Post-infectious Non-paraneoplastic Autoimmune Retinopathy in Paediatric age group: A high index of suspicion, Multimodal Imaging and Early treatment can restore vision.

PURPOSE: To report a case of non-paraneoplastic Auto-immune retinopathy (npAIR) in a paediatric patient who showed excellent visual recovery with early diagnosis and prompt treatment. METHODS: Retrospective Case report. RESULTS: A five year old girl presented to us with bilateral profound vision loss of sub-acute onset following an episode of high fever, without any previous visual abnormality. A diagnosis of npAIR was made based on history, clinical findings and multimodal imaging. Intravenous methylprednisolone was started urgently followed by oral steroid. Visual acuity showed good improvement along with gradual restoration of anatomy of retinal layers in Optical Coherence Tomography (OCT) over a period of three months. CONCLUSION: Our case highlights the importance of suspecting npAIR in paediatric patients presenting with sudden bilateral painless progressive loss of vision without prior visual difficulties and the role of multimodal imaging to aid in diagnosis. The recovery of vision with restoration of photoreceptor layer also shows the nature of the disease to recover with early intervention despite a negative anti-retinal antibody test but with features highly suggestive of npAIR.

Crohn's Disease Presenting as Metabolic Myopathy: A Case Report.

Myopathies associated with systemic diseases results from several different disease processes. Myopathy as the initial presenting symptom in Crohn's disease is a rare presentation. We report a 20-yearr-old lady who presented with a painful proximal myopathy. On examination, she was malnourished with pallor, angular cheilitis, Bitots spots, and bilateral pitting pedal edema. Laboratory evaluation showed iron deficiency anemia, hypoalbuminemia, and very low vitamin D levels with elevated creatine phosphokinase levels. A possibility of osteomalacic metabolic myopathy due to vitamin D deficiency was considered. The malabsorption workup was negative. A colonoscopic biopsy showed noncaseating granulomatous inflammation suggestive of Crohn's disease. With supplementary therapy and specific treatment, she was asymptomatic at 6-months follow-up with no residual neurological deficits. A detailed history and an algorithmic approach will be very useful in making the differential diagnosis in any patient presenting with muscle weakness in myopathy associated with systemic illness.

Seronegative limbic encephalitis in association with Sjögren's syndrome: a rare case report.

Limbic encephalitis is often due to an autoimmune or paraneoplastic disease and is always a diagnostic challenge. We report a 31-year-old lady who presented with fever and proximal weakness to start with and afterwards developed refractory focal onset seizures and worsening cognition despite optimum treatment. Evaluation revealed hypokalemia with a normal anion gap metabolic acidosis. Magnetic resonance imaging (MRI) brain showed features of limbic encephalitis. Cerebrospinal fluid (CSF) showed lymphocytosis and CSF autoimmune, paraneoplastic and viral encephalitis panel were negative. However a blood ANA profile clinched the diagnosis when SS-A and Ro 52 were strongly positive. She was given steroids and subsequently plasma exchange. A labial gland biopsy confirmed the diagnosis of Sjögren syndrome. In cases of autoimmune limbic encephalitis with no identifiable cause, serological screening for rheumatological disorders is recommended. Sjögren syndrome is a rare aetiology for autoimmune limbic encephalitis. A detailed history and a step wise approach is always the key to the right diagnosis.

Comparison of thrombolytic agents in treatment of patients with acute ischemic stroke; findings from a single centre follow up study in real-life settings.

BACKGROUND AND OBJECTIVES: Health outcome data of thrombolysis in patients with acute ischemic stroke in real life-settings in India are scarce. We studied the clinical profile, risk factors and functional outcome of patients with acute ischemic stroke (AIS) who were thrombolysed. METHODS: In a single centre retrospective study from January 2017 to June 2020, we analysed the data of adult patients with AIS presented within 4.5 h of symptom onset. We included patients if they had NIHSS score ≥4, modified Rankin score of 2 or less before the stroke onset and without evidence of haemorrhage. Modified Rankin score of two or less at the end of three months was defined as the primary efficacy outcome. The development of symptomatic intracerebral haemorrhage was considered as the primary safety outcome. We tried to analyse the primary safety and efficacy outcomes between two thrombolytic agents. RESULTS: Ninety patients (Tenecteplase = 61; Alteplase, n = 29) underwent stroke thrombolysis during the study period. The mean age was 64.3 years in Tenecteplase group and 63.2 years in Alteplase group. Twenty patients were aged more than 75 years. Hypertension was the most common comorbidity in both the groups (72% and 72.4%). Median mRS score at 3-months was 1 in Tenecteplase group and 0.5 in Alteplase group (p < 0.001), however there was no statistically significant difference between both treatment groups in terms of NIHS score at 24 h (70.4% vs 51.7%, p = 0.08), functional recovery calculated with mRS at 3-month (83.6% vs 79.3%, p = 0.62) or in terms of symptomatic ICH (9.8% and 17.2% p = 0.36). CONCLUSION: Tenecteplase appears to have similar clinical outcomes as Alteplase for stroke thrombolysis. Given the relatively low-cost and ease of administration, Tenecteplase may be better than Alteplase for management of acute ischemic stroke.

Neurological Disorders Seen During Second Wave of SARS-CoV-2 Pandemic from Two Tertiary Care Centers in Central and Southern Kerala.

