Maria Immacolata Pirastru

Multiple sclerosis epidemiology in Sardinia : evidence for a true increasing risk

Objectives– To update prevalence and incidence rates of MS among Sardinians. Materials and methods– The present work is a “spider” kind of population based survey, conducted over the interval 1968–97, on patients with MS (Poser criteria) living in the province of Sassari, Northern Sardinia (454,904 population). Results– A crude total prevalence rate of 144.4 per 100,000, an onset‐adjusted prevalence rate of 149.7 per 100,000 and an average annual incidence rate of 8.2 for the period 1993–7 were found. Conclusion– Repeated epidemiological assessments of MS in Sardinia over decades have shown that the island is at high risk for MS. The present work highlights that MS incidence in Sardinia has been increasing over time. Although a substantial and widely spread improvement in MS case ascertainment can be postulated as the reason for such observations, a comparison between our data and those recently reported from a more industrialized province in Northern Italy seems to prove an at least partially real increase in MS risk among Sardinians and favours the hypothesis of a MS “Sardinian focus” as related to its latitude.

Incidence of multiple sclerosis in the town of Sassari, Sardinia, 1965 to 1985: Evidence for increasing occurrence of the disease

Prevalence studies carried out in Sardinia from 1975 suggest an increasing occurrence of MS. However, it is questionable whether this increase represents a real change in MS incidence or simply reflects longer survival. Data from 79 patients indicated the average annual incidence for the period 1965 through 1985 was 3. 4 per 100, 000. On December 31, 1985, the prevalence rate was 69 per 100, 000. Evaluation of MS temporal trends showed a significant increase in MS incidence during the study period: values ranged around two per 100, 000 in the triennial periods 1965–1967, 1968–1970, and 1971–1973, and around five in each triennium from 197-7 onward.

Incidence of multiple sclerosis in Macomer, Sardinia, 1912–1981: Onset of the disease after 1950

Intensive search of all cases of MS occurring in the Sardinian commune of Macomer since 1912 indicated that MS was absent up to the early 1950s. All 13 ascertained cases had clinical onset in the years 1952–1981. During this period, the average annual incidence was 4.8 per 100,000; the highest incidence was found in the period 1957–1961, slowly decreasing up to 1981. MS was probably introduced after 1945, when the centuries-old isolation of Macomer ended and the native population came into contact with individuals from high-and medium-risk areas.

Amyotrophic lateral sclerosis in Sardinia, Italy: an epidemiologic study

The authors carried out an epidemiologic study on amyotrophic lateral sclerosis in Sardinia for the years 1957 through 1990. The duration of the disease and survival were significantly shorter in bulbar form. The distribution of ALS in various areas of the island was found to be not at all homogeneous. Mean yearly incidence showed no significant variations in the decades 1971–80 and 1981–90. In the last decade, an increase of bulbar forms was observed.

Wernicke’s encephalopathy in a woman on slimming diet

Wernicke’s encephalopathy (WE) is characterized in its classic form by ophthalmoplegia, ataxia, and mental changes, a triad not frequently encountered in clinical practice.1 It results from a deficiency in vitamin B1, or thiamine, an essential coenzyme in intermediate carbohydrate metabolism.1 WE may occur in chronic alcoholism, when the dietary intake of thiamine is inadequate, and may complicate any condition in which there is poor nutrition.1 We report a case of WE in a woman on an apparently balanced slimming diet for about 1 year, with no other definite risk factors. A 30-year-old right-handed woman presented with the acute onset of ataxia, vertigo, nausea, double vision in any gaze direction, and oscillopsia in January 1999. At admission she was alert and oriented. Her weight was 73.0 kg. She reported a loss in weight of 25 kg in the past 11 months, following a slimming diet by “Herbalife International of Europe” (Los Angeles, CA, 1998). This diet consisted of a dietetic meal (200 kcal) plus a normal …

Amyotrophic lateral sclerosis in Sardinia (Italy): Epidemiologic features from 1957 to 2000

To evaluate epidemiological variables of amyotrophic lateral sclerosis (ALS) in Sardinia (Italy) in the 1991–2000 periods and compare them with the preceding decades.

Upbeat nystagmus as an early sign of cerebellar astrocytoma

SummaryA boy with a left-hemispheric cerebellar astrocytoma had upbeat nystagmus exhibiting increasing-velocity slow phases. The nystagmus improved after excision of the tumour.

Familial autoimmune MuSK positive myasthenia gravis

The familial occurrence of autoimmune myasthenia gravis (MG) with anti-acetylcholine receptor antibodies (AChR Ab) has been rarely reported. Different antibody specificity in family members has also been described. To our knowledge, a familial form of MuSK Ab positive MG has never been reported. We studied the HLA allele profile in a family with two sisters affected by MuSK positive MG.

MACRO SQUARE WAVE JERKS INDUCED BY BINOCULAR FIXATION ATTEMPTS

Quantitative eye movement recordings in a patient with suspected multiple sclerosis who had alternating esotropia, horizontal nystagmus, downbeat nystagmus, and saccadic dysmetria, showed occasional macro square wave jerk intrusions and oscillations in the fixating eye during spontaneous monocular fixation, and binocular macro square wave jerk oscillations during the effort to fuse.