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Biomedical subjects

S Schaeffer

Publications and source records attributed to S Schaeffer.

At least 19 recordsLinked to original sources

Disorders in intentional gestural organization in Alzheimer's disease: combined or selective impairment of the conceptual and production systems?

This study examines disorders which affect the intentional gestural organization in Alzheimer's disease. An original and prospective protocol which assessed both the conceptual and production systems (with reference to the Roy and Square model 1985) was presented to 16 patients with Alzheimer's disease (mild to moderate dementia) and 40 healthy elderly subjects. Our study showed impairment of both systems in the Alzheimer's patients group. Two patients presented reverse performance profiles, which were characterized in one case by preservation of the conceptual system and impairment of the production system and in the other by the reverse dissociation. The findings demonstrated that disorders in the intentional gestural organization in Alzheimer's disease usually affect both the conceptual and production systems. The hypothesis concerning the existence of two functionally distinct systems underlying the organization of intentional body movement was corroborated (1) by a lack of significant correlation between the scores obtained in the assessment of the two systems and (2) by the demonstration of the double dissociation.

Aged↗

Approaches to the sellar and parasellar region: anatomic comparison of endonasal-transsphenoidal, sublabial-transsphenoidal, and transethmoidal approaches.

Various surgical approaches have been described for treating lesions which arise in the sellar region. The popular transnasal-transsphenoidal and transethmoidal-transsphenoidal approaches avoid the morbidity associated with transcranial operations while allowing enough exposure to successfully remove most lesions that arise in and around the sella. The goal of the present study was to accurately quantify the amount of exposure to the sellar and suprasellar region that the transethmoidal-transsphenoidal, endonasal-transphenoidal, and sublabial-transsphenoidal approaches provide. Each of the three approaches was performed on 14 formalin fixed heads with the aid of the operative microscope. The distances of relevant surgical landmarks, and the amount of exposure superior and anterior to the dorsum sella as well as the lateral exposure obtained through each of these approaches was carefully recorded. The mean distances were then used to calculate the volume of exposure offered by each approach. It was found that the sublabial-transsphenoidal approach afforded the greatest volume of exposure superior and anterior to the dorsum sella. The endonasal-transsphenoidal and the transethmoidal approaches were both found to offer less suprasellar volume exposure, with the transethmoidal approach offering the least. The authors believe the information obtained through this study illustrates some important anatomical relationships which can be used to advantage by the surgeon to tailor the most appropriate approach, depending upon the precise location of the lesion either within the sella or suprasellar region.

Brain Neoplasms↗

Nitrous oxide anesthesia-associated myelopathy.

BACKGROUND: The role of nitrous oxide exposure in neurologic complications of subclinical cobalamin deficiency has been reported, but few cases are well documented. OBSERVATION: Two weeks after surgery for prosthetic adenoma, a 69-year-old man developed ascending paresthesia of the limbs, severe ataxia of gait, tactile sensory loss on the 4 limbs and trunk, and absent tendon reflexes. After a second surgical intervention, the patient became confused. Four months after onset, the patient had paraplegia, severe weakness of the upper limbs, cutaneous anesthesia sparing the head, and confusion. Moderate macrocytosis, low serum B12 levels, and a positive Schilling test result led to the diagnosis of pernicious anemia. Results of electrophysiologic examinations showed a diffuse demyelinating neuropathy. Magnetic resonance imaging of the spinal cord disclosed hyperintensities of the dorsal columns on T2-weighted images. CONCLUSIONS: Pernicious anemia can result in severe neurologic symptoms with only mild hematologic changes. The role of nitrous oxide anesthesia in revealing subclinical B12 deficiency must be emphazised. Magnetic resonance imaging of the spinal cord might be helpful in making the diagnosis.

Adenoma↗

Patient delay in seeking care for heart attack symptoms: findings from focus groups conducted in five U.S. regions.

