Current Trends of Leprosy in a Tertiary Care Centre in North Kerala: A 10 Year Observational Retrospective Study

After the declaration of elimination of leprosy as a public health problem, leprosy services in India have been integrated with General Health-Care System, possibly resulting in readuced focus. This study was conducted to analyze the trends in the disease over a 10-year period in a tertiary care hospital in North Kerala in post elimination era. Of the 133 Hansen’s disease (HD) cases, HD-BT (borderline tuberculoid) was the most common type, followed by, HD-LL (lepromatous lepromatous). Leprosy reactions were seen in 37.59% cases, and deformities were noticed in 19 (13.53%) cases, of which 13 (66.67%) had grade 2 disability. While analyzing slit skin smear results, 25.56% were AFB (Acid Fast Bacilli) positive. On histopathological evaluation of 129 biopsied cases, 115 (89.13%) cases were consistent with clinical diagnosis. The presence of a significant number of smear-positive cases in a post elimination era, particularly lepromatous spectrum, is a matter of serious concern. Community based studies will be necessary to understand the reality at population level and for devising appropriate strategy to break the chain in transmission.

Evaluation of dried blood spots as a feasible alternative to plasma for the detection and quantification of hepatitis c virus in a tropical setting: A pilot study

Introduction: Confirmatory diagnosis of hepatitis C virus (HCV) infection (HCV RNA detection) is essential before start of the therapy. HCV RNA detection is not available in many parts of India. Shipment of plasma from distant places to referral laboratories may affect HCV RNA titres. Dried blood spots (DBS) provide an easy alternative for transporting samples to centres where HCV RNA testing is done. Aim: Evaluation of DBS as a feasible alternative to plasma for HCV diagnosis. Methods: In this cross-sectional study, 40 consecutive patients' blood samples were collected from patients referred from the Liver Clinic. Whole blood was spotted onto two Whatman 903TM cards. One card was incubated at ?37癈 and other at 4癈 for 15 days, after drying. DBS was eluted and run in Abbott RealTime HCV assay. HCV was also quantified using the Abbott ARCHITECT HCV core antigen assay for 29 of the study patients. Results were compared with normal plasma values. Results: The median log HCV RNA value (in log10IU/mL) of plasma was 5.74, with normalised DBS it was 4.92 (?37癈) and 4.66 (4癈); difference in plasma and DBS median log values was 0.82 (?37癈) and 1.08 (4癈) logs, respectively. Interclass correlation values were 0.943, P < 0.0001 (?37癈) and 0.950, P < 0.0001 (4癈), showing high agreement. The median HCV core antigen value (in fmol/L) for plasma was 325.35, whereas it was 4.77 (?37癈) and 4.64 (4癈) for DBS samples. Conclusions: DBS can be used for sampling patients from distant resource-limited settings as an alternative to plasma for HCV RNA estimation. Larger studies are required to evaluate the feasibility of DBS in the Indian subcontinent, especially for HCV core antigen estimation.

Candida spp. other than Candida albicans: a major cause of fungaemia in a tertiary care centre.

This study was conducted to determine the frequency of different Candida spp. isolated from different parts of the hospital, associated risk factors and mortality rate. A total of 59 cases were selected for prospective analysis over a period of one and half years. Blood samples collected were processed by BACTEC (9240) method. Candidaemia was diagnosed by positive blood culture at least from two blood culture samples or from a clinically significant single blood culture sample. Candida spp. were identified by standard techniques. Most frequent isolates were C. tropicalis (35.6%), C. parapsilosis (28.8%), C. glabrata (11.9%) and C. pelliculosa (11.9%). Candida albicans was isolated only in 3.4% cases. Neonatology department accounted for highest number of isolates (27.1%), followed by gastrointestinal surgery (15.3%) and cardiac surgery (13.6%). Mortality was noted in 16.9%. Probable risk factors determined were intensive care unit stay (74.6%), antibiotic therapy (50.8%), central line (42.4%), urinary catheter (32.2%), ventilator (23.7%), malignancy (20.3%) and abdominal surgery (15.3%).

Late prosthetic valve endocarditis due to Cardiobacterium hominis, an unusual complication.

We report a case of prosthetic valve endocarditis caused by Cardiobacterium hominis in a patient who had undergone atrial septal defect closure and mitral valve replacement of the heart in 1978. He presented with pyrexia of unknown origin and congestive cardiac failure. Investigations revealed infective endocarditis of prosthetic valve in mitral portion. Blood culture samples grew C. hominis. The patient was empirically started on vancomycin and gentamicin intravenously and ceftriaxone was added after isolation of the organism. Though subsequent blood cultures were negative, patient remained in congestive cardiac failure and died due to complications.

