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Biomedical subjects

L Bettoni

Publications and source records attributed to L Bettoni.

At least 19 recordsLinked to original sources
PubMed1994

Reliability of EEG in the diagnosis of Creutzfeldt-Jakob disease.

Although EEG is generally considered a useful tool for the diagnosis of Creutzfeldt-Jakob disease (CJD), some cases have been reported where the EEG was non-specific. We reviewed a series of 15 CJD patients, observed in our institute in the period 1975-91. In 12 cases the diagnosis was confirmed on post-mortem examination. The prominent aspect of the present series was the homogeneity of clinical, neurophysiological and neuropathological data. All patients showed the presence of periodic sharp wave complexes (PSWC) and EEG reactivity to external stimuli or drugs was uniform. The EEG can give essential information for the diagnosis of CJD if 2 basic conditions are satisfied: (1) serial recordings are performed in relation to the different stages of the disease, and (2) not only the presence of PSWC is considered, but also the reactivity of EEG to dynamic events such as the response to external stimuli and drugs, and the level of consciousness.

Adult↗
PubMed1994

Steadiness of amyotrophic lateral sclerosis in the province of Parma, Italy, 1960-1990.

A clinical and epidemiological study on amyotrophic lateral sclerosis (ALS) was conducted in the province of Parma, Italy, from 1960-1990. A total of 121 cases were collected from hospital records. The average annual incidence was 0.98 per 100,000 inhabitants, with a male/female ratio of 1.1. Age-specific incidence was maximal in the age group 60-69 years. No difference between rural and urban areas was found. Prevalence on October 26th, 1981 was 2.5 per 100,000. Mean age at onset was 60 years, with no significant sex difference. Mean duration of the disease was 30 (sd 21.4) months. Bulbar forms were significantly (p < 0.05) shorter than conventional forms, with a mean duration of 23.4 (sd 21.4) months. Age at onset did not influence prognosis. A comparison of three decades was made, to verify whether possible variations of the disease had occurred with time. From our data a definite stability was found in such epidemiological parameters as incidence, prevalence, mean duration and mortality of ALS in the period.

Adult↗
PubMed1994

Post-anoxic theta and alpha pattern coma.

Theta pattern coma is a rare EEG finding, described in the course of post-anoxic or post-traumatic coma and interpreted as a "malignant" variant of alpha pattern coma. A case of isolated theta pattern coma, followed by alpha pattern coma is reported. Short temporal succession of such patterns would seem to confirm the hypothesis of a close relationship between them. Possible physiological mechanisms involved in the generation of the patterns are discussed.

Alpha Rhythm↗
PubMed1993

Peroneal muscular atrophy with parkinsonism, ptosis, and congenital strabismus.

Peroneal muscular atrophy (PMA) may be occasionally associated with other neurodegenerative features including parkinsonism. We report the association of PMA of neuronal type with parkinsonism, ptosis and congenital strabismus in a 62-year-old Sicilian woman. The complete syndrome was present only in the proband, but variously combined features were present in ten other family members over four generations, with likely autosomal dominant inheritance. Although a similar syndrome of PMA, ptosis, parkinsonism and dementia was already reported, this family showed a previously undescribed combination of features in view of the presence of congenital strabismus.

Blepharoptosis↗
PubMed1993

Unilateral trismus caused by vertebrobasilar dolichoectasia.

Vertebrobasilar dolichoectasia is a relatively uncommon pathological entity and generally asymptomatic. We report a quite unusual case of unilateral motor trigeminal involvement with trismus, due to VD. Apart from tetanus or local morbid conditions of the mouth, trismus is often attributed to disturbed programming and co-ordination of the masticatory muscles within the mesencephalic nucleus. The possibility of truncal compression of the trigeminal motor root by vertebrobasilar dolichoectasia being responsible for masseter muscle spasm, in analogy with the pathogenetic mechanisms proposed in hemifacial spasm, is proposed as an alternative explanation in the present case.

Aged↗
PubMed1993

Acute esotropia from small thalamic hemorrhage.

A 74 year-old patient developed transient acute esotropia, upward gaze palsy and imbalance of standing and gait. Computed Tomography (CT) demonstrated a small left thalamic hemorrhage in the absence of midbrain lesions. The role of thalamic lesion in the genesis of monocular supranuclear palsy is discussed.

Acute Disease↗
PubMed1990

Considerations on a group of 13 patients with Creutzfeldt-Jakob disease in the region of Parma (Italy)

We report a group of 13 cases of Creutzfeldt-Jakob disease treated in the region of Parma (Italy) between 1975 and 1984. An extensive study did not point to any common source of infection. The clinical stereotypy and distinctive neuropathology in this temporo-spatially confined group of patients might be stressed, but the possibility of infection by a single strain of the CJD agent remains speculative.

Aged↗
PubMed1988

Myokymia in the course of Bell's palsy. An electromyographic study.

Facial myokymia has been described in association with many morbid conditions, most frequently multiple sclerosis (MS) and brainstem tumors (BST). Very few reports deal with myokymia in the course of Bell's palsy, despite high frequency of the disease. A series of 88 consecutive patients with Bell's palsy is presented, in which serial EMG controls were performed in the initial phases of facial palsy. Twenty three showed myokymic activity detected by EMG, while clinically evident myokymia could be observed only in 8. EMG features did not reveal any peculiarity as compared with myokymic discharges reported in other pathological situations. Considering the high incidence of myokymic activity reported in the course of Bell's palsy, it is the authors' opinion that an occasional finding of myokymia, both clinically evident or detected by EMG, should not necessarily lead one to suspect serious aetiologies.

