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Volume 2(2); October 2009
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Invited Review
Positron Emission Tomography in the Differential Diagnosis of Parkinsonism
Juha O Rinne
J Mov Disord. 2009;2(2):53-57.
DOI: https://doi.org/10.14802/jmd.09015
  • 9,304 View
  • 72 Download
  • 2 Citations
AbstractAbstract PDF

Positron emission tomography (PET) studies on presynaptic dopaminergic function can reveal hypofunction in early Parkinson’s disease (PD) which may help in the early diagnosis especially in patients with mild symptoms. This hypofunction can be detected with fluorodopa (reflecting mainly aromatic amino acid decarboxylase activity of nigrostriatal terminals) or dopamine transporter ligands. These studies can also help to distinguish PD from essential tremor. However, investigations of presynaptic dopaminergic function are not useful in the differential diagnosis of parkinsonian syndromes. PET ligands, such as fluorodeoxyglucose (reflecting glucose metabolism) and dopamine receptor ligands, reflecting striatal neuronal function are better in this respect. Cardiac sympathetic function studies represent a new and interesting approach to improve differential diagnosis of parkinsonian syndromes but more studies are needed in larger patient populations with longer follow-up to evaluate the usefulness of these investigations. Multitracer approach combining ligands reflecting different aspects of dopaminergic neurotransmission and other physiological function will increase differential diagnostic accuracy.

Review Article
Secondary Dystonia-Clinical Clues and Syndromic Associations
Susanne A Schneider, Kailash P Bhatia
J Mov Disord. 2009;2(2):58-63.
DOI: https://doi.org/10.14802/jmd.09016
  • 16,411 View
  • 368 Download
  • 9 Citations
AbstractAbstract PDF
Background:

Dystonia is a hyperkinetic movement disorder defined by involuntary sustained muscle spasms and unusual postures. Etiologically, dystonic syndromes can be broadly divided into primary and secondary forms, dystonia-plus syndromes and heredodegenerative forms. In particular, diagnosis of secondary dystonic syndromes can be challenging in view of the variety of causes.

Purpose:

The purpose of this article is to highlight some clinical clues and syndromic associations as well as investigational findings which may be helpful in the approach to a patient with suspected secondary dystonia.

Methods:

We outline characteristic clinical and neuroimaging findings which may be directive in the diagnostic process of dystonia patients and facilitate making the correct diagnosis, thus allowing initiating the best treatment.

Results:

Secondary causes of dystonia include, among others, strategic brain lesions of various origins, metabolic disease, neurodegenerative conditions, and previous exposure to drugs or toxins. Presence of clinical signs including prominent oromandibular involvement, eye movement disorders, retinitis pigmentosa, deafness, peripheral neuropathy, parkinsonism or progressive dementia should alert the clinician to consider a secondary cause. Strategic lesions within the basal ganglia, but also within the brainstem, cerebellum or cortical areas may underlie dystonia and should thus be excluded.

Conclusions:

When thorough clinical examination reveals features atypical of primary dystonia, syndromic associations may help the clinician to narrow down the list of differential diagnosis. Directive investigations like neuroimaging may confirm the clinical suspicion.

Original Articles
Usefulness of Diffusion-Weighted MRI for Differentiation between Parkinson’s Disease and Parkinson Variant of Multiple System Atrophy
Eun Joo Chung, Eung Gyu Kim, Jong Seok Bae, Choong Ki Eun, Kwang Sig Lee, Minkyung Oh, Sang Jin Kim
J Mov Disord. 2009;2(2):64-68.
DOI: https://doi.org/10.14802/jmd.09017
  • 10,263 View
  • 62 Download
  • 14 Citations
AbstractAbstract PDF
Background and Purpose:

Several studies have reported that diffusion-weighted imaging (DWI) is able to help discriminate a Parkinson variant of multiple system atrophy (MSA-p) from Parkinson’s disease (PD) on the basis of the increased regional apparent diffusion coefficient (rADC). We analyzed the usefulness of DWI by using the rADC for differential diagnosis between MSA-p and PD and investigated the correlation between the rADC value and clinical features of MSA-p and PD.

