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  1. Article: The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study.

    Ojha, Pawan T / Gaikwad, Abhijeet M / Singh, Rakesh / Jagiasi, Kamlesh / Nagendra, Shashank

    Annals of Indian Academy of Neurology

    2023  Volume 26, Issue 4, Page(s) 395–400

    Abstract: Background: Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, and in over 80% of cases, antibodies are identified against the nicotinic type of acetylcholine receptor (AChR) on the muscle endplate. Despite the ... ...

    Abstract Background: Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, and in over 80% of cases, antibodies are identified against the nicotinic type of acetylcholine receptor (AChR) on the muscle endplate. Despite the availability of various treatment options, patients with MG experience relapses and remission during the course of the disease. Aims and Objective: To understand the clinical profile, predictors of outcomes in acetyl choline receptor (AChR) antibody positive generalized MG.
    Methods: This is a retrospective, single-centre, observational study of 108 patients with AChR positive generalized MG. We collected data on clinical and demographical profiles, treatments received, and treatment responses from those who fulfilled inclusion criteria over a mean follow up period of 33.75 ±7.30 months. Clinical outcomes were studied in terms of the type of remission and crisis or disease exacerbations patients had, considering different variables and treatment received.
    Results: We found the commonest initial symptoms were ocular or oculo-bulbar, which progressed to generalized MG in the first year of disease onset. 36 (33.3%) patients experienced a crisis requiring mechanical ventilation within a mean period of 9.4 ±4.77 months from the disease onset. Multivariate regression analysis showed late-onset MG (age of onset between 50-70 years) and treatment with rituximab were better correlated with remission, (odd ratio of 4.7; 95 % CI ,1.12 -12.6;
    Conclusion: In this study of AChR antibody positive generalized MG patients, we found that nearly one-third of them experienced myasthenic crisis despite receiving the best medical care. Rituximab appeared to be effective in the treatment of refractory MG and those who failed thymectomy. Thymectomy was associated with better outcomes in patients, both with or without a thymoma.
    Language English
    Publishing date 2023-08-25
    Publishing country India
    Document type Journal Article
    ZDB-ID 2240174-X
    ISSN 1998-3549 ; 0972-2327
    ISSN (online) 1998-3549
    ISSN 0972-2327
    DOI 10.4103/aian.aian_186_23
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Myosin Myopathy Presenting as Chronic Progressive External Ophthalmoplegia.

    Maniyar, Aamna M H / Singh, Rakesh K / Ojha, Pawan T / Chaudhary, Gaurav S / Mahto, Anuradha P / Shah, Arjun G

    Annals of Indian Academy of Neurology

    2023  Volume 26, Issue 6, Page(s) 1024–1025

    Language English
    Publishing date 2023-11-03
    Publishing country India
    Document type Journal Article
    ZDB-ID 2240174-X
    ISSN 1998-3549 ; 0972-2327
    ISSN (online) 1998-3549
    ISSN 0972-2327
    DOI 10.4103/aian.aian_552_23
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Atypical Optic Neuritis: The Potential Red Flags.

    Ojha, Pawan T / Barvalia, Prachi P / Singh, Rakesh / Soni, Girish / Kadam, Nikhil / Nagendra, Shashank / Aglave, Vikram / Jagiasi, Kamlesh

    Neurology India

    2022  Volume 70, Issue 5, Page(s) 1982–1987

    Abstract: Background: Many potential causes of optic nerve inflammation exist, including typical and atypical causes, which require different management strategies.: Objective: The objective of this study is to identify red flags that help differentiate ... ...

