Temporal Lobe Encephalitis Need not Always be Herpes Simplex Encephalitis: Think of Tuberculosis.

Historically, temporal lobe encephalitis is considered as a pathognomonic feature of Herpes simplex encephalitis. This rule may not always be true and we believe that clinicians should keep their differential open. We here report once such. Case of a 36-year-old Indian male who developed altered sensorium following a prodrome of headache and fever. Examination and imaging suggested Temporal Lobe Encephalitis (TLE). Herpes encephalitis was considered and he was started on anti-virals awaiting lumbar puncture reports. Cerebrospinal fluid (CSF) analysis for Herpes Polymerase Chain Reaction (PCR) turned out to be negative. Later, to our surprise PCR for tuberculosis (TB) was positive. CSF was 100% lymphocytic and Adenosine deaminase was 12. He was started on 5 drug anti-tuberculosis regimen following which he showed a significant clinical improvement. Given the prevalence of tuberculosis in the sub-continent, clinicians must be aware of this diagnostic possibility when a patient with TLE does not respond to anti-virals. Apart from disease specific therapy, multi-disciplinary approach involving speech therapy is warranted. An early aetiological characterization of TLE has both diagnostic and prognostic implications, failing which patient may succumb.

Delayed Diagnosis of Left-Sided Diaphragmatic Hernia in an Elderly Adult with no History of Trauma.

Diaphragmatic Hernia (DH) is the herniation of abdominal contents into the thorax through a rent in the diaphragm. Acquired DH most commonly occurs following a blunt or penetrating trauma to the abdomen with former being common than the later. Very rarely DH can be spontaneous and be asymptomatic until it's very extensive. A 78-year-old presented with breathlessness and chest pain of one month duration. There was dull note to percussion and absent breath sounds in left lower zone. Auscultation revealed bowel sounds in left infra-axillary and mammary area. Electrocardiogram and laboratory data suggested acute myocardial infarction. Coronary angiogram showed a triple vessel disease. Roentgenogram was simulating pneumonic consolidation but presence of air shadows was the thing against pneumonic consolidation. CT imaging of the thorax revealed an extensive left diaphragmatic hernia with viscera and left kidney as its contents. He was initially taken up for CABG and surgery for diaphragmatic hernia was planned at a later date. This case is important for its extensive nature and for its rarity as acquired DH rarely occurs spontaneously. Chest roentgenogram must be read cautiously in all such cases to look for this entity. Auscultation for bowel sounds in the thorax is a diagnostic clue.

Relation between Vitamin D Deficiency and Severity of Chronic Obstructive Pulmonary Disease-A Case Control Study.

INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is a chronic inflammatory disorder characterized by irreversible and progressive limitation of expiratory airflow. COPD is now considered as a systemic disease with several extra-pulmonary manifestations which include osteoporosis, muscle weakness, arrhythmia, stroke, cancer etc. The role of vitamin D in skeletal integrity is well established. Role of it in several other diseases like autoimmune, infectious, respiratory and cardiovascular diseases has been increasingly recognised. Data on vitamin D status in COPD in Indian population was limited while those comparing vitamin D with stage, pack year and body mass index were lacking. AIM: To assess Vitamin D in subjects with COPD and matched controls and also to study the variability in serum vitamin D levels with COPD disease characteristics. MATERIALS AND METHODS: A total of 162 patients aged 18-65 years from south India presenting to kasturba hospital were included in the study. Serum 25- hydroxy-vitamin D were measured in 81 COPD patients (combined COPD stage A-D) and compared with 81 age and gender matched controls. Level < 20 ng/ml defined deficiency. Risk estimate with Odds ratio and association of COPD characteristics with Vitamin D was assessed using logistic regression analysis. RESULTS: COPD patients had an increased risk for vitamin D deficiency compared to controls after adjustment for age and gender(OR =2.687 (1.40,5.13)). Variables associated with lower 25(OH) D levels in COPD patients were higher pack year (p=0.001), current smoking status (p=0.026), Low BMI (p=0.02), and GOLD stage III- IV (p=0.001). CONCLUSION: COPD was associated with an increased risk of vitamin D deficiency, and there was a significant association between vitamin D levels and Combined COPD stage severity. Also, a higher pack year and a low BMI are associated with lower levels of vitamin D.