BACKGROUND: Patient delay in seeking health care for heart attack symptoms is a continuuing problem in the United States. METHODS: Investigators conducted focus groups (N = 34; 207 participants) in major U.S. regions (NE, NW, SE, SW, MW) as formative evaluation to develop a multi-center randomized community trial (the REACT Project). Target groups included adults with previous heart attacks, those at higher risk for heart attack, and bystanders to heart attacks. There were also subgroups reflecting gender and ethnicity (African-American, Hispanic-American, White). FINDINGS: Patients, bystanders, and those at higher risk expected heart attack symptoms to present as often portrayed in the movies, that is, as sharp, crushing chest pain rather than the more common onset of initially ambiguous but gradually increasing discomfort. Patients and those at higher risk also unrealistically judge their personal risk as low, understand little about the benefits of rapid action, are generally unaware of the benefits of using EMS/9-1-1 over alternative transport, and appear to need the "permission" of health care providers or family to act. Moreover, participants reported rarely discussing heart attack symptoms and appropriate responses in advance with health care providers, spouses, or family members. Women often described heart attack as a "male problem," an important aspect of their underestimation of personal risk. African-American participants were more likely to describe negative feelings about EMS/9-1-1, particularly whether they would be transported to their hospital of choice. CONCLUSIONS: Interventions to reduce patient delay need to address expectations about heart attack symptoms, educate about benefits and appropriate actions, and provide legitimacy for taking specific health care-seeking actions. In addition, strategy development must emphasize the role of health care providers in legitimizing the need and importance of taking rapid action in the first place.

Adaptation, Psychological↗

The 'Heart Attack Survival Kit' project: an intervention designed to increase seniors' intentions to respond appropriately to symptoms of acute myocardial infarction.

The purpose of this study was to test the effectiveness of a 'Heart Attack Survival Kit', disseminated via two different delivery methods, designed to increase seniors' intentions to call 911 and take an aspirin in response to a cardiac emergency. Twelve-hundred seniors were randomly assigned to (1) receiving a Kit via a home visit by an Emergency Medical Technician (EMT), (2) receiving a Kit via direct mail or (3) a control group. All participants were telephoned and asked how they would respond to a cardiac emergency. Results showed that respondents in the intervention group (EMT and direct mail group combined) reported a greater frequency of the recommended coping response to AMI (39%) than respondents in the control group (10%) (P < 0.000). Within intervention groups, 47% in the EMT group and 30% in the direct mail group (P < 0.000) reported intentions to take the appropriate response to AMI. The results suggest that a Heart Attack Survival Kit, especially when delivered door-to-door by EMTs, can be an effective way of educating seniors about cardiac emergencies.

Aged↗

Carvedilol prevents remodeling in patients with left ventricular dysfunction after acute myocardial infarction.

OBJECTIVE: The aim of the study was to assess the effects of carvedilol, a vasodilating nonselective beta-blocker, on the indexes of left ventricular remodeling after acute myocardial infarction in those with left ventricular dysfunction. METHODS AND RESULTS: Forty-nine patients with predischarge left ventricular ejection fraction <45% after acute myocardial infarction were evaluated in a double-blind, randomized, placebo-controlled, parallel group study (selected from the database of the Carvedilol Heart Attack Pilot Study: CHAPS). Patients received medication after thrombolysis and continued to do so for 6 months. Two-dimensional echocardiography was performed before discharge (7 to 10 days after admission) and at 3 months after acute myocardial infarction. Analysis of variance showed that wall thickness opposite the site of infarction decreased from (mean +/- SD) 12.3 +/- 2.1 mm to 11.0 +/- 2.4 mm with carvedilol compared with 11.6 +/- 1.9 mm to 12.2 +/- 1.9 mm with placebo (P =.01). Left ventricular mass changed from 235 +/- 74 g to 217 +/- 64 g with carvedilol compared with 227 +/- 80 g to 252 +/- 85 g with placebo ( P =.02). Carvedilol prevented alteration of sphericity index (ratio of long and short axis of left ventricle) that changed from 1.65 +/- 0.29 to 1.66 +/- 20 with carvedilol compared with 1.58 +/- 0.33 to 1.39 +/- 0.19 with placebo (P =.02); alteration was also prevented of wall thickening abnormality at infarct site, which changed from 9.2 +/- 3.1 cm2 to 9.1 +/- 3.5 cm 2 with carvedilol compared with 10.3 +/- 3.3 cm2 to 13.5 +/- 4.6 cm2 with placebo (P =.002). CONCLUSION: Carvedilol administered early after acute myocardial infarction results in attenuation of left ventricular remodeling in patients with persistent left ventricular dysfunction before discharge.

