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JNMA J Nepal Med Assoc 2022 Jun 1;60(250):551-554. Epub 2022 Jun 1.

Department of Neurosurgery, Nepal Medical College and Teaching Hospital, Attarkhel, Kathmandu, Nepal.

Cerebral hemangiopericytomas are very rare mesenchymal tumours arising from pericytes surrounding the blood vessels in the brain. Most patients present with headaches, focal neurological findings and focal seizures with or without generalisation. Our patient chiefly complained of an uncontrollable movement of her right hand that was initially fleeting but later became continuous. Read More

Neurologia (Engl Ed) 2022 May 6;37(4):263-270. Epub 2021 Apr 6.

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

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 analyse the relationship between EPC duration and outcomes. Read More

Epilepsy Behav Rep 2022 9;19:100540. Epub 2022 Apr 9.

Department of Radiology, Star Imaging Centre and Research Centre, Pune, India.

Rasmussen's encephalitis (RE) is a rare chronic inflammatory disease of the brain resulting in unilateral hemispheric atrophy with drug-resistant focal epilepsy associated with a variable degree of progressive hemiparesis and cognitive decline. The precise etiology of RE is unknown but presumed to have a neuroinflammatory pathobiological basis. Only surgery halts progression of the disease, but may occur at the expense of a fixed but otherwise inevitable neurological deficit. Read More

BMJ Case Rep 2022 Apr 29;15(4). Epub 2022 Apr 29.

Pediatric Critical Care, University of Illinois College of Medicine at Peoria (UICOMP), Peoria, Illinois, USA.

Status epilepticus (SE) is a life-threatening medical emergency which is frequently encountered in the critical care setting and can be refractory to treatment. Refractory status epilepticus (RSE) is defined as SE that has failed to respond to adequately used first-line and second-line antiepileptic medications. Super refractory status epilepticus is defined as SE that persists for 24 hours or more after the use of an anaesthetic agent or recurs after its withdrawal. Read More

Cureus 2022 Feb 19;14(2):e22394. Epub 2022 Feb 19.

Department of Neurosciences, School of Medical Sciences, Hospital Universiti Sains Malaysia, Kota Bharu, MYS.

Cerebral venous sinus thrombosis (CVST) is a rare type of cerebrovascular disease that affects mainly young to middle-aged adults. The main clinical presentation of CVST includes progressive headache, focal neurological deficit, disturbance of conscious level, and epileptic seizures, which can occur early or late in the disease process. Generalized seizure has been recognised as the most common seizure subtype among patients with CVST and epileptic seizures. Read More

World Neurosurg 2022 Jun 17;162:e517-e525. Epub 2022 Mar 17.

Department of Neurological Surgery, Cleveland Clinic, Cleveland, Ohio, USA; The Charles Shor Center for Epilepsy, Cleveland Clinic, Cleveland, Ohio, USA. Electronic address:

Objective: The objectives of this study were to determine the relationship between the severity of pathology and seizure outcomes in patients who underwent hemispherectomy for Rasmussen encephalitis (RE) and to investigate which clinical factors correlated with severity of pathology.

Methods: In this retrospective cohort study, we collected and reviewed pathology and clinical variables. We ascertained seizure outcomes using Engel's classification, and Pardo stages were used to grade pathology. Read More

J Int Med Res 2022 Mar;50(3):3000605221081429

Jupiter hospital, Pune, India.

The purpose of this review is to create more awareness regarding the epileptic manifestations of non-ketotic hyperglycaemia, which are not widely recognised, and to assist understanding of the pathophysiology involved. Given that type II diabetes is one of the common causes of morbidity worldwide, it is important to appreciate the various neurological manifestations of non-ketotic hyperglycaemia.Here, I present two cases and review the existing literature. Read More

Clin Neurophysiol 2022 05 25;137:59-62. Epub 2022 Feb 25.

