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Biomedical subjects

D Gambi

Publications and source records attributed to D Gambi.

At least 55 records · Page 3Linked to original sources

Benign monomelic amyotrophy of lower limb: report of three cases.

Three patients with wasting confined to a single lower limb are reported. The characteristic features were: sporadic occurrence, insidious onset with slow progression and in 2 cases arrested course for at least 4 years, wasting out of proportion with disability, absence of sensory, pyramidal tract or bulbar signs. CK, motor and sensory conductions, and lumbar MRI were normal. Muscle CT showed selective loss of muscle tissue and fat replacement in posterior leg muscles. Quantitative electromyography and histologic findings revealed neurogenic features not only in the affected legs, but also in clinically uninvolved limbs. Monomelic amyotrophy of lower limb is a variant of spinal muscular atrophy with a benign course. However, as in the early stages of the disease there are no distinctive clinical or laboratory findings with other motor neuron diseases, the diagnosis of monomelic amyotrophy may be made only retrospectively after a prolonged observation.

Adult↗

P3 recordings in patients with bilateral temporal lobe lesions.

We recorded event-related potentials to an acoustic "oddball" paradigm from 19 scalp derivations in five patients (three women and two men; age range, 44 to 56) who had global amnesia following encephalitis. CT and MRI showed severe bilateral lesions of anterior and midtemporal lobes. A P3 component, with a peak latency within the normal limits for age-matched controls, was recorded to "target" stimuli in four patients from all leads except Fp1, Fp2, F7, F8, T3, and T4. In the fifth patient, the P3 peak was delayed.

Adult↗

Evoked potentials (EPs) in experimental allergic encephalomyelitis: a study of EP modifications during the course of a controlled disease.

24 juvenile strain 13 guinea pigs were sensitized with homologous spinal cord homogenate in complete Freund's adjuvant in order to obtain Experimental Allergic Encephalomyelitis (EAE) in the Chronic Relapsing (CR-EAE), Chronic Progressive (CP-EAE) and Acute (A-EAE) forms. Visual, Brain-Stem Acoustic, Somatosensory Evoked Potentials were recorded in these animals, prior to the sensitizing injection and during the course of the disease. The aim of our study was to observe modifications of Evoked Potentials during progression of a demyelinating disease. Latency delays of Evoked Potentials appeared 15 days after the sensitizing injection, preceding or simultaneously with clinical alterations and when demyelinated plaques were not detected in anatomical specimens.

Acute Disease↗

Three-dimensional transcranial Doppler in acute ischemic stroke in the territory of the middle cerebral artery: clinical and CT correlation.

We studied 34 patients with acute ischemic stroke in the territory of the middle cerebral artery (MCA) by three-dimensional transcranial Doppler (TCD-3D). The parameters analyzed were: mean blood flow velocity, systolic and diastolic velocities; indices of pulsatility, hemisphere asymmetry and pulsatility transmission. Of the 34 patients 11 presented marked slowing of flow velocity in the MCA on the infarct side with an asymmetry index (AI) of over 40%, 8 patients with slightly reduced flow velocity in the MCA and an AI of 25-40%, 2 patients in whom there was indirect evidence of collateral circulations in the anterior cerebral artery distribution together with slowing of MCA flow; 5 patients had stenosis of the MCA, 9 patients showed no alterations of the Doppler parameters. The correlation between neurological symptom pattern and AI was significant (r = 0.76). Noninvasive, easy to perform, performable at once and reliable, TCD-3D is a great improvement on traditional transcranial Doppler and is especially useful in assessing the hemodynamics of the cerebral circulation in ischemic stroke.

Acute Disease↗

Persistence of a P3 component in severe amnestic syndrome.

Event-related potentials (ERPs) to an odd-ball paradigm were recorded from 19 scalp derivations in a patient (47 years old, housewife) affected by complete amnestic syndrome following limbic encephalitis. CT scans and magnetic resonance imaging showed severe bilateral lesions of anterior, mid-temporal lobes and frontal lobes. A P3 component, with a peak latency inside normal limits for age-matched controls, was recorded to 'target' stimuli from all leads except Fp1, Fp2, F7, F8, T3 and T4.

Amnesia↗

CD21+ (B2 antigen+) cell decrement and CD4+CD29+ (helper-inducer) cell increment suggest an activation of cell immune reactivity in multiple sclerosis.

Two-color flow cytometric analysis on peripheral blood lymphocytes of 35 untreated multiple sclerosis (MS) patients, 17 other medical disease (OMD) patients and 14 healthy control (HC) subjects was performed to evaluate the levels of different T and B cell subpopulations. In MS patients we observed an increase in CD4+CD29+ helper-inducer cells but this increase was not related to the different phases of the disease. We hypothesize that this change is related to the reduction of CD21+ cells expressing B2 antigen, a 140 kDa molecule disappearing after B cell activation. An increased level of CD4+CD45RA- (helper-inducer-like cells) and a reduction of CD4+CD29- (suppressor-inducer-like cells) were also present in our patients. These findings demonstrate an immune 'disequilibrium' in MS, which is linked with an increased level of CD25+ cells expressing the interleukin-2 (IL-2) receptor. IL-2, besides being a T cell growth factor, is also a B cell growth factor. These data let us hypothesize that an activation of the immune response is present in MS.

