Abstract:
:Six patients with congenital myotonia and 4 patients with myotonic dystrophy have been examined clinically before and after the administration of N-propyl-ajmalin, an alkaloid frequently used as a cardiac antiarrhythmic drug. All patients but one reported a good to moderate improvement of their myotonic muscle stiffness. This was verified by measuring the time the patients needed to ascend a flight of stairs and by recording the speed of opening the hand. The amplitude of the compound muscle action potential decreased during repetitive nerve stimulation in myotonic patients. This decrease was not influenced by N-propyl-ajmalin. It seems to be due to the increased after-depolarization observed in myotonic fibres which causes partial inactivation of the Na-carrying system. From one patient a muscle biopsy was taken and intracellular potentials were measured with a microelectrode. Almost all muscle cells investigated showed myotonic activity which was completely abolished by addition of 10(-5) g/ml N-propyl-ajmalin to the bathing fluid. The development and duration of "warm-up" is illustrated and a possible electrophysiological basis is discussed.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Birnberger KL,Rüdel R,Struppler Adoi
10.1007/BF00316381subject
Has Abstractpub_date
1975-09-01 00:00:00pages
99-110issue
2eissn
0340-5354issn
1432-1459journal_volume
210pub_type
杂志文章abstract::A myasthenia gravis (MG) patient who seems to have recovered can later have recurrence of myasthenic signs. Clearly clinical remission does not always correspond to the normalization of all the factors involved in the pathogenesis of the disease. In ten patients who had apparently recovered from MG, electromyographic ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313771
更新日期:1982-01-01 00:00:00
abstract::The symptomatology of 18 patients with the severest clinical course after subarachnoid hemorrhage (SAH) is described and analyzed. Seventeen patients died, five with an acute bulbar syndrome with cardiac arrest, and 12 with irreversible breakdown of brain function. One patient had an apallic syndrome with minimal sign...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313005
更新日期:1979-01-01 00:00:00
abstract:OBJECTIVES:The outcome of aphasia at 3 months is variable in patients with moderate/severe stroke. The aim was to predict 3-month aphasia outcome using prediction models including initial severity in addition to the interaction between lesion size and location at the acute phase. METHODS:Patients with post-stroke apha...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09259-3
更新日期:2019-06-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10004-4
更新日期:2020-11-01 00:00:00
abstract::The long-duration response (LDR) to levodopa is an important component of the therapeutic response to the drug in Parkinson's disease (PD). Some characteristics are peculiar: it is independent from peripheral pharmacokinetics of levodopa, but it is dependent on the intervals between doses and on the size of each dose....
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-010-5731-0
更新日期:2010-11-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-010-5667-4
更新日期:2010-11-01 00:00:00
abstract::Cerebral venous thrombosis (CVT) affects mainly working-aged individuals. Functional recovery after CVT is generally considered good with about 3/4 of patients achieving short-term independence. However, vascular events, long-term functional outcome, and employment after CVT remain poorly investigated. We identified c...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-015-7996-9
更新日期:2016-03-01 00:00:00
abstract::The APOE*4 allele of the apolipoprotein E gene increases the risk of Alzheimer's disease (AD), but whether it also affects the course of the disease is controversial. However, all studies on this issue until now have been based on patients at various stages of disease. In the present population-based study, 97 patient...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050351
更新日期:1999-04-01 00:00:00
abstract::Four patients from three unrelated families, with clinical and electrophysiological findings compatible with the diagnosis of hereditary motor and sensory neuropathy, are presented. The molecular analysis showed that the affected individuals were homozygous for the mutation in the X25 gene, characteristic of Friedreic...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0902-2
更新日期:2002-11-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10086-0
更新日期:2020-12-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00878874
更新日期:1995-05-01 00:00:00
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journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150170117
更新日期:2001-08-01 00:00:00
abstract::Clinical data and the serum and cerebrospinal fluid (CSF) findings of 71 patients with Guillain-Barré syndrome (GBS), 7 with Fisher syndrome and 24 with chronic inflammatory polyradiculoneuropathy (CIP), were analysed. Isoelectric focusing of serum and CSF together with different formulae and diagrams were applied to ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314019
更新日期:1986-08-01 00:00:00
abstract:OBJECTIVES:Recent retrospective studies found sleep disorders, including obstructive sleep apnea and its symptoms to occur more often in patients following aneurysmal subarachnoid hemorrhage, but studies investigating the incidence of subarachnoid hemorrhage in patients with diagnosed obstructive sleep apnea [OSA] comp...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09265-5
更新日期:2019-06-01 00:00:00
abstract::Temporal lobe epilepsy has been usually associated with progressive brain atrophy due to neuronal cell loss. However, recent animal models demonstrated a dual effect of epileptic seizures with initial enhancement of hippocampal neurogenesis followed by abnormal astrocyte proliferation and neurogenesis depletion in the...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09546-z
更新日期:2019-12-01 00:00:00
