- Understanding patient-reported outcome measures in Huntington disease: at what point is cognitive impairment related to poor measurement reliability? [Journal Article]
- QLQual Life Res 2018 Jun 16
- CONCLUSIONS: Results indicate that the psychometric reliability of PROs can be compromised as HD symptoms progress and cognition declines. Clinicians should consider PROs in conjunction with other types of assessments when total cognition scores exceed critical thresholds.
- Conscious sedation with midazolam intravenously for a patient with Parkinson's disease and unpredictable chorea-like dyskinesia. [Journal Article]
- BJBr J Oral Maxillofac Surg 2018 Jun 13
- Oral surgery can be difficult in patients with chorea-like dyskinesia, which is common in those on long-term levodopa medication for Parkinson's disease, and we know of no conclusive evidence to indi...
Oral surgery can be difficult in patients with chorea-like dyskinesia, which is common in those on long-term levodopa medication for Parkinson's disease, and we know of no conclusive evidence to indicate whether conscious sedation with midazolam is effective in such cases. We report a patient in whom levodopa-induced chorea-like dyskinesia disappeared when midazolam was given intravenously for conscious sedation.
- A Review of Scales to Evaluate Sleep Disturbances in Movement Disorders. [Review]
- FNFront Neurol 2018; 9:369
- Patients with movement disorders have a high prevalence of sleep disturbances that can be classified as (1) nocturnal sleep symptoms, such as insomnia, nocturia, restless legs syndrome (RLS), periodi...
Patients with movement disorders have a high prevalence of sleep disturbances that can be classified as (1) nocturnal sleep symptoms, such as insomnia, nocturia, restless legs syndrome (RLS), periodic limb movements (PLM), obstructive sleep apnea (OSA), and REM sleep behavior disorder; and (2) diurnal problems that include excessive daytime sleepiness (EDS) and sleep attacks. The objective of this review is to provide a practical overview of the most relevant scales that assess these disturbances to guide the choice of the most useful instrument/s depending on the line of research or clinical focus. For each scale, the reader will find a brief description of practicalities and psychometric properties, use in movement disorder cohorts and analyzed strengths and limitations. To assess insomnia, the Pittsburgh Sleep Quality Index, a generic scale, and three disease-specific scales: the Parkinson Disease Sleep Scale (PDSS), the PDSS-2, and Scales for outcomes in Parkinson's disease (PD)-Sleep-Nocturnal Sleep subscale are discussed. To evaluate nocturia, there are no specific tools, but some extensively validated generic urinary symptom scales (the Overall Bladder Questionnaire and the Overactive Bladder Symptom Score) and some PD-specific scales that include a nocturia item are available. To measure RLS severity, there are currently four domain-specific generic scales: The International Restless Legs Scale, the Johns Hopkins Restless Legs Severity Scale, the Restless Legs Syndrome-6 measure, a Pediatric RLS Severity Scale, and the Augmentation Severity Rating Scale (a scale to evaluate augmentation under treatment) and several instruments that assess impact on quality of sleep and health-related quality of life. To evaluate the presence of PLM, no clinical scales have been developed to date. As far as OSA, commonly used instruments such as the Sleep Apnea Scale of the Sleep Disorders Questionnaire, the STOP-Bang questionnaire, and the Berlin Questionnaire are reviewed. Three scales have been extensively used to assess EDS: the generic Epworth Sleepiness Scale, the Stanford Sleepiness Scale, and the PD-specific Scales for outcomes in PD-Sleep-Daytime sleepiness subscale. To date, only the Inappropriate Sleep Composite Score specifically evaluates propensity to sleep attacks.
- Neurodegeneration as the presenting symptom in 2 adults with xeroderma pigmentosum complementation group F. [Journal Article]
- NGNeurol Genet 2018; 4(3):e240
- CONCLUSIONS: These cases illustrate the role of NER dysfunction in neurodegeneration and how adult-onset neurodegeneration could be the major symptom bringing XP-F patients to clinical attention. XP-F should be considered by neurologists in the differential diagnosis of patients with adult-onset progressive neurodegeneration accompanied by global brain atrophy and a history of heightened sun sensitivity, excessive freckling, and skin malignancies.
- A need to apply unified criteria to choreoballism associated with glycemic derangement. [Letter]
- PRParkinsonism Relat Disord 2018 Jun 05
- Drug treatment. [Journal Article]
- HCHandb Clin Neurol 2018; 155:371-377
- There are no approved disease-modifying therapies for any of the inherited cerebellar ataxias. Drug treatment in childhood ataxia is still very limited. Effective treatments are available for only a ...
There are no approved disease-modifying therapies for any of the inherited cerebellar ataxias. Drug treatment in childhood ataxia is still very limited. Effective treatments are available for only a few rare metabolic hereditary disorders. Symptomatic management of associated tremor, spasticity, dystonia, or chorea can follow the medication recommendations in general usage. The foundation of management of cerebellar ataxia in adults or children remains rehabilitation.
- IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes. [Journal Article]
- FNFront Neurol 2018; 9:329
- IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms wherea...
IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms whereas dysautonomy and cognitive decline usually appear in later stages of the disease. We report a case of a patient with IgLON5-associated encephalitis presenting with rapidly progressive cognitive decline and atypical inflammatory lesions on brain magnetic resonance imaging, oligoclonal bands on cerebrospinal fluid, anti-IgLON5 antibodies exclusively of the IgG1 class, and a fierce inflammatory reaction on brain biopsy, who responded favorably to immunotherapy.
- Long-term outcome of Gamma Knife radiosurgery for symptomatic brainstem cavernous malformation. [Journal Article]
- WNWorld Neurosurg 2018 Jun 01
- CONCLUSIONS: GKS for s-BSCM is safe and effective alternative to surgical resection for reducing the rate of recurrent hemorrhage. Because the annual hemorrhage rate increases more than 5 years after GKS, clinicians should monitor patients closely to determine their subsequent treatment.
- [Chorea due to chronic subdural hematoma]. [Journal Article]
- RSRinsho Shinkeigaku 2018 Jun 01
- An 86-year-old woman presented with generalized chorea in the face and extremities, which gradually progressed for two weeks. Cranial CT revealed a chronic subdural hematoma (CSDH) that covered the l...
An 86-year-old woman presented with generalized chorea in the face and extremities, which gradually progressed for two weeks. Cranial CT revealed a chronic subdural hematoma (CSDH) that covered the left parietal lobe. Discontinuation of amantadine did not improve the chorea. The hematoma was evacuated and the chorea completely subsided in a week. The pathogenesis leading to chorea in CSDH remains unclear. A unilateral hematoma presenting with generalized chorea similar to the present patient and two others with unilateral CSDH causing ipsilateral hemichorea have been reported. The rarity of these movement disorders due to CSDH indicates that these patients had a preclinical dysfunction within neuronal networks interconnecting basal ganglia the cerebral cortex. Our findings confirmed that CSDH could cause chorea, and further neuroimaging to evaluate cerebrovascular disease, taking a detailed family history and obtaining information about current medications might reveal factors likely to precipitate the development of chorea.
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- Effect of Risperidone on the Motor and Functional Disability in Children With Choreoathetoid Cerebral Palsy. [Journal Article]
- PNPediatr Neurol 2018 Apr 20
- CONCLUSIONS: Risperidone is a promising drug to manage children with choreoathetoid cerebral palsy and is well tolerated in children.