Two female patients with the typical clinical and electrophysiological features of the stiff-man syndrome, both responded to steroid treatment. ACTH infusion produced an immediate clinical relief of muscle contracture and cramps, with parallel marked reduction of the EMG pattern of continuous spontaneous activity in agonist and antagonist muscles. Apart from this effect, a more delayed response to oral prednisone was observed in both cases and steroid-dependence in one of them, who also exhibited instrumental and laboratory findings suggesting an inflammatory process. These data lead us to consider a possible dysimmune pathogenesis of some cases with the stiff-man syndrome.
Maximum motor conduction velocity of the median, ulnar and peroneal nerves and maximum sensory conduction velocity of the median nerve have been studied in 635 children, below 12 years of age, free from peripheral nervous system disease. The children fell into four age-group: from 0 to 1 year; from 1 to 3 years: from 3 to 6 years; from 6 to 12 years. No normal values were recorded for the sensory conduction velocity of the median nerve under the age of one year. The motor conduction velocity values significantly rise for the median and ulnar nerves up to 1 year, for the peroneal nerve up to 3 years. The sensory conduction velocity values of median nerve increase significantly up to 6 years.
Peroneal motor and sural sensory conduction velocities (MNCVs/SNCVs), somatosensory evoked potentials to median nerve stimulation (MN-SEPs) and motor evoked potentials (MEPs) to transcranial stimulation were examined in 138 HIV-infected patients (in the different stages of the disease), 20 seronegative intravenous drug abusers (IVDAs), and 20 healthy subjects. Findings of peroneal MNCV slowing in patients ranged from 16% (asymptomatic HIV patients) to 63% (AIDS) and of sural SNCV slowing from 13% to 40%. Altered MN-SEPs ranged from 10% to 30%, and MEPs ranged from 44% to 72%, mostly due to a prolongation of the central motor conduction time (CMCT). All seronegative IVDAs showed patterns within the normal range. Electrophysiological techniques were helpful in demonstrating early and subclinical alterations in HIV patients.
In children and adolescents, schizophrenia is one of the ten main causes of disability-adjusted life years. The identification of people at Clinical High Risk of developing Psychosis (CHR-P) is one of the most promising strategies to improve outcomes. However, in children and adolescents research on the CHR-P state is still in its infancy and the clinical validity of at-risk criteria appears understudied in this population. Furthermore, only few studies have evaluated the psychopathological, neuropsychological, neuroimaging characteristics and, especially, long-term outcomes of adolescents at high risk. We present here the protocol of an innovative longitudinal cohort study of adolescents aged 12-17. The sample will consist of patients admitted to a third level neuropsychiatric unit, belonging to one of the following three subgroups: 1) adolescents with established Diagnostic and Statistical Manual of Mental Disorder–Fifth Edition psychosis, 2) adolescents with CHR-P, and 3) adolescents with psychiatric symptoms other than established psychosis or CHR-P. The primary aim of our study is to evaluate the 2-year prognosis across the three groups. We will measure transition to psychosis (or the stability of the diagnosis of psychosis in the psychotic group), the risk of development of other psychiatric disorders, as well as socio-occupational functioning at outcome. The secondary aim will be to explore the effect of specific predictors (clinical, neuropsychological and neuroimaging factors) on the prognosis. At baseline, 1-year and 2-year follow-up participants will be assessed using standardized semi-structured interviews and instruments. Psychopathological and functioning variables, as well as neuropsychological domains will be compared across the three subgroups. Moreover, at baseline and 2-year follow-up all recruited patients will undergo a 3-Tesla magnetic resonance imaging examination and diffusion tensor imaging parameters will be analyzed. We believe that this study will advance our ability to predict outcomes in underage CHR-P samples. In particular, our data will enable a better understanding of the clinical significance of CHR-P in adolescents, and shed new light on prognostic factors that can be used to refine the prediction of clinical outcomes and the implementation of preventive interventions.
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