Azathioprine-induced Veno-occlusive Hepatotoxicity in a Patient with Myasthenia Gravis.

INTRODUCTION: Myasthenia gravis (MG) is an autoimmune disorder of post-synaptic neuromuscular junction characterised by fatigable muscle weakness and is treated with prednisolone with or without other immunosuppressants, including azathioprine (AZA). Veno-occlusive hepatotoxicity of AZA is a rare complication in MG. CASE REPORT: We report a 35-year-old man with MG who was treated with pyridostigmine, prednisolone, and AZA for 5 years. He presented with abdominal pain and increased fatiguability for 7 days. His serum bilirubin and liver enzymes were elevated, and ultrasound revealed a dilated hepatic vein and portal vein suggestive of veno-occlusive liver disease. The clinical symptoms, liver functions, and ultrasound of the hepatobiliary system normalized after withdrawal of AZA. CONCLUSION: A possibility of AZA veno-occlusive hepatoxicity should be considered in an MG patient if presented with abdominal pain, elevated bilirubin and transaminases and ultrasound showing dilatation of hepatic veins. Physicians should be aware of this complication because this toxicity is reversible following dose reduction or withdrawal of AZA.

IMPETUS Stroke: Assessment of hospital infrastructure and workflow for implementation of uniform stroke care pathway in India.

BACKGROUND: India accounts for 13.3% of global disability-adjusted life years (DALYs) lost due to stroke with a relatively younger age of onset compared to the Western population. In India's public healthcare system, many stroke patients seek care at tertiary-level government-funded medical colleges where an optimal level of stroke care is expected. However, there are no studies from India that have assessed the quality of stroke care, including infrastructure, imaging facilities, or the availability of stroke care units in medical colleges. AIM: This study aimed to understand the existing protocols and management of acute stroke care across 22 medical colleges in India, as part of the baseline assessment of the ongoing IMPETUS stroke study. METHODS: A semi-structured quantitative pre-tested questionnaire, developed based on review of literature and expert discussion, was mailed to 22 participating sites of the IMPETUS stroke study. The questionnaire assessed comprehensively all components of stroke care, including human resources, emergency system, in-hospital care, and secondary prevention. A descriptive analysis of their status was undertaken. RESULTS: In the emergency services, limited stroke helpline numbers, 3/22 (14%); prenotification system, 5/22 (23%); and stroke-trained physicians were available, 6/22 (27%). One-third of hospitals did not have on-call neurologists. Although non-contrast computed tomography (NCCT) was always available, 39% of hospitals were not doing computed tomography (CT) angiography and 13/22 (59%) were not doing magnetic resonance imaging (MRI) after routine working hours. Intravenous thrombolysis was being done in 20/22 (91%) hospitals, but 36% of hospitals did not provide it free of cost. Endovascular therapy was available only in 6/22 (27%) hospitals. The study highlighted the scarcity of multidisciplinary stroke teams, 8/22 (36%), and stroke units, 7/22 (32%). Lifesaving surgeries like hematoma evacuation, 11/22 (50%), and decompressive craniectomy, 9/22 (41%), were performed in limited numbers. The availability of occupational therapists, speech therapists, and cognitive rehabilitation was minimal. CONCLUSION: This study highlighted the current status of acute stroke management in publicly funded tertiary care hospitals. Lack of prenotification, limited number of stroke-trained physicians and neurosurgeons, relatively lesser provision of free thrombolytic agents, limited stroke units, and lack of rehabilitation services are areas needing urgent attention by policymakers and creation of sustainable education models for uniform stroke care by medical professionals across the country.

Study Protocol: IMPETUS: Implementing a Uniform Stroke Care Pathway in Medical Colleges of India: IMPETUS Stroke.

