Parkinson's disease in Ferrara, Italy, 1967 through 1987.

Epidemiological surveys on Parkinson's disease that have been carried out in different parts of the world have suggested that the disease is uniformly distributed in white populations. The position with regard to the Mediterranean peoples is still controversial, because of the large variation of the frequencies observed in the different areas that have been investigated. We therefore studied the frequency of Parkinson's disease in the Local Health Service of Ferrara, northeastern Italy (mean population, 187,000). Based on 394 patients, the mean incidence per year for the period from 1967 through 1987 was 10.01/100,000. The incidence rate of Parkinson's disease among cases with early onset was found to be statistically higher in rural areas as compared with urban ones (6.32/100,000 vs 3.11/100,000). Moreover, the study revealed a significantly higher incidence rate among agricultural workers (20.6/100,000). These results would seem to give further support to the hypothesis of a possible causal role of environmental factors that are mainly linked to agriculture, most likely due to the continual exposure to toxic agents in this area. However, further studies, which are not exclusively epidemiological, are necessary before any conclusions may be drawn, because many confounding variables may account for the results from surveys of this type.

The risk of Parkinson disease in Mediterranean people.

On the basis of previous epidemiologic studies, Parkinson disease was thought to be evenly distributed throughout the world. These studies, however, were conducted only on North European populations. The position with regard to the Mediterranean peoples was still unknown, and we therefore studied the frequency of Parkinson disease on the island of Sardinia, where some ethnic groups of the Mediterranean stock are represented. Based on 967 accepted cases, the prevalence 100,000 population on January 1, 1972, was 65.6; the average annual incidence for the period 1961 through 1971 was 4.9. These figures are one-half of the figures established for North Europeans. Our findings suggest racial differences in predisposition to Parkinson disease. Some Negroid features are present in Sardinians. If, as seems likely, Africans prove to be relatively unsusceptible to the disease, the risk for Sardinians and other Mediterranean ethnic groups might be intermediate between North Europeans and Africans.

Interference of concomitant tasks on simple reaction time: attentional and motor factors.

Simple reaction times to lateralized unstructured visual stimuli were measured in normal subjects while they were carrying out concomitant left hemisphere tasks. Three tasks were used. In the first task the subjects had to pay attention to strings of digits, acoustically presented, and detect letters randomly interspersed among the digits; in the second task the subjects had to memorize strings of acoustically presented digits; in the third task they had to repeat each digit of a string as soon as they heard it. In spite of the greater difficulty of the first two tasks with respect to the third one, only this last task, the only one requiring a verbo-motor response, produced a significant disadvantage for the left hemisphere responses to light. It is argued that in RT experiments a specific left hemisphere interference takes place when the secondary task requires the organization of a motor response

Alexia without agraphia: a study of a case of verbal alexia without accompanying colour-naming defect.

A 47-year-old man developed a persistent form of alexia without agraphia as the result of a haemorrhagic intracerebral lesion in the left inferior temporo-occipital region, due to the rupture of an arteriovenous malformation. Surgical evacuation of the haematoma and excision of the malformation did not produce any modification of the reading deficit, which remained unchanged during a 4-year follow-up. The reading deficit was restricted to words and the patient was able to read only letter-by-letter, so that the whole words were reconstructed from the auditory names of the letters. So far, the disconnection explanation is the standard explanation of alexia without agraphia and the present case of verbal alexia may be regarded as being within this overall category. However, this explanation meets with unanswered questions that suggest more flexible interpretations. Neurolinguistic studies have questioned the unique character of alexia without agraphia as a clinical entity and, in contrast to the disconnection hypothesis, support the notion that the different varieties of alexia that are traditionally described represent distinctive syndromes, each with its own clinical features and pathophysiological basis. In this context, the reading properties in this case seem fully compatible with a deficit of the visual word-form system postulated by Warrington and Shallice, that is, the relatively early stage of the reading process through which a word-form or equivalent unit is attained. This system might be lateralized to the left hemisphere, as suggested by the fact that this case, like other cases of verbal alexia, had sustained damage to the left hemisphere and did not show any differential preservation of the reading of concrete words.

Oral aluminum and neuropsychological functioning. A study of dialysis patients receiving aluminum hydroxide gels.

