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A 45-year-old woman presented with a 10 year history of asymmetrical facial flushing and sweating after exertion or in hot weather. During these episodes the right side of her face remained dry and white, while the left side normally flushed. Sweating was impaired on the left side in the limbs and trunk. She also had areflexia in the lower limbs and slow pupillary reactions to light and darkness, as seen in Adie's syndrome. The topography of the sweating disorder suggested that the lesion involved the sympathetic pathways at the level of spinal cord. The relationship with the harlequin syndrome and related disorders is discussed.

242 Background: The supportive care and rehabilitative needs of cancer patients are highly varied and complex, and optimal care planning often requires the provision and coordination of multiple modalities. To address these needs, an innovative model for multi-disciplinary evaluation and management within a survivorship clinic was piloted. Methods: Between November 2013 and May 2014, 50 patients who had completed cancer therapy with curative intent were seen in a multi-disciplinary survivorship clinic (MDSC) sequentially by an advanced practice practitioner (nurse practitioner or physician assistant), oncology social worker, and oncology rehabilitation therapist. Data regarding specific care planning resulting from this encounter, compliance with recommended therapies, and patient-reported satisfaction were collected. These data were compared against the remaining cohort of patients (N = 402) who completed curative intent cancer therapy in our cancer program during the same period. Results: Rate of referral for rehabilitation services (83.3% vs 13.9%, p < 0.01) and compliance with recommended therapy visits (85.7% vs 44.6%, p < 0.01) were significantly increased among patients seen within the MDSC pilot when compared with similar patients who were not seen in this setting. The number of referred therapy modalities was also increased among patients seen in the MDSC pilot, median 2 (range 1-3 modalities) vs 1 (range 1-2 modalities), respectively. Of patients who completed an assessment following the multi-disciplinary visit, 86% scored their overall experience as excellent which was the highest possible score. Conclusions: Patients seen within an innovative pilot program of multi-disciplinary survivorship care planning appreciated increased rates of referral for rehabilitation services, were referred for a greater number of therapy modalities, and were more compliant with recommended interventions than similar patients who were not seen in this setting. This patient cohort also reported high rates of satisfaction with the pilot program.

OBJECTIVETo evaluate tumor control and complications associated with low-dose radiosurgery for vestibular schwannomas.METHODSBetween December 1993 and January 2000, 47 patients with vestibular schwannomas were treated at our center with gamma knife radiosurgery. The marginal tumor doses ranged from 7.5 to 14.0 Gy (median, 12.0 Gy) for patients treated after microsurgery and from 10.0 to 15.0 Gy (median, 12.0 Gy) for patients in whom radiosurgery was the primary treatment. The median maximum tumor diameter was 18 mm (range, 3–50 mm). Evaluation included audiometry, neurological examination, and serial imaging tests. A survey was conducted at the time of analysis.RESULTSFollow-up data were available for 45 patients and ranged from 1 to 7 years (median, 3.6 yr). In 43 patients (96%), tumor control (no radiographic progression or surgical resection) was observed. All 33 previously untreated patients had tumor control. Transient facial weakness, experienced in two patients (4%), had resolved completely within 6 months. No patient developed trigeminal neuropathy. Hearing was diminished from baseline in 12% of patients with useful hearing (Gardner-Robertson Class III). However, all patients with pretreatment hearing Gardner-Robertson Class I or II maintained testable hearing (Class I to III) at the most recent examination.CONCLUSIONLow-dose radiosurgery in this series provided comparable local control and decreased incidences of complications in relation to other reports. Additional follow-up will allow more definitive conclusions to be reached regarding the ultimate rates of tumor control and hearing preservation. Nevertheless, the current dose used for vestibular schwannomas at the University of Maryland Medical Center is 12.0 Gy to the tumor periphery.

The value of an early initial coadministration of levodopa (L-dopa) and lisuride in Parkinson's disease was the main goal of the present study. Eighty-two patients with recently diagnosed idiopathic Parkinson's disease were randomized into two groups for treatment with L-dopa alone or L-dopa + lisuride. The trial was double-blinded for the first year and open for the following 4 years. Selegiline (10 mg/day b.i.d.) was added in both groups at the end of the first year. Outcome measures were evolution of L-dopa dosage and Unified Parkinson's Disease Rating Scale scores and subscores, and incidence of motor complications. The dropout rate was higher in the L-dopa group (63.4%) than in the combination group. Motor improvement was better (p0.01) in the L-dopa + lisuride group. Expected motor complications were rare, moderate and equivalent in the two groups despite a difference in L-dopa dosage (446.7 vs. 387.5 mg/day). Long-term follow-up demonstrated the L-dopa-sparing effect of lisuride (average 1 mg/day), the beneficial effect of early combination therapy on motor status and the paucity of motor complications in both groups.

