Journal of Pediatric Neurology - Volume 8, issue 2

Authors: Khairkar, Praveen | Bang, Govind | Agarwal, Amit | Lakhkar, Bhavna | Jagzape, Tushar

Article Type: Research Article

Abstract: Acute disseminated encephalomyelitis is a rare inflammatory demyelinating entity affecting central nervous system. This immune mediated disease shows variable presentation of motor, sensory, visual, gait, and memory disturbances that usually follow vaccinations or some viral or bacterial infections. Review of literature showed few case reports of acute disseminated encephalomyelitis presenting as psychiatric disorders like acute Korsakoff's syndrome, conversion disorder and acute psychosis. However, none of them had been reported in pre-adolescent age …group to the best of our knowledge. We herein report the first case of depersonalization disorder in a child suffering from acute disseminated encephalomyelitis. Show more

Keywords: Depersonalization disorder, acute disseminated encephalomyelitis, pre-adolescence, neuropsychiatry

DOI: 10.3233/JPN-2010-0391

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 211-214, 2010

Authors: Singh, Paramjeet | Vyas, Sameer | Singhal, Manphool | Ray, Munni | Khandelwal, Niranjan

Article Type: Research Article

Abstract: Clinical presentations of childhood cerebral sinovenous thrombosis are nonspecific and variable. Computed tomography venography and magnetic resonance imaging with magnetic resonance venography are the investigation of choice for the diagnosis of sinovenous thrombosis. We present an 8-month-old female child with a history of loose stools and vomiting. She subsequently developed altered consciousness and abnormal jerky movements. Magnetic resonance imaging showed sub acute hemorrhagic bithalamic infarcts with deep sinus venous thrombosis. A brief …review of the deep sinus venous thrombosis including the present case is presented. Show more

Keywords: Cerebral venous thrombosis, magnetic resonance imaging

DOI: 10.3233/JPN-2010-0392

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 215-218, 2010

Authors: Gondaliya, Dushyant R. | Shrama, Surendra K. | Kundu, Sumita | Roy, Supriya

Article Type: Research Article

Abstract: Hallervorden-Spatz disease (HSD) is a rare neurodegenerative disorder of autosomal recessive inheritance with age of onset between 1 year and 10~years and a life span of about 10 years after onset. The main clinical picture is characterized by extra pyramidal dysfunction-dystonia, rigidity and choreoathetosis. Other corroborative findings include corticospinal tract involvement, intellectual impairment, visual problems and seizures. Typical findings in magnetic resonance imaging (MRI) of brain help in early diagnosis of HSD. …Here, we are presenting a case of HSD with positive familial history, characteristic clinical features and MRI findings. In this report, we will discuss the clinical features of the patient with special emphasis on MRI findings and compare it with available literature. Show more

Keywords: Hallervorden-Spatz disease, basal ganglia, iron deposition

DOI: 10.3233/JPN-2010-0389

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 219-222, 2010

Authors: Koul, Roshan | Al Futaisi, Amna | Alzri, Faisal | Jain, Rajeev

Article Type: Research Article

Abstract: A 2-year-old boy presented with left sided partial motor seizures with progressive hemiparesis. Over time, he lost speech. Magnetic resonance imaging revealed progressive atrophy of the right brain hemisphere. Monthly immunoglobulin infusion in addition to antiepileptic drugs resulted in some improvement of his weakness and speech.

Keywords: Rassmussen's encephalitis, hemiparesis, seizures, immunoglobulin

DOI: 10.3233/JPN-2010-0393

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 223-226, 2010

Authors: Gosalakkal, Jayaprakash A. | Prasad, Balky A.

Article Type: Research Article

Abstract: Arteriovenous malformations (AVM) of the brain are uncommon congenital lesions with a complex array of arterial to venous shunts. The common clinical manifestations include hemorrhage, seizures, headache and congestive heart failure. We recently saw a child who presented with bilateral ptosis, a rather rare and unusual manifestation of AVM. We discuss the reported ocular manifestations of AVM and the possible pathogenesis of the ptosis in this child.

Keywords: Ptosis, arteriovenous malformations

DOI: 10.3233/JPN-2010-0394

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 227-229, 2010

Authors: Rajapakse, Thilinie | Ramaswamy, Vijay | Scozzafava, James | Sinclair, D. Barry

Article Type: Research Article

Abstract: Diffusion weighted imaging is currently the standard of care in the early imaging of hypoxic-ischemic brain injury. Its use has been well described in adults; however, there is a paucity of data regarding its use and reliability in the immature brain. Here we report a case of a 17-month-old who died from a diffuse hypoxic ischemic injury secondary to asphyxiation where there was a failure of diffusion-weighted imaging to show diffuse injury. The electroencephalogram, neurological examination …and computerized tomography all suggested global hypoxic-ischemic injury, yet the diffusion weighted imaging showed only focal ischemia at 48 hours post injury. We discuss possible mechanisms underlying the failure of diffusion-weighted imaging to provide an accurate measure of the severity of this child's injury, and highlight the importance of the neurological examination in determination of prognosis in hypoxic-ischemic brain injury. Show more

Keywords: Hypoxic-ischemic brain injury, diffusion weighted imaging, ischemia, neurological outcome

DOI: 10.3233/JPN-2010-0397

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 231-235, 2010

Authors: Mandal, Subinay | Hembram, Debasish

Article Type: Research Article

Abstract: Caudal dysgenesis constitutes a heterogeneous spectrum of congenital caudal anomalies, including varying degrees of developmental defects of the vertebral column, as well as anorectal and genitourinary anomalies. Herein, we are reporting a case of caudal regression syndrome with imperforate anus, bilateral renal hypoplasia, vestigial tail and esophageal duplication cyst in a baby of non-diabetic mother, with history of non-consanguineous marriage.

Keywords: Caudal regression syndrome, congenital anomaly, sirenomelia, vestigial tail

DOI: 10.3233/JPN-2010-0396

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 237-240, 2010

Authors: Sharma, Sanjay | Nathani, Inder | Sahu, Chandradev

Article Type: Research Article

DOI: 10.3233/JPN-2010-0318

Citation: Journal of Pediatric Neurology, vol. 8, no. 2, pp. 241-242, 2010