The fate of rice crop residues and context-dependent greenhouse gas emissions: Model-based insights from Eastern India.

Crop residue burning is a common practice in many parts of the world that causes air pollution and greenhouse gas (GHG) emissions. Regenerative practices that return residues to the soil offer a 'no burn' pathway for addressing air pollution while building soil organic carbon (SOC). Nevertheless, GHG emissions in rice-based agricultural systems are complex and difficult to anticipate, particularly in production contexts with highly variable hydrologic conditions. Here we predict long-term net GHG fluxes for four rice residue management strategies in the context of rice-wheat cropping systems in Eastern India: burning, soil incorporation, livestock fodder, and biochar. Estimations were based on a combination of Tier 1, 2, and 3 modelling approaches, including 100-year DNDC simulations across three representative soil hydrologic categories (i.e., dry, median, and wet). Overall, residue burning resulted in total direct GHG fluxes of 2.5, 6.1, and 8.7 Mg CO2-e in the dry, median, and wet hydrologic categories, respectively. Relative to emissions from burning (positive values indicate an increase) for the same dry to wet hydrologic categories, soil incorporation resulted in a -0.2, 1.8, or 3.1 Mg CO2-e change in emissions whereas use of residues for livestock fodder increased emissions by 2.0, 2.1, or 2.3 Mg CO2-e. Biochar reduced emissions relative to burning by 2.9 Mg CO2-e in all hydrologic categories. This study showed that the production environment has a controlling effect on methane and, therefore, net GHG balance. For example, wetter sites had 2.8-4.0 times greater CH4 emissions, on average, than dry sites when rice residues were returned to the soil. To effectively mitigate burning without undermining climate change mitigation goals, our results suggest that geographically-target approaches should be used in the rice-based systems of Eastern India to incentivize the adoption of regenerative 'no burn' residue management practices.

Tillage and crop establishment effects on weeds and productivity of a rice-wheat-mungbean rotation.

Weeds are one of the key threats in sustaining the productivity of the rice-wheat cropping system in the Indo-Gangetic Plains. The development of sound integrated weed management technologies requires knowledge of mechanisms that influence weed flora composition and weed seedbank dynamics. A long-term study was initiated in 2015 at Patna, Bihar, India to evaluate the effect of seven tillage and crop establishment methods on weed density, weed seedbank composition, and crop productivity in rice-wheat-mungbean rotation. All the treatments included zero-till mungbean after wheat. Tillage and crop establishment methods had differential effects on weed and weed seedbank composition. In rice, zero-till direct-seeded rice recorded 62% lower emergence of Cyperus iria, 82-90% of Echinochloa colona, and 81-83% of total weeds compared to tilled systems, but the system of rice and wheat intensification favoured E. colona. In wheat, the system of wheat intensification favoured the Phalaris minor and Solanum nigrum. Zero-till rice and wheat reduced the seedbank of Trianthema portulacastrum by 95%, and total weed seedbank by 62% compared to the system of rice and wheat intensification. Nearly, 72% of C. iria seeds, 62% of grasses, and 64% of broad-leaved weeds were in 0-15 cm soil layer. Zero-till direct-seeded rice produced a 13% lower rice grain yield than conventional puddled transplanted rice. Compared to the system of wheat intensification, zero-till wheat under triple zero-till systems produced an 11.5% higher grain yield. Managing weed seedbank is a long-term endeavour. The present study revealed that tillage and crop establishment methods influence weed density and diversity. Under zero-till rice-wheat system, rice yield decreases marginally, but the system productivity maintains due to improvement in succeeding wheat yield. This system is also helpful in reducing the weed flora density and soil weed seedbank. Regular monitoring and management of emerging pests such as armyworm (Mythimna separata) are, however, required. The study suggests that the adoption of triple zero-tillage can be a viable option for reducing the weed density and weed seedbank concurrently increasing the system productivity of the rice-wheat-mungbean cropping system in eastern Indo-Gangetic Plains.

Can yield, soil C and aggregation be improved under long-term conservation agriculture in the eastern Indo-Gangetic plain of India?