Critical illness polyneuropathy (CIP) is a reported cause of varying degrees of neuromuscular weakness in patients with multiple organ failure. Little is known concerning predictive factors of neurological recovery. The critical care conditions, neurological explorations and 2-year clinical follow-up of 19 patients who suffered from severe forms (quadriplegia or quadriparesis) of CIP were analyzed. Characteristics of patients who recovered clinically were compared with those of patients who did not. Two patients died within 2 months, 11 recovered completely, 4 remained quadriplegic and 2 remained quadriparetic. All patients suffered from sepsis, multiple organ dysfunction syndrome and a catabolic state before the onset of CIP. Outcome appears difficult to predict with clinical or electrophysiological data. Three parameters were significantly correlated with poor recovery: longer length of stay in the critical care unit, longer duration of sepsis and greater body weight loss. A relationship seems to exist between the severity of CIP and that of sepsis and its associated hypercatabolism. The favorable outcome usually attributed to CIP must be reconsidered. The authors recommend aggressive measures against sepsis to limit CIP and its sequelae.

The concept of memory centers is based on a multidisciplinary approach to memory disorders and dementia, especially Alzheimer's disease, a public health challenge. The first memory centers were established in the 80s in the US and the British Isles. The aims of these centers are to make a diagnosis (and to reassure the 'worried well'), to detect dementia, to provide a service for their management, to educate care takers, to evaluate new therapies, and to contribute to clinical and basic research. Follow-up is crucial. The first memory centers all experienced long delay to diagnosis of dementia and found that Alzheimer's disease was the first cause of consultation. These observations led to the creation of such centers in many countries. A survey of 20 French memory centers defined the "ideal memory center": an identified structure with a clinic and a day-care unit for diagnosis and follow-up, with neurological, psychological, psychiatric, and geriatric skills. It must be part of a medical and social network for the management of dementia and participate in a network of memory centers at the regional and national level. Relationships between dementia and somatic diseases, frequent in demented patients, are still poorly known. Dementia interferes with the clinical expression, the management, and the prognosis of somatic diseases, some of which, such as cardiovascular conditions, are possibly linked with dementia. Conversely, somatic diseases may rapidly worsen the cognitive state and induce delirium, leading to hospitalization. Medical wards are not all appropriate. Memory centers must also be involved in these care, educational and research issues.

Donepezil has been shown to be well tolerated and to improve cognition and global function in patients with mild to moderately severe Alzheimer's disease (AD). The current trial was undertaken to investigate further the efficacy and safety of donepezil, in a multinational setting, in patients with mild to moderately severe AD. This 30-week, placebo-controlled, parallel-group study consisted of a 24-week, double-blind treatment phase followed by a 6-week, single-blind, placebo washout. Eight hundred and eighteen patients with mild to moderately severe AD were randomly allocated to treatment with single, daily doses of 5 or 10 mg donepezil, or placebo. The two primary efficacy measures were: a cognitive performance test, the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog) and a global evaluation, the Clinician's Interview-Based Impression of Change with caregiver input (CIBIC plus). Secondary outcome measures included the Sum of the Boxes of the Clinical Dementia Rating Scale (CDR-SB), a modified Interview for Deterioration in Daily living activities in Dementia (IDDD) and a patient rated quality of life assessment. Statistically significant improvements in cognitive and global function were observed, as evaluated by ADAS-cog and CIBIC plus, respectively, in both the 5 and 10 mg/day donepezil groups, compared with placebo. Treatment-associated changes were also observed in functional skills, as shown by improved scores on the CDR-SB and the complex-tasks component of the IDDD. A dose-response effect was evident, with the 10 mg/day donepezil group demonstrating greater benefits in all outcome measures than the 5 mg/day group. Donepezil was well tolerated by this patient population and did not produce any clinically significant laboratory test abnormalities. The results of this study confirm that donepezil is effective and well tolerated in treating the symptoms of mild to moderately severe AD.

An 82-year-old woman developed acute onset isolated paralysis of the right ninth, tenth, eleventh and twelfth cranial nerves (Collet-Sicard syndrome). The polynevritis was associated with major weight loss, increased blood pressure, nephropathy, distal arteriopathy and an inflammatory syndrome, suggesting panarteritis nodosa. Neuromuscular biopsy results confirmed the diagnosis. Collet-Sicard syndrome resulted from ischemia in the territory of the ascending pharyngeal artery.

Memory complaints are frequent and not always the result of a memory impairment. Such complaints must be taken into account since they always reflect some disorder, at least of attentional processes. Excepting primary psychiatric disorders, biological and imaging exams are required in addition to a neuropsychological and behavioral assessment. Finding the cause of the memory complaints is not an end in itself. The purpose is to cure or at least to relieve the consequences of this and related symptoms with pharmacological, neuropsychological and psychological therapies, social help, information and support. Patients (especially in case of dementia) and families need follow-up care by a multidisciplinary staff (neurologists, psychiatrists, psychologists, speech therapists, nurses, nurse's aids and social workers) who play a role of prevention for intercurrent diseases, social and legal counselling and help anticipate the future. Multidisciplinary memory units are dedicated to the diagnosis and care of memory complaints and disorders. We describe here the operative function of these centers which should be integrated into a health care network centered around patients with memory complaints and their families.

