Otogenic Cerebellar Abscess with Polymicrobial Anaerobic Infection in a Young Female- A Rare Presentation.

Brain abscess is a serious clinical condition caused by a localized collection of pus within the brain tissue. This typically occurs as a result of an infection that originates from a nearby area, such as an ear, sinus, or dental infection, or an infection in the bloodstream. Streptococcus and Staphylococcus species are the most common organisms implicated in brain abscesses. Apart from aerobic growth, cases of mixed infections of both aerobic and anaerobic organisms are also commonly reported in the literature. Herein we report a 23-year-old immunocompetent female with chronic otitis media who presented with cerebellar abscess where the aerobic growth was sterile and anaerobic culture revealed pure growth of dual anaerobes viz… Peptostreptococcus and Bacteroides thetaiotaomicron. This case highlights the importance of prompt diagnosis and management of polymicrobial anaerobic infection in cases of brain abscess.

Physical Activity, Sedentary Behavior, Sleep and Screen Time of Healthy Under-Fives Attending Selected Immunization Clinics and Anganwadis of South Kerala, India.

OBJECTIVE: To estimate the proportion of healthy under-fives whose physical activity, sedentary behavior, sleep and screen time adhered to WHO 2019 recommendations and to identify risk factors for non-adherence. METHODS: A cross-sectional study was conducted among 480 healthy children (6 mo - 4 years) who attended 20 selected urban anganwadis or immunization clinics in South Kerala, India. Sociodemographic, anthropometric and outcome variables (duration of physical activity, sedentary behavior, sleep and screen time) were collected for all participants. RESULTS: Physical activity, sedentary behavior, sleep and screen time recommendations were adhered by 63.3%, 22.7%, 82.2% and 22.7% under-five children, respectively. Risk factors for inadequate physical activity were female sex, nuclear family, maternal education below college level, unskilled maternal occupation/housewife, unskilled paternal occupation and low monthly income. Risk factors for non-adherence to recommended sedentary behavior duration included joint family, paternal education college level/above, unskilled maternal occupation/housewife, unskilled paternal occupation and low monthly income. CONCLUSION: Under-fives should reduce sedentary behaviors and screen time and spend more time on physical activities.

Time to Attain Full Enteral Feeds Among Preterm Fetal Growth Restricted Neonates With Absent/Reversed End-Diastolic Flow.

OBJECTIVES: To determine the difference in time to attainment of full enteral feeds between fetal growth restricted (FGR) preterm neonates with and without absent/reversed end-diastolic flow (AREDF). Secondary objectives were to compare the short-term outcomes including the incidence of necrotizing enterocolitis (NEC) and feed intolerance between the two groups and to determine the factors affecting the time to attainment of full enteral feeds (FEF) among preterm FGR neonates. METHODS: A prospective cohort study was conducted among consecutive preterm FGR neonates delivered at 28-36 weeks gestation admitted in level III NICU. An umbilical artery doppler ultrasound was performed antenatally for all participants to detect AREDF. FGR neonates with AREDF were taken as the study group and those without AREDF were taken as the comparison group. Time to attain FEF was defined as time taken to establish enteral feeds of 150 ml/kg/day and tolerating it for the next 3 consecutive days. Delayed attainment of FEF was taken as ≥10 days needed to attain FEF. RESULTS: The median (IQR) time to attainment of full feeds was longer among neonates with AREDF compared to those without AREDF [12 (8, 16.5) vs 8 (5, 10) days; P < 0.001]. Neonates with AREDF had more feed intolerance [RR, 95% CI = 1.51 (1.13 - 2.02); P = 0.004], higher mortality [RR, 95% CI = 2.5 (1.02 - 6.2); P = 0.036], prolonged time to regain birth weight [15 (11.5, 19) days, P = 0.035], longer NICU stay [10 (7, 15), P < 0.001] and longer hospital stay [33 (23, 49), P < 0.001]. Also, neonates with AREDF had more hypoglycemia [RR, 95% CI=2.15 (1.2-3.7); P = 0.004], hypoxic ischemic encephalopathy [RR, 95% CI 5.05 (1.13 - 22.4); P = 0.016], hypothyroidism [RR, 95% CI= 8.08 (1.02 - 63.4), P = 0.016], cholestasis (P = 0.007), prolonged parenteral nutrition requirement [10 (7, 15) days, P < 0.001] and oxygen requirement [4.5 (2, 8) days, P < 0.001]. Multivariable logistic regression showed, AREDF [aOR 95% CI 2.91 (1.49 - 5.68), P = 0.002], lower gestational age [aOR 95% CI 0.724 (0.604 - 0.867), P < 0.001] and thrombocytopenia at birth [aOR 95% CI 2.625 (1.342 - 5.136), P = 0.005] are significant predictors of delayed attainment of full feeds among preterm FGR neonates. CONCLUSION: Preterm FGR neonates with AREDF are slower to attain FEF, have more feed intolerance, higher mortality, need longer time to regain birth weight, prolonged NICU stay and hospital stay. AREDF, lower gestation, sepsis and thrombocytopenia at birth are significant predictors of delayed full feed attainment among preterm FGR neonates. It is essential to devise strategies to reduce morbidity and mortality among this group of preterm neonates.

