Abstract:
:We present 2 sibs with autosomal recessive spastic paraparesis and severe amyotrophy of the distal limb muscles. Elaborate neurophysiologic studies disclosed slight to moderate slowing of motor conduction, moderate to severe reduction of motor action potentials, denervation potentials, and increased distal motor latencies. This syndrome, not having been reported since the papers by Ormerod (5) in 1904 and Holmes (6) in 1905, constitutes another rare, benign, complicated form of hereditary spastic paraparesis.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Bruyn RP,Scheltens P,Lycklama à Nijeholt J,de Jong JMdoi
10.1111/j.1600-0404.1993.tb04133.xsubject
Has Abstractpub_date
1993-06-01 00:00:00pages
443-5issue
6eissn
0001-6314issn
1600-0404journal_volume
87pub_type
杂志文章abstract:MATERIALS AND METHODS:In a prospective study we evaluated patients with first generalized tonic-clonic seizure (GTCS) (n = 16, age: 31 +/- 11 years, 8 women) and patients with vaso-vagal syncope (VVS) (n = 17, age: 32 +/- 13 years, 8 women), diagnosed on the basis of past history and clinical presentation who had serum...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1997.tb00084.x
更新日期:1997-03-01 00:00:00
abstract::This article reports the case of a man who developed a pure motor paraplegia following epidural anesthesia with a pattern of slow recovery over subsequent months. Reviewing the available literature on post-epidural paraplegia we noted a number of potential etiologies and analyzed their role in its causation. On the ba...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:
更新日期:1985-10-01 00:00:00
abstract::A case of necrotizing brain stem encephalitis due to listeria monocytogenes is described in a 48-year-old man who had brain stem encephalitis of complicated course and with selective destruction of the vasomotoric and respiratory centers. He developed that very rare Ondine's curse syndrome, being only able to breathe ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1988.tb05949.x
更新日期:1988-06-01 00:00:00
abstract:OBJECTIVES:To address the relationship between years lived with a disability (YLDs), prevalence and cost of neurological diseases, and to test whether there is a European North-South gradient for national health expenditure, disability, costs and prevalence of neurological diseases. MATERIALS AND METHODS:Information o...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12339
更新日期:2015-07-01 00:00:00
abstract:OBJECTIVE:To clarify how chronic solvent abuse affects cognitive function using magnetic resonance imaging as an index of brain damage. MATERIAL AND METHODS:A total of 25 chronic solvent abusers underwent magnetic resonance imaging with quantitative neurometry and Wechsler Adult Intelligence Scales revised. RESULTS:T...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1997.tb00235.x
更新日期:1997-07-01 00:00:00
abstract::Amino acid concentrations in plasma of patients with Huntington's disease (HD) were determined in 16 patients and 21 age- and sex-matched healthy controls. Alanine and isoleucine were significantly decreased in HD plasma whereas arginine, histidine, leucine, lysine, ornithine, proline, serine, threonine, tyrosine, and...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1995.tb00438.x
更新日期:1995-03-01 00:00:00
abstract::Symptoms of acute renal failure were observed in an anorexia patient who had a history of frequent vomiting. The acute renal failure might have resulted from rhabdomyolysis subsequent to a disturbance of electrolyte balance. This acute renal failure might have resulted in death if not treated. We postulate that even a...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1990.tb00935.x
更新日期:1990-01-01 00:00:00
abstract::X-linked adrenoleukodystrophy/adrenomyeloneuropathy presents a wide variation of clinical manifestations and may mimic several diseases. A screening investigation by measuring plasma saturated very long-chain fatty acids was performed in two groups of patients. Among six patients with hereditary spastic paraparesis on...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1992.tb04015.x
更新日期:1992-02-01 00:00:00
abstract:OBJECTIVES:Strokes represent the most common etiology of epilepsy in patients over the age of 60 years, with an incidence of 2-4% occurring in different studies. MATERIALS AND METHODS:In this observational study, 1,428 patients were included who had stroke and were admitted to our Stroke Unit between the years 1996 an...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2006.00642.x