Methods:

Twelve patients with PD and 10 with MSA-p were studied. The rADC value was determined in different brain regions, including the dorsal putamen (DP) and middle cerebellar peduncles (MCP).

Results:

The rADC values of the DP showed a greater increase in MSA-p patients than in PD patients (p=0.03). MSA-p patients also presented increased rADC values of the MCP compared with PD patients (p=0.0001). In particular, the sensitivity, specificity and positive predictive values of the MCP rADC were higher than those of the DP rADC. However, DP and MCP rADC values were not correlated with clinical features in either MSA or PD patients.

Conclusions:

DWI discriminated between PD and MSA-p based on rADC values in DP and MCP. The MCP rADC value, in particular, could better discriminate MSA-p from PD.

Anticholinergic Agents Can Induce Oromandibular Dyskinesia
Hee-Young Shin, Won Tae Yoon, Won Yong Lee
J Mov Disord. 2009;2(2):69-71.
DOI: https://doi.org/10.14802/jmd.09018
  • 17,016 View
  • 70 Download
  • 2 Citations
AbstractAbstract PDF
Background and Purpose:

Oromandibular dyskinesia (OMD) can occur spontaneously or they can be induced by the conventional dopamine receptor antagonists. Anticholinergic medications have rarely been reported to cause OMD in parkinsonian or non-parkinsonian patients.

Methods:

We analyzed the clinical features of two parkinsonian and one non-parkinsonian patients who experienced OMD after anticholinergic medication.

Results:

Each patient of our cases developed oromandibular symptoms in the temporal regions that were related to the addition of anticholinergic agents, and the symptoms were relieved following the discontinuation of the causative anticholinergic drugs. In one of our case, levodopa alone did not cause dyskinesia but augmented dyskinesia associated with anticholinergics.

Conclusions:

Here we report two parkinsonian and one non-parkinsonian patients with OMD induced by the use of anticholinergic agents. In our cases, we could not find any other precipitating or actual secondary causes for the OMD symptoms in our patients. Furthermore, the fact that the OMD in our cases were ameliorated with cessation of anticholinergics suggests that it may be anticholinergic-induced.

Autonomic Dysfunctions in Parkinsonian Disorders
Hyo-Jin Bae, Sang-Myung Cheon, Jae Woo Kim
J Mov Disord. 2009;2(2):72-77.
DOI: https://doi.org/10.14802/jmd.09019
  • 31,125 View
  • 81 Download
  • 4 Citations
AbstractAbstract PDF
Background and Purpose:

Symptoms of autonomic dysfunctions are common in the patients with parkinsonian disorders. Because clinical features of autonomic dysfunctions are diverse, the comprehensive evaluation is essential for the appropriate management. For the appreciation of autonomic dysfunctions and the identification of differences, patients with degenerative parkinsonisms are evaluated using structured questionnaire for autonomic dysfunction (ADQ).

Methods:

Total 259 patients, including 192 patients with [idiopathic Parkinson’s disease (IPD, age 64.6 ± 9.6 years)], 37 with [multiple system atrophy (MSA, 62.8 ± 9.1)], 9 with [dementia with Lewy body (DLB, 73.9 ± 4.3)], and 21 with [progressive supranuclear palsy (PSP, 69.4 ± 9.6)]. The ADQ was structured for evaluation of the presence of symptoms and its severity due to autonomic dysfunction, covering gastrointestinal, urinary, sexual, cardiovascular and thermoregulatory domains. Patients were also evaluated for the orthostatic hypotension.