Primate malaria of human importance.

Nonhuman primate (NHP) malaria poses a major threat to the malaria control programs. The last two decades have witnessed a paradigm shift in our understanding of the malaria caused by species other than the traditionally known human Plasmodium species - Plasmodium falciparum, Plasmodium vivax, Plasmodium malariae, and Plasmodium ovale. The emergence of the malaria parasite of long-tailed macaque monkeys, Plasmodium knowlesi, as the fifth malaria species of humans has made the scientific community consider the risk of other zoonotic malaria, such as Plasmodium cynomolgi, Plasmodium simium, Plasmodium inui, and others, to humans. The development of knowledge about P. knowlesi as a pathogen which was earlier only known to experimentally cause malaria in humans and rarely cause natural infection, toward its acknowledgment as a significant cause of human malaria and a threat of malaria control programs has been made possible by the use of advanced molecular techniques such as polymerase chain reaction and gene sequencing. This review explores the various aspects of NHP malaria, and the association of various factors with their emergence and potential to cause human malaria which are important to understand to be able to control these emerging infections.

Scedosporium Infection in Recipients of Kidney Transplants from Deceased Near-Drowning Donor.

Scedosporium aurianticum infection developed in 2 recipients of kidney transplants in India, acquired from the same deceased near-drowning donor. Given the substantial risk for death associated with Scedosporium infection among solid-organ transplant recipients, safety protocols for organ transplantation from nearly drowned donors should be thoroughly revaluated and refined.

Profile of cardiac lesions among laboratory confirmed congenital rubella syndrome (CRS) infants: a nationwide sentinel surveillance, India, 2016-22.

Background: The phenotypical profile of cardiovascular malformations in patients with congenital rubella syndrome (CRS) is varied. We aimed to describe the profile of cardiac defects among CRS patients detected in the sentinel CRS surveillance in India during 2016-22. Methods: Sentinel sites enrolled infants with suspected CRS based on presence of cardiac defects, hearing impairment, eye signs, or maternal history of febrile rash illness. Suspected CRS cases underwent detailed systemic examination, including echocardiography and serological investigation for rubella. Cardiac defects were categorized as 'Simple' or 'Complex' as per the National Heart, Lung, and Blood Institute classification. We compared the distribution of cardiac defects among laboratory confirmed CRS cases and seronegative discarded cases. Findings: Of the 4578 suspected CRS cases enrolled by 14 sites, 558 (12.2%) were laboratory confirmed. 419 (75.1%) laboratory confirmed cases had structural heart defects (simple defects: n = 273, 65.2%, complex defects: n = 144, 34.4%), with ventricular septal defect (42.7%), atrial septal defect (39.4%), patent ductus arteriosus (36.5%), and tetralogy of Fallot as the commonest defects (4.5%). Laboratory confirmed CRS cases had higher odds of left to right shunt lesions (OR = 1.58, 95% CI: 1.15-2.17). This was mainly on account of a significant association of PDA with CRS (OR = 1.77, 95% CI: 1.42-2.21). Mortality was higher among CRS patients with complex heart defects (HR = 2.04, 95% CI: 1.26-3.30). Interpretation: Three-fourths of the laboratory confirmed CRS cases had structural heart defects. CRS patients with complex cardiac defects had higher mortality. Detecting CRS infection early and providing timely intervention for cardiovascular defects is critical for the management of CRS patients. Funding: Ministry of Health and Family Welfare, Govt of India, through Gavi, the Vaccine Alliance.

