Search PubMed⌕ Search

Biomedical subjects

M Verny

Publications and source records attributed to M Verny.

At least 37 records · Page 2Linked to original sources
PubMed1998

Retrospective application of a set of clinical diagnostic criteria for the diagnosis of multiple system atrophy.

We estimated the accuracy of a modified commonly used set of clinical diagnostic criteria for the diagnosis of multiple system atrophy (MSA) by retrospectively applying the criteria to the features recorded by six neurologists who had evaluated 105 autopsy-confirmed cases (16 MSA and 89 non-MSA disorders). Cases were abstracted from the records of the patients' first visit to an academic center, and were presented as clinical vignettes to six neurologists, each of whom recorded the main clinical features of the presented clinical vignette on a standardized form. Sensitivity and positive predictive values were chosen as validity outcome measures and were calculated by comparing the applied diagnostic criteria to the neuropathologic information. Of note, most MSA patients in this study (mainly those with Shy-Drager type) had not received levodopa therapy since the primary neurologists often had not perceived a need to administer this treatment. The validity of the retrospectively applied criteria for the diagnosis of possible MSA (sensitivity: median, 53%, range, 50-69%; positive predictive value: 30%, 28-39%) and probable MSA (sensitivity: 44%, 31-60%; positive predictive value: 68%, 54-80%) at the first visit was suboptimal. The best, still not perfect, accuracy for this set of diagnostic criteria was obtained when six out of eight features (sporadic adult onset, dysautonomia, parkinsonism, pyramidal signs, cerebellar signs, no levodopa response, no cognitive dysfunction, or no downward gaze supranuclear palsy) were present (median sensitivity, 59%; range, 50-75%; positive predictive value: 67%, 53-83%). This is the first study to validate criteria for the clinical diagnosis of MSA. Our data suggest that it is difficult to achieve an early and accurate clinical diagnosis of this disorder. The probability of correctly diagnosing MSA increases when at least six features of this modified set of criteria are present or when requiring the set for probable MSA.

Adult↗
PubMed1998

Accuracy of the clinical diagnosis of postencephalitic parkinsonism: a clinicopathologic study.

The accuracy of the clinical diagnosis of postencephalitic parkinsonism (PEP) is unknown. We determined the validity of the clinical diagnosis of PEP by presenting 105 records with neuropathologic diagnoses of PEP (n = 7), progressive supranuclear palsy (n = 24), Parkinson's disease (n = 15), dementia with Lewy bodies (n = 14), multiple system atrophy (n = 16), corticobasal degeneration (n = 10), Creutzfeldt-Jakob disease (n = 4), and other dementia disorders (n = 15), as clinical vignettes to six neurologists unaware of the autopsy findings. The neurologists' own clinical diagnoses were compared with neuropathologic diagnoses for measures of diagnostic accuracy, including reliability (kappa statistics), sensitivity and positive predictive values for the first and last visits. The group reliability for the diagnosis of PEP was almost perfect (kappa = 0.91, 0.9). The mean sensitivity at the first visit was 86% (range, 71-100%) with minimal change at the last visit (83%; range, 71-100%). Positive predictive values remained unchanged (100%). The high reliability, sensitivity and positive predictive values of the clinical diagnosis of PEP indicate that neurologists identify this disorder even when they report that they have never evaluated a case. In our data set, the best predictors for the diagnosis of PEP included onset below middle age; symptom duration lasting more than 10 years, and the presence of oculogyric crisis. History of encephalitis lethargica, present in most PEP cases, was an important individual diagnostic predictor. Copyright 1998 Lippincott Williams & Wilkins

Journal Article↗
PubMed1998

Ideomotor apraxia test: a new test of imitation of gestures for elderly people.

Ideomotor apraxia is one of the earliest disturbances in Alzheimer's disease (AD); no test of this disturbance has yet been validated for elderly people. We propose a test of meaningless gesture imitation and its relevance in elderly people is studied. The Ideomotor Apraxia Test (IAT) consists of showing 10 gestures. Each item is graded from 0 to 3. IAT was carried out on 55 patients with AD (mean age: 86.8 +/- 6.8 (71-100); mean MMS score: 14.6 +/- 6.3) and 26 elderly patients without cognitive impairment (mean age: 84.1 +/- 7.5 (70-100); mean MMS score: 27.5 +/- 1.9). The inter-rater reliability, a threshold, the apparent sensitivity and specificity and the relationship between the MMS score and the ideomotor apraxia score were determined. The mean apraxia score was 14.9 +/- 7.3 in the AD group and 28 +/- 1.6 in the normal group (P < 0.0001). Inter-rater agreement is excellent (P = 0.995). The threshold is 27. The apparent sensitivity and specificity are very good (95% and 88%, respectively). The correlation between the MMS score and ideomotor apraxia score is excellent (r = 0.83 (95% CI; 0.72-0.91). Age and educational level do not influence ideomotor apraxia score. IAT is the first test that evaluates meaningless gesture imitating capacity in an elderly population. It is an easy and quick-to-perform test, useful routinely for diagnosis of AD. It could contribute to the early diagnosis of AD and is correlated with the severity of cognitive impairment. Copyright 1998 Lippincott Williams & Wilkins

