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Tremor Other Hyperkinet Mov (N Y) 2022 4;12:14. Epub 2022 May 4.

Department of Neurology, University Hospital "12 de Octubre", Madrid, Spain.

Background: Multiple sclerosis (MS), a subset of chronic primary inflammatory demyelinating disorders of the central nervous system, is closely associated with various movement disorders. These disorders may be due to MS pathophysiology or be coincidental. This review describes the full spectrum of movement disorders in MS with their possible mechanistic pathways and therapeutic modalities. Read More

Brain Sci 2022 Mar 29;12(4). Epub 2022 Mar 29.

Department of Neurology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu 501-1194, Japan.

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is a type of autoimmune corticosteroid-responsive meningoencephalitis that occurs with or without myelitis. Movement disorders have been reported in GFAP-A patients but have not been characterized. In this study, we examined the characteristics of movement disorders in GFAP-A patients. Read More

eNeurologicalSci 2022 Jun 7;27:100395. Epub 2022 Mar 7.

Department of Neurology, Faculty of Medicine, University of Yamanashi, Japan.

We report a 77-year-old woman with a thymoma, anti-LGI1antibody associated encephalitis (LGI1 encephalitis), and MG accompanied by positive anti-acetylcholine receptor antibodies (AchR Ab) and anti-titin antibodies (titin Ab). She was treated with thymomectomy followed by immunosuppressive therapy, which resulted in immediate amelioration of motor weakness and gradual improvement of cognitive impairment over the next two years. LGI1 Ab were positive at two months after thymomectomy, followed by negative conversion demonstrated on 1 year examination. Read More

J Clin Neurol 2022 Mar;18(2):235-237

Department of Neurology, Ajou University Medical Center, Ajou University School of Medicine, Suwon, Korea.

Neurology 2022 05 9;98(18):767-772. Epub 2022 Mar 9.

From the Department of Neurology (A.S.B., A.F., J.M.B., B.R.), Department of Ophthalmology (A.F.), Department of Neurosurgery (J.M.B.), Yale School of Medicine, New Haven, CT.

A 73-year-old woman presented with transient episodes of dysarthria and horizontal diplopia. She had stereotactic radiosurgery 18 years prior for a retroclival meningioma. Neurologic examination was notable for right-sided tongue deviation, tongue fasciculations, and intermittent impaired abduction of the right eye. Read More

Clin Chim Acta 2022 May 3;530:74-80. Epub 2022 Mar 3.

Clinical Research Center for Children Neurodevelopmental disabilities of Hunan Province, Changsha 410008, China. Electronic address:

Background: Heterozygous variants of KCNQ2 can cause KCNQ2 associated neurodevelopmental disorder, mainly are benign (familial) neonatal or infantile epilepsy (B(F)NE or B(F)IE) and developmental epileptic encephalopathy(DEE). Moreover, some intermediate phenotypes, including intellectual disability (ID), and myokymia are related to the gene.

Methods: We collected a non-syndromic ID male patient with a novel KCNQ2 missense variant. Read More

Oper Neurosurg (Hagerstown) 2021 Dec 31. Epub 2021 Dec 31.

Department of Neurosurgery, Nakamura Memorial Hospital, Sapporo, Japan.

Background: Few reports have shown that superior oblique myokymia (SOM) may result from vascular compression of the trochlear nerve and may be curable using microvascular decompression (MVD).

Objective: To report the clinical characteristics and surgical treatment of 2 cases of SOM and provide a review of the related literature.

Methods: Two patients with SOM were treated using MVD with the lateral supracerebellar infratentorial approach. Read More

SN Compr Clin Med 2022 10;4(1):29. Epub 2022 Jan 10.

School of Optometry, The University of Faisalabad, Faisalabad, Pakistan.

The purpose of this study is to report eyelid myokymia in patients recently recovered from COVID-19 disease. A cohort of 15 patients who developed eyelid myokymia during or immediate post-recovery of systemic disease were evaluated. Demographic, clinical characteristics, effect of age, and hospitalization on the disease course were studied. Read More

Pract Neurol 2022 06 6;22(3):233-234. Epub 2022 Jan 6.

Department of Neurology, King's College Hospital, London, UK.

JAMA Neurol 2022 01;79(1):80-81

Department of Neurology, Institute of Post Graduate Medical Education and Research, Kolkata, India.

Mov Disord 2022 02 31;37(2):430-432. Epub 2021 Oct 31.