Results:

Although dementia with Lewy body (DLB) patients were oldest and duration of disease was longest in IPD, total ADQ scores of MSA and PSP (23.9 ± 12.6 and 21.1 ± 7.8) were significantly increased than that of IPD (15.1 ± 10.6). Urinary and cardiovascular symptom scores of MSA and gastrointestinal symptom score of PSP were significantly worse than those of IPD. The ratio of patient with orthostatic hypotension in IPD was 31.2% and not differed between groups (35.1% in MSA, 33.3% in DLB and 33.3% in PSP). But the systolic blood pressure dropped drastically after standing in patients with MSA and DLB than in patients with IPD and PSP.

Conclusions:

Patients with degenerative parkinsonism showed widespread symptoms of autonomic dysfunctions. The severity of those symptoms in patients with PSP were comparing to that of MSA patients and worse than that of IPD.

Case Reports
Paroxysmal Chorea as a Relapse of Myelopathy in a Patient with Neuromyelitis Optica
Sang-Soo Lee, Ho-Sung Han, Dong-Ick Shin
J Mov Disord. 2009;2(2):78-79.
DOI: https://doi.org/10.14802/jmd.09020
  • 18,764 View
  • 74 Download
AbstractAbstract PDF

Movement disorders secondary to intrinsic spinal cord disease are rare. Paroxysmal chorea has not yet been reported in the neuromyelitis optica (NMO). We report a 43-year-old woman with relapsing-remitting cervical myelopathy who developed paroxysmal chorea during clinical exacerbation of NMO. MRI scan of the cervical spine revealed a long segmental enhancing lesion, but brain MRI did not show any responsible abnormalities. Acute exacerbation of recurrent myelopathy in NMO may be associated with transient movement disorder.

A Case of Vascular Hemichorea Responding to Topiramate
Jee-Ae Kim, San Jung, Min-Ju Kim, Seok-Beom Kwon, Sung-Hee Hwang, Ki-Han Kwon
J Mov Disord. 2009;2(2):80-81.
DOI: https://doi.org/10.14802/jmd.09021
  • 15,549 View
  • 43 Download
  • 3 Citations
AbstractAbstract PDF

Although vascular chorea often comes into remission spontaneously, a few patients may remain with persistent movement disorder. Most movements respond well to neuroleptics as well as other antidopaminergic drugs, but some patients show poor responses to those neuroleptics. Topiramate is a widely used of broad-spectrum anticonvulsant possessing a complex mechanism of action. It has been proven to enhance gamma-aminobutyrate acid activity and to be effective in the control of other movement disorders. We describe a 63-year-old woman with intractable vascular hemichorea which was controlled with anti-convulsant, topiramate.

Hemidystonia as an Initial Manifestation of Leptomeningeal Metastasis
Hyun-Jung Jung, Seong-Min Choi, Byeong-Chae Kim
J Mov Disord. 2009;2(2):82-85.
DOI: https://doi.org/10.14802/jmd.09022
  • 16,624 View
  • 75 Download
  • 1 Citations
AbstractAbstract PDF

A 76-year-old woman gradually developed action dystonia of the left hand and foot. Leptomeningeal metastasis of the right fronto-parietal area associated with gastric adenocarcinoma was found on the brain magnetic resonance imaging (MRI) and positron emission tomography (PET) studies. We discuss the mechanisms involved in the development of secondary hemidystonia and review dystonia associated with cortical lesions.

Disabling Head Tremor in a Patient with DYT1 Mutation
Jae-Hyeok Lee, Dae-Seong Kim, Jae-Wook Cho, Kyung-Pil Park
J Mov Disord. 2009;2(2):86-87.
DOI: https://doi.org/10.14802/jmd.09023
  • 6,222 View
  • 37 Download
  • 1 Citations
AbstractAbstract PDF

Dystonic head tremor is known to be a feature in some patients with DYT1 mutation. However, isolated tremor of the head without relevant cervical dystonia has rarely been described. We report here a patient with the three-bp GAG deletion in the DYT1 gene (904_906delGAG) who had severe head tremor in the frame of a generalized limb dystonia.

