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Biomedical subjects

J Touchon

Publications and source records attributed to J Touchon.

At least 19 recordsLinked to original sources

Prevention trials in Alzheimer disease: one step forward?

Finding strategies that prevent or delay the onset of dementia in Alzheimer disease (AD) will be a challenge for the years to come. Prevention trials in AD pose several unresolved questions, including methodologic, scientific, medical, regulatory, and ethical issues. A critical concern is the benefit and relative risk of giving a treatment to non-demented patients or to asymptomatic subjects. Some trials are under way and will perhaps move the field of prevention of dementia one big step forward.

Alzheimer Disease↗

Mild cognitive impairment (MCI) in medical practice: a critical review of the concept and new diagnostic procedure. Report of the MCI Working Group of the European Consortium on Alzheimer's Disease.

Mild cognitive impairment (MCI) was proposed as a nosological entity referring to elderly people with mild cognitive deficit but no dementia. MCI is a heterogeneous clinical entity with multiple sources of heterogeneity. The concept of MCI was reviewed and a diagnostic procedure with three different stages was proposed by the European Consortium on Alzheimer's Disease Working Group on MCI. Firstly, MCI should correspond to cognitive complaints coming from the patients or their families; the reporting of a relative decline in cognitive functioning during the past year by a patient or informant; cognitive disorders as evidenced by clinical evaluation; absence of major repercussions on daily life; and absence of dementia. These criteria, similar to those defined during an international workshop in Stockholm, make it possible to identify an MCI syndrome, which is the first stage of the diagnostic procedure. Secondly, subtypes of MCI had to be recognised. Finally, the aetiopathogenic subtype could be identified. Identifying patients at a high risk for progression to dementia and establishing more specific and adapted therapeutic strategies at an early stage, together with more structured overall management, is made possible by the diagnostic procedure proposed.

Activities of Daily Living↗

[Chemotherapy treatment for anti-Hu paraneoplastic syndrome without active malignancy].

INTRODUCTION: Anti-Hu associated paraneoplastic neurological syndromes are rare and characterized by poor prognosis. The research and treatment of a related cancer, a small-cell lung cancer most of the time, remains the best therapeutic strategy. CASE REPORT: We describe the clinical course of a paraneoplastic subacute sensory neuronopathy associated with anti-Hu antibodies in a male smoker treated by an early chemotherapy active against a small-cell lung cancer although no tumor could be found at repeated evaluations. In spite of this treatment, the neurological state deteriorated with the appearance of a cerebellar degeneration, and limbic encephalitis which resulted in a loss of autonomy. A small-cell lung cancer was found and treated 65 months after the onset of the neurological symptoms. The treatment of the underlying malignancy, when it can be found, is still considered as the optimal treatment for paraneoplastic neurological syndromes. Although no tumor could be found, we treated our patient with an empirical chemotherapy active against the most frequent malignancy associated to anti-Hu syndrome in a smoker man, without any improvement. CONCLUSION: Active and repeated research for a cancer related to an anti-Hu neurological syndrome and its treatment are undispensable. For our patient without any identified cancer empirical chemotherapy treatment was unable to stop neurological worsening. When no tumor can be identified by conventional imaging techniques, an early FDG-PET scan should be considered and then repeated if normal.

Antibodies↗

[Consensus statement on severe dementia].

Under the auspices of the French Society of Gerontology and Geriatrics, a multidisciplinary group of experts, including geriatricians, neurologists, epidemiologists, psychiatrists, pharmacologists, and public health specialists developed consensus recommendations about care for patients with severe dementia. They defined 21 recommendations for general practitioners, long-term care physicians, and specialists, based on the knowledge currently available (2005). The aim of care at all stages is to mitigate the quality-of-life of patient, caregiver, and family insofar as possible, combining care and future planning until the end of life. Management, to take into account problems including nutritional status, behavior disorders, and ability (or inability) to perform activities of daily living, must be global, multidisciplinary, and coordinated and must optimize use of local medical and social resources. The group also stressed the importance of clinical research to improve knowledge of disease course and assess management strategies and recommended specific area for research.

Aged↗

[Consensus statement on severe dementia].