Neurology Department, Coimbra University Hospital Centre, Coimbra, Portugal.

Neurology 2022 02 29;98(5):e563. Epub 2021 Oct 29.

From the Department of Neurology (M.T.J.P., N.A.S., M.P.G.B.), Maastricht University Medical Center; and GROW-School for Oncology and Developmental Biology (M.P.G.B.), Maastricht University, the Netherlands.

Tremor Other Hyperkinet Mov (N Y) 2021 20;11:36. Epub 2021 Sep 20.

Department of Neurology and Clinical Neurophysiology, Amsterdam University Medical Centers, Amsterdam Neuroscience, University of Amsterdam, Amsterdam, The Netherlands.

Background: Hemifacial spasm is diagnosed on a clinical base, with certain atypical features alerting the physician for mimics.

Phenomenology Shown: Hemifacial neuromyotonia/myokymia characterized by tonic hemifacial contraction followed by multifocal undulating hemifacial twitches.

Educational Value: These features are a red flag for (post-irradiation) facial neuromyotonia/myokymia which generally responds well to low dose carbamazepine. Read More

Mov Disord Clin Pract 2021 Oct 26;8(7):1126-1128. Epub 2021 Aug 26.

Department of Neurology AIIMS New Delhi India.

Pediatr Neurol 2021 12 6;125:40-47. Epub 2021 Sep 6.

Division of Pediatric Neurology, University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Lemoyne, Pennsylvania. Electronic address:

Fasciculations are the most common form of spontaneous muscle contraction. They frequently occur in healthy individuals. However, there are a minority of situations that fasciculations are observed in association with specific neurologic disorders. Read More

Neurol Clin 2021 11;39(4):1083-1096

Department of Neurology, Emory University School of Medicine, 12 Executive Park Drive Northeast, Room 150H, Atlanta, GA 30329, USA. Electronic address:

Peripheral nerve hyperexcitability (PNH) typically presents with complaints of muscle twitching, cramps, and muscle stiffness. Symptoms and signs indicating central and/or autonomic nervous system dysfunction also may be reported. An electroclinical spectrum exists, spanning from the milder cramp-fasciculation syndrome to more severe syndromes characterized by continuous muscle fiber activity. Read More

Front Neurol 2021 9;12:703970. Epub 2021 Sep 9.

Department of Neurology and Epileptology, Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.

Among genetic paroxysmal movement disorders, variants in ion channel coding genes constitute a major subgroup. Loss-of-function (LOF) variants in , the gene coding for K1.1 channels, are associated with episodic ataxia type 1 (EA1), characterized by seconds to minutes-lasting attacks including gait incoordination, limb ataxia, truncal instability, dysarthria, nystagmus, tremor, and occasionally seizures, but also persistent neuromuscular symptoms like myokymia or neuromyotonia. Read More

Cureus 2021 Aug 2;13(8):e16844. Epub 2021 Aug 2.

General Medicine, Government Medical College and Hospital, Chandigarh, Chandigarh, IND.

Patients with snakebites have highly variable presentations, and delayed diagnosis may lead to unfavorable outcomes. Here, we describe the case of a snakebite in a 23-year-old male who presented with myokymias. On management with mechanical ventilation and anti-snake venom, the patient improved and was discharged. Read More

Neurol Clin Pract 2021 Jun;11(3):e267-e276

Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.

Objective: Morvan syndrome is characterized by central, autonomic, and peripheral hyperexcitability due to contactin-associated protein 2 (CASPR2) antibody. Our objective was to study the clinical spectrum, electrophysiologic, autonomic, polysomnographic, and neuropsychological profile in patients with CASPR2-related Morvan syndrome.

Methods: Serum and CSF samples that were CASPR2 antibody positive from 2016 to 2019 were assessed. Read More

Neurology 2021 Aug 11. Epub 2021 Aug 11.

Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy. IRCCS, Institute of Neurological Sciences of Bologna, Bologna, Italy.

Objective: Anti-IgLON5 disease is a recently described neurological disease that shares features of autoimmunity and neurodegeneration. Abnormal movements appear to be frequent and important but have not been characterized and are under-reported. Here we describe the frequency and types of movement disorders in a series of consecutive patients with this disease. Read More

Clin Neurophysiol 2021 08 13;132(8):1830-1844. Epub 2021 May 13.

UK Dementia Research Institute, Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience, King's College London, UK; King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK.