Congenital rubella syndrome surveillance in India, 2016-21: Analysis of five years surveillance data.

Background: In India, facility-based surveillance for congenital rubella syndrome (CRS) was initiated in 2016 to estimate the burden and monitor the progress made in rubella control. We analyzed the surveillance data for 2016-2021 from 14 sentinel sites to describe the epidemiology of CRS. Method: We analyzed the surveillance data to describe the distribution of suspected and laboratory confirmed CRS patients by time, place and person characteristics. We compared clinical signs of laboratory confirmed CRS and discarded case-patients to find independent predictors of CRS using logistic regression analysis and developed a risk prediction model. Results: During 2016-21, surveillance sites enrolled 3940 suspected CRS case-patients (Age 3.5 months, SD: 3.5). About one-fifth (n = 813, 20.6%) were enrolled during newborn examination. Of the suspected CRS patients, 493 (12.5%) had laboratory evidence of rubella infection. The proportion of laboratory confirmed CRS cases declined from 26% in 2017 to 8.7% in 2021. Laboratory confirmed patients had higher odds of having hearing impairment (Odds ratio [OR] = 9.5, 95% confidence interval [CI]: 5.6-16.2), cataract (OR = 7.8, 95% CI: 5.4-11.2), pigmentary retinopathy (OR = 6.7, 95 CI: 3.3-13.6), structural heart defect with hearing impairment (OR = 3.8, 95% CI: 1.2-12.2) and glaucoma (OR = 3.1, 95% CI: 1.2-8.1). Nomogram, along with a web version, was developed. Conclusions: Rubella continues to be a significant public health issue in India. The declining trend of test positivity among suspected CRS case-patients needs to be monitored through continued surveillance in these sentinel sites.

Extensive Multifocal Emphysematous Osteomyelitis of Spine: A Rare Case and a Review of Literature.

BACKGROUND: Emphysematous osteomyelitis (EO) is an extremely rare form of osteomyelitis which is complicated mainly by infection with gas-forming organisms. The common causative agents of this disease are mainly members of Enterobacteriaceae family, the most common are Escherichia coli and Klebsiella pneumoniae along with anaerobes. A total of 48 cases of EO have been reported in the literature till now globally and none have documented the isolation of Corynebacterium amycolatum. CASE PRESENTATION: We report a rare case of emphysematous osteomyelitis of the spine and pelvis due to Escherichia coli along with the isolation of Corynebacterium amycolatum from the same pus samples on two consecutive occasions in a 50-year-old female with uncontrolled diabetes mellitus, who was successively treated with antibiotics and drainage of pus. We also did a brief review of the literature of all cases reported till now. CONCLUSION: The role of Corynebacterium amycolatum in the etiology of emphysematous osteomyelitis needs to be evaluated further in future studies as we cannot completely ignore its isolation in two consecutive samples as a mere contaminant.

In-vitro Susceptibility Testing Methods for Ceftazidime-avibactam against Carbapenem-resistant Enterobacterales: Comparison with Reference Broth Microdilution Method.

INTRODUCTION: ß-lactam antibiotics, mainly cephalosporins, and carbapenems, have been the mainstay of treatment for infections caused by Enterobacterales. However, their role in treating clinical infections has become limited because of the increase in resistance. There is a need to have cost-effective and rapid methods for antimicrobial susceptibility testing methods for newer antibiotics like ceftazidime-avibactam against carbapenem-resistant Enterobacterales (CRE), which can be applied in routine clinical microbiology laboratories. With this aim, the present study was conducted to compare the disk diffusion and gradient diffusion, i.e., the E-test method with the reference broth microdilution (BMD) method for in-vitro testing of ceftazidime-avibactam against CRE. MATERIALS AND METHODS: A total of 111 CRE isolates from various clinical samples were included. Conventional PCR (Polymerase Chain Reaction) was done for the detection of genes encoding carbapenemases and to see their expression, modified carbapenem inactivation method (mCIM) along with EDTA (Ethylenediaminetetraacetic acid) carbapenem inactivation method (eCIM) was done. RESULTS: 42.3% (47/111) isolates were resistant to ceftazidime-avibactam by the standard broth microdilution method; however, 45.9% (51/111) were resistant by both disk diffusion and E-test. In 5.4% of isolates (similar in both methods), microbroth dilution method results did not match with E-strip and disk diffusion. Very major errors (VME) by both disk diffusion and E-test were found in 2.1% (1/47), and major errors (ME) were found in 7.8% (5/64) isolates (similar isolates in both methods). The overall categorical agreement (CA) rate was 94.6% for both E-test and disk diffusion, and the essential agreement (EA) rate was 90.1% (100/111) for E-test. 98% (109/111) of CRE harbored carbapenemase genes either singly (30.3%) or in combination with others (69.7%). CONCLUSION: In conclusion, for CRE, E-test and the disk diffusion method for ceftazidimeavibactam depicted an acceptable performance as an alternative to the reference broth microdilution method.