Journal Article↗
PubMed1998

Dementia with Lewy bodies.

The presence of a high number of Lewy bodies--the morphological marker of Parkinson's disease--in the cerebral cortex of some cases of dementia has been frequently observed in association to Alzheimer type lesions (mainly senile plaques) and changes in the substantia nigra, that may be held responsible for the frequently associated symptoms of parkinsonism. The term "dementia with Lewy body" (DLB) has recently been suggested by a consensus conference and indicates that the pathogenetic mechanism of the dementia remains poorly understood. Marked fluctuations of alertness and of the cognitive performances, moderate parkinsonism and episodes of visual hallucinations may lead to suspect this diagnosis in cases of dementia. Unexplained falls, syncopes, delirium or alterations of consciousness may also be observed, and the patients may then be admitted in departments of internal medicine or geriatrics. The Lewy body is an intraneuronal spherical inclusion, present in Parkinson's disease. It is observed in the brainstem (substantia nigra, locus coeruleus, dorsal nucleus of the Xth nerve) and in the nucleus basalis of Meynert. The cortical Lewy bodies have a different aspect, but retain their antigenic characteristics: they are, in particular, stained by the antiubiquitin antibodies. Recently, they were found to be also labeled by antisynuclein antibodies. A mutation of the synuclein gene was recently identified in cases of familial Parkinson's disease. Clinically as well as pathologically, DLB may thus be difficult to distinguish from Alzheimer's disease on the one hand, and from Parkinson's disease, on the other. That diagnosis, however, is associated with a poor prognosis and should lead to specific therapeutic measures.

Aged↗
PubMed1997

What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study.

BACKGROUND: The presentation of symptoms for multiple system atrophy (MSA) varies. Because there are no specific markers for its clinical diagnosis, the diagnosis rests on the results of the neuropathologic examination. Despite several clinicopathologic studies, the diagnostic accuracy for MSA is unknown. OBJECTIVES: To determine the accuracy for the clinical diagnosis of MSA and to identify, as early as possible, those features that would best predict MSA. DESIGN: One hundred five autopsy-confirmed cases of MSA and related disorders (MSA [n=16], non-MSA [n=89]) were presented as clinical vignettes to 6 neurologists (raters) who were unaware of the study design. Raters identified the main clinical features and provided a diagnosis based on descriptions of the patients' first and last clinic visits. METHODS: Interrater reliability was evaluated with the use of kappa statistics. Raters' diagnoses and those of the primary neurologists (who followed up the patients) were compared with the autopsy-confirmed diagnoses to estimate the sensitivity and positive predictive values at the patients' first and last visits. Logistic regression analysis was used to determine the best predictors to diagnose MSA. RESULTS: For the first visit (median, 42 months after the onset of symptoms), the raters' sensitivity (median, 56%; range, 50%-69%) and positive predictive values (median, 76%; range, 61%-91%) for the clinical diagnosis of MSA were not optimal. For the last visit (74 months after the onset of symptoms), the raters' sensitivity (median, 69%; range, 56%-94%) and positive predictive values (median, 80%; range, 77%-92%) improved. Primary neurologists correctly identified 25% and 50% of the patients with MSA at the first and last visits, respectively. False-negative and -positive misdiagnoses frequently occurred in patients with Parkinson disease and progressive supranuclear palsy. Early severe autonomic failure, absence of cognitive impairment, early cerebellar symptoms, and early gait disturbances were identified as the best predictive features to diagnose MSA. CONCLUSIONS: The low sensitivity for the clinical diagnosis of MSA, particularly among neurologists who followed up these patients in the tertiary centers, suggests that this disorder is underdiagnosed. The misdiagnosis of MSA is usually due to its confusion with Parkinson disease or progressive supranuclear palsy, thus compromising the research on all 3 disorders.

