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Neurology AND Movement disorders [keywords]
- Hemifacial spasm and postural abnormalities; clinical and posturographical analyses. [JOURNAL ARTICLE]
- Acta Neurol Belg 2014 Sep 17.
Hemifacial spasm (HFS) is defined as an involuntary, irregular clonic, or tonic movement of muscles innervated by the ipsilateral seventh cranial nerve. It is reported that the coexistence of non-motor- and motor-related symptoms can be seen in patients with HFS. Postural disturbances were investigated in some movement disorders; however, postural abnormalities due to HFS had not been reported before. In this study, we aimed to investigate the postural abnormalities in patients with HFS. In this cross-sectional, controlled study, Tinetti Balance and Gait Test (TBGT) scores and static posturography were performed on fifteen patients with HFS and fifteen healthy age- and sex-matched controls. The total TBGT score and TBGT-balance score were found to be significantly lower in the patient group than in the control group (p values were, respectively, 0.046 and 0.011). The ratio of the patients with high risk of falling was 40 %, and the difference was found to be significantly higher in the patient group (p value = 0.008). In Fourier analyses, a significant difference was found in the medium to high frequencies (F5-6) when the posturographic evaluation was performed on a solid ground with closed eyes, head rotated to right, and head rotated to the left positions (p values were, respectively, 0.045 and 0.007). The stability index of the HFS group was significantly higher than the control group when tested on the neutral, head right, and head left positions (p values were, respectively, 0.004, 0.049, and 0.003). In conclusion, our study showed that the patients with HFS have more balance and falling problems than the controls, which can be both clinically and posturographically determined.
- Sydenham Chorea and PANDAS in South Africa: Review of Evidence and Recommendations for Management in Resource-Poor Countries. [JOURNAL ARTICLE]
- J Child Neurol 2014 Sep 16.
In South Africa, and worldwide, rheumatic fever represents a public health problem. Improved diagnosis and management of Sydenham chorea, a major manifestation of acute rheumatic fever is key to prevention of rheumatic heart disease. This article reviews Sydenham chorea from its original description to current opinions. Recommendations are founded on expert opinion as class 1 data is lacking. This South African perspective is relevant to resource-poor settings globally insofar as it provides diagnosis and management recommendations for primary- and secondary-level healthcare professionals who care for patients in such environments. Four basic tenets of care are recommended, namely, elimination of the streptococcal infection, symptomatic treatment, immunological treatment, and nonpharmacologic interventions. A user-friendly outcome measurement tool, viable for use in low-resource settings is presented. Introduction of this tool may lead to increased awareness of the neuropsychiatric manifestations of poststreptococcal movement disorders in Africa, where reports are limited.
- Subthalamic nucleus-deep brain stimulation for early motor complications in Parkinson's disease-the EARLYSTIM trial: Early is not always better. [JOURNAL ARTICLE]
- Mov Disord 2014 Sep 16.
Subthalamic nucleus deep brain stimulation (STN-DBS) has revolutionized the management of disabling motor complications in Parkinson's disease. The EARLYSTIM trial applied this treatment to patients who had been experiencing motor complications for less than three years. STN-DBS significantly improved all primary and secondary outcome measures while best medical therapy failed to provide any improvement at the two-year follow-up time point. On face value these results strongly favor the application of STN-DBS far earlier than is currently applied, when patients are just beginning to experience problems with motor complications. Here we review the application of early DBS and the EARLYSTIM trial from the perspectives of clinical issues, health economics and study design and patient expectation of benefit. We conclude that the most relevant issue is not when to operate but on whom and that early is not always better. © 2014 International Parkinson and Movement Disorder Society.
- NREM sleep alpha and sigma activity in Parkinson's disease: Evidence for conflicting electrophysiological activity? [JOURNAL ARTICLE]
- Clin Neurophysiol 2014 Aug 27.
