Rivista di neurologia最新文献

We describe a 55 years old man affected by eyelid ptosis, mild ophthalmoplegia externa and severe dysphagia owing to pharyngoesophageal dyskinesia. Skeletal and eyelid constrictor muscles EMG showed myogenic alterations. Muscle histological findings of ragged red fibers and oxidative histochemical alterations, together with ultrastructural investigation clue for mitochondrial myopathy diagnosis. This, considering the unusual clinical aspects of the case, confirms as mitochondrial disease can widely vary in their phenotypical expression.

T cell clones derived from the cerebrospinal fluid of patients with multiple sclerosis were investigated for their ability to produce IL2, IL4, IFN gamma and TNF alpha. As controls, liver infiltrating T lymphocyte clones from patients with chronic active hepatitis were used. All CSF clones (both CD4+ and CD8+) produced high amounts of IFN gamma and particularly of TNF alpha. TNF was synthesized in a significantly higher amount than control clones. Moreover, they were capable of secreting IL2 but not IL4. From our results we conclude that CSF-CD4+ T clones could constitute a subset with functional properties similar to those of the Th1/inflammatory cells of the mouse. The unusually high amount of TNF produced by CSF derived T cell clones strongly suggests a significant role for this cytokine in MS immunopathogenesis.

Recent findings suggest that patients affected by Alzheimer's disease (AD) have evident alterations of smooth-pursuit ocular movements induced by a sinusoidal predictable target. For a better understanding of the possible modifications of this performance in patients with AD we evaluated, using a quantitative method, the smooth-Pursuit induced by ramps with constant, unpredictable velocities. Twelve patients with probable AD were studied. These patients were compared with twelve age matched healthy volunteers. Visual inspection of our recordings shows that AD patients have frequent, large saccades intrusions which in some cases appeared to disrupt smooth tracking, particularly for low target speeds. Furthermore, the smooth-pursuit eye velocity induced by target of high speed is significantly reduced in respect to that present in our control group. Some significant correlations between altered smooth-pursuit parameters and abnormal psychometric scores were noted.

In order to verify the sensory nerve conduction velocity (SNCV) by median nerve palmar stimulation regard to each single finger, we tested 48 patients with typical CTS symptoms, confirmed by neurophysiological examination. The control group consisted of twenty-seven healthy volunteers, of similar mean age. The SNCVs were obtained by stimulating the II finger (M2), III (M3), IV (M4) and II and III intermetacarpo-phalangeal site (respectively P2 and P3) for median nerve; IV finger (U4) and IV intermetacarpophalangeal site (P4) for ulnar nerve. Data show that P2 was normal in 3 cases (6.2%), as was M2; M3 in 2 cases (4.2%) and P3 only in one case (2.1%). No sensory nerve action potential was recorded in 3 case (6.2%) during P3 stimulation, in comparison to 14 cases (29.2%) during M4 stimulation. When each case was examined separately, the behaviour of palmar SNCV resulted similar to the corresponding fingers. Finally, the ratio between SNCV values of P2/P3, with respect to controls, showed a further more severe damage of P3 compared to the P2, generally preferred by other authors.

A 45-year-old woman with mild thrombocytosis secondary to iron-deficiency anemia suffered from hemispheric infarction in absence of vascular, cardiac or coagulation pathologies. The modest raise of the platelets count seemed as the sole possible cause for the cerebral infarction. We wish to emphasize the inconstant benignity of mild non essential thrombocytosis at levels less than 1 million/cu.mm.

Sleep-EEG of two subjects affected by Progressive Supranuclear Palsy was studied. Morphological, reduced and poorly organized sleep transients (spindles) in stage 2 and decreased REMs during desynchronized sleep were observed. The quantitative sleep analysis showed a poor and fragmented sleep with an increase of stage 1 and stage 3 and a decrease of stage 2 and REM sleep.

Tardive dyskinesia consists of abnormal involuntary movements at the oro-facial area (mouth, tongue, maxillary) or generalized choreoathetotic disorders of the limbs and trunk occurring in at least 10-20% of chronically neuropsychiatric patients exposed to neuroleptics. Age (over 50), gender (female), affective disorders, individual predisposition, type of drug, dosage and duration of neuroleptic exposure (over 3 months), anticholinergics, appear to be risk factors. In this brief review some current pathophysiological mechanisms and clinical therapeutical trials are also discussed.

A 68 year-old hypertensive woman carried out, linked with the featuring complaints of temporal arteritis-polymyalgia rheumatica complex, an orthostatic hypotension caused, probably, by a spread immunocomplex damage. The presence of depression in Horton's Arteritis and its improvement with the complete recovery would show, perhaps, a central catecholaminergic failure.