Motor band sign in a Huntington disease phenocopy.

We report a 68-year-old lady who presented with Huntington phenocopy with generalized chorea and was genetically proven to have Spinocerebellar ataxia (SCA)17. MRI Brain demonstrated motor band sign, which is most commonly reported in motor neuron disease. This is the first case of motor band sign with SCA 17 and highlights the widening spectrum of radiological signs in SCA 17.

Primary CNS vasculitis (PCNSV): a cohort study.

Primary CNS Vasculitis (PCNSV) is a rare inflammatory disorder affecting the blood vessels of the central nervous system. Patients present with a combination of headaches, seizures, and focal neurological deficits. There is usually a diagnostic delay.

Evaluation of the Impact of Integrated Care and Self-Management After Deep Brain Stimulation in Parkinson's Disease.

Background: Parkinson's disease (PD) is a progressive neurodegenerative disorder with a myriad of motor and non-motor symptoms. Although deep brain stimulation (DBS) has a dramatic impact in the lives of people with PD, care delivery remains complex. There is a lack of evidence on the implementation and role of integrated care and self-management support in people with PD with chronic DBS.

Spectrum of Movement Disorders of Late-Onset Niemann-Pick Disease Type C.

Niemann-Pick disease type C (NPC), is a rare lysosomal storage disorder, which has a variable presentation based on the age of onset. We describe five adult/adolescent-onset NPC cases presenting with a range of movement disorders along with vertical supranuclear gaze palsy as part of the clinical presentation. A diagnostic delay of 4-17 years from the symptom onset was found in this case series.

Do Imaging Markers of Cerebral Small Vessel Disease Predict Hematoma Volume and Outcome in Acute Intracerebral Hemorrhage?

Background And Purpose: Cerebral small vessel disease (CSVD) markers have not been widely studied in relation to hematoma volume and growth in hypertensive intracerebral hemorrhage (ICH). The objectives to assess the relationship of white matter hyperintense lesions (WMHL), microbleeds (MBs), and cortical siderosis (CSS) with hematoma volume, hematoma expansion (HE), and 3 months outcome in patients with hypertensive ICH.

Methods: All consecutive acute hypertensive supratentorial ICH presenting to the emergency were prospectively recruited.

Clinical Features, Gender Differences, Disease Course, and Outcome in Neuromyelitis Optica Spectrum Disorder.

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an astrocytopathy with a predilection for the optic nerve, spinal cord, and brainstem. In this ambispective study, we evaluate clinical characteristics, responses to therapy, and disability outcomes in patients with NMOSD.

Methods: Patients diagnosed as NMOSD and following up for at least 1 year at a tertiary care center in India were recruited.

Clinical and laboratory evidence of Lyme disease in North India, 2016-2019.

Background: Lyme disease is endemic to parts of the Americas, Europe and Asia. However, only a handful of sporadic cases have been reported from India. In this study, we systematically evaluated the clinical and epidemiological features of Lyme disease in North India.

High-Pressure Normocephalus-Raised Intracranial Pressure with False Localizing Signs.

Normal-sized ventricles and absence of papilledema do not rule out shunt failure and raised intracranial pressure (ICP). Raised ICP can present with false localizing signs which may be cranial nerve palsies or extensive polyradiculopathy. Our patient with a history of ventriculoperitoneal (VP) shunt presented with rapidly progressive vision loss without papilledema, as well as multiple cranial nerve palsies and radiculopathy.

Therapeutic Update on Huntington's Disease: Symptomatic Treatments and Emerging Disease-Modifying Therapies.

Huntington's disease (HD) is a monogenic neurodegenerative disorder that presents with progressive motor, behavior, and cognitive symptoms leading to early disability and mortality. HD is caused by an expanded CAG repeats in exon 1 of the huntingtin (HTT) gene. The corresponding genetic test allows a clinical, definite diagnosis in life and the identification of a fully penetrant mutation carrier in a premanifest stage.

Effect of yoga as add-on therapy in migraine (CONTAIN): A randomized clinical trial.