A Cerebellar Tremor in a Patient with Human Immunodeficiency Virus-1 Associated with Progressive Multifocal Leukoencephalopathy
Hee-Jin Kim, Jae-Jung Lee, Phil Hyu Lee
J Mov Disord. 2009;2(2):88-90.
DOI: https://doi.org/10.14802/jmd.09024
  • 18,504 View
  • 44 Download
  • 1 Citations
AbstractAbstract PDF

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system (CNS) caused by JC virus infection in oligodendrocytes, especially in patients with acquired immunodeficiency syndrome (AIDS). Movement disorders associated with PML are very rare. Here, we report a case of PML in an AIDS patient who presented with a cerebellar tremor, caused by lesions in the cerebellar outflow tract. A cerebellar tremor can be a rare clinical manifestation in patients with PML.

A Case of Adrenoleukodystrophy Presenting as Progressive Cerebellar Dysfunction
Seunguk Jung, Jong Won Chung, Ji Young Yun, Han-Joon Kim, Beom Seok Jeon
J Mov Disord. 2009;2(2):91-94.
DOI: https://doi.org/10.14802/jmd.09025
  • 12,032 View
  • 78 Download
  • 3 Citations
AbstractAbstract PDF

X-linked adrenoleukodystrophy (X-ALD) is a hereditary neurological disorder affecting the nervous system and adrenal cortex. The phenotype of X-ALD ranges from the rapidly progressive cerebral form to milder adrenomyeloneuropathy. However, cerebellar manifestations are rare. We report a case of adrenoleukodystrophy presenting as progressive cerebellar dysfunction resembling olivopontocerebellar degeneration, with a review of the literature

A Case of Painful Hemimasticatory Spasm with Masseter Muscle Hypertrophy Responsive to Botulinum Toxin
Jin-Hyuck Kim, Seok-Won Han, Yun Joong Kim, Jooyong Kim, Mi-Suh Oh, Hyeo-Il Ma, Byung-Chul Lee
J Mov Disord. 2009;2(2):95-97.
DOI: https://doi.org/10.14802/jmd.09026
  • 12,425 View
  • 66 Download
  • 5 Citations
AbstractAbstract PDF

Hemimasticatory spasm (HMS) is a rare disorder of the trigeminal nerve characterized by paroxysmal involuntary contractions of the unilateral jaw-closing muscles. HMS has been frequently described in association with facial hemiatrophy or localized scleroderma. A 42-year-old female presented with involuntary paroxysmal spasms of the left face, of 6 months duration. Her lower face on the left was markedly hypertrophied without skin lesions. An electrophysiological study indicated that the masseter reflexes and masseteric silent period were attenuated on the affected side. Surface electromyography demonstrated irregular bursts of motor unit potentials at high frequencies up to 200 Hz. Magnetic resonance imaging of the head showed marked hypertrophy of the left masseter muscle. Biopsy of the hypertrophied masseter muscle was normal. Repeated local injections of botulinum toxin noticeably reduced the size of the hypertrophied muscle as well as improved the patient’s symptoms.

Chorea in the Both Lower Limbs Associated with Nonketotic Hyperglycemia
Young-Hee Sung, Ki-Hyung Park, Yeung-Bae Lee, Hyeon-Mi Park, Dong-Jin Shin
J Mov Disord. 2009;2(2):98-100.
DOI: https://doi.org/10.14802/jmd.09027
  • 17,212 View
  • 77 Download
  • 4 Citations
AbstractAbstract PDF

Hemichorea-hemiballism (HC-HB) is a complication of non-ketotic hyperglycemia (NKH); in NKH patients, the frequency of occurrence of HC-HB is greater than that of bilateral chorea. We report the case of a hyperglycemic patient who showed chorea in both the lower limbs. Magnetic resonance imaging (MRI) of the brain revealed high signal intensity on T1-weighted images of the bilateral dorsolateral putamen. The abnormal involuntary movements disappeared after oral administration of haloperidol. Our case report that chorea associated with NKH is correlated with the topography of the basal ganglia.


JMD : Journal of Movement Disorders