In-vitro susceptibility testing methods for the combination of ceftazidime-avibactam with aztreonam in metallobeta-lactamase producing organisms: Role of combination drugs in antibiotic resistance era.

Resistance in Gram-negative organisms has become one of the leading threats in recent years. Of the different mechanisms described in the literature, resistance due to beta-lactamases genes have been overcomed by the use of a beta-lactamase inhibitor in combination with a beta-lactam antibiotic. When this combination is insufficient to counter metallo-beta-lactamases, a third antibiotic, has been added to restore susceptibility. One such recent combination is ceftazidime-avibactam with aztreonam. In this study, 60 isolates of multidrug-resistant organisms producing metallo-beta-lactamases were included to perform in-vitro antibiotic susceptibility testing against ceftazidime-avibactam and aztreonam alone and in combination using three different methods. Individual testing revealed 100% (60/60) resistance to both ceftazidime-avibactam and aztreonam in all the isolates. The disk diffusion method showed an inhibition zone size of 21 mm in all the isolates, with 16 isolates showing an increase in inhibition zone size of >16 mm. In the E-test fixed ratio method, MICs of ceftazidime-avibactam and aztreonam when used alone ranged from 8/4 µg l-1 to ≥256/4 µg l-1 and 16 µg l-1 to 256 µg l-1, respectively, but in combination, these MICs were reduced to 0.016/4 µg l-1 to 2/4 µg l-1 with FIC < 0.5 in all the isolates. Similar results were obtained with the E-test agar dilution method with more than a 16-fold reduction in MIC in all the isolates when avibactam concentration was fixed at 4 µg l-1. All three methods showed a 100% correlation with each other. The current study depicted the usefulness of combining ceftazidime-avibactam with aztreonam against organisms producing metallo-beta-lactamases and that disk diffusion methods can be used as a method for performing in-vitro antibiotic susceptibility testing of this combination.

Relationship Between COVID-19 Lockdown and Epidemiology of Neonatal Sepsis.

BACKGROUND: We compared the hospital-based epidemiology of neonatal sepsis after the coronavirus disease 2019 lockdown (LD) versus historical epochs and the LD period versus phases of unlocking. METHODS: This retrospective cohort study was conducted in a level 3 neonatal unit. We compared neonates born in three 24-week periods-Group LD: 22 March 2020 to 5 September 2020-the reference group, Group pre-LD: 29 September 2019 to 14 March 2020 and Group temporally corresponding to LD in 2019 (corres-LD): 24 March 2019 to 7 September 2019. We also studied linear trends from LD phase 1.0 until Unlock 4.0. The key outcome was culture-positive sepsis. RESULTS: There were 1622, 2744 and 2700 subjects in groups LD, pre-LD and corres-LD, respectively. The incidence of any culture-positive sepsis in pre-LD was higher than LD [odds ratio (95% CI) = 1.61 (1.02-2.56)]. This was mainly due to a statistically significant reduction in Acinetobacter baumannii sepsis, with incidence rate differences of pre-LD versus LD [0.67 (95% CI: 0.37-0.97), P = 0.0001] and corres-LD versus LD [0.40 (95% CI: 0.16-0.64), P = 0.0024]. Groups pre-LD and corres-LD had higher proportion of multi-drug resistant (MDR)/extreme drug resistance/pan drug resistance sepsis than LD [77%, 77% and 44%, respectively (P values of both groups vs. LD = 0.01)]. From LD 1.0 to unlock 4.0, there were fewer episodes of MDR sepsis (Plinear trends = 0.047). On multivariable analysis, group pre-LD (vs. reference group LD), male sex, birth weight and Apgar score independently predicted culture-positive sepsis. CONCLUSIONS: LD favorably impacted the epidemiology of neonatal sepsis in a hospital setting, with less A. baumannii and MDR sepsis, which persisted during unlocking.