Introduction: In India, a national program for stroke (national programme for the control of cardiovascular diseases, diabetes, cancer, and stroke) and stroke management guidelines exist. Its successful implementation would need an organized system of stroke care in practice. However, many challenges exist including lack of awareness, prehospital notification systems, stroke ready hospitals, infrastructural weaknesses, and rehabilitation. We present here a protocol to investigate the feasibility and fidelity of implementing a uniform stroke care pathway in medical colleges of India. Methods and Analysis: This is a multicentric, prospective, multiphase, mixed-method, quasi-experimental implementation study intended to examine the changes in a select set of stroke care-related indicators over time within the sites exposed to the same implementation strategy. We shall conduct process evaluation of the implementation process as well as evaluate the effect of the implementation strategy using the interrupted time series design. During implementation phase, education and training about standard stroke care pathway will be provided to all stakeholders of implementing sites. Patient-level outcomes in the form of modified Rankin Scale score will be collected for all consecutive patients throughout the study. Process evaluation outcomes will be collected and reported in the form of various stroke care indicators. We will report level and trend changes in various indicators during the three study phases. Discussion: Acute stroke requires timely detection, management, and secondary prevention. Implementation of the uniform stroke care pathway is a unique opportunity to promote the requirements of homogenous stroke care in medical colleges of India.

Alien-hand syndrome with mirror movements in hereditary diffuse leukoencephalopathy with spheroids.

Hereditary diffuse leukoencephalopathy with spheroids is a rare genetic disorder caused by mutations of the colony-stimulating factor 1 receptor gene. It is an adult-onset leukodystrophy, with a wide spectrum of neurological and psychiatric manifestations that includes Parkinsonism, dementia, seizures, limb weakness, spasticity and abnormal motor behaviour. Alien-hand syndrome and mirror movements are rare manifestations of this and other neurodegenerative disorders. We describe a woman with progressive limb and trunk rigidity, Parkinsonism and dementia, who also had involuntary left arm levitation (part of the posterior variant of alien-hand syndrome) and left-hand mirror movements. We discuss the different types of alien-hand syndrome, and the likely mechanisms of arm levitation and mirror movements.

Impact of COVID-19 pandemic and lockdown in a cohort of myasthenia gravis patients in India.

OBJECTIVE: Myasthenia gravis (MG) is characterized by fluctuating muscle weakness due to immune mediated damage of acetylcholine receptor (AchR). COVID-19 infection, mental stress and non-availability of drugs following lockdown may worsen myasthenic symptoms. We report the impact of COVID 19, and lockdown on the physical and mental health, and quality of sleep in a cohort of MG. METHODS: Thirty-eight MG patients were telephonically interviewed 2months after the declaration of lockdown in India. The difficulty in procuring drugs, complications, and worsening in the MG Foundation of America (MGFA) stage were noted. The patients were enquired about MG Quality of Life 15 (MGQOL15), MG Activity of Daily Living (MGADL), Hospital Anxiety and Depression Scale (HADS) and Pittsburgh Sleep Quality Index (PSQI) using a prefixed questionnaire. Their pre-COVID parameters were retrieved from our earlier trial data, which was completed 4months back. The scores of the above mentioned parameters before and after COVID were compared. RESULTS: Their median age was 45 years, and the median duration of treatment for MG was 4.5years. Eleven (28.9 %) patients were hypertensive and 3(7.9 %) diabetic. All were on prednisolone and 18(47.4 %) received azathioprine. None developed COVID, but three had other infections. Two patients needed hospitalization because of wrong medication in one and severe anxiety-insomnia in another. Following COVID19 and lockdown, MG patients had worsening in MGQOL15, MGADL, HADS and PSQI scores. Pittsburgh Sleep Quality Index score correlated with MGQOL15 and dose of acetylcholine esterase inhibitors. CONCLUSION: COVID-19 and lockdown were associated with anxiety, depression, poor MGQOL and sleep especially in severe MG patients.

Lymphocytic Hypophysitis Mimicking Pituitary Macroadenoma.

Lymphocytic hypophysitis is an inflammatory /autoimmune disorder that primarily involves the pituitary gland and the pituitary stalk. The common clinical presentations include headache, nausea, vomiting, fatigue, features of hypopituitarism and diabetes insipidus as well as diplopia, orbital pain and bitemporal hemianopia. We report a case of lymphocytic hypophysitis which presented as hemichorea. Neuroimaging showed a mass in the sella tursica region which, on histopathological examination was suggestive of lymphocytic hypophysitis. After excision of this mass, patient showed marked improvement in his symptoms albeit he developed panhypopituitarism. The patient was treated with pituitary hormonal replacement therapy and is currently asymptomatic.