Spinal segmental myoclonus following spinal surgery.

A 44-year-old male was admitted with L5/S1 spondylodiscitis complicated by a posterior epidural abscess that was compressing the thecal sac with severe narrowing of the canal and compression of the cauda equine. He underwent computed tomography (CT) guided drainage followed by L5/S1 decompression laminectomy and was started on a 6-week course of intravenous antibiotics with good response. He remained well and afebrile with inflammatory markers showing improvement. During this period, he developed intermittent myoclonic movements of right lower limb with severe pain over the back radiating to the gluteal region which hindered his rehabilitation potential. He was diagnosed with spinal segmental myoclonus based on clinical findings and history of recent spinal surgery. He was successfully treated with a course of clonazepam and continues to make functional improvements during his rehabilitation program.

Spinal Segmental Myoclonus in Primary Progressive Multiple Sclerosis.

Background: A wide variety of associated movement disorders has been described in multiple sclerosis. Phenomenology Shown: A 57-year-old woman with primary progressive multiple sclerosis developed spinal segmental myoclonus associated with focal myelitis. Educational Value: Movement disorders in multiple sclerosis are phenomenologically diverse and have varied pathophysiological mechanisms, making it essential to identify them to initiate appropriate treatment.

How Do I Find Clues About Where Myoclonus Is Originating?

Myoclonus is defined as a brief and jerky shock-like involuntary movement caused by abrupt muscle contraction or sudden cessation of ongoing muscular activity. Myoclonus can be generated by abnormal activity in different parts of the nervous system, both peripheral and central, including cortical and subcortical structures. According to the presumed neural generator, myoclonus is classified as cortical, subcortical (including myoclonus-dystonia and brainstem/reticular myoclonus), spinal (including segmental spinal and propriospinal myoclonus), and peripheral. The identification of myoclonus subtypes, and therefore its potential source, is clinically important because it can guide diagnosis and treatment. In this video lecture (Video), we reviewed how to determine myoclonus origin. We first reviewed the clinical features typical of each myoclonus subtype. We, then, explored the electrophysiological techniques that can aid in the differential diagnosis of myoclonus, based on its origin. In conclusion, we provided a clinical and electrophysiological overview on how to find clues about neural generators of myoclonus.

Prolonged-release fampridine for the treatment of myoclonus after cervical myelitis: a case report.

Prolonged-release fampridine (PR-FAM), a potassium channel blocker, is approved for improving walking ability in patients with multiple sclerosis (MS). Beyond this, positive effects on other MS symptoms like fatigue, cognition, and tremor have been described. To our knowledge, a positive effect of PR-FAM on spinal myoclonus has not been described so far. Here, we report a 32-year-old female with myoclonus after cervical myelitis affecting both hands which markedly improved after administration of PR-FAM. Treatments used before such as carbamazepine or levetiracetam had to be withdrawn because of intolerable side effects or lack of efficacy. The positive effect of PR-FAM could be confirmed by transient suspension. PR-FAM may be considered as a treatment option in refractory spinal myoclonus after myelitis in selected cases.

Subpial Lumbar Lipoma Associated with Retained Medullary Cord.

Subpial lipomas, which are also known as nondysraphic intramedullary spinal cord lipomas, are not associated with spinal dysraphism resulting from the failed primary neurulation. Retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that originates from the late arrest of secondary neurulation. We treated a 6-year-old boy presented with myoclonus of the lower limbs, who had subpial lipoma at the lumbar cord, just rostral to the low-lying conus, which was tethered by a cord-like structure (C-LS) continuous from the conus and extending to the dural cul-de-sac. Following cord untethering from C-LS and minimal debulking of the lipoma, the myoclonus was improved. Histological examination of C-LS revealed a large central canal-like structure in the neuroglial core and the diagnosis of RMC was made. Subpial lipomas can be incidentally coexistent with spinal dysraphism resulting from the failed secondary neurulation, such as RMC.

Treatment of Spinal Myoclonus Due to Degenerative Compression Myelopathy with Cervical Spinal Cord Stimulation: A Report of 2 Cases.

