Ataxia-teleangectasia: neurophysiological studies in 8 patients.

Eight patients affected by Ataxia-Teleangectasia (AT) were studied with a set of neurophysiological tests. The study was done two times between 2 and 10 years. The most characteristic feature was a severe involvement of the posterior spinal cord: it was associated with a moderate peripheral neuropathy as well as a severe peripheral neuropathy. These data could indicate that the dying back phenomena are not the only pathogenetic basis of the spinal cord degeneration in this disease.

Early involvement of central nervous system in type I diabetic patients.

OBJECTIVE: To investigate whether the asymptomatic involvement of the central and peripheral nervous systems may be an early complication of diabetes. RESEARCH DESIGN AND METHODS: We studied early impairment of the central and peripheral nervous system pathways in 15 type I diabetic patients with good metabolic control and short disease duration and in 10 healthy control subjects using a set of neurophysiological tests. RESULTS: Results in diabetic subjects showed 1) impairment of motor (7% and somatosensory (13%) pathways of the central nervous system, 2) impairment of motor and sensory conduction velocities (40-60%), and 3) normal values of the vibration perception threshold and cardiovascular autonomic tests. CONCLUSIONS: The damage is more evident in peripheral sites where hyperglycemia and aldose reductase pathways are more active. Instead, several episodes of hypoglycemia, which occur in type I diabetic patients in good metabolic control, may cause alterations of brain nervous cells.

[5-fluorouracil, cisplatin and retinol palmitate in the management of advanced cancer of the oral cavity. Phase II study]. / 5-Fluorouracile, cis-platino e retinolo palmitato nel trattamento delle neoplasie avanzate del cavo orale. Studio di fase II.

23 patients, with advanced oral cavity cancer, radically unresectable or relapsing after surgery and/or radiotherapy, were treated with a chemotherapeutic regimen, including 5-Fluorouracil 1000 mg/m2 and cis-platinum 20 mg/m2 for 5 consecutive days. Retinol palmitate was administered at the dose of 15.000 IU b.i.d. in chemotherapy intervals. In complete or partial responders, radiotherapy was planned on primary tumor. Chemotherapy treatment was well tolerated with G3 haematological toxicity in 30% of patients and G2 gastrointestinal toxicity in 20% of patients. Seven patients (31.8%) had a complete response (CR), 7 patients had a partial response (PR) (31.8%), stable disease was observed in 3 patients (SD) (13.7), while 5 patients progressed (PD) (22.7%). Median time to treatment failure was 5.6 months. Median survival, from start of chemotherapy, was 8 months 237 days (range 8 days-4 years). 12 patients received consolidation chemotherapy on the primary disease site. No patient developed a second tumor. A combined approach, in the treatment of oral cavity cancer, may give prolonged responses, with acceptable toxicity, without interfering on the quality of life.

Spinal cord inhibitory mechanisms in Parkinson's disease.

We studied two spinal cord inhibitory mechanisms, recurrent (Renshaw) inhibition and reciprocal inhibition, in seven patients with asymmetric Parkinson's disease in order to determine their contribution to the pathogenesis of rigidity. Recurrent inhibition, studied in the leg, did not differ from that found in normal subjects. All three periods of reciprocal inhibition, studied in the forearm, were present but reduced in magnitude compared with those observed in normal subjects. The arms, whether more symptomatic or less symptomatic, gave similar results. The diminution of all three periods of reciprocal inhibition is similar to the findings in patients with dystonia and is apparently indicative of an abnormal supraspinal influence on spinal mechanisms in these two disorders of basal ganglia function.

Contribution of electromyography to the diagnosis, treatment and prognosis of cervical disc disease: a study of 114 patients.

The aim of this study is to verify whether EMG has the same diagnostic and prognostic capacity in cervical root syndrome as it does in lumbo-sacral syndromes. Our population includes 114 patients affected by a cervico-brachialgia with EMG denervation provoked by cervical disc disease. 26 patients were submitted to a myelography confirming the discal origin of the syndrome: there were 3 cases of disc protrusion and 23 cases of cervical spondylosis. The agreement between EMG and myelographical data was significant in 20 cases (76.9%). There were no significant differences of evolution between operated and non-operated cases. EMG failed to provide prognostic criteria as it does in lumbo-sacral syndromes: it must hence be concluded that all patients presenting cervico-brachialgia and EMG signs of denervation should in any case be admitted to the hospital for exploratory examination. In this context, the particular role of EMG is to establish the degree of the muscular impairment, the number of roots involved, the duration of the syndrome and--recently--the identification of the intervertebral space to be explored by spinal CT.