Aged↗
PubMed1997

[Multifocal motor neuropathy: an individualized disease of the peripheral nervous system].

Multifocal motor neuropathy is a peripheral nervous system disease described among chronic inflammatory demyelinating polyneuropathies. It is characterized according to both clinical criteria, including chronic asymmetric and multifocal deficit which starts and remains prominent in the upper limbs, and electrophysiological criteria, including persistent multifocal motor conduction blocks in motor nerves. High titers of serum antiganglioside GM1 antibodies are discovered in nearly 40% of cases. Steroids and plasma exchange are not efficient. High doses of intravenous immunoglobulins (i.v.Ig) improved symptoms in the majority of open and controlled published studies. The quality of the response to i.v.Ig may worsen in some patients after a variable number of infusions, leading to immunosuppressive treatments mainly with oral or intravenous cyclophosphamide. Its etiology is unknown but the frequent presence of anti-GM1 antibody high serum titers, the pathological findings in some rare morphological studies, and the response to i.v.Ig favor the hypothesis of an autoimmune disorder.

Demyelinating Diseases↗
PubMed1997

Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders? A clinicopathological study.

The difficulty in differentiating progressive supranuclear palsy (PSP, also called Steele-Richardson-Olszewski syndrome) from other related disorders was the incentive for a study to determine the clinical features that best distinguish PSP. Logistic regression and classification and regression tree analysis (CART) were used to analyse data obtained at the first visit from a sample of 83 patients with a clinical history of parkinsonism or dementia confirmed neuropathologically, including PSP (n = 24), corticobasal degeneration (n = 11), Parkinson's disease (PD, n = 11), diffuse Lewy body disease (n = 14). Pick's disease (n = 8) and multiple system atrophy (MSA, n = 15). Supranuclear vertical gaze palsy, moderate or severe postural instability and falls during the first year after onset of symptoms classified the sample with 9% error using logistic regression analysis. The CART identified similar features and was also helpful in identifying particular attributes that separate PSP from each of the other disorders. Unstable gait, absence of tremor-dominant disease and absence of a response to levodopa differentiated PSP from PD. Supranuclear vertical gaze palsy, gait instability and the absence of delusions distinguished PSP from diffuse Lewy body disease. Supranuclear vertical gaze palsy and increased age at symptom-onset distinguished PSP from MSA. Gait abnormality, severe upward gaze palsy, bilateral bradykinesia and absence of alien limb syndorme separated PSP from corticobasal degeneration. Postural instability successfully classified PSP from Pick's disease. The present study may help to minimize the difficulties neurologists experience when attempting to classify these disorders at early stages.

Fixation, Ocular↗
PubMed1997

Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study.

The accuracy of the clinical diagnosis of corticobasal degeneration (CBD) is unknown. To determine its diagnostic accuracy, we presented 105 cases with known neuropathologic diagnoses, including CBD (n = 10), progressive supranuclear palsy (PSP, n = 24), Parkinson's disease (n = 15), diffuse Lewy body disease (n = 14), multiple system atrophy (n = 16), postencephalitic parkinsonism (n = 7), Pick's disease (n = 7), Creutzfeldt-Jakob disease (n = 4), Alzheimer's disease (n = 4), vascular parkinsonism (n = 3), and Whipple's disease (n = 1), as clinical vignettes to six neurologists unaware of the autopsy findings. Reliability was measured with the kappa statistics. The neurologists' clinical diagnoses were compared with clinicopathologic diagnoses for sensitivity, specificity, and positive predictive values at first and last clinic visits. The group reliability for the diagnosis of CBD significantly improved from moderate for the first visit (mean = 34 months after onset) to substantial for the last (68 months after onset). For the first visit, mean sensitivity for CBD was low (35%), but specificity was near-perfect (99.6%). For the last visit, mean sensitivity minimally increased (48.3%), and specificity remained stable. False-negative misdiagnoses mainly occurred with PSP. False-positive diagnoses were rare. The extremely low sensitivity of the clinical diagnosis of CBD suggests that this disorder is markedly underdiagnosed. Although the validity of the clinical diagnosis might have been improved if neurologists could have examined these patients, more important is that this disorder was misdiagnosed by the primary neurologists. In our data set, the best predictors for the diagnosis of CBD included limb dystonia, ideomotor apraxia, myoclonus, and asymmetric akinetic-rigid syndrome with late onset of gait or balance disturbances.

