Journal of Pediatric Neurology - Volume 6, issue 3

Authors: Mahajan, Vikas | Singhi, Pratibha | Khandelwal, N.

Article Type: Research Article

Abstract: Goldenhar syndrome is a rare disorder characterized by a wide spectrum of physical features particularly craniofacial. We report an infant with developmental delay, and seizures who had features of Goldenhar syndrome and had an arachnoid cyst. Though the syndrome itself is not very rare, the presence of arachnoid cyst and hydrocephalus prompted us to report this case. Arachnoid cyst relates to developmental errors in the neural crest and thus presence of arachnoid cyst in the index …case supports the argument of neural migration defect in the pathogenesis in Goldenhar syndrome. Only one such case in the world literature has been reported earlier. Show more

Keywords: Goldenhar syndrome, arachnoid cyst, hydrocephalus

Citation: Journal of Pediatric Neurology, vol. 6, no. 3, pp. 261-264, 2008

Authors: Kumar, Raj | Kalra, Samir Kumar | Das, Nitu Kumar | Poddar, Banani | Mahapatra, Ashok Kumar

Article Type: Research Article

Abstract: Grisel's syndrome is a rare condition characterized by atlantoaxial subluxation following an inflammatory process in the head and neck region. It occurs more commonly in children and usually presents with cervical pain and torticollis, in addition to symptoms of the primary infection. A case of Grisel's syndrome is discussed here who suddenly developed quadriplegia with severe respiratory compromise and ultimately diagnosed as reducible atlantoaxial subluxation with hematomyelia. Hematomyelia was extending from medulla …to the third cervical vertebrae level of cord. The case was managed successfully by posterior fusion. Though the clinical improvement occurred following posterior fusion, but there was no optimum radiological reduction of the reducible atlantoaxial subluxation. This case emphasizes the importance of posterior fusion in Grisel's syndrome where an offending posterior indentation of upper cervical cord by dislocated posterior arch, if dealt with; the clinical improvement can be achieved even without sufficient anterior reduction of subluxation. The reasons of hematomyelia and nonreduction atlantoaxial subluxation are discussed here. Show more

Keywords: Grisel's syndrome, hematomyelia, atlantoaxial subluxation, posterior fusion

Citation: Journal of Pediatric Neurology, vol. 6, no. 3, pp. 265-268, 2008

Authors: Kumar, Raj | Vaıd, Vivek Kumar | Kalra, Samir Kumar | Mahapatra, Ashok Kumar

Article Type: Research Article

Abstract: Situs inversus (also called situs transversus) is a rare congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The association of spinal dyraphism with situs inversus totalis is rare and only few cases have been reported till date. The authors report an infant who presented with meningomyelocele, with split cord malformation type-1 and situs inversus totalis. It is hypothesized that defects of the midline and laterality defects (e.g., situs …inversus) are etiologically related and determining genetic locus or implicating various chemical agents that are known to produce situs inversus independently, as the causative factor for spinal dysraphism would further enhance our understanding about the pathogenesis of the above conditions and hence help in better management. A thorough physical examination supplemented by imaging studies of the entire neuraxis with a high index of suspicion for associated developmental abnormalities in a patient with congenital spinal abnormality is necessary. Show more

Keywords: Situs inversus totalis, split cord malformation, meningomyelocele

Citation: Journal of Pediatric Neurology, vol. 6, no. 3, pp. 269-272, 2008

Authors: Mahjoubi, Frouzandeh | Akbari, Mohammad Taghi | Babamohammadi, Gholamreza

Article Type: Research Article

Abstract: Here we describe clinical and cytogenetic data on 2-year-old female child with partial trisomy for the distal part of the long arm of chromosome 10 (10q22–>qter) and a concomitant monosomy 3(p25–>pter) as a result of a maternal balanced reciprocal translocation. Her karyotype was ascertained as 46,XX,der(3)t(3;10)(p25;q22). The father had normal karyotype. The mother had an apparently balanced translocation involving chromosome 3 and 10 [46,XX,t(3;10)(p25;q22)]. This is the second reported case of partial …trisomy 10q and partial trisomy 3p. Clinical features of this case and a few published cases will be reviewed briefly. Show more

Keywords: Trisomy 10q, monosomy 3p

Citation: Journal of Pediatric Neurology, vol. 6, no. 3, pp. 273-276, 2008

Authors: Agarwal, Arun L. | Biswas, Devdutta | Agrawal, Sanjay

Article Type: Research Article

Abstract: Viral encephalitis is often followed by cognitive sequelae including intellectual decline whose pathophysiology remains obscure till date. Based on recent laboratory studies implicating the cholinergic system in viral encephalitis, the authors postulate the hypothesis that cholinergic deficit might account for some of the cognitive sequelae in at least some types of viral encephalitis, which might be benefited by central acetyl cholinesterase inhibitors. The case of an adolescent with postencephalitic intellectual decline responding …robustly to donepezil is presented. Show more

Keywords: Donepezil, central acetylcholinesterase inhibitors, encephalitis, mental retardation

Citation: Journal of Pediatric Neurology, vol. 6, no. 3, pp. 277-281, 2008

Authors: Idris, Badrisyah | Theophilus, Sharon Casilda | Muthu, Nyanaveelan | Hup, Chan Kin | Abdullah, Jafri Malin | Tharakan, John

Article Type: Research Article

Abstract: Stroke resulting from traumatic brain injury in children is uncommon. Three such cases are reported here, to bring this entity to attention and to highlight their good prognosis. Possible mechanisms that may lead to stroke in children following head trauma are discussed No other causes of stroke were identified, and magnetic resonance angiography and Doppler ultrasound ruled out injury to the carotid vessels in all three cases.

Keywords: Children, stroke, head injury

Citation: Journal of Pediatric Neurology, vol. 6, no. 3, pp. 283-285, 2008

Authors: Kumar, Raj | Singhal, Namit | Sahu, Rabi Narayan | Kalra, Samir Kumar | Sharma, Kumudini | Mahapatra, Ashok Kumar

Article Type: Research Article

Abstract: Orbital abscess following trivial blunt trauma to the eye are uncommon. Few cases are known and reported in the literature. The clinical presentation can be myriad varying from localized presentation to children with marked systemic toxicity. Visual loss is the most dreaded complication and a rapid deterioration in vision may occur if the treatment is delayed. Radiological investigations usually offer a prompt diagnosis but considerable degree of confusion may occur, especially in those cases that lack …systemic manifestations of infective pathology. Aggressive medical and surgical treatment is mandatory for a favorable outcome in these cases. We report the case of a child who presented to us with orbital abscess of the left eye following a trivial blunt trauma. No other infective focus was identified. Subsequent imaging revealed abscess of the superior rectus muscle. The child improved following intravenous antibiotics and ultrasonography guided aspiration of the abscess. The clinical and radiological presentation of such cases, management and outcome and a brief review of literature are discussed. Show more

Keywords: Orbital abscess, superior rectus, blunt trauma, non-axial proptosis, visual loss

Citation: Journal of Pediatric Neurology, vol. 6, no. 3, pp. 287-292, 2008