摘要:

ABSTRACT—The acute malignant hyperthermia crisis appears to be mediated by asudden and sustained rise in the concentration of sarcoplasmic calcium, leading to a prolonged muscle contraction. It is not known whethr clcium metabolism in skeletal muscie is abnormal in malignant hyperthermia susceptible (MHS) subjects under normal condition. Ahpermetabolic state in skeletal muscle can be assessed in vivo by measuring relaxation rate. In the present study the relaxation rate was measured in the adductor policis muscle after electerical stimulation of the ulnar nerve. Ten MHS subjects with no clinical symptoms of muscle disease were investigated. Relaxation rates were found to be significantly higher in the MH‐susceptible subects compared with controls even under normal condition (p0.0001). Determination of relaxtion rate could thus be considered a possible addition to those methods currently available for the diagnosis of

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07898.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—Intrathecal IgG synthesis and CSF oligoclonal bands were reexamined after 18–24 months in 66 patients with multiple sclerosis; 40 of them received azathioprine (AZA) 2.5 mg/Kg/die; all received a course of dexamethasone (DEXA) during clinical relapses. The IgG Index was significantly reduced in the group treated with AZA, especially in patients with short disease duration, low disability and high IgG index. Changes observed in CSF banding pattern were not significant. These results suggest an effect of AZA on IgG synthesis, as reported byin vitrostud

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07899.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—Dopaminergic receptor properties in the striatum of patients with Parkinson s disease (PD) and Huntington's chorea (HD) were studied by positron emission tomography (PET), using11C‐N‐methyl‐spiperone as a dopamine D2receptor ligand. The time‐dependent regional radioactive uptake in the caudate nucleus and the putamen was measured and fitted to a 3‐compartment pharmacokinetic model. The rate constant k3for specific binding to the receptor compartment in the striatum was determined in relation to the binding in regions with a low density of specific binding sites, such as the cerebellum and the frontal cortex–k3, which is a measure of the receptor density, was reduced in one patient with HD but less affected in PD in comparison with healthy controls. The pattern of k3values calculated from the 6 PD patients is discussed in relation to any side‐to‐side differences in dopamine receptor densities in hemiparkinsonism and to possible “hypersensitivity” of dopamine receptors in the early stage of the disease and down‐regulation i

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07900.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—A 3‐year intensive search for selected neuromuscular disorders in Benghazi, yielded 34 patients with Duchenne's muscular dystrophy (25 index cases), 19 with limb‐girdle muscular dystrophy (13 index cases), 4 with facioscapulohumeral muscular dystrophy (3 index cases), 3 with opthalmoplegia‐plus (all index cases), 13 with polymyositis, 41 with hereditary motor and sensory neuropathy (HMSN) (17 index cases) and 27 with Guillain‐Barré Syndrome (GBS). The age‐adjusted prevalence rates, on 31 December 1985, per 100,000 population were 6 for Duchenne dystrophy, 3.7 for limb‐girdle dystrophy, 0.8 for facioscapulohumeral dystrophy, 0.6 for opthalmoplegia‐plus and 7.9 for HMSN (6.4 and 1.5 for Types I and II, respectively). The adjusted average annual incidence of polymyositis was 8.8/mill population; the peak incidence was observed in females in the age‐group 20–40. A mean age‐adjusted incidence rate for GBS of 1.7/100,000 population per year has been found. The peak age‐specific incidence of GBS occurred in the third decade; the sex‐dependent difference in the incidence was not significant. No indigenous forms of the disease were encountered and the clinical features differed little from the descriptions in literature. The large family size and high rate of consanguineous marriages contribute to the high frequency of familial disorders, especially those with autos

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07901.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—Twenty‐four patients with Guillain‐Barré syndrome were prospectively evaluated for evidence of autonomic dysfunction. It occurred in 16 (66.7%) patients, usually during the peak period of paralysis, in the form of either excess or inadequate activity of sympathetic and/or parasympathetic nervous systems. Its clinical manifestations comprised of sinus tachycardia (33.3%), bradycardia (8.3%), hypertension (33.3%), postural hypotension (35%), urinary sphincteric disturbances (20.8%) and anhydrosis of lower limbs (12.5%). Assessment of cardiovascular responses to autonomic function tests revealed impaired alterations in heart rate during deep breathing (31.6%), Valsalva's manoeuvre (28.6%), sustained handgrip (25%), cold‐pressor test (36.4%), postural change (35%) and atropine test (20%); and impaired rise in blood pressure during firm handgrip (25%) and cold‐pressor test (36.6%). ECG abnormalities were noticed in 8 (33.3%) patients. They comprised of depressed ST segment in 5, inverted T wave in 3, tall T wave in 2 and prolonged QTc in 2 patients. Two patients died of respiratory failure. Autonomic dysfunction in Guillain‐Barré syndrome did not appear to have any prognostic significance as there was no significant difference in autonomic dysfunction between good—and bad—outcome g

