemic or atrophy-gliosis chronic focal alterations. In the remaining lesions such as tumors, vascular malformations, cysts and diffuse atrophies, where often an urgent diagnosis is necessary, both tests were equally sensitive. EEG showed alterations in 80% of patients and agreed with results of CT scan and MRI in about 80% of cases.

The passive (tilting) orthoclinostatic test was performed on 44 young subjects affected by type 1 insulin-dependent diabetes. In 11 cases the test was repeated after a 3-4 years interval. The results, compared with age matched controls, showed in the diabetic patients a supersensitivity of the two antagonist systems, increasing with age and duration of the disease, up to an overexcitability and a functional exhaustion. It would thus seem that diabetic dysautonomia involves not only the peripheral nervous system but also the hypothalamic centers, with an impoverishment of the capacity of self-regulation. The hypersympathicotonus in diabetic young patients seems to be only apparent, as a result of the hypersensitivity of an overstimulated system.

The authors, in this case, stress the importance that cases of N.M.S. can arise as possible complication of sulpiride treatment, a drug of large use and with less unwished extrapyramidal effects that other neuroleptics.

In myotonic dystrophy alteration in membrane excitability characterizes, in addition to the dystrophic process, modifications of contractile function detectable after fatigue. To verify in which extent sarcolemmal activation or contractility mechanisms are involved in fatigue, some electrophysiological and dynamometer parameters have been studied in tibialis anterior of 5 myotonic dystrophy patients. Evaluation has been performed basally and after protocol consisting of intermittent isometric voluntary contractions lasting 15', at two different muscle lengths, optimal and short. Administration at several recovery times of 20 and 50 Hz tetanizing sequences shows how processes distal to membrane excitability are mainly responsible for fatigue at optimal length, while relative potentiation of excitability preserves the muscle from excessive force loss at short length.

A 41 year-old-man developed diplopia, bilateral ptosis, paralysis of conjugate eye movement upwards and horizontal, with dilated and fixed pupils, after an influenza syndrome. Radiological, cerebrospinal fluid and electrophysiological data were normal, except BAEPs. The different aetiologies and pathogenesis are discussed.

Propofol is a new anaesthetic agent commonly used because of its rapid pharmacokinetic. Lately, anecdotal reports suggest its utility in the treatment of convulsive status epilepticus. We describe four cases of convulsive status due to severe encephalopathy of various etiology. They were resistant to diazepam and other drugs and remitted only after treatment with propofol. The infusion, even protracted to 8 days, did not cause any toxic or proconvulsive side-effects. The very short duration of its central depressant action permitted monitoring of the underlying neurological status whenever needed.