806 results match your criteria: "Myokymia"

Background And Importance: Superior oblique myokymia (SOM) is a rare, acquired aberration of the innervation of the superior oblique, resulting in episodic monocular contraction of the superior oblique muscle characterized by intermittent rotatory eye movement causing diplopia and oscillopsia. Several treatment modalities have been described to treat SOM, including medication and surgical interventions. There is a paucity of reports describing microvascular decompression (MVD) of the trochlear nerve near the root entry zone for the treatment of a neurovascular conflict.

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Background: Myoclonus 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.

Objective: The 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.

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Metformin-induced eyelid myokymia.

Indian J Pharmacol

September 2024

Department of Obstetrics and Gynaecology, Latifa Hospital, Dubai Health Authority, Dubai, UAE.

Metformin, an insulin-sensitizer, is used in the treatment of polycystic ovarian syndrome (PCOS) in obese women as it helps in weight loss and regularizes the menstrual cycle. However, impaired absorption of Vitamin B12 due to metformin has also been hypothesized. Here, we report the case of a 39-year-old woman prescribed metformin for PCOS, presenting with an unusual symptom of eyelid myokymia.

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Background And Importance: We describe, to our knowledge, the first report of fully endoscopic microvascular decompression (MVD) of the trochlear nerve in a patient with superior oblique myokymia (SOM).

Clinical Presentation: A 51-year-old female presented with multiple years of intermittent, "jumpy," and "shimmering" visual disturbances. She was diagnosed with SOM.

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Isolated Hemifacial Spasm as the Presenting Sign of Cerebral Glioblastoma.

Ophthalmic Plast Reconstr Surg

December 2024

Department of Ophthalmology and Visual Science, The University of Texas Health Science Center at Houston, Houston, Texas, U.S.A.

The authors report a rare case of a 58-year-old female with mild right-sided hemifacial spasms and eyelid myokymia and a concomitant high-grade glial mass. This report outlines the clinical presentation, diagnostic approach, and management of right hemifacial spasms and eyelid myokymia. The patient had a 5-month history of gradually worsening right hemifacial spams accompanied by mild right lower eyelid twitching.

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Introduction: The treatment options available for Superior Oblique Myokymia (SOM) previously included surgery and systemic drugs, with the addition of beta blockers from the early 1990's. This case review aims to examine the efficacy of topical timolol (0.5%) as a treatment for SOM in the short and long term.

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Case Summary: A 16-year-old spayed female domestic shorthair cat with methimazole-treated hyperthyroidism presented with a chronic progressive history of a stiff gait progressing to recumbency. A neurological examination revealed continuous excessive muscle tone with myokymia, which exacerbated with exercise and persisted during general anaesthesia. An electromyographic study revealed myokymic discharges in all tested muscles, as well as complex repetitive discharges, fibrillation potentials and positive sharp waves.

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Small Complex Rearrangement in -Related Axonal Neuropathy.

Genes (Basel)

November 2024

Department of Neuroscience, IRCCS Stella Maris Foundation, 56128 Pisa, Italy.

Background: Autosomal recessive inherited pathogenetic variants in the histidine triad nucleotide-binding protein 1 () gene are responsible for an axonal Charcot-Marie-Tooth neuropathy associated with neuromyotonia, a phenomenon resulting from peripheral nerve hyperexcitability that causes a spontaneous muscle activity such as persistent muscle contraction, impaired relaxation and myokymias.

Methods: Herein, we describe two brothers in whom biallelic variants were identified following a multidisciplinary approach.

Results: The younger brother came to our attention for clinical evaluation of moderate intellectual disability, language developmental delay, and some behavioral issues.

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Neuromyotonia, also known as Isaac syndrome, is a rare neurological disorder characterized by continuous muscle activity, stiffness, and spontaneous muscle contractions, it is very rare in children. We report a 16-year-old female patient with neuromyotonia. She presented with pain, stiffness, autonomic symptoms and muscle myokymia in both lower limbs.

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Article Synopsis
  • Morvan syndrome is a rare autoimmune disorder primarily affecting males, characterized by symptoms like muscle twitching, autonomic disturbances, and severe sleep issues, often linked to specific autoantibodies.
  • Current treatments include various immunotherapies, but no long-term cure exists, prompting interest in alternative therapies like traditional Chinese medicine.
  • Three patients with serum Caspr2-positive Morvan syndrome showed some relief from traditional treatments but experienced disease recurrence, indicating the complexity and unpredictability of the condition.
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Article Synopsis
  • Isaacs' syndrome is a rare condition that causes persistent muscle activity, leading to symptoms like twitching, stiffness, and cramps, and is associated with peripheral nerve hyperexcitability.
  • A study involving 12 patients examined their clinical signs, diagnostic tests, antibody presence (like LGI1 and CASPR2), and the search for related cancers, which ultimately returned inconclusive results.
  • The patients responded well to treatments like carbamazepine or gabapentin, along with immunotherapies, resulting in full recovery within 3-6 months.
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Article Synopsis
  • Morvan syndrome, or Morvan's fibrillary chorea, is a rare neurological condition linked to cancer, showing symptoms that affect the CNS, peripheral nerves, and autonomic functions.
  • A case study highlighted an adult female who suddenly experienced chest pain, weakness, and muscle spasms, revealing positive VGKC and amphiphysin antibodies in her blood.
  • The diagnosis of Morvan syndrome involves recognizing myokymia, positive VGKC antibodies, and neuropsychiatric symptoms, with this case demonstrating a unique link to amphiphysin positivity.
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Introduction:  Previous studies have shown that isolated eyelid myokymia (EM) is usually caused by stress, fatigue, and caffeine consumption. The purpose of this study was to evaluate the association between EM and digital screen time, uncorrected refractive error, intraocular pressure (IOP), and blood electrolyte levels.

