Multiple mononeuropathy as the initial presentation of systemic lupus erythematosus — nerve biopsy and response to plasma exchange

Summary A total of 15 patients affected by idiopathic dystonia (7 with generalized and 8 with focal or segmental dystonia) were subjected to therapy with bromocriptine at low doses, pimozide and trihexyphenidyl. The symptoms were evaluated by giving a progressive score in relation to the intensity of the dystonic symptom to each of the body segments involved by the dystonia. Bromocriptine did not significantly modify the dystonia. Pimozide showed a slight nonsignificant improvement of the dystonic symptoms. Trihexyphenidyl was effective in the generalized dystonias, in agreement with previous reports in the literature. The variation in the pharmacological results could be due to the diversity of the dystonic syndromes, which comprise cases that are different in age at onset, site of dystonic symptoms, and evolution.

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Zusammenfassung Fünfzehn von idiopathischer — und zwar 7 von generalisierter und 8 von fokaler und segmentarischer — Dystonie befallenen Patienten unterzogen sich verschiedenen pharmakologischen Behandlungen mit kleinen Mengen Bromocriptine, Pymozide und Triesifenidile. Die Symptome wurden durch eine fortlaufende Punktzahl bezeichnet, so daß deren Schätzung von der Intensität des Symptoms Dystonie in jedem einzelnen befallenen Körperteil abhing. Die Dystonien wurden durch Bromocriptine nicht bedeutend geändert.Pymozide führte zu einer geringeren, doch unbedeutenden, Besserung der dystonischen Symptome.Triesifenidile wirkte auf die generalisierten Dystonien, in Übereinstimmung mit einigen Literaturangaben.Die Veränderlichkeit der pharmakologischen Ergebnisse wurde auf die Verschiedenheit der dystonischen Syndrome zurückgeführt, unter denen man Fälle versammelt, die sich durch Anfangsalter, Sitz der dystonischen Symptome und Entwicklungsart voneinander unterscheiden.

     

Guillain-Barré syndrome in a course of early cutaneous type of Lyme borreliosis: diagnostic and therapeutic difficulties

A case is reported of a 33-year-old man in whom Guillain-Barré syndrome (GBs) developed three weeks after a tick's sting. At the sting site typical for an early cutaneous type of Lyme borreliosis -- erythema migrans -- appeared. The demyelinating polyradiculoneuropathy of GBs occurred after disappearance of erythema migrans, and was manifested by progressive neuropathic symmetrical limb weakness with distal numbness and pain, and bilateral facial paralysis. The GBs was confirmed by electrophysiological examination and elevated protein concentration with a normal range of cells in the cerebrospinal fluid. Antibodies IgM and IgG against Borrelia burgdorferi in the blood serum and cerebrospinal fluid assessed using immunoenzymatic assay, MEIA, were negative on account of their early search. The above findings suggested that the GBs appearance after the probable Borrelia burgdorferi infection was in fact due to that infection. The patient recovered after treatment with plasma-phoresis and corticosteroid therapy followed by intravenous immunoglobulin, and physiotherapy. This is the first case in the Polish neurological literature of GBs with an early skin form of borreliosis which developed after the tick's sting.     

Cocultures of meningeal and astrocytic cells—A model for the formation of the glial-limiting membrane

The glial-limiting membrane at the border of the central nervous system (CNS) consists of glial endfeet covered by a basal lamina. The formation of the glia limitans seems to be controlled by adjacent meninges but only little is known about this interaction. In the present study astrocytes and meningeal cells were investigated in vitro to see if cocultures of these cells can serve as a suitable model for the differentiation of the glial-limiting membrane and can be used to define the conditions under which the glial-limiting membrane develops. The following observations were made in cocultures of meningeal and astrocytic cells of two-day-old rats: (i) epithelioid astrocytes were transformed into stellate cells; (ii) single colonies of proliferating epithelioid astrocytes were generated; (iii) the area around these colonies becomes devoid of meningeal cells, which seem to form a circular border around the astroglial islands; (iv) from the glial colonies long thin glial processes grow towards the surrounding meningeal cells, terminating at the site of contact; (v) in the contact zone between meningeal cells and astrocytes irregular shaped deposits of electron dense material resembling a basal lamina were seen. These observations indicate that indeed a structure resembling a glial-limiting membrane develops in cocultures of meningeal and astrocytic cells. Its formation depends on the balance of growth promoting effects of meningeal cells on astrocytes and growth inhibiting effects of astrocytes on meningeal cells. Both activities can be enriched from conditioned media of pure astrocytic or meningeal cell culture. The proposed model of meningo-astrocytic cocultures may be a helpful instrument for further investigations on the formation of the glia limitans.     

