[Sheehan's syndrome: an erratic diagnosis in the early postpartum]. / Le syndrome de Sheehan: un diagnostic difficile en post-partum immédiat.

Post-partum pituitary necrosis (Sheehan's syndrome) is a rare complication of the post-partum haemorrhage. The diagnosis can be erratic and often delayed. We report the case of a patient who had headaches and meningitis signs few hours after a post-partum haemorrhage. Magnetic Resonance Imaging (MRI) performed at day 2 showed a specific hypophysitis. The onset of asthenia, loss of weight, polyuro-polydipsy, persistent amenorrhoea and absence of lactation led to hormonal investigation. This permitted to diagnose global antehypopituitarism associated with diabetes insipidus. Progressive pituitary atrophy due to necrosis was found using MRI follow-up over 1 year. Our report summarises pathophysiological features of Sheehan's syndrome and early clinical and biological signs are discussed. MRI of the sellar region may be useful to early suspect the diagnosis.

[Analysis of 12 cases of McArdle's disease diagnosed after 30 years]. / Maladie de MacArdle diagnostiquée après 30 ans : à propos de 12 cas.

PURPOSE: McArdle's disease (MAD) or glycogen storage disease type V, usually starts in childhood or adolescence. Generally diagnosis is made before the early adulthood because patients present well defined syndrome and are constrained. METHOD: We retrospectively investigated all MAD cases diagnosed in the biochemical laboratory from Debrousse Hospital in Lyon, during 40 years (1962-2002). We then selected patients whose diagnosis had been made after 30 years. RESULTS: Fifteen patients answered our criteria but only 11 files could be analysed. A twelfth patient (service of internal medicine--Royan) supplemented the series. We sought the reasons of a late diagnosis: early age of beginning but few symptoms (7 cases), age of beginning higher than 20 years (5 cases including 3 after 45 years). The principal symptoms were muscular deficit and muscular pains (8 cases) and second wind phenomenon (7 cases). Creatinine phosphokinase level was constantly high. Ischemic effort test when it was carried out was constantly abnormal. Conversely electromyogram was often normal (5 cases). Several biopsies were necessary in a third of the cases to evoke the diagnosis, particularly among the patients with late onset symptoms. CONCLUSION: Diagnosis of metabolic MAD is generally easy if the interrogation finds inaugural symptoms in childhood or adolescence even if the patient consults very late in the life. The diagnosis can become much more difficult if it begins late in life (atypical symptoms, need for several muscular biopsy).

Are semantic errors actually semantic?: Evidence from alzheimer's disease.

Patients with Alzheimer's disease (AD) produce a high rate of semantic errors when naming to confrontation. This is considered to be one of the many consequences of their semantic memory deficit. However, it has been shown, in aphasic patients with focal lesions, that semantic errors could arise from impairment to any one of the levels in the naming process. To check this hypothesis in AD, we assessed in 15 patients the capacity to name and access semantic knowledge (by multiple-choice probe questions) about 14 objects presented successively in the visual, tactile, auditory, and verbal modalities. In the visual naming task, 33 errors were recorded: 26 (78.8%) were semantic and 7 (21.2%) were unrelated errors. Of the 26 semantic errors, 8 were related to a deficit of the semantic knowledge related to the item and 17 to a deficit in the retrieval of the phonological form of the word. One was associated with a deficit of access to semantic knowledge in the visual modality. The 7 unrelated errors were associated with a loss of semantic knowledge for 4 and deficit of access to the phonological form for 3. In conclusion, this study shows that semantic errors do not systematically reflect a deficit of semantic knowledge in Alzheimer's disease. It also seems that unrelated errors are more frequently related to semantic deficits than semantic errors in this population.

Superior written over spoken picture naming in a case of frontotemporal dementia.

Two main hypotheses have been proposed regarding the role of phonology in written word production. According to the phonological mediation hypothesis, the retrieval of the lexical phonological representation of a word is an obligatory prerequisite to the retrieval of its spelling. Therefore, deficits to the phonological lexicon should affect both spoken and written picture naming. In contrast, the orthographic autonomy hypothesis posits that the lexical orthographic representations of words can be accessed without any necessary phonological mediation. In support of this view, cases of preserved written naming despite impaired lexical phonology have been reported following brain damage. In this report, we replicate this basic pattern of performance in case YP, a 60-year-old woman with a pattern of frontotemporal dementia. As her disease progressed, YP's ability to write down the names of pictures remained very good despite a severe decline in oral naming. Further testing indicated that this deficit was not primarily due to an articulatory or post-lexical phonological deficit. YP's case provides strong additional support for the orthographic autonomy hypothesis. The significance of this case with respect to the characterization of dementia syndromes is discussed.