    Abstract Background: Many potential causes of optic nerve inflammation exist, including typical and atypical causes, which require different management strategies.
    Objective: The objective of this study is to identify red flags that help differentiate typical from atypical optic neuritis (ON).
    Materials and methods: This prospective study included 66 patients (100 eyes) with immune-mediated ON from January 2016 to June 2019, carefully excluding the nonimmune causes. The clinico-radiological features, investigations, therapy, and outcome were analyzed.
    Results: We evaluated 33 cases each of typical and atypical ON. The typical group included 29 idiopathic ON and four associated with multiple sclerosis. Atypical ON included 19 neuromyelitis optica (NMO), seven MOG-associated ON (MOG-ON), and others due to Sjogren's syndrome, granulomatous polyangiitis, sarcoidosis, and IgG4 disease. Atypical ON occurred significantly and more frequently with extremes of ages (<10 or >70 years), bilateral simultaneous or severe vision loss with early disc pallor, multiple attacks, symptoms/neuro-imaging indicating non-MS disease e.g., long segment ON/myelitis, large confluent lesions, the involvement of optic tract, chiasma, area postrema or diencephalon, and (pachy) meningitis. Systemic involvement and poor outcomes despite steroids and second-line immunosuppression were observed more often in the atypical ON.
    Conclusions: The red flags indicating atypical ON are onset at extremes of age, multiple attacks, bilateral simultaneous or severe to very severe vision loss, early disc pallor, neurological symptoms, or imaging abnormalities suggesting non-MS disease, systemic involvement, and poor steroid responsiveness. The awareness might help the clinician promptly identify and escalate therapy to ensure a better outcome.
    MeSH term(s) Aged ; Humans ; Autoantibodies ; Myelin-Oligodendrocyte Glycoprotein ; Neuromyelitis Optica/complications ; Optic Neuritis/diagnosis ; Pallor/complications ; Prospective Studies ; Vision Disorders
    Chemical Substances Autoantibodies ; Myelin-Oligodendrocyte Glycoprotein
    Language English
    Publishing date 2022-11-10
    Publishing country India
    Document type Journal Article
    ZDB-ID 415522-1
    ISSN 1998-4022 ; 0028-3886
    ISSN (online) 1998-4022
    ISSN 0028-3886
    DOI 10.4103/0028-3886.359189
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article: Validation of a New Graphic Facial Nerve Grading System: FAME Scale.

    Ojha, Pawan T / Nagendra, Shashank / Ansari, Afroz / Kadam, Nikhil Dhananjay / Mathur, Ajay / Gopinathan, Neeraja / Ojha, Nishu / Patel, Hardik / Bansode, Akshay / Kalel, Orpah / Morwani, Kishan P / Jagtap, Digvijay / Kumar, Sumant / Vij, Vinod

    Annals of Indian Academy of Neurology

    2022  Volume 25, Issue 3, Page(s) 464–472

    Abstract: Background and objective: Most of the existing qualitative facial nerve grading systems are very subjective while the quantitative grading systems are more complex, require longer data input time and specific software. There is a need for having a ... ...

    Abstract Background and objective: Most of the existing qualitative facial nerve grading systems are very subjective while the quantitative grading systems are more complex, require longer data input time and specific software. There is a need for having a scoring system with graphic criteria to improve the subjectivity, reliability and convenience. We aimed to develop and validate such a reliable graphic scale for use in Bell's palsy.
    Methods: Face videos of patients with unilateral facial paralysis were recorded using smartphones and analyzed for six items including five voluntary facial movements apart from complications of facial palsy (synkinesis, hyperkinesis, and contracture). 15 videos were used for pilot study, 75 for the development of scale and 110 for its validation. Each video was rated on two separate occasions by 3 independent raters, a score of 0-4 was assigned to each item using the graphic scoring criteria, and a composite score was obtained (range 0-24). Five disease severity categories: normal (score 0), mild (score 1-6), moderate (score 7-12), severe (score: 13-18) and profound facial weakness (score: 19-24).
    Results: The proposed scale and its component items had high inter-rater and intra-rater reliability (Kappa >0.7). Good correlation (Pearson co-efficient >0.7) was seen among the voluntary movements. The proposed scale is a valid tool to score motor deficits and complications of facial palsy.
    Conclusions: The proposed scale is a valid and reliable graphic scale to describe facial motor dysfunction and its secondary defects.
    Language English
    Publishing date 2022-04-06
    Publishing country India
    Document type Journal Article
    ZDB-ID 2240174-X
    ISSN 1998-3549 ; 0972-2327
    ISSN (online) 1998-3549
    ISSN 0972-2327
    DOI 10.4103/aian.aian_662_21
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Myelin Oligodendrocyte Glycoprotein (MOG) Antibody-Associated CNS Demyelination: Clinical Spectrum and Comparison with Aquaporin-4 Antibody Positive Neuromyelitis Optica Spectrum Disorder.

