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Volume 5(1); April 2012
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Original Articles
Preliminary Study of Intravenous Amantadine Treatment for Ataxia Management in Patients with Probable Multiple System Atrophy with Predominant Cerebellar Ataxia
Jinyoung Youn, Hyeeun Shin, Ji Sun Kim, Jin Whan Cho
J Mov Disord. 2012;5(1):1-4.
DOI: https://doi.org/10.14802/jmd.12001
  • 7,243 View
  • 85 Download
  • 4 Citations
AbstractAbstract PDF
Background and Purpose:

Multiple system atrophy with predominant cerebellar ataxia is a disabling neurologic disease. However, effective management has not yet been established. We conducted a short-term, open-label preliminary study to assess the benefits of intravenous amantadine treatment in patients with probable multiple system atrophy with predominant cerebellar ataxia.

Methods:

Twenty patients (10 male, 10 female) with probable multiple system atrophy with predominant cerebellar ataxia received 400 mg of amantadine by intravenous per day for 5 days. Ataxia severity was evaluated by the International Cooperative Ataxia Rating Scale before and after intravenous amantadine therapy and all subjects reported subjective improvement after intravenous amantadine treatment using a patient global impression scale. We analyzed the total and subscale scores by the ataxia scale and patient global impression scale.

Results:

The mean age was 57.4 years (range: 47–72) and the mean disease duration was 30.8 months (range: 11–79). The ataxia severity significantly decreased after intravenous amantadine therapy from 42.5 to 37.3 (p < 0.001). The mean patient global impression scale for improvement was 2.9 and there were no side effects of intravenous amantadine treatment observed. When we assessed responders, the duration of intravenous amantadine effect was more than 1 month in 4 subjects of 7 responders.

Conclusions:

Our findings suggest that intravenous amantadine treatment can be a safe management option in cerebellar ataxia, although the mechanism is unclear. Thus, further double-blind, long-term studies with a larger sample size are needed.

Reorganization of the Human Somatosensory Cortex in Hand Dystonia
Maria Jose Catalan, Kenji Ishii, William Bara-Jimenez, Mark Hallett
J Mov Disord. 2012;5(1):5-8.
DOI: https://doi.org/10.14802/jmd.12002
  • 7,281 View
  • 52 Download
  • 1 Citations
AbstractAbstract PDF
Background and Purpose:

Abnormalities of finger representations in the somatosensory cortex have been identified in patients with focal hand dystonia. Measuring blood flow with positron emission tomography (PET) can be use to demonstrate functional localization of receptive fields.

Methods:

A vibratory stimulus was applied to the right thumb and little finger of six healthy volunteers and six patients with focal hand dystonia to map their receptive fields using H215O PET.

Results:

The cortical finger representations in the primary somatosensory cortex were closer to each other in patients than in normal subjects. No abnormalities were found in secondary somatosensory cortex, but the somatotopy there is less well distinguished.

Conclusions:

These data confirm prior electrophysiological and functional neuroimaging observations showing abnormalities of finger representations in somatosensory cortex of patients with focal hand dystonia.

Botulinum Toxin Clinic-Based Epidemiologic Survey of Adults with Primary Dystonia in East China
Li Wang, Xingyue Hu, Chunfeng Liu, Yiwen Wu, Changqing Wang, Zhiqiang Wang, Jun Chen
J Mov Disord. 2012;5(1):9-13.
DOI: https://doi.org/10.14802/jmd.12003
  • 11,461 View
  • 54 Download
  • 2 Citations
AbstractAbstract PDF
Background and Purpose:

Primary focal or segmental dystonia is a rare clinical condition. The clinical features of dystonia have not been evaluated in China. We performed a study to investigate the epidemiology of primary dystonia and its clinical variants in an adult population.

Methods:

A Botulinum Toxin Clinic-based study was conducted in the period 18 May through 8 October 2010 in East China. We identified 523 dystonia patients from the Movement disorders and Botulinum Toxin clinic Cases.

Results:

The most common focal dystonia were blepharospasm (59%), cervical dystonia (35%), limb dystonia (3%), oromandibular dystonia (2%) and laryngeal dystonia (1%). Males with primary dystonia were noted to have earlier age of onset. A female predominance was noted for most of the primary dystonias with a male to female ratio (M : F) ranging from 1 : 1.48 to 1 : 3.

Conclusions:

The epidemiological features of dystonia in East China we collected were similar to the report in Japan which contrasts partly with that reported in Europe.

