Ethical issues in end of life treatments for patients with dementia.

Dementia is a terminal disease, associated with great suffering and difficult decisions in the severe stage. The decision-making process is characterized by uncertainty because of lack of scientific evidence in treatments and by the need to reconcile conflicting points of view. In intercurrent diseases, aggressive interventions are used without consideration of its futility; in comparison with cancer, several consequences of physicians' attitude not to consider dementia as a terminal disease have been reported, especially concerning pain relief. Lack of evidence of artificial nutrition and hydration effectiveness makes advance care planning relevant.

Ethical questions in the treatment of subjects with dementia. Part I. Respecting autonomy: awareness, competence and behavioural disorders.

The document deals with some ethical issues raised by the treatment of demented people. In particular the conceptual and empirical aspects of the assessment of awareness and competence of these patients are analysed, as well as the dilemmas related to the treatment of behavioral disorders.

Left caloric vestibular stimulation ameliorates right hemianesthesia.

BACKGROUND: Left caloric vestibular stimulation (CVS) transiently reduces impairments of right-brain-damaged patients with left unilateral neglect, including left hemianesthesia, contralateral to the side of the lesion (contralesional). Conversely, no effect on right contralesional hemianesthesia in left-brain-damaged patients is seen with right CVS. This discrepancy is unexplained. METHODS: The authors explored the effect of CVS on right- and left-brain-damaged patients with hemianesthesia. One left-brain-damaged patient had an fMRI study during tactile stimulation before and after left CVS. The same fMRI touch study, without CVS, was performed in neurologically unimpaired subjects. RESULTS: A transient remission of right hemianesthesia associated with left brain damage was observed, provided that cold CVS was administered to the left ear. In the left-brain-damaged patient studied with fMRI, left CVS modulated the neural response to right hand tactile stimuli of a portion of the secondary somatosensory area (SII) of the right hemisphere. In neurologically unimpaired subjects, fMRI scans showed that the same part of area SII in the right hemisphere was activated by ipsilateral right-sided touches and to a larger extent than area SII in the left hemisphere by left-sided touches. CONCLUSIONS: Left caloric vestibular stimulation is effective on both left and right hemianesthesia because it modulates the hemisphere that has a more complete representation of, or is capable to attend to, the whole somatosensory surface of the body. These results suggest a hardwired hemispheric asymmetry in hand representation, starting from a somatotopically organized brain region such as area SII.

Diagnostic value of sural nerve matrix metalloproteinase-9 in diabetic patients with CIDP.

Patients with diabetes mellitus (DM) may develop chronic inflammatory demyelinating polyneuropathy (CIDP), which may be difficult to distinguish from diabetic neuropathy (DNP). Here the authors show that immunoreactivity for matrix metalloproteinase-9 on sural nerve biopsies may help to identify CIDP-DM. In a pilot study on 10 CIDP-DM patients with IV immunoglobulins and tight glycemic control, the CIDP-DM patients had a better outcome than DNP patients treated with tight glycemic control only.

Pathophysiology, clinical manifestations and supportive care of metastatic brain cancer.

Brain metastases (BrM) are tumours that originate in tissues outside the central nervous system and spread secondarily to involve mainly the brain. The management of patients with cerebral metastases is complex, costly, and in some instances controversial. Furthermore, even in patients with widespread systemic cancer, the symptoms of the disease are often controllable while the symptoms of the BrM may be disabling. The treatment of BrM is one of the few areas of neuro-oncology where real progress has been made in the last twenty years. Moreover, the costs of managing this disease are rising, as therapies become more intensive and the number of patients with BrM increases. Modern neuroradiological imaging techniques, which are able to discover BrM earlier in the course of systemic cancer, and the greater efficacy of specific treatments, which lengthens survival, have increased the prevalence. The aggressive treatment of BrM may add some benefits to the patient, but its excessive cost leads to the necessity for accurate cost-effectiveness analysis. The latter begins with a complete understanding of the disease: its diagnosis, natural history and results of various modalities of treatment. While the development of BrM usually indicates a poor prognosis for the patient, advances in supportive care have made it possible to reverse most of the neurological symptoms and to give patients a meaningful extension of useful life.

Thrombolysis for acute ischemic stroke: the patient's point of view.

BACKGROUND: The use of thrombolytic therapy for acute ischemic stroke is still controversial. A major problem is balancing the improvement in functional ability against the risk of early death from cerebral hemorrhage. Our aim was to assess whether patients who have had a stroke, and their proxies, would give consent to thrombolysis if this therapy were introduced into clinical practice for acute ischemic stroke in Italy. METHODS: A 10-item questionnaire was administered by personnel not directly involved in the care of patients in 12 Italian hospitals. Interviews were carried out with at least 10 consecutive stroke inpatients per center in the postacute phase and to their proxies. RESULTS: There were 685 responders (377 female), median age 58 years (range 18-98); 332 were patients and 353 proxies. Responders were representative of the Italian population as a whole as regards mean age and sex, education and marital status; 59% of responders (95% confidence interval 55-62%) would agree to thrombolysis in the case of stroke. There was more uncertainty among proxies than patients, especially when the decision had to be taken for a relative (41 vs. 17% could not decide, p < 0.001). The preference for thrombolysis was higher among more educated people (p = 0.001) and was not influenced by sex, age and marital status. Overall, 81% of responders would prefer to risk dying rather than remain severely disabled. CONCLUSION: Thrombolytic therapy is feasible in Italy as there is ample willingness to trade off a better functional outcome with a higher risk of death. Education is the main sociodemographic determinant of independent decision-making, as only those with an adequate cultural level are able to discriminate between one treatment option and another. The proxy's uncertainty about how to interpret a patient's preference regarding consent to thrombolytic therapy should be tackled, since proxies play a key role in making patients' preferences known in case of incompetence after an acute stroke.