Deteriorating soil health, diminishing soil organic carbon (SOC), development of subsurface hard compact layer and declining system productivity are barriers to achieving sustainable production in the traditional rice-wheat cropping system (TA) in the eastern Indo-Gangetic Plain of India. Conservation agriculture (CA), which favours minimum soil disturbance, crop residue retention and crop diversification could be a viable alternative to the TA to address most of those major problems. With that in mind, a long-term experiment is being implemented at ICAR-RCER, Patna, Bihar, India, with four treatments: (a) TA, (b) full CA (fCA) and (c and d) partial CA (pCA1 and pCA2), differing in crop establishment methods, cropping system and crop residue management in a randomized complete block design. Measurement of soil health parameters was carried out in the 11th year of the experiment. The results revealed a beneficial effect of CA and 46 and 40% increase in SOC concentration and stock, respectively, under fCA over TA in the 0-7.5-cm soil layer. The effect of partial CA (pCA1 and pCA2) was variable, but an increasing trend was always observed under pCA compared to TA. There was an enrichment in SOC content of aggregates under CA irrespective of size class; however, no relation was found between SOC content and aggregate diameter. The contribution of macroaggregates to SOC stock was larger (36-66%) under CA in the 0-7.5-cm soil layer. Adoption of CA improved the macroaggregate content, MWD and GMD of aggregates, and aggregation ratio. Soil macropore content was greater under fCA, whereas other parameters were similar among treatments. The impact of CA was mostly limited to 0-7.5 cm soil layer and a maximum up to 15 cm soil depth while evaluation until 60 cm soil depth was realized. The yield of rice in CA was comparable to or higher than in TA, whereas the system rice equivalent yield was always higher (38-53%) under CA than under the conventional practices. Therefore, a CA-based cropping system must be encouraged, to increase SOC status, improve aggregation stability and, consequently, sustain or increase system productivity, in order to achieve food and nutritional security in the eastern Indo-Gangetic Plain of India. HIGHLIGHTS: Effects of long-term conservation agriculture (CA) on soil C, aggregation and yield were evaluated.CA improved SOC concentration and stock by 46 and 40%, as well as macroaggregate SOC stock by 36-66%.Macro-aggregation and mean weight diameter improved in CA but was mostly limited to a shallow soil depth.CA can be promoted for sustainability of a rice-wheat system due to higher productivity (38-53%).

An impact of agronomic practices of sustainable rice-wheat crop intensification on food security, economic adaptability, and environmental mitigation across eastern Indo-Gangetic Plains.

In the eastern Indo-Gangetic Plains (EIGP), conventional rice-wheat system has led to a decline in productivity, input-use efficiency, and profitability. To address these, a four-year field study was conducted to evaluate the performance of tillage and crop establishment (TCE) methods in rice-wheat-greengram rotation. The treatments included: 1) random puddled transplanted rice (RPTR) - conventional-till broadcast wheat (BCW) - zero-till greengram (ZTG); 2) line PTR (LPTR) - conventional-till drill sown wheat (CTW) - ZTG; 3) machine transplanted rice in puddled soil (CTMTR) - zero tillage wheat (ZTW) - ZTG; 4) machine transplanted rice in zero-till wet soil (ZTMTR) - ZTW - ZTG; 5) system of rice intensification (SRI) - system of wheat intensification (SWI) - ZTG; 6) direct-seeded rice (DSR) - ZTW - ZTG; and 7) zero-till DSR - ZTW - ZTG. During the initial two years, conventional rice system (PTR) recorded a 16.2 % higher rice grain yield than DSR system. Whereas in the fourth year, the rice yields under DSR and PTR were comparable. As compared to SRI/SWI, the average wheat yield in ZT system was significantly high, whereas in rice, SRI/SWI system was comparable with CT system. ZTW after non-puddled rice was at par to CTW after PTR. The ZT wheat produced 4.6 % more yield than CT system. DSR production system consumed 6.8 % less water compared to transplanted system. On the system basis, 10.8 % higher net returns were recorded with CA-based system compared to conventional system. The system energy productivity under CA-based production system was 14-36 % higher than PTR-based systems. CA-based system also led to 8-10 % lower global warming potential (GWP) than conventional methods. The current study indicated that as compared to conventional system, a significant gain in productivity, profitability and energy-use efficiency, and reduction in the environmental mitigation are possible with emerging alternative TCE methods. Long-term expansion and further refinement of these technologies in local areas need to be explored for the second green revolution.

Sustainable agriwaste management at farm level through self-reliant farming system.