Sleep EEG spectral patterns were investigated in eight newly diagnosed, non-depressed, non-demented, drug-naïve Parkinson's disease patients compared to nine controls.Mean relative spectral power density calculated for 0.25Hz frequency bins and for classical EEG frequency bands.Differences between patients and controls were most prominent in non-REM sleep, specially around 8.6Hz (slow alpha), 12.5Hz (fast alpha/slow sigma) and 15Hz (fast sigma). Slow alpha showed lower p-values over frontal and occipital electrodes, whereas fast sigma activity was more important on central and parietal sites. Significantly increased NREM sleep alpha activity was found in left and right frontal (Mann-Whitney U=12,000, p=.021; U=14,000, p=.036), left and right central (U=14,000, p=.036), left parietal and left occipital (U=13,000, p=.027; U=15,000, p=.046) areas. Increased sigma activity was found in right frontal (U=14,000, p=.036), left central (U=12,000, p=.021), left and right parietal (U=12,000, p=.021; U=13,000, p=.027) and left occipital (U=15,000, p=.046) areas.Concomitantly increased scalp EEG alpha and sigma activity was found during NREM sleep in initial Parkinson's disease.These non-REM sleep microstructure changes may represent evidence for altered electrophysiological mechanisms leading to sleep-wake instability in early disease stages.
- Tauopathy PET and amyloid PET in the diagnosis of chronic traumatic encephalopathies: studies of a retired NFL player and of a man with FTD and a severe head injury. [JOURNAL ARTICLE]
- Transl Psychiatry 2014.:e441.
Single, severe traumatic brain injury (TBI) which elevates CNS amyloid, increases the risk of Alzheimer's disease (AD); while repetitive concussive and subconcussive events as observed in athletes and military personnel, may increase the risk of chronic traumatic encephalopathy (CTE). We describe two clinical cases, one with a history of multiple concussions during a career in the National Football League (NFL) and the second with frontotemporal dementia and a single, severe TBI. Both patients presented with cognitive decline and underwent [(18)F]-Florbetapir positron emission tomography (PET) imaging for amyloid plaques; the retired NFL player also underwent [(18)F]-T807 PET imaging, a new ligand binding to tau, the main constituent of neurofibrillary tangles (NFT). Case 1, the former NFL player, was 71 years old when he presented with memory impairment and a clinical profile highly similar to AD. [(18)F]-Florbetapir PET imaging was negative, essentially excluding AD as a diagnosis. CTE was suspected clinically, and [(18)F]-T807 PET imaging revealed striatal and nigral [(18)F]-T807 retention consistent with the presence of tauopathy. Case 2 was a 56-year-old man with personality changes and cognitive decline who had sustained a fall complicated by a subdural hematoma. At 1 year post injury, [(18)F]-Florbetapir PET imaging was negative for an AD pattern of amyloid accumulation in this subject. Focal [(18)F]-Florbetapir retention was noted at the site of impact. In case 1, amyloid imaging provided improved diagnostic accuracy where standard clinical and laboratory criteria were inadequate. In that same case, tau imaging with [(18)F]-T807 revealed a subcortical tauopathy that we interpret as a novel form of CTE with a distribution of tauopathy that mimics, to some extent, that of progressive supranuclear palsy (PSP), despite a clinical presentation of amnesia without any movement disorder complaints or signs. A key distinguishing feature is that our patient presented with hippocampal involvement, which is more frequently seen in CTE than in PSP. In case 2, focal [(18)F]-Florbetapir retention at the site of injury in an otherwise negative scan suggests focal amyloid aggregation. In each of these complex cases, a combination of [(18)F]-fluorodeoxyglucose, [(18)F]-Florbetapir and/or [(18)F]-T807 PET molecular imaging improved the accuracy of diagnosis and prevented inappropriate interventions.
- Understanding Parkinson disease: An evolving case study. [JOURNAL ARTICLE]
- Nurse Pract 2014 Oct 15; 39(10):1-10.
Thirty years ago, Parkinson disease was described as a shortage of the neurotransmitter dopamine. Today, understanding of this disorder includes possible genetic influences, premorbid and nonmotor issues, and a variety of neurologic, cognitive, and psychiatric symptoms. Using a case study, this article presents the current science of Parkinson disease.
- Ping-Pong Gaze: Sherrington Would Not Have Done It Better. [JOURNAL ARTICLE]
- JAMA Neurol 2014 Sep 15.
- Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia. [JOURNAL ARTICLE]
- Future Neurol 2014 Mar 1; 9(2):187-196.
The cerebellum is an important structure for accurate control and timing of movement, and Purkinje neurons in the cerebellar cortex are key players in cerebellar motor control. Cerebellar dysfunction can result in ataxia, a disorder characterized by postural instability, gait disturbances and motor incoordination. Cerebellar ataxia is a symptom of a number of conditions, and the emerging evidence that Purkinje neuron dysfunction, in particular, abnormal Purkinje neuron repetitive firing, is a major driver of motor dysfunction in a subset of dominantly inherited ataxias is dicussed. Abnormalities in Purkinje neuron excitability that are observed in mouse models of each of these disorders, and where appropriate describe studies linking particular ion channels to aberrant excitability are also discussed. Common mechanisms of dysfunction and speculate about potential therapeutic targets, suggesting that Purkinje neuron firing abnormalities are a novel target for improving motor dysfunction in patients with some forms of dominantly inherited ataxia are proposed.