Adrenergic beta-Antagonists↗

Episodic memory in transient global amnesia: encoding, storage, or retrieval deficit?

OBJECTIVES: To assess episodic memory (especially anterograde amnesia) during the acute phase of transient global amnesia to differentiate an encoding, a storage, or a retrieval deficit. METHODS: In three patients, whose amnestic episode fulfilled all current criteria for transient global amnesia, a neuropsychological protocol was administered which included a word learning task derived from the Grober and Buschke's procedure. RESULTS: In one patient, the results suggested an encoding deficit, and in two others, a storage deficit. CONCLUSIONS: The encoding/storage impairment concerning anterograde amnesia documented in our patients stands in clear contrast with the impairment in retrieval which must underly the retrograde amnesia that also characterises transient global amnesia. This dissociation in turn favours the idea of a functional independence among the cognitive mechanisms that subserve episodic memory.

Aged↗

Cluster headache-like attack as an opening symptom of a unilateral infarction of the cervical cord: persistent anaesthesia and dysaesthesia to cold stimuli.

A 54 year old man experienced excruciating left retro-orbital pain with lacrimation and redness of the eye representative of a cluster headache attack. This was followed by left hemiparesis with plegia of the lower limb and left Horner's syndrome. Five days later the hemiparesis recovered while the patient developed hypoanaesthesia to cold stimuli that evoked painful burning dysaesthesia on the right side below the C4 level. MRI disclosed a discrete infarct in the left lateral aspect of the cord at C2 level concomitant to a left vertebral artery thrombosis. This limited infarct and the clinical symptoms suggest a hypoperfusion in the peripheral arterial system of the left hemicord, supplied both by the anterior and posterior spinal arteries. Cluster headache-like attack and persistent dysaesthesia to cold stimuli are discussed respectively in view of the central sympathetic involvement and partial spinothalamic system dysfunction.

Anesthesia↗

Axonal phenotype of Charcot-Marie-Tooth disease associated with a mutation in the myelin protein zero gene.

A French family had Charcot-Marie-Tooth disease type 2 (CMT2) which was characterised by late onset of peripheral neuropathy involvement, Argyll Robertson-like pupils, dysphagia, and deafness. Electrophysiological studies and nerve biopsy defined the neuropathy as axonal type. Genetic analysis of myelin protein zero (MPZ) found a mutation in codon 124 resulting in substitution of threonine by methionine. One of the patients, presently 30 years old, showed only Argyll Robertson-like pupils as an objective sign but no clinical or electrophysiological signs of peripheral neuropathy.

Adolescent↗

Paraneoplastic cerebellar syndrome and optic neuritis with anti-CV2 antibodies: clinical response to excision of the primary tumor.

OBJECTIVE: To describe a patient with a paraneoplastic cerebellar syndrome and optic neuritis with circulating anti-CV2 antibodies and clinical improvement after excision of a small cell lung carcinoma. DESIGN: Report of a case. SETTING: A 62-year-old man simultaneously developed a severe cerebellar syndrome and a bilateral optic neuritis predominantly in the left eye (visual acuity, 20/25 in the right eye; < 20/400 in the left eye; and bilateral swelling of the optic discs). MAIN OUTCOME AND RESULTS: Anti-CV2 antibodies, recently described as associated with paraneoplastic neurological syndrome, were detected in the patient's serum sample. These antibodies were demonstrated to react with the cytoplasm of a subpopulation of oligodendrocytes in the white matter of rat brain in the cerebellum, brainstem, spinal cord, and optic chiasm. The patient was found to have a small cell lung carcinoma, which was removed. After excision of the tumor, the cerebellar syndrome improved dramatically and the papilledema disappeared despite aftereffects of the optic neuritis. CONCLUSIONS: These findings were consistent with the diagnosis of a paraneoplastic neurological syndrome, although both optic neuritis and remission of the cerebellar syndrome are uncommon patterns of paraneoplastic syndromes. CV2 antigen expression by the oligodendrocytes of the cerebellum, brainstem, spinal cord, and optic chiasm correlated with the clinical syndrome observed in our patient. However, the precise pathophysiological role of anti-CV2 antibodies is still unknown.