Nine dialysis patients with significantly increased serum-aluminum levels due to chronic ingestion of aluminum hydroxide gels and eleven dialysis patients with normal serum-aluminum levels were tested neuropsychologically for generalized functions (intelligence, reasoning, memory) and for more specific abilities (visual memory, verbal and reading fluency, manual dexterity). All tests did not reveal any significant difference in neurophyscholigical functioning between the two groups. This finding seems to indicate that oral aluminum is not neurotoxic for man, even under circumstances of renal failure. This contradicts the idea that oral aluminum plays a role in etiology of dialysis dementia. However, the possibility cannot be excluded that aluminum overload in the present sample was not sufficient to induce changes in CNS functioning. Thus, until the importance of oral aluminum has been decided, it seems wise to keep all sorces of aluminum overload as low as possible.

The geographical distribution of multiple sclerosis, rheumatoid arthritis, rheumatic heart disease and poststreptococcal nephritis in Sardinia: climatic and socioeconomic factors.

The comparative geography of multiple sclerosis (MS) and nonneurological diseases considered to be autoimmune is of great interest. But there are few appropriate investigations. Some have found an increase in the frequency of MS, rheumatoid arthritis and rheumatic heart disease depending on the geographic latitude. Nevertheless, the significance of the latitude effect as an indicator of a possible etiological relationship between MS and these conditions has been questioned. In this paper, the frequency of MS, rheumatoid arthritis, rheumatic heart disease and poststreptococcal nephritis is reported from Sardinia where appreciable differences in climatic and socioeconomic conditions exist. There was a positive correlation of the distribution of MS with the distribution of rheumatic heart disease and poststreptococcal nephritis. On the other hand, no correlation was found with the distribution of rheumatoid arthritis.

Clinical and audiological findings in a case of auditory agnosia.

A case is reported of severe agnosia for verbal and non-verbal sounds without associated aphasic disorder. A CT scan revealed bilateral, temporal lobe lesions from two ischaemic accidents that had occurred 9 months apart. The search for subtle deficits in the patient showed normal sensitivity to changes in the intensity and frequency of simple sounds; in contrast, his ability to discriminate sound duration and musical note sequences was severely impaired. The simultaneous recording of the whole auditory-evoked response pattern revealed no abnormality in the early components, which reflect the activation of the auditory nuclei and pathways of the brain stem. However, the middle and late components were delayed and slowed. These results and others in the literature suggest that the neocortex in man, as in other mammals, plays an essential role in the temporal aspects of hearing. Also, the two main ingredients commonly recognized in auditory agnosia, i.e. word deafness and the inability to interpret non-verbal sounds, are caused by the disruption of elementary, bilaterally represented cortical functions which start the processing of every kind of auditory information.

Systemic lupus erythematosus presenting with neurological disorders.

Six patients are described who developed a wide variety of neurological manifestations heralding systemic lupus erythematosus (SLE), which included epileptic seizures, stroke, peripheral polyradiculoneuropathy similar to Guillain-Barré syndrome, transverse myelopathy and multifocal disorders with remitting course mimicking multiple sclerosis. The peculiarity of these cases was that the neurological disorders remained the only manifestations of SLE for many years and the nervous system appeared to be the main target even after the development of systemic SLE. In five patients the prognosis was favourable and corticosteroid treatment led to prolonged remission.

Cognitive function and regular dialysis treatment.

The purpose of this investigation was to assess the intellectual function of patients on regular dialysis treatment by using neuropsychological tests such as the Wechsler Adult Intelligence Scale (WAIS) and the Wechsler Memory Scale (WMS). Studies were performed on 54 patients; 21 patients were retested after at least 12 months. We found no relationship between the neuropsychological scores and the blood levels of small molecules, the hemoglobin concentrations, the serum aluminium levels, and the levels of formal education. We found, however, an impairment of neuropsychological performance, mainly for memory function, related to the duration of dialysis and, possibly, to parathormone levels. We conclude that standard hemodialysis strategies are not able to prevent the development of intellectual impairment in uremic patients and that the WMS could be used as a sensitive objective measure of the comparative adequacy of various long-term dialysis treatments.

Phonological dyslexia, lexical analogy, and functional models of oral reading: a critique of Bradley and Thomson.

We discuss some of the interesting features of the investigation of a phonological dyslexic patient's ability to read aloud nonwords embedded in words reported by Bradley and Thomson (1984, Brain and Language, 22, 292-302). We highlight some problems of their interpretation, tentatively suggest an alternative explanation in terms of lexical analogy, and conclude by suggesting some prescriptive conditions for the legitimacy of extending models of normal functioning to account for cognitive neuropsychological data.

