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PubMedDate not supplied

[Assessment of the prognostic capacity improvement of the 5th edition of the TNM in patients with nasopharyngeal carcinoma].

OBJECTIVE: Evaluate the changes included in the 5th edition of the TNM classification in patients with nasopharyngeal carcinoma. PATIENTS AND METHODS: Retrospective study of 103 patients with nasopharyngeal carcinoma treated with radical intention in our centre from 1985 to 1997. A reclassification according to the criteria defined in the 5th edition of the TNM was carried out. The loco-regional control and survival obtained when patients were classified with the 4th and 5th editions of the TNM were compared. RESULTS: The use of the 5th edition of the TNM led to a decrease in local category (T) and stage grouping, improving the prognostic capacity in local control and survival. The changes included in the 5th edition of the TNM did not modify the regional classification in a essential way. CONCLUSIONS: The changes included in the 5th edition of the TNM improved the classification of patients with nasopharyngeal carcinoma.

Adult↗
PubMed2001

The atherosclerosis epidemic: methodology, nosology, and clinical practice.

An electronic search shows that there is a small but respectable amount of research on psychiatric and neurologic nosology. There is a also a small body of literature on endocrinologic nosology, but cardiologists almost never write about the nosology of heart disease. It does not keep them awake at night. Our understanding of the 20th-century epidemic of coronary artery disease (CAD), which dominates cardiology, has nevertheless been frustrated by problems of nosology. One of the purposes of this article is to discuss how our understanding of the epidemic has depended on classification of disease events and on the methods used to detect them. The other purpose is to show how current reclassifications of CAD and the major metabolic condition conducive to CAD, type 2 diabetes, each affect the practice of cardiology and medicine.

Adult↗
PubMed1985

Noninvasive prediction of hyaline membrane disease: an optimized classification of sonographic placental maturation.

Accurate prediction of fetal pulmonary maturity by means of a less invasive procedure than amniocentesis would be desirable. Sonographic diagnosis of a Grade III placenta has been reported to be an excellent predictor of fetal lung maturity. The standard classification of placental grading assigns grade according to the most advanced portion of the placenta. Using this classification, we studied 230 patients. In 80 pregnancies with Grade III placenta, three of the neonates developed respiratory distress syndrome. With reclassification of the placentas as immature, (no Grade III areas), intermediate, (only a portion of the placenta being Grade III), or mature, (Grade III placenta throughout), it was found that no neonatal hyaline membrane disease occurred in the 41 pregnancies with mature placentas, whereas 12% of the neonates in the immature group and 8% in the intermediate group developed hyaline membrane disease. These findings suggest that when sonographic examination of the placenta shows both Grade III and non-Grade III sections, there is still a risk for an immature amniotic fluid lecithin/sphingomyelin ratio and neonatal hyaline membrane disease. The placentas should be considered mature only when Grade III changes are present in all sections examined by ultrasound.

Amniotic Fluid↗
PubMed1999

Evaluation of TMN classification for lung carcinoma with ipsilateral intrapulmonary metastasis.

BACKGROUND: Staging for lung cancer based on the TNM classification is an important predictive factor for prognosis. Recently, lung cancer with ipsilateral intrapulmonary metastasis (PM) was reclassified according to the revision of the TNM classification. To evaluate the prognostic importance of the new staging system for PM, we analyzed the postoperative survival of patients with non-small cell lung carcinoma. METHODS: Of 1,002 consecutive patients who underwent operation for primary lung cancer between June 1984 and December 1996, we reviewed the medical record of 889 patients who underwent complete resection for non-small cell lung cancer. RESULTS: We considered 89 patients (10.0%) to have synchronous ipsilateral PM. After reclassification to the former staging system revised in 1992, 5 patients were classified as stage I, 29 as stage IIIA, 48 as stage IIIB, and 7 as stage IV. In the new staging system revised in 1997, 48 patients were recategorized as stage IIIB, and 41 as stage IV. The 5-year survival of patients without PM (49.5%) was significantly better than that of patients with PM in primary-tumor lobe (29.6%, p = 0.002) or in nonprimary-tumor ipsilateral lobe (23.4%, p = 0.0002). Although the survival of patients with stage IV cancer without PM was significantly worse than that of patients with the new (1997) stage IV cancer with PM (p = 0.02), it was similar to that of patients with the former (1992) stage IV cancer with PM. The survival of PM patients with N0 or N1 disease was significantly better than that of PM patients with N2 or N3 disease (p = 0.001). Furthermore, in patients with the new (1997) stage IIIB cancer, the survival of N0 disease was better than that of N2 disease (p = 0.007). CONCLUSIONS: Inasmuch as the prognosis of non-small cell carcinoma in patients with PM strongly correlated with N factor rather than PM factor, N factor should be reflected in a staging designation. We therefore consider the new TNM classification for PM reclassified in 1997 to be less acceptable for surgical-pathologic staging than the revision in 1992.