Autoantibodies↗

[Multiple and unilateral paralysis of the cranial nerves revealing 2 cerebellar infarctions].

A 86 year old man suffered multiple palsies of the right V, VI, VII, VIII, IX and X cranial nerves preceded for several weeks by transient diplopia, facial palsy and vertigo. The CT scan and MRI showed two infarcts sitting in the territories of the right postero-inferior cerebellar artery and the right antero-inferior cerebellar artery. A cerebellar syndrome developed several days later. Although cranial nerves palsies are very commun following infarcts of the cerebellar arteries, their occurrence without other neurological deficit, especially cerebellar syndrome, seems to be rare.

Aged↗

[Multifocal motor neuropathies with conduction blocks. 39 cases].

Clinical, biological and electrophysiological features from a cohort of 39 multifocal motor neuropathies with conduction blocks (NMM with CB) have been studied. There were 29 males and 10 females with an average of 47.3. At the first evaluation, the mean duration of the symptoms was of 8 years with extremes between 1 and 28. Pain and paresthesias were present in respectively 10 and 18 p. 100 of the patients. Fasciculations and cramps were observed in more than 2/3 of the cases. Three patients had tremor at rest. Upper limb muscular weakness was the predominant initial symptom (84.6 p. 100). The weakness always affected distal and unilateral muscles. Radial and cubital nerve distribution are mainly affected and in half of the cases an unilateral motor deficit in the lower limb was associated. Muscle atrophy was frequent (74 p. 100) and rapidly developed in the first 2 years. Reflexes were decreased or absent in 64 p. 100. In 78 p. 100 of cases, biological study showed normal serum immunoelectrophoresis and CSF. IgM anti-GM1 antibodies were found in 24/36 patients. Very high titres were found in 5 cases. All patients had CB in upper limbs. The preferential localizations of the CB were equally at the median and ulnar nerves. Only 7 patients had CB localized to the lower limbs. In many cases, marked reduction of the motor amplitude prevented the detection of CB, marked reduction of the motor amplitude prevented the detection of CB. Moderate fibrillation potentials were found in 28 p. 100 of patients. Giant muscular unit potentials were frequent (21/39). F-waves in nerve with CB were always abnormal with marked increased latencies. Late responses sometimes seemed to be repeater F-waves. Axon reflexes were detected in 5 cases. The late responses abnormalities could precede the block. Clinical, biological and electrophysiological described arguments could may distinguish NMM with CB from motor neuron disease and relate them to the group of chronic demyelinating neuropathies.

Adult↗

[Clinical and cognitive study of apraxia in cortico-basal atrophy. A selective disorder of the production system].

The aim of this study was to characterize the different processes underlying apraxic disorders in cortico-basal degeneration. Three patients were submitted to a prospective paradigm elaborated in the theoretical framework proposed by Roy and Square (1985). This cognitive model postulates a two-steps system in the control mechanisms in limb praxis: the conceptual system and the production system. Our results, strictly similar for the three patients, showed a sparing of the conceptual system and an impairment of the production system with a dramatic lack in the control of the temporal and spatial aspects of the gestures. These results suggest a dysfunction of the premotor cortex including the supplementary motor area.

Aged↗

[Gesture apraxia disclosing carbon monoxide poisoning].

A seventy year old right handed woman presented progressive limb apraxia and headache due to carbon monoxide poisoning. Thereafter, when in hospital for one week, she developed akinetic mutism, coma and died. Limb apraxia has been rather uncommonly reported in carbon monoxide poisoning. The akinetic mutism observed after the patient had been removed from intoxication could be analysed as a delayed encephalopathy related to a chronic carbon monoxide poisoning.

Aged↗