Annually 500 M t organic wastes are produced in India from the agriculture sector. Transportation of bulky organic manures for centralized collection, processing and distribution to farms is cost prohibitive. Hence, recycling of agricultural wastes using vermicompost technology at the farm level is a practical way of managing agriwaste for meeting the plant nutrient requirement. Our experience with a 1.584 ha farm for three years (2015-2016 to 2017-2018) revealed that 8.1 t vermicompost was produced in three batches from 24 t agriwastes produced within the farm area. The system productivity by recycling these farm generated agriwastes and run-off water was 18.05 t (≈11.4 t ha-1) rice equivalent yield which was higher by 2.6 times as compared to rice fallow (4.46 t ha-1). Also, the net return from this system (Indian rupees 70141 ha-1) was higher by 2.3 times, after considering the fixed cost towards construction of a water recycling pond. An increase in carbon stock in soil for the four years study period was 0.66 Mg ha-1 year-1 with the agriwaste recycling system under organic nutrition. For the inorganic fertilizer plot, the increase in carbon stock was 0.53 Mg ha-1 year-1. A decrease in bulk density from 1.56 to 1.46 Mg m-3, increase in water holding capacity from 0.43 to 0.52 cm3 cm-3 and increase in available P and K content in soil from 38.0 and 174.7 kg ha-1 to 45.8 and 186.5 kg ha-1, respectively, were noted. Thus, recycling of agricultural waste at the farm level is useful in improving soil health and crop productivity.

Association of Child Neurology (AOCN) Consensus Statement on the Diagnosis and Management of Febrile Seizures.

JUSTIFICATION: Febrile seizures are quite common in children but there are controversies in many aspects of their diagnosis and management. METHODS: An expert group consisting of pediatric neurologists and pediatricians was constituted. The modified Delphi method was used to develop consensus on the issues of definitions and investigations. The writing group members reviewed the literature and identified the contentious issues under these subheadings. The questions were framed, pruned, and discussed among the writing group members. The final questions were circulated to all experts during the first round of Delphi consensus. The results of the first round were considered to have arrived at a consensus if more than 75% experts agreed. Contentious issues that reached a 50-75% agreement was discussed further in online meetings and subsequently voting was done over an online platform to arrive at a consensus. Three rounds of Delphi were conducted to arrive at final statements. RESULTS: The expert group arrived at a consensus on 52 statements. These statements pertain to definitions of febrile seizures, role of blood investigations, urine investigations, neuroimaging, electroencephalography (EEG), cerebrospinal fluid analysis and screening for micronutrient deficiency. In addition, role of rescue medications, intermittent anti-seizure medication and continuous prophylaxis, antipyretic medication and micronutrient supplementation have been covered. CONCLUSIONS: This consensus statement addresses various contentious issues pertaining to the diagnosis and management of febrile seizures. Adoption of these statements in office practice will improve and standardize the care of children with this disorder.

Clinical and molecular spectrum associated with Polymerase-γ related disorders.

BACKGROUND: POLG pathogenic variants are the commonest single-gene cause of inherited mitochondrial disease. However, the data on clinicogenetic associations in POLG-related disorders are sparse. This study maps the clinicogenetic spectrum of POLG-related disorders in the pediatric population. METHODS: Individuals were recruited across 6 centers in India. Children diagnosed between January 2015 and August 2020 with pathogenic or likely pathogenic POLG variants and age of onset <15 years were eligible. Phenotypically, patients were categorized into Alpers-Huttenlocher syndrome; myocerebrohepatopathy syndrome; myoclonic epilepsy, myopathy, and sensory ataxia; ataxia-neuropathy spectrum; Leigh disease; and autosomal dominant / recessive progressive external ophthalmoplegia. RESULTS: A total of 3729 genetic reports and 4256 hospital records were screened. Twenty-two patients with pathogenic variants were included. Phenotypically, patients were classifiable into Alpers-Huttenlocher syndrome (8/22; 36.4%), progressive external ophthalmoplegia (8/22; 36.4%), Leigh disease (2/22; 9.1%), ataxia-neuropathy spectrum (2/22; 9.1%), and unclassified (2/22; 9.1%). The prominent clinical manifestations included developmental delay (n = 14; 63.7%), neuroregression (n = 14; 63.7%), encephalopathy (n = 11; 50%), epilepsy (n = 11; 50%), ophthalmoplegia (n = 8; 36.4%), and liver dysfunction (n = 8; 36.4%). Forty-four pathogenic variants were identified at 13 loci, and these were clustered at exonuclease (18/44; 40.9%), linker (13/44; 29.5%), polymerase (10/44; 22.7%), and N-terminal domains (3/44; 6.8%). Genotype-phenotype analysis suggested that serious outcomes including neuroregression (odds ratio [OR] 11, 95% CI 2.5, 41), epilepsy (OR 9, 95% CI 2.4, 39), encephalopathy (OR 5.7, 95% CI 1.4, 19), and hepatic dysfunction (OR 4.6, 95% CI 21.3, 15) were associated with at least 1 variant involving linker or polymerase domain. CONCLUSIONS: We describe the clinical subgroups and their associations with different POLG domains. These can aid in the development of follow-up and management strategies of presymptomatic individuals.