Strategy for patients with co-existence of meningioma and intracerebral aneurysm,especially unruptured aneurysm (–seven cases and review of the literature–)

BackgroundIntracerebral aneurysms co-existing with meningiomas are rare. Treatment strategies for intracerebral aneurysms co-existing with meningiomas have not yet been established.MethodsWe studied 62 patients with intracerebral aneurysms co-existing with meningiomas in the literature including our seven cases, evaluated the various managements and outcomes, and discussed the strategy for intracerebral aneurysms, especially unruptured cases, co-existing with meningiomas. The aim of this study was to develop a guide for the management of non-subarachnoid hemorrhage (SAH) intracerebral aneurysms co-existing with meningiomas.ResultsMost intracerebral aneurysms co-existing with meningiomas are unruptured. Of course, aneurysms presenting with SAH should be treated first followed by the resection of meningiomas. In addition, intracerebral aneurysms inside or adjacent to meningiomas have a high risk of intraoperative rupture during the surgery for meningiomas, and it may be necessary to treat them first followed by the resection of meningiomas with one or two-step surgery.In nine out of 62 patients, ten intracerebral unruptured aneurysms were not treated; however, no intracerebral aneurysms ruptured during the follow-up period, and outcomes of these patients were good in eight and poor in only one.ConclusionsIntracerebral unruptured aneurysms remote from meningiomas may be treated according to the guidelines for unruptured aneurysms.In advance of microsurgery and endovascular techniques, both lesions should be treated, if possible.     

Parkinsonism associated with Sj?gren's syndrome: three cases and a review of the literature.

Sj?gren's syndrome (SS) is a common multisystem autoimmune disorder. As with other autoimmune disorders such as systemic lupus erythematosus (SLE), SS has been associated with a wide range of neurologic abnormalities. Parkinsonism has been reported previously in five SS patients. We present three additional cases of SS with parkinsonism.     

Editor’s Choice——The influence of traditional Chinese medicine monomers and electroacupuncture on glial cell injury

Glial cells in the central nervoussystem play an important role inimmune and inflammatory reactions.Astrocytes exhibit specific spatialpatterns and complex molecularlevel changes, and also have a dualeffect on ischemic neuronal injury     

The relationship between job-anxiety and trait-anxiety—A differential diagnostic investigation with the Job-Anxiety-Scale and the State-Trait-Anxiety-Inventory

Job-related anxiety, in contrast to general trait-anxiety, is by its very nature associated with problems of participation at work. The aim of this study is to investigate the relation between general trait-anxiety and specific job-related anxiety and to examine whether job-anxiety and trait-anxiety are differently associated with sick leave. 190 inpatients of a psychosomatic and orthopaedic rehabilitation center with mental and somatic disorders filled in the Job-Anxiety-Scale (JAS) and the State-Trait-Anxiety-Inventory (STAI-T). Additionally, informations on age, gender, the current duration of sick leave in weeks, employment status, duration of unemployment, and position at the workplace were collected.Highest scores of job-anxiety were found for the JAS-dimensions “job-related worries” and “health anxieties”, followed by “cognitions of insufficiency,” “stimulus-related anxieties,” and “social anxieties.” JAS and STAI-T were significantly correlated. Job-anxiety, in contrast to trait-anxiety, was significantly related to duration of sick leave. Women showed higher scores on the STAI-T but not on the JAS.It can be concluded that job-anxiety is related to but not identical with trait-anxiety. Job-anxiety is important to understand sick leave and appears as a multidimensional and clinically important phenomenon.     

The influence of parental offending on the continuity and discontinuity of children’s internalizing and externalizing difficulties from early to middle childhood

Purpose

Although parental criminal offending is a recognized risk factor for conduct problems among offspring, its impact on the continuity and discontinuity of children’s behavioural and emotional difficulties during the early development is less well known. We used data from a large, population-based record-linkage project to examine the relationship between parental offending and the continuity and discontinuity of children’s conduct, attentional, and emotional difficulties from early to middle childhood while also considering the role of timing of the parental offending exposure.