Adult↗
PubMed1997

Clinical experience with paraspinal mapping. II: A simplified technique that eliminates three-fourths of needle insertions.

OBJECTIVE: To simplify and minimize the quantified needle examination of the paraspinal muscles (paraspinal mapping [PM]) without compromising sensitivity or specificity. DESIGN: Nonrandomized prospective trial. SETTING: Electrodiagnostic laboratory of a university spine center and of a private practice in a small community. SUBJECTS: One hundred fourteen consecutive persons referred for electrodiagnosis of spinal or lower extremity disorders who had PM data and 35 previously reported asymptomatic volunteers. INTERVENTION: Abbreviated PM protocols were simulated by progressively eliminating data from the 45 needle insertions of the original PM. Simulations involving 35, 15, 13, and 5 insertions resulted in different normal values (95% of asymptomatic volunteers) and different scores in patients. The resulting reclassification of patients as normal or abnormal was compared with the original protocol and with clinical data. MAIN OUTCOME MEASURES: False positive and false negative rates of the simulations compared with the original protocol. RESULTS: Abbreviated protocols involving 30, 15, 13, and 5 needle insertions had normal cutoff scores of less than 5, less than 4, less than 3, and less than 2, respectively, with 2%, 2%, 4%, and 8% false positive rates and 3%, 8%, 13%, and 21% false negative rates compared with the original. In many cases clinical information correlated better with the abbreviated test results than the original PM. CONCLUSIONS: The third protocol compared well with the original PM, and in a limited number of patients with imaging studies demonstrated 92% sensitivity and 92% specificity. By eliminating the iliocostalis, longissimus, and lowest multifidus needle explorations, 73% fewer needle insertions were used. We recommend that this new technique, now called "miniPM," be used in most clinical settings.

Electromyography↗
PubMedDate not supplied

Typing Chlamydia psittaci--a review of methods and recent findings.

When the present chlamydial classification was established it was recognized that a wide variety of types were contained within the arbitrary designation Chlamydia psittaci. Early workers relied mostly on observations of growth characteristics to differentiate the types of C. psittaci isolated from a wide range of different hosts. The differences between isolates were confirmed serologically using a variety of tests of which the most sensitive was the micro-immunofluorescence (MIF) test which was able to recognize nine immunotypes among the mammalian isolates alone. This approach has recently been improved by the use of monoclonal antibodies in the MIF test which has confirmed most of the mammalian immunotypes and divided the avian strains into four groups. Studies on the nucleic acid of C. psittaci isolates show clear differences in the size distribution of DNA fragments produced by restriction endonuclease digestion of the genomes of the various types. Most importantly, studies of DNA/DNA homologies showed that at least four of the types identified by biological, serological and restriction endonuclease tests were sufficiently different to be considered separate species. Most recently, attention has been focused on DNA sequence comparisons of C. psittaci genes amplified by the polymerase chain reaction (PCR). The usual target has been the major outer membrane protein gene for which much sequence information is now available. The combination of PCR and MIF with monoclonals has provided a set of practical techniques with which all chlamydial isolates can be detected and typed with relative ease. It is likely that these developments will lead to the reclassification of the genus and, hopefully, a rapid increase of our understanding of the diseases caused by C. psittaci.

