Somatosensory evoked potentials in Charcot-Marie-Tooth disease.

SEPs by median nerve stimulation have been performed in 18 adult patients (12 males and 6 females) affected by CMTD (type I, 13 patients; type II, 5 patients). All patients underwent MCV studies (median, ulnar, peroneal nerve), SCV studies (median and sural nerve), VEP, BAEP. N9 and N13 peaks were not detectable in 7/13 and 5/13 cases (HMSN type I) while cortical N19 were always recorded. Latency values of all responses were moderately or markedly delayed in all cases with HMSN type I, but proved normal or slightly delayed in HMSN type II cases. The prolonged latencies were mainly related to slowing of peripheral conduction. N9-N13 inter-peak was abnormally prolonged in 2 cases and N13-N19 in 2 other cases; both were prolonged in another case. In another 3 cases an abnormal BAEP was recorded. The few patients with abnormal CCT and BAEP probably belong to a borderline form between HMSN and hereditary ataxias.

Neurological symptoms of the sick building syndrome: analysis of a questionnaire.

Sick building syndrome (SBS) is an excess of work-related irritations of the skin and mucous membranes and of symptoms such as headache and fatigue in those working in modern air-conditioned buildings. We aimed to analyse the neurological symptoms, especially headache, in workers with potential SBS. The most frequent symptoms were headache and dry eyes. Sex was a major factor of difference: women report more symptoms than men. A positive correlation emerged between the number of symptoms and the asthenia scale score. Only 11 (8.2% of the whole sample) and 37 (27.4%) workers met all the IHS criteria for migraine and tension-type headache respectively. At least one symptom of SBS was present in 92.6% of workers. A negative correlation emerges between air conditioning and headache during working hours. No correlation emerges between the workplace comfort indicator and SBS and asthenic symptoms while a negative correlation was found between migraine and tension-type headache and comfort in the workplace. SBS symptoms are very frequent among all workers but headache is the primary symptom.

[Antibiotic prophylaxis in surgery. Results of a 4-year personal case series]. / Antibioticoprofilassi in chirurgia. Risultati di 4 anni su casistica personale.

A prospective study of surgical infections in our Department of Surgery in the years 1986-1989 is reported. 2719 patients entered the study: they represent the total number of patients operated in the above said period (628 urgent and 2091 elective operations). As suggested in 1964 by Altmeier, surgical procedures were classified in four groups according to the potential risk of intraoperative contamination. For every septic complication observed pertinent cultures were performed and responsible pathogens identified. We present our protocol of antibacterial prophylaxis which distinguishes antibiotics given as "ultra short term", "short term", "antibiotic prophylaxis". The results obtained and particularly the total number of infections (3.9%), and the percentage of infections in group 3 (4.6%) and in group 4 (23.1%) procedures validate the usefulness of antibiotic prophylaxis in these patients. In group 1 and group 2 patients the usefulness of antibiotic prophylaxis seems very doubtful.

[Cancer of the male breast with atypical ductal hyperplasia and contralateral gynecomastia]. / Cancro della mammella maschile con iperplasia duttale atipica e ginecomastia controlaterale.

A case of right mammary invasive ductal carcinoma in young man with atypical ductal hyperplasia and gynecomastia of the second breast is described. The review of the existing literature stress the rarity of this observation. Nevertheless, in all cases of male mammary carcinoma the accurate examination must always include the contralateral breast to search the possible precancerous lesions or etiologic factors.

[Primary melanoma of the esophagus]. / Il melanoma primitivo dell'esofago.

A case of primary malignant melanoma of the esophagus is described. Primary malignant melanoma of the esophagus is rare, reaching only 0.1% of all esophageal tumors. The differential diagnosis is often difficult: the clinical features are similar to those of squamous cell carcinoma and, because of its potentially widespread submucosal growth, the bioptic and/or cytologic examinations are frequently negative. The surgical approach with esophagectomy is actually the treatment of choice. However, the prognosis is poor with a 5-year survival of 4.2%.

[A case of recurrent, metastatic Merkel's tumor localized in the gluteal region]. / Su un caso di tumore a cellule di Merkel a localizzazione glutea, recidivo, metastatizzato.

A case of recurrent metastatic Merkel cell carcinoma of the buttock is described and after a short review of the literature some clinical and therapeutic features of this rare tumor are given.

[Primary lymphoma of the spermatic cord]. / Linfoma primitivo del funicolo spermatico.

A case of primary malignant lymphoma of the spermatic cord in an eighty-nine-year old man is described. The mass simulated a groin hernia and was misdiagnosed elsewhere. The biopsy stated the presence of high malignant centroblastic lymphoma of the spermatic cord. The patient died few weeks after the diagnosis. The review of the literature permits to stress the rarity of this observation: only seven cases of funicular lymphoma are reported up to now. In all cases there are problems in differential diagnosis with other more frequent inguinocrural pathologies. The tumour of the spermatic cord is therefore an intraoperatory diagnosis. The surgical approach is the treatment of choice but it must be supported by a proper chemotherapy. In any case the prognosis is poor and depends on tumour's staging, clinical behaviour and response to the therapy.

