99mTc-pyrophosphate muscle uptake in four siblings with Becker's disease.
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Biomedical subjects
Publications and source records attributed to B Rossi.
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The presence of clinical and electromyographic (EMG) features of neuromuscular hyperexcitability (NMHE) and of the commonly associated neurovegetative disturbances (NVD) were investigated in 49 patients with primary fibromyalgia (PF) and in a control group of 33 patients with rheumatoid arthritis (RA). At least two clinical features of NMHE were present in 39%, and at least three NVD in 63% of PF patients. In contrast, only 1 RA control had two NMHE features (p greater than 0.005) and three NVD (p less than 0.001). Moreover, a significant post-ischemic spontaneous EMG hyperactivity was observed in 11 PF patients, and in only 1 control with RA (p less than 0.05). Finally, in patients with PF the number of tender points were correlated with psychological tests for depression (p less than 0.02), and the number of NVD. The present study shows that in patients with PF there is a large prevalence of NMHE complaints and NVD. The potential underlying pathogenetic mechanisms are also discussed.
Stimulation of the first trigeminal branch using a 10 ms train of 5 pulses of 0.1 ms duration is performed on 17 healthy subjects (19-65 years of age) in order to assess the relationship between polysynaptic components of the blink reflex and perceptive and pain thresholds (ths). The mean value of tactile ths (Pth) is 2.22 +/- 0.86 mA; while R2, R3 and pain threshold values are 1.25 +/- 0.34, 3.52 +/- 1.94 and 4.78 +/- 2.77 times the Pth respectively. The pain ths average is 1.47 +/- 0.92 with regard to the R3 threshold. The R2 ths are very close to those of tactile perception, while those of R3 appear significantly related to the pain ths.
An interaction between pain modulation and arterial pressure control has been proposed on the basis of experimental data in man and animal. Eight hypertensive patients and eight normotensive volunteers were investigated by electrical stimulation of the first trigeminal branch and dental pulp, to evaluate nociceptive sensation and reflex responses. A significant threshold increase of pain sensation and R2, R3 polysynaptic components of the blink reflex, has been found in hypertensive patients.
To investigate whether pain and paresthesias could identify two different subclasses of small-fibre diabetic neuropathy, and to evaluate their relation to the metabolic control, we tested nerve conduction velocity (NCV) of median nerve (sensitive-SM, and motor-MM) and deep peroneal nerve (DP) in 48 diabetics (24 IDDM, 24 NIDDM) reporting pain (group A) or paresthesias (group B) that might be due to diabetic polyneuropathy. Glycated haemoglobin (HbA1c) was also assessed. No difference between group A and group B was found either in NCV, in all nerves tested, or in HbA1c. No relation was observed between NCV of nerves tested and HbA1c, duration of diabetes, age and type of diabetes in both groups.
This study was conducted on 15 outpatients with idiopathic Parkinson's disease, 10 on chronic levodopa therapy and 5 drug free, all complaining of painful syndromes. We investigated the perceptive and pain thresholds and the threshold of polysynaptic components of the blink reflex in patients and 8 controls. Our data indicate that as regards the perceptive, pain and R2, R3 thresholds, patients with and without levodopa therapy do not differ significantly from controls.
Since previous papers about the frequency of sexual dysfunctions (SD) in diabetic males did not consider the genetical and clinical heterogeneity of diabetes mellitus, we studied the prevalence and the pathogenesis of SD in 77 non insulin dependent diabetics (NIDD). The diagnostic procedures consisted of physical and psychological examinations, compilation of a questionnaire, evaluation of autonomic and peripheral nervous systems, measurement of penile arterious flow, dosage of hormonal and metabolic parameters. SD were present in 52% of patients: 24% of them had organic impotence, 28% psychological sexual impairment. Age, duration of diabetes and metabolic control were superimposable in the patients with and without SD. Most patients with organic impotence had an alteration of parasympathetic nervous system; less frequently a reduced penile arterious flow was observed. The relief of such a high prevalence of SD in NIDD males suggests that future studies should consider the various groups of diabetics separately.
Two family are described affected by a mild myotonic syndrome, characterized by almost exclusively presence of myotonic phenomenon, in absence of symptoms as muscular weakness, hypotrophy or hypertrophy ("myotonia levior"). The nosographic classification of this disease in the congenital myotonias is today uncertain, and it could be a case of a low expressivity variant of the dominant form (Thomsen disease), or the heterozygotic phenotype of the recessive form (Becker disease). Family history, clinical and electrophysiological data obtained in the patients seem to support first hypothesis, providing an useful element for a correct genetic counselling in these cases.
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In a group of spasmophilic subjects, symptoms imputable to the syndrome were compared with EMG signs of ischemic neuromuscular hyperexcitability, to evaluate their significance by logistic statistical analysis. Subjects with a duration of postischemic EMG spontaneous activity greater than the mean of normal values + 2.5 DS were considered positive for a spasmophilic syndrome. The following symptoms were selected as positive by the analysis: palpitations, nail and hairs abnormalities, headache, lipothymia; thermoregulation disorders and tremors were negatively related to the syndrome, while sleep disorders and asthenia emerged as positive in the logistic analysis but not significantly so.
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Four patients presenting ulcerative mutilating acropathy (UMA) (Thévenard's disease or Denny-Brown's hereditary sensory neuropathy) are reported: in 3 cases (2 of them are siblings) the neuropathy was familiar with a dominant transmission, the fourth case was sporadic. A fifth patient, member of one of the families with UMA, had a sensory motor neuropathy (type I of Charcot Marie Tooth disease) without acropathies. Following examinations have been performed: EMG and motor and sensory nerve conduction velocities in 4 cases, investigation of content of free aminoacids in serum and cerebrospinal fluid in 3 cases, kinetic arteriographies of the legs in 1 case, histology of sural nerve in 1 case. All patients had a mixed motor and sensory impairment. Neurophysiological tests showed in some cases a neural damage, in others a neuronal compromission, this suggesting different diseases. The presence of kiphoscoliosis, oligophrenia and loss of hearing, in some siblings of one family, is ascribed to an overlap with Friedreich disease. Links between Thévenard, Charcot Marie Tooth and Friedreich diseases are discussed.
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82 children (47 male and 35 female), aged between 2 and 12 years, with signs and symptoms consistent with spasmophilia, were investigated with an emg technique to better define boundaries of tetanic syndrome. In all subjects an ischaemic test was performed to the upper limb and the spontaneous emg activity from thenar eminence was recorded both during and after ischaemia. Moreover a clinical examination, with particular attention to objective signs of spasmophilia, were performed and serum electrolytes determined. Electromyographic spontaneous activity was noticed in 46 children (24 female, 22 male), 31 of them were hypocalcemic. The frequency of the various clinical manifestations is reported and correlated with emg and humoral findings. Significant difference between sexes in the incidence of tetanic syndrome where not noticed. More frequent symptoms for spasmophilia are tensive headache and cramps, abdominal spasms, psycomotos instability and anxiety; Chvostek's sign is frequent but no specific. The other symptoms assume relevance only if present in association. The role of electromyographic criteria in the detection of tetanic syndrome is stressed.
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