Animals↗
PubMed1983

Differential diagnostic value in hepatobiliary disease of serum conjugated bile acid concentrations and some routine liver tests assessed by discriminant analysis.

Serum concentrations of glycine and taurine conjugates of cholic, chenodeoxycholic, and deoxycholic acid were measured with a HPLC-enzymatic assay in 104 patients with hepatobiliary diseases. The capability of discriminating between four major diagnostic categories by the serum bile acid concentrations, alone and in conjunction with five commonly used biochemical liver tests, was evaluated by stepwise linear discriminant analysis. The serum bile acid pattern alone was inferior to the routine liver tests in separating the diagnostic groups. When the two sets of analytes were combined, the generalized group distance (Rao's V) was significantly increased, showing that the serum bile acid pattern contained discriminatory information in addition to the routine liver tests. The gain in correct reclassification of patients obtained by adding the serum bile acids, however, was only marginal.

Adolescent↗
PubMed1998

Clinicopathological characteristics of prostatic adenocarcinoma in men with atypical prostate needle biopsies.

PURPOSE: Atypical or nondefinitive diagnoses comprise 1.5 to 10% of all prostate needle biopsies and many men with atypical biopsy have carcinoma on rebiopsy. We characterize the clinical and pathological features of these men and the tumors, and compare them to those of other men who had more than 1 biopsy. MATERIALS AND METHODS: All prostate needle biopsies done at our institution between 1989 and 1996 on men with a followup biopsy were reviewed and the clinicopathological features were correlated. RESULTS: A total of 343 men had more than 1 biopsy during this period. Of the biopsies 64 were atypical and followup (repeat biopsy) was available for 59. Men with an atypical diagnosis were more likely to have carcinoma (34%) and to be diagnosed subsequently earlier (270 days) than those with an initial negative diagnosis (22%, 603 days). No significant differences were noted in patient age, results of digital rectal examination, initial or followup serum prostate specific antigen, subsequently identified tumor size or Gleason score on needle biopsy or at resection. Although on review as many as 38% of the original atypical foci could be reclassified, this reclassification did not significantly change the results of rebiopsy. CONCLUSIONS: Men with an atypical diagnosis on prostate biopsy are significantly more likely to have carcinoma on rebiopsy than men with an initial negative diagnosis, and the second biopsy should be performed at a significantly shorter interval. The tumors that are subsequently identified in these men are similar to those identified in men without an atypical biopsy.

Adenocarcinoma↗
PubMed1995

Relationship between low estrogen receptor values and other prognostic factors in primary breast tumors.

BACKGROUND: The current study compared the immunocytochemical expression of estrogen (ER) and progesterone (PgR) receptors by malignant breast cells to the hormone receptor concentrations reported from radioligand assays. These values were examined in relation to DNA ploidy and the fraction of cells in S phase. METHODS: ER and PgR concentrations, DNA ploidy, and S-phase fractions were measured by standard techniques with 124 samples of invasive ductal carcinoma. Suspensions of tumor cells were examined by immunocytochemical assay (ICA) for the percentages of ER and PgR positive cells. RESULTS: Twenty-six of the 38 tumors from patients 50 years of age or younger were classified as high S-phase fraction, and 28 tumors had aneuploid levels of DNA. The 20 ER positive tumors each contained less than 100 fmol/mg. Thirty-nine of the 86 tumors from patients older than 50 years were classified as high S phase, and 41 were aneuploid. Sixty-five samples were considered ER positive by radioligand assay. ICA showed that tumors in either age group with less than 40 fmol/mg did not contain ER positive cells. The proportion of samples with PgR levels between 10 and 100 fmol/mg was small, and fewer PgR positive tumors were categorized as negative when examined by ICA for receptor containing cells. The reclassification of the hormone receptor status of a tumor based on ICA appeared to be independent of S-phase and ploidy values. CONCLUSIONS: Tumors that are classified as ER or PgR positive based on accepted cutoff values for radioligand assays may actually be receptor negative because the tumors do not appear to contain receptor positive cells.

