Thymectomy for myasthenia gravis: A pathological analysis.

BACKGROUND: Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most commonly caused by antibodies to the acetylcholine receptors, often with underlying thymic pathology. AIMS: This study is aimed at analyzing the pathological spectrum of the excised thymuses in patients with myasthenia. MATERIALS AND METHODS: This was a retrospective 10-year study of 68 thymectomy specimens performed as a part of the treatment of patients with MG. STATISTICAL ANALYSIS: Nil. RESULTS: There were 47 males and 21 females (male to female ratio of 2.2:1) with a mean age of 41 years. Only three patients presented with ocular myasthenia. The thymus was normal in 9 patients (13.2%) and atrophic in 17 patients (25%). Follicular hyperplasia and thymomas were seen in 6 and 36 patients, respectively. CONCLUSION: The thymectomies performed in patients of MG had a fairly variable spectrum on histology; the thymic tumors were predominantly of the cortical phenotype.

Rare and Treatable Cause of Early-Onset Refractory Absence Seizures.

Glut-1 transporter deficiency syndrome (GLUT1-DS) is a rare disorder caused by the mutation in SLC2A1 gene, which results in impaired glucose transport into the brain. It has a broad spectrum of phenotypic presentation ranging from cognitive decline, microcephaly, and refractory seizures to complex movement disorder. Recognition of this disorder is necessary as it is refractory to antiepileptic drugs (AEDs) and responds significantly to ketogenic diet. We report a case of 7-year-old girl who presented with paroxysmal eye movements in infancy with early-onset absence epilepsy (EOAE), which worsened in early morning and on fasting and was found to be refractory to four AEDs. She had mild developmental delay and subtle ataxia. Cerebrospinal fluid showed hypoglycorrhachia, and molecular analysis identified deletion in exon 4 of SLC2A1 gene (p.leu169del), thus confirming GLUT1-DS. She had a near-complete seizure control on ketogenic diet. Thus, GLUT1-DS should be suspected in all cases of refractory generalized seizures specially EOAE, especially if it worsens on fasting, is associated with development delay, positive family history, or paroxysmal movement disorder.

Double - Trouble- Relapsing Leishmaniasis in a Virologically Suppressed HIV Positive Patient.

We present a case of a middle aged male, with long standing retroviral disease on second line ART (Anti-Retroviral Therapy) with three episodes of visceral leishmaniasis diagnosed on bone marrow examination treated with a combination of liposomal amphotericin B and miltefosine.

Case Series of Pancreatitis with Uncommon Presentations.

Acute pancreatitis is an inflammatory disease characterized by local tissue injury which can trigger a systemic inflammatory response. Vascular complications of pancreatitis are a major cause of morbidity and mortality. Pulmonary embolism in acute pancreatitis has been reported to be very rare. Cardiovascular complications include shock, hypovolemia, pericardial effusion, and even nonspecific ST-T changes in the electrocardiogram (ECG) mimicking acute myocardial infarction. Acute pancreatitis complicated with acute myocardial infarction has rarely been reported and the precise mechanisms of myocardial injury remain unclear. Here we report two cases of acute pancreatitis one with acute pulmonary thrombo embolism and other with acute myocardial injury.

Isolated right ventricular cardiomyopathy with autoimmune hypothyroidism: a rare association in an adolescent.

A 13-year-old girl presented with progressive dyspnoea and palpitation, diagnosed on echocardiography as primary right ventricular cardiomyopathy with atrial fibrillation. Her thyroid profile was positive for antithyroid microsomal antibody, and antithyroid peroxidase antibodies were suggestive of autoimmune hypothyroidism. She was managed with furosemide, digoxin, acenocoumarol and thyroxine following which she showed significant improvement. This is a rare case of isolated right ventricular cardiomyopathy and its association with autoimmune hypothyroidism presenting at the age of 13.