Under the auspices of the French Society of Gerontology and Geriatrics, a multidisciplinary team including geriatritians, neurologists, epidemiologists, psychiatrists, pharmacologists and public health specialists developed a consensus on care for patients with severe dementia. They defined 21 recommendations for general practitioners, long-term care physicians and specialists based on knowledge available in 2005. At all stages of the disease, the objective of care is to improve as much as possible quality-of-life for the patient and his/her family, including a life project until the end of life. It is always possible to do something for these patients and their family: nutritional status, behavior disorders, and incapacities to deal with basic activities of daily life have to be taken in consideration. Resource allocation and proximity care have to be targeted. Research areas necessary to improve the care of patients with severe dementia has been selected.

Aged↗

Dissociation in circadian rhythms in a pseudohypersomnia form of fatal familial insomnia.

The authors present clinical, sleep, and neuroendocrine features of a patient with genetically confirmed fatal familial insomnia (D178N mutation with heterozygosity at codon 129 of the prion protein gene). The patient exhibited pseudohypersomnia behavior instead of insomnia. There was profound alteration in the sleep-wake cycle with a clear dissociation in the disappearance of circadian and neuroendocrine rhythms, findings unrelated to abnormalities in the hypocretinergic system.

Adrenocorticotropic Hormone↗

Effect of age on MSLT results in patients with narcolepsy-cataplexy.

OBJECTIVE: To measure the effect of age on Multiple Sleep Latency Test (MSLT)characteristics, sleep latency, and number of sleep-onset REM periods (SOREMP) in two large populations of narcoleptic patients with similar genetic backgrounds. METHODS: Clinical and polygraphic information on the severity of the condition was obtained on 236 well-defined narcolepsy-cataplexy-human leukocyte antigen DR2-positive patients from Montpellier (France) and on 147 similar patients from Montreal (Canada). RESULTS: The results show a progressive decrease in the number of SOREMP with age and a progressive increase in the mean sleep latency on the MSLT as a function of age. This finding is also related to the severity of cataplexy as assessed from the clinical history with a progressive decrease in the frequency of cataplexy attacks with age. These results may reflect the progressive increase in sleep latency seen in normal aging and suggest that clinical improvement might be due to changes in the neural mechanisms responsible for SOREMP, which may weaken with age. CONCLUSIONS: The progressive decrease in the number of SOREMP and increase in the mean sleep latency on the MSLT as a function of age suggest that the current criteria used for diagnosis may be too stringent in older patients. The major influence of age on MSLT results should therefore be taken into account when diagnosing a narcoleptic patient.

Adolescent↗

Prediction of transition from cognitive impairment to senile dementia: a prospective, longitudinal study.

OBJECTIVE: The purpose of this investigation was to replicate the statistical approach used in a previous investigation (Toronto study) within a French population to determine the best predictive model for Alzheimer's disease (AD). METHOD: Data from neuropsychological tests from two prospective studies were entered into a regression model. RESULTS: Replication of the statistical approach in the Montpellier sample produced a three-test model with a specificity of 99% and sensitivity of 73%. This model consisted of a delayed auditory verbal recall test, a construction test, a category fluency test and provides probability estimates for the transition to dementia in individual cases. CONCLUSION: The models derived from these two longitudinal studies provide an empirical basis for the selection of tests for the definition of mild cognitive impairment of the Alzheimer type (MCI-A). The small set of tests derived are suitable for use in general practice.

Adult↗

CSF hypocretin-1 levels in narcolepsy, Kleine-Levin syndrome, and other hypersomnias and neurological conditions.

OBJECTIVE: To determine the role of CSF hypocretin-1 in narcolepsy with and without cataplexy, Kleine-Levin syndrome (KLS), idiopathic and other hypersomnias, and several neurological conditions. PATIENTS: 26 narcoleptic patients with cataplexy, 9 narcoleptic patients without cataplexy, 2 patients with abnormal REM-sleep-associated hypersomnia, 7 patients with idiopathic hypersomnia, 2 patients with post-traumatic hypersomnia, 4 patients with KLS, and 88 patients with other neurological disorders. RESULTS: 23 patients with narcolepsy-cataplexy had low CSF hypocretin-1 levels, while one patient had a normal hypocretin level (HLA-DQB1*0602 negative) and the other two had intermediate levels (familial forms). One narcoleptic patient without cataplexy had a low hypocretin level. One patient affected with post-traumatic hypersomnia had intermediate hypocretin levels. The KLS patients had normal hypocretin levels while asymptomatic, but one KLS patient (also affected with Prader-Willi syndrome) showed a twofold decrease in hypocretin levels during a symptomatic episode. Among the patients without hypersomnia, two patients with normal pressure hydrocephalus and one with unclear central vertigo had intermediate levels. CONCLUSION: Low CSF hypocretin-1 is highly specific (99.1%) and sensitive (88.5%) for narcolepsy with cataplexy. Hypocretin ligand deficiency appears not to be the major cause for other hypersomnias, with a possible continuum in the pathophysiology of narcolepsy without cataplexy and idiopathic hypersomnia. However, partial hypocretin lesions without low CSF hypocretin-1 consequences cannot be definitely excluded in those disorders. The existence of normal hypocretin levels in narcoleptic patients and intermediate levels in other rare aetiologies needs further investigation, especially for KLS, to establish the functional significance of hypocretin neurotransmission alterations.