Adult↗
PubMed2000

The implementation of visual routines.

Many visual tasks can be decomposed into a sequence of simpler subtasks. Ullman suggested that such subtasks are carried out by elemental operations that are implemented by specialized processes in the visual brain [Ullman, S. (1984). Visual routines. Cognition (18), 97-159]. According to this hypothesis, there are a limited number of elemental operations that, since they can be applied sequentially, may nevertheless give rise to a large number of visual routines. Examples of such elemental operations are visual search, texture segregation and contour grouping. Here we attempt to delineate how such elemental operations are implemented in the visual brain. When an image appears, feedforward processing rapidly leads to an activity pattern that is distributed across many visual areas. Thereafter, elemental operations come into play, and these are implemented by the modulation of firing rates. Firing rate modulations effectuate grouping of neural responses into coherent object representations. Moreover, they permit transfer of information from one operator to the next, which allows flexibility in the sequencing of operations. We discuss how the elemental operations provide a tool to relate cortical physiology to psychophysics, and suggest a reclassification of pre-attentive and attentive processes.

Animals↗
PubMed2002

Prostate cancer involving the bladder neck: recurrence-free survival and implications for AJCC staging modification. American Joint Committee on Cancer.

OBJECTIVES: In the American Joint Committee on Cancer (AJCC) TNM staging system, prostate cancer involving the bladder neck after radical prostatectomy is considered pT4 disease, suggesting a high risk of recurrence. The recurrence risk with pathologic invasion of the bladder neck, however, has not been definitively compared with that associated with extra-organ disease. We therefore compared the recurrence risk in cases with bladder neck involvement with that of cases with extraprostatic extension and/or seminal vesicle invasion. METHODS: The study cohort was composed of 1123 men with clinically localized prostate cancer treated with prostatectomy as monotherapy. The preoperative prostate-specific antigen (PSA) level, bladder neck involvement, margin positivity, Gleason score, and other pathologic categories were assessed as covariates contributing to the PSA-recurrence risk in univariate and multivariable models. RESULTS: Bladder neck involvement was found in 60 (5%) of 1123 cases. In univariate analysis, the bladder neck was the site-specific margin with the greatest PSA-recurrence risk of focal involvement (relative risk 1.52, 95% confidence interval [CI] 1.15 to 2.00, P = 0.0030). The PSA-recurrence relative risk with extraprostatic extension was 3.05 (95% CI 2.13 to 4.38, P <0.0001) and with seminal vesicle invasion was 8.59 (95% CI 5.76 to 12.82, P <0.0001). In the multivariable model, the PSA-recurrence risk with bladder neck involvement (relative risk 1.19, 95% CI 0.72 to 1.96, P = 0.5) was not a significant independent prognostic factor. Extraprostatic extension (relative risk 2.25, 95% CI 1.54 to 3.27, P <0.0001) and seminal vesicle invasion (relative risk 4.12, 95% CI 2.57 to 6.62, P <0.0001) were significant independent predictors of PSA recurrence. CONCLUSIONS: Any staging system should be evidence based. The current AJCC system for staging bladder neck involvement, however, is contrary to the available evidence. Reclassification of bladder neck involvement as part of the pT3 category should be considered.

Analysis of Variance↗
PubMed2001

De novo acute myeloid leukemia in the elderly; a consistent fraction of long-term survivors by standard-dose chemotherapy.

To clarify the characteristics of de novo acute myeloid leukemia (AML) among the elderly, we reviewed 112 patients over 60 years old (median age 72 years) who were treated at hospitals in Nagasaki Prefecture with a population of 1.5 million between 1987 and 1994. Reclassification of morphological diagnosis revealed that the proportion of M3 was lower but that of M6 and the incidence of cases with trilineage dysplasia (TLD), known as poor prognostic features, were higher in the elderly than in patients less than 60 years old. Similarly, chromosomal data showed a lower frequency of favorable karyotypes such as t(8;21) and t(15;17) in the elderly. The overall survival of all 112 patients was 10.3% at 5 years. Multivariate analysis indicated that good performance status (PS), low WBC at diagnosis, standard dose multi-drug chemotherapy and all-trans retinoic acid (ATRA) treatment for M3 patients, and morphological findings without TLD were significantly correlated with longer survival. Most of the long-term survivors were found among those who received standard dose therapy in this series, although no consensus has been established how to treat elderly AML patients. We propose that a prospective controlled trial is necessary to confirm the role of standard dose chemotherapy for elderly patients with de novo AML.

