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AJNR Am J Neuroradiol 2020 05 7;41(5):917-922. Epub 2020 May 7.

From the Departments of Radiology and Division of Neuroradiology (F.G.G., B.H., C.A.P.F.A., S.R.T., J.S.M.-S., A.V., G.Z.).

Pathogenic variants in the () cause a diverse group of pathologies known as -related disorders. In this report, we describe brain MR imaging findings and electroencephalogram correlates of 13 children with -related disorders at diagnosis and follow-up. At diagnosis, all patients had seizures and 12 had abnormal MR imaging findings. Read More

Neuroradiology 2020 Jul 26;62(7):895-899. Epub 2020 Mar 26.

Department of Neurology, University Medical Center Bonn, Bonn, Germany.

Epileptic seizures associated with hyperglycemia have a rare but characteristic MR imaging pattern which however is frequently missed. It consists of a T2 hypointensity and an apparent diffusion coefficient (ADC) decrease of the white matter underlying the epileptic cortex; the cortex itself may be DWI hyperintense and show a blood-brain barrier disruption. Prompt diagnosis is relevant since treatment of the hyperglycemic state rather than treatment with anti-epileptic drugs frequently interrupts the seizures. Read More

J Neurol Sci 2020 Jun 2;413:116762. Epub 2020 Mar 2.

Division of Neurology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. Electronic address:

Intern Med 2020 Jun 5;59(11):1445-1449. Epub 2020 Mar 5.

Department of Neurology, Japanese Red Cross Medical Center, Japan.

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been associated with steroid-responsive cortical encephalitis and comorbid generalized epilepsy. A 44-year-old woman developed repeated epilepsia partialis continua (EPC) without generalized seizures and was anti-MOG antibody-positive. Radiological abnormalities were detected in the bilateral medial frontoparietal cortices, but there were no cerebrospinal fluid abnormalities. Read More

Seizure 2020 Jan 28;76:153-155. Epub 2020 Jan 28.

Oxford Epilepsy Research Group, NIHR Biomedical Research Centre, Nuffield Department of Clinical Neuroscience, John Radcliffe Hospital, Oxford OX3 9DU, UK. Electronic address:

Acta Neurol Belg 2020 Feb 10. Epub 2020 Feb 10.

Department of Rheumatology, Shin Ill Medical Clinic, Seoul, South Korea.

Am J Med Genet A 2020 04 25;182(4):705-712. Epub 2020 Jan 25.

Faculty of Medicine, Department of Pediatrics, Division of Pediatric Metabolism, Hacettepe University, Ankara, Turkey.

Phosphomannomutase 2 deficiency (PMM2-CDG) is an autosomal recessive congenital disorder of glycosylation, characterized by multisystem phenotypes, mostly including neurological involvement. In Turkey, due to high rates of consanguinity, many patients with autosomal recessive disorders have homozygous variants and these diseases are more common, compared to Europe. However, published reports of PMM2-CDG from Turkey are scarce. Read More

World Neurosurg 2020 Apr 16;136:140-145. Epub 2020 Jan 16.

Department of Neurosurgery, Tianjin Medical University General Hospital, Tianjin, R.P. China. Electronic address:

Background: This is a rare case of a patient presenting with epileptic seizures and headaches who was diagnosed with spontaneous intracerebral dermoid cyst rupture via radiographic imagery, and rupture was confirmed via a pathology report.

Case Description: We report the case of a woman aged 26 years who presented with a history of chronic headache for 9 years without other symptoms, and progressive worsening of her headache had occurred for 1 month prior to admission. Radiologic examination showed a large mass located in the left temporal fossa and a large amount of homogeneous matter in the subarachnoid space of the ipsilateral cerebral hemisphere, then the tumor was completely excised. Read More

Epileptic Disord 2019 Dec;21(6):603-607

Department of Radiology, Marqués de Valdecilla University Hospital,, Biomedical Research Institute (IDIVAL), Santander, Cantabria, Spain.

