Journal of Pediatric Neurology - Volume 11, issue 2

Authors: Das, Suman | Sarkar, Nirmalya | Chatterjee, Rita | Chatterjee, Kaushani | Chatterjee, Anish

Article Type: Research Article

Abstract: Chronic inflammatory demyelinating polyneuropathy is an immune mediated disorder of more than 2 mo duration. A 5-year-old male child presented with subacute onset of ascending weakness, hyporeflexia and respiratory insufficiency requiring mechanical ventilation. He had suffered from an exactly similar episode 1 yr back. Cerebrospinal fluid showed albuminocytological dissociation. Nerve conduction velocity study showed combined demyelinating and axonal type of polyradiculoneuropathy. Sural nerve biopsy revealed demyelination and axonal degeneration. The child responded …well to intravenous immunoglobulin in the acute phase and prednisolone was used to maintain the child in remission. Show more

Keywords: Chronic inflammatory demyelinating polyradiculoneuropathy, Guillian-Barre syndrome, demyelination, intravenous immunoglobulin

DOI: 10.3233/JPN-130609

Citation: Journal of Pediatric Neurology, vol. 11, no. 2, pp. 127-130, 2013

Authors: Carbunar, Olimpia | Sinsioco, Claudine | Bicknese, Alma

Article Type: Research Article

Abstract: Spinal cord infarction is rare in children. Most described cases are in association with aortic surgery or arterial hypotension. In children, infarction can be confused with the more common disorder transverse myelitis as the magnetic resonance imaging findings and clinical presentation are often similar. We describe here a case of spontaneous spinal cord infarct initially treated as transverse myelitis.

Keywords: Spinal cord infarction, cerebrovascular disease, childhood stroke, transverse myelitis

DOI: 10.3233/JPN-130610

Citation: Journal of Pediatric Neurology, vol. 11, no. 2, pp. 131-133, 2013

Authors: Balasubramaniam, Shanti | Salomons, Gajja S. | Blom, Henk J.

Article Type: Research Article

Abstract: Methylenetetrahydrofolate reductase (MTHFR) is a key regulatory enzyme in the remethylation of homocysteine to methionine. S-adenosylmethionine, formed from methionine and adenosine triphosphate, is the methyl donor in crucial reactions for brain development and function. MTHFR deficiency is the most prevalent inborn error of folate metabolism and is characterized by hyperhomocysteinemia and hypomethioninemia. A broad clinical spectrum includes psychomotor retardation, microcephaly, seizures, central respiratory failure and death. Premature atherosclerosis, arterial and venous …thrombosis or psychiatric manifestations occur in the late onset forms. We report a female neonate with severe MTHFR deficiency presenting with neonatal encephalopathy, congenital microcephaly, seizures, central hypoventilation with gross cerebral atrophy, hypomyelination and pontine hypoplasia on magnetic resonance imaging of brain. She succumbed at 10 wk of age despite treatment with oral S-adenosylmethionine, betaine, folinic acid, riboflavin and hydroxycobalamin. Show more

Keywords: MTHFR deficiency, remethylation of homocysteine, severe neurological impairment, microcephaly, seizures, central hypoventilation

DOI: 10.3233/JPN-130611

Citation: Journal of Pediatric Neurology, vol. 11, no. 2, pp. 135-140, 2013

Authors: Patnaik, Sibabratta | Mohapatra, Manoranjan | Satpathy, Dina K. | Das, Swarnalata | Mohanty, Aswini K.

Article Type: Research Article

Abstract: An 8-month-old boy presented with high fever with seizures and weakness of legs. Cerebrospinal fluid analysis revealed acute pyogenic meningitis and magnetic resonance imaging revealed anterior sacral meningocele with epidural abscess. He was managed by surgery through posterior approach along with antibiotics. Clinical presentation, differential diagnosis, diagnostic modalities and management of this rare clinical condition is briefly discussed in this article.

Keywords: Anterior sacral meningocele, epidural abscess, pre-sacral space, Currarino triad, staphylococcal meningitis

DOI: 10.3233/JPN-130612

Citation: Journal of Pediatric Neurology, vol. 11, no. 2, pp. 141-145, 2013

Article Type: Correction

DOI: 10.3233/JPN-130613

Citation: Journal of Pediatric Neurology, vol. 11, no. 2, pp. 147-147, 2013