Long-Term Efficacy of T3 Analogue Triac in Children and Adults With MCT8 Deficiency: A Real-Life Retrospective Cohort Study.

CONTEXT: Patients with mutations in thyroid hormone transporter MCT8 have developmental delay and chronic thyrotoxicosis associated with being underweight and having cardiovascular dysfunction. OBJECTIVE: Our previous trial showed improvement of key clinical and biochemical features during 1-year treatment with the T3 analogue Triac, but long-term follow-up data are needed. METHODS: In this real-life retrospective cohort study, we investigated the efficacy of Triac in MCT8-deficient patients in 33 sites. The primary endpoint was change in serum T3 concentrations from baseline to last available measurement. Secondary endpoints were changes in other thyroid parameters, anthropometric parameters, heart rate, and biochemical markers of thyroid hormone action. RESULTS: From October 15, 2014 to January 1, 2021, 67 patients (median baseline age 4.6 years; range, 0.5-66) were treated up to 6 years (median 2.2 years; range, 0.2-6.2). Mean T3 concentrations decreased from 4.58 (SD 1.11) to 1.66 (0.69) nmol/L (mean decrease 2.92 nmol/L; 95% CI, 2.61-3.23; P < 0.0001; target 1.4-2.5 nmol/L). Body-weight-for-age exceeded that of untreated historical controls (mean difference 0.72 SD; 95% CI, 0.36-1.09; P = 0.0002). Heart-rate-for-age decreased (mean difference 0.64 SD; 95% CI, 0.29-0.98; P = 0.0005). SHBG concentrations decreased from 245 (99) to 209 (92) nmol/L (mean decrease 36 nmol/L; 95% CI, 16-57; P = 0.0008). Mean creatinine concentrations increased from 32 (11) to 39 (13) µmol/L (mean increase 7 µmol/L; 95% CI, 6-9; P < 0.0001). Mean creatine kinase concentrations did not significantly change. No drug-related severe adverse events were reported. CONCLUSIONS: Key features were sustainably alleviated in patients with MCT8 deficiency across all ages, highlighting the real-life potential of Triac for MCT8 deficiency.

Disease characteristics of MCT8 deficiency: an international, retrospective, multicentre cohort study.

BACKGROUND: Disordered thyroid hormone transport, due to mutations in the SLC16A2 gene encoding monocarboxylate transporter 8 (MCT8), is characterised by intellectual and motor disability resulting from cerebral hypothyroidism and chronic peripheral thyrotoxicosis. We sought to systematically assess the phenotypic characteristics and natural history of patients with MCT8 deficiency. METHODS: We did an international, multicentre, cohort study, analysing retrospective data from Jan 1, 2003, to Dec 31, 2019, from patients with MCT8 deficiency followed up in 47 hospitals in 22 countries globally. The key inclusion criterion was genetically confirmed MCT8 deficiency. There were no exclusion criteria. Our primary objective was to analyse the overall survival of patients with MCT8 deficiency and document causes of death. We also compared survival between patients who did or did not attain full head control by age 1·5 years and between patients who were or were not underweight by age 1-3 years (defined as a bodyweight-for-age Z score <-2 SDs or <5th percentile according to WHO definition). Other objectives were to assess neurocognitive function and outcomes, and clinical parameters including anthropometric characteristics, biochemical markers, and neuroimaging findings. FINDINGS: Between Oct 14, 2014, and Jan 17, 2020, we enrolled 151 patients with 73 different MCT8 (SLC16A2) mutations. Median age at diagnosis was 24·0 months (IQR 12·0-60·0, range 0·0-744·0). 32 (21%) of 151 patients died; the main causes of mortality in these patients were pulmonary infection (six [19%]) and sudden death (six [19%]). Median overall survival was 35·0 years (95% CI 8·3-61·7). Individuals who did not attain head control by age 1·5 years had an increased risk of death compared with patients who did attain head control (hazard ratio [HR] 3·46, 95% CI 1·76-8·34; log-rank test p=0·0041). Patients who were underweight during age 1-3 years had an increased risk for death compared with patients who were of normal bodyweight at this age (HR 4·71, 95% CI 1·26-17·58, p=0·021). The few motor and cognitive abilities of patients did not improve with age, as evidenced by the absence of significant correlations between biological age and scores on the Gross Motor Function Measure-88 and Bayley Scales of Infant Development III. Tri-iodothyronine concentrations were above the age-specific upper limit in 96 (95%) of 101 patients and free thyroxine concentrations were below the age-specific lower limit in 94 (89%) of 106 patients. 59 (71%) of 83 patients were underweight. 25 (53%) of 47 patients had elevated systolic blood pressure above the 90th percentile, 34 (76%) of 45 patients had premature atrial contractions, and 20 (31%) of 64 had resting tachycardia. The most consistent MRI finding was a global delay in myelination, which occurred in 13 (100%) of 13 patients. INTERPRETATION: Our description of characteristics of MCT8 deficiency in a large patient cohort reveals poor survival with a high prevalence of treatable underlying risk factors, and provides knowledge that might inform clinical management and future evaluation of therapies. FUNDING: Netherlands Organisation for Health Research and Development, and the Sherman Foundation.