Bardet-Biedl syndrome: multiple fingers with multiple defects!

Bardet-Biedl syndrome (BBS) is a rare congenital ciliopathy characterised by rod-cone dystrophy, postaxial polydactyly, central obesity, mental retardation, hypogonadism and renal dysfunction. A 45-year-old Indian man presented with New York Heart Association class 2 dyspnoea of 3 months duration. He was blind since childhood. He was obese, cyanosed, and had clubbing and polydactyly. Systemic examination revealed presence of wide and fixed split second heart sound with systolic murmur in the left parasternal area. Work up unmasked the presence of secondary polycythaemia, atypical retinitis pigmentosa and partial atrioventricular defect. He was diagnosed to have BBS based on clinical and radiological features. This case is interesting for its rarity and also for the peculiarity of its cardiovascular association. Polydactyly with a suspicious clinical background is the clue and by itself warrants the clinician to search for occult anomalies. Clinicians must be aware of this syndrome, for which an early diagnosis and a multidisciplinary approach will significantly improve mortality and morbidity in patients.

Hypokalaemic Periodic Paralysis- A Prospective Study of the Underlying Etiologies.

BACKGROUND: Hypokalaemic periodic paralysis (HPP) is a rare muscular disorder characterised by episodic weakness associated with hypokalaemia. The disease can either be inherited or acquired and misdiagnosis of the disease is quite common. Most of the data available on the disease is from the western world. Studies reporting aetiological, clinical and metabolic profiles of Indian population are sparse. Hence we tried to provide insights of the disease among the Indian population. AIM: To study the aetiological, clinical and metabolic profile of patients diagnosed with Hypokalaemic Periodic Paralysis (HPP). MATERIALS AND METHODS: This is an observational and analytical study on HPP diagnosed patients, during September 2011 to September 2014 in Kasturba Hospital, Manipal. A total of 23 patients were studied. Detailed history, clinical evaluation and metabolic workup for secondary causes of HPP were analysed. RESULTS: Of the 23 patients, 57% had primary HPP while 43% had secondary HPP. The group of patients with primary HPP comprised of 92% males and 8% females with mean age of 28 years and the mean duration of symptoms of 18 hours. The group with secondary HPP comprised of 70% males and 30% females with mean age of 38.7 years and the mean duration of symptoms of 60 hours. The secondary causes of HPP were thyrotoxicosis (50%), infective diarrhea (20%), Crohn's disease (10%), renal tubular acidosis (RTA) Type I (10%) and Conn's syndrome (10%). CONCLUSION: In our study primary HPP was found to be more common than secondary HPP. Males were predominantly affected in both groups. HPP should be ruled out before starting therapy for Guillain-Barré Syndrome (GBS).

Hunter syndrome with its typical heart: a close mimic to rheumatic heart.

A 24-year-old man presented with New York Heart Association (NYHA) grade 3 dyspnoea. He appeared dwarf-like with coarse facial features. General examination revealed cubitus valgus, claw hand, genu valgus, hallus valgus and equinovarus deformity of the foot. Systemic examination revealed cardiomegaly, a pansystolic mitral regurgitation (MR) murmur, hepatosplenomegaly and a normal IQ. Examination suggested multisystem disease involving the dermatological, musculoskeletal, cardiac and gastrointestinal system. Echocardiography showed thickened mitral and aortic valves, and moderate MR. We considered this as a storage disorder, particularly the mucopolysaccharidosis, because of its typical cardiac involvement. Further evaluation confirmed the diagnosis of Hunter syndrome. The patient was considered for enzyme replacement therapy, following which he improved. This rare disease must be considered whenever a physician encounters a young patient with multisystem involvement. In view of the availability of disease-specific therapy, an early diagnosis and prompt treatment with a multidisciplinary approach can improve the quality of life of these patients.