Somato-sensory evoked potentials in diabetes type I.

Twenty-nine young patients with insulin dependent diabetes were studied. Clinical findings, metabolic profile, and neurophysiological data were correlated. Nerve conduction velocity (NCV) and Somato-sensory Evoked Potential (SEPs) were recorded stimulating the median nerve and the posterior tibial nerve bilaterally. High incidence of distal neuropathy was present in our cases plus a significant alteration of the plexus conduction, at the level of brachial plexus and of the cauda. We conclude that even in young diabetic patients the damage can be multifocal, and SEP technique could contribute to a more accurate study of neurological complication in the diabetic disease.

Radiation-induced brachial plexopathy: clinical and electromyographical (EMG) considerations in 13 cases.

The purpose of this study was to determine the clinical and electromyographical characteristics of the initial picture, and the subsequent course of the radiation-induced brachial plexopathy. The AA. studied 13 females affected by infiltrating carcinoma of the breast, who underwent a radical mastectomy with removal of the axillary cavity, followed by radiotherapy (total dose was 50 Gy per cycle). The symptom-free interval between the end of radiotherapy and the appearance of symptoms varied considerably (from one month to 15 years). The initial clinical picture presented pain (5 cases), paresthesia (6 cases) and motor deficit (8 cases). EMG examination showed a pluriradicular denervation in 10 cases (with a very frequent damage of the lower roots of the plexus) and a monoradicular denervation in 3 cases. In 6 cases it was possible to carry out a follow-up with repeated tests for 3-17 years after radiotherapy. The role of the EMG in the diagnosis and prognosis of this plexopathy is then discussed.

H-reflex recovery curve and reciprocal inhibition of H-reflex in different kinds of dystonia.

We studied the H-reflex recovery curve and reciprocal inhibition of the H-reflex bilaterally in the upper limb of 5 patients with generalized dystonia, 5 patients with blepharospasm, 10 patients with spasmodic torticollis, and 14 patients with writer's cramp. We compared the results with those obtained from a group of healthy volunteers. The recovery curve of the H-reflex was normal in patients with writer's cramp or blepharospasm, but showed an increase of the physiologic recovery at a 200 msec delay in patients with spasmodic torticollis or generalized dystonia. Reciprocal inhibition of the H-reflex showed a decrease in the amount of inhibition in all the patient groups and a facilitation of the H-reflex during the 3rd period of inhibition in the patients with spasmodic torticollis or generalized dystonia.

H-reflex in nonhomonymous muscles in the human forearm.

In 6 healthy volunteers, stimulation of the median nerve at the elbow elicited H-reflexes in muscles innervated by the ulnar nerve (flexor digitorum profundus of the 4th and 5th digits of the hand) as well as the median nerve (flexor carpi radialis). This finding offers direct physiologic evidence in humans of monosynaptic excitation from group Ia afferents to nonhomonymous muscles, a phenomenon for which only limited indirect evidence existed previously.

[Antitetanic immunity in a sample of the Tuscan population]. / Immunità antitetanica in un campione di popolazione Toscana.

In order to assess protection level against tetanus, the antitoxin titre was evaluated, by indirect haemagglutination test, in a sample, stratified by sex and age, of 805 subjects of three communes of Tuscany. On the whole the frequency of the serum protected is the 46.1% and it varies according to sex and age: under 20 years it is more than 90%, but it decreases in the successive ages, less in the male sex, probably owing to the vaccination made during the military service. The greatest percentage of immune subjects was observed in the most industrialized area. The less protected professional categories were pensioned people and housewives (25.8%). No differences of serum protection were found between those who practise or do not, recreative activities at the risk (sport, horticulture and gardening). No correlation was observed between the vaccinal anamnesis and the serological test.