Acute Disseminated Encephalomyelitis Presenting as Bilateral Ptosis in a Sri Lankan Child.

INTRODUCTION: Acute disseminated encephalomyelitis is a rare inflammatory demyelinating disease characterized by acute onset polyfocal neurological deficits associated with encephalopathy. It commonly presents with fever, meningism, seizures, ataxia, motor deficits, and bladder dysfunction. Although cranial neuropathies, including optic neuritis and facial nerve palsies, have previously been reported, children presenting with bilateral ptosis is extremely rare. Here, we report a 3-year-old child with acute disseminated encephalomyelitis presenting with acute onset bilateral ptosis due to involvement of the single central levator subnucleus of the oculomotor nerve. Case Presentation. A 3-year-old Sri Lankan boy presented with drooping of the upper eyelids for three days and unsteady gait for two days. He did not have seizures, blurring of vision, limb weakness, swallowing or breathing difficulties, or bladder dysfunction. On examination, he had bilateral ptosis, gait ataxia, and dysmetria. His vision, eye movements, and examination of other cranial nerves were normal. MRI brain revealed high signal intensities involving the subcortical white matter of parietal and occipital lobes, midbrain in the area of single central levator subnucleus of the oculomotor nerve, cerebellar vermis, and right cerebellar hemisphere. Based on the clinical features suggesting polyfocal neurological involvement of the midbrain and cerebellum and characteristic MRI findings, the diagnosis of acute disseminated encephalomyelitis was made. He responded well and rapidly to high-dose intravenous methylprednisolone and showed a complete clinical and radiological recovery. CONCLUSION: This case report describes a rare presentation of acute disseminated encephalomyelitis, bilateral ptosis due to involvement of the single central levator subnucleus of the oculomotor nerve. It highlights that the presenting manifestations of acute disseminated encephalomyelitis can be subtle and vary; however, timely diagnosis and treatment result in complete recovery.

Presenting status of children with classical congenital adrenal hyperplasia over two decades (1999-2018) in the absence of newborn screening in Sri Lanka.

OBJECTIVES: Although new-born screening (NBS) for classical congenital adrenal hyperplasia (C-CAH) has been available for decades, it is not widely implemented. We assessed the usefulness of introducing NBS for C-CAH, by analyzing presenting status of infants with C-CAH, over the past two decades, in Sri Lanka. METHODS: This retrospective clinic-based study, from the largest tertiary children's hospital in Sri Lanka, analyzed initial presenting features of children with C-CAH from 1999 to 2018, in the absence of NBS for CAH, and included gender-based comparisons. RESULTS: Features suggestive of impending adrenal-crisis were seen at initial presentation in >80 % (dehydration 70%, hyponatremia 65%, hyperkalemia 47%, vomiting 45%, hypoglycemia 22%, collapse 20%). Hyperpigmentation was seen in 78%, and consanguinity in 27%. There were fewer affected males (n = 12) compared to females (n = 28). Most girls (96%) had virilized genitalia, and 16 faced uncertainty about gender at birth. Median age at diagnosis was 20 days. More than 70% of children had SW-CAH (males = 9 and females = 20). There were fewer males with SW-CAH, and all had features of impending adrenal crisis, including severe hyponatremia in 50%, while 62% of girls also developed hyponatremia and 33% had hyperkalemia, prior to treatment. Treatment of SW-CAH was initiated at a median age of 30 days in boys, and 10 days of age in girls. CONCLUSION: Many boys and girls with C-CAH from Sri Lanka presented late with impending adrenal crisis. Males were diagnosed later, and some possibly succumbed to C-CAH undiagnosed. These findings support including CAH in NBS programs to avert preventable childhood morbidity and mortality.

Effect of Prior Symptomatic Dengue Infection on Dengue Haemorrhagic Fever (DHF) in Children.

Pathogenesis of dengue haemorrhagic fever is not fully understood, but it is thought that there is antibody enhancement during the secondary infection, which causes severe dengue haemorrhagic fever (DHF). Therefore, patients who have DHF should have a documented history of symptomatic dengue infection in the past. A retrospective descriptive-analytical study was conducted at the University Paediatric Unit at Lady Ridgeway Hospital for Children, Colombo, Sri Lanka. All children who had fulfilled the criteria for DHF admitted to the unit from April 2018 to September 2018 were recruited into the study. Relevant data were collected from bed head tickets. One hundred and eighty-four children were included in the final analysis. Thirty-three (17.9%) had a past history of documented symptomatic dengue infection, while 82.1% did not have a documented dengue infection. Twelve patients had dengue shock syndrome, and none of them had previously documented symptomatic dengue fever. Dextran was used in 96 patients in the critical phase. Twelve (42%) patients with past documented symptomatic dengue fever needed dextran while 84 (54.9%) patients without a documented past history of dengue fever needed dextran. In our clinical observation, we noticed that children with DHF mostly did not have a documented symptomatic prior dengue infection, while those with a documented symptomatic prior infection had a milder subsequent illness. In fact, the majority (82.1%) of patients with DHF did not have documented previous symptomatic dengue infection. It was also observed that the clinical course of subsequent dengue infection was less severe in patients with previously documented symptomatic dengue fever. This finding should be further evaluated in a larger scale study minimizing the all-confounding factors. This fact is more important in selecting recipients for vaccines against the dengue virus, which are supposed to produce immunity against the virus without causing the severe disease.