Longitudinally Extensive Transverse Myelitis (LETM) Following Varicella Infection in a 13-Year Immunocompetent Child.

Varicella infection in children is a common self-limited illness with neurological complications in less than 0.1% of cases. Longitudinally extensive transverse myelitis (LETM) is uncommon in children following infection with varicella-zoster virus. We describe a case of 13-yr immunocompetent girl with LETM following varicella infection shown a dramatic clinical response to a combination of acyclovir and pulse steroids.

Development and validation of AIIMS modified INCLEN diagnostic instrument for epilepsy in children aged 1 month-18 years.

OBJECTIVES: There is shortage of specialists for the diagnosis of children with epilepsy, especially in resource limited settings. Existing INCLEN (International Clinical Epidemiology Network) instrument was validated for children aged 2-9 years. The current study validated modifications of the same including wider symptomatology and age group. METHODS: The Modified INCLEN tool was validated by a team of experts by modifying the existing tools (2-9 years) to widen the age range from 1 month to 18 years and include broader symptomatology in a tertiary care teaching hospital of North India between January and June 2015. A qualified medical graduate applied the candidate tool which was followed by gold standard evaluation by a Pediatric Neurologist (both blinded to each other). RESULTS: A total of 197 children {128 boys (65%) and 69 girls (35%)}, with a mean age of 72.08 (±50.96) months, completed the study. The sensitivity, specificity, positive and negative predictive value, positive and negative likelihood ratio of the modified epilepsy tool were 91.5% (84.5-96.1), 88.6% (80.0-93.5), 89.7% (81.9-95.3), 90.8% (83.7-95.7), 8 (6.6-9.8) and 0.09 (0.07-0.12) respectively. SIGNIFICANCE: The new modified diagnostic instruments for epilepsy is simple, structured and valid instruments covering 1month to 18 years for use in resource limited settings with acceptable diagnostic accuracy. All seizure semiologies as well as common seizure mimics like breath-holding spells are included in the tool. It also provides for identification of acute symptomatic and febrile seizures.

Development of All India Institute of Medical Sciences-Modified International Clinical Epidemiology Network Diagnostic Instrument for Neuromotor Impairments in Children Aged 1 Month to 18 Years.

INTRODUCTION: There is shortage of specialists for the diagnosis of children with neuromotor impairments (NMIs), especially in resource limited settings. Existing International Clinical Epidemiology Network (INCLEN) instrument for diagnosing NMI have been validated for children aged 2-9 years. The current study modified the same including wider symptomatology and age group (1 month to 18 years). METHODS: The Modified INCLEN diagnostic tool (INDT) was developed by a team of experts by modifying the existing tool to widen the age range (1 month to 18 years) and include broader symptomatology (inclusion of milestones from the first 2 years of life and better elucidation of cerebellar and extrapyramidal features) in a tertiary care teaching hospital of North India between January and April 2015. A trained medical graduate applied the candidate tool, which was followed by gold standard evaluation by a Pediatric Neurologist (both blinded to each other). RESULTS: A total of 197 children (102 with NMI and 95 without NMI) were enrolled for the study. The sensitivity, specificity, positive and negative predictive values, positive and negative likelihood ratio of the modified NMI tool were 90.4% (82.6-95.5), 95.5% (88.7-98.7), 95.5% (88.9-98.7), 90.3% (82.4-95.5), 19.9 (12.1-32.6), and 0.13 (0.08-0.12), respectively. CONCLUSION: The All India Institute of Medical Sciences modified INDT NMI tool is a simple and structured instrument covering a wider symptomatology in the 1 month to 18 years age group with acceptable diagnostic accuracy.

