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Spinal myoclonus is a sudden, brief, and involuntary movement of segmental or propriospinal muscle groups. Spinal myoclonus has occasionally been reported in patients undergoing opioid therapy, but the pathophysiology of opioid-induced myoclonus has not been elucidated yet. Here, we present two patients with spinal segmental myoclonus secondary to ischemic and radiation myelopathy. Conventional medications did not help treat persistent myoclonus in both legs. Continuous intrathecal morphine infusion was implanted for pain control in one patient, which relieved spinal myoclonus entirely. This experience led to the application of this method with a second patient, leading to the same gratifying result. Spinal myoclonus reemerged as soon as the morphine pumps were off, which confirmed the therapeutic role of opioids. In contrast to the opioid-induced myoclonus, these cases show a benefit of opioids on spinal myoclonus, which could be explained by synaptic reorganization after pathologic insults in the spinal cord.
Analgesics, Opioid ; Dyskinesias ; Humans ; Leg ; Methods ; Morphine* ; Myoclonus* ; Spinal Cord ; Spinal Cord Diseases

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Metronidazole-Induced Craniocervical Myoclonus with Reversible Bilateral Dentate Nucleus Lesions.

Hyun Chang LEE ; Young Eun KIM ; Hyeo Il MA

Journal of Movement Disorders.2017;10(1):67-68. doi:10.14802/jmd.16021

No abstract available.
Cerebellar Nuclei* ; Myoclonus*

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Multifocal Myoclonus as a Manifestation of Acute Cerebral Infarction Recovered by Carotid Arterial Stenting.

Hyangkyoung KIM ; Jun Soo BYUN ; Mark HALLETT ; Hae Won SHIN

Journal of Movement Disorders.2017;10(1):64-66. doi:10.14802/jmd.16040

No abstract available.
Cerebral Infarction* ; Myoclonus* ; Stents*

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Camptocormia with Transient Ischemic Attack.

Ju Hee OH ; Dong Woo RYU ; Si Hoon LEE ; Joong Seok KIM

Journal of Movement Disorders.2017;10(1):62-63. doi:10.14802/jmd.16043

No abstract available.
Ischemic Attack, Transient*

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Suspected Perinatal Depression Revealed to be Hereditary Diffuse Leukoencephalopathy with Spheroids.

Josefine BLUME ; Robert WEISSERT

Journal of Movement Disorders.2017;10(1):59-61. doi:10.14802/jmd.16050

Early motor symptoms of neurodegenerative diseases often appear in combination with psychiatric symptoms, such as depression or personality changes, and are in danger of being misdiagnosed as psychogenic in young patients. We present the case of a 32-year-old woman who presented with rapid-onset depression, followed by a hypokinetic movement disorder and cognitive decline during pregnancy. Genetic testing revealed a mutation in the colony-stimulating factor 1 receptor gene, which led to the diagnosis of hereditary diffuse leukoencephalopathy with spheroids. Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is probably an under-recognized disease. HDLS should be considered in patients with rapidly progressing parkinsonian symptoms and dementia accompanied by white matter lesions.
Adult ; Dementia ; Depression* ; Diagnosis ; Female ; Genetic Testing ; Humans ; Leukoencephalopathies* ; Macrophage Colony-Stimulating Factor ; Movement Disorders ; Neurodegenerative Diseases ; Parkinsonian Disorders ; Pregnancy ; White Matter

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Familiar Hyperekplexia, a Potential Cause of Cautious Gait: A New Korean Case and a Systematic Review of Phenotypes.

Yoonju LEE ; Nan Young KIM ; Sangkyoon HONG ; Su Jin CHUNG ; Seong Ho JEONG ; Phil Hyu LEE ; Young H SOHN