Acute Disease↗
PubMed2001

Dysplastic versus proliferative CMML--a retrospective analysis of 91 patients from a single institution.

In chronic myelomonocytic leukemia (CMML) segregation of two subtypes has been suggested depending on WBC count-myelodysplastic (MD-CMML) and myeloproliferative (MP-CMML). In a retrospective analysis of 91 (60/31) previously untreated CMML patients, we compared the presenting clinical, haematological, laboratory and bone marrow features and examined the clinical impact of this reclassification. LDH values and bone marrow cellularity were significantly increased in MP-CMML. Median survival was significantly longer for patients with MD-CMML, progression rate was higher for MP-CMML. Patients with MD-CMML had longer median preleukemic duration; after transition to AML, MP-CMML patients had longer median survival. In MDS phase anemia was more common in MP-CMML and thrombocytopenia more common in MD-CMML whereas transfusion rates showed no difference. Evaluation of prognostic scoring systems for both groups confirmed that patients' characteristics and outcome could be well compared. Our data suggest that segregation into MD-CMML and MP-CMML is justified.

Aged↗
PubMed2002

Perinatal cortical and subcortical visual loss: mechanisms of injury and associated ophthalmologic signs.

PURPOSE: To determine whether term and preterm injuries to the retrogeniculate visual system are associated with recognizable patterns of ophthalmologic abnormalities and whether these patterns can be attributed to cortical (gray matter) or subcortical (white matter) injury. DESIGN: A retrospective case series. PARTICIPANTS: One hundred children with clinical and neuroimaging signs of perinatal posterior visual pathway injury who were examined at Arkansas Children's Hospital Eye Clinic between 1989 and 1999. METHODS: We reviewed magnetic resonance images or computed tomographic scans from 50 children with cortical (predominantly or exclusively involving cortical gray matter) and 50 children with subcortical (predominantly or exclusively involving subcortical white matter) perinatal injury to the retrogeniculate visual system. Ophthalmologic abnormalities were analyzed retrospectively in each group. MAIN OUTCOME MEASURES: Conjugate gaze deviation, type of strabismus, abnormal eye movements, and optic disc morphology. RESULTS: Horizontal conjugate gaze deviation, exotropia, and a normal optic disc appearance were significantly more common in cortical than in subcortical visual loss. Tonic downgaze, esotropia, and optic nerve hypoplasia (with or without coexisting pallor) were significantly more common in subcortical than in cortical visual loss. CONCLUSIONS: Perinatal cortical and subcortical visual loss produce differing profiles of ophthalmologic dysfunction. A reclassification of periventricular leukomalacia and other forms of retrogeniculate white matter injury as subcortical visual loss would increase diagnostic specificity.

Blindness, Cortical↗
PubMed2002

Electrophysiologic and phenotypic features of an autosomal cone-rod dystrophy caused by a novel CRX mutation.