Adult↗

HTLV-1-associated myeloneuropathy in an Italian.

A 58-year old man presented with slowly progressive spastic paraparesis, ataxia, absent ankle jerks, bladder disturbances, impairment of vibration sense and mental deterioration. Electrophysiological studies documented axonal sensory neuropathy, posterior column and optic nerve involvement. Serum tests for anti-HTLV-1 antibodies were negative but HTLV-1 proviral sequences were consistently demonstrated in white blood cell genomic DNA using the polymerase chain reaction technique. Western blot and polymerase chain reaction assays of sera and DNA from family members were negative for HTLV-1. The most likely cause of infection in this patient was a blood transfusion received 2 years before onset of symptoms. This is the second Italian case of HTLV-1 associated myelopathy and the fourth reported in white subjects living in Europe.

Amino Acid Sequence↗

Mapping of event-related potentials to auditory and visual odd-ball paradigms in patients affected by different forms of dementia.

The paper reports the results of recordings and maps of event-related potentials (ERPs) in patients with Alzheimer's disease (AD), progressive supranuclear palsy (PSP) and in subjects affected by dementia in multiple sclerosis (MS). ERPs were recorded from 19 scalp electrode derivations using both visual and acoustic paradigms. In 43% of AD patients, ERPs were normal; in 20%, although present, ERP components were delayed, while in the other 37% none of the N2 and P3 peaks could be recorded, because of abnormal topography of potentials on the scalp. In patients with PSP, the normal ERP sequence was not identified. In patients with MS delayed ERPs (50%), abnormal topography of ERPs (30%) and absence of ERPs (20%) were observed. The follow-up of AD patients showed a progressive alteration of ERPs. ERP topography alterations were observed in AD, PSP and MS patients with poorest cognitive performances.

Acoustic Stimulation↗

Multiple sclerosis: an immune system activation disease.

Two-color flow-cytometric analysis on peripheral blood lymphocytes of 46 untreated multiple sclerosis patients (MS), 36 other medical disease patients (OMD) and 19 healthy control subjects (HC) was performed to know the relationships between T and B cell subpopulations. In MS patients we observed an increase of total lymphocyte count and an increase of CD4+CD29+ cells, which are adjuvant to B cell in antibody production. We hypothesized this change is related to the reduction of CD21+ cells, expressing B2 antigen which disappears after B cell activation. The unperfect balance of immune system in MS was also demonstrated by the increased level of CD25+ cells in relapsing-remitting patients and by the decreased level of CD4+ CD45RA+ (suppressor inducer) cells in progressive patients.

Adult↗

CNS involvement in chronic inflammatory demyelinating polyneuropathy: an electrophysiological and MRI study.

Eight consecutive patients with chronic inflammatory demyelinating polyneuropathy (CIDP) were investigated by visual evoked potential (VEP), brainstem auditory evoked response (BAER) and magnetic resonance imaging (MRI) to assess central nervous system (CNS) involvement. VEPs were abnormal in 6 patients and BAERs in 2. MRI showed changes suggestive of CNS demyelination in 2 cases. Our findings suggest the existence of a combined central and peripheral demyelinating syndrome and emphasize the possibility of a common pathogenetic mechanism for both.

Adult↗

24-month follow-up of multiple sclerosis patients treated with cyclophosphamide.

15 patients with remitting-progressive multiple sclerosis received a course of intravenous cyclophosphamide followed by a booster dose every 60 days for 2 years. There were no severe short or medium term side effects. Half of the patients presented clinical stabilization and substantially unchanged Kurtzke rating at 2 year follow-up. Since the aim was stabilization only, our findings argue for continuation of the study in young patients with very short shift.

Adult↗

F response in vascular and degenerative upper motor neuron lesions.

The F response represents the recurrent discharge of a small percentage of the motoneuron pool activated antidromically by any single impulse delivered to the nerve. We studied F waves from median nerve stimulation in 22 controls, in 30 patients with spasticity after cerebrovascular accident (CVA), and in 4 patients with familial spastic paraplegia (FSP). The following parameters were analyzed: median nerve conduction velocity, median nerve M response amplitude (M), average amplitude of 16 F responses (F16); persistence of F response (Fp) defined as the percentage of measurable responses to 16 stimuli, average amplitude of F response expressed as percentage of maximal M amplitude: (F16/M%). Results showed that Fp, F16 and F16/M% values were significantly increased on the spastic side of CVA patients and in FSP. Our findings indicate that after upper motor neuron lesions (whatever etiology and course) a change in the frequency of recurrent discharge and/or in the total number of lower motor neurons capable of backfiring occurs. Therefore, F response reflects the hyperexcitability state of the lower motor neurons in spasticity.

