One Europe, one neurologist?

Eur J Neurol. 2007 Mar;14(3):241-7. One Europe, one neurologist? Grisold W, Galvin R, Lisnic V, Lopes Lima J, Mueller E, Oberndorfer S, Vodusek DB; UEMS-EBN and EFNS Education Committee. Department of Neurology and Ludwig Boltzmann Institute for Neurooncology , Kaiser Franz Josef Hospital of the City of Vienna, Vienna, Austria. [email protected] Abstract In recent years, there has been a major shift in emphasis within neurology from being a largely diagnostic discipline to one much more actively involved in treating disease. There have been major scientific advances leading to new and effective treatments. There is also a much greater awareness of the burden of neurological disease (Olesen J, Leonardi M. European Journal of Neurology 2003; 10: 471) and informed sufferers are requesting specific intervention. There is wide variation in the delivery of neurological services throughout Europe. This is reflected in manpower levels, the place of neurology related to other medical specialties and different mixes of hospital and private office practice. These differences have been thrown into sharper focus by the recent expansion of the European Union (EU). Initial training in neurology is given to undergraduate/pre-graduate students. Post-graduate education is delivered within a residency program leading to specialist qualification and certification. We now recognize that this is only the beginning of a life long program of continuous education and development (CME/CPD). National and international exchange programs facilitate the growth of knowledge and promote professional harmony and cooperation. The free migration of medical specialists has been an aspiration but remains limited by cultural, linguistic, personal, professional, political and economic factors. Two bodies, the European Board of Neurology (EBN-UEMS) http://www.uems-neuroboard.org (Union Européenne des Médecins Spécialistes) and the European Federation of Neurological Societies (EFNS) http://www.efns.org are actively involved in harmonising and developing neurology at the European level. PMID: 17355542 [PubMed - indexed for MEDLIN

First Experience and the Effectiveness of Immunomodulating Treatment in Inflammatory Demyelinating CNS Diseases : Analysis of Nine Patients

Publisher Copyright: © 2015 by Lana Vainšteine.Therapeutic plasma exchange (TPE) is used in many neurological disorders to remove immunoglobulin and other immunologically active substances. We observed patients that were admitted in Riga East Clinical University Hospital "Gaiezers", Clinic of Neurology and Neurosurgery, Multiple Sclerosis Unit, and were diagnosed with relapsing remitting multiple sclerosis (MS), according to McDonald criteria 2010 (five patients), Neuromyelitis optica (NMO) spectrum disorders (three patients) and one with NMO, according to Wingerchuk 2006 criteria. All relapses were confirmed according to clinical criteria. Visual acuity was assessed by an ophthalmologist, and neurological status by a neurologist. All patients received at least 1 cycle of 1000 mg methylprednisolone intravenous for five to seven days. The expanded disability status scale score in the MS patient group was in range 4.0-9.0 before TPE and 3.5-6.5 range after TPE. Best improvement was observed in the MS group: mean symptom reduction of 20%. Patients with NMO spectrum disorder had an EDSS score of 8.0-8.5 range on admission and 6.5-8.0 range after TPE. After one month, one patient in the NMO spectrum disorder group had good response to TPE and EDSS was 3.5, two patients had only slight improvement (EDSS scores 8.0 and 7.5). Condition of patients with NMO did not improve even after a month.publishersversionPeer reviewe

Australian family carer responses when a loved one receives a diagnosis of Motor Neurone Disease—“Our life has changed forever”

While the experiences of family members supporting a person with a terminal illness are well documented, less is known about the needs of carers of people with neurological diseases, in particular, Motor Neurone Disease (MND). This paper describes the qualitative data from a large Australian survey of family carers of people with MND, to ascertain their experiences of receiving the diagnosis. The aim of the study was to describe the experiences of family carers of people with MND in receiving the diagnosis in order to inform and improve ways in which the diagnosis is communicated. Anonymous postal surveys were sent to people with MND in Australia and their family carers respectively. The perceived ability/skills of neurologists was assessed using a five‐point scale from excellent to poor. Attributes of communication of bad news was measured by the SPIKES protocol. Each survey question invited further written responses. Eight hundred and sixty‐four questionnaires were posted to people with MND and their family carers, with assistance from MND associations. One hundred and ninety‐six family carers submitted responses, of which 171 (88%) were patient‐carer dyads. Analyses were conducted on 190 family carers. Five themes emerged from reading and re‐reading written responses: frustrations with the diagnosis; giving information; family carer observations of the neurologist; the setting; and what would have made the diagnosis easier? The delivery of the diagnosis is a pivotal event in the MND trajectory. Satisfaction for patients and their family carers is related to the neurologists showing empathy and responding appropriately to their emotions, exhibiting knowledge and providing longer consultations. Neurologists may benefit from education and training in communication skills to adequately respond to patients’ and families’ emotions and development of best practice protocols