Secondary anaerobic infection in a case of multidrug resistant tubercular paraspinal abscess: A rare presentation.

INTRODUCTION: Paraspinal abscesses are most commonly caused by Staphylococcus aureus and some Gram-negative bacteria. In developing countries, Mycobacterium tuberculosis (MTB) contributes to almost 50 % of cases. Even in proven cases of tubercular paraspinal abscesses, secondary infection of aerobic or anaerobic bacteria is possible and should be carefully evaluated for proper management. CASE REPORT: A type I diabetes mellitus patient presented with chronic backache and lower limb weakness and radiological investigations showed paraspinal collections suggestive of tuberculosis. The patient was then started on anti-tubercular drugs, she initially responded and then showed gradual deterioration in the form of increased pain, fever and pus discharge. Aerobic cultures of pus were sterile and anaerobic culture grew Peptoniphilus asaccharolyticus sensitive to metronidazole. Appropriate treatment had resulted in clinical improvement. CONCLUSION: Suspicion about co-infection with aerobic or anaerobic bacteria should be high even in proven cases of tubercular paraspinal abscess not improving despite proper anti-tubercular therapy.

Bacterial coinfections and secondary infections in COVID-19 patients from a tertiary care hospital of northern India: Time to adhere to culture-based practices.

OBJECTIVE: Bacterial co-pathogens are common in various viral respiratory tract infections, leading to increased disease severity and mortality. Still, they are understudied during large outbreaks and pandemics. This study was conducted to highlight the overall burden of these infections in COVID-19 patients admitted to our tertiary care hospital, along with their antibiotic susceptibility patterns. MATERIAL AND METHODS: During the six-month study period, clinical samples (blood samples, respiratory samples, and sterile body fluids, including cerebrospinal fluid [CSF]) of COVID-19 patients with suspected bacterial coinfections (at presentation) or secondary infections (after 48 hours of hospitalization) were received and processed for the same. RESULTS: Clinical samples of 814 COVID-19 patients were received for bacterial culture and susceptibility. Out of the total patient sample, 75% had already received empirical antibiotics before the samples were sent for analysis. Overall, 17.9% of cultures were positive for bacterial infections. Out of the total patients with bacterial infection, 74% (108/146) of patients had secondary bacterial infections (after 48 hours of hospitalization) and 26% (38/146) had bacterial coinfections (at the time of admission). Out of the 143 total isolates obtained, the majority (86%) were gram-negative organisms, of which Acinetobacter species was the commonest organism (35.6%), followed by Klebsiella pneumoniae (18.1%). The majority (50.7%) of the pathogenic organisms reported were multidrug resistant. CONCLUSION: The overall rate of secondary bacterial infections (SBIs) in our study was lower (7.9%) than reported by other studies. A rational approach would be to adhere to the practice of initiating culture-based guidance for antibiotics and to restrict unnecessary empirical antimicrobial therapy.

WHO 2009 Warning Signs as Predictors of Time Taken for Progression to Severe Dengue in Children.

OBJECTIVES: To identify WHO 2009 warning signs that can predict time taken for progression to severe dengue in a pediatric population. DESIGN: Prospective analytical study over 1 year and 2 months. SETTING: Tertiary care center. PARTICIPANTS: 350 children aged 1 mo-12 y with serologically confirmed dengue without co-morbidities/co-infections; conse-cutive sampling. PROCEDURE: At admission, clinical and laboratory details were noted. Disease progression, time of onset of each warning sign, hematocrit, and platelet counts were recorded daily till discharge/ death. If progressing to severe dengue, its time of onset was noted. Time to event analysis with Log Rank test, Kaplan Meier plots and Cox Proportional Hazards Model was done. OUTCOME MEASURES: Primary outcome was time interval from onset of first warning sign to onset of severe dengue (defined as per WHO 2009 guidelines). Predictors were WHO 2009 warning signs: abdominal pain, lethargy, persistent vomiting, mucosal bleed, clinical fluid accumulation, hepatomegaly >2 cm, hematocrit ≥0.40 and platelet count <100x109/L. RESULTS: Among 350 children followed up completely till discharge/death, 90 developed severe dengue (event) while 260 did not (censored). Median age of study population was 7.75 y. Clinical fluid accumulation [(P=0.002, Hazard Ratio (HR) 2.19, 95% CI 1.33-3.60)] and hematocrit ≥0.40 [(P=0.009, HR (95%CI) 1.715, (1.13-2.60)] were significant in univariate analysis. Final multivariate model includes clinical fluid accumulation [(P=0.02, HR (95%CI) 1.89, (1.116-3.202)], hematocrit ≥0.40 (P=0.07), mucosal bleed (P=0.56) and persistent vomiting (P=0.32). CONCLUSION: WHO warning signs that predict time taken for progression to severe dengue in children include clinical fluid accumulation, hematocrit ≥0.40, persistent vomiting and mucosal bleed. Study results have implications in policy making and practice guidelines to triage children attending a health care facility with or without warning signs.