PURPOSE: To reexamine a large Albertan family previously reported with a progressive cone dystrophy with variable phenotype and to map the disorder using molecular genetic techniques. DESIGN: Observational case series. PARTICIPANTS: Twenty-nine subjects (10 affected) from four generations of a large kindred were clinically examined. Twenty-three of these individuals, as well as two unaffected spouses, were included in the molecular genetic study. Subject ages ranged from 17 to 91 years of age. METHODS: Disease status and associated ocular abnormalities were assessed primarily by measurement of visual acuity, color vision, fundus photography, and both full-field and multifocal electroretinography (ERG and mfERG). Linkage of the disorder to the rhodopsin gene was studied using microsatellites. A mutational screen of the CRX gene was performed to identify coding sequence changes. MAIN OUTCOME MEASURES: Visual acuity and color discrimination were reduced in clinically affected individuals; full-field flash ERG was used to measure function of both cones and rods. mfERG and fundus photography allowed documentation of the observed macular changes. RESULTS: We noted a variable, adult-onset macular dystrophy, progressing in some cases to a retinitis pigmentosa-like phenotype. Both photopic and scotopic full-field ERG amplitudes were reduced by approximately 50%, demonstrating involvement of both photoreceptor systems. A reduced b-wave amplitude with a relatively preserved a-wave was observed at both cone and rod levels. Macular involvement was confirmed by mfERG. The rhodopsin locus was excluded by haplotype analysis. A novel frameshift mutation was detected in exon III of the CRX retinal homeobox gene. ERG and molecular genetic findings were consistent with the reclassification of this disease as an autosomal dominant cone-rod dystrophy (CRD) CONCLUSIONS: We report a novel CRX mutation causing autosomal dominant CRD. Observed ERG changes suggest that this mutation primarily impairs inner retinal function. Because retinal expression of CRX is limited to photoreceptors, this dysfunction may be the result of faulty photoreceptor communication with second-order retinal neurons. We propose misexpression of gated cation channels caused by altered CRX activity as one putative mechanism by which a sole photoreceptor defect may selectively impair neurotransmission without disrupting the upstream events of phototransduction.

Adolescent↗
PubMed1998

Early mortality of undiagnosed but prevalent (in 1983-1984) HIV infection in Lothian injectors who tested hepatitis B surface antigen positive (group A) or negative but were high risk for blood-borne virus transmission (group B) in 1983-1984.

OBJECTIVE: To estimate the early death-rate in HIV infected injectors whose HIV infection was during the injection-related HIV outbreak in Lothian region in Scotland in 1983-1984, which was coincident with Hepatitis B transmissions. SETTING: Regional Virus Laboratory in Edinburgh. SAMPLES: Sera from 1983-1984, originally received for Hepatitis B surface antigen testing, from individuals aged 15-55 years who were positive for Hepatitis B surface antigen in 1983-1984 (group A: census) or tested negative but were at high risk for blood-borne virus transmission according to their reason for testing (group B: 50% sample). METHODS: Survival status of individuals in groups A and B who had not been diagnosed with HIV disease by the end of December 1995 was checked against the deaths' records of the Registrar General for Scotland. Stored sera from 1983-1984 for patients who had died early (that is: in 1983-1984) were tested anonymously for HIV and Hepatitis C antibodies; and prior to testing, causes of death were scored by RPB according to the likelihood of their being HIV or drugs related. RESULTS: Three early deaths were found in group A patients who were not known to be HIV infected. None of the deaths was likely to be HIV-related; the sera were not tested in order not to risk deductive disclosure. Twenty-four early deaths were found in group B patients who were not known to be HIV-infected, five of whom were both HIV and Hepatitis C antibody positive, and one other was HIV antibody negative but Hepatitis C positive. Reclassification after unlinked anonymous testing and multiplying up of the group B results (to account for 50% sample) gave the early death rate (that is: in 1983-1986) as 15/155 (10%) for HIV-infected drug users (95% CI: 6%-13%). CONCLUSION: Injection-related outbreaks of HIV infection in Lothian in 1983-1984 and at Glenochil Prison in 1993 were each associated with substantial--estimated 10%--early death-rate in HIV-infected injectors. Both HIV outbreaks were coincident with Hepatitis B transmissions, which may be relevant. Further investigations of the death-rate within 2 years of HIV infection are warranted in other exposure categories than injection-related and for injectors who have been immunized against Hepatitis B.

Adolescent↗
PubMed2005

Studies on a German (Münster) version of the temperament auto-questionnaire TEMPS-A: construction and validation of the briefTEMPS-M.