更新日期:2006-07-01 00:00:00
abstract::During a thorough surveillance of viral infections of the central nervous system an outbreak of aseptic meningitis was discovered in the western part of Finland in late 1985. The 21 diseased young adults were carefully studied by different virological methods. A presumed viral etiology, in all cases of enteroviral ori...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1987.tb05457.x
更新日期:1987-05-01 00:00:00
abstract::Two new components of the human SEP upon stimulation of the contralateral median nerve at the wrist have been identified. Such components have been called N16 and N17, according to their polarity and latency. N16 and N17, as well as the N14-P15 complex, are generated by separate subcortical dipoles. Particularly, they...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1978.tb02896.x
更新日期:1978-12-01 00:00:00
abstract:OBJECTIVES:Multiple sclerosis (MS) patients suffer from high levels of anxiety. However, it is unclear which factors are related to anxiety, since study results are inconsistent, and the associated factors have not been examined comprehensively. In this study, we investigated the demographic, disease-related and psycho...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13191
更新日期:2020-03-01 00:00:00
abstract:OBJECTIVE:To evaluate the clinical course of patients with myasthenia gravis (MG) up to 3 months after double filtration plasmapheresis (DFP). MATERIAL AND METHODS:We recorded the MG score and measured the level of acetylcholine receptor antibody (AchRAb) at baseline and day 1 (D1), week 1 (W1), 1 month (M1), 2 (M2) a...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2003.00107.x
更新日期:2003-09-01 00:00:00
abstract:BACKGROUND:Some recent studies have found an association between foetal malformations in earlier antiepileptic drug (AED)-exposed pregnancies and an increased hazard of such malformations in subsequent pregnancies. We investigated this matter further, and also considered the possible role of spontaneous abortions in pr...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12672
更新日期:2017-03-01 00:00:00
abstract:OBJECTIVE:To validate the diagnosis of idiopathic intracranial hypertension (IIH) from the Swedish National Patient Register (NPR) and investigate the incidence of IIH, as well as co-morbidities and medication use in a large Swedish population-based sample. METHODS:We searched the NPR to find all patients ≥18 years ol...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12742
更新日期:2017-11-01 00:00:00
abstract::Twenty-four consecutive patients with familial amyloidosis with polyneuropathy (type 1), who were at different stages of the disease were investigated. The purpose was to report the electrophysiological features and to compare them with those found in other generalized neuropathies; 12 cases were familiar and 12 cases...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1981.tb00753.x
更新日期:1981-02-01 00:00:00
abstract::An epidemiological survey of multiple sclerosis was carried out in the western part of Poland. The estimated prevalence rate on December 31, 1981 was compared with that established on the same territory on January 1, 1965. The disease was less frequent in 1981. The prevalence rate for definite cases was only 42.87 ver...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1985.tb00866.x
更新日期:1985-08-01 00:00:00
abstract::We studied clinically and electrophysiologically 8 patients affected by orthostatic tremor (OT), which is an unusual movement disorder consisting of shaking movements of the legs and trunk in the standing position. We failed to find any cause in 6 of the 8 cases. In 2 patients OT was clearly secondary to neurologic di...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1990.tb00944.x
更新日期:1990-02-01 00:00:00
abstract:OBJECTIVES:The GM2 gangliosidoses are a group of genetic disorders caused by the accumulation of ganglioside GM2 in neuronal cells. We examined the alpha- and beta-subunits of beta-hexosaminidases by a non-radioisotopes detecting system to evaluate whether it was a useful method for understanding of the pathophysiologi...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2002.01097.x
更新日期:2002-06-01 00:00:00