Dementia with Lewy Bodies (DLB) is an entity which now fulfils clinical and neuropathological criteria according to international consensus guidelines (McKeith et al., 1996). It is now possible, in clinical practice, to consider the diagnosis of DLB from the beginning of the management of a demented patient. The clinical, diagnostic and therapeutic aspects of DLB are investigated in a prospective manner. Visual hallucinations, fluctuations and extrapyramidal symptoms seem to have, in association with the progressive cognitive decline, some particularities. However their specificity should be correlated with neuropathological data. Other symptoms, such as repeated falls or syncope, neuroleptic sensitivity, systematized delusions or other modalities of hallucinations, are probably additional arguments giving more predictive value to the association of the major symptoms. The role of neuropsychological patterns at the beginning of the cognitive decline is shown. There are some recent concordant results of functional imaging in DLB. The neuropathological aspects of DLB and the links with Parkinson pathology and especially Alzheimer pathology are emphasized.

Frontotemporal dementia (FTD) is poorly recognized clinically. Of the 1,517 patients examined at the Lille Outpatients Memory Clinic data bank (1991-1995), 74 fulfilled the criteria of the Lund and Manchester groups for FTD. They accounted for 5 p, 100 of all patients, 1 for 10 probable or possible Alzheimer's disease. Mean patient age was 63 years, duration of the disease was 5 years, mean Mini Mental State score was 23; 45 p. 100 belonged to the active population. Behavioral disorders occurred before the cognitive decline and remained the major feature. All patients had at least 3 of the following symptoms: self-control impairment, affective disorder, loss of interest and self-neglect. Memory impairment consisted of correct encoding and impaired retrieval processes, without major storage impairment. No patients had spatial disorientation. Language was usually reduced, EEG was normal. Two diagnoses were confirmed by autopsy: both consisted of aspecific frontal and temporal degeneration, 41 p. 100 of the patients were referred by a general practitioner, 30 p. 100 by a psychiatrist, 16 p. 100 by a neurologist, 2 p. 100 by other specialists, and 11 p. 100 following the advice of their relatives. FTD had never been suspected. Alzheimer's disease or non specified degenerative dementia was suspected in 2/3 of patients and a psychiatric disorder in 1/3. With the advent of novel pharmacological agents for the treatment of dementing disorders and for research purposes, the identification and accurate differentiation of FTD from Alzheimer's disease and psychiatric disorder is essential. Therefore, the role of multidisciplinary memory clinic is crucial to differentiate FTD from other degenerative dementias.

A group of French expert met on the 7th and 8th of February 1998 in order to establish a consensus attitude for Alzheimer's disease diagnosis and treatment. Members were drawn from primary care, geriatrics, neurology and psychiatry. They used the consensus statement of the American Association for Geriatrics, the Alzheimer's Association and the American Geriatrics Society published in JAMA, in October 1997 as a source of data for further consideration. Alzheimer's disease in the most common etiology of dementia. Main clinical features are cognitive impairment and psycho-behavioral disorders. Diagnosis must be one of inclusion and not exclusion. It is based on interviews of informants and family members and office-based clinical assessment. After a physical examination, cognitive function must be evaluated using the Mini-Mental State Examination. A laboratory evaluation should include a complete blood cell count, blood chemistry and determination of thyroid-stimulating hormone. In addition, noncontrast computed tomography head scans are adequate in most cases. Available pharmacologic treatments are not curative but are given to improve quality of life and enhance cognition and behavior. Two cholinesterases inhibitors, tacrine and donepezil, are the only agents officially authorized for treatment of the cognitive impairment in Alzheimer's disease. Mood and behaviour disorder also have to be treated by both pharmacologic and nonpharmacologic strategies. Only pharmacologic treatments will be detailed here. The consensus statements established by this group of experts will be reevaluated each year, considering the new available data on Alzheimer's disease.

A 59 year-old-man had ataxia and hemiparesis of progressive onset which resolved in a few weeks. CT scan and magnetic resonance imaging showed white matter multiple lesions. The cerebrospinal fluid was consistent with an inflammatory disease. The diagnosis of multiple sclerosis was evoked. However, the patient had chronic brucellosis and serological rates increased and decreased according to the clinical and radiological signs. Thus, encephalitis caused by brucellosis was evoked. The mechanism is assumed to be a demyelinating process caused by a deep focus reactivation.

Purpose: Several recent open studies have provided encouraging results as to the efficacy of intravesical installations of capsaicin for neurogenic hyperreflectivity. The present trial was performed to verify these results under controlled conditions. Intravesical installation of capsaicin represents a new therapeutic hope for the treatment of the neurogenic hyperrelfexic bladder. Method: This randomized, double-blind study compared the results of the intravesical installation of 30 mg capsaicin in 100 ml of 30% alcohol (experimental group) with those of installing 100 ml 30% alcohol alone (control group). On day 0 and day 30, urodynamic and biopsic examinations were performed in all subjects of each group. Patients: All the subjects included in the study had a functionally disabling form of neurogenic hyperreflexic bladder resistant to the usual therapies. Cystoscopy and retrograde cystography were performed to exclude any patient who presented with a tumor-like lesion or had vesicoureteral reflux. Results: Twelve paraplegic of tetraplegic subjects, seven women and five men whose average age was 46, were included. Eight had multiple sclerosis, and four had sustained a traumatic spinal cord injury. The patients were randomly separated into two groups of six. Initially, there was no clinical or urodynamic differences in these groups. Installation immediately triggered side effects and during the first 7 days (suprapubic burning sensation, sensory urgency, hot flushes, autonomic hyperreflexia, hematuria) in five of the six subjects in both groups. Bladder biopsy revealed no significant deterioration. On day 30, there was improvement in all of the experimental-group of patients with significant regression of leakage (P=0.002) and of sensory urgency (P=0.01). Only one control subject had amelioration. Urodynamic examination showed a rise in bladder capacity from 172.5 to 312.3 ml in the experimental group, significantly greater (P=0.03) than the rise from 129 to 175.3 ml observed in the control group. Conclusion: This trial fully confirms the efficacy of intravesical installations of capsaicin, an efficacy obtained at the cost of nonnegligible side effects. An intermediate-term follow-up of this treatment will be necessary before considering more widespread use of this agent.