Method

Data for 19,208 children and their parents were drawn from the New South Wales Child Development Study. Multinomial regression analyses tested associations between mother’s and father’s history and timing of any and violent offending, and patterns of continuity or discontinuity in offspring emotional, conduct, and attentional difficulties between ages 5 and 11 years.

Results

Maternal and paternal offending each conferred a significantly increased risk of all the patterns of developmental difficulties, including those limited to age 5 only (remitting problems), to age 11 only (incident problems), and to difficulties present at both ages 5 and 11 years (persisting problems). Greatest odds were observed for persisting conduct problems. Paternal offending that continued through early and middle childhood had the greatest association with child difficulties, while the timing of maternal offending had a less prominent effect on child developmental difficulties.

Conclusion

Parental offending is a strong risk factor for early and pervasive behavioural and emotional problems in offspring, and may be a key indicator of high risk for later antisocial behaviour.

     

The genetics of epilepsy—The past,the present and future

A brief history of human geneticsSixty years is an appropriate yardstick for many reasons, not least for the remarkable advances in medicine, public health, psychology and biological disciplines. Particularly relevant is the approaching 60th anniversary of the discovery of the structure of DNA, which unlocked the driving force of nature and spawned a plethora of scientific discoveries and economic development through the Bitoech industry. Prior to 1953, and before Watson and Crick burst into the Cambridge pub with their eureka moment, it was known that chromosomes were important, the first principles of clinical cytogenetics were emerging and the rules of heritable traits were well-advanced, but without the basic framework or mechanism. Human Molecular Genetics arrived when the first mutations were linked to human disorders reflecting the advances in understanding the genetic code, assembly of protein building blocks and methodological advances in reading the physical code (all be it very difficult process at the time). Accelerated by the introduction of recombinant gene technology in the 1980s, and in conjunction with the development of linked genetic marker maps, the catalogue of genes associated with disease has risen exponentially with classical examples such as sickle cell disease, cystic fibrosis and Huntington's disease. The advances approached super-sonic dimensions when genes were found in Mendelian families, and mapping strategies were adopted using the variation map of the human genome (SNP's, di-nucleotide repeats), in addition to targeted candidate gene approaches aided by the significant database resources available to investigators. Super-sonic gave way to light-speed with the publication of the 3 billion letters of the genetic code which constitutes the human genome, followed quickly by genomes in plants, bacteria, pathogens, fruits and vegetables, and a menagerie of eukaryotic and prokaryotic animals, often representing model systems for genomic and pathophysiological research. In short don’t blink or you’ll miss the next revolution – too late, it's just happened!     

The effect of neuroleptic treatment and of high dosage diazepam therapy onβ-endorphin immunoreactivity in plasma of schizophrenic patients

Summary Synapsin I (Protein I), a neuron-specific phosphoprotein enriched in presynaptic nerve terminals, has been used as a quantitative marker for the density of nerve terminals in five brain regions (caudate nucleus, cingulate gyrus, hippocampus, mesencephalon and putamen) from patients who had suffered from Alzheimer disease/senile dementia of Alzheimer type (AD/ SDAT), from patients with multi-infarct dementia (MID), and from agematched controls. Samples were obtained at autopsy. Lower levels of Synapsin I were observed in the hippocampus of patients with AD/SDAT but not with MID. There were no significant differences in Synapsin I levels between patients and controls in any of the other four brain regions examined.     

Papillary glioneuronal tumor—a rare entity: report of four cases and brief review of literature

Purpose

Papillary glioneuronal tumors (PGNT) have been recently included as a distinct entity in the WHO classification of tumors of the central nervous system. Their molecular pathogenesis is not clear. In the current study, we present the morphological, immunohistochemical, and molecular features of four cases of PGNT reported over the past 11?years.

Methods

Over a period of 11?years (January 2000–February 2010), there were four cases of PGNT, which were reviewed for histomorphological features. TP53 and IDH1 mutations were assessed using antibodies against p53 protein and for mutant IDH1^R132H protein, respectively. Immunohistochemistry was also performed for epidermal growth factor receptor (EGFR) protein. Fluorescence in situ hybridization assay was used for analyzing 1p/19q deletion status.