BACKGROUND: Based on classic German concepts of a continuum between depressive, hyperthymic, cyclothymic, and irritable temperaments and affective disorder (and adding an anxious type to the four), Akiskal and co-workers developed the Temperament Evaluation of Memphis, Pisa, Paris and San Diego both in interview (TEMPS-I) and auto-questionnaire (TEMPS-A) versions. It is the aim of the present analyses to validate a brief German version of TEMPS-A. METHODS: A total of 1056 students of the Westfalische-Wilhelms-Universitat in Munster, Germany, filled out the long 110-item version of the TEMPS-A (Munster translation by Erfurth: TEMPS-M) modified into a five gradation Likert format and with the items randomized. Based on this data we constructed a brief version of the TEMPS-M. In a second study, a sample of 151 students were recruited who filled out the briefTEMPS-M twice, approximately 1 month apart. RESULTS: Our psychometric procedures resulted in the retention of 35 items from the original 110. The proposed five-factor structure of the original TEMPS-A was upheld, with relatively few item reclassification (mainly due to some overlap between depressive and anxious traits). Internal consistency (Cronbach alpha values ranging from 0.69 to 0.84) and test-retest reliability were shown. Most importantly, all temperaments in the briefTEMPS-M correlated quite well (Pearson r values ranging from 0.49 to 0.72) with their respective original versions in the longer TEMPS-M. As for construct validity, significant correlation was shown with the Beck Depression Inventory for all but the hyperthymic temperament; the hyperthymic, cyclothymic and irritable correlated highest with the self-report Manic Inventory. LIMITATIONS: The study sample of university students was selective. CONCLUSIONS: We were able to construct a brief German version of the TEMPS-A auto-questionnaire. We submit this shorter version will be suitable for both clinical (psychiatric and general medical) and neurobiological research, as well as in studies on temperament features in selected populations, e.g., allowing comparisons between regions or different (German-speaking) countries.

Adolescent↗
PubMed1999

Pathogenesis of Guillain-Barré syndrome.

Recent neurophysiological and pathological studies have led to a reclassification of the diseases that underlie Guillain-Barré syndrome (GBS) into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor and sensory axonal neuropathy (AMSAN) and acute motor axonal neuropathy (AMAN). The Fisher syndrome of ophthalmoplegia, ataxia and areflexia is the most striking of several related conditions. Significant antecedent events include Campylobacter jejuni (4-66%), cytomegalovirus (5-15%), Epstein-Barr virus (2-10%), and Mycoplasma pneumoniae (1-5%) infections. These infections are not uniquely associated with any clinical subtype but severe axonal degeneration is more common following C. jejuni and severe sensory impairment following cytomegalovirus. Strong evidence supports an important role for antibodies to gangliosides in pathogenesis. In particular antibodies to ganglioside GM1 are present in 14-50% of patients with GBS, and are more common in cases with severe axonal degeneration associated with any subtype. Antibodies to ganglioside GQ1b are very closely associated with Fisher syndrome, its formes frustes and related syndromes. Ganglioside-like epitopes exist in the bacterial wall of C. jejuni. Infection by this and other organisms triggers an antibody response in patients with GBS but not in those with uncomplicated enteritis. The development of GBS is likely to be a consequence of special properties of the infecting organism, since some strains such as Penner 0:19 and 0:41 are particularly associated with GBS but not with enteritis. It is also likely to be a consequence of the immunogenetic background of the patient since few patients develop GBS after infection even with one of these strains. Attempts to match the subtypes of GBS to the fine specificity of anti-ganglioside antibodies and to functional effects in experimental models continue but have not yet fully explained the pathogenesis. T cells are also involved in the pathogenesis of most or perhaps all forms of GBS. T cell responses to any of three myelin proteins, P2, PO and PMP22, are sufficient to induce experimental autoimmune neuritis. Activated T cells are present in the circulation in the acute stage, up-regulate matrix metalloproteinases, cross the blood-nerve barrier and encounter their cognate antigens. Identification of the specificity of these T cell responses is still at a preliminary stage. The invasion of intact myelin sheaths by activated macrophages is difficult to explain according to a purely T cell mediated mechanism. The different patterns of GBS are probably due to the diverse interplay between antibodies and T cells of differing specificities.

Animals↗