Journal of Pediatric Neurology and Neuroscience

Case Report Pages 79-83

Kabuki Syndrome: A Rare Genetic Multisystem Disorder in Bangladesh

Authors: Zeena Salwa, Md Masudur Rahman and AT Nizam Uddin Ahmed

Abstract: Purpose of reporting the case of a Bangladeshi female child diagnosed as Kabuki syndrome with all her clinical features, laboratory tests and genetic report. This case highlights the existence of the syndrome with its complexities. Management of the patient is supportive by multidisciplinary team for better outcomes as there is no cure till now. Prevention approaches with promotion of growth and development of the affected children should be provided.

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Case Report Pages 73-78

Pediatric RCVS in Newly Diagnosed all Treated with Intra- Arterial Nicardipine

Authors: Devin Simon, MD, Jaclyn Peraino, BS, Kabelo Thusang, MD and Suhad Khalil, MD

Abstract: Reversible Cerebral Vasoconstriction Syndrome (RCVS) is an uncommon cause of severe headache and other complications in the pediatric population. Diagnosis includes obtaining vessel imaging to confirm vasospasm in the cerebral vasculature. Therapy oftentimes requires calcium channel blockers administered through an intra-arterial approach. Resolution of symptoms and vasospasm are expected at approximately 3 months.

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Commentaries Pages 73-75

Step-By-Step Approach in Managing Sleep Problem in Children with Intellectual Disability

Authors: CHOW Chit Kwong

Abstract: We must emphasize the first step of management is correct identification of children with sleep disorders, and those with risk factors should receive extra attention. Children with ID are already a high risk group for developing sleep disorders we propose all of them should be routinely screened. This can be in form of standardized questionnaire such as Children's Sleep Habits Questionnaire (CSHQ)

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Case Report Pages 55-72

Food Color Additives in the US Food Supply: Review of Neurobehavioral Safety

Authors: G Craig Llewellyn, PhD, J Kim Penberthy, PhD and Julia M Parker, BA

Abstract: Currently approved food additives, including color additives, have a long history of safe use in human consumables. Many food additives are added to increase the flavor or other sensory appeal of food items and have typically been viewed as enhancing the experience of eating or drinking. Research on the impact of food color additives (FCAs) upon neurobehavior, especially focusing on hyperactive behavioral symptoms in children and adolescents

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Case Report Pages 54

X-Linked Periventricular Nodular Heterotopia a Rare Cause of Headache and Right Sided Hemiparesis in a 7-Year-Old Girl

Authors: Christine Loftis, BS, Aleksandr Chernyy, MD and Randy Williamson, MD

Abstract: X-linked Periventricular Nodular Heterotopia (X-linked PVNH) is a neuronal migration disorder that occurs because nerve cells fail to migrate during fetal development. The disorder is caused by a defect in the FLNA gene which is responsible for making the protein filamin A which is responsible for cellular structure

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Short Communication Pages 51-53

Authors: Nobuhiko Aoki, MD

Abstract: This entity is characterized by an extremely stereotypical clinical profile illustrated by the following hypothetical case: A Japanese male infant aged 5 ~12 months falls and hits his occipital region against a soft surface as he tries to stand up by putting his hands on the corner of the living room table. Immediately after the fall, the baby begins to cry, and the mother holds him in her arms and notes generalized convulsions. The baby is taken to the emergency room by ambulance, where a CT reveals an acute subdural hematoma. Retinal hemorrhage is also noted. Shortly thereafter he regains full activity and no neurological sequelae are noted.

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Case Report Pages 47-50

Infantile Acute Subdural Hematoma with Retinal Hemorrhage Caused by Minor Occipital Impact Witnessed by an ICU Nurse: A Case Report

Authors: Nobuhiko Aoki, M.D

Abstract: An acute subdural hematoma (ASDH) in infants without external signs of trauma is differently recognized as shaken baby syndrome (SBS) or abusive head trauma (AHT) in the United States or as infantile acute subdural hematoma (i-ASDH) due to minor head trauma in Japan. The aim of the present case report is to document the existence of i-ASDH as a discrete clinical entity differing from SBS and AHT.

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Review Article Pages 43-46

Diffuse Leptomeningeal Glioneuronal Tumor: Case Report and Review of Literature

Authors: Neelan J Marianayagam, MD, PhD, Netanel Ben-Shalom, MD, Helen Toledano, MD, Nevo Marglit, MD, Shalom Michowiz, MD and Gustavo Rajz, MD

Abstract: In this study, we describe a rare case of disseminated CNS malignancy of childhood. A seven-year-old boy with headache, vomiting and gait disturbances was diagnosed with non-obstructive hydrocephalus and papilledema. Initial MRI revealed no significant findings, however subsequent studies revealed bilateral extradural and leptomeningeal enhancement.

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