abstract::Paralytic poliomyelitis presenting with quadriparesis, transient encephalitis and bulbar symptoms in 2 patients in close contact with recently vaccinated children with trivalent live oral polio vaccine is described. Symmetrical lower motor neuron involvement of deltoid muscles with electromyographic confirmation was f...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1987.tb03569.x
更新日期:1987-09-01 00:00:00
abstract:OBJECTIVES:To estimate the prevalence of thyroid diseases and the cumulative risk of thyroid diseases during a follow-up period after myasthenia gravis (MG) diagnosis compared with non-MG controls. MATERIALS AND METHODS:We used the Taiwan National Health Insurance Database linked to Registry of Catastrophic Illness da...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13254
更新日期:2020-09-01 00:00:00
abstract::Two patients who developed rhabdomyolysis secondary to generalized seizures are presented. Minor traumas of skeletal muscles due to seizures may be associated with myoglobinuria and the development of acute renal failure. The diagnosis rhabdomyolysis may easily be confirmed by following serum creatine kinase. Myoglobi...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1989.tb03746.x
更新日期:1989-03-01 00:00:00
abstract:OBJECTIVE:To evaluate the epidemiological and clinical features of motor neuron disease (MND) in a region (835,000 inhabitants) of south-western Greece. PATIENTS AND METHODS:The medical records of all patients diagnosed with adult-onset MND at the Department of Neurology of the University Hospital of Patras from 1990 ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2004.00362.x
更新日期:2005-02-01 00:00:00
abstract::The effects of bromocriptine in patients with Parkinson's disease manifesting various problems in levodopa therapy were tested in a double-blind manner with the collaboration of 59 institutions. The slow and low principle was in part adopted. Either bromocriptine or placebo was added to levodopa. Twenty-nine % of the ...
journal_title:Acta neurologica Scandinavica
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0404.1985.tb00858.x
更新日期:1985-08-01 00:00:00
abstract:OBJECTIVES:Interindividual variability in intestinal absorption and bioavailability might contribute to inadequate control of seizures under treatment with carbamazepine and phenytoin. We therefore correlated intestinal expression levels and genetics of CYP3A4, CYP2C9/19, MDR1 and MRP2 with dose requirement and plasma ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2006.00761.x
更新日期:2007-04-01 00:00:00
abstract:OBJECTIVE:We evaluated the differences in brain morphology among patients with juvenile myoclonic epilepsy according to the occurrence of absence seizures. MATERIALS AND METHODS:Twenty-one juvenile myoclonic epilepsy patients with (n = 6) and without (n = 15) absence seizures were enrolled. We analyzed whole-brain T1-...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12436
更新日期:2016-02-01 00:00:00
abstract:INTRODUCTION:Cerebral small vessel disease (SVD) contributes to dementia and disability in the elderly, and may negatively affect stroke outcomes. We aimed to evaluate to what extent single features and global burden of SVD detected with magnetic resonance (MR) are associated with worse outcomes in patients with ischae...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12745
更新日期:2017-07-01 00:00:00
abstract:OBJECTIVES:Although the recovery from Guillain-Barré syndrome (GBS) is good in most patients, some develop permanent severe disability or even die. Early predictors would increase the likelihood to identify patients at risk for poor outcome at the acute stage, allowing them intensified therapeutic intervention. MATERI...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12927
更新日期:2018-08-01 00:00:00
abstract::A 54 year-old woman showed the unusual association of a Guillain-Barré syndrome and opsoclonus, a rare eye movement characterized by involuntary bursts of jerking, ataxic and multidirectional saccades, without an intersaccadic interval. Since opsoclonus is a phenomenon described exclusively in CNS diseases, the case r...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1992.tb04061.x
更新日期:1992-05-01 00:00:00
abstract::Electromyograms (EMGs) are usually considered artifacts during electroencephalographic (EEG) recording for determination of electrocerebral silence (ECS). Appearance of scalp motor unit activity, however, may reflect residual brainstem function and can introduce difficulty in establishing a secure diagnosis of brain d...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1986.tb04638.x
更新日期:1986-08-01 00:00:00