BACKGROUND: Spinal myoclonus (SM) is a rare hyperkinetic movement disorder that is either idiopathic or secondary to spinal cord lesions. The treatment is either symptomatic only or addresses the underlying etiology. We describe 2 patients with SM with compression myelopathy who were treated with spinal cord stimulation (SCS). CASE DESCRIPTION: The first patient was a 39-year-old man with cervicobrachial pain owing to compression myelopathy at level C5-6 underwent fusion. A year later, he developed neurologic deficits including left-limb dominant tetraparesis and involuntary movements of the right leg. Despite ventral fusion at C5-7 due to progressive myelopathy, the involuntary movements extended to both left extremities. A paddle electrode was placed at level C5-6. SM disappeared immediately under stimulation, and the effect continued even after 24 months. The second patient, a 57-year-old man, underwent fusion at level C5-6 in 1998. Since then, he experienced a persistent tremor in his left hand. After 20 years, he developed cervicobrachial pain of the right upper limb with paresis. Compression myelopathy at segment C6-7 was treated with fusion plating. Six months later, pain returned in both upper limbs, and the tremor extended discretely to his right side. A paddle electrode for SCS was placed at level C7-Th1. SM disappeared immediately under stimulation, and the effect persisted after 10 months. Both patients reported sustained pain reduction. CONCLUSIONS: SCS might offer a more selective medicament-free therapy option for SM. The activation of intraspinal networks and replacement of supraspinal descending influences are mechanisms of SCS in this disorder.

Spinal Myoclonus: Is It An Anesthetic Mystery? / Mioclonía espinal: ¿un misterio anestésico? ¡Se acepta el reto!

The goal is to present a descriptive study related an unprecedent case of spinal myoclonus (SM) following subarachnoid anesthesia (SA). SM are sudden, brief, involuntary non-generalized spasms that can be an adverse effect of drug administration via neuraxial routes. Female, 67y, ASA II, proposed for hip replacement surgery, with normal preoperative exams. 7min after SA with 10mg of bupivacaine 0,5%, no motor blockade observed, and patient complained of unbearable pain in legs and perineum and bilateral, asymmetrical and arrhythmic myoclonic movements in the lower limbs. The latter solved after 48h of general anesthesia and rocuronium perfusion, amongst other therapeutics. Accordingly, intrathecal bupivacaine appears to be the SM most likely cause, regarding the absence of neurologic and electrolyte disorders, spinal cord direct trauma, drug exchange and normal perioperative examination, imaging and laboratory testing.It is mandatory to always take the patients' anaesthetic histories and recognize, treat and report rare anaesthetic complications.

Large fasciculation can clinically manifest as spinal myoclonus; electromyographic and dynamic echomyographic studies of four cases with motor neuron disease.

OBJECTIVE: Patients with motor neuron disease rarely present with fasciculation which is large enough to be clinically recognized as myoclonus. This study is aimed at elucidating the features of large fasciculation manifesting as myoclonus by using surface electromyography (surface EMG) and dynamic echomyography (dynamic Echo). METHODS: Four patients with amyotrophic lateral sclerosis, two of whom clinically presented with both fasciculation and myoclonus, were studied by using the surface EMG and the dynamic Echo. RESULTS: At rest, all patients had fasciculation in atrophic muscles, and the surface EMG showed occasional discharges of different waveforms corresponding to fasciculation. During voluntary gentle muscle contraction, the surface EMG showed repetitive discharges in the contracting muscle, which were constant in size and waveform within each muscle. The muscle Echo at rest revealed occasional contractions of a small number of muscle fibers corresponding to fasciculation. During voluntary muscle contraction, the number of muscle fibers involved in the involuntary motor phenomena was larger in the patients who clinically presented with myoclonus compared with other patients who clinically presented only with fasciculation. In a patient who presented with myoclonus, there was no contraction in the antagonist muscle. CONCLUSIONS: Fasciculation involving a large number of muscle fibers clinically manifests as spinal myoclonus. SIGNIFICANCE: Fasciculation involving a large number of muscle fibers can be a cause of spinal myoclonus.

Spinal Myoclonus Responding to Continuous Intrathecal Morphine Pump.

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.

A case of bulbospinal muscular atrophy with large fasciculation manifesting as spinal myoclonus.

OBJECTIVE: This paper reports a patient with bulbospinal muscular atrophy (BSMA) who presented with spinal myoclonus, documented by video and surface electromyography. CASE REPORT: A 66-year-old man had progressive gait disturbance, dysphagia, and easy fatigability of all extremities over a period of 4 years. Neurologically, muscle atrophy, fasciculation, and weakness were observed in the bulbar and limb muscles. When the knees were kept in mild flexion in the supine position, fasciculation of the thigh adductor muscles was so large that it caused shock-like involuntary movements of the legs, corresponding to spinal myoclonus. A genetic test revealed 41 repeats of CAG in the androgen receptor gene, and the diagnosis of BSMA was made. SIGNIFICANCE: The present case suggests that extremely large fasciculation can cause spinal myoclonus.