Results

All the tumors showed the characteristic biphasic morphology. Rare findings included minigemistocyte-like cells in one, angiomatous areas in three, focal necrosis in one, and a high MIB-1 labeling index of 12 and 13?%, respectively, in two of the cases. All lacked EGFR, IDH1 expression, and 1p/19q deletions. Interestingly, antibody for p53 labeled the tumor cells, mainly those showing glial differentiation, in two cases. At a mean follow-up of 30?months, there was no evidence of disease progression except in one case which recurred after 24?months.

Conclusion

PGNT are rare CNS neoplasms. Despite showing focal morphological features reminiscent of oligodendroglial tumors and presence of astrocytic component, they usually lack the common genetic alterations involved in the pathogenesis of gliomas. Multi-institutional pooling of cases may aid in elucidating their oncogenetic pathway.     

Monocytes and plasma tissue factor levels in normal individuals and patients with deep venous thrombosis of the lower limbs: potential diagnostic tools?

INTRODUCTION: Tissue factor (TF) is the main physiological initiator of blood coagulation; it is membrane-bound on monocytes (mTF) and free in plasma (pTF). Abnormal expression of TF by monocytes has been implicated in various diseases. We therefore quantified monocytes expressing TF and pTF levels in patients with lower-limb deep venous thrombosis (DVT). MATERIALS AND METHODS: DVT was confirmed by Duplex Scan. Blood mTF levels under resting condition (baseline), after incubation without (unstimulated) and with (stimulated) lipopolysaccharide (LPS), and total mTF levels were determined by flow cytometry using two analytical methods (Histogram and Quadrant-Statistics). Plasma TF levels were measured using an enzyme-linked immunoabsorbent assay (ELISA). Results were compared with age-matched controls. RESULTS: Histogram analysis in patients with DVT showed significantly elevated mTF levels for baseline, unstimulated and total mTF over controls. For Quadrant-Statistics, DVT patients also showed significantly raised baseline, unstimulated, stimulated and total mTF. Similarly, pTF levels were significantly raised in subjects with DVT compared to controls. Baseline mTF levels correlated with pTF levels by Histogram and Quadrant-Statistics analysis. Using the relative operating characteristic (ROC) curve, baseline mTF and pTF assays displayed sensitivity and specificity in detecting DVT. Quadrant-Statistics baseline mTF and pTF gave the best discrimination. CONCLUSIONS: The TF assays used in this study showed acceptable sensitivity and specificity and are cost-effective and practical. Therefore, they should be considered in patients with, or at risk of, DVT.     

The relationship between obsessive-compulsive disorder and anxiety disorders: A question of diagnostic boundaries or simply severity of symptoms?

BackgroundA growing number of studies are questioning the validity of current DSM diagnoses, either as “discrete” or distinct mental disorders and/or as phenotypically homogeneous syndromes. In this study, we investigated how symptom domains in patients with a main diagnosis of obsessive-compulsive disorder (OCD), panic disorder (PD) and social anxiety disorder (SAD) coaggregate. We predicted that symptom domains would be unrelated to DSM diagnostic categories and less likely to cluster with each other as severity increases.MethodsOne-hundred eight treatment seeking patients with a main diagnosis of OCD, SAD or PD were assessed with the Dimensional Obsessive-Compulsive Scale (DOCS), the Social Phobia Inventory (SPIN), the Panic and Agoraphobia Scale (PAS), the Anxiety Sensitivity Index-Revised (ASI-R), and the Beck Depression and Anxiety Inventories (BDI and BAI, respectively). Subscores generated by each scale (herein termed “symptom domains”) were used to categorize individuals into mild, moderate and severe subgroups through K-means clusterization and subsequently analysed by means of multiple correspondence analysis.ResultsBroadly, we observed that symptom domains of OCD, SAD or PD tend to cluster on the basis of their severities rather than their DSM diagnostic labels. In particular, symptom domains and disorders were grouped into (1) a single mild “neurotic” syndrome characterized by multiple, closely related and co-occurring mild symptom domains; (2) two moderate (complicated and uncomplicated) “neurotic” syndromes (the former associated with panic disorder); and (3) severe but dispersed “neurotic” symptom domains.ConclusionOur findings suggest that symptoms domains of treatment seeking patients with OCD and anxiety disorders tend to be better conceptualized in terms of severity rather than rigid diagnostic boundaries.