Journal of Movement Disorders.2017;10(1):53-58. doi:10.14802/jmd.16044

Familial hyperekplexia, also called startle disease, is a rare neurological disorder characterized by excessive startle responses to noise or touch. It can be associated with serious injury from frequent falls, apnea spells, and aspiration pneumonia. Familial hyperekplexia has a heterogeneous genetic background with several identified causative genes; it demonstrates both dominant and recessive inheritance in the α1 subunit of the glycine receptor (GLRA1), the β subunit of the glycine receptor and the presynaptic sodium and chloride-dependent glycine transporter 2 genes. Clonazepam is an effective medical treatment for hyperekplexia. Here, we report genetically confirmed familial hyperekplexia patients presenting early adult cautious gait. Additionally, we review clinical features, mode of inheritance, ethnicity and the types and locations of mutations of previously reported hyperekplexia cases with a GLRA1 gene mutation.
Accidental Falls ; Adult ; Apnea ; Clonazepam ; Gait* ; Genetic Background ; Glycine Plasma Membrane Transport Proteins ; Humans ; Nervous System Diseases ; Noise ; Phenotype* ; Pneumonia, Aspiration ; Receptors, Glycine ; Reflex, Startle ; Sodium ; Stiff-Person Syndrome* ; Wills

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Exosome-Based Delivery of miR-124 in a Huntington's Disease Model.

Soon Tae LEE ; Wooseok IM ; Jae Jun BAN ; Mijung LEE ; Keun Hwa JUNG ; Sang Kun LEE ; Kon CHU ; Manho KIM

Journal of Movement Disorders.2017;10(1):45-52. doi:10.14802/jmd.16054

OBJECTIVE: Huntington's disease (HD) is a genetic neurodegenerative disease that is caused by abnormal CAG expansion. Altered microRNA (miRNA) expression also causes abnormal gene regulation in this neurodegenerative disease. The delivery of abnormally downregulated miRNAs might restore normal gene regulation and have a therapeutic effect. METHODS: We developed an exosome-based delivery method to treat this neurodegenerative disease. miR-124, one of the key miRNAs that is repressed in HD, was stably overexpressed in a stable cell line. Exosomes were then harvested from these cells using an optimized protocol. The exosomes (Exo-124) exhibited a high level of miR-124 expression and were taken up by recipient cells. RESULTS: When Exo-124 was injected into the striatum of R6/2 transgenic HD mice, expression of the target gene, RE1-Silencing Transcription Factor, was reduced. However, Exo-124 treatment did not produce significant behavioral improvement. CONCLUSION: This study serves as a proof of concept for exosome-based delivery of miRNA in neurodegenerative diseases.
Animals ; Cell Line ; Exosomes ; Huntington Disease* ; Methods ; Mice ; MicroRNAs ; Neurodegenerative Diseases ; Transcription Factors

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Psychodynamic Psychotherapy for Functional (Psychogenic) Movement Disorders.

Vibhash D SHARMA ; Randi JONES ; Stewart A FACTOR

Journal of Movement Disorders.2017;10(1):40-44. doi:10.14802/jmd.16038

OBJECTIVE: As the literature for the treatment of functional (psychogenic) movement disorders (FMD) is sparse, we assessed clinical outcomes in patients with FMD who underwent treatment with psychodynamic psychotherapy (PDP). METHODS: A retrospective analysis of the data of patients with FMD who were referred for PDP from 2008−2014 at Emory University Medical Center was performed. RESULTS: Thirty patients were included, mean age at presentation was 50 years (SD 13.9) and majority were female (27/30). Most common movement disorder was involuntary shaking/jerky movements (50%) and tremor (43%). Mean duration of symptoms was 3.2 years and mean number of PDP visits was 4.9. PDP lead to good outcomes in 10, modest in 8, and poor in 9. Three patients lost to follow up. Mean duration of symptoms between two groups (good vs. poor) was not statistically significant (p = 0.11), mean number of PDP visits showed a trend towards significance (p = 0.053). In all cases of good outcomes precipitants of the movement disorder were identified and a majority (60%) was receptive of the diagnosis and had good insight. CONCLUSION: PDP lead to improvement in 60% of the patients which is encouraging as the treatment is challenging. This study supports heterogeneous causes of FMD including varied roles of past/recent events and demonstrates importance of psychological approaches such as PDP. Treatment with PDP should be considered in some patients with FMD but predicting who will respond remains a challenge. Further long term prospective studies with large sample size and placebo control are needed.
Academic Medical Centers ; Conversion Disorder ; Diagnosis ; Female ; Humans ; Lost to Follow-Up ; Movement Disorders* ; Prospective Studies ; Psychotherapy, Psychodynamic* ; Retrospective Studies ; Sample Size ; Tremor

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