Motor epilepsia partialis continua is a widely described variant of simple focal motor status epilepticus. However, few studies have addressed associated pathophysiological anomalies that may help us understand the cortical organization, basic functioning and control of voluntary movement. We describe the clinical, video-EEG and neuroimaging findings from two cases of motor epilepsia partialis continua that support the hypothesis of the coexistence of both classic body and complex motor map models in the cortical organization of voluntary movement in humans. Read More

Neurohospitalist 2020 Jan 30;10(1):58-63. Epub 2019 May 30.

Department of Neurology, University of South Florida, Tampa, FL, USA.

We present the case of an 18-year-old woman with B-cell acute lymphoblastic leukemia (ALL) who developed hemorrhagic stroke and epilepsia partialis continua due to acute cerebral vein thrombosis (CVT). The patient had 10 risk factors for CVT (including use of asparaginase chemotherapy for the ALL) and also unfortunately had 4 biomarkers for poor prognosis for outcome post-CVT diagnosis. Immediate transfer to a Comprehensive Stroke Center allowed for hyperacute neurointerventional clot extraction with rapid restoration of the patency of the superior sagittal sinus. Read More

J Neurovirol 2020 Jun 5;26(3):437-441. Epub 2019 Dec 5.

Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, Piazzale Santa Maria della Misericordia, 15, 33010, Udine, Italy.

Idelalisib, a selective phosphatidylinositol 3-kinase delta (PI3Kδ) inhibitor, is a newly approved second-line drug for patients with chronic lymphocytic leukemia. Recent clinical trials have suggested a possible association between idelalisib treatment and development of progressive multifocal leukoencephalopathy (PML) due to John Cunningham virus (JCV) reactivation. Nevertheless, clinical course and radiological and pathological features of idelalisib-induced PML still need to be clarified. Read More

Epilepsy Behav Rep 2019 25;12:100339. Epub 2019 Oct 25.

Department of Biological and Medical Psychology, University of Bergen, Jonas Lies vei 21, 5009 Bergen, Norway.

We report a 15-year-old female with POLG-related mitochondrial disease who developed severe multifocal epilepsia partialis continua, unresponsive to standard anti seizure drug treatment and general anesthesia. Based on an earlier case report, we treated her focal seizures that affected her right upper limb with 20-min sessions of transcranial direct current stimulation (tDCS) at an intensity of 2 mA on each of five consecutive days. The cathode was placed over the left primary motor cortex, the anode over the contralateral orbitofrontal cortex. Read More

Nephron 2020 13;144(3):156-160. Epub 2019 Nov 13.

Division of Pediatric Nephrology, Ludwig-Maximilians University, Dr. v. Hauner Children's Hospital, Munich, Germany,

Autosomal-dominant tubulointerstitial kidney disease -(ADTKD) describes tubulointerstitial kidney disease with autosomal-dominant inheritance. In 2017, the term mitochondrial tubulointerstitial kidney disease (MITKD) was introduced for tubulointerstitial kidney disease caused by mitochondrial DNA (mtDNA) mutations. To date, there are few mutations described in literature causing MITKD, one of them is m. Read More

Zhonghua Er Ke Za Zhi 2019 Nov;57(11):844-851

Department of Neurology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.

To summarize the clinical and genetic characteristics of children with mitochondrial epilepsy. Clinical data of 62 children who were clinically and genetically diagnosed with mitochondrial epilepsy by the Department of Neurology, Beijing Children's Hospital from October 2011 to December 2018 were analyzed retrospectively, and the control of epilepsy was followed up. test or χ(2) test were used to analyze the related factors affecting the prognosis of epilepsy between the effective group and the ineffective group. Read More

Oper Neurosurg (Hagerstown) 2020 May;18(5):E175-E180

Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital, Memphis, Tennessee.