    Ojha, Pawan T / Aglave, Vikram B / Soni, Girish / Jagiasi, Kamlesh A / Singh, Rakesh K / Singh, Rakeshsingh Kamleshsingh / Nagendra, Shashank

    Neurology India

    2020  Volume 68, Issue 5, Page(s) 1106–1114

    Abstract: Background: The clinical phenotypes of myelin oligodendrocyte glycoprotein (MOG) antibody disease, its disease course, and treatment are poorly understood and much work needs to be done towards this.: Objective: To characterize the clinico-radiologic ...

    Abstract Background: The clinical phenotypes of myelin oligodendrocyte glycoprotein (MOG) antibody disease, its disease course, and treatment are poorly understood and much work needs to be done towards this.
    Objective: To characterize the clinico-radiologic spectrum and treatment outcomes of MOG antibody disease and differentiate it from aquaporin-4 (AQP-4) antibody positive neuromyelitis optica spectrum disorders (NMO-SD).
    Methods: A single-center, observational study from Western India during 2017-2019, of 48 patients with either MOG antibody positive (21 patients) or AQP-4 antibody positive (27 patients) central nervous system demyelination.
    Results: MOG antibody group had median age 32.2 years, no gender bias, median disease duration 40 months, relapses in 9 patients (43%), and median 2.5 (1-16) episodes per patient. Onset phenotypes included isolated bilateral optic neuritis (ON) (43%), isolated unilateral ON (19%), acute brainstem syndrome (19%), simultaneous ON with myelitis (9%), isolated myelitis (5%), and acute disseminated encephalomyelitis optic neuritis (ADEM-ON) (5%). Characteristic neuroimaging abnormalities were anterior segment longitudinally extensive ON, upper brainstem, and thoracic cord involvement (both short and long segment lesions). Most patients (86%) responded well to steroids, only 3/21 required rescue immunotherapy. In total, 6 out of 46 eyes affected developed permanent visual disability, while one patient had motor disability. The features differentiating MOG from AQP-4 antibody group were: no female predilection, preferential optic nerve involvement, characteristic neuroimaging abnormalities, and favorable therapeutic response and outcome.
    Conclusions: MOG disease commonly presents as severe ON, myelitis, acute brainstem syndrome, ADEM or their combinations. Early identification, treatment, and maintenance immunosuppression are necessary. It can easily be differentiated from NMO-SD using clinico-radiological features and therapeutic response.
    MeSH term(s) Adult ; Aquaporin 4 ; Autoantibodies ; Disabled Persons ; Humans ; India ; Motor Disorders ; Myelin-Oligodendrocyte Glycoprotein/analysis ; Neuromyelitis Optica/diagnostic imaging
    Chemical Substances Aquaporin 4 ; Autoantibodies ; Myelin-Oligodendrocyte Glycoprotein
    Language English
    Publishing date 2020-10-24
    Publishing country India
    Document type Journal Article ; Observational Study
    ZDB-ID 415522-1
    ISSN 1998-4022 ; 0028-3886
    ISSN (online) 1998-4022
    ISSN 0028-3886
    DOI 10.4103/0028-3886.294831
    Database MEDical Literature Analysis and Retrieval System OnLINE

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