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Volume 4(2); October 2011
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Review Article
MicroRNAs in Experimental Models of Movement Disorders
Soon-Tae Lee, Manho Kim
J Mov Disord. 2011;4(2):55-59.
DOI: https://doi.org/10.14802/jmd.11011
  • 18,849 View
  • 48 Download
  • 4 Citations
AbstractAbstract PDF

MicroRNAs (miRNAs) are small RNAs comprised of 20–25 nucleotides that regulates gene expression by inducing translational repression or degradation of target mRNA. The importance of miRNAs as a mediator of disease pathogenesis and therapeutic targets is rapidly emerging in neuroscience, as well as oncology, immunology, and cardiovascular diseases. In Parkinson’s disease and related disorders, multiple studies have identified the implications of specific miRNAs and the polymorphisms of miRNA target genes during the disease pathogenesis. With a focus on Parkinson’s disease, spinocerebellar ataxia, hereditary spastic paraplegia, and Huntington’s disease, this review summarizes and interprets the observations, and proposes future research topics in this field.

Original Articles
Putaminal Hypointensity in the Parkinsonian Variant of Multiple System Atrophy: Simple Visual Assessment Using Susceptibility-Weighted Imaging
Jae-Hyeok Lee, Seung-Kug Baik
J Mov Disord. 2011;4(2):60-63.
DOI: https://doi.org/10.14802/jmd.11012
  • 14,693 View
  • 177 Download
  • 16 Citations
AbstractAbstract PDF
Background and Purpose

Susceptibility-weighted imaging (SWI) has been shown to be superior in its ability to demonstrate brain mineralization than other conventional MR imaging. The goal of our study was therefore to assess the frequency and extent of putaminal hypointensity in parkinsonian variant MSA using SWI.

Methods

11 patients with multiple system atrophy-parkinsonian type (MSA-p), 30 patients with Parkinson’s disease (PD), and age matched 30 controls were investigated using 3 Tesla MRI. The pattern of putaminal hypointensity was measured using a visual grading scale and scored from 0 to 3.

Results

Hemi- or bilateral putaminal hypointensity (a score of ≥ 2) and hyperintense rim were recognized in 81.8% and 54.5% of 11 MSA-p, respectively. The scores of putaminal hypointensity of MSA-p were significantly higher than other groups (p < 0.001), a score of ≥ 2 differentiated MSA-p from other groups. And all five patients with early disease stage also showed these characteristic findings.

Conclusions

SWI appears to be useful for depicting putaminal hypointensity even in early stage of MSA-p. This finding suggests that iron deposition associated putaminal degeneration can occur early in the disease process.

Lateralized Effects of Unilateral Thalamotomy and Thalamic Stimulation in Patients with Essential Tremor
Mi J. Kim, Sang R. Jeon, Sung R. Kim, Myoung C. Lee, Sun J. Chung
J Mov Disord. 2011;4(2):64-67.
DOI: https://doi.org/10.14802/jmd.11013
  • 14,195 View
  • 67 Download
  • 5 Citations
AbstractAbstract PDF
Background and Purpose

Stereotactic thalamotomy has been an effective surgical procedure in the treatment of medically refractory essential tremor (ET), however, little is known about the bilateral effects of unilateral ventralis intermedius (Vim) thalamotomy and Vim deep brain stimulation (DBS). We studied the lateralized effects of unilateral Vim thalamotomy and Vim DBS in ET patients.

Methods

Vim thalamotomy was performed in 6 patients and Vim DBS in 6. Patients were evaluated preoperatively and at 3 and 6 months postoperatively using the Clinical Rating Scale for Tremor (CRST).

Results

The contralateral Part A (tremor localization/severity rating) and Part B (specific motor tasks/function rating) subscores, and axial subscores of CRST significantly improved after unilateral Vim thalamotomy or Vim DBS. On the side ipsilateral to surgery, ET patients demonstrated no significant improvements in the Part A and Part B subscores of CRST. The Part C (functional disabilities resulting from tremor) subscores and total scores of CRST were significantly improved after surgery.

Conclusions

Vim thalamotomy and DBS may be equally effective for the management of contralateral and axial tremor in ET patients, but both interventions may not improve tremor on the side ipsilateral to surgery.

Analysis of the Substantia Innominata Volume in Patients with Parkinson’s Disease with Dementia, Dementia with Lewy Bodies, and Alzheimer’s Disease
Hee Jin Kim, Ji Eun Lee, Soo Jeong Shin, Young Ho Sohn, Phil Hyu Lee
J Mov Disord. 2011;4(2):68-72.
DOI: https://doi.org/10.14802/jmd.11014
  • 10,789 View
  • 72 Download
  • 9 Citations
AbstractAbstract PDF
Background and Purpose

The substantia innominata (SI) contains the nucleus basalis of Meynert, which is the major source of cholinergic input to the cerebral cortex. We hypothesized that degeneration of the SI and its relationship to general cognitive performance differs in amyloidopathy and synucleinopathy.

Methods

We used magnetic resonance imaging (MRI)-based volumetric analysis to evaluate the SI volume in patients with amnestic mild cognitive impairment (aMCI), Alzheimer’s disease (AD), Parkinson’s disease-mild cognitive impairment (PD-MCI), PD with dementia (PDD), dementia with Lewy bodies (DLB), and healthy elderly controls. The correlation between SI volume and general cognitive performance, measured using the Korean version of the Mini-Mental State Examination (K-MMSE), was examined.