Epilepsy Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. Electronic address:

J Mol Neurosci 2022 May 11;72(5):1125-1132. Epub 2022 Mar 11.

Pediatric Neurology Division, Children's Medical Center, Pediatrics Center of Excellence, Myelin Disorders Clinic, Tehran University of Medical Sciences, Tehran, Iran.

Introduction: Coenzyme Q10 deficiency can be due to mutations in Coenzyme Q10-biosynthesis genes (primary) or genes unrelated to biosynthesis (secondary). Primary Coenzyme Q10 deficiency-4 (COQ10D4), also known as autosomal recessive spinocerebellar ataxia-9 (SCAR9), is an autosomal recessive disorder caused by mutations in the ADCK3 gene. This disorder is characterized by several clinical manifestations such as severe infantile multisystemic illness, encephalomyopathy, isolated myopathy, cerebellar ataxia, or nephrotic syndrome. Read More

Clin Imaging 2022 May 19;85:48-54. Epub 2022 Feb 19.

Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.

Rasmussen encephalitis (RE) is a rare, chronic, idiopathic, progressive, inflammatory, neurodegenerative disease process and typically seen in pediatric cohort. Although primarily a disease affecting children, adult cases with RE have also been reported. It manifests as drug refractory epilepsia partialis continua (EPC). Read More

Brain Dev 2022 Jun 28;44(6):401-404. Epub 2022 Feb 28.

National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.

Background: Rasmussen syndrome (RS) is a rare neurological disorder characterized by unilateral chronic inflammation, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. There has been no detailed pathological evaluation or finding, including focal cortical dysplasia, for bilateral RS.

Case Report: A 13-year-old boy presented with status epilepticus with focal to bilateral tonic clonic seizure starting from the left upper limb. Read More

Clin Case Rep 2022 Feb 15;10(2):e05460. Epub 2022 Feb 15.

Neurology Ward Department of Internal Medicine Hôpital Provincial Général de Référence de Bukavu (HPGRB) Bukavu Democratic Republic of the Congo.

We report the case of an 18-year-old girl who presented with several episodes of simple partial motor seizures compatible with the diagnosis of epilepsia partialis continua. In addition to ketotic hyperglycemia, tuberculous meningoencephalitis was diagnosed based on clinical, biological, and brain imaging findings. The seizures ceased after normalization of glycemia. Read More

Epilepsy Behav Rep 2022 28;18:100511. Epub 2021 Nov 28.

University of California, San Francisco, Department of Neurosurgery, 400 Parnassus Ave 8 Floor, San Francisco, CA 94143, United States.

We present a case of a 10-month-old girl undergoing repetitive TMS (rTMS) for the treatment of drug-resistant epilepsy. A 10-month-old girl, later diagnosed with pathogenic POLG1 mutations, presented to our institution with chronic progressive EPC (epilepsia partialis continua) manifesting as a frequent, left-sided, synchronous continuous jerking of the arms and legs. The seizures were drug-resistant to multiple antiseizure medications and epilepsy surgery, responding only to continuous anesthesia. Read More

J Coll Physicians Surg Pak 2022 Jan;32(1):108-110

Department of Paediatric Medicine Unit-1, Mayo Hospital/KEMU, Lahore, Pakistan.

Rasmussen's encephalitis (RE) is a rare chronic progressive inflammatory disease of the brain that results in difficult-to-control seizures (mostly focal: epilepsia partialis continua), cognitive decline and progressive loss of neurological function including speech, motor skills with eventual paralysis of one half of the body (hemiparesis) and encephalitis. It is a disease that usually affects a single hemisphere and presents commonly at an early age. It poses a lot of challenges, both in diagnosis as well as treatment. Read More

Clin Neurol Neurosurg 2022 02 21;213:107099. Epub 2021 Dec 21.