Executive/attentional performance and measures of schizotypy in patients with schizophrenia and in their nonpsychotic first-degree relatives.

Previous studies of executive/attentional functions have found impairments in nonpsychotic first-degree relatives of patients with schizophrenia. The aims of this study were: (1) to replicate these findings by three laboratory measures of attention/information processing - a continuous performance test (DS-CPT), a forced-choice span of apprehension task (SPAN), and a digit symbol substitution test (DSST), and by a series of neuropsychological tests sensitive to prefrontal cortical damage - Trail Making A and B, verbal fluency (VFT), Stroop Color and Word Test (Stroop), and Wisconsin Card Sorting Test (WCST); (2) to investigate whether such executive/attentional deficits are associated with schizotypal traits assessed using the social anhedonia, physical anhedonia, perceptual aberration and magical ideation scales (Chapman, L.J., Chapman, J.P., Raulin, M.L. 1976. Scales for physical and social anhedonia. J. Abnorm. Psychol. 85, 374-382; Chapman, L.J., Chapman, J.P., Raulin, M.L., 1978. Body-image aberration in schizophrenia. J. Abnorm. Psychol. 87, 399-407; Eckblad, M., Chapman, L.J., 1983. Magical ideation as an indicator of schizotypy. J. Consult. Clin. Psychol. 51, 215-225). In both patient and relative groups, performance was significantly poorer on the DSST, VFT and Trail B, and the reaction time on the SPAN was significantly longer. These neuropsychological impairments were present as much in siblings as in parents of schizophrenic patients; age did not appear to cancel differences between the relative and control groups. In the relative group, the four scores of schizotypy were at an intermediate level between those of patient and control groups, and the social anhedonia and perceptual aberration scores tended to be significantly different between the relative and the control groups. Only two significant correlations were found between the neuropsychological performance and the measures of schizotypy.

Working memory functioning in medicated Parkinson's disease patients and the effect of withdrawal of dopaminergic medication.

Cognitive impairments in Parkinson's disease (PD) could be explained by a central executive (CE) deficit in A. D. Baddeley's (1986) working memory model. To test this hypothesis, verbal, spatial, and double span tasks were given to 12 medicated PD patients and control participants, with varying recall delays. The double span task was assigned to explore the coordinating and integrating function of the CE, and lengthening the recall delay was expected to implicate more attentional resources. PD patients had lower spans relative to controls in all tasks. However, the more specific implication of the CE was difficult to prove. One reason could be that PD patients were on dopaminergic treatment when tested. To control this effect, 12 PD patients on and off medication were studied in a second experiment using the same tasks. PD patients off medication had lower spans only in the double task; this result underlines the role of dopamine on working memory processes.

Identification without manipulation: a study of the relations between object use and semantic memory.

The role of semantic knowledge in object utilisation is a matter of debate. It is usually presumed that access to semantic knowledge is a necessary condition for manipulation, but a few reports challenged this view. The existence of a direct, pre-semantic route from vision to action has been proposed. We report the case of a patient with a disorder of object use in everyday life, in the context of probable Alzheimer's disease. This patient was also impaired when manipulating single objects. He showed a striking dissociation between impairment in object use and preserved capacity to perform symbolic and meaningless gestures. To elucidate the nature of the disorder, and to clarify the relations between semantic knowledge and object use, we systematically assessed his capacity to recognise, name, access semantic knowledge, and use 15 common objects. We found no general semantic impairment for the objects that were not correctly manipulated, and, more importantly, no difference between the semantic knowledge of objects correctly manipulated and objects incorrectly manipulated. These data, although not incompatible with the hypothesis of a direct route for action, are better accommodated by the idea of a distributed semantic memory, where different types of knowledge are represented, as proposed by Allport (Allport, D. A. Current perspectives in dysphasia, pp. 32-60. Churchill Livingstone, Edinburgh, 1985).

Cognitive executive dysfunction in patients with obstructive sleep apnea syndrome (OSAS) after CPAP treatment.

We have previously described impairments of cognitive executive functions in 17 patients with OSAS in comparison with 17 normal controls, as assessed by various frontal-lobe-related tests. In the present study, 10 of these OSAS patients treated with continuous positive airway pressure (CPAP) were reevaluated after 4-6 months of treatment. Neuropsychological tasks explored attention, short-term memory span, learning abilities, planning capacities, categorizing activities, and verbal fluency. Patients were found to have normalized most of their cognitive executive and learning disabilities, but all the short-term memory tests remained unchanged. These findings are discussed in light of the contribution of the frontal-lobe-related systems to short-term memory functions, and the eventual pathogenic role played by sleep fragmentation and nocturnal hypoxemia, which are related to the occurrence of apneic and hypopneic events. In conclusion, short-term memory impairment was persistent in OSAS patients despite CPAP treatment for 4-6 months.