- Movement disorders of probable infectious origin. [Journal Article]
- Ann Indian Acad Neurol 2014 Jul; 17(3):292-7.
Movement disorders (MDs) associated with infections remains an important debilitating disorder in the Asian countries.The objective of the following study is to report the clinical and imaging profile of a large cohort of patients with MDs probably associated with infection.This was a chart review of 35 patients (F:M-15:20) presenting with MD in the Neurology services of National Institute of Mental Health and Neurosciences, India. The demographic profile, type of infection, time from infection to MD, phenomenology of MD and magnetic resonance imaging (MRI) findings were reviewed.The mean age at presentation was 22.6 ± 13.3 years, (5-60), age of onset of MD was 15.7 ± 15 years, and duration of symptoms was 6.9 ± 8.1 years (42 days to 32 years). The mean latency of onset of MD after the infection was 5.9 ± 4.2 weeks. The phenomenology of MD were: (1) Pure dystonia-28.6%, (2) dystonia with choreoathetosis-22.9%, (3) Parkinsonism-14.6%, (4) pure tremor, hemiballismus, myoclonus and chorea-2.9% each, and (5) mixed MD-22.9%. Most often the MD was generalized (60%), followed by right upper limb (31.4%) and left upper limb (8.6%). A viral encephalitic type of neuroinfection was the most common infection (85.7%), which was associated with MD. Abnormalities of brain MRI, seen in 79.2%, included signal changes in (1) thalamus-52.0%, (2) putamen and subcortical white matter-16% each, (3) pons-12%, (4) striatopallidum, striatum and grey matter-8% each, and (5) caudate, cerebellum, lentiform nucleus, midbrain and subthalamic nucleus-4.0% each.MDs associated with infection were the most often post-encephalitic. Dystonia was the most common MD, and thalamus was the most common anatomical site involved.
- Alterations in Polysomnographic (PSG) profile in drug-naïve Parkinson's disease. [Journal Article]
- Ann Indian Acad Neurol 2014 Jul; 17(3):287-91.
We studied the changes in Polysomnographic (PSG) profile in drug-naïve patients of Parkinson's disease (PD) who underwent evaluation with sleep overnight PSG.This prospective study included 30 with newly diagnosed levodopa-naïve patients with PD, fulfilling the UK-PD society brain bank clinical diagnostic criteria (M:F = 25:5; age: 57.2 ± 10.7 years). The disease severity scales and sleep related questionnaires were administered, and then patients were subjected to overnight PSG.The mean duration of illness was 9.7 ± 9.5 months. The mean Hoehn and Yahr stage was 1.8 ± 0.4. The mean Unified Parkinson's Disease Rating Scale (UPDRS) motor score improved from 27.7 ± 9.2 to 17.5 ± 8.9 with sustained usage of levodopa. Nocturnal sleep as assessed by Pittsburgh Sleep Quality Index (PSQI) was impaired in 10 (33.3%) patients (mean PSQI score: 5.1 ± 3.1). Excessive day time somnolence was recorded in three patients with Epworth Sleepiness Scale (ESS) score ≥ 10 (mean ESS score: 4.0 ± 3.4). PSG analysis revealed that poor sleep efficiency of <85% was present in 86.7% of patients (mean: 68.3 ± 21.3%). The latencies to sleep onset (mean: 49.8 ± 67.0 minutes) and stage 2 sleep (36.5 ± 13.1%) were prolonged while slow wave sleep was shortened. Respiration during sleep was significantly impaired in which 43.3% had impaired apnoea hyperpnoea index (AHI) ≥5, mean AHI: 8.3 ± 12.1). Apnoeic episodes were predominantly obstructive (obstructive sleep apnea, OSA index = 2.2 ± 5.1). These patients had periodic leg movement (PLM) disorder (56.7% had PLM index of 5 or more, mean PLMI: 27.53 ± 4 9.05) that resulted in excessive daytime somnolence.To conclude, sleep macro-architecture is altered in frequently and variably in levodopa-naïve patients of PD and the alterations are possibly due to disease process per se.