Unitary attention in callosal agenesis.

The interhemispheric organisation of two specific components of attention was investigated in three patients affected by partial or complete agenesis of the corpus callosum. A visuospatial component of attention was explored using a visual search paradigm in which target and distractors were displayed either unilaterally within a single visual hemifield, or bilaterally across both visual hemifields in light of prior work indicating that split-brain patients were twice as fast to scan bilateral displays compared to unilateral displays. A central component of attention was explored using a psychological refractory period (PRP) paradigm in which two visual stimuli were presented laterally at various stimulus onset asynchronies (SOAs), with each stimulus associated with a different speeded two-alternative choice task. The stimulus-response compatibility in the second task was systematically manipulated in this paradigm, in light of prior work indicating that split-brain patients exhibited a close-to-normal PRP effect (i.e., slowing of the second response as SOA is decreased), with, however, abnormally decreasing effects of the manipulation of the response mapping on the second task speed as SOA was decreased. The present results showed that, although generally slower than normals in carrying out the two tasks, the performance of each of the three acallosal patients was formally equivalent to the performance of a matched control group of normal individuals. In the visual search task, the search rate of the acallosal patients was the same for unilateral and bilateral displays. Furthermore, in the PRP task, there was more mutual interference between the lateralised tasks for the acallosal patients than that evidenced in the performance of the matched control group. It is concluded that the visuospatial component and the central component of attention in agenesis of the corpus callosum are interhemispherically integrated systems.

Pure agraphia: a discrete form of aphasia.

A 62 year old, right handed man developed a pure agraphia as the result of a left temporal lobe stroke. Isolated writing disturbances persisted for seven months until he had a second cerebrovascular accident resulting in total aphasia and right hemiplegia. A CAT scan obtained four months after the first episode showed a localised dilatation of the posterior portion of the left Sylvian cistern and patchy areas of low absorption in the left temporal lobe. The report supports suggestions that localised damage to the language area can produce a pure agraphia as the sole detectable disorder of language organisation.

Voluntary lid closing inability. Release of a compulsive reaction to the exploration of the environment.

Voluntary lid closing inability has been observed in two patients with right-sided frontal ischemic damage. The patients developed a transient inability to close their eyelids voluntarily at the same time as a transient forced grasping in the left hand and left hemiparesis not affecting the face. Automatic and reflex lid closures were retained as well as the ability to keep the eyes closed and to reopen them readily on command. In previous reports, inability to close eyelids voluntarily has been attributed to apraxia, paralysis, or motor impersistence. The localization of the lesion observed in our patients suggests other pathogenetic hypotheses similar to compulsive gaze [19]. It is conceivable that the voluntary lid closing inability produced by frontal lobe lesions is due to the release of a compulsion to maintain the lids elevated in the waking state, because of the overactive effect of the visual stimuli.

[Cognitive tasks and simple reaction times: new observations]. / Compiti cognitivi e tempi di reazione semplici: nuovi dati.

Simple reaction times to lateralized unstructured visual stimuli were studied in normal subjects while they were carrying out a concomitant task. In one task strings of four digits, randomly chosen from 1 to 9, were acoustically presented to the subjects who had to repeat them at the end of each presentation, in a second task again strings of four digits were presented and the subjects were asked to pay attention to the stimuli without memorizing them. In order to compel the subjects to listen carefully to the stimuli, in the second experiment, intermixed with the digits, a letter was occasionally presented and the subjects had to repeat it. It was found that the memory task facilitates reaction times mediated by the left hemisphere, whereas no hemispheric differences were found in the purely attentional task.

[Gerstmann's syndrome: report of a case]. / La sindrome di Gerstmann: considerazioni su un caso.

In recent years, several authors have questioned the reliaty of Gerstmann syndrome and have claimed that "aphasia might be regarded as the true Grundstörung of the Gerstmann symptoms". This paper describes a case of Gerstmann syndrome ictally risen with aphasia in a patient suffering from a tumour of the left PTO region. After the remission of aphasia, the syndromic association typical of the Gerstmann syndrome lasted up the exitus.

Pure "aphasic" agraphia due to damage of the left superior parietal lobule.