Department of Clinical Neurological Sciences, London Health Sciences Centre, Western University, London, Ontario, Canada; Department of Pathology and Laboratory Medicine, London Health Sciences Centre, Western University, London, Ontario, Canada. Electronic address:

Epilepsia partialis continua (EPC) is a rare phenomenon in multiple sclerosis (MS). We describe a patient with relapsing-remitting MS and three episodes of EPC, with refractoriness to anti-seizure drugs but corticosteroid-responsiveness. No lesions likely attributable to her episodes of EPC were seen on 1. Read More

Neurohospitalist 2022 Jan 23;12(1):147-150. Epub 2021 Jul 23.

Department of Neuromedicine, Bangur Institute of Neurosciences, Kolkata, India.

Seizures and involuntary movements are relatively rare, but well-known neurological complications of non-ketotic hyperglycemia. While hemichorea-hemiballism secondary to diabetic striatopathy is increasingly being reported, unilateral caudate atrophy resulting from chronic vascular insufficiency/insult in a backdrop of poorly controlled diabetes mellitus is sparsely described in literature. We herein report a 75-year-old woman with poorly controlled diabetes mellitus who presented with concurrent epilepsia partialis continua involving left side of her face and hemichorea on the right side in the context of non-ketotic hyperglycemia. Read More

Epileptic Disord 2022 Feb;24(1):203-207

Saint-Luc University Hospital, Department of Neurology, Brussels, Belgium, Saint-Luc University Hospital, Centre for Refractory Epilepsy, Brussels, Belgium.

The role of neuroinflammation in epileptogenesis is extensively investigated, but short-term effects of seizures on established CNS pathologies are less studied and less predictable. We describe the case of a woman with previous recurrent episodes of focal cerebral haemorrhage of unknown cause who developed a pseudo-tumoural oedema triggered by provoked focal status epilepticus. A brain biopsy revealed that the underlying condition was primary angiitis of the CNS. Read More

Epileptic Disord 2022 Feb;24(1):176-182

Scientific Institute, IRCCS E. Medea, Bosisio Parini, Lecco, Italy.

Dynamin-1-like (DNM1L) is a gene located on chromosome 12p11.21 that encodes for dynamin-related protein (DRP1), a GTPase involved in mitochondrial and peroxisomal fusion, which plays a pivotal role in brain development. The missense variant, p. Read More

J Family Med Prim Care 2021 Sep 30;10(9):3502-3504. Epub 2021 Sep 30.

Department of Neurology, Bangur Institute of Neurosciences, Kolkata, West Bengal, India.

We report a case of a 14-year-old non-immunised girl with a prior history of measles infection presenting with afebrile seizures progressing to epilepsia partialis continua (EPC), quadriparesis and headache. Further evaluation revealed Human immunodeficiency virus (HIV) seropositivity with elevated anti-measles antibody titres in Cerebrospinal fluid (CSF). Electroencephalography showed focal epileptiform activity and Magnetic resonance imaging (MRI) of the brain revealed bilateral, asymmetrical long repetition time MRI (TR) hyperintensities involving juxtacortical white matter in both parietal lobes, left temporal and also in the left basal ganglia without any contrast enhancement or Diffusion weighted imaging (DWI) restriction. Read More

Caspian J Intern Med 2021 ;12(Suppl 2):S464-S466

Department of Neurology, School of Medicine, Babol University of Medical Sciences, Babol, Iran.

Background: Arachnoid cysts are congenital or acquired cerebrospinal fluid (CSF) filled intra arachnoidal lesions, included 1% of all infantile intracranial masses and were discovered incidentally in MRI or CT-scan. The vast majority of these lesions are generally asymptomatic but some patients with arachnoid cyst have headache, dizziness, seizure (or epilepsy), vestibular symptoms and cognitive impairment.

Case Presentation: We present a case of a 43-year-old woman who has late onset epilepsia partialis continua and had right spastic cerebral palsy due to huge arachnoid cyst. Read More

Pract Neurol 2021 Dec 9;21(6):543-545. Epub 2021 Nov 9.