Rationnel La precision et la securite des pancreas artificiels utilisant la voie sous-cutanee, dependront largement de la qualite de la pompe utilisee et de la moindre inertie du systeme Perfusion d’insuline/Reponse glycemique. Les pompes actuelles (PA) comportent un pousse-seringue progressant par acoups, et une tubulure. Une patch pompe (PP) avec moteur electromagnetique, sans tubulure, permettrait-elle d’ameliorer la biodisponibilite de l’insuline ? Dans le cadre du programme Diabeloop, notre objectif etait 1) de comparer la precision d’une PP de type Jewel-Debiotech a celle de PA sur le debit de base (DB) d’insuline in vitro 2) d’en evaluer l’impact lors d’un bolus prandial in vivo Patients et methodes La precision du DB a ete evaluee in vitro par pesee continue du liquide perfuse, avec une balance haute precision Sartorius MC5. Les donnees ont ete enregistrees (logiciel Infuscale) et traitees conformement a la norme IEC/EN601-2-24. L’impact sur le repas a ete evalue par un bolus de 6U d’insuline Aspart administre soit avec PP soit avec PA, au petit-dejeuner, chez 14 patients diabetiques type 1 sous pompe, a 15 jours d’intervalle Resultats La precision d’un DB de 1 U/h, sur de courtes periodes (15/30 min), mesuree par la methode des courbes en trompette et la repetabilite de la perfusion, etaient significativement meilleures avec la PP qu’avec les PA (p Conclusion La PP Jewel-Debiotech assure un DB plus precis sur le court terme que les PA. Le bolus prandial parait peu modifie. L’impact lors d’ajustements insuliniques minimes mais repetes dans les conditions de boucle fermee reste a evaluer

Positron emission tomography (PET) studies in dementia suffer from methodological differences. Most PET studies concern resting state metabolism in Alzheimer's disease and its correlations with neuropathology and neuropsychology. Only few clinically based PET studies on sensitivity and specificity of specific findings in specific dementing disorders have been reported. The role of PET in dementia for routine clinical purposes seems limited at present. Future studies should evaluate the potential of PET in preclinical diagnosis and to study treatment effects.

The patient was a 30 year-old man. He had no previous history. For several months he experienced a slowly progressive horizontal diplopia which was the expression of a bilateral third cranial nerve palsy with an intact intrinsic component. Muscular or neuro-muscular pathology as myasthenia was initially suspected but not confirmed. CT scan and MRI revealed an atypical left temporo-insular lesion which led us to discuss a chronic inflammatory pathology as sarcoidosis or a tumoral process. Finally cerebral biopsy showed a high grade oligodendroglioma. Symptomatology was attributed to infiltration of the peduncles from this tumor. Such a case has never been seen before. Early neuroradiological explorations would be useful in case of clinical suspicion of cranial nerve palsy.

Oculopharyngeal muscular dystrophy (OPMD) was described by Taylor in 1915. This autosomal dominant inheritance affection begins at the age of 40-50 years associating bilateral ptosis and dysphagia. In 1980, Tome and Fardeau described rimmed vacuoles and typical intranuclear tubulo-filamentous inclusions in the muscle biopsy. We report two cases (brother and sister) of clinical and histological OPMD with mitochondrial abnormalities (Ragged red fibers) associated with classical OPMD lesions. Those observation remind the question already vised since a long time by some authors, of the signification of mitochondrial abnormalities in OPMD.

Assessment of a new rehabilitation method for unilateral neglect syndrome (UNS), using a specific device (Bon Saint Come's device) that associates exploratory reconditioning with voluntary trunk rotation.Study 1, randomized control trial during 2 months; study 2, nonrandomized control trial during 2 months.Neurorehabilitation units in private and public center.In study 1, 22 consecutive patients with UNS resulting from recent stroke (3 months) were randomly assigned to an Experimental Group (11 patients) or to a Control Group (11 patients). The 2 groups were very similar in terms of general and neurological data. In study 2, 5 consecutive patients with chronic UNS resulting from an old stroke (6 months) showing the same characteristics were included.In study 1, patients in Group E followed the experimental program 1 hour a day for 1 month (20 hours) and Group C followed usual neurorehabilitation during the same time. In study 2, every patient followed the experimental program 1 hour a day for 1 month (20 hours).Assessment in both studies was done at day 0, day 30, and day 60 using a battery of UNS tests (Albert, Scheckenberg, bell) and an activities of daily living (ADL) test (the Functional Independence Measure [FIM]). Mean scores of each test were compared between the 2 groups with the Wilcoxon nonparametric test.In study 1 all UNS test results and the FIM improved significantly more in Group E than in Group C. In Group E, UNS disappeared in 5 patients and improved in 6. In Group C, UNS disappeared in 1 patient, improved in 4, and was unchanged in 6. In Study 2, UNS remitted in 2 patients, improved in 2 patients, and was unchanged in 1.The Bon Saint Come method seems to significantly improve recent and chronic UNS, as well as ADL function. These encouraging results could have resulted from a synergistic effect of spatial reconditioning and voluntary trunk rotation. It must be assessed by a new study with more patients.