Methods: Between February 2023 and June 2024, 103 eyes of 103 patients who applied to the ophthalmology outpatient clinic with complaints of eyelid twitching lasting for more than two weeks and 103 eyes of 103 healthy individuals as a control group were included in the study.

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Neuromyotonia.

Handb Clin Neurol

August 2024

UCL Queen Square Institute of Neurology, Department of Neuroinflammation, and National Hospital for Neurology and Neurosurgery, London, United Kingdom. Electronic address:

Article Synopsis
  • * The disorder can be genetic or acquired, with a significant focus on autoimmune causes such as CASPR2 and LGI1 antibody diseases, Guillain-Barré syndrome, and others.
  • * Treatment strategies involve addressing any underlying tumors, using immune suppression therapies for autoimmune cases, and sodium channel blockers for symptom management.
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Article Synopsis
  • This paper discusses the concept of spectral resonance in the Fitzhugh-Nagumo (FHN) system when influenced by periodic signals and unbounded Gaussian noise.
  • It reveals a connection between stochastic resonance and spectral resonance based on the standard deviation of inter-spike intervals (ISI), highlighting how different signal frequencies affect the ISI distribution.
  • The study suggests using the entropy function as an alternative metric to detect spectral resonance and relates ISI distributions from the FHN system to real EMG measurements in conditions like myokymia and neuromyotonia, indicating potential uses in identifying various neural activities.
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BACKGROUND Morvan fibrillary chorea (Morvan syndrome) is a rare disorder marked by a collection of neurological symptoms such as myokymia, peripheral nerve excitability, neuromyotonia, autonomic instability, memory impairment, and delirium. Morvan syndrome is suspected to occur through antibodies directed against voltage gated potassium channels (VGKC), and has been linked with several autoimmune conditions and hematologic malignancies. We present a case of Morvan syndrome in association with monoclonal B cell lymphocytosis.

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Oral management for a patient with trismus accompanied by Isaacs' syndrome: a case report.

BMC Oral Health

June 2024

Section of Geriatric Dentistry and Perioperative Medicine in Dentistry, Division of Maxillofacial Diagnostic and Surgical Science, Faculty of Dental Science, Kyushu University, Fukuoka, Japan.

Article Synopsis
  • * A patient with trismus due to Isaacs' syndrome had difficulty receiving dental treatment initially and later sought help at a specialized hospital where medication was given to alleviate pain and mouth opening issues.
  • * After undergoing plasmapheresis, the patient had temporary relief allowing for successful tooth extraction and dental restoration, highlighting the need for teamwork between neurologists and dentists in managing such cases.
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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated neuropathy affecting peripheral nerves and nerve roots. It is characterized by symmetric weakness involving both proximal and distal muscles; it can be relapsing-remitting or progressive in course. The clinical manifestations of CIDP are various and may present with atypical features, like myokymia, tremor, or tremor-like phenomena, which may mislead the clinician in diagnosis.

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Topiramate-induced ocular complications: case series.

Rom J Ophthalmol

April 2024

Department of Ophthalmology, Father Muller Medical College, Mangalore, India.

Unlabelled: Several ocular adverse effects have been attributed to Topiramate, a sulfonamide derivative. It can cause problems in the eye such as choroidal effusion syndrome, acute angle closure glaucoma, myopic shift, visual field defects, and Myokymia. If not identified early, it can be vision-threatening.

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Both gain- and loss-of-function variants of KCNA1 are associated with paroxysmal kinesigenic dyskinesia.

J Genet Genomics

August 2024

Department of Medical Genetics and Center for Rare Diseases, and Department of Neurology, and Zhejiang Key Laboratory of Rare Diseases for Precision Medicine and Clinical Translation in Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, China; MOE Frontier Science Center for Brain Science and Brain-machine Integration, School of Brain Science and Brain Medicine, Zhejiang University, Hangzhou, Zhejiang 310012, China. Electronic address:

KCNA1 is the coding gene for Kv1.1 voltage-gated potassium-channel α subunit. Three variants of KCNA1 have been reported to manifest as paroxysmal kinesigenic dyskinesia (PKD), but the correlation between them remains unclear due to the phenotypic complexity of KCNA1 variants as well as the rarity of PKD cases.

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Background: We used electromyography to characterize hypoglossal nerve function among radiation-treated head and neck cancer survivors with later onset unilateral tongue immobility.

Methods: Patients with unilateral tongue immobility without evidence of recurrent cancer were seen at a tertiary academic institution between February and September 2021. All patients were at least 2 years post-treatment with radiation therapy for head and neck squamous cell carcinoma.

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A 61-year-old man presented with 2 days of "flickering" vision. The symptom resolved with closure of the left eye. Examination demonstrated involuntary high-frequency, low-amplitude intorting movements of the left eye, consistent with superior oblique myokymia (Video 1).

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