Once myasthenic, always myasthenic? observations on the behavior and prognosis of myasthenia gravis in a cohort of 100 patients.

BACKGROUND: The natural history of myasthenia gravis [MG] is unpredictable: In the first few years the disease course is worst with subsequent gradual disease stabilization. However, some patients tend to have continued disease activity or resurgence of the disease many years into the illness. The factors correlating with disease course need further evaluation. AIMS: To study the patterns of remissions, worsening and exacerbations in patients with MG and correlate various factors affecting them. SETTINGS AND DESIGN: Retrospective, Institute Review Board (IRB) approved study in tertiary referral neurology center. MATERIALS AND METHODS: Hundred patients with acquired MG confirming the inclusion criteria were studied. Pharmacological remissions, complete stable remissions, exacerbations, worsening episodes were analyzed with respect to age of onset, disease extent, disease severity at onset and worst of illness, acetyl choline receptor antibody positivity, thymectomy status, period of disease, pharmacotherapy and crisis episodes. RESULTS AND CONCLUSIONS: In this cohort the percentage of new remission rates per year steadily declined after the first year. Ocular myasthenia had lesser clinical worsening episodes and high chance of complete stable remission. Generalized disease had less chance drug free remission. The risk of episodes of worsening persisted at a steady rate over a period of time, being maximum in the first year. The risk of exacerbations was unpredictable and could occur after prolonged clinical quiescence, often was related to reduction of immunosuppression. The disease course did not differ significantly in the juvenile and adult age-groups. There was a strong case for permanent immunomodulation in MG.

Sensory Neuronopathies: Clinical Presentation, Management, and Outcome.

BACKGROUND AND OBJECTIVES: Sensory neuronopathies (SNNs) are a rare group of pure sensory disorders causing asymmetrical, multifocal pattern of sensory loss with distinct clinical and electrophysiological features. We aimed to study the clinical features and etiology and share the experience of treating SNN from our center. METHODS: A prospective observational study was conducted over 3 years. Patients with predominant sensory complaints and electrophysiological evidence of neuropathy were evaluated. Possible/probable SNN was diagnosed using Camdessanch´e criteria. Detailed workup was done to determine the etiology and treatment given accordingly. Follow-up was done and response to treatment was assessed using modified Rankin Scale grading at 12 months. RESULTS: Fourteen patients with SNN were studied. Two (14.3%) patients were diagnosed with Sjogren's syndrome (SS-SNN), two (14.3%) with paraneoplastic syndrome, one (7.1%) with leprous ganglionitis, and nine (64.3%) were idiopathic sensory neuronopathy (I-SNN) cases. Improvement occurred in nine (64.3%), stability in three (21.4%), and worsening in two (14.3%) patients. Out of 11 SS-SNN and I-SNN patients, eight showed improvement on follow-up, seven with injection rituximab (RTX) and one with azathioprine. We found positive correlation between RTX treatment and improvement on follow-up (P = 0.0256). Six (66.66%) out of nine I-SNN patients had early initiation of immunotherapy, of which all improved. There was positive correlation between early treatment initiation time in I-SNN patients and improvement (P = 0.0119). CONCLUSIONS: Promising results were noted in SS-SNN and I-SNN patients with intensive treatment approach using RTX. Hit hard and early treatment approach is crucial for achieving improvement in sensory neuronopathies.

Are syncopes in sitting and supine positions different? Body positions and syncope: a study of 111 patients.

CONTEXT: Syncope is a common cause of transient loss of consciousness. In the analysis of patients having syncope, body position has not been systematically studied and correlated with triggers, prodromal symptoms and circumstances. This correlation is important in differentiating syncope from its mimics. AIMS: To study syncope with respect to body positions, triggers, prodromal symptoms and circumstances. SETTINGS AND DESIGN: Prospective study set in Neurology Department of Tertiary Care Center. MATERIALS AND METHODS: Patients fulfilling guidelines set by The Task Force for the Diagnosis and Management of Syncope of the European Society of Cardiology (ESC) were recruited. Detailed clinical history, examination and investigations (ECG, 2D-ECHO, Head Up Tilt Test, Holter monitor, EEG, MRI Brain) were carried out. RESULTS: Out of the 111 recruited patients, 67 developed syncope in standing, 16 in sitting, 23 in both standing and sitting, 1 in both sitting and supine and 4 in all three positions. Prodromal symptoms were present in 81% while triggers in 42% and circumstances in 41% of patients. Black out, sweating, dizziness and headache were most common prodromal symptoms. Intense pain, smell and fear were most common triggers while prolonged standing, hot crowded room and fasting were most common circumstances associated with syncope. CONCLUSIONS: Against common belief, syncope can occur in sitting as well as in supine position. Emotional triggers were commoner in patients with syncope in supine and sitting positions while prodromal symptoms and circumstances were similar for all positions. Syncope should be considered in body positions other than standing.

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

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.