Adolescent↗

Orthodromic median and ulnar fourth digit sensory conductions in mild carpal tunnel syndrome.

Because the fourth digit (D4) has a dual innervation, median and ulnar D4 sensory conduction velocity (SCV) comparison may be useful in diagnosing the carpal tunnel syndrome (CTS). We studied 50 control hands and 41 hands with recent onset symptoms and signs of CTS but normal median distal motor latency and normal SCV from the second digit (D2). In CTS, D4 SCV was significantly slower than D2 SCV and D4 median and ulnar sensory conduction difference was abnormal in 38 hands (92%). In 36 CTS hands (87%), but in no control hand, a double peak potential could be recorded over the median after D4 stimulation providing an immediate visual confirmation of the diagnosis of CTS. Comparing median and ulnar D4 SCV is a very sensitive method to detect early or mild CTS and should be used whenever conventional electrodiagnostic studies are normal or borderline.

Adolescent↗

Experimental allergic encephalomyelitis in guinea pig: variability of response to intradermal emulsion injection.

Different forms of experimental allergic encephalomyelitis were obtained in 4 groups of guinea pigs: 7 adult Hartley guinea pigs (Group I), 12 adults of the same strain (Group II), 6 juvenile strain 2 guinea pigs (Group III) and 6 juvenile strain 13 animals (Group IV), by the injection of emulsions. Groups I and II received emulsions containing 250 mg and 500 mg respectively of fresh isologous spinal cord tissue, complete Freund adjuvant (CFA) and saline solution while Groups III and IV received an emulsion containing 120 mg of isologous spinal cord, CFA, saline solution and 15 mg of Mycobacterium tuberculosis. The increased antigen load induced a disease with delayed onset and prolonged progressive course (C-P-EAE) in Groups I and II, although 8 animals showed no symptoms of illness. The findings in C-P-EAE were large demyelinated plaques, perivenous fibrosis and large areas of infiltration. Demyelinated areas occurred within the spinal cord white matter only in two asymptomatic animals. C-P-EAE was obtained in 4 of the Strain 2 animals. In conclusion, the increased antigen load induced a range of lesions in Hartley guinea pigs, although not all animals were affected. C-P-EAE was induced also in strains of guinea pig other than the Heartley strain. These different reactions may have been the outcome of partial or complete inactivation of the cell-mediated response to the inoculated antigens.

Acute Disease↗

Orthostatic tremor: report of two cases and an electrophysiological study.

Two patients with legs tremor present on standing, but none on walking or sitting, are reported. Tremor was not exclusive or orthostatism and was also evoked by strong tonic contraction of leg muscles. Synchronous EMG bursts were recorded in antagonistic muscle groups at 8-10 Hz in the first patient and at 16 Hz in the second. EMG activity was synchronous in corresponding muscles of both legs. The occurrence of EMG activity was not influenced by stimulation of nerve afferent fibers. We suggest that this movement disorder may be an exaggeration of physiological tremor due to synchronization of motor units by spontaneous oscillations in central structures.

Aged↗

GM1 ganglioside therapy in acute ischemic stroke. Italian Acute Stroke Study--Hemodilution + Drug.

Eleven of 31 clinical centers participating in the Italian Acute Stroke Study--Hemodilution carried out a preliminary study on the effectiveness of ganglioside GM1 in acute stroke; 502 patients were randomized to GM1 (GM1, n = 121), GM1 plus hemodilution (GM1 + H, n = 128), placebo (P, n = 130), or placebo plus hemodilution (P + H, n = 123) groups less than or equal to 12 hours after onset of a hemispheric cerebral infarct. The patients were treated for 15 days and were evaluated on Days 21 and 120 after the onset of stroke. Intention-to-treat analysis failed to show any differences in neurologic deficit, mortality, or neurologic disability among the groups. Efficacy analysis showed a significantly higher degree of neurologic improvement in GM1 group patients compared with patients in the P group during the first 15 days. GM1-treated patients (GM1 and GM1 + H groups) showed a significantly higher degree of neurologic improvement during the first 10 days compared with the placebo-treated patients (P and P + H groups). These differences were no longer statistically significant at Day 120. Our results provide a rationale for the planning of a larger, multicenter trial of GM1 ganglioside in acute stroke.

Aged↗

Neurophysiological studies of L-acetylcarnitine administration in man.

Systemic administration of L-acetylcarnitine HCl (LAC) increases in human subjects the amplitude of visual evoked potentials (VEPs) obtained with patterned elements of 7.5 min of visual angle, of steady-state VEPs obtained with intermittent luminous stimulation, of EEG theta, alpha and beta bands. The latency of the "cognitive" P300 potential obtained with an auditory "oddball" paradigm was also reduced by LAC injection, while the amplitude of this potential was increased. These results were obtained in control volunteers and in patients affected by different forms of dementias. The modifications induced by LAC appear 10-15 min after the i.v. injection and last for 50-90 min. These results parallel previously described findings of animal experiments and suggest an effect of LAC on cholinergic neurotransmission.

Acetylcarnitine↗