Background And Importance: Presurgical mapping of eloquent cortex in young patients undergoing neurosurgery is critical for timely intervention, surgical planning, and minimizing postoperative deficits. However, invasive direct cortical stimulation has limited success in young children and noninvasive modalities, such as magnetoencephalography and functional MRI, require sedation, often precluding localization of critical language cortices. Transcranial magnetic stimulation (TMS), a noninvasive brain stimulation technique, is well suited to evaluate language areas in young children because it does not require the patient to remain still during mapping. Read More

J Med Case Rep 2019 Jun 24;13(1):191. Epub 2019 Jun 24.

Forth Valley Royal Hospital, Larbert, and Usher Institute of Population Health Sciences and Informatics, The University of Edinburgh, Edinburgh, UK.

Background: We describe a patient copresenting with epilepsia partialis continua, tuberculosis, and hemophagocytic lymphohistiocytosis. To our knowledge, this is the first documented case of this triad.

Case Presentation: A 54-year-old black South African woman presented to a hospital in Scotland with an acute history of right-sided facial twitching, breathlessness, and several months of episodic night sweats. Read More

Epileptic Disord 2019 Jun;21(3):302-306

Department of Neurology, St Stephen's Hospital, New Delhi.

Epilepsia partialis continua (EPC) of abdominal muscles is a rare entity with variable clinical localization and aetiology. A 25-year-old man presented with sudden onset of intermittent focal myoclonic movements involving the abdominal muscles on the right side exclusively, lasting from 20 minutes to an hour. Brain MRI revealed a ring-enhancing lesion, suggestive of cysticercal granuloma over the left precentral gyrus. Read More

Brain Stimul 2019 Sep - Oct;12(5):1101-1110. Epub 2019 May 14.

Department of Neurology, Christian-Doppler University Hospital, Paracelsus Medical University, Centre for Cognitive Neuroscience, Salzburg, Austria; Institute of Public Health, Medical Decision Making and HTA, UMIT, Private University for Health Sciences, Medical Informatics and Technology, Hall in Tyrol, Austria.

Rationale: Refractory status epilepticus (RSE) is the persistence of status epilepticus despite second-line treatment. Super-refractory SE (SRSE) is characterized by ongoing status despite 48 h of anaesthetic treatment. Due to the high case fatality in RSE of 16-39%, off label treatments without strong evidence of efficacy in RSE are often administered. Read More

Seizure 2019 Jul 13;69:228-234. Epub 2019 May 13.

Department of Pediatrics, Peking University First Hospital, No. 1 of Xian Men Street, Xicheng District, Beijing, 100034, China. Electronic address:

Purpose: To summarize the clinical features and neuroimaging changes of epilepsy associated with TBC1D24 mutations.

Methods: Genetic testing was conducted in all epilepsy patients without acquired risk factors for epilepsy. Epilepsy patients identified with TBC1D24 compound heterozygous mutations by next-generation sequencing (NGS) epilepsy panel or whole exome sequencing (WES) were enrolled. Read More

J Investig Med High Impact Case Rep 2019 Jan-Dec;7:2324709619848816

1 Louisiana State University Health Sciences Center, New Orleans, LA, USA.

Refractory seizures or status epilepticus (RS/SE) continues to be a challenge in the inpatient setting. Failure to abort a seizure with antiepileptic drugs (AEDs) may lead to intubation and treatment with general anesthesia exposing patients to complications, extending hospitalization, and increasing the cost of care. Studies have shown a key role of inflammatory mediators in seizure generation and termination. Read More

Acta Neurol Scand 2019 Sep 6;140(3):184-193. Epub 2019 Jun 6.

Department of Pediatric Neuroscience, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Objectives: To determine the prevalence of epilepsy in children with early-onset mitochondrial diseases (MDs) and to evaluate the epileptic phenotypes and associated features.

Materials And Methods: Children affected by MD with onset during the first year of life were enrolled. Patients were classified according to their mitochondrial phenotype, and all findings in patients with epilepsy versus patients without were compared. Read More

Mol Cytogenet 2018 10;11:42. Epub 2018 Aug 10.