Department of Neurology, UC Gardner Neuroscience Institute, Cincinnati, Ohio, USA

Epilepsia partialis continua manifests as low-frequency, rhythmic involuntary movements of a focal body part. We report a young man, HIV-positive and with syphilis, who developed right-hand epilepsia partialis continua associated with a small left-sided cortico-subcortical frontal lesion. A pen and paper test provided 'mechanographic' data on frequency, amplitude and rhythmicity of the hand movements, helping distinguish it from other causes of low-frequency repetitive hand movements. Read More

J Neurosci Rural Pract 2021 Oct 16;12(4):817-820. Epub 2021 Sep 16.

Department of Endocrinology, Command Hospital, Lucknow, Uttar Pradesh, India.

Eur Rev Med Pharmacol Sci 2021 Oct;25(20):6277-6282

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

Objective: Isolated abdominal epilepsia partialis continua (EPC) without the involvement of other body parts is rarely seen. Abdominal EPC usually occurs either as a part of hemibody EPC or as an evolution of refractory EPC after initial treatment. As the isolated abdominal EPC was rarely reported up to date, the data regarding its pathophysiology and management are limited. Read More

Neuropediatrics 2021 Oct 21. Epub 2021 Oct 21.

Center for Pediatric Neurology, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, United States.

Ann Med Surg (Lond) 2021 Aug 4;68:102606. Epub 2021 Aug 4.

Pediatric University Hospital, Division of Neurology, Damascus, Syria.

Rasmussen encephalitis (RE) is a rare disease of unknown etiology that causes severe chronic unihemispheric inflammatory disease of the central nervous system mainly in children. It leads to intractable seizures, cognitive decline and progressive neurological deficits in the affected hemisphere. We report two cases of RE, as defined by fulfillment of the 2005 Bien criteria. Read More

Neurol Sci 2021 Sep 8;42(9):3911-3913. Epub 2021 Jun 8.

Department of Neurology, Institute of Human Behaviour and Allied Sciences, Delhi, 110095, India.

Seizure 2021 07 1;89:56-58. Epub 2021 May 1.

Department of Epileptology, University Hospital Bonn, Bonn, Germany.

Ann Clin Transl Neurol 2021 06 6;8(6):1353-1361. Epub 2021 May 6.

Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.

No clear evidence-based treatment paradigm currently exists for refractory and super-refractory status epilepticus, which can result in significant mortality and morbidity. While patients are typically treated with antiepileptic drugs and anesthetics, neurosurgical neuromodulation techniques can also be considered. We present a novel case in which responsive neurostimulation was used to effectively treat a patient who had developed super-refractory status epilepticus, later consistent with epilepsia partialis continua, that was refractory to antiepileptic drugs, immunomodulatory therapies, and transcranial magnetic stimulation. Read More

Epileptic Disord 2021 Apr;23(2):385-391

Department of Pediatric Neurology, King Fahad Specialist Hospital, Dammam, Saudi Arabia.

Epilepsia partialis continua (EPC) is a form of focal motor status epilepticus, associated with multiple etiologies. Etiology-specific treatments, such as hemispherotomy for Rasmussen encephalitis, lesionectomy for focal cortical dysplasia, and metabolic correction for non-ketotic hyperglycemia, have proven to be efficacious in treating EPC, but, in general, EPC is difficult to treat and often drug-resistant, and there is little evidence to guide therapy. We report the successful treatment of EPC with perampanel in two pediatric patients. Read More

Neurocase 2021 04 14;27(2):165-168. Epub 2021 Apr 14.

Neurology Department, Massachusetts General Hospital, Harvard Medical School, Boston, USA.

Classic onset of CLN1 disease is within the first year of life with developmental arrest, epilepsy and rapid progression. In an atypical variant of CLN1 disease onset is later in the juvenile epoch. Although epilepsy in the juvenile form of CLN1 often is less severe than in typical CLN1, treatment of seizures and status epilepticus may be challenging. Read More