Basal Ganglia↗
PubMed1997

What are the obstacles for an accurate clinical diagnosis of Pick's disease? A clinicopathologic study.

Several studies have evaluated the reliability and validity of the clinical diagnosis of Alzheimer's disease (AD) using well-defined neuropathologic criteria, but none has attempted to evaluate the diagnostic accuracy of Pick's disease. We determined the accuracy of the clinical diagnosis of Pick's by presenting 105 autopsy-confirmed cases of Pick's (n = 7) and related disorders (non-Pick's, n = 98) as clinical vignettes in randomized order to six neurologists who were unaware of the autopsy findings. The group of raters had moderate to fair agreement for the diagnosis of Pick's as measured by the kappa statistics. The sensitivity for the diagnosis of Pick's for the first visit (mean, 53 months after onset) and last visit (mean, 78 months after onset) was low (range, 0 to 71%), but specificity was near-perfect. Median positive predictive values at both visits were 83 to 85%. False-negative misdiagnoses mainly involved AD. False-positive diagnoses were rare and occurred with corticobasal degeneration (first visit) and with dementia with Lewy bodies (last visit). Pick's was also misdiagnosed by primary neurologists. The best clinical predictors for the early diagnosis of Pick's included "frontal" dementia, early "cortical" dementia with severe frontal lobe disturbances, absence of apraxia, and absence of gait disturbance at onset. However, the first neurologic evaluation in some of the Pick's cases took place in advanced stages of the disease. Our findings suggest that this disorder is underdiagnosed in clinical practice. Although the low sensitivity for the clinical diagnosis of Pick's is disappointing, our data suggest that when clinicians suspect Pick's, their diagnosis is almost always correct. Absence of awareness of the main features of this disorder and of specificity of the frontal lobe syndrome may partially explain the low detection of Pick's disease.

Aged↗
PubMed1997

[Dementia syndromes and length of stay of elderly patients in internal medicine].

Dementia is frequently observed in elderly patients admitted in Internal Medicine Units. Study of prospective data allowed comparison between 199 demented patients and 601 non demented ones, for life conditions, hospitalization parameters, way of discharge and associated diseases. Age and sex ratio are equal. Demented patients live more frequently in geriatric institutions and require more often nurse care at home. For them, social care is more frequent during hospitalization. There is no significant difference for mean duration of stay, discharge in step down unit and mortality. In both groups the predictive factors for the duration of stay are the need for social care during hospitalization and the number of comorbidities. In patients with degenerative dementia, hypertension and cardiopathies are less frequent than among other patients. These data suggest that adequate care, including social care, may produce the same duration of stay and way of discharge for demented and non demented elderly patients in Internal Medicine Units.

Aged↗
PubMed1996

Progressive supranuclear palsy: a clinicopathological study of 21 cases.

The symptoms and signs used to diagnose progressive supranuclear palsy (PSP) should be easily identifiable by neuropathologists and neurologists as well as by movement disorder experts. The presence, at the time of death, of symptoms and signs that are used in published clinical criteria for the diagnosis of this disorder was searched for in 21 pathologically confirmed typical PSP cases. The following items, present in at least 80% of pathologically confirmed cases, can be considered as the most accurate clinical data for the diagnosis of PSP: non-familial parkinsonism, not improved by L-dopa therapy, with vertical voluntary gaze palsy; postural instability and falls; pseudobulbar palsy and dementia with frontal lobe-like syndrome; and a progressive course of less than 10 years. The definite diagnosis of PSP must be clinicopathological, and these minimal clinical data may be used for this purpose.

Adult↗
PubMed1996

The significance of cortical pathology in progressive supranuclear palsy. Clinico-pathological data in 10 cases.

Several observations suggest that the cortical lesions of progressive supranuclear palsy (PSP) play a role in the clinical symptoms and signs of the disease, although direct evidence for this is lacking. We addressed the significance of these lesions in a series of 10 cases of definite PSP. Tau positive cortical lesions were constant, and the mean density of neurofibrillary tangles (NFTs) was highest in the precentral and angular gyri. Their cortical distribution was singular: they were mainly located in the deepest cortical layers, affecting small as well as large neurons. They were weakly and rarely labelled with anti-ubiquitin antibodies. The tau metabolism impairment appeared more diffuse than previously thought, since neuropil threads, tau positive astrocytic tangles and oligodendroglial inclusions were also seen in the cortex and the subcortical structures. A factorial analysis of NFT density in cortex and subcortex isolated two factors, cortical and subcortical, both linked to the pedunculopontine nucleus. This suggests a prominent role of this nucleus in the spread of the lesions. A pathological subgroup exhibited mild lesions in the pedunculopontine nucleus, which were always associated with mild cortical density of NFTs. In this subgroup, the clinical data were unusual: absence of oculomotor palsy and axial rigidity in one case, of dementia in another, and presence of tremor at rest in the third case. The presence of cortical NFTs could be related to the selective involvement of cortical pathways perhaps connected with the pedunculopontine nucleus.