Summary of cerebrospinal fluid routine parameters in neurodegenerative diseases

In neurodegenerative diseases, cerebrospinal fluid analysis (CSF) is predominantly performed to exclude inflammatory diseases and to perform a risk assessment in dementive disorders by measurement of tau proteins and amyloid beta peptides. However, large scale data on basic findings of CSF routine parameters are generally lacking. The objective of the study was to define a normal reference spectrum of routine CSF parameters in neurodegenerative diseases. Routine CSF parameters (white cell count, lactate and albumin concentrations, CSF/serum quotients of albumin (Qalb), IgG, IgA, IgM, and oligoclonal IgG bands (OCB)) were retrospectively analyzed in an academic research setting. A total of 765 patients (Alzheimer’s disease (AD), Parkinson’s disease (PD), Parkinson’s disease dementia (PDD), vascular dementia (VD), frontotemporal lobar degeneration (FTLD), progressive supranuclear palsy (PSP), multisystem atrophy (MSA), motor neuron diseases (MND), spinocerebellar ataxia (SCA), Huntington’s disease (HD)) and non-demented control groups including a group of patients with muscular disorders (MD). The main outcome measures included statistical analyses of routine CSF parameters. Mildly elevated Qalb were found in a small percentage of nearly all subgroups and in a higher proportion of patients with PSP, MSA, VD, PDD, and MND. With the exception of 1 MND patient, no intrathecal Ig synthesis was observed. Isolated OCBs in CSF were sometimes found in patients with neurodegenerative diseases without elevated cell counts; lactate levels were always normal. A slightly elevated Qalb was observed in a subgroup of patients with neurodegenerative diseases and does not exclude the diagnosis. Extensive elevation of routine parameters is not characteristic and should encourage a re-evaluation of the clinical diagnosis

Neuromuscular abnormality and autonomic dysfunction in patients with cerebrotendinous xanthomatosis

<p>Abstract</p> <p>Background</p> <p>Cerebrotendinous xanthomatosis (CTX) is a rare lipid-storage disease. Neuromuscular abnormality and autonomic system (ANS) dysfuction in CTX are rarely examined in large-scale studies in the literature. We studied the peripheral nervous system, myopathology, and autonomic system of four CTX patients and performed a literature review of the reported CTX patients with peripheral neuropathy.</p> <p>Methods</p> <p>Four biochemically and genetically confirmed CTX patients, belonging to two families, were included for study and all received nerve conduction study (NCS), muscle biopsy for histopathologic and ultrastructural study, skin biopsy for intraepidermal nerve fiber (INEF) density measurement, autonomic testings including sympathetic skin response, R-R interval variation and head-up tilt test using an automated tilt table to record the changes of blood pressure and heart rate in different postures. The Q-Sweat test was also applied for the detection of sweat amount and onset time of response. The clinical characteristics, study methods and results of 13 studies of peripheral neuropathy in CTX patients in the literature were also recorded for analysis.</p> <p>Results</p> <p>The results of NCS study showed axonal sensory-motor polyneuropathy in three CTX cases and mixed axonal and demyelinating sensor-motor polyneuropathy in one. The myopathological and histopathologic studies revealed mild denervation characteristics, but the ultrastructural study revealed changes of mitochondria and the membranous system, and increased amounts of glycogen, lipofuscin and lipid deposition. The ANS study revealed different degrees of abnormalities in the applied tests and the INEF density measurement showed small fiber neuropathy in three of the four CTX patients. The literature review of peripheral neuropathy in CTX revealed different types of peripheral neuropathy, of which axonal peripheral neuropathy was the most common.</p> <p>Conclusions</p> <p>Peripheral neuropathy, especially the subtype of axonal sensori-motor neuropathy, is common in patients with CTX. Evidence of lipid metabolic derangement in CTX can be reflected in ultrastructural studies of muscles. With an adequate multi-parametric evaluation, a high incidence of ANS abnormalities can be seen in this rare lipid-storage disease, and a high incidence of small fiber involvement is also reflected in the IENF density measurement of skin biopsies.</p

Impairment and disability in 20 CIDP patients according to disease activity status.

Twenty patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) meeting the EFNS/PNS criteria were examined in order to assess differences/similarities between the various grading systems according to CIDP disease activity status (CDAS). A principal component (PC) analysis and the correlations between the following scores were performed: Neurological Symptom Score; MRC sum score; Neurological Impairment Score; Hammersmith Functional Motor Scale; Inflammatory Neuropathy Cause and Treatment (INCAT) Sensory Sum Score; Overall Disability Sum Score; INCAT Disability Score; Rasch-built Overall Disability Scale. Our analysis outlined two main sets of scales, with high influence in the top two PCs. The first PC that best explained the variability within the cohort consisted of CDAS, general disability scores and motor scores; these parameters were also strongly correlated amongst each other. The second PC explained less the variability and consisted mainly of sensory scores and disease duration; these parameters did not correlate with the scores of the first PC or with the CDAS. Our findings suggest separating screening for motor and sensory deficits when evaluating CIDP patients, as only the motor scores correlate with CDAS