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07902.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—Two hundred consecutive patients affected by essential tremor (ET), referred to the Movement Disorders Center of the University of Bologna between January 1977 and December 1981, were studied to identify the different clinical features of this disorder, and its associated neurological signs. In this series, 169 patients suffered from typical essential tremor (TET); in the other 31 cases uncommon clinical features were detected: 25 presented postural, action and rest tremor (continuous tremor: CT); 5 had orthostatic tremor (OT) and I was affected by writing tremor (WT). Buccolinguofacial dyskinesias (BLFD) were associated with ET in 19 cases; 2 presented dystonia, and in 6 mild extrapyramidal signs were detected. Uncommon types of tremor: ET plus BLFD and ET associated with extrapyramidal signs had an higher incidence in older people. No patient developed Parkinson's disease (PD) during the follow‐up period (2–5 y

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07903.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—To support the hypothesis of an immunopathogenesis of polyneuropathy in Waldenström's macroglobulinaemia (MW), serum IgM fractions of MW patients were applied intraperitoneally to mice for 17 days. Sections of liver, kidney,M. glutaeus maximus,central nervous system (CNS) and bothNn. ischiadiciwere examined for IgM, IgG, C3 and as control IgD with PAP‐immunostaining. IgM deposits were found in every organ except the CNS. In peripheral nerves larger amounts were visualized in perineurium and endoneural space, whereas myelin lamellae and periaxon did not stain. Therefore, perhaps our investigation reveals a greater permeability of the blood‐nerve barrier (BNB) compared with the blood‐brain barrier (BBB). The involvement of the monoclonal IgM of MW, which has been shown to reactin vitrowith peripheral nerve constituents, appears possible in the pathogenesis of polyne

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07904.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—Thirty‐seven cases that showed bilateral basal ganglia calcification (BGC) were found in 5987 patients. These cases (0.6%) were studied in relation to their CT findings, underlying diseases and epilepsy. CT findings of BGC were divided into “localized” type (33 cases) and “diffuse” type (4 cases). The number of patients with the “localized” type clearly seemed to increase with age. The M:F ratio of the “localized” type was 1:2. The “localized” type was seen in both idiopathic BGC and familial BGC. The “diffuse” type was seen in hypoparathyroidism only. The specific relationship of these two types of BGC to underlying diseases, however, does not fully agree with results so far reported. We experienced a case with familial BGC during this study that appears to be only the 15th so far reported. Partial epilepsy occurred in 75% of epilepsy with BGC, but there seemed to be no direct relationship betwe

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07905.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—Immunoglobulin G (IgG) and albumin from unconcentrated cerebrospinal fluid (CSF) and serum of patients with multiple sclerosis (MS) and non‐MS controls were quantitated using enzyme‐linked immunosorbent assay (ELISA). The IgG levels, IgG/albumin ratios and IgG indexes were significantly increased in CSF of MS patients compared to those of non‐MS controls. The method is sensitive, rapid and reproducible and can be applied to routine laboratory use for quantitation of IgG and albumin in unconcentrated CSF from humans as well as in experimental animals used as models for demyelinating d

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07906.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

ABSTRACT—Psychiatric patients with tardive dyskinesia (TD) may be difficult to distinguish from those with Huntington's Disease (HD), who frequently have psychiatric symptoms. This study compared 14 patients with HD with 21 patients (15 schizophrenics and 6 with affective disorder), matched for involuntary movements, using a quantitated neurological examination and other objective and semi‐objective tests. The HD group was significantly more impaired on measures of voluntary movement and saccadic eye movements. When the psychiatric group was subdivided, voluntary motor impairment was most marked in schizophrenics with both TD and parkinsonism. The implications of these findings are discus

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1987.tb07907.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY