Abstract:
BACKGROUND:The symptomatic treatment of myotonia and myalgia in patients with dystrophic and non-dystrophic myotonias is often not satisfactory. Some patients anecdotally report symptoms' relief through consumption of cannabis. METHODS:A combination of cannabidiol and tetrahydrocannabinol (CBD/THC) was prescribed as compassionate use to six patients (four patients with myotonic dystrophy types 1 and 2, and 2 patients with CLCN1-myotonia) with therapy-resistant myotonia and myalgia. CBD/THC oil was administered on a low dose in the first 2 weeks and adjusted to a higher dose in the following 2 weeks. Myotonia behaviour scale (MBS), hand-opening time, visual analogue scales (VAS) for myalgia and myotonia, and fatigue and daytime sleepiness severity scale (FSS, ESS) were performed weekly to monitor treatment response. RESULTS:All patients reported an improvement of myotonia especially in weeks 3 and 4 of treatment: MBS improved of at least 2 points in all patients, the hand-opening time variously improved in 5 out of 6 patients. Chronic myalgia was reported by both DM2 patients at baseline, one of them experienced a significant improvement of myalgia under treatment. Some gastrointestinal complaints, as abdominal pain and diarrhoea, improved in 3 patients; however, 4 out of 6 patients reported new-onset constipation. No other relevant side effect was noticed. CONCLUSIONS:These first empirical results suggest a potentially beneficial role of CBD/THC in alleviating myotonia and should encourage further research in this field including a randomized-controlled trial on larger cohorts.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Montagnese F,Stahl K,Wenninger S,Schoser Bdoi
10.1007/s00415-019-09593-6subject
Has Abstractpub_date
2020-02-01 00:00:00pages
415-421issue
2eissn
0340-5354issn
1432-1459pii
10.1007/s00415-019-09593-6journal_volume
267pub_type
杂志文章abstract:BACKGROUND:The significance of neutrophil-to-lymphocyte ratio (NLR) has been explored in different diseases. Few studies addressed its role in patients with multiple sclerosis (MS), with promising results regarding its association with disease activity or disability. OBJECTIVES:We aimed at confirming the role of NLR a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10322-7
更新日期:2021-01-03 00:00:00
abstract:BACKGROUND:Progression of parkinsonian motor impairment is usually rapid and relentless in multiple system atrophy (MSA). However, it may also be subject to considerable variation. Prospective natural history studies using validated rating scales are required to accurately determine the progression of parkinsonism in M...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0617-2
更新日期:2005-01-01 00:00:00
abstract:BACKGROUND:Spinal muscular atrophy (SMA) is caused by a homozygous deletion of the survival motor neuron (SMN)1 gene. The nearly identical SMN2 gene plays a disease modifying role. SMA is classified into four different subtypes based on age of onset and clinical course (SMA types 1-4). The natural history of early onse...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0929-0
更新日期:2008-09-01 00:00:00
abstract:OBJECTIVE:To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6. METHODS:Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched health...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0907-6
更新日期:2008-08-01 00:00:00
abstract::A method for the preparation of a potent and specific peroxidase-labelled antihuman IgG antibody is described. CSF cells from patients with various neurological diseases were stained. Only in cases of inflammatory diseases was IgG detectable. The stained cells were so-called lymphoid cells. As the demonstrable IgG can...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313346
更新日期:1980-01-01 00:00:00
abstract::Freezing of gait (FOG) is a disabling motor symptom experienced by a large proportion of patients with Parkinson's disease (PD). While it is known that FOG contributes to lower health-related quality of life (HRQoL), previous studies have not accounted for other important factors when measuring the specific impact of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7524-3
更新日期:2015-01-01 00:00:00
abstract:OBJECTIVE:Amyotrophic lateral sclerosis type 8 (ALS8) is a familial form of motor neuron disease, with predominance of lower motor neuron degeneration, and is caused by mutation of the vesicle-associated membrane protein-associated protein B (VAPB). We aimed to compare the cognitive profile of patients with ALS8 and he...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09369-y
更新日期:2019-08-01 00:00:00
abstract::The aim of the study was to identify the main factors that impact mobility impairment in multiple sclerosis (MS) patients in Italy. Clinicians from a large number of Italian MS centers took part in a Delphi process aimed at obtaining consensus statements among the participants. Large consensus was obtained for stateme...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7230-6
更新日期:2014-03-01 00:00:00
abstract::Pontocerebellar hypoplasia type 2 (PCH2) is a very rare autosomal recessive disorder. We report two unrelated female patients born to consanguineous parents presenting with this condition. Patient 1 showed a classical clinical/neuroradiological phenotype of PCH2 with dyskinesia/dystonia. Patient 2 had a neonatal onset...
