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Last update 08 May 2025

Cerebral cortex myoclonus

Last update 08 May 2025

Basic Info

Synonyms

Cerebral cortex myoclonus, Cerebral cortex myoclonus (disorder), Cortical myoclonus

Introduction

Cortical myoclonus mainly affects the distal upper limbs and face, which reflects the largest cortical representations of these body areas. It is often focal, but may be multifocal, bilateral or generalized, as a consequence of intracortical and transcallosal spreading of abnormal activity. It typically occurs on voluntary action and may affect speech and gait. Cortical myoclonic jerks are stimulus sensitive, typically to touch, but sensitivity to visual stimuli is also described. Most patients with cortical myoclonus have both positive myoclonus and NM, occurring either independently or together as a complex of the two kinds of myoclonus. If cortical myoclonus is prolonged and lasts for hours, days or weeks, it is called epilepsia partials continua and is considered to be a rare form of focal epileptic status. Focal cortical myoclonus almost always points to an underlining lesion of the sensori-motor cortex, which produces hyperexcitability (e.g. vascular, inflammatory or neoplastic). [PMID:21339907]

Clinical Trials associated with Cerebral cortex myoclonus

CTR20190082

/ CompletedNot Applicable

吡拉西坦片800mg随机、开放、单剂量、两制剂、两周期自身交叉空腹/餐后状态下健康人体生物等效性正式试验

[Translation] A randomized, open, single-dose, two-formulation, two-period self-crossover bioequivalence study of piracetam tablets 800 mg in healthy volunteers in the fasting/fed state

观察健康受试者空腹及餐后状态下单剂量口服受试制剂吡拉西坦片（800mg，生产企业：江西亿友药业有限公司）后血药浓度的经时过程，估算相应的药代动力学参数；并以吡拉西坦片（800mg，UCB Pharma SA公司生产，商品名：Nootropil）为参比制剂，进行人体相对生物利用度和生物等效性试验，为该药一致性评价申报和临床应用提供参考依据。

[Translation]

The time course of blood drug concentration after a single oral dose of the test preparation piracetam tablets (800 mg, manufactured by Jiangxi Yiyou Pharmaceutical Co., Ltd.) was observed in healthy subjects in the fasting and postprandial states, and the corresponding pharmacokinetic parameters were estimated. Piracetam tablets (800 mg, produced by UCB Pharma SA, trade name: Nootropil) were used as the reference preparation for human relative bioavailability and bioequivalence studies, providing a reference for the consistency evaluation application and clinical application of the drug.

Start Date04 Dec 2018

Sponsor / Collaborator

Jiangxi Yiyou Pharmaceutical Co., Ltd.

NCT00001663

/ CompletedPhase 1IIT

Treatment of Cortical Myoclonus With Repetitive Transcranial Magnetic Stimulation

Myoclonus is a condition related to epilepsy of involuntary twitching or jerking of the limbs.
The purpose of this study is to determine if stimulation of the brain with magnetic pulses can decrease myoclonus. Researchers believe that this may be possible because in studies on normal volunteers, magnetic stimulation made areas of the brain difficult to activate for several minutes. In addition, early studies on patients with myoclonus have shown magnetic stimulation to be effective at decreasing involuntary movements.
Transcranial Magnetic Stimulation (TMS) is a non-invasive technique that can be used to stimulate brain activity and gather information about brain function. It is very useful when studying the areas of the brain and spinal cord related to motor activity (motor cortex and corticospinal tract). Repetitive transcranial magnetic stimulation (rTMS) involves the placement of coil of wire (electromagnet) on the patient's scalp and rapidly turning on and off the electrical current. The changing magnetic field produces weak electrical currents in the brain near the coil. This permits non-invasive, relatively localized stimulation of the surface of the brain (cerebral cortex). The effect of magnetic stimulation varies, depending upon the location, intensity and frequency of the magnetic pulses.
Researchers plan to use rTMS for 10 days on patients participating in the study. The 10 day period will be broken into 5 days of active repetitive magnetic stimulation and 5 days of placebo "ineffective" stimulation. At the end of the 10 day period, if the results show that rTMS was beneficial, patients may undergo an additional 5 days of active rTMS.