Objective: To evaluate the effectiveness of yoga as an adjuvant to conventional medical management on clinical outcomes in patients with migraine.

Methods: CONTAIN was a prospective, randomized, open-label superiority trial with blinded endpoint assessment carried out at a single tertiary care academic hospital in New Delhi, India. Patients enrolled were aged 18-50 years with a diagnosis of episodic migraine and were randomized into medical and yoga groups (1:1).

Anti-myelin oligodendrocyte glycoprotein antibody associated disease spectrum - A north Indian tertiary care centre experience and review of literature.

Introduction: MOG antibody associated disease is a relatively new disorder for which the full clinical spectrum is being described and the literature is evolving. The current study outlines the observations on a cohort of patients diagnosed with this clinical entity.

Methods: This is a retrospective review of prospectively followed up patients with MOG antibody positive neurological illness.

Validation of ICH and ICH-GS Scores in an Indian Cohort: Impact of Medical and Surgical Management.

Objective: Prognostic scores help in predicting mortality and functional outcome post intracerebral hemorrhage (ICH). We aimed to validate the ICH and ICH-GS scores in a cohort of Indian patients with ICH and observe the impact of any surgical intervention on prognostication.

Methods: This was an ambispective observational study of primary ICH cases enrolled between January 2014 and April 2018.

Progress in Pharmacological and Surgical Management of Tourette Syndrome and Other Chronic Tic Disorders.

Background: Tourette syndrome (TS) and other chronic tic disorders are clinically heterogenous and cause physical discomfort, social difficulties, and emotional distress. In addition to tics, TS patients have a variety of behavioral comorbidities, including obsessive-compulsive disorders and attention-deficit hyperactivity disorders. TS treatment is multidisciplinary, involving behavioral therapy, oral medications, and botulinum toxin injections.

Leptomeningeal leukaemia misdiagnosed as tubercular meningitis.

Chronic meningitis is a common syndrome with multiple aetiological causes. It can be associated with visionproblems as well as multifocal involvement of the central nervous system. Often it presents with constitutional symptoms as well.

Quality of Life and Its Determinants in Adult Drug Refractory Epilepsy Patients Who Were Not Candidates for Epilepsy Surgery: A Correlational Study.

Background And Purpose: This study was performed to elucidate quality of life (QOL) and its determinants in adult drug refractory epilepsy (DRE) patients who were not candidates for epilepsy surgery.

Methods: A correlational study was performed at the center of excellence, epilepsy between July 2014 to June 2016. All consecutive DRE patients who were not candidates for epilepsy surgery were enrolled.

Anticancer Drugs for Parkinson's Disease: Is It a Ray of Hope or Only Hype?

Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by the death of dopaminergic (DA) neurons in the substantia nigra. To develop therapeutic strategies to halt or slow the neurodegenerative process, it is imperative that we understand the pathogenesis of PD. With the current state of knowledge, multiple pathological pathways such as oxidative stress, inflammation due to microglial activation, apoptotic pathway activation via Abelson (c-Abl)tyrosine kinase enzyme, and DA toxins have been incriminated in causing DA neuronal death in PD.

Langerhans cell histiocytosis presenting as a rapidly evolving frontotemporal syndrome.

Langerhans cell histiocytosis (LCH) is a rare disorder in adults which usually manifests with involvement of multiple organ systems, including the central nervous system. We describe an unusual case of biopsy-proven LCH presenting with frontotemporal-dominant cognitive impairment with hypothalamic involvement, along with multisystem disease. We propose that the dementia was probably an immune-mediated process triggered by LCH which responded dramatically to high-dose steroids.

Proportion and spectrum of movement disorders in adolescent and adult patients of autoimmune encephalitis of non-neoplastic aetiology.

We aimed to study the proportion of patients with movement disorders in seropositive autoimmune encephalitis of non-neoplastic aetiology and also to describe the spectrum of movement disorders in them. We prospectively screened 362 patients of age >12 years with encephalitis of unknown aetiology for a panel of antibodies for autoimmune encephalitis. Demographic and clinical characteristics with focus on the movement disorders were recorded.