Case Reports
Hypomania Induced by Subthalamic Nucleus Stimulation in a Parkinson’s Disease Patient: Does It Suggest a Dysfunction of the Limbic Circuit?
Ji Seon Kim, Hee Jin Kim, Ji-Young Lee, Jong Min Kim, Ji Young Yun, Beom S. Jeon
J Mov Disord. 2012;5(1):14-17.
DOI: https://doi.org/10.14802/jmd.12004
  • 11,192 View
  • 61 Download
  • 5 Citations
AbstractAbstract PDF

The aim of this report was to describe a case of hypomania after deep brain stimulation of the subthalamic nucleus (STN DBS) in a Parkinson’s disease (PD) patient. 59-year-old man with a 15-year history of PD underwent bilateral implantation of electrodes to the STN. Immediately after surgery, his motor function was markedly improved and his mood was elevated to hypomania. Fusion images of the preoperative MRI and postoperative CT scan showed that the electrodes were located in the medial portion of the STN. In this case, behavioral mood change was related to the deep brain stimulation. Moreover, the anatomical location and the functional alteration of the STN after the DBS surgery might be related to the regulatory system of the associative and limbic cortico-subcortical circuits.

Apparently Ipsilateral Parkinsonism in a Patient with Chronic Subdural Hematoma
Tae Hwan Roh, Dokyung Lee, Il Ki Hong, Deog Yoon Kim, Tae-Beom Ahn
J Mov Disord. 2012;5(1):18-20.
DOI: https://doi.org/10.14802/jmd.12005
  • 6,186 View
  • 68 Download
  • 1 Citations
AbstractAbstract PDF

Symptomatic parkinsonism secondary to ipsilateral lesion is rarely reported. Although the contribution of the contralateral lesions was assumed in some cases, the pathomechanism remains undetermined. Herein we report a patient with a subdural hematoma, who developed parkinsonism in the ipsilateral hemibody. Structural and functional imaging suggests the contralateral dopaminergic dysfunction as the major culprit of apparently ipsilateral parkinsonism.

An Elderly Case of Acute Cerebellitis after Alleged Vaccination
Kang Min Park, Si Eun Kim, Sung Eun Kim
J Mov Disord. 2012;5(1):21-23.
DOI: https://doi.org/10.14802/jmd.12006
  • 11,174 View
  • 60 Download
  • 1 Citations
AbstractAbstract PDF

Acute cerebellitis (AC) is a benign and self-limiting inflammatory disease. It typically occurs as a primary infectious or postinfectious disorder. Although AC mostly presents in early childhood, it can appear in adult. A 66-year-old man admitted to our hospital because of limb and gait ataxia. Three weeks ago, he took an influenza vaccination. There was no abnormality on brain MRI with contrast enhancement, but Technetium-99m hexamethyl propylene amine oxime-single photon emission computed tomography (HMPAO-SPECT) showed markedly cerebellar asymmetry, suggesting hypoperfusion in the right cerebellum. Influenza vaccination can cause AC in the elderly and brain HMPAO-SPECT imaging is more useful than MRI in identifying patients with AC.

Psychogenic Balance Disorders: Is It a New Entity of Psychogenic Movement Disorders?
Jong Sam Baik, Myung Sik Lee
J Mov Disord. 2012;5(1):24-27.
DOI: https://doi.org/10.14802/jmd.12007
  • 14,181 View
  • 89 Download
  • 2 Citations
AbstractAbstract PDF

The various reported psychogenic dyskinesias include tremor, dystonia, myoclonus, gait disorder, Parkinsonism, tics, and chorea. It is not easy to diagnose psychogenic movement disorders, especially in patients with underlying organic disease. We describe three patients with balance and/or posture abnormalities that occur when they stand up, start to move, or halt from walking, although their gaits are normal. One had an underlying unilateral frontal lobe lesion. All patients improved dramatically after receiving a placebo-injection or medication. These abnormal features differ from the previously reported features of astasia without abasia and of psychogenic gait disorders, including recumbent gait. We describe and discuss the patients’ unique clinical characteristics.

Levodopa-Induced Facial Dystonia in a Case of Progressive Supranuclear Palsy
Eun Joo Chung, Sang Jin Kim
J Mov Disord. 2012;5(1):28-32.
DOI: https://doi.org/10.14802/jmd.12008
  • 14,689 View
  • 61 Download
  • 2 Citations
AbstractAbstract PDF

Progressive supranuclear palsy (PSP) is frequently misdiagnosed as other Parkinsonism because of clinical heterogeneity of PSP. We present here a case of a 67-year-old male patient with frontotemporal dementia-like cognitive impairment including language difficulties and abnormal behaviors. He showed severe facial dystonia after the levodopa treatment. Herein, we describe an unusual case of a patient presenting with PSP which, we believe could contribute to our knowledge about atypical leveodopa-induced facial dystonia in PSP.


JMD : Journal of Movement Disorders