[Absence of frontal lobe dysfunction and working memory deficits in young schizophrenic patients]. / Absence de dysfonctionnement frontal et de trouble de la mémoire de travail chez de jeunes schizophrènes.

The impairment in tasks requiring intact frontal lobe functions has been repeatedly shown in schizophrenics. However, the relative roles of confounding factors, like duration of the disease, social withdrawal, or antidopaminergic medication, are not clearly demonstrated. We studied the performance of 12 young active patients, with chronic residual schizophrenia that had recent onset, and 12 control subjects, with frontal lobe tests and with a battery designed to explore working memory. The results show normal performance in schizophrenia. The small number of patients does not allow definitive conclusions, but this study suggests that a frontal dysfunction may not be present early in the evolution of schizophrenia in active patients.

One or several semantic system(s)? Maybe none: evidence from a case study of modality and category-specific "semantic" impairment.

Following cerebral anoxia, EC a 55-year-old patient, exhibited a severe and clear-cut pattern of semantic impairments without general intellectual deficit or perceptual difficulty. EC demonstrated a complex neuropsychological picture including a massive visual agnosia and a complete lack of imagery, both of which involved all categories of objects (living and non living) and a category-specific word comprehension deficit limited to animal names. Findings are discussed in the light of the theoretical frameworks currently available in the area of neuropsychology. It is argued that neither the single nor the multiple view of semantics fully succeed in providing a satisfactory account of the data and a tentative interpretation of the whole pattern of impairment is proposed in the general framework of non abstractive conceptions of meaning.

The phonological loop in medicated patients with Parkinson's disease: presence of phonological similarity and word length effects.

OBJECTIVE: To test the verbal subsystem of Baddeley's working memory model (the phonological loop) in patients with Parkinson's disease. METHODS: Fifteen patients with idiopathic Parkinson's disease and 15 controls were tested with a span paradigm to assess the effects reflecting the functioning of the phonological loop: the phonological similarity effect (in verbal and visual presentation), and the word length effect (in visual presentation). RESULTS AND CONCLUSIONS: The patients did not show any dysfunction of the phonological loop, reflected by the presence of phonological similarity and word length effects, but had lower spans than controls. The implications of these results for the working memory model are discussed.

[Hypoglossal-facial anastomosis. A report of 60 cases]. / Anastomose hypoglosso-faciale. A propos de 60 cas.

The authors report their experience of 60 cases of hypoglosso-facial anastomosis. The results of this retrospective series were analysed by the same examiner according to the House and Brackmann classification. The surgical technique is rapidly described, with emphasis on the important points. The results are analysed as a function of the interval between the anastomosis and facial paralysis: better and more rapid results are obtained when surgery is performed early (80% of grade 3 with immediate surgery versus 50% in very late surgery after more than 4 years). However, grade 3 or 4 can be obtained in every case, even in the case of very late surgery. Other favourable prognostic factors were revealed by this study: specialized rehabilitation and especially the patient's psychological must be integrated in this nerve transfer. In view of these good results and the limited adverse effects (atrophy of the hemi-tongue, eye-mouth synkinesias), hypoglosso-facial anastomosis must be part of the therapeutic strategy of total, permanent facial paralysis.

[Marchiafava-Bignami disease: development with flare-up]. / Maladie de Marchiafava-Bignami: évolution par poussées.

Marchiafava-Bignami disease is a complication of chronic ethanol intoxication. The clinical course may be acute, rapidly leading to death, or marked by a progressive dementia. A form with favorable outcome is also described. During the evolution of chronic forms, acute worsenings are possible, with subsequent partial recovery. We report a clinico-radiological case, where a first episode was followed by a good outcome. After a second episode, 3 years later, the patient developed a dementia with predominant frontal lobe signs. This case suggests that, even after a favorable outcome, a long-term poor prognosis cannot be ruled out.

[Capillary and cavernous hemangioma disclosed by painful amyotrophy of the thigh]. / Hémangiome capillaire et caverneux révélé par une amyotrophie douloureuse de la cuisse.

We report the case of an intramuscular cavernous and capillary hemangioma of the thigh revealed by pains evolving for 2 years and by an amyotrophy. After 21 months of investigation, the surgical excision and the histological examination only established the dia Thigh MRI. T1-weighted axial and sagittal sections with gadolinium infusion.

Working memory in medicated patients with Parkinson's disease: the central executive seems to work.