Two cases of valproate-induced hyperammonemic encephalopathy without hepatic failure.

We report two children with localization related epilepsies, who presented with somnolence, seizure exacerbation, behavioral alteration, decline in speech and cognitive abilities, and ataxia while being treated with a combination of valproate and topiramate, but had previously tolerated valproate with other antiepileptic drugs. These children had elevated serum ammonia, normal transaminase levels, and generalized slowing of EEG background activity during encephalopathy, which promptly reverted back to normal along with clinical improvement following withdrawal of valproate. To our knowledge, this is the first documentation of valproate-induced hyperammonemic encephalopathy enhanced by topiramate from India. We intend to alert internists, pediatricians, psychiatrists and neurologists about this underrecognized adverse effect of antiepileptic drug polytherapy.

Blindness, ophthalmoplegia and extensive radiculopathy: an unusual clinical syndrome in intracranial sino-venous thrombosis.

Isolated intracranial hypertension is a common manifestation of intracranial sino-venous thrombosis (ISVT). Markedly elevated intracranial tension presents with unusual features including cranial neuropathies and radiculopathy. We report two cases with ISVT, which presented with headache, papilledema, progressive visual loss, complete ophthalmoplegia and flaccid areflexic quadriparesis along with a normal sensorium. Magnetic resonance imaging (MRI) of the brain and cervical spinal cord showed no lesions that could account for the neurological deficits. Markedly elevated lumbar CSF pressure was noted in both cases. Nerve conduction study favored radiculopathy in one case and was normal in the other. Raised intracranial pressure was found to be the sole cause for the clinical manifestations. Visual impairment persisted in one patient despite lumbo-peritoneal shunting while the other died of septicemia. To our knowledge there are no previous reports of a syndrome comprising blindness, ophthalmoplegia and flaccid quadriplegia due to intracranial hypertension in ISVT.

Component therapy.

The dramatic advances that have taken place in recent years in the care of sick and premature infants also have been matched by a similar increase in the use of blood transfusion therapy. Haematological features indicate that a newborn has a blood volume of 85-125 ml/kg the foetal haemoglobin is 60-85% and average Hb in full term infant is 18 gm/dl. By 2-3 months it falls to 11-12 g/dl the main cause of anemia are iron poor diet, weaning diets recurrent or chronic infections and hemolytic episodes in malarious areas. The red cells transfusions are usually top up transfusions, exchange transfusions, partial exchange transfusions. Top up- are for investigational losses and correction of mild degrees of anemias, upto to 5-15 ml/kg. They comprise 90% of all neonatal transfusions and are used in low birth babies in special care units for a maximum of 9-10 episodes. The walk in donor programs once popular are not much in vogue. The threshold for transfusion is 8-10 g/dl Hb for upto 5 weeks. Exchange transfusions are done for correction of anemia, removal of bilirubin, removal of antibodies and replacement of red cells. Ideally plasma reduced red cells that are not older than 5 days are used. It is prepared by removal of 120 ml of standard whole blood donation. The advantage of fresh cells is that hyperkalemia is avoided and good post transfusion survival acceptable red cell oxygen affinity. However it has to be screened for sickle cell disease and G6PD deficiency. Indications for exchange transfusion are kernicterus, neonatal hemolysis, G6PD deficiency, ARDS, neonatal sepsis, DIC and neonatal isoimmune thrombocytopaenia. Complications include over transfusion, perforation of major vessels, hypocalcaemia, citrate toxicity, hypothermia, hypoglycaemia, thrombocytopenia, necrotizing enterocolitis, GVHD, bacterial, viral infections. Partial exchange transfusions are done for symptomatic anemia, where Hb<10 g/dl, it is indicated in polycythemia and hyperviscosity syndromes. Exchange volume = Blood volume x (observed Hct-Desired HCt) divided observed Hct. Points to consider-there is weak expression of ABO antigens so particular care while grouping. Transfusing volumes should be 2-5 ml/kg/hour in paediatric bags of 50-100 ml with infusion devices. Platelet transfusion are indicated in neonatal throbocytopaenia, thrombocytopaenia due to sepsis, DIC, bacterial pathogens, CMV, TORCHS, Obstetric conditions such as pre eclampsia, intrauterine death abruption placenta birth injury hypoxia schock neonatal iso immune thrombocytopaenia and maternal ITP. Administration 1 RDE/pack per 2.5 kg single dose of fresh platelets less than 24hrs which contains 55 x 10(9) cells. This also contributes fresh plasma so is useful for coagulation defects also, though there is a risk of CMV and GVHD due to leucocyte contamination. Granulocyte concentrate; Gravity leucopheresis-1:8 ratio of 60 ml of 6% HES made to stand for 1hr.

The significance of corpora amylacea in mesial temporal lobe epilepsy.