Prospective follow-up study of chronic polyneuropathy of undetermined cause.

Forty consecutive patients with chronic sensorimotor polyneuropathy of undetermined cause were followed to determine disease progression and prognosis. They had had neurological symptoms for at least 1 year before presentation. Neurophysiological examination in all patients showed chronic axonal degeneration, which was confirmed by sural nerve biopsy. Patients were reviewed every 3 months. Laboratory tests were performed every 6 months for the first 2 years and then annually. Neurophysiological examination was performed annually in all patients. Patients were followed up for at least 4 years. In no instance was a possible etiological factor detected during follow-up. The clinical and electrophysiological findings had a slowly progressive course. We suggest that patients with a chronic polyneuropathy of undetermined cause despite detailed investigations do not require further extensive and expensive laboratory tests and neurophysiological studies during follow-up.

The effect of anticonvulsant drugs on blood levels of methotrexate.

The reduced bioavailability of chemotherapeutic agents is one of the reasons that explains the limited efficacy of adjuvant chemotherapy in high grade glioma patients. We report how even the results of high dose sequential chemotherapy can be influenced by antiepileptic drugs.

Chorea as a symptom of neuroborreliosis: a case study.

Borrelia burgdorferi (Bb) can cause a large number of neurological symptoms. Although extrapyramidal disturbances are rare (representing less than 2% of all neurological complications), diffuse choreic dyskinesias have been described during the course of mild encephalitis. The data published in the literature suggest that there are clinical and neurological analogies between neuroborreliosis and multiple sclerosis (MS). The presence of specific anti-Bb antibodies in cerebrospinal fluid is a discriminating factor that allows a diagnosis of neuroborreliosis to be made. We describe the case of a patient with Lyme disease, characterised by widespread chorea and behavioural disturbances. Emphasis is placed on the atypical onset and evolution, the difficulties encountered in formulating a diagnosis, and the uncertainties concerning the pathophysiology and clinical/neuroradiological correlations of the disease.

Kennedy's disease: clinical and molecular study of two Italian families.

Kennedy's disease, or spinal and bulbar muscular atrophy (SBMA), is a rare X-linked motoneuron disorder with variable signs of androgen insensitivity. It is associated with the expansion of a trinucleotide CAG repeat within the androgen receptor (AR) gene. We here report our clinical and molecular findings in two Italian families with Kennedy's disease. The increased size of the CAG repeat was demonstrated in four affected males and seven carrier females.

Superficial siderosis of the central nervous system after brachial plexus injury. Case report.

Superficial siderosis is a rare condition characterized by deposition of hemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. It is associated with cerebrospinal fluid abnormalities consistent with recurrent bleeding into the subarachnoid space. The usual symptoms are hearing loss, ataxia, spastic paraparesis, sensory and sphincter deficits, and mental deterioration. A case is presented of severe superficial siderosis of the central nervous system in a 51-year-old man who had suffered a brachial plexus injury at the age of 20 years. The diagnosis was made by means of magnetic resonance imaging 16 years after the initial symptoms, which comprised bilateral hearing loss and anosmia. Subarachnoid bleeding was due to traumatic pseudomeningocele of the brachial plexus, a very unusual cause of superficial siderosis. This case is interesting insofar as the surgical treatment prevented further bleeding and possibly progression of the disease.

Before M.A.S.T.-I: analysis of the population of a participating center.

Between 1 January 1989 and 31 December 1990 568 consecutive patients were admitted to our unit for ischemic stroke (defined on clinical criteria and with CT or MR evidence of ischemic areas or exclusion of hemorrhage or tumor). The interval between onset of symptoms and arrival in hospital was under 6 h in 270 cases (47.5%). We classified our population according to the clinical criteria proposed by Bamford et al. by subgroup as follows: LACI (27.5%); PACI (24.5%); TACI (30.6%); POCI (17.4%). The mortality in our population was 10.9%. The distribution of the risk factors considered was: hypertension in 387 cases (67%), diabetes in 158 cases (27.8%), NVAF in 95 cases (16.7%). Our data show that about a half of all patients with ischemic stroke can expect to receive treatment at the acute stage.

Atypical features and prognosis of Wallenberg syndrome: longitudinal study.

The lessons of a retrospective study of 24 consecutive cases of Wallenberg syndrome observed during a 6-year span are described: the high frequency of signs and symptoms not considered typical, such as headache, diplopia, supranuclear homolateral facial paresis and homolateral brachiocrural hemiparesis; the unfavorable short term course in a high percentage of cases, and the risk of sudden respiratory arrest.

Transient global amnesia: a clinical and EEG study.

33 cases of transient global amnesia are reported. An interaction between organic and functional factors in determining the syndrome is supposed.