OBJECTIVE: To determine whether a deficit of the central executive can explain the attentional deficits of patients with Parkinson's disease. METHODS: Fifteen patients with idiopathic Parkinson's disease and 15 controls were given a dual task paradigm minimising motor demands and combining verbal, visual, or spatial span with two conditions of articulatory suppression. RESULTS: Although the spans were systematically lower in medicated parkinsonian patients than in controls, suggesting a decrease of central processing resources, there was no direct evidence for a deficit of the central executive. CONCLUSIONS: A deficit of the central executive either is not an inevitable feature of the disease, or is dependent on the nature of task (visuomotor v cognitive), or is corrected by dopaminergic medication.

[Frontal lobe dysfunction and depressive state: relation to endogenous character of depression]. / Dysfonctionnement frontal et état dépressif: relation avec le caractère endogène de la dépression.

A dysfunction of dorsolateral prefrontal cortex (DLPF) in major depression is suggested by functional imagery and comparative neuropsychology. However, assessment of frontal lobe syndrome with DLPF-dependent tests led to controversial results. To clarify these findings, we administered 5 of these tests (Wisconsin Card Sorting Test, Stroop Test, Trail Making Test, Tower of Toronto, verbal fluency) to 16 major depressive subjects and their 16 controls, before and after 21 days of treatment. Furthermore, we tried to assess the prognostic value of frontal lobe dysfunction, and its relation with the endogenous or exogenous nature of the depression on the one hand, the severity of the depression on the other hand. Our results suggest that the presence of a frontal lobe syndrome (defined by impaired performances at 3 tests or more) is only noted in endogenous depression; after treatment, no impairment is detected. No correlation is found with the severity of the depression. Frontal lobe syndrome does not seem to indicate poorer prognosis for current depressive episode.

Calculation and number processing in mild Alzheimer's disease.

Calculation and number processing abilities in 17 patients suffering from a mild form of dementia of Alzheimer's type (DAT) were studied by means of a standardized multitask assessment battery, the EC301 (Deloche et al., 1994). Patients were selected from a larger sample by using a specific visuo-perceptive task to control the confounding effects of deficits in analysing digit serial order. Language and memory skills were evaluated by means of standardized testing procedures. The EC301 overall score showed impaired performance in 12 cases. Calculation and number processing scores were highly correlated with Mini-Mental State Examination and language performance. However, multiple single-case analyses indicated heterogeneous patterns of preserved/impaired abilities with respect to the three cognitive areas under investigation (calculation, memory, and language) and to the different components of the calculation and number processing system.

[Deficiency in the reproduction and learning proper names after left tubero-thalamic ischemic lesion]. / Déficit de la production et de l'apprentissage des noms propres après lésion ischémique tubéro-thalamique gauche.

Predominant impairment or preservation of category-specific naming and comprehension is not rare in aphasics. Much less frequent is a selective inability to generate proper names. To our knowledge, only one such case has been reported after a left thalamic lesion, located in the ventral anterior nucleus, the mamillo-thalamic tractus and the genu of the internal capsule. We report a new case of selective inability to generate proper names after a left tubero-thalamic infarct. A 65-year old right-handed man presented with a selective impairment in producing proper names, both from photographs or descriptions and on tests of verbal fluency. The deficit was obvious both for persons names and for geographical names. The rest of the neuropsychological testing was remarkable only for a mild verbal amnesia, affecting only serial material (list of words), a reduced fluency for flowers, fruits and musical instruments, difficulties in learning of new words, and a dissociation between preserved learning for words on a test of learning of words and occupations (Cohen, 1990). This anomia for proper names could result from an indirect frontal-lobe dysfunction, preventing voluntary activation of the phonological representation of proper names.

Deficits of cognitive executive functions in patients with sleep apnea syndrome.

Impairment of cognitive executive functions previously has been suspected to occur in association with sleep apnea syndrome (SAS), as suggested by some neuropsychological studies. However, such functions have not been assessed directly. In the present study, 17 patients with SAS were evaluated with various focused frontal lobe-related tests in comparison with 17 normal controls. Such tasks explored attention, short-term memory spans, learning abilities, planning and programming capacities, categorizing activities and verbal fluency. Patients were found to have a significantly decreased ability to initiate new mental processes and to inhibit automatic ones, in conjunction with a tendency for preservative errors. They were also affected with deficits of verbal and visual learning abilities and had reduced memory spans. Such defects were further evaluated via logistic regression against two criteria of the severity of the disease: the number of apneas and hypopneas per hour of sleep and the level of nocturnal hypoxemia. Memory deficits were rather related to the former, whereas typical frontal lobe-related abnormalities seemed rather consistent with the latter. These findings are discussed in light of data from the literature concerning cognitive impairments described for patients with isolated daytime sleepiness versus hypoxemia, as illustrated in other pathological or physiological circumstances.