Electromyography↗
PubMed1987

[Prevalence and incidence of multiple sclerosis in the province of Parma, 1965-1980. Preliminary data].

Recent epidemiological studies in small communities report very high prevalence rates of multiple sclerosis (MS), suggesting that Italy should be included among high risk areas for MS. Such data are not confirmed when populations exceeding 300.000 are considered. The total prevalence rate of probable MS in the province of Parma was 23.07 per 100.000 on the prevalence day Dec. 31, 1980. Up to date, very few studies deal with incidence of MS, due to the fact that in many cases it is difficult to establish the exact outburst of the disease. Yet, incidence will indicate the real risk for the disease for a given period in a more accurate manner. Mean annual incidence of MS in the province of Parma in the period 1965-80 was 1.14 per 100.000. A previous survey in the period 1950-59 reported, for the same area, an incidence rate of 1.01 per 100.000, versus a prevalence rate of 12.33 per 100.000. In our opinion, when comparison of data between two different areas, in different periods, is to be made, incidence rates for MS should be preferred to prevalence studies.

Adolescent↗
PubMed1986

Progressive multifocal leukoencephalopathy (PML): clinical and pathological findings in two short-duration patients.

The clinical features and neuropathological findings of two patients suffering from progressive multifocal leukoencephalopathy (PML) are reported. These patients had cancer and died two months and one month after onset of their neurological symptoms, respectively. In both demyelination was discovered only as a result of autopsy due to different reasons: the CAT-scan findings were misinterpreted in the first patient, while in the second diagnosis was overshadowed by the sudden progress towards a fatal outcome preceded by myoclonus and other encephalitis-like manifestations. The major findings were the extreme paucity of the mesodermal elements on the one hand, and the modest spread of the affected areas on the other. It is pointed out that, whatever the size of the lesions, characters were the same and that their formation could hardly be traced in time. The intriguing similarities between PML and several types of demyelination obtained experimentally using certain virus strains are remarked.

Aged↗
PubMed1985

Hypercalcemic encephalopathy in the course of hyperthyroidism.

A hypercalcemic condition can be observed in association with hyperthyroidism. The case of a patient suffering from hypercalcemia and hyperthyroidism is reported. A confusional state and EEG alterations, among which diffuse monomorphic delta rhythms were remarkable, are shown. As soon as normalization of calcemia was achieved, a rapid clinical and EEG improvement took place. A hypothetical interpretation is proposed, according to which a prolonged, though inconstant, and mild hypercalcemia in the course of hyperthyroidism could determine an encephalopathy, concealing in some way thyrotoxic symptoms.

Brain Diseases↗
PubMed1984

Cerebral mycosis: clinico-pathological report of four cases observed in fifteen months.

Cerebral mycosis is a rare condition, difficult to diagnose in life, but is not easily missed at post-mortem examination. We report clinical and pathological findings in four patients with long duration prodromes, seizures and psychiatric symptoms. All of them had been misdiagnosed. Actinomycotic granules in two and Aspergillus filaments in the other patients were ascertained at histological examination. Two of the patients were in their twenties and apparently healthy. We suggest that the possibility of mycotic infection should be considered even in cases where no lowering of the body's defences can be postulated. Cell mediated immunity defects may be assumed as underlying these infections. Therefore we would suggest the importance of immunological tests for type IV immune responsiveness in all intracranial infective processes of undefined etiology.

Actinomycosis↗
PubMed1983

Amyotrophic lateral sclerosis in the province of Parma, Italy: a clinical and epidemiological study in the period 1960-1980.

A clinical and epidemiological study on ALS was conducted in the province of Parma, Italy, in the period 1960-1980. A total of 78 cases was collected from hospital records. The average annual incidence was 0.93 per 100,000 with no difference between rural and urban areas. On prevalence day (October 24, 1971) a prevalence rate of 2.27 was found. Mean age at onset was 59 years, with no sex difference. In 70% of cases the onset was between 50 and 70 years, this datum being statistically significant (p less than 0.001). Mean duration of the disease was 30.4 +/- 20 months, with no difference for sex and age at onset. A comparison was then made between two decades (1961-70 vs 1971-80), in order to verify the clinical impression of a worsening of some ALS parameters in recent years. Although statistical evaluation did not show any significant data, a tendency to a worsening of some parameters of the disease may be postulated.

Age Factors↗
PubMed1983

Pseudomyotonia of the striated urethral sphincter.

Pseudomyotonic discharges of the striated urethral sphincter are infrequent findings. We herein report on 9 patients with dissimilar anatomical-clinical conditions. In some patients a definite neurological lesion was ascertained, whereas in the remaining patients no neurological impairment could be determined clinically and instrumentally. The possibility of spontaneous resolution of the phenomenon is an interesting clinical feature.

Adolescent↗
PubMed1981

Meige syndrome: a clinical and EMG study.

2 patients showing oral-facial asymptomatic dyskinesia are reported. Clinically observed involuntary movements are the same as those described by Henry Meige in 1910 for the first time. An outstanding EMG feature is the recording of spontaneous potential bursts, both short and long in duration, that show synchronism when activity is registered from different facial muscles at the same time. Clinical and EMG data allow close comparison between involuntary movements in the course of Meige syndrome and idiopathic hemifacial spasm.

Blepharospasm↗