Genetics Laboratory, University of Oklahoma Health Sciences Center, 1122 NE 13th Street, Suite 1400, Oklahoma City, OK 73104 USA.

Background: The clinical features of Down syndrome vary among individuals, with those most common being congenital heart disease, intellectual disability, developmental abnormity and dysmorphic features. Complex combination of Down syndrome phenotype could be produced by partially copy number variations (CNVs) on chromosome 21 as well. By comparing individual with partial CNVs of chromosome 21 with other patients of known CNVs and clinical phenotypes, we hope to provide a better understanding of the genotype-phenotype correlation of chromosome 21. Read More

Neurologia 2019 May 1. Epub 2019 May 1.

Unidad de Epilepsia, Servicio de Neurología, Hospital Clínico San Carlos, Madrid, España; Instituto de Investigación Sanitaria, Hospital Clínico San Carlos (IdISSC), Madrid, España.

Introduction: Timing is one of the most important modifiable prognostic factors in the management of status epilepticus. Epilepsia partialis continua (EPC) is a status epilepticus subtype of highly variable, occasionally prolonged, duration. The aim of this study was to analyze the relationship between EPC duration and outcomes. Read More

Eur Rev Med Pharmacol Sci 2019 Apr;23(7):3001-3004

IRCCS "NEUROMED", Pozzilli (IS), Italy.

Epilepsia partialis continua (EPC) is a rare form of focal motor status epilepticus characterized by continuous muscular twitches or jerks involving a limited part of the body, usually facial region and distal limb. Although the cerebrovascular disease is known to be one of the most common causes of this condition, other reported cases with predominant abdominal involvement have different aetiologies, including, tumors, focal cortical dysplasia, and central nervous system infections. No cases of epilepsia partialis continua of the abdominal wall occurred after brain surgery have been previously reported. Read More

Pediatr Neurol 2019 10 8;99:94. Epub 2019 Feb 8.

Pediatric Neurology Division, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

Neurohospitalist 2019 Apr 15;9(2):93-99. Epub 2018 Oct 15.

Department of Nuclear Medicine, Municipal Hospital Landshut, Germany.

A compilation of 6 distinctive F-fluorodeoxyglucose positron emission tomography (PET) combined with computed tomography (CT) findings in the acute setting of neurohospital care is presented. In case 1, PET/CT allowed the final diagnosis of circumscribed ischemic infarction by demonstrating a clear pattern of luxury perfusion. In case 2, diagnosis of thalamic abscess was made, whereby PET/CT demonstrated an empty zone. Read More

J Neurol Neurosurg Psychiatry 2019 Aug 22;90(8):920-928. Epub 2019 Feb 22.

Neurology, Krembil Brain Institute; Movement Disorders Centre and the Edmond J Safra Program in Parkinson's Disease, Toronto, Ontario, Canada

Seizures and movement disorders (MDs) are distinct neurological conditions presenting with abnormal movements. Despite sharing an overlap in phenomenology, these movements have different origins. In order to explore the overlaps and the narrow boundaries between these two conditions, we performed a review of the literature to explore the risk of seizures in MDs. Read More

Epilepsy Behav Case Rep 2019 26;11:77-80. Epub 2019 Jan 26.

Division of Neurology, Department of Internal Medicine, National Yang-Ming University Hospital, No.169, Siaoshe Rd., Yilan City, Yilan County 26058, Taiwan.

Creutzfeldt-Jakob disease (CJD) presents with seizures as an early symptom in only approximately 3% of cases. These seizures often present as nonconvulsive status epilepticus (NCSE) or epilepsia partialis continua (EPC). Here, we describe a case of probable sporadic CJD (sCJD) in an 83-year-old man whose manifest an unusual presentation of left-hand tonic seizures without evolution to EPC, as well as brain MRI findings interpreted as peri-ictal changes, which led to an initial misdiagnosis of focal epilepsy. Read More

Clin Neurophysiol 2019 04 25;130(4):439-444. Epub 2019 Jan 25.

