Abstract：
:A 47-year-old female developed proximal limb weakness after hysterectomy for uterine fibromatosis. Muscle strength slowly improved, but relapse occurred at age 52. She presented with progressive gait difficulty, proximal limb weakness, and painful calves. Family history was not contributory. Cranial nerves, deep tendon reflexes, and sensation were normal. Serum creatine kinase was normal. An IgG kappa monoclonal protein was found. Nerve conduction studies were normal, but EMG showed brief small polyphasic motor unit action potentials with early recruitment in proximal muscles. Muscle biopsy showed abundant rods, atrophic muscle fibres, and type 1 fibre predominance. The sarcolemma was immunoreactive for IgG kappa. Plasmapheresis was unsuccessful, but methylprednisolone and azathioprine led to moderate improvement of muscle strength, associated with reduced monoclonal protein levels. This is the third case report, describing the association of monoclonal gammopathy and late-onset nemaline myopathy. Presence of a monoclonal protein at the sarcolemma and responsiveness to immunosuppressive treatment are suggestive of a dys-immune origin.
journal_name
Acta Neurol Belgjournal_title
Acta neurologica Belgicaauthors
Deconinck N,Laterre EC,Van den Bergh PYkeywords:
subject
Has Abstractpub_date
2000-03-01 00:00:00pages
34-40issue
1eissn
0300-9009issn
2240-2993journal_volume
100pub_type
杂志文章abstract：:The etiology of chronic subdural hematoma (CSDH) in patients is diverse. The primary objective of this article was to discuss one of the causes, spontaneous intracranial hypotension with spinal cerebrospinal fluid (CSF) leak, which is usually neglected by the neurosurgeon. All the consecutive 15 patients who underwent...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-016-0597-2
更新日期：2016-12-01 00:00:00
abstract：:For a long-term period (25-30 years) the author sums up the results obtained by cervical discectomy through a ventral route followed by interbody arthrodisis. In the disc diseases, the radicular syndromes gave excellent results, the medullar syndromes good results. In the cervical injuries, the results were always goo...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1987-03-01 00:00:00
abstract：:Multiple sclerosis (MS) is a neuro-immunological demyelinating disease. From the immunological aspects, it is well accepted that T cells play a pivotal role in the etiology of the disease. T helper (Th) 1 and Th17 cells are thought to be the main pathogenic T cells in the pathogenesis of MS and are known as effector T...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,meta分析
doi：10.1007/s13760-018-1028-3
更新日期：2019-03-01 00:00:00
abstract：:The first applications of positron emission tomography (PET) for the study of brain tumours appeared early in the development of this technology. New trends in these particular PET applications tend to take into account the histological heterogeneity of these tumours and the necessity to integrate PET data in their su...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：
更新日期：1997-09-01 00:00:00
abstract：:Similar alterations in the neuronal cytoskeleton (paired helical filaments) and modifications in the molecular forms of acetylcholinesterase (specific decrease in the G4 form) have been detected in the elderly rat as well as in patients suffering from SDAT. These observations challenge the specificity of some manifest...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1989-05-01 00:00:00
abstract：PURPOSE:A retrospective evaluation of the ictal temporal tracer uptake and the perfusion pattern in other neo- and subcortical structures was performed to define typical extratemporal changes of rCBF that might further aid the differentiation of temporal and extratemporal seizures. METHOD:Ictal and interictal rCBF bra...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1998-12-01 00:00:00
abstract：:Clinical records and EEGs of 114 consecutive patients with clinical diagnosis of Alzheimer's disease have been reviewed. The EEGs of 15 patients (13%) contained triphasic waves. Triphasic waves were always atypical as they occurred singularly or in short bursts, and/or they had occipital predominance, and/or they were...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1990-01-01 00:00:00
abstract：:To analyze the clinical characteristics and PRRT2 gene mutation of self-limited familial infantile epilepsy and evaluate the treatment responses of different antiepileptic drugs in self-limited familial infantile epilepsy. We reviewed the clinical feature and genetic mutation results and treatment responses of two sib...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-020-01348-9
更新日期：2020-08-01 00:00:00
abstract：:On the basis of eight case studies conducted since 1980 the authors define the concept of subdural hematoma showing a level on the CAT scan image. The concept throws some light on the etiopathogenesis of certain hematoma. ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1983-08-01 00:00:00
abstract：:For the most part, the central nervous system is unable to regenerate. The majority of injuries of vascular, inflammatory, degenerative and traumatic aetiology lead to an irreversible loss of central nervous system function. The paper presents the role of Nogo-A, MAG and OMgp proteins in the inhibition of central nerv...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：10.1007/s13760-015-0425-0
更新日期：2015-12-01 00:00:00
abstract：:Chorea paralytica (or chorea mollis) is a very rare variant of Sydenham's chorea, characterized by a profound hypotonia, resulting in severe disability. Given the rarity of this condition, data on its prognosis are lacking. Most reports suggest that the delay from onset to recover total autonomy is long, usually sever...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-013-0214-6
更新日期：2013-12-01 00:00:00
abstract：:The authors present the clinico-pathological correlation in a women who developed at the age of 43 years progressive pain in the left hemiface with motor and sensitive disorders of the left trigeminal nerve. Progressive involvement of the left IIIrd, IVth and Vth cranial nerves appeared with persistence of intractable...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1980-01-01 00:00:00
abstract：:n-Hexane gives cause to one of the most common toxic polyneuropathies seen in poorly ventilated factories. It is a sensory-motor polyneuropathy ending up with axonal degeneration. Nerve biopsy reveals paranodal axonal swelling and secondary myelin retraction in early stages. Myelin retraction imitates demyelination ca...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-019-01137-z