Temporal lobe epilepsy (TLE) associated with mesial temporal sclerosis (MTS), mesial TLE (MTLE), is the commonest medically refractory adult epilepsy syndrome. Corpora amylacea (CoA) have been shown to be a marker of MTS. We compared 9 patients with MTS who had dense deposition of CoA in their hippocampi with 25 patients with MTS who did not have CoA. The patients with CoA were significantly older and they showed a trend towards having a significantly longer duration of epilepsy. The postoperative seizure outcome at 2 years was not different in the 2 groups. Our results could indicate the progressive nature of the pathology of MTS, probably indicating excitotoxic damage due to recurrent seizures, but they need to be verified by clinicopathological correlation among a larger number of patients with MTLE.

Absence of GABRA1 Ala322Asp mutation in juvenile myoclonic epilepsy families from India.

An Ala322Asp mutation in the GABRA1 gene was recently reported to be responsible for causing the autosomal dominant (AD) form of juvenile myoclonic epilepsy (JME) in a French-Canadian family. To study if JME families from India exhibiting the AD mode of inheritance carry the Ala322Asp mutation, we examined 35 unrelated JME-affected individuals from such families for the Ala322Asp mutation in GABRA1. Ala322Asp mutation was not observed in any of these JME-affected individuals, suggesting that this mutation is unlikely to be a predominant mutation involved in causation of epilepsy. To evaluate the possibility of other mutation(s) in and around GABRA1 that may predispose to JME, we compared the allele frequencies at two marker loci, D5S2118 and D5S422, flanking GABRA1, in probands and 100 matched population controls. One of the allele frequencies at D5S422 shows a significant difference between the cases and controls (chi-square = 11.44, d.f. = 1, P = 0.0007), suggesting genetic association between JME and genes located in the proximity of the DNA marker.

Idiopathic hypertrophic cranial pachymeningitis.

Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. The diagnosis of idiopathic hypertrophic cranial pachymeningitis was based on neuroimaging findings of thickened enhancing dura, exclusion of known causes and histopathologic findings compatible with nonspecific inflammation in the meningeal biopsies. Corticosteroid therapy was effective in all cases in inducing a complete or partial remission of the neurologic symptoms and signs. We describe the clinical, radiological and pathological features of idiopathic hypertrophic cranial pachymeningitis and discuss the relationship of this entity with other inflammatory fibrosclerotic disorders to explain the pathogenesis. A high index of suspicion, prompt confirmation of the diagnosis by meningeal biopsy, and early institution and long-term maintenance of steroid therapy may help to prevent irreversible neurologic sequelae, especially blindness.

Selection of ideal candidates for epilepsy surgery in developing countries.

In order to become cost-effective, epilepsy surgery centers in developing countries will have to achieve excellent results by selecting candidates destined to have a seizure-free outcome using locally available limited technology and expertise, without compromising on patient safety. Our experience illustrates that this goal can be accomplished by selecting patients, whose epileptogenic zone can be unquestionably established, based on history, magnetic resonance imaging, and interictal and ictal scalp electroencephalogram findings. Patients with mesial temporal lobe epilepsy, and those with circumscribed potentially epileptogenic lesions belong to this category. A stepwise approach by reserving more difficult to treat patients to later date as experience develops, or by referring them to a better-equipped center, will help each center to understand its capabilities and limitations and to move forward. It would be essential to work with and educate the local public and professionals, if the epilepsy surgery program in a developing region were to have a lasting impact, It is encouraging to note that, despite major challenges, in the last one-decade, several epilepsy centers in the developing world have not only successfully implemented epilepsy surgery programs, but also have produced results comparable to that from developed countries at a fractional cost.

Selective intra arterial thrombolysis in acute carotid territory stroke.

The safety and efficacy of selective intraarterial administration of urokinase in five male patients, (age range 30 to 65 years, mean 41.2 years), with occlusive stroke involving the carotid territory and a normal cranial computed tomography scan was evaluated. The time elapsed before treatment ranged from one to 10 hours. Digital subtraction angiography disclosed distal internal carotid artery occlusion in one patient and occlusion of the middle cerebral artery or its branches in the others. The urokinase dose ranged from 120,000 to 500,000 units. In two patients who received thrombolytic treatment within three hours of the onset of symptoms, there was a 100% recanalisation associated with excellent neurological recovery. In the remaining three patients, recanalisation rate varied from 0 to 50% with partial recovery in two and no recovery in one patient. None had a haemorrhagic transformation of the infarct. Although no firm conclusions can be drawn because of the small number of patients studied, selective intraarterial urokinase therapy appears to be safe and useful in patients with carotid territory stroke if undertaken early. Only through a multicenter, randomized, controlled trial, enough number of patients can be recruited to verify these observations.