Neurology, Neurophysiology and Neurobiology Unit, Department of Medicine, Università Campus Bio-Medico di Roma, Rome, Italy. Electronic address:

Objective: The pathophysiology of epilepsia partialis continua (EPC) is still unclear, a thalamo-cortical circuit dysfunction has been hypothesized. The aim of present study is the functional evaluation of the thalamo-cortical network in EPC by means of the study of low- and high-frequency somatosensory evoked potentials (LF-SEP and HF-SEP).

Methods: Median LF-SEP and HF-SEP were recorded in 3 patients with EPC and in 2 patients with rolandic lesions without EPC (non-EPC). Read More

Epileptic Disord 2019 Feb;21(1):108-111

Department of Neurology, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires.

A female patient with electroclinical and neuroradiological features compatible with Rasmussen syndrome developed a particular clinical and EEG pattern. As the seizures were refractory to valproate at 750 mg/kg/day, oxcarbazepine (OXC) at 30 mg/kg/day was added. Seizures became more frequent and on neurological examination, no hemiparesis was detected. Read More

Epilepsy Res 2019 03 9;151:24-30. Epub 2019 Jan 9.

Section of Epilepsy, Department of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and Chang Gung University College of Medicine, Taoyuan, Taiwan; Department of Neurology, Xiamen Changgung Hospital, China. Electronic address:

Objective: To report the long-term seizure control and safety of open-loop electrical cortical stimulation in patients with refractory focal epilepsy of diverse etiologies.

Methods: Six patients who received a therapeutic trial of cortical stimulation were included retrospectively. The frequency of seizures was recorded before and after implantation. Read More

Epilepsy Behav Case Rep 2019 28;11:54-57. Epub 2018 Nov 28.

Department of Neurosurgery, UT Health San Antonio, San Antonio, TX, United States of America.

We describe a 23-year-old woman with previous right temporal lobe surgeries for underlying cortical dysplasia, presenting with right hemispheric seizures and epilepsia partialis continua (EPC). After anti-seizure medication adjustments, she developed focal status epilepticus with progressive EEG and neuroimaging changes. Cerebrospinal fluid and serum autoimmune panels were negative except for an elevated serum acetylcholine-receptor antibody titer, but she underwent immunosuppressive therapy. Read More

Epilepsy Behav Case Rep 2019 28;11:43-46. Epub 2018 Nov 28.

Department of Neurology, Mayo Clinic, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA.

Both variegate and acute intermittent porphyria can manifest with various neurological symptoms. Although acute symptomatic seizures have been previously described, they are typically tonic-clonic and focal impaired awareness seizures. Convulsive status epilepticus and epilepsia partialis continua are rare and have been described on a case report basis. Read More

Eur J Paediatr Neurol 2019 Mar 21;23(2):240-247. Epub 2018 Dec 21.

Pediatric Diabetology Unit, Department of Maternal and Infantile Health, Sapienza University of Rome, Italy.

We provide an overview on the current knowledge about the association between epilepsy and type 1 diabetes mellitus (T1DM). People with T1DM have a 2-6-fold higher risk of epilepsy than the general population. The onset of T1DM anticipates the onset of epilepsy by a mean period between 1,5 and 2,8 years. Read More

Zhongguo Dang Dai Er Ke Za Zhi 2018 Dec;20(12):1008-1014

Department of Emergency, Xiangya Hospital, Central South University, Changsha 410008, China.

Objective: To investigate the etiology and clinical features of epilepsia partialis continua (EPC) in children.

Methods: A retrospective analysis was performed for the clinical features, diagnosis and treatment of six children with EPC, and the clinical and laboratory features and prognosis were compared between the children with different etiologies.

Results: There were five girls and one boy, with an onset age ranging from one year and seven months to nine years. Read More

Can J Neurol Sci 2018 Dec 17:1-7. Epub 2018 Dec 17.

1Division of Neurology,Krembil Brain Institute,University Health Network, Toronto Western Hospital,University of Toronto,Toronto, Ontario,Canada.