更新日期：2019-04-13 00:00:00
abstract：:The EEG of 48 patients suffering from either an unilateral or a bilateral thrombosis of the carotid artery are compared, and the most discriminative data of the recordings in these two conditions are stressed. ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1976-01-01 00:00:00
abstract：:Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic process that typically occurs in the setting of immune dysregulation. In contrast to the characteristic pattern involving parieto-occipital and posterior frontal regions, predominant involvement of the infratentorial brain occurs in a minority of PRES...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-015-0431-2
更新日期：2015-12-01 00:00:00
abstract：:Sleep disorders are very common in Parkinson's disease (PD), being associated with several other conditions, mainly psychiatric disorders. The present study was designed to assess sleep quality in Brazilian patients with PD and to evaluate whether sleep changes are associated with clinical variables, especially neurop...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-017-0868-6
更新日期：2018-03-01 00:00:00
abstract：:A case of femoral palsy associated with chronic pain sensible to Carbamazepine, secondary to iliacus haematoma during anticoagulant therapy is reported. Late femoral nerve decompression followed by transcutaneous neurostimulation permit the normalization of the sensory nerve conduction and a complete clinical recovery...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1985-11-01 00:00:00
abstract：:A 54-year-old female patient presented acute hallucinatory psychotic episodes with irritative EEG for seven years before an initial state of complex partial mal. A single convulsive episode occurred 14 years after the beginning of the psychiatric symptoms. The intercritical EEG showed independent temporal lobe foci pr...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1989-01-01 00:00:00
abstract：:Spinal cord stimulation is an accepted treatment for neuropathic pain. Technical advances in electrode design and better patient selection have led to better and sustained pain control by these devices. Multilead electrical stimulation is the latest innovation in implantable electrostimulation (Mattrix, Medtronic Minn...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1998-06-01 00:00:00
abstract：:The percentage of echinocytes in the blood from patients affected by Duchenne muscular dystrophy, in mothers carriers of the dystrophic state, and in Steinert dystrophic patients, has been investigated after addition of low levels of lysolecithin. The echinocyte content was significantly higher in both Duchenne patien...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1981-07-01 00:00:00
abstract：:Early onset dementia (EOD) is a major diagnostic challenge as it often presents with atypical features and may be attributed to treatable diseases. Primary degenerative dementias (Alzheimer's disease-AD, frontotemporal lobar degeneration-FTLD, Lewy body dementia-LBD), although traditionally considered to affect older ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-014-0310-2
更新日期：2015-03-01 00:00:00
abstract：:Among the last 7000 consecutive autopsies from our department, nine cases of primary reticulum cell sarcoma (non hodgkinian malignant lymphoma) of the central nervous system were discovered. Another case was found by cerebral biopsy. There were six males and four females (mean age 43.5 years). Mean duration of illness...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1976-07-01 00:00:00
abstract：:The aim of this study was to investigate personality, by means of the Rorschach Psychodiagnostic test, in a consecutive series of fourteen patients with Parkinson's disease (PD) submitted to bilateral deep brain stimulation of the subthalamic nucleus (DBS STN). Patients were evaluated pre-operatively and 1 year after ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：2008-03-01 00:00:00
abstract：:Smoking is associated with increased multiple sclerosis (MS) risk. In addition, some studies have reported that smoking is associated with anxiety and depression. However, the associations between smoking, walking, and fatigue are needed to be investigated. The objective was to investigate the associations between cig...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-020-01341-2
更新日期：2020-03-28 00:00:00
abstract：BACKGROUND:FDG-PET in ALS most typically demonstrates a primary (and sometimes also supplementary) motor cortex hypometabolism, often associated with more diffuse cortical hypometabolism involving mostly the dorsolateral prefrontal cortex, the medial and lateral premotor cortices, and the bilateral insular cortex invol...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：2011-12-01 00:00:00
abstract：: ...
journal_title：Acta neurologica Belgica
pub_type：
doi：10.1007/s13760-018-1035-4
更新日期：2018-12-01 00:00:00
abstract：:Multiple sclerosis (MS) often starts in the form of clinically isolated syndrome (CIS) and only some of the CIS patients progress to relapsing-remitting MS (RRMS). Biomarkers to predict conversion from CIS to MS are thus greatly needed for making correct treatment decisions. To identify a predictive cerebrospinal flui...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-018-0954-4
更新日期：2019-03-01 00:00:00
abstract：:Three patients with hypereosinophilia showed different forms of peripheral neuropathy: severe polyneuropathy of prevalently sensory type (case 1), mild sensory neuropathy (case 2), acute mononeuropathy of the median nerve with subclinical polyneuropathy (case 3). Hypereosinophilia was probably idiopathic, however the ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1991-01-01 00:00:00
abstract：:Electrophysiological tests (visual and somatosensory evoked potentials, flicker fusion test) and electrophoretic examination of cerebrospinal fluid were performed in 17 patients who previously suffered from one or more attacks of optic neuritis. The association of the tests revealed the demyelinating inflammatory path...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1983-03-01 00:00:00
abstract：:The primary source of malignant intracerebral nerve sheath tumors is still unclear We report the imaging and MR spectroscopic findings in a 39-year-old man with a very rare brain stem tumor MR examination revealed the presence of intraaxial brain stem tumor with a partial exophytic growth. On pathological examination,...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：2008-06-01 00:00:00