Aged↗
PubMed1996

Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study.

OBJECTIVE--To analyse the natural history of progressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) and clinical predictors of survival in 24 patients with PSP confirmed by necropsy, who fulfilled the NINDS criteria for a neuropathological diagnosis of typical PSP. METHODS--Patients were selected from the research and clinical files of seven medical centres involving tertiary centres of Austria, England, France, and the United States. Clinical features were analysed in detail. The patients' mean age at onset of PSP was 63 (range 45-73) years. RESULTS--The most frequent clinical features (occurring in at least 75% of the patients) were early postural instability and falls, vertical supranuclear palsy, akinetic-rigid predominant parkinsonian disorder characterised by symmetric bradykinesia and axial rigidity unrelieved by levodopa, pseudobulbar palsy, and frontal release signs. Occasionally, segmental dystonia or myoclonus were described, but neither aphasia nor alien limb syndrome was reported. Fractures occurred in 25% of the patients but were unrelated to the severity of the gait or to the presence of falls. Median survival time was 5.6 (range 2-16.6) years. Onset of falls during the first year, early dysphagia, and incontinence predicted a shorter survival time. Age at onset, sex, early onset of dementia, vertical supranuclear palsy, or axial rigidity had no effect on prognosis of survival. Pneumonia was the most common immediate cause of death. PSP was most often clinically misdiagnosed as Parkinson's disease. Errors in diagnosis suggest that PSP is underdiagnosed. CONCLUSION--Progressive onset of early postural instability with falls or supranuclear vertical palsy in the fifth decade, should suggest the diagnosis of PSP. Onset of falls during the first year are emphasised, as they could lead to an early diagnosis and influence the prognosis of patients with PSP. Whether appropriate treatment of the dysphagia could prolong the survival of PSP patients needs to be explored.

Aged↗
PubMed1996

Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).

We assessed the validity and interrater reliability of neurologists who, using four different sets of previously published criteria for the clinical diagnosis of progressive supranuclear palsy (PSP), also called Steele-Richardson-Olszewski syndrome, rated 105 autopsy-proven cases of PSP (n = 24), Lewy body disease (n = 29), corticobasal ganglionic degeneration (n = 10), postencephalitic parkinsonism (n = 7), multiple system atrophy (n = 16), Pick's disease (n = 7), and other parkinsonian or dementia disorders (n = 12). Cases were presented in random order to six neurologists. Information from each patient's first and last visits to the medical center supplying the case was presented sequentially to the rater, and the rater's diagnosis was compared with the neuropathologic diagnosis of each case. Interrater agreement for the diagnosis of PSP varied from substantial to near perfect, but none of the criteria had both high sensitivity and high predictive value. Because of these limitations, we used a logistic regression analysis to identify the variables from the data set that would best predict the diagnosis. This analysis identified vertical supranuclear palsy with downward gaze abnormalities and postural instability with unexplained falls as the best features for predicting the diagnosis. From the results of the regression analysis and the addition of exclusionary features, we propose optimal criteria for the clinical diagnosis of PSP.

Aged↗
PubMed1994

Cortical tangles in progressive supranuclear palsy.

Ten cases of PSP were examined for the presence of neocortical and hippocampal lesions. Samples from 10 cortical areas were stained by Bodian's method and by tau, ubiquitin and beta A4 immunocytochemistry. For the sake of comparison, 5 Alzheimer's cases were studied with the same techniques. Neocortical tangles, star-like tufts of fibers, and neuropil threads were seen in all the cases of PSP. They were stained by Bodian's technique and labelled by an anti-tau, but not by a polyclonal anti-ubiquitin antibody. Senile plaques (Bodian's technique), diffuse or focal amyloid deposits (beta-A4 immunohistochemistry) were rare or absent. The density of tangles was the highest in area 4 and the lowest in area 17. In area 4, the tangles were mainly located in layers V-VI. By contrast, the Alzheimer's tangles had a bimodal distribution (layers III and V-VI). These results favor the specificity of cortical alterations in PSP.

Aged↗