A patient with intractable epilepsy, previous right frontal resection, and active vagus nerve stimulation (VNS) developed new onset quasi-continuous twitching around the left eye. Electroencephalography showed no correlate to the orbicularis oculi twitches apart from myographic potentials at the left supraorbital and anterior frontal electrodes. Magnetoencephalography was performed using spatiotemporal signal space separation to suppress magnetic artifacts associated with the VNS apparatus. Read More

Mult Scler Relat Disord 2019 Feb 3;28:31-33. Epub 2018 Dec 3.

Department of Neurology, St Vincent's Hospital, 390 Victoria St, Darlinghurst, Sydney, NSW 2010, Australia.

Secondary autoimmune disorders are a recognised complication of alemtuzumab treatment for multiple sclerosis. We report a case of autoimmune encephalitis manifesting as a polymorphic epilepsia partialis continua / status epilepticus seven months after the second course of alemtuzumab in a patient with previous autoimmune hypothyroidism and immune thrombocytopenic purpura. An MRI revealed multifocal cortical abnormalities and neuronal loss was evident on biopsy. Read More

Epileptic Disord 2018 Dec;20(6):468-478

Department of Neurosurgery, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.

The aim of this study was to analyse the electroclinical and imaging findings and outcome of patients with Rasmussen syndrome (RS) with atypical manifestations. We conducted a retrospective, descriptive study of 10 of 44 consecutive patients with RS with atypical features, followed between 1999 and 2017. Six patients were boys and four were girls. Read More

Eur J Med Genet 2019 Dec 27;62(12):103591. Epub 2018 Nov 27.

Division of Child Neurology and Psychiatry, Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy. Electronic address:

Background: Mutations in the KCTD7 gene have been associated with progressive myoclonus epilepsy and, in a single patient, with the so-called "Neuronal Ceroid Lipofuscinosis 14" (characterised by myoclonic seizures, cognitive regression, optic atrophy leading to visual loss, and progressive cortical and cerebellar atrophy).

Clinical Reports: We describe two new patients carrying two novel pathogenic mutations in the KCTD7 gene. Patient 1 (NM_153033. Read More

Ann Indian Acad Neurol 2018 Jul-Sep;21(3):225-227

Department of Medical Imaging, Taipei Medical University Hospital, Taipei, Taiwan.

Epilepsia partialis continua (EPC) is a rare epileptic syndrome, presenting as continuous focal motor seizures for a period of minutes, hours, or days. EPC may develop in patients with cerebral cortical lesions and occasionally may develop in patients with metabolic disorders, such as nonketotic hyperglycemia (NKH). Here, we report a case of EPC following NKH, showing an unusual magnetic resonance imaging (MRI) finding of concurrent hypointensity on susceptibility-weighted image (SWI) and T2-weighted image (T2WI) with leptomeningeal and cortical enhancement, which have never been reported. Read More

Can J Neurol Sci 2018 11 24;45(6):703-706. Epub 2018 Sep 24.

2Department of Neurology,Franz Tappeiner Hospital,Merano,Italy.

Zhonghua Bing Li Xue Za Zhi 2018 Sep;47(9):676-681

Department of Pathology, Beijing Haidian Hospital/Haidian Section of Peking University Third Hospital, Beijing 100080, China.

To investigate the clinicopathologic features of Rasmussen syndrome (RS) and to raise awareness of this rare disease. Clinicopathologic data of 4 cases of RS were retrospectively analyzed at Beijing Haidian Hospital from 2008 to 2016. The clinical manifestations included epilepsia partialis continua and progressive neurologic deficits in all patients. Read More

J Neuroimmunol 2018 10 24;323:119-124. Epub 2018 Jul 24.