Myoclonus-like involuntary movements following cesarean delivery epidural anesthesia.

Spinal myoclonus following neuraxial anesthesia is rare. This report describes a case of myoclonus-like involuntary movement that occurred during the recovery from epidural anesthesia for a cesarean delivery. The patient's symptom improved with the administration of benzodiazepine, and the patient recovered with no neurological sequelae. In conclusion, epidural anesthesia can cause spinal myoclonus, which can be treated with a benzodiazepine.

A Case of Hemiabdominal Myoclonus.

Myoclonus consists of sudden, brief, involuntary jerky muscular contractions. Central and peripheral nervous system lesions are involved in the pathogenesis of this movement disorder. Symptomatic or secondary spinal myoclonus is the most common form. A 68-year-old woman was diagnosed with hemiabdominal spinal myoclonus. Occasional and very mild involuntary repetitive movements of the hemiabdomen began immediately after surgery for uterine cancer. After surgery for laparocele, secondary to the uterine cancer surgery, performed under spinal anesthesia, there was severe worsening of movements. Neuroradiological investigations failed to demonstrate spinal injury, while neurophysiological studies showed impairment of the right central somatosensory pathway. Considering the low resolution of magnetic resonance imaging in the evaluation of thoracic level, we suggest an extensive neurophysiological evaluation in patients with spinal myoclonus.

Familial segmental spinal myoclonus: a rare clinical feature of Friedreich's ataxia.

INTRODUCTION: Friedreich's ataxia (FRDA) is the most common autosomal recessive inherited ataxia. It is characterized by onset before the age of 25 year, progressive limb and truncal ataxia, lower limb areflexia, extensor plantars, dysarthria and impaired posterior column sensations. Other important associated features are skeletal deformity, hypertrophic cardiomyopathy and diabetes mellitus. Most of the patients (98%) have an unstable homozygous trinucleotide (GAA) expansion in intron-1 of chromosome 9 and 2% patients are compound heterozygous for GAA expansion and point mutations. CASE DESCRIPTION: We observed an adolescence onset FRDA exhibiting spinal segmental myoclonus (SSM) in a family. Triplet repeat primed polymerase chain reaction (TP-PCR) demonstrated unstable expansion of >66 GAA repeats. CONCLUSIONS: SSM is a unique and rare manifestation of FRDA. This might be the first case report of SSM in FRDA patient.

Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes.

Propriospinal myoclonus (PSM) is a rare type of spinal myoclonus characterized by muscle jerks that usually start in the midthoracic segments and then slowly propagate up and down into the spinal cord, resulting in repetitive and irregular jerky flexion, or extension of the trunk, neck, knees and hips. PSM can be symptomatic, but up to 80% of reported cases appear idiopathic. PSM tends to occur especially while the subject is lying down. PSM at sleep onset was first described by experts in sleep medicine. The original electrophysiological features included fixed pattern of muscle activations, slow spinal cord conduction (5-15 m/s), electromyographic burst duration less than 1000 ms, synchronous activation of agonist and antagonist muscles and no involvement of facial muscles. PSM has been reported to be a functional (psychogenic) movement disorder in a number of cohorts. The differential diagnosis between idiopathic PSM and the functional forms is not always straightforward. A consistent polymyographically documented muscle activation pattern may be supportive but by no means sufficient and additional neurophysiological investigations are required. PSM should be differentiated from other movement disorders involving the abdomen and trunk, or occurring at sleep-wake transition. This article offers a comprehensive overview of the spectrum of PSM phenotypes.

Spinal-generated movement disorders: a clinical review.

Spinal-generated movement disorders (SGMDs) include spinal segmental myoclonus, propriospinal myoclonus, orthostatic tremor, secondary paroxysmal dyskinesias, stiff person syndrome and its variants, movements in brain death, and painful legs-moving toes syndrome. In this paper, we review the relevant anatomy and physiology of SGMDs, characterize and demonstrate their clinical features, and present a practical approach to the diagnosis and management of these unusual disorders.

Recurrent spinal myoclonus after two episodes of spinal anesthesia at a 1-year interval -A case report-.

Spinal myoclonus is an unusual, self-limiting, adverse event that may occur during spinal anesthesia. The exact cause and underlying biochemical mechanism of spinal myoclonus remain unclear. A few cases of spinal myoclonus have been reported after administration of intrathecal bupivacaine. We report a case in which spinal myoclonus recurred after two episodes of spinal anesthesia with bupivacaine at a 1-year interval in a 35-year-old woman. The myoclonus was acute and transient. The patient recovered completely, with no neurologic sequelae.