abstract::Natalizumab (TYSABRI(®)), a specific α4-integrin antagonist, is approved as a second-line treatment of relapsing-remitting MS (RRMS) patients who fail therapy with interferons or as first-line treatment of patients with highly active relapsing-remitting disease. Since the market introduction of natalizumab as a monoth...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-011-6116-8
更新日期:2011-11-01 00:00:00
abstract::Bcl2-associated athanogene 3 (BAG3) gene mutations cause dilated cardiomyopathy and myofibrillar myopathy. Recently, a novel c.625C>T (p.Pro209Ser) mutation in BAG3 was reported to cause axonal Charcot-Marie-Tooth (CMT) disease in three families. Here, we describe two patients with adult-onset and moderate CMT in a Ch...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09680-8
更新日期:2020-04-01 00:00:00
abstract:INTRODUCTION:PCT is used in the diagnosis of acute neurological syndromes, particularly stroke. We aimed to evaluate PCT abnormalities in patients with acute epileptic seizures or status epilepticus (SE). METHODS:We collected patients undergoing acute PCT for the suspicion of acute ischemic stroke (AIS), who received ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9095-1
更新日期:2018-12-01 00:00:00
abstract::The diagnosis of neurosarcoidosis is often difficult; the imaging signs of spinal cord sarcoidosis sometimes mimic those of cervical spondylotic myelopathy, which is common in elderly persons. We examined the characteristics of spinal cord sarcoidosis in Japanese patients with neurosarcoidosis. This case series identi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6080-3
更新日期:2011-12-01 00:00:00
abstract::Isolated floccular infarction is extremely rare, and impairments of the vestibulo-ocular reflex (VOR) have not been explored in humans with isolated floccular lesions. The purpose of this study was to examine and report selective impairment of VOR in response to high acceleration using head impulse in a patient with i...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6837-y
更新日期:2013-06-01 00:00:00
abstract::We performed the first population-based case-control study on clinical risk factors and drug exposure in Guillain-Barré syndrome (GBS). Sixty patients with GBS were collected through an incidence survey performed in the Emilia-Romagna region of northern Italy. GBS patients were compared with 109 hospital controls (HC)...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050504
更新日期:1999-11-01 00:00:00
abstract::A clinical and electrophological study was performed on 30 patients with chronic carbon disulphide poisoning. Although the measurements of motor conduction velocity and of terminal latency were within the normal range in the subclinical stage, estimation of nerve excitability threshold showed distal motor hypoexcitabi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313208
更新日期:1980-01-01 00:00:00
abstract::The concentration of neopterin was measured in serum samples taken from individuals infected with HTLV-I: 5 from asymptomatic individuals, 1 from a patient with adult T-cell leukaemia and 30 from patients with tropical spastic paraparesis (TSP). In addition, cerebrospinal fluid (CSF) was available from 22 of the TSP p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00810351
更新日期:1992-05-01 00:00:00
abstract::Two sexually active female patients presented with acute meningitis. The CSF abnormalities were severe and persistent. In spite of the absence of genital lesions, serological studies revealed a primary infection by herpes simplex virus type 2. An immunoblot study revealed intrathecal synthesis of anti-herpes antibodie...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314743
更新日期:1990-08-01 00:00:00
abstract::Our knowledge of the vestibular sensory system, its functional significance for gaze and posture stabilization, and its capability to ensure accurate spatial orientation perception and spatial navigation has greatly benefitted from experimental approaches using a variety of vertebrate species. This review summarizes t...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-015-7909-y
更新日期:2016-04-01 00:00:00
abstract:OBJECTIVE:We aimed to assess the effectiveness of conservative treatments in children with tension-type headaches (TTH). METHOD:We searched Medline, Embase and the Cochrane Library and performed reference checking. Two independent authors assessed trial quality and extracted data. Analysis was carried out according to...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-005-0940-7
更新日期:2005-10-01 00:00:00
abstract::Sera from 23 patients with acute Guillain Barré syndrome (GBS), 15 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and from 40 age-matched blood donors were analysed for antibodies to acidic glycosphingolipids from human brain and peripheral nerve. Antibodies to ganglioside LM1, the majo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00315684
更新日期:1991-04-01 00:00:00
abstract::The finding of a mutation in the alpha-synuclein gene in a rare autosomal dominant form of idiopathic Parkinson's disease (IPD), has prompted increased interest in identifying genes that account for the more common sporadic form. A number of association studies have suggested that functional polymorphisms in genes tha...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150050258
更新日期:1998-10-01 00:00:00
abstract::Multiple Sclerosis (MS) is an inflammatory disease of the Central Nervous System with multifocal areas of demyelination. Although its etiology and pathogenesis remain controversial, several lines of evidence indicate that MS is mediated by a misdirected immune response against one or several myelin proteins. The invol...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150200026
更新日期:2002-04-01 00:00:00
abstract:BACKGROUNDS:Wake-Up Stroke (WUS) patients are generally excluded from thrombolytic therapy (rTPA) due to the unknown time of stroke onset. This study aimed to investigate the effects of rTPA in WUS patients during every day clinical scenarios, by measuring ischemic lesion volume and functional outcomes compared to non-...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09603-7
更新日期:2020-03-01 00:00:00