A case of pure agraphia, due to an ischaemic lesion of the left superior parietal lobule, is reported. The neuropsychological analysis of writing performances suggests an aphasic nature of this patient's pure agraphia. The authors discuss the role played by diffuse and/or localized brain lesions in writing function, emphasizing that the left superior parietal lobule in man may be crucial for the sensorimotor linguistic integration needed for writing.

[Tactile recognition and cognitive tasks. Preliminary results]. / Riconoscimento tattile e compiti cognitivi. Datti preliminari.

In a basic experiment, a tactual recognition task was given to 28 normal blindfolded subjects. The subject were asked to explore the stimuli with the left or the right hand, and to choose out of 4 cavities the target form. Two cognitive concurrent tasks varied in terms of their verbal (left hemisphere) and non-verbal (right hemisphere) attributes were then given to other two groups of subjects (both of 24 Ss) performing the basic task. A consistent right hemisphere advantage was obtained in experiment I. For the non-verbal condition left hand performance was disrupted as well as for the concurrent verbal task condition. These findings were interpreted in terms of brain lateralisation of cognitive functions. For the "verbal" condition the results suggests that in case of a different task difficulties the normal mode of operation of the hemispheres is one of cooperative interaction, affecting, in this case, the hemisphere doing the processing.

The distribution of multiple sclerosis in Sardinia.

The incidence of M.S. in Sardinia has been found to be the highest in Southern Italy. On "prevalence day", October 21st, 1971, the incidence was 12.48/100,000 inhabitants, whereas the rates in Campania and in Puglia, (regions of Southern Italy at the same latitude as Sardinia) the rates were 3.3 and 4.0 respectively. In the present study, a significant female predominance was found, the rates for women and men being 15.88 and 9.03 respectively. The incidence of MS in the study area was found to be significantly higher both in areas with a cool humid climate and in communities with the lowest socioeconomic conditions. A significantly higher incidence was computed for communities with less than 5,000 inhabitants. MS incidence had no correlation with the occupational status and dietary habits, which are uniformly distributed throughout the island. The disease started at au average of 27.3 years and its duration on "prevalence day" was 13.4 years. The age of onset was found to be low in comparison with other population studies in Europe and in North-Central Italy. The age-specific curves of onset and on "prevalence day" showed two peaks, one in the second-third decade and one in the forth-fifth decade. Most of the patients with an earlier onset showed a complete disappearance of the symptoms after the first bout.

Studies on epidemiological, clinical, and etiological aspects of ALS disease in Sardinia, Southern Italy.

This investigation was conducted to clarify the epidemiology of ALS disease in Sardinia. During the years 1965-1974, the average annual incidence was found to be 0.64/100,000 inhabitants. On prevalence day, October 24th, 1974, the prevalence rate was 1.56/100,000 inhabitants. A significant male predominance was found, the average annual incidence rates for men and women being 0.88 and 0.40, respectively. The peak in both sexes was reached between 60 and 69 years. ALS distribution in the study area was uniform but its occurrence was significantly higher among agricultural workers (5.28/100,000). ALS started on average at 56.58 years and its duration was 2.5 years, being significantly longer in patients under 40-years-old. The distribution of the various clinical forms was: 66 per cent conventional forms, 20 percent bulbar and 14 per cent pseudo-polyneuritic. In the bulbar type, a female predominance was found. About 96 percent of cases were sporadic and 4 per cent familial. Familial cases presented no difference from sporadic cases. Trauma was present in 10.5 percent of the cases and gastrointestinal disfunction in 13 per cent. This probably reflects some relationship between trauma and ALS, and between malnutrition and ALS. No combination of ALS, dementia and parkinsonism was observed. Dementia was associated with ALS in four cases and Parkinson's disease in one case, separately. The combination of other disease states with ALS in the present study may be simple coincidence.

Uremic neuropathy: correlations between electroneurographic parameters and serum levels of parathyroid hormone and aluminum.

An electrophysiological investigation on 29 dialysis patients was performed with the aim of verifying whether serum parathyroid hormone (PTH) and aluminum (Al) levels may play a role in the pathogenesis of uremic neuropathy. All patients except 1 showed electrophysiological abnormalities. Both PTH and Al levels were found to be significantly correlated with the time on dialysis. On the contrary, the values of each single electroneurographic parameter did not show any correlation with the duration of dialysis, PTH levels and Al levels. The authors conclude that PTH and Al does not seem to play a role in the etiology of uremic neuropathy.