One of the main features of numerous neurodegenerative disorders is the presence of specific inclusions in the cerebral cortex. In most of these disorders, these inclusions correspond to the aggregation of abnormal filaments. Neurofibrillary tangles (NFT) are commonly found in Alzheimer’s disease (AD), amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC), head injury, Hallervorden-Spatz disease and progressive supranuclear palsy (PSP). Pick’s disease (PiD), a form of fronto-temporal dementia, is characterized by the presence of chromatolytic neurons and Pick bodies (PB; Brion et al. 1991). Despite many microscopic and ultrastructural differences, these inclusions share similar antigenic properties (Dickson et al. 1985; Delacourte et al. 1990; Hof et al. 1994a, b). Tau immunoreactivity is observed in NFT of most of these neurodegenerative disorders and anti-Tau antibodies immmunolabel PB (Hof et al. 1994 a).

In the authors experience of a memory clinic, about 2/3 of the patients fulfilled the criteria for dementia and among the demented patients 2/3 had probable Alzheimer's disease. Vascular dementia is the second cause of dementia in elderly people, but two other degenerative disorders fulfilling the NINCDS-ADRDA criteria for Alzheimer disease (Mc Khann et al., 1984) account for degenerative dementia. There is now a consensus for the clinical diagnosis and the neuropathological aspects of these two diseases: Dementia with Lewy Bodies (Mc Keith et al., 1996) and fronto-temporal dementia (the Lund and Manchester groups, 1994). The authors emphasize the clinical aspects of those two diseases at an early stage in comparison with dementia of Alzheimer type.

We developed a disruptive behavior questionnaire designed for persons living close to the patient in order to assess behavior disorders in Alzheimer type dementia.The study included 111 patients with criteria for diagnosis of Alzheimer type dementia. The questionnaire was used to assess the patients 15 days after withdrawal of psychotropic drugs. A partially-structured interview was also conducted by a psychiatrist in 20 cases.There was no relationship between the different behavior disorders and the mini-mental-state score or the social and cultural situation. Answers to the questionnaire and responses given during the interview were nearly identical.There has been little effort to assess behavior disorders compared with cognitive disorders in patients with Alzheimer type dementia. Behavior assessment should be an integral part of patient evaluation since cognitive and non-cognitive disorders are independent and certain therapeutics may be effective in behavior disorders.

Central nervous system lesions resulting from sarcoidosis occur in 5% of cases, but supratentorial mass lesions are uncommon. We report 3 cases of intracranial pseudo-tumoral lesions, due to sarcoidosis: 1 woman and 2 men. Clinical features included left facial myoclonus, headache and vertigo, right hemiparesis and unique general seizure. Sarcoidosis was diagnosed upon conjonctival biopsy in the first case, infiltrative lesions of the lungs and mediastinal lymph nodes in the second case, and intracerebral lesion plus lymph nodes biopsies in the last case. In the first case, CT scan and MRI images showed diffuse subcortical high signal, suggesting pachymeningitis with vasogenic oedema. In the second case, GT scan and MRI revealed numerous small granuloma in the left rolandic area, and one in the striatum. In the third case, CT scan showed an enlargement of the left temporal horn, due to an enhanced left periventricular lesion. MRI with gadolinium showed that the lesion encircled the ventricle horn. In all cases, clinical and radiological improvement was obtained after corticoid therapy.

Between 1967 and 1994 48 patients have been treated at our institution for acute rupture. Two patients died before operation, in two cases the operative attempt had to be abandoned with irreversible cardiopulmonary arrest, and in 44 patients surgical correction could be performed, The rupture was complete in 14 cases and incomplete in 30. In 25 patients surgery consisted of direct suture, in one patient an aortoplasty was performed, and in 18 patients a dacron tube interposition was necessary. In 7 cases the clamp and repair technique was applied, and in 37 cases extracorporeal circulation was installed. The hospital mortality amounted to 18% and was mainly contributable to the concomitant injuries. Two patients presented with paraplegia at admission, both died; postoperative paraplegia occurred in two instances, once after 48 hours. The late results were in the majority of the cases influenced by orthopedic or neurological sequelae, only one patient had to be reoperated for a false aneurysm. Our results and the review of the literature lead to the following conclusions: In spite of an optimized emergency care system, many patients with aortic rupture die before any surgical intervention. The installation of extracorporeal circulation can decrease the incidence of postoperative paraplegia; in our experience the administration of heparin does not increase the risk of bleeding from concomitant injuries. Direct suture represents the correction of choice. The long-term results are excellent.