Background and Objective: SARS-CoV-2 infections present with predominant respiratory symptoms. Only a few anecdotal reports of neurological involvement have come out from India so far. Adverse neurological events following immunization (AEFI) were also reported. We present the neurological symptoms seen either in association with vaccination or COVID-19 infection during the second wave. Methods: This was a retrospective study that included consecutive COVID-19 patients' admissions during the second wave of COVID-19 pandemic in two tertiary health care centres in Kerala. Neurological symptoms two weeks prior or thirty days after a positive status of antigen or RTPCR was termed as COVID-19-Associated Neurological Disorders (CAND) and those with neurological symptoms within one month of COVID-19 vaccination was termed as Post-Vaccinal Neurological Disorders (PVND). Results: During the study period, 1270 COVID-19 admissions were reported. We identified neurological symptoms in 42 patients (3.3%), of which 35 were CAND and 7 were PVND. Stroke was the most common (50%), followed by seizures and peripheral nervous system disorders (14.2% each). Encephalitis/demyelination (11.9%) and COVID-19-associated infections (9.5%) were also seen. Conclusion: During the SARS-CoV-2 pandemic, CAND and PVND have been emerging. Association of some of these may be fortuitous; however it is worth mentioning as pathogenic mechanisms of COVID-19 affecting various organ systems still remain unclear. Moreover, this may be helpful in future studies designing management options.

Hypophysitis: A Rare Cause of Impaired Consciousness in a Middle-Aged Woman.

BACKGROUND: Hypophysitis occurs predominantly in women, usually during pregnancy or postpartum period. Hypophysitis involving the anterior pituitary often presents with adrenal insufficiency. DESIGN/METHODS: We describe a 43-year-old woman who presented with recurrent episodes of alteration of sensorium due to hyponatremia. She had a 3-month history of amenorrhea and on evaluation had evidence of pan-hypopituitarism. The magnetic resonance images of the brain showed mild pituitary enlargement and thickened pituitary stalk with homogenous contrast enhancement indicating hypophysitis. She was treated with oral steroids and thyroxine supplements. She remained asymptomatic at 15-month follow-up on low-dose oral steroids and thyroxine supplements. RESULTS: Hypopituitarism due to hypophysitis resulted in recurrent episodes of hyponatremia in our patient. CONCLUSION: Our case highlights the importance of a systematic search for the etiology for hyponatremic encephalopathy.

Paradoxical Embolism in a Case of Hereditary Hemorrhagic Telangiectasia: Case Report with Literature Review.

Ischemic stroke involving multiple artery territory with normal vessels gives a clue for an embolic stroke. Paradoxical embolization is a recognized cause of ischemic stroke. We describe a 48-year-old male who presented with acute onset giddiness, mild dysarthria, right hemiparesis, and ataxia. Clinical examinations indicated left cerebellar signs with right hemiparesis. Additionally, the patient had significant clubbing and mild cyanosis. A strong family history of epistaxis and hemoptysis was noted. His Magnetic resonance imaging of the brain showed acute left cerebellar and left frontal cortical infarct with normal vessels. The routine stroke and cardiac workup were normal. Chest X-ray showed bilateral lower zone opacities. Contrast-enhanced computerized tomogram (CT) of the chest showed bilateral pulmonary arteriovenous malformation. Paradoxical embolism due to pulmonary arteriovenous malformation resulted in ischemic stroke in our patient. He fulfilled all of the four criteria for diagnosing definite hereditary hemorrhagic telangiectasia. Our case highlights the importance of a systematic search for paradoxical embolism in a cryptogenic stroke.

Mineralizing Angiopathy of Lenticulostriate Arteries with Infantile Basal Ganglia Infarct Following Minor Head Trauma: A Case Series.

Acute arterial ischemic stroke after minor head trauma has been reported in the past, mostly in infants. Most of these affected children had basal ganglia infarct on imaging. Investigations for other etiologies of stroke were noncontributory in most of the cases. Thin-slice computed tomography scan may show mineralizing angiopathy of lenticulostriate arteries. We report a clinical series of four infants who presented with the classical features of this distinct clinico-radiological entity. Clinical characteristics and risk factors at the time of stroke were described in detail. The long-term outcome on standard antiplatelet therapy is reported. None of the children had stroke recurrence during follow-up. The current literature on this clinico-radiological syndrome is reviewed in detail. In the typical cases, extensive etiological workup may not be warranted.

A pragmatic study on efficacy, tolerability and long term acceptance of ketogenic diet therapy in 74 South Indian children with pharmacoresistant epilepsy.

PURPOSE: Significant challenges exist for Ketogenic Diet (KD) programs in many populations, mainly due to the variations in local dietary preferences. Here we report a single center experience of KD therapy in a cohort of South Indian children with pharmaco-resistant epilepsies. METHODS: Children aged 0-18 years, enrolled in the KD program for pharmacoresistant epilepsies of Amrita institute of Medical Sciences, Kochi, Kerala, India (2010 - 2015) were included in this pragmatic study. Diet efficacy was evaluated according to reduction in seizure frequency and in the number of antiepileptic drugs (AED). Duration of retention, reasons for discontinuation and the rate of adverse events were used for assessing KD tolerability. RESULTS: Seventy four children were enrolled in the KD program. Four children could not complete the initiation process. Median age at KD initiation was 4.2years. 53 children had developmental delay. 89% were on 3 or more AEDs. Baseline seizure frequency was >5/day in 52 children. KD was continued for a median duration of 10.43 months. At the last contact, 59.4% reported seizure reduction of more than 50%. More than 90% reduction was noted in 25 children (33.7%). 6(8.1%) of them became completely seizure free. Four children expired during the study period and four children reported major adverse events necessitating KD withdrawal. Main reasons for discontinuation of KD were poor compliance, lack of response to diet and relapse of seizures. CONCLUSION: KD may be a safe and effective option for children with pharmacoresistant epilepsies even while on a traditional carbohydrate rich South Indian diet.