[Efferent loop obstruction by a phytobezoar after gastrectomy]. / Occlusione da fitobezoar dell'ansa efferente in gastroresecato.

The authors present one case of intestinal obstruction due to phytobezoar of the efferent loop, in a previously undergone gastric surgery patient. They report, in the light of bezoar's peculiar location, that is usually it found in the stomach or small bowel.

[Multiple primary malignant neoplasms]. / Le neoplasie multiple maligne primitive (NMMP).

Multiple primary malignant neoplasms (MPMN) are tumors that arise independently in the same organ or tissues and different organs; they may present simultaneously (synchronous) or at least 6 months later (metachronous). The authors report the case observed: 20 multiple neoplasms in 5 years (5.8% of the tumors). In the light of the international literature they analyze some aspects of the problem, particularly with regard to the classification, etiopathogenesis, neoplasm associations and possible genetic character for some of there.

[Contusion of the liver. Importance of associated lesions in polytraumatized patients]. / Les contusions du foie. Importance des lésions associées chez les polytraumatisés.

Between January 1984 and June 1993, we treated 120 contusions of the liver in a situation of polytrauma. There were 24 patients in Stage I, 47 in Stage II, 22 in Stage III, 13 in Stage IV and 14 in Stage V according to the Organ Injury Scaling Committee of the American Association for the Surgery of Trauma. A total of 107 patients were operated. Polytrauma related mortality was high. Besides the gravity of the liver lesion, prognosis was a function of other associated intra or extra abdominal lesions. In our series, other associated lesions were the cause fo death in 26 patients (64%) and 15 deaths (36%) were directly related to the hepatic lesion. The gravity of the Stage V lesions was related both to the state of shock of operation and the difficulties in reestablishing haemostasis. Packing decreased the effect of hypovolaemia and coagulopathy. The prognosis of supra hepatic venous lesions and hepatic resections remain disastrous. Our surgical schema has changed towards more conservative surgery and, when haemodynamic stability has been achieved, to abstention and careful monitoring. Different extra-hepatic trauma causing damage to other organs directly compromises simple hepatic lesions. The result of our series confirms the correlation between mortality and the gravity of the polytrauma as evaluated according to the Injury Severity Score proposed by Baker.

[Infantile seborrheic dermatitis and related syndromes. Diagnostic and pathogenetic problems]. / La dermatite seborroica infantile (D.S.I.) e sindromi affini. Problemi diagnostici e patogenetici.

Infantile seborrhoeic dermatitis is a dermatosis affecting infants characterized by little or no itching and by completely typical skin symptoms, distribution and evolution. Infantile seborrhoeic dermatitis should therefore be differentiated from atopic dermatitis and from diaper-rash although interferences be possible. In fact the high frequency of the atopic dermatitis and of diaper rash may cause these two manifestations to sometimes superimpose on infantile seborrhoeic dermatitis. This in turn causes rather complex clinical situations that may be very hard, but not impossible to diagnose if use is made of laboratory findings to support clinical features. The pathogenesis and etiology of infantile seborrhoeic dermatitis are still debated although recent results assign the dominant role to Candida albicans. This mycete also plays a relevant role in the genesis of diaper rash and particularly of Leiner's exfoliative erythrodermas, which can be interpreted as a generalized and erythrodermic expression of infantile seborrhoeic dermatitis. Systematic cultural investigations and appropriate antimycotic treatments, that would stimulate antibody response, will allow to clarify in the near future this interesting etiological problem, with important therapeutical consequences.

An immunohistochemical study of normal and neoplastic canine sertoli cells.

Immunohistochemical studies of human fetal Sertoli cells (SCs) have shown transient expression of cytokeratin (CK) and desmin (DES) that is replaced after birth by expression of vimentin (VIM) and inhibin-α (INH-α). Human Sertoli cell tumours (SCTs) are characterized by re-expression of CK and DES. The aim of the present study was to evaluate immunohistochemically the expression of VIM, INH-α, CK and DES in normal and neoplastic canine SCs. Normal testicular tissue from three adult dogs, one 6-month-old puppy and two neonatal pups was examined in addition to samples from 21 canine SCTs. VIM was not expressed by neonatal SCs, but was present in SCs from the puppy, the adult dogs and in all SCTs. Conversely, INH-α was expressed by neonatal SCs and most SCTs, but not by normal SCs of adult dogs and the puppy. DES and CK were expressed only by some SCTs. These results show that, contrary to findings in man, canine SCs do not express VIM at the time of birth. SCs from neonatal dogs do express INH-α, but such expression was lost in the puppy and the adult dogs. Canine SCs therefore differ from human SCs, as expression of INH-α characterizes immature SCs, whereas the expression of VIM characterizes mature SCs. Canine SCTs may express CK and DES, suggesting that the neoplastic cells undergo de-differentiation during transformation.

