Category: Myoclonus
Objective: A 36-years-old woman with a spinal cord diffuse intramedullary astrocytoma WHO grade II, with multifocal brain parenchymal involvement. After insertion of her ventriculo-peritoneal shunt two months ago for hydrocephalus and brain edema, the patient had also been experiencing persisting involuntary contractions of her abdominal muscles (Video 1) that were painful and irregular in nature. We prescribed clonazepam for the abdomen convulsions without response. Revision of the ventriculo-peritoneal shunt also did not decrease the frequency or intensity of the abdomen convulsions.
Background: Myoclonus, defined as involuntary muscular contractions or inhibition in a muscle or group of muscles.The source of myoclonus can be found anywhere along the neuroaxis, and can be identified as cortical, cortical-subcortical, subcortical-supraspinal, spinal, or peripheral type based on its physiological type and affected anatomical location.1 Segmental spinal myoclonus may be caused by hyperexcitability induced by viral irritation, myelitis, multiple sclerosis, syringomyelia, malignancy, spinal cord trauma, or vascular lesions.1,2,3 Segmental spinal myoclonus is usually confined to one or more contiguous myotomes and occurs irregularly, resistant to supraspinal influences such as sleep or voluntary movement.1 We describe a patient with segmental spinal myoclonus induced by malignancy that is temporarily relieved by lumbar puncture.
Method: We did magnetic resonance imaging (MRI) of her brain, showing no hydrocephalus, over-shunting, or obvious interval change compared to previous MRI studies three months ago. CSF study was then done to excluded CNS infection or inflammation.
Results: During lumbar puncture, complete cessation of abdominal convulsions was also observed once the lumbar puncture needle has entered the subarachnoid space. Abdominal convulsions did not recur for the duration the needle was in place but were observed again immediately after removal of the lumbar puncture needle. Thoraco-lumbar spinal MRI studies showed diffuse CSF drop metastases at the surface of the spinal cord, cauda equina, and S1-2 thecal sac with some loculated CSF collection at the C7-T8 posterior intradural-extramedullary CSF space.
Conclusion: We describe a patient with segmental spinal myoclonus induced by malignancy that is temporarily relieved by lumbar puncture.
References: 1. Caviness, J.N. and Brown, P. (2004). Myoclonus: current concepts and recent advances. The Lancet Neurology. 3(10): 598-607. doi: 10.1016/S1474-4422(04)00880-4 2. Kojovic, M. (2011). Myoclonic disorders: a practical approach for diagnosis and treatment. Therapeutic Advances in Neurodegenerative Disorders. 4(1): 47-62. doi: 10.1177/1756285610395653 3. Alroughani, R.A., Ahmed, S.F., Khan, R.A., and Al-hashel, J.Y. (2015). Spinal segmental myoclonus as an unusual presentation of multiple sclerosis. BioMed Central Neurology. 15:15. doi: 10.1186/s12883-015-0271-y
To cite this abstract in AMA style:
Y. Huang, A. Chuang. A Novel Case of Persistent Segmental Spinal Myoclonus Due to Spinal Astrocytoma Temporarily Relieved by Diversion of Cerebrovascular Fluid [abstract]. Mov Disord. 2020; 35 (suppl 1). https://www.mdsabstracts.org/abstract/a-novel-case-of-persistent-segmental-spinal-myoclonus-due-to-spinal-astrocytoma-temporarily-relieved-by-diversion-of-cerebrovascular-fluid/. Accessed September 7, 2024.« Back to MDS Virtual Congress 2020
MDS Abstracts - https://www.mdsabstracts.org/abstract/a-novel-case-of-persistent-segmental-spinal-myoclonus-due-to-spinal-astrocytoma-temporarily-relieved-by-diversion-of-cerebrovascular-fluid/