Central nervous system (CNS) neurosarcoidosis was considered to be infrequent. Clinical and radiological polymorphism explained the delay before diagnosis. With magnetic resonance imaging (MRI), diagnosis is more easily performed, especially in paucisymptomatic cases. A review of the most important clinical and radiological studies and three personal cases are reported. This underlines variable characteristics of hemispheric localizations, especially pseudotumoral forms.

Introduction Le 21 juillet 2009, l'article 51 de la loi HPST autorisait la cooperation interprofessionnelle avec transferts d'activites ou d'actes de soins, reorganisant le mode d'intervention aupres des patients. La telemedecine, pratique medicale a distance, utilise les technologies de l'information et de la communication pour renforcer le suivi des patients. Une etude multicentrique a evalue, sur 6 mois, la faisabilite d'une cooperation interprofessionnelle pour renforcer entre deux consultations conventionnelles de diabetologue, le suivi des patients diabetiques insulinotraites, munis d'un carnet glycemique electronique. Objectif 54 patients ont ete suivis par une equipe pluridisciplinaire par protocole de cooperation. Des infirmiers avec une formation adaptee posaient un diagnostic sur les resultats glycemiques teletransmis. Ils realisaient des actes derogatoires tels que l'ajustement des doses d'insuline, le traitement des accidents hypoglycemiques, hyperglycemiques. Pour repondre aux exigences legislatives, tous les actes etaient traces et evalues dans un logiciel (ePEP). Selon le niveau de gravite, l‘intervention etait para medicale ou medicale, engendrant ou pas des appels telephoniques. Resultats Chaque patient a beneficie de 16 telesurveillances. L'infirmier les a appeles en moyenne 2 fois. 11 teleconsultations medicales vers 13 % des patients, et 0,8 consultation physique ont ete realisees. Les patients ont stabilise leur HbA1c avec de rares hypoglycemies severes (seulement 2) et seuls 5 patients ont ete hospitalises pendant 4,8 jours (2 pour desequilibre glycemique). Il faut noter a l'inclusion une utilisation du carnet electronique depuis 20 mois. Cette prise en charge pluridisciplinaire a satisfait les patients, le score des questionnaires DQOL a augmente de 2,5 points pendant la periode de l'etude. Conclusion : Ce suivi des patients par protocole de cooperation avec des infirmiers realisant des actes derogatoires, selon l'article 51, ouvre la voie a une nouvelle prise en charge. Elle economise du temps medical, rare et cher, tout en permettant une stabilite glycemique poursuivie dans le temps sans cout supplementaire.

Introduction Dans le diabete de type 1 (DT1) traite par pompe S/C, il n'existe pas de recommandations precises sur l'ajustement des doses d'insuline en cas d'activite physique (AP). L'objectif de ce travail etait de valider des algorithmes d'adaptation des doses d'insuline, prenant en compte l'intensite, la duree de l'activite physique et son delai par rapport au repas. Patients et methodes Vingt patients DT1 sous pompe avec HbA1c Resultats Les 20 patients (H 10, IMC 24,5±3kg/m 2 , âge 47±11 ans, HbA1c initiale 7,9 %, duree d'evolution du diabete 18±9,5 ans, sous pompe depuis 5,1±4,2 ans), pratiquaient en moyenne 3,6h d'AP/sem. Tous ont fait l'integralite des epreuves basales et prandiales. Pour chaque patient, chaque type d'epreuve (base/prandiale) et chaque niveau d'AP, l'option d'ajustement qui n'entrainait pas d'hypoglycemie a ete identifiee et retenue pour l'elaboration d'algorithmes generaux d'ajustement, prenant en compte l'horaire de l'AP par rapport au repas, son intensite et sa duree. Conclusion Des algorithmes simples et personnalises d'adaptation des doses d'insuline ont pu etre definis permettant a des patients DT1 traites par pompe de pratiquer une AP de loisirs sans deteriorer leur equilibre glycemique ni augmenter leur risque d'hypoglycemie.

Introduction La telemedecine (TM) vise a definir une nouvelle organisation qui assure le maintien de la qualite des soins malgre une demographie medicale en baisse et une necessaire maitrise des couts. La loi HPST rend possible l’intervention de paramedicaux pour renforcer le suivi de patients aux schemas insuliniques complexes, selon un protocole de cooperation interprofessionnelle, definissant les modalites d’une delegation de tâche medecin/infirmier. L’objectif de l’etude ePEP est d’evaluer sur 6 mois, la faisabilite d’une telle delegation formalisee autour d’un plan d’education personnalise (PEP) electronique, pour le suivi de patients diabetiques equipes du carnet electronique DIABEO. Patients et methodes Le logiciel ePEP respectant un PEP a ete developpe, definissant precisement les tâches de l’infirmier et les niveaux d’intervention respectifs de l’infirmier et du medecin, en fonction d’alertes prealablement definies. Ce logiciel a ete utilise pour le suivi sur 6 mois de patients sous schema basal-bolus, utilisant deja pour la plupart le systeme DIABEO. Resultats Les 54 patients suivis dans 10 centres de diabetologie (DT1 96 %, H 39 %, pompe 54 %, âge 39 ± 12 ans, anciennete du diabete 18 ± 15ans, HbA1c initiale 8,0 ± 1,3 %) qui ont beneficie du suivi infirmier (16 telesurveillances/patient), n’ont eu que 42 consultations (CS) medicales pendant l’etude (0,8CS/patient) et 11 teleconsultations. Deux seulement ont ete hospitalises pour desequilibre glycemique. Au plan metabolique, l’HbA1c ne s’est pas deterioree ; l’incidence des hypoglycemies severes s’est averee extremement faible (2 au total), aucune n’ayant entraine d’hospitalisation. Les patients ont semble satisfaits de cette modalite de suivi (taux de satisfaction moyen mesure par questionnaire DQOL : +2,5 points en fin d’etude). Conclusion La cooperation interprofessionnelle medecin/infirmier formalisee a travers un PEP electronique, couple a un carnet electronique de type DIABEO, s’est averee non seulement faisable mais aussi efficace pour le maintien de l’equilibre glycemique avec economie de temps medical specialise et satisfaction des patients.