Development of a Prognostic Prediction Model to Determine Severe Dengue in Children.

OBJECTIVE: To develop a prognostic prediction model using the seven warning signs highlighted by WHO revised Dengue fever classification 2009 to determine severe dengue in children. METHODS: In this prospective analytical study conducted in a tertiary care centre, consecutive sampling of all children aged 1mo to 12y admitted with serologically confirmed Dengue was done from May 2015 through August 2016. After excluding 27 patients with co-infections and co-morbidities, 359 patients were followed up daily to assess clinical and laboratory progression till discharge/ death. Independent predictors were abdominal pain or tenderness, persistent vomiting, lethargy, mucosal bleed, clinical fluid accumulation, hepatomegaly >2 cm and rising hematocrit concurrent with platelet count <100 × 109/L. Outcome measure was severe dengue defined as per WHO guidelines 2009. RESULTS: Among 359 children, 93 progressed to severe dengue. In univariate analysis, significant predictors were clinical fluid accumulation (OR 4.773, p = 0.000, 95%CI 2.511-9.075), persistent vomiting (OR 1.944, p = 0.010, 95%CI 1.170-3.225), mucosal bleed (OR 2.045, p = 0.019, 95%CI 1.127-3.711) and hematocrit ≥0.40 concurrent with platelet count <100 × 109/L (OR 2.985, p = 0.000, 95%CI 1.783-4.997). The final multivariable model included clinical fluid accumulation (aOR 3.717, p = 0.000, 95%CI 1.901-7.269), hematocrit ≥0.40 concurrent with platelet count <100 × 109/L (aOR 2.252, p = 0.004, 95%CI 1.302-3.894) and persistent vomiting (p = 0.056) as predictors of severe dengue. CONCLUSIONS: Among seven WHO warning signs, predictors of severe dengue as suggested by the present multivariable prediction model include clinical fluid accumulation, persistent vomiting and hematocrit ≥0.40 concurrent with platelet count <100 × 109/L.

Klippel-Trenaunay syndrome and gestational trophoblastic neoplasm.

BACKGROUND: Klippel-Trenaunay syndrome is a non-heritable venous malformation with bone and soft tissue hypertrophy and cutaneous nevi. CASE CHARACTERISTICS: Neonate with Klippel Trenaunay syndrome born to a mother with past history of Gestational trophoblastic neoplasm. OBSERVATION: Antenatally, a fetal vascular malformation was identified ultrasonologically at 29 weeks gestation. Acute myeloid leukemia was diagnosed in mother at 33 weeks gestation. MESSAGE: A rare association of Klippel Trenaunay syndrome and gestational trophoblastic neoplasm with the possible role of either hyperglycosylated Human Chorionic Gonadotropin or chemotherapy as a link is highlighted.

Clinical response to antibiotics among children with bloody diarrhea.

WHO recommends ciprofloxacin as the drug of choice for bloody diarrhea. We retrospectively analyzed antibiotic response in 100 children with bloody diarrhea admitted between 2006-2010. Cotrimoxazole (n=55) had higher chance of attaining improved appetite and normal activity in 48 h, hospitalization of <3d, blood disappearance in ≤5d and not requiring a second antibiotic compared to others (n=45). Older antimicrobials should be tried in all possible situations.

Radiological clue to diagnosis of Canavan disease.

Canavan disease is an autosomal recessive leukodystrophy characterized by early onset developmental delay, initial hypotonia progressing to hypertonia, macrocephaly and blindness. The authors present an infant with these clinical features. MRI brain shows white matter changes with characteristic involvement of subcortical U fibres and MR spectroscopy shows the characteristic peak of N- acetyl aspartate. The importance of specific clinical features and imaging in the diagnosis of different leukodystrophies in resource and access limited settings is suggested.