Pregnancy in women with epilepsy : preliminary results of Kerala registry of epilepsy and pregnancy.

Eighty-five women with epilepsy were followed up for reproductive functions under the registry of epilepsy and pregnancy. 32 of them had completed the pregnancy. Their mean age was 26 years and mean seizure frequency was 0.7 during current pregnancy. Nineteen of them (59.4%) had generalized epilepsy. Nine of them were not on any anti epileptic drugs (AED), 23 women were on various AEDs, 19 being on monotherapy. Only 40% of the women were taking folic acid during pregnancy. Pregnancy ended as spontaneous abortion in one patient. Nearly one third required cesarean section. Majority (87.5%) had term babies. Three (10.7%) babies had birth asphyxia. Six babies (21.4%) had low birth weight. Congenital malformations were detected in four cases (12.5%). Malformations included neural tube defects, talipes equinovarus and other minor anomalies. These babies were exposed to sodium valproate, carbamazepine or phenobarbitone. The risk of malformation was significantly greater (p<0.05) when the mother had generalized epilepsy. The odds ratio for risk of malformation was much higher with sodium valproate (6) than that with carbamazepine (1.2) or phenobarbitone (0.8). Majority of women with epilepsy had safe pregnancy and childbirth without any aggravation of epilepsy.

Brainstem abscess complicating tetralogy of Fallot successfully treated with antibiotics alone.

Medically treated brainstem abscess in a 11 year old boy with tetralogy of Fallot is reported. There was a complete resolution of the lesion without any neurologic sequelae during parenteral antibiotic therapy with crystalline penicillin, chloramphenicol and metronidazole. The pathogenesis and management of cardiogenic brain abscesses in general and brainstem abscess in particular has been reviewed.

Pathology of temporal lobe epilepsy: An analysis of 100 consecutive surgical specimens from patients with medically refractory epilepsy.

The neuropathological features of temporal lobe epilepsy were studied utilising 100 consecutive surgical specimens from patients with medically refractory complex partial seizures. A wide spectrum of neuropathological changes was recorded in 98 specimens. Fifty-eight specimens showed features of Ammon's horn sclerosis. Diffuse accumulation of corpora amylacea were demonstrated in the resected temporal lobes from 54 patients. Six patients had neoplastic lesions of temporal lobe. One unique case of dysembryoplastic neuroepithelial tumour showed a melanotic component within the tumour. The neuropathological features were regarded as nonspecific in 31% of cases. Our results indicate that a majority of patients with medically intractable epilepsy of temporal lobe origin reveal significant neuropathological features. Careful documentation of the neuropathological features and its correlation with radiological, electrophysiological and pre- and post-surgical clinical features will help in predicting the seizure outcome after temporal lobectomy for medically refractory epilepsy.

A high rate of caesarean sections in an affluent section of Chennai: is it cause for concern?

BACKGROUND: While rising Caesarean section rates have been the subject of much attention and debate worldwide, there is not much information available on this rate and its potential adverse impact in India. METHODS: Our survey was a standard Expanded Programme on Immunization 30-cluster design, carried out in an urban educated, middle/upper class population in Chennai. Mothers of 210 children aged 12-36 months were interviewed and data collected on immunization and breast-feeding practices. Since the mode of delivery was one of the questions, we could generate population-based data on the Caesarean section rate and its influence on breast-feeding. RESULTS: Of the 210 babies, 95 (45%, 95% confidence interval: 39.1-51.3) had been delivered by Caesarean section. Two hundred and six of 210 babies (98%) had been breast-fed at some time. However, babies born by Caesarean section tended to be started late on breast-feeds were given prelacteal feeds more often, and colostrum less often when compared to babies delivered vaginally (all statistically significant). CONCLUSIONS: Our study revealed a very high rate of Caesarean section in the selected metropolitan population. On purely scientific grounds, a rate of 40% to 50% is extremely difficult to justify. Though not conclusive, the data also suggest that Caesarean section may be adversely affecting some aspects of breast-feeding. There is a need for more data and audits on Caesarean section rates in India, and a wider debate on its potential adverse impact on the health of mothers and newborns.

Mitochondrial myopathies--a clinicopathological study.

Mitochondrial myopathies are heterogeneous group of clinical disorders that can affect multiple systems besides skeletal muscles. The mitochondrial abnormalities in the skeletal muscles are morphologically identified by the presence of characteristic Ragged-red fibers (RRF) in the cryostat sections of the muscle stained with modified Gomori's trichrome stain. In this retrospective study, clinical and histopathological features in six patients with mitochondrial myopathies have been analysed. The utility of histochemical methods in confirming the diagnosis of mitochondrial myopathy has been emphasised.