Journal of Pediatric Neurology - Volume 8, issue 4

Authors: Hussain, Nahin | Pugalenthi, Arun | Gosalakkal, Jayaprakash

Article Type: Research Article

Abstract: Cerebral sinovenous thrombosis (CSVT) is a rare and under-diagnosed complication of ulcerative colitis. We report a case of a 14-year-old male child, who presented with a history of worsening headaches, vomiting and decreased level of consciousness. He was diagnosed to have CSVT with infarction on magnetic resonance imaging of the brain. He was treated with low molecular weight heparin and discharged home with a mild right-sided hemiparesis. At a year follow-up, his neurological examination was normal. …The use of antithrombotic therapy, in particular low molecular weight heparin can be effective in reducing the mortality and morbidity associated with CSVT. Nevertheless, randomized placebo-controlled studies are necessary for assessing its efficacy and absence of risk in children. Show more

Keywords: Ulcerative colitis, cerebral sinovenous thrombosis

DOI: 10.3233/JPN-2010-0422

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 403-406, 2010

Authors: Ruiz-Juretschke, Fernando | Hinojosa, José | Muñoz, Alberto | Martinez-Anton, Amalia | Esparza, Javier

Article Type: Research Article

Abstract: The Currarino triad is a rare hereditary caudal malformation complex consisting of sacral bone defect, anorectal malformation and presacral mass, which is transmitted in an autosomal dominant pattern with variable phenotypic expression. We report a case of complete Currarino triad with anterior meningocele that was studied with intrathecal gadolinium-diethylene triamine pentaacetic acid magnetic resonance imaging, a novel technique that may be useful for surgical decision and planning in disorders involving the cerebrospinal …fluid pathways. Show more

Keywords: Currarino triad, anterior myelomeningocele, intrathecal gadolinium, tethered cord

DOI: 10.3233/JPN-2010-0425

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 407-410, 2010

Authors: Akopova-Larbi, Rosana | Turki, Ilhem Ben Youssef | Kraoua, Ichraf | Gouider-Khouja, Neziha

Article Type: Research Article

Abstract: Aicardi-Goutières syndrome (AGS) is a rare infantile encephalopathy characterized by basal ganglia calcifications, leukodystrophy, chronic pleocytosis and elevated levels of interferon-alpha in cerebrospinal fluid. AGS is a clinically and genetically heterogeneous syndrome. We report an early onset case of AGS. Consanguinity, similar picture in the family and course suggested a familial metabolic encephalopathy. Extended basal ganglia calcifications and leukodystrophic changes on cerebral imaging, cerebrospinal fluid lymphocytosis and high interferon-alpha level allowed to …diagnose AGS. We reviewed the literature and discussed the pathophysiology, differential diagnosis and treatment of this disorder. Show more

Keywords: Infantile encephalopathy, microcephaly, basal ganglia calcifications, leukodystrophy, CSF lymphocytosis

DOI: 10.3233/JPN-2010-0420

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 411-415, 2010

Authors: Kalane, Umesh Dinkar | Sawant, Sangeeta | Shilpa, Amin Alpa, Sule | Tanuja, Karande | Kumar, Santosh

Article Type: Research Article

Abstract: Morning glory disc anomaly is a rare condition of the optic disc coloboma characterized by an enlarged, excavated, funnel shaped disc with an elevated rim, resembling a morning glory flower. Morning glory syndrome consists of morning glory disc anomaly with Basal encephaloceles, with other midline anomalies such as hypertelorism, broad nasal root, cleft lip, and cleft palate and short stature due to hypopituitarism. Morning glory syndrome is very rarely described to be associated with the vascular …anomalies. We report a case of morning glory syndrome associated with the hypoplasia of the left common carotid artery throughout its course, which is rarely described in the literature. Show more

Keywords: Morning glory disc, morning glory syndrome, basal encephalocele

DOI: 10.3233/JPN-2010-0426

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 417-420, 2010

Authors: Pavlou, Evangelos | Hatzistilianou, Maria | Stamou, Maria | Fidani, Liana | Charitandi, Afrodites | Athanasiadou, Fani

Article Type: Research Article

Abstract: An 11-year-old boy with Henoch-Schonlein purpura complicated by hypertension-induced encephalopathy is reported. Steroid therapy (oral prednisolone 2 mg/kg per day) was started immediately after diagnosis because of gastrointestinal involvement. A few days later the patient developed hypertension followed by generalized tonic-clonic seizures. A peripheral lesion in the posterior gray and white matter was seen on magnetic resonance imaging. The nature and location of the lesions and the normalization of the patient's magnetic …resonance imaging reported a few months later was consistent with a posterior predominant parieto-occipital encephalopathy described in the literature as posterior reversible encephalopathy syndrome. The occurrence of this very rare syndrome in childhood requires close monitoring and normalization of blood pressure in patients in order to prevent central nervous system manifestations. Show more

Keywords: Henoch-Schonlein purpura, childhood, posterior encephalopathy

DOI: 10.3233/JPN-2010-0424

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 421-424, 2010

Authors: Karim, Nayeem | Slack, Gregory | Morse, Richard P.

Article Type: Research Article

Abstract: Epileptic seizures are generally considered a cortically based phenomenon, with motor expression mediated through the corticospinal tracts, yet half of patients with hydranencephaly have seizures. The mechanisms mediating expression of seizures in hydranencephalic infants have not been well studied, but they are assumed to reflect subcortical networks or represent non-epileptic brainstem release phenomena. Children with hydranencephaly thus provide a useful model for studying the clinical features of subcortically mediated seizures. We studied …two infants with hydranencephaly using video-electroencephalogram in order to determine if the seizures arose from remnants of cortical tissue or subcortical structures, and to analyze features of seizures of subcortical origin. Show more

Keywords: Hydranencephaly, brain stem release phenomena, seizures, epilepsy, subcortical networks, audiogenic seizures

DOI: 10.3233/JPN-2010-0427

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 425-431, 2010

Authors: Abdwani, Reem | Koul, Roshan | Al Azri, Faisal | AL-Futaisi, Amna

Article Type: Research Article

Abstract: We report a 10-year-old girl with juvenile dermatomyositis who presented with generalized anasarca, an unusual initial manifestation, rarely reported. Anasarca has been linked with severity of the disease activity in some reports. The patient was treated with pulse methylprednisolone, methotrexate and intravenous immunoglobulin. Disease activity was completely controlled within 3 months of therapy. It is crucial for pediatricians to recognize generalized edema as a presenting feature of juvenile dermatomyositis in the absence …of the other clinical and laboratory features of other common causes of anasarca. We advocate early diagnosis and prompt aggressive treatment to decrease morbidity. Show more

Keywords: Juvenile dermatomyositis, anasarca, edema

DOI: 10.3233/JPN-2010-0421

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 433-435, 2010

Authors: Maller, Vinod G. | Chowdhury, Veena | Singh, Sapna | Kottiyath, Vivek C. | Khandelwal, Girish | Jain, Rajat

Article Type: Research Article

DOI: 10.3233/JPN-2010-0423

Citation: Journal of Pediatric Neurology, vol. 8, no. 4, pp. 437-439, 2010