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pub_type: 杂志文章
doi:10.1007/s004150200069
更新日期:2002-05-01 00:00:00
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pub_type: 杂志文章,meta分析,评审
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abstract:BACKGROUND:The vegetative state, also known as the unresponsive wakefulness syndrome, is one of the worst possible outcomes of acquired brain injury and confronts rehabilitation specialists with various challenges. Emergence to (minimal) consciousness is classically considered unlikely beyond 3-6 months after non-traum...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09542-3
更新日期:2019-12-01 00:00:00
abstract::Rapid-eye-movement (REM) sleep behavior disorder (RBD) is a parasomnia characterized by complex motor activity associated with dreaming during REM sleep. RBD may be idiopathic or associated with various neurological diseases involving the brainstem. The association of RBD and limbic system impairment was unclear. We r...
journal_title:Journal of neurology
pub_type: 信件
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更新日期:2009-07-01 00:00:00
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journal_title:Journal of neurology
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doi:10.1007/s00415-020-10241-7
更新日期:2020-09-26 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0430-y
更新日期:2005-03-01 00:00:00
abstract::To determine the epidemiological, clinical, and genetic characteristics of congenital hypomyelinating leukodystrophies, including Pelizaeus-Merzbacher disease (PMD), we conducted a nationwide epidemiological survey in Japan. A two-step survey targeting all medical institutions specializing in pediatric neurology and c...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7263-5
更新日期:2014-04-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5458-y
更新日期:2010-07-01 00:00:00
abstract::We analysed the kinetics and clinical impact of binding antibodies (BAbs) and neutralizing antibodies (NAbs) to three interferon beta (IFNbeta) products in patients with relapsing-remitting MS (RRMS). Patients with RRMS received IFNbeta-1b 8 MIU subcutaneously (SC) every other day, intramuscular (IM) IFNbeta-1a 30 mcg...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0312-8
更新日期:2004-03-01 00:00:00
abstract::Production of reactive oxygen species after cerebral blood flow disruption may enhance tissue damage through multiple molecular pathways. Changes in nitric oxide (NO) metabolism and oxidative stress status were investigated in 47 patients with ischemic stroke by measuring plasma nitric oxide (NO) and peroxynitrite (ON...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-007-0700-y
更新日期:2008-01-01 00:00:00
abstract::Oculomotor dysfunction in Parkinson's disease (PD) is mainly characterized by a fragmentation of memory-guided gaze shifts (target is reached by several hypometric saccades). Since this phenomenon can also be observed in normal subjects, we scrutinized its pathophysiological significance in PD patients. We recorded ho...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0691-7
更新日期:2002-06-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00936890
更新日期:1995-02-01 00:00:00
abstract::The neurochemical abnormalities underlying vascular parkinsonism (VP) have not been well characterised. A better understanding may help to optimize pharmacological interventions. Since VP patients generally have a poorer response to l-Dopa than Parkinson's disease (PD) patients, we investigated whether levels of relev...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7126-5
更新日期:2013-12-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-7012-5
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abstract::Auto-oxidation of levodopa generates toxic metabolites, such as free radicals, semiquinones and quinones. In vitro, levodopa is a powerful toxin that is lethal to cultures of neurones. This raises the concern that levodopa may also be toxic in vivo, and that chronic treatment with levodopa could induce further damage ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00022914
更新日期:2000-04-01 00:00:00
abstract::Biomarkers beyond clinical assessment are needed in patients who suffer from amyotrophic lateral sclerosis (ALS). Here, single-voxel proton magnetic resonance spectroscopy ((1)H MRS) of the gray matter of the motor cortex and the white matter including the pyramidal tracts was used to investigate concentrations of N-a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0495-2
更新日期:2007-08-01 00:00:00
abstract::Two siblings with Lafora disease (LD) are described: one with epilepsy, myoclonus, EEG abnormalities, severe dementia and many Lafora bodies (LBs) in muscle and skin tissue; the other with myoclonus, epilepsy, EEG abnormalities and LBs in muscle and in skin tissue, without dementia. The findings suggest that the diagn...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314784
更新日期:1991-07-01 00:00:00
abstract::Additional autoimmune diseases in people with multiple sclerosis (MS) and their relatives have been studied many times. Studies have employed different designs, and yielded conflicting results. We performed a systematic review, and calculated overall risk of additional autoimmune diseases in people with MS and their f...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-012-6790-1
更新日期:2013-05-01 00:00:00
abstract::Cerebellar ataxias (CAs) represent a heterogeneous group of sporadic or inherited disorders. The clinical spectrum of CAs is continuously expanding. Our understanding of the mechanisms leading to the clinical deficits has improved over these last decades, in particular thanks to progress in genetics, neuroimaging and ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-09717-3
更新日期:2020-04-01 00:00:00
abstract::Myasthenia gravis patients usually present with fluctuating, asymmetrical stress-dependent weakness in the absence of other neurological disturbances. In weak patients psychopathological disturbances are frequently reported and misdiagnosed as a psychiatric disorder. The question to what extent psychiatric symptoms ar...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-007-2022-5
更新日期:2007-05-01 00:00:00
abstract::Psychiatric complaints are common in Parkinson's disease (PD), and have a significant influence in disease outcome and quality of life. Little attention has been paid to psychiatric symptoms at early stage disease. We aimed to screen a population of early stage PD patients for psychiatric symptoms and to study the rel...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6140-8
更新日期:2012-01-01 00:00:00
abstract::We assessed the prevalence of vertebral artery (VA) stenosis or occlusion and its influence on outcome in patients with acute basilar artery occlusion (BAO). We studied 141 patients with acute BAO enrolled in the Basilar Artery International Cooperation Study (BASICS) registry of whom baseline CT angiography (CTA) of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7583-5
更新日期:2015-02-01 00:00:00