Results

Compared to control subjects, the mean normalized SI volume was significantly decreased in all of the other groups. The normalized SI volume did not differ between the subjects with PDD and DLB, whereas it was significantly smaller in subjects with PDD (p = 0.029) and DLB (p = 0.011) compared with AD. In subjects with PD-related cognitive impairment (PD-MCI, PDD, or DLB), there was a significant positive correlation between the SI volume and K-MMSE score (r = 0.366, p < 0.001), whereas no correlation was seen in subjects with AD-related cognitive impairment (aMCI or AD).

Conclusions

Our data suggest that the SI loss is greater in synucleinopathy-related dementia (PDD or DLB) than in AD and that the contribution of the SI to cognitive performance is greater in synucleinopathy than in amyloidopathy.

Case Reports
Neuroleptic Malignant Syndrome in a Patient with Corticobasal Degeneration
Myung Jun Lee, Chul Hyoung Lyoo, Myung Sik Lee
J Mov Disord. 2011;4(2):73-74.
DOI: https://doi.org/10.14802/jmd.11015
  • 8,862 View
  • 49 Download
AbstractAbstract PDF

Parkinson’s disease is a principal underlying disease of neuroleptic malignant syndrome (NMS) occurring in parkinsonian disorders, but NMS may occur in patients with progressive supranuclear palsy and multiple system atrophy. We report first patient with corticobasal degeneration (CBD) who developed NMS after abrupt reduction of antiparkinsonian medication and concurrent infection. It should be kept in mind that the prevention of infectious illness, which is common complication in parkinson-plus syndrome, is important, and dose reduction or withdrawal of anti-parkinsonian medications should be carefully performed even in the patients with CBD who are expected to be unresponsive to levodopa treatment.

Oromandibular Dyskinesia as the Initial Manifestation of Late-Onset Huntington Disease
Dong-Seok Oh, Eun-Seon Park, Seong-Min Choi, Byeong-Chae Kim, Myeong-Kyu Kim, Ki-Hyun Cho
J Mov Disord. 2011;4(2):75-77.
DOI: https://doi.org/10.14802/jmd.11016
  • 14,596 View
  • 57 Download
  • 4 Citations
AbstractAbstract PDF

Huntington’s disease (HD) is a neurodegenerative disorder characterized by a triad of choreoathetosis, dementia and dominant inheritance. The cause of HD is an expansion of CAG trinucleotide repeats in the HD gene. Typical age at onset of symptoms is in the 40s, but the disorder can manifest at any time. Late-onset (≥ 60 years) HD is clinically different from other adult or juvenile onset HD and characterized by mild motor problem as the initial symptoms, shorter disease duration, frequent lack of family history, and relatively low CAG repeats expansion. We report a case of an 80-year-old female with oromandibular dyskinesia as an initial manifestation of HD and 40 CAG repeats.

Concomitant Appearance of Pisa Syndrome and Striatal Hand in Parkinson’s Disease
Sanjay Pandey, Manmohan Mehndiratta
J Mov Disord. 2011;4(2):78-79.
DOI: https://doi.org/10.14802/jmd.11017
  • 17,239 View
  • 67 Download
  • 4 Citations
AbstractAbstract PDF

Pisa syndrome is (PS) usually seen in patients receiving antipsychotic drugs and characterised by lateral flexion of trunk and axial dystonia. It is believed that antipsychotic drugs lead to dopamine blockage causing PS. We describe a Parkinson’s disease patient who was doing well with levodopa/carbidopa for 3 years and developed lateral flexion of trunk. His abnormal posture used to completely improve upon lying down position. He also had striatal hand deformity suggestive of focal dystonia.

Vocal Polyps in Tourette Syndrome
Michael P Chu, Karen PM Chu, Kevin Fung
J Mov Disord. 2011;4(2):80-81.
DOI: https://doi.org/10.14802/jmd.11018
  • 6,294 View
  • 30 Download
  • 1 Citations
AbstractAbstract PDF

Hoarseness and dysphonia are often a result of vocal cord polyps which in turn, are linked to vocal trauma. We report the case of vocal polyps in the setting of a 27-year old male with a history only remarkable for Tourette syndrome. We review the literature regarding etiology and pathophysiology of vocal cord lesions and propose vocal tics in Tourette syndrome as an under-recognized etiology. In this way, we also review therapies that may aid in treating not only the vocal cord lesions but also particularly in the setting of vocal tics.

Dopa Responsive Slow Orthostatic Tremor in Parkinson’s Disease
Suk Yoon Lee, Eun Joo Chung, Yeo Jung Kim, Sang Jin Kim
J Mov Disord. 2011;4(2):82-84.
DOI: https://doi.org/10.14802/jmd.11019
  • 13,838 View
  • 80 Download
  • 4 Citations
AbstractAbstract PDF

Slow orthostatic tremor (OT) occurred to longer and lower frequency regular rhythmic bursts in leg muscle upon standing. The slow OT was often able to clinically confused with orthostatic myoclonus. We described a Parkinson’s disease patient with levodopa responsive slow OT. She showed abnormal movements of more regular rhythms and stable frequency on both legs on standing. These symptoms were aggravated at off state and improved by increasing levodopa.


JMD : Journal of Movement Disorders