Ratan Gupta

Janani Shishu Suraksha Karyakram and out of pocket expenditure

Sir, We read with interest the article on out-of-pocket expenditure (OOPE) by Mondal et al.1 The authors reiterate the need to provide fi nancial protection for ensuring universal institutional delivery at zero expense through the Janani Shishu Suraksha Karyakram (JSSK). Different variables were indicated for higher OOPE in the study. However, consideration of economic status of the benefi ciaries would have enriched the study. As pointed out previously, a higher amount during institutional delivery is incurred by economically well-off families.2,3 While it is true that the study analyzed the amount of expenditure and it might be a proxy for wealth index, but the severity of economic burden precipitated by that expenditure would remain unclear from such analysis. In fact, the greatest effect of free care would be expected among those having the least capacity to spend. As evident from literature, a signifi cant reduction in OOPE was experienced among the fi nancially weaker sections, after the introduction of the JSSK.4

Two Brothers with Multiple Cranial Nerve Palsies

To the Editor: Two developmentally normal adolescent brothers presented with sudden onset ptosis and external ophthalmoplegia. In addition, the first sibling had neck stiffness and the second had left upper motor neuron type facial nerve palsy. Rest of the neurological and systemic examinations were normal. There was no preceding insect or snake bite, ingestion of canned food or mushrooms or sleeping in an ill-ventilated room with burning fuel. There was no preceding joint pain, skin rash, sore throat or neck swelling, fever, loose stools or vomiting. There was, however, history of recent visit to a nearby grassland. Hemogram, Venereal Disease Research Laboratory (VDRL), Electrocardiogram of both were normal. Cerebrospinal fluid analysis revealed 10 mononuclear cells, protein of 50 mg%with CSF sugar of 58 against blood sugar of 80 mg% in the first, and similar findings in the second sibling. Contrast Enhanced Computed Tomography and Nerve Conduction studies of both siblings were normal. MRI brain could not be done because of financial constraints. IgM antibody to Borrelia burgdoferi was 2.10 U/ml (<0.90 U/mL) and IgG antibody to Borrelia burgdoferiwas 1.20 U/ml (<0.90 U/mL). They received injection crystalline penicillin followed by doxycycline for 3 wk with resolution of ptosis, external opthalmoplegia and neck signs. However, second sibling’s facial nerve palsy persisted at discharge. Lyme disease is caused by Borrelia burgdorferi, and is transmitted by deer ticks of the genus Ixodes. Stage 2 or early disseminated Lyme disease presents as neuroborreliosis presenting with cranial neuropathy or meningeal irritation or myocarditis. The presentation in two siblings simultaneously first raised suspicion of a snake bite or a poisoning. However, snake envenomation was ruled out due to presence of meningeal signs, absence of history of bite, absence of local swelling or fang marks and resolution without any antidote. Moreover, multiple cranial nerve palsies with meningeal signs did not fit into toxidrome except isolated cranial nerve palsies in mushroom poisoning, carbon monoxide poisoning and lead poisoning. Meningeal signs were not explained by any of these. Moreover, there was no suggestive exposure history and resolution occurred without chelation but with antibiotics. Instead, the presentation was consistent with stage 2 neuroborelliosis. The diagnosis was clinched by the positive Lyme serology and prompt response to antibiotics. The Centers for Disease Control (CDC) recommends a two-step testing process for diagnosis. Screening is done by ELISA and Western blot is used for supplementation. False positive results may occur with syphilis, other rickettsial diseases, asymptomatic individuals living in endemic areas, immunization with recombinant outer-surface protein A (OspA) Lyme disease vaccine or tick-borne relapsing fever (Borrelia hermsii) that appeared less likely in the present scenario. There are isolated case reports of its presentation in India as neuroretinitis from South India [1], an acute febrile illness from Bhopal [2] and isolated dermatological manifestations [3]. In a seroprevalence study of B burgdorferi infection in NorthEastern states of India [4], 13 % of 500 cases were positive for B burgdorferi specific IgG. Due to migrant populations and a large number of the urban poor residing near grassy or woody populations, this disease may be an emerging infectious disease in India and a high index of suspicion is required to diagnose this potentially treatable zoonotic infection.

Rare association of lissencephaly with hirschprung's disease

Lissencephaly (LIS) and HSCR disease both have been described in the literature widely in isolation. Both the conditions are attributed to neurocristopathies. Their cooccurrence in a child is very uncommon in the literature with only three cases reported so far. Whether this association is actually by chance or is overlooked and is attributed to other etiologies of constipation is still not proven. However, this co-occurrence has been emphasized rarely. Therefore, we describe this rare association.

Hypokalemic periodic paralysis and distal renal tubular acidosis associated with renal morphological changes

We report an unusual case of 5-yrs-old girl presenting with recurrent episodic weakness with documented hypokalemia, polyuria and failure to thrive. The child was finally diagnosed as having distal renal tubular acidosis. Imaging studies revealed associated hypoechoic spaces in renal medulla. Long term treatment with alkali and maintenance of normokalemia lead to regression of these morphological changes.