Start Date01 Jan 1997

Sponsor / Collaborator

National Institute of Neurological Disorders & Stroke

100 Clinical Results associated with Cerebral cortex myoclonus

100 Translational Medicine associated with Cerebral cortex myoclonus

0 Patents (Medical) associated with Cerebral cortex myoclonus

400

Literatures (Medical) associated with Cerebral cortex myoclonus

01 Apr 2025·The Neurohospitalist

Scrub Typhus Meningoencephalitis Presenting as Generalized Convulsive Status Epilepticus with Basal Ganglia and Extra-limbic Cortical Involvement, Complicated by Cortical Multifocal Myoclonus

Article

Author: Ghosh, Ritwik ; Mukhopadhyay, Arpan ; Biswas, Uttam ; León-Ruiz, Moisés ; Benito-León, Julián ; Bheeman, Raghul ; Sarkar, Ritun

Background Scrub typhus is an acute febrile infectious disease highly prevalent in the Asia Pacific region, often referred to as the “tsutsugamushi triangle.” This mite-borne rickettsial zoonosis is caused by Orientia tsutsugamushi, an intracellular Gram-negative organism that primarily targets endothelial cells. The resulting vasculitis leads to multisystem involvement. In terms of neurological manifestations, meningoencephalitis is the most common presentation of scrub typhus. Other frequent neurological manifestations include cranial nerve paresis, transverse myelitis, and polyneuropathy. Status epilepticus, while reported, is a rare presenting feature of this infection. Although scrub typhus has been documented to present as limbic encephalitis, there have been no previous descriptions in the literature of neuroradiological patterns affecting the basal ganglia or extra-limbic cortices in this condition. Case Report We report a case of a 23-year-old previously healthy woman who presented with scrub typhus meningoencephalitis. The condition manifested as encephalitis with involvement of the basal ganglia and extra-limbic cortices. She presented with generalized convulsive status epilepticus, which was complicated by cortical multifocal myoclonus. Discussion Scrub typhus can be a significant diagnostic challenge, potentially presenting with generalized convulsive status epilepticus and mimicking both clinical and radiological features of arboviral encephalitides, such as those caused by West Nile and Japanese encephalitis viruses. Furthermore, as demonstrated in this case, its radiological presentation can resemble that of autoimmune encephalitis. Given that scrub typhus is amenable to treatment with antibiotics, such as doxycycline and azithromycin, which do not increase seizure risk, it should be considered in the differential diagnosis for patients presenting with seizures or encephalitis, especially in endemic areas.

05 Mar 2025·Cureus

Hemifacial Spasms or Focal Aware Seizures: The Role of Video-EEG

Article

Author: Duarte-Celada, Walter R ; Davila-Siliezar, Pamela ; Moncayo, Juan A ; Ferdous, Jannatul

Hemifacial spasms (HFS) are common in neurological practice but can be mistaken for focal aware seizures without appropriate diagnostic workup. We present the case of a man in his 60s admitted for congestive heart failure (CHF) exacerbation, who developed left-sided weakness and HFS during hospitalization. Although his motor symptoms improved after receiving tenecteplase (TNK), the HFS persisted, prompting the use of continuous video-EEG to differentiate between cortical myoclonus and HFS. Video-EEG is essential for distinguishing HFS from focal aware seizures, identifying high-frequency ipsilateral muscle artifacts in HFS versus interictal or ictal EEG patterns contralateral to facial contractions in seizures. This case highlights key features to aid neurologists in differentiating these conditions.

01 Mar 2025·Movement Disorders Clinical Practice

Diagnostic Utility of Clinical Neurophysiology in Jerky Movement Disorders: A Review from the MDS Clinical Neurophysiology Study Group

Review

Author: Rocchi, Lorenzo ; Ganos, Christos ; Hamada, Masashi ; Merchant, Shabbir ; Phielipp, Nicolas ; Latorre, Anna ; Chen, Robert ; van der Veen, Sterre ; Tijssen, Marina A.

AbstractBackgroundMyoclonus and other jerky movement disorders are hyperkinetic disorders, the diagnosis of which heavily relies on clinical neurophysiological testing. However, formal diagnostic criteria are lacking, and recently the utility and reliability of these tests have been questioned.ObjectiveThe aim of this review was to assess the utilization of clinical neurophysiology testing to identify possible gaps and boundaries that might guide the development of new methods for a more precise diagnosis and in‐depth understanding of myoclonus.MethodsWe reviewed electrophysiological features of cortical myoclonus, subcortical myoclonus (ie, myoclonus associated with dystonia, brainstem myoclonus), excessive startle reflex, spinal myoclonus (ie, spinal segmental and propriospinal myoclonus), peripheral myoclonus and mimics of myoclonus of peripheral origin (hemifacial spasm, minipolymyoclonus, myokymia), functional jerky movements, chorea, and tics.ResultsElectrophysiological features that support the recognition of myoclonus subtypes, such as muscle burst duration, muscle pattern of activation, measures of cortical excitability, or movement‐related cortical potentials, have been identified. These significantly contribute to the diagnosis of jerky movement disorders, but their reliability is uncertain. Despite the significant advancements, several unresolved questions persist. Factors contributing to this include the absence of systematic neurophysiological assessment and standardized methods, alongside the limited number of patients investigated using these techniques.ConclusionAlthough clinical neurophysiology remains the “gold standard” for defining and diagnosing myoclonus, our review highlighted the need to enhance the quality and reliability of neurophysiological testing in jerky movement disorders. Further studies including larger cohorts of patients recruited from different centers, employing standardized and optimized electrophysiological techniques, are warranted.

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