This 44-year woman was admitted for weight loss and global intellectual slowing. She had mild chronic alcoholic neuropathy. She was discontinued alcoholic consumption for 6 months and was given disulfiram (1.5 g/day) since then. She developed over a 5-day period acute neuropathy, confusion and extrapyramidal symptoms with oculo-cephalogyric and dystonic movements and myoclonus. Electromyography revealed a severe polyneuropathy. After disulfiram withdrawal, confusion and extrapyramidal symptoms disappeared within a few days, but sensitivo-motor deficit improved more slowly. Nerve biopsy was suggestive of a pure axonal neuropathy.

Propriospinal myoclonus is a rare and relatively little studied complication of spinal cord injury. We report two patients with an extension-producing myoclonus presenting with tetraplegia caused by cervical trauma. Rhythmic extension jerks of the trunk and lower limbs appeared several weeks after their injury in a context of severe spasticity. The characteristics of these jerks were determined by polymyography of 12 muscles. They lasted between 306 and 1127 ms with a frequency of 0.3 to 0.5 Hz. By comparing latencies their origin was found to be in the lumbar cord from which there was a slow (2 m s-1) upward and downward spread. Oral treatment with baclofen and sodium valproate was partially successful in one patient, but ineffective in the other. Intrathecal 75 or 100 micrograms baclofen produced a striking, complete disappearance of myoclonus prompting the implantation and successful use of a baclofen pump in one patient. These two new cases suggest the existence of a lumbar generator in which myoclonic extension jerks originate, and demonstrate a new therapeutic alternative in intrathecal baclofen for patients resistant to oral medication.

A 33-year-man with an encephalopathy of unknown aetiology, had an history of epilepsia for 30 years. Different types of seizures were seen, including grand mal and frontal attacks. Epilepsia was associated with mental retardation and behavioral disorders. At the age of 33, he was admitted for repetitive general convulsions. Epilepticus status lasted for two weeks and improved with vigabatrin et clonazepam. General seizures, frontal motor convulsions with arms and trunk antepulsion, and dacrystic attacks were seen. The latter seemed to be like normal crying because they were accompanied by lacrimation, contorted and mournful facies, and sobbing sounds. One year later, repetitive cardiac arrests occurred during a new epilepticus status. Cardiac arrests, observed on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns included theta and delta activity with rhythmic slow wave epileptic activity, predominating on right side, in temporal areas. CT scan was normal. MRI showed right cerebral atrophy, prevailing in the temporo-mesial region, with right temporal horn enlargement. This case report of dacrystic seizures, the first one with MRI study, suggests that temporo-mesial structures of the non-dominant hemisphere may be involved in dacrystic and asystolic attacks.

A 66 year-old man presented with a myelitis associated with eosinophilia in blood and cerebrospinal fluid. Specific serological procedures in blood and stool examinations led to the diagnosis of myelitis due to Strongyloides stercoralis. Physiopathology of medullary lesions is discussed. To our knowledge, this is the first report of myelitis in the course of Strongyloides infection.

Introduction De part son expansion mondiale, d’allure « epidemique » liee aux modifications du mode de vie, la prevention du diabete de type 2(DT2) sera une des preoccupations majeures des systemes de sante dans l’avenir. De caractere familial, les futurs DT2 sont les enfants de DT2 (prevalence de 20–30%). Il est urgent de disposer d’outils predictifs. Nous proposons d’etablir une equation de risque de survenue du DT2, a partir du genotypage des genes connus a risque transmis par les parents, combines a des elements simples (habitudes alimentaires, activite physique, diabete gestationnel). Patients et methodes Nous devons collecter, dans une etude multicentrique, les donnees genetiques et cliniques sur 2 generations adultes comportant au moins un diabete diagnostique par generation (1 000 cas). La fratrie non atteinte servira de temoins (2 000 cas). Au CERITD, promoteur de l’etude, en collaboration avec l’investigateur principal, une infirmiere de recherche sera en charge d’organiser le recrutement et la motivation des familles, de les identifier dans la base de donnees du service, de les contacter, de coordonner les autres centres, d’organiser les rendez-vous, de realiser les enquetes retrospectives (en collaboration avec des enqueteurs genetiques), et d’effectuer les prelevements, tout en respectant les bonnes pratiques cliniques. L’infirmiere recherche sera disponible via un numero vert pour repondre aux questions des patients et les accompagner dans le recrutement de leur famille. Resultats La validation d’une telle equation debouchera sur la realisation d’une biopuce ciblant les genes a risque, couple a un algorithme impliquant des parametres cliniques simples permettant de fournir un risque chiffre de diabete a venir chez les enfants de diabetiques et permettre la mise en place de mesure de prevention hygieno-dietetique voire pharmacologique precoces et prolongees. Conclusion La reussite de Descendance repose sur la rigueur organisationnelle du circuit patient, de la coordination entre les differents centres pour eviter les deperditions de patients et renforcer la fiabilite des donnees.