Bilateral ophthalmoplegia in a child with migraine.

BACKGROUND: In children, migraine with or without aura is a common entity, however variants like recurrent painful optic neuropathy (RPON) is rarely encountered. CASE RESULT: A 9 year old boy presented with headache for 1 week and restricted movements and drooping in both eyes for last 3 days. On examination he had bilateral ophthalmoplegia and ptosis. History of migrainous headache was present in the patient as well as his mother. His MRI brain with venogram, serum autoimmune markers, serum and urine toxicology screen and repetitive nerve stimulation test were normal. He received intravenous pulse followed by oral steroids for 6 weeks and was started on antimigraine prophylaxis. Eighteen months since the attack, he has improved completely with mild asymmetric mydriasis persisting. DISCUSSION AND CONCLUSION: This may represent first attack of RPON in a child with migraine. Rarely this may herald the onset of migraine as well, index of suspicion should be high as it is a diagnosis of exclusion and a treatable entity.

Finger drop sign: Rare presentation of a common disorder.

BACKGROUND: Guillain Barre syndrome (GBS) commonly presents with limb weakness and occasional cranial nerve, respiratory or autonomic involvement. Isolated or predominant bilateral finger drop as presenting feature has never been reported in the pediatric age group. CASE: A 9-year-old boy presented with deformity of both hands for 7 days and leg pain with difficulty in getting up from floor for 3 days. On examination he had bilateral clawing with subtle hip flexor weakness and hyporeflexia. His nerve conduction study revealed motor axonal neuropathy. His serum lead levels and autoimmune markers were within normal limits. His cerebrospinal fluid examination revealed albuminocytological dissociation. He was diagnosed as GBS and was given intravenous immunoglobulin. He improved completely over next 8 weeks. CONCLUSIONS: GBS is one of the commonest causes of acquired neuropathy in the tropics. In resource limited setting, where electrophysiological facilities may not be available, identification of finger drop sign may help in correct management.

Isolated cerebellar involvement in vitamin B12 deficiency: a case report.

Deficiency of vitamin B12 causes megaloblastic anemia and nervous system demyelination. Structures affected in the nervous system include spinal cord, cranial and peripheral nerves, and brain white matter. A 9-year-old boy presented with knuckle hyperpigmentation and oral ulcers for 3 years, pallor and easy fatigability for 6 months, gait abnormalities for 3 months, and abnormal speech and behavioral abnormalities for 3 days. On examination, he had physical signs of megaloblastic anemia, mood swings with intermittent hallucinations, and features of cerebellar impairment. Blood investigations revealed megaloblastic anemia, and pernicious anemia was ruled out. Brain magnetic resonance imaging (MRI) revealed bilateral cerebellar signal changes. He received treatment for vitamin B12 deficiency and appropriate nutritional counseling. Three months later, he showed significant clinical and radiologic resolution. To our knowledge, isolated cerebellar involvement as the sole neurologic manifestation of vitamin B12 deficiency has not been described previously in children.

Leukodystrophy presenting as acute-onset polyradiculoneuropathy.

BACKGROUND: Sulfatides, the most abundant glycosphingolipids, are a major component of myelin. They are degraded by the combined action of sphingolipid activator protein and arylsulfatase A. Deficiency of either of these entities causes metachromatic leukodystrophy (MLD). On the basis of age of onset, this entity is divided into late infantile, juvenile, and adult subtypes. Late infantile form, the commonest subtype, can exhibit peripheral neuropathy as the initial manifestation. The other two forms usually manifest peripheral neuropathy later in the disease course. PATIENT: A 1.5-year-old girl with preexisting isolated motor delay presented with acute-onset ascending flaccid quadriparesis, ptosis, and respiratory failure. Ptosis and respiratory failure responded completely to intravenous immunoglobulin, whereas quadriparesis showed minimal improvement. Nerve biopsy revealed metachromatic granules with demyelination, and serum arylsulfatase A levels were undetectable. CONCLUSION: The severity and nature of the disease coupled with the response to immunotherapy makes this case unusual. This child may represent either an atypical presentation of MLD with coincidental response to immunotherapy or an episode of immune mediated neuropathy in an individual with already diseased nerves due to MLD.