Possessing a discrete functional repertoire, the anterior horn cell can be in one of two electrophysiological states: on or off. Usually under tight regulatory control by the central nervous system, a hierarchical network of these specialist neurons ensures muscular strength is coordinated, gradated and adaptable. However, spontaneous activation of these cells and their axons can result in abnormal muscular twitching. Read More

J Neuroophthalmol 2021 Jun;41(2):176-193

Department of Ophthalmology, University of Alabama School of Medicine (LBK, MSV, MT), Birmingham, Alabama; The Shiley Eye Institute, University of California San Diego (LBK), San Diego, California; and The Jules Stein Eye Institute, University of California, Los Angeles (JLD), Los Angeles, California.

Abstract: This review of disorders of the fourth cranial nerve includes discussion on anatomy, examination techniques, congenital and acquired etiologies, differential diagnosis, and management options. The findings of the superior oblique muscle on orbital MRI in patients with fourth nerve palsy have had a major impact on our understanding of this cranial neuropathy. In addition, briefly reviewed are rare disorders of the fourth nerve: superior oblique myokymia, Brown syndrome, and ocular neuromyotonia. Read More

BMC Neurol 2021 May 3;21(1):185. Epub 2021 May 3.

Department of Neurology and Research Center of Neurology in Second Affiliated Hospital, Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China.

Background: Morvan syndrome (MoS) is a rare autoimmune syndrome associated with antibodies against two kinds of potassium channel proteins, contactin associated protein-like 2 (CASPR2) and leucine-rich glioma inactivated protein 1 (LGI1). MoS patients with only LGI1-antibody seropositivity have rarely been reported. Here, we describe a 64-year-old male MoS patient with only LGI1-antibody seropositivity. Read More

Neurology 2021 07 26;97(1):e111-e112. Epub 2021 Apr 26.

From the Departments of Neurology (M.S., F.S., A.S.C.) and Neuroradiology (P.P.), Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental; and CEDOC-NOVA Medical School (A.S.C.), Universidade Nova de Lisboa, Portugal.

J Neuroimmunol 2021 06 7;355:577569. Epub 2021 Apr 7.

Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. Electronic address:

Objective: To analyze the clinical characteristics and outcomes of patients diagnosed with acquired neuromyotonia and who were treated with tacrolimus.

Methods: A single center, retrospective study was performed on patients with acquired meuromyotonia whose treatment included tacrolimus. The clinical information, antibody tests, and electromyography results were reviewed. Read More

J Vet Intern Med 2021 May 26;35(3):1218-1230. Epub 2021 Mar 26.

Department of Small Animal Medicine and Surgery, University of Veterinary Medicine Hannover, Hannover, Germany.

Movement disorders are a heterogeneous group of clinical syndromes in humans and animals characterized by involuntary movements without changes in consciousness. Canine movement disorders broadly include tremors, peripheral nerve hyperexcitability disorders, paroxysmal dyskinesia, and dystonia. Of these, canine paroxysmal dyskinesias remain one of the more difficult to identify and characterize in dogs. Read More

Intern Med 2021 Aug 8;60(16):2687-2691. Epub 2021 Mar 8.

Department of Neurology, Kobe City Medical Center General Hospital, Japan.

We herein report a 48-year-old woman receiving eribulin mesylate for breast cancer who presented with gait disorder, distal limb paresthesia, and weakness progressing monthly. A nerve conduction study indicated demyelination with multifocal conduction block. Considering the immune-mediated pathology of her condition, she was administered intravenous immunoglobulin. Read More

Muscle Nerve 2021 06 24;63(6):861-867. Epub 2021 Mar 24.

Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA.

Introduction: Myokymic discharges are classically associated with nerve injury from prior radiation but may occur in other neuromuscular disorders. Using quantitative analysis we aimed to identify the spectrum of conditions in which myokymic discharges are present and determine if there are electrophysiological features that distinguish postradiation from nonradiation causes of myokymia.

Methods: We reviewed the clinical history of all patients examined in our electromyography labs with myokymic discharges recorded from June 2017 to February 2020. Read More

Brain Circ 2020 Oct-Dec;6(4):225-241. Epub 2020 Dec 29.

Department of Medicine, Federal University of Fronteira Sul, Passo Fundo, RS, Brazil.

Neurocysticercosis (NCC) is a specific form of cysticercosis that affects the central nervous system. It is caused by the tapeworm , which is often found in pigs. NCC is considered one of the "great simulator/mimickers" of other diseases. Read More