Central serotonin depletion may contribute to the anxiety, restlessness, irritability, and affective disturbance seen in a variety of psychiatric conditions, particularly dementia of the Alzheimer's type (DAT) in which brain concentrations of both 5-hydroxytryptamine (5-HT) and its 5-hydroxyindoleacetic acid (5-HIAA) metabolite are reduced.Trazodone, a serotonergic antidepressant with alpha 2-adrenergic blocking activity, was administered to 13 patients with DAT in an open 10-week pilot study at a dose of 25 mg t.i.d. Behavioral and affective disturbance was assessed pretreatment and posttreatment using semistructured interview and Jouvent's Depressed Mood and Gottfries-Brane-Steen scales.Irritability, anxiety, restlessness, and affective disturbance were all decreased (p.05). No side effects were observed. Mean Mini Mental State scores were unaffected by treatment.The hypothesis that trazodone corrects behavioral and affective disturbance induced by serotonin depletion in DAT requires confirmation in a double-blind placebo-controlled trial.

We report two cases of Marchiafava-Bignami disease studied by CT and MRI. Both patients displayed persistent confusion and a disconnection syndrome but had a favourable outcome. In both cases, CT with intravenous contrast medium revelaed enhancement of the corpus callosum in the early stage. MRI showed gadolinium uptake in both the genu and splenium of the corpus callosum in one case, 8 days after the onset. Three weeks after admission, cystic lesions appeared in the corpus callosum on MRI T1-weighted sagittal images in both cases. Contrast medium uptake may be useful in the diagnosis of Marchiafava-Bignami disease.

Some 176 consecutive carotid endarterectomies performed during 1987 were assessed after 11.5 and 44 months. There were four perioperative deaths. At mean follow-up of 32.5 months the 50% restenosis rate progressed from 9.7% to 11.9%. Of the 168 carotid arteries with a normal patency at discharge, 36 showed progression of stenoses as judged by duplex scanning during the observation period of 44 months. Twenty-nine stenoses were present within 1 year and seven developed between 12 and 44 months. Successive assessments revealed marked differences in the evolution of restenoses which usually depended on the degree of severity reached at the end of the first year: 44% progressed, 28% regressed and only 28% remained stable. The risk of late occlusion in vessels with a50% restenosis at 1 year was below 1% and the risk of progressing to a stenosis50% was 3.3%. Assessment at 56 months was limited to patients who had a restenotic lesion during the first 44 months. It confirmed that the disease was still unstable with progression in 7% of cases and regression in 10%. This study did not demonstrate any significant restenosis after 20 endarterectomies using the eversion technique compared with an incidence of 13.4% after 156 standard endarterectomies.

In patients with Alzheimer's disease, morphological neuroimaging techniques usually reveal signs of global cerebral atrophy which gradually worsen over time and depends on age and severity of the cognitive decline. Because of the lack of artifacts and of a more appropriate angle, magnetic resonance imaging scans may visualize a prominent atrophy of the medial temporal lobes, including hippocampal structures. Hippocampal atrophy is relatively specific of Alzheimer's disease before 65 and is related to the severity of memory disorders. White matter changes in patients without cerebrovascular risk factors are not more severe in patients with presenile Alzheimer's disease than in age-matched controls. They are, however, more severe in patients with senile onset than in age-matched controls. These findings suggest that white matter changes in patients with senile onset are consistent with a diagnosis of Alzheimer's disease.

Huntington's disease is a hereditary disease with autosomal dominant transmission which generally occurs in adults. The gene was discovered in 1983 and the genetic abnormality in March 1993. The most commonly recognized clinical manifestation is choreiform movements although other signs often appear more invalidating to family and friends. Cognitive decline, modifications in behaviour, and sometimes psychiatric disturbances are perceived as the major handicap in everyday life. In this review, emphasis has been placed on late onset forms of the disease. Recent research has focused on morphologic and functional imagery, the neuropathologic stages of selective neurone loss beginning in the striatum, and the role of excitotoxic amino-acids. Certain ethical considerations must be addressed when determining prognosis. Predictive tests and follow-up must be prepared and conducted scrupulously and proposed to voluntary, informed, major subjects at risk. Tests should be performed by a qualified laboratory working on anonymous samples, independent of the clinical team, and should be given to the subject orally by a genetic counsellor. A prenatal test may be requested by parents at risk. The question of treatment, which to date can only offer symptomatic relief, should be re-addressed in light of the recent discovery of the mutation published in March 1993.