Adolescent↗

["The 5 words": a simple and sensitive test for the diagnosis of Alzheimer's disease].

OBJECTIVE: Alzheimer's disease (AD) is under-diagnosed in France. Today only an estimated 50% of patients are identified. Diagnosis of AD is particularly difficult because the amnesic syndrome, characteristic of the disease, is often confused with memory dysfunction that is frequent during the process of aging. The improvement in the diagnostic conditions of AD relies on the availability of a simple and reliable tool for screening memory disorders of organic origin. METHOD: The 5-word test studies the recall of a short list, which the physician ensures the patient has registered. Its construction permits the identification of patients exhibiting objective memory disorders. A validation study has been conducted in 86 patients suffering from AD and 126 persons complaining of functional memory disorders. RESULTS: The study has shown the sensitivity (91%) and specificity (87%) of the 5-word test in identifying patients with AD. CONCLUSION: This is a rapid (2 minutes) and simple test that is easy to use in medical practice for the screening of AD.

Age Factors↗

[Alzheimer's disease: a public health problem: yes, but a priority?].

Alzheimer's Disease is a major Public Health problem for many reasons. First, it is a frequent disease since, in France, the prevalence was estimated at about 400.000 cases, and the annual incidence at 100.000 cases. The frequency of the disease increases, in particular due to the ageing of the population. This disease has major consequences on the life of the patient and his/her caretaker. The cost of the disease is important, estimated at about 50 milliards of French francs. Pharmaceutical treatment and other interventions are possible in particular to delay the nursing home placement. On the other hand, this disease is often ignored, under-diagnosed, underestimated and exposed to inequality in resorting to care. In summary, Alzheimer's Disease (AD) has all the criteria required for a major public health problem. In spite of this observation, AD is not yet considered as a priority for health authorities, although attitudes are changing.

Accidents, Traffic↗

[REM Sleep behavioral disorder].

Rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia which occurs only during REM sleep. RBD is characterized by the loss of skeletal muscle atonia, which is the principal feature of REM sleep, and by abnormal behavior representing the attempted enactment of dreaming. Clinically, it consists of abnormal behavior, frequently violent, that may lead to injuries. A polysomnographic study is necessary to assess the diagnosis showing the absence of REM sleep atonia and related abnormal behavior. RBD usually affects older men. Its exact etiological factors remain unknown. However, RBD is sometimes linked with drug use or exposure to toxic conditions. Moreover, the association with neurological disorders is frequently reported, particularly cerebrovascular, tumoral or neurodegenerative disease. Neurodegenerative disorders with parkinsonism are frequently involved. In Lewy body dementia, RBD is particularly frequent. In many cases, RBD may have a predictive value, preceding other symptoms of neurodegenerative disorders with parkinsonism. The biochemical mechanisms underlying RBD still remain unclear but could involve the dopamino-cholinergic balance and the serotoninergic pathway.

Anticonvulsants↗

[Mild cognitive impairment: imaging data].

The diagnosis of Mild Cognitive Impairment (MCI) as defined by Petersen et al. (1997), is based uniquely on clinical observations. Cerebral imaging, both morphological and functional, may in fact facilitate diagnosis, particularly with regard to the differentiation of sub-types of MCI and the identification of prodromal AD (MCI-AD). Volumetric Magnetic Resonance Imagery (MRI) examination of structures affected early in AD such as entorhinal cortex, the temporal lobe and, above all, the hippocampus, are especially useful. Hypoactivity within these regions, especially of the temporal lobe and posterior cingulate gyrus by Positon Emission Tomography, and more recently monophotonic emission tomoscintigraphy, also appears to have diagnostic utility. Studies of cholinergic system activity by functional imaging may also be of future value in MCI-AD diagnosis. MRI used in conjunction with other techniques may be of significant value, in particular the use of spectro-MRI and functional MRI. This latter technique, leading to the development of cognitive activation paradigms, is particularly promising.

Aged↗