Legal and assistance aspects of Alzheimer's disease: analysis of 100 cases.

Caring for patients with disabling cognitive diseases, such as Alzheimer's disease (AD) and other progressive dementias, has a number of legal and social welfare implications. The two main problems to be discussed with patients and caregivers are the need for a legal guardian and requests for government financial support, both of which depend on the patient's progressive loss of autonomy and increasing need for assistance. In order to study the presence of these two support measures, we considered 100 AD patients (56 women and 44 men) divided in four groups on the basis of the stage of the disease: mild (25), moderate (34) and severe (32), or death (9). We investigated the number of caregivers for each patient (and their relationship with the patient), the presence of a legal guardian, and whether government financial support had been obtained. The number of caregivers increased for each patient as the disease advanced (54% with moderate and 67% with severe disease had > or =1 caregiver), but only 11% of the patients had a guardian. The caregivers were most likely to be family members (70% were spouses, 45% offspring). Only 23% of the patients with moderate and 62% with severe disease received government financial support. Our data concerning the care of incompetent people (as AD patients progressively become) in juridical (guardianship/trusteeship/proxy/power of attorney) and social terms (government financial support) show that such aspects are not sufficiently taken into account until the patients reach a severe disease or have died.

Cognitive impairment in patients suffering from relapsing-remitting multiple sclerosis with EDSS < or = 3.5.

OBJECTIVES: Previous papers have mainly demonstrated the presence and the frequency of cognitive impairment in patients suffering from relapsing-remitting multiple sclerosis. The purpose of this study was to investigate subjects with the relapsing-remitting form of the disease and mild clinical disability (EDSS < or = 3.5), so as to quantify this deficit when the illness does not yet interfere with daily living and the ability to work. METHODS: Fifty patients and 50 healthy controls were submitted to a wide neuropsychological battery, including Wechsler Memory Scale I- (WMS), Benton Visual Retention Test D- (BVRT), Raven Coloured Progressive Matrices (RCPM), Kohs' test (KT), Judgement of Lines Orientation H- (JLO), Facial Recognition (FR) and Aachner Aphasie Test (AAT). They also underwent Clinical Depression Scale (CDQ) and State-Trait Anxiety Inventory (STAI). RESULTS: The results show the presence of significant memory impairment on both WMS (P = 0.000) and BVRT (P = 0.000) in patients compared with controls. Patients were also impaired in abstract reasoning and problem-solving deficit (KT P = 0.003; RCPM P = 0.000) and in FR (P = 0.019). Cognitive decline correlated with illness duration (r = 0.761), but was independent of EDSS (r = 0.085). CONCLUSION: Cognitive decline was present even when physical disability was not yet severe, but it was mild and did not limit patients' ability to work. The cognitive impairment outlined was of the subcortical type and correlated with illness duration. This study emphasizes the importance of cognitive examination in clinical practice. It is suggested that a complete neurological examination include tests on memory and abstract reasoning.

Long-term interferon-beta treatment for multiple sclerosis.

The aim of our study was to analyze the dropout rate in patients with relapsing-remitting multiple sclerosis (RRMS) under long-term treatment with the three commercially available interferon beta (IFNbeta) preparations. According to the drug taken, we divided 122 RRMS patients into 4 groups: Betaferon group, 56 patients taking INFbeta-1b (24 MIU weekly, subcutaneous injections); Avonex group, 38 patients taking IFNbeta-1a (6 MIU weekly, intramuscularly); Rebif group, 18 patients taking INFbeta-1b (18 MIU subcutaneously). Ten patients who shifted from Betaferon to Avonex were included in a fourth group. Dropouts were registered every trimester with the related cause. Data were evaluated using Kaplan-Meier survival analysis and log-rank test. During the observation period of five years, 48 patients (39.9%) dropped out: 48% of the patients in Betaferon group withdrew at a median of 758 days, 26% of the Avonex group at 356 days; 38% of the Rebif group at 421 days, and 40% of those who shifted from Betaferon to Avonex at 259 days. The differences between groups were not significant on survival analysis. Patients receiving higher dose treatment (Betaferon and Rebif groups) dropped out mainly for clinical adverse events; conversely, patients receiving lower dose therapy (Avonex group) dropped out most often for inefficacy. Patients who shifted to a lower dose treatment (fourth group) had a dropout rate similar to that of the initial treatment. Our data showed that one-third of the patients stopped the therapy, mostly for adverse events and then for inefficacy, while the remaining two-thirds were still on treatment without problems up to 5 years of follow-up. Compliance seems related to the dose of the drug, but further analysis is needed to confirm our data.

Pancreatic encephalopathy: a 7-year follow-up case report and review of the literature.

Pancreatic encephalopathy is a rare complication of acute pancreatitis. Clinical features include focal neurological signs and acute onset of dementia. This picture can fluctuate over time: cyclic progression with remission and relapses has been described. We present the case of a 43-year-old man who, after an acute episode of pancreatitis, experienced five relapses, with alternating focal signs. The patient has improved, but cognitive impairment persists after a 7-year follow-up.

Apolipoprotein E and intronic polymorphism of presenilin 1 and alpha-1-antichymotrypsin in Alzheimer's disease and vascular dementia.

Apolipoprotein E (ApoE) genotypes, presenilin 1 (PS-1) and alpha(1)-antichymotrypsin (ACT) polymorphism and the association of the genotypes were examined in patients with Alzheimer's disease (AD, n = 121) or vascular dementia (VD, n = 68) in comparison with elderly controls (n = 125). The frequency of the ApoE epsilon 4 allele was significantly increased both in late-onset AD (0.35) and in VD (0.17); the frequency of ApoE epsilon 2 was significantly reduced in AD, but it was similar in VD and controls. The presence of the allele 1 of PS-1 intronic polymorphism was not associated with AD or VD and was not influenced by the ApoE genotypes. Also, the frequency of allele A of the intronic polymorphism of ACT was similar in AD, VD and controls and it was not altered by ApoE or PS-1 genotypes. The results confirm the association between ApoE epsilon 4 and AD and indicate an increase in ApoE epsilon 4 in Vd, too. A potential protective role of ApoE epsilon 2 is also suggested for late-onset AD but not for VD. No association was shown between ACT allele A and PS-1 allele 1 in AD or VD.

Therapeutic considerations in cerebellopontine angle lipomas inducing hemifacial spasm.

Lipoma is a very rare tumour at the cerebellopontine angle. We report a case of incomplete hemifacial spasm, associated with a lipoma involving and compressing both facial and acoustic nerves at their origin in the brainstem. The patient was treated with medical therapy (botulinum toxin A) and surgery. We present a review of the last ten years of the literature, with particular regard to management.

Human chromosome 7 carries the beta 2 interferon gene.

A cDNA clone (pAE20-4) corresponding to the 1.3-kilobase human beta 2 interferon mRNA was used as a probe in blot-hybridization experiments of DNA from a panel of human-rodent somatic cell hybrids containing overlapping subsets of human chromosomes. The DNA hybridization experiments showed that the human beta 2 interferon gene is located on human chromosome 7. This assignment is consistent with previous experimental data in which the expression of the translationally active 1.3-kilobase beta 2 interferon mRNA was assayed in various somatic cell hybrids. Blot-hybridization experiments using DNA from different human cell strains and cell lines reveal distinct EcoRI restriction fragment length polymorphisms of the human beta 2 interferon gene.