Department of Neurology, Sir Charles Gairdner Hospital, Nedlands, Australia. Electronic address:

Antibodies against glutamic acid decarboxylase (GAD) are reported in association with numerous neurological conditions including temporal lobe epilepsy and limbic encephalitis. We report a case of Anti-GAD-Antibody associated encephalitis presenting with epilepsia partialis continua (EPC) progressing to a fulminant encephalopathy preferentially affecting the frontal lobes associated with coma and refractory status epilepticus. The abnormalities identified on MRI included marked bilateral frontal lobe involvement which has not been reported in other auto-immune encephalitides and may be specific for Anti-GAD-Antibody associated encephalitis. Read More

J Neurovirol 2018 12 5;24(6):773-775. Epub 2018 Sep 5.

Clinical Unit of Neurology, Department of Medicine, Surgery and Health Sciences, University Hospital and Health Services of Trieste, University of Trieste, Trieste, Italy.

Epilepsia partialis continua (EPC) is a rare entity, first described in 1894 by Koževnikov, as a variant of simple focal motor status epilepticus. EPC is most frequently characterized by motor symptoms, but as recently described, non-motor manifestations may occur, such as somatosensory symptoms or aura continua. EPC in adults has been attributed to various etiologies: infectious, vascular, neoplastic, and metabolic. Read More

Case Rep Neurol 2018 May-Aug;10(2):193-198. Epub 2018 Jul 24.

Division of Neuroradiology, Department of Radiology, Chi-Mei Medical Center, Tainan, Taiwan, ROC.

We present the case of a middle-aged man suffering from epilepsia partialis continua 3 weeks before the start of cognition decline, visual disturbance, and pyramidal dysfunction. The epilepsia partialis continua was difficult to control, and the underlying cause was uncertain even after thorough surveys for infection, inflammation, autoimmunity, and neoplasm. However, progressive signal intensity changes were noted over the involved cortical gyri, bilateral caudate, and putamen on serial magnetic resonance diffusion-weighted images, which were compatible with sporadic Creutzfeldt-Jacob disease. Read More

Epileptic Disord 2018 Aug;20(4):301-312

Department of Neurology, Christian Doppler Klinik, Paracelsus Medical University, Salzburg Austria, Centre for Cognitive Neuroscience Salzburg, Austria, Department of Public Health Technology Assessment, UMIT - University for Health Sciences, Medical Informatics and Technology, Hall.i.T., Austria.

Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. We describe a 22-year-old woman with EPC manifesting with isolated toe movements, prevalent over the left side and initially misdiagnosed as psychogenic, clinically almost indistinguishable from those observed in "painful legs and moving toes syndrome". The continuous involuntary movements with EMG correlates of twitches lasting <100 ms, the sharp waves over fronto-central regions on EEG, and the marked asymmetry in somatosensory evoked potentials with higher cortical amplitude over the right side following peripheral stimulation over the left foot confirmed the epileptic nature of the symptoms, leading to the diagnosis of EPC. Read More

Clin Neurol Neurosurg 2018 08 9;171:143-150. Epub 2018 Jun 9.

Istanbul University, Istanbul Faculty of Medicine, Department of Neurology, Turkey.

Objective: Epilepsia partialis continua (EPC) is a special form of cortical epilepsy. Several studies have described the ictal and interictal electroencephalography (EEG) findings in patients with EPC; however, lateralizing and localizing values of these findings have been evaluated rarely. This study investigated the correlation of semiologic and EEG findings, and outcomes in patients with EPC. Read More

Epileptic Disord 2018 Jun;20(3):189-194

Department of Neurology, Donostia University Hospital, San Sebastián, Spain.

Opercular myoclonic-anarthric status epilepticus (OMASE) is a rare form of epilepsia partialis continua presenting as fluctuating dysarthria, or even anarthria. The condition is caused by an epileptogenic lesion involving the opercular cortex of either hemisphere. Speech impairment is secondary to bilateral epileptic activity affecting the glossopharyngeal muscles. Read More

Neurologia 2018 Jun 8. Epub 2018 Jun 8.

Servicio de Neurología, Hospital Universitario 12 de Octubre, Madrid, España.