Neuropathies associated with monoclonal gammapathies.

There is increasing evidence that monoclonal proteins are implicated in the development of peripheral neuropathy. Approximately ten percent of patients with peripheral neuropathy of unknown cause have a monoclonal protein and this rate is significantly higher than prevalence rates of monoclonal protein in comparable segments of the general population. Extensive clinical, electrophysiological and immunopathological evidences indicate that peripheral neuropathy associated with monoclonal protein are heterogeneous, including: 1. the demyelinating, predominantly sensory neuropathies associated with anti-MAG antibodies; 2. the axonal, sensory neuropathies associated with anti-sulfatide and anti-chondroitin sulfate antibodies; 3. the motor neuropathies associated with anti-GM1 antibodies. Patients with chronic polyneuropathies should be evaluated for underlying plasma cell dyscrasia.

The prevalence of dementia and cognitive deficit in a rural population of 2442 residents in northern Italy. A door-to-door survey.

A population study to estimate the prevalence of dementia was carried out amongst the residents of four rural towns near Pavia in northern Italy. The population included a 40% sample of the residents between 65 and 69 years old and all residents of 70 years old or older. A door-to-door neuropsychological screening was followed by neurological examination on individuals scoring low at the screening. Of the 1670 subjects who completed the screening, 42.3% scored under the cut-off point. The prevalence of cognitive deficit was higher in women, even accounting for age. Of those who scored under the cut-off point, 336 underwent neurological examination, yielding a prevalence of dementia of 15% (from 1.6% between the ages of 65 and 69; 35% at the age of 85) in women and 5% (from 7% between the ages of 75 and 79; 27% at the age of 85 or more) in men.

Myoclonus epilepsy and ragged-red fibers: blood mitochondrial DNA heteroplasmy in affected and asymptomatic members of a family.

By a rapid PCR-based method to assess the 8344 mtDNA mutation associated with MERRF disease, we have studied DNA from blood samples of 10 individuals belonging to a family spanning four generations in which one patient showed the complete MERRF phenotype, three other members were less severely affected, while the remaining were unaffected. The percentage of mutant mtDNA was quantified by laser-densitometric scanning of the negative photographic sheets of the agarose gels. The results showed that the MERRF patient had 53% of mutated mtDNA while the two less affected patients had 62% and 14% of mutated mtDNA, respectively. However, a high percentage of mutated genomes (up to 64%) was also found in some unaffected relatives. These results show that although on one hand the mutation is probably the primary cause of the disease, on the other hand the relative amount of mutated mtDNA in blood samples is not indicative of its clinical expression.

An Italian-Australian comparison of quality of life among people with intellectual disability living in the community.

This paper reports the measurement of both objective and subjective quality of life in samples of people either with intellectual disability or from the general adult population, drawn from Australia and Italy. Measures were made using the Comprehensive Quality of Life Scale. Generally, it was found that the scores for all groups were comparable. This finding is consistent with the provision of generally decent objective living conditions in both countries and subjective well-being homeostasis, wherein subjective quality of life is held within a predictable range. These results are discussed in the context of measurement difficulties imposed by the need for simple Likert scales and subjective data which are strongly negatively skewed.

Middle latency auditory evoked potentials improve the detection of abnormalities along auditory pathways in multiple sclerosis patients.

Brain-stem and middle latency auditory evoked potentials (BAEPs and MLAEPs) have been studied in 34 multiple sclerosis (MS) patients. We were able to detect a central nervous system auditory pathway involvement in 17 (50%) of the patients: 38% by BAEPs alone (I-V inter-peak latency) and 47% by MLAEPs alone (Na and Pa peak latency). Five patients had abnormal MLAEPs with normal BAEPs whereas the opposite was detectable in only 1 patient. In addition, most MLAEP parameters in the MS group statistically differed from those obtained in the control group. Therefore, our results demonstrated that the auditory pathway impairment could frequently be located at a rostral level along the auditory radiation. In conclusion, even if only Na and Pa components were considered, MLAEPs succeeded in improving the sensitivity of the auditory evoked potential examination without increasing the false positive rate.

A community-based outbreak of Salmonella enterica serotype Typhimurium associated with salami consumption in Northern Italy.

An outbreak of Salmonella enterica serotype Typhimurium belonging to phage type (PT) 193 occurred in autumn 1995 and involved 83 individuals in a large area of Northern Italy (Lombardy Region). Epidemiological and microbiological investigations of strains isolated from clinical and food specimens revealed that the vehicle was a batch of salami, produced on 4 September 1995 by a local firm. The outbreak was contained when the batch was withdrawn from sale. Insufficient ripening of the salami had allowed the salmonella to survive, emphasizing the need to define criteria for the ripening process of foods such as salami.