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Biomedical subjects

Rajesh RamachandranNair

Publications and source records attributed to Rajesh RamachandranNair.

8 recordsLinked to original sources

Mirror movements following cortical resection of polymicrogyria in a child with intractable epilepsy.

Mirror movements may be congenital or acquired. There are few reports of acquired mirror movements in pediatric patients. Further, mirror movements in children with epilepsy have rarely been reported. A 9-year old male, with intractable partial epilepsy resulting from polymicrogyria of the right hemisphere, underwent cortical resection of the right frontotemporoparietal region for a malformation of cortical development. He developed left hemiplegia and mirror movements in the left hand in the postoperative period. Four months after surgery, he remained seizure-free with mild residual left-sided hemiplegia and persistent mirror movements. Mechanisms postulated for mirror movements include aberrant pyramidal tract development and transcallosal inhibitory pathways. The latter mechanism might have contributed to the mirror movements observed in this child. This study is the first report of mirror movements following focal cortical resection for intractable epilepsy due to polymicrogyria.

Cerebral Cortex↗

Recurrent de novo mutations of SCN1A in severe myoclonic epilepsy of infancy.

Mutations in the voltage-gated sodium channel gene SCN1A are a major cause of severe myoclonic epilepsy of infancy (Dravet syndrome) and generalized epilepsy with febrile seizures plus. This study reports the identification of six de novo SCN1A mutations in patients with severe myoclonic epilepsy of infancy, including a tetranucleotide deletion in exon 26. The same deletion was previously observed in two unrelated patients and appears to result from slipped-strand mispairing of a direct repeat during deoxyribonucleic acid replication. Review of the literature indicates that recurrent mutations account for 25% of SCN1A mutations in severe myoclonic epilepsy of infancy, including six sites of deamination at CpG dinucleotides.

Adolescent↗

Incomplete alpha coma pattern in a child.

Only 19 cases of alpha coma pattern in the pediatric age group had been reported. Recently the concept of reactivity has been included in the definition of alpha coma. Electroencephalography reveals reactivity to painful stimuli in incomplete alpha coma. In adults, this pattern has a better prognosis than the complete pattern with no reactivity. A 5-year-old child had coma secondary to hypoxic brain damage after surgery. His electroencephalogram documented an incomplete alpha coma pattern. He remained in a vegetative state. This study is the first report of a child with incomplete alpha pattern documented on electroencephalogram.

Alpha Rhythm↗

A reappraisal of rhythmic coma patterns in children.

OBJECTIVE: This study was designed to determine the prevalence of rhythmic coma patterns in comatose children and to ascertain the prognostic significance of reactive rhythmic coma patterns. METHODS: We retrospectively analyzed and classified electroencephalogram (EEGs) in comatose children between two months and 18 years of age during the period 1996 - 2003 according to modified Young's classification. Outcome at one-year was scored according to the Paediatric Cerebral and Overall Performance Category Scale. Outcomes were compared using Fisher's exact test and Mann-Whitney test. RESULTS: Analysis of 63 electroencephalogram (EEG) records in 38 patients showed rhythmic patterns in 19 records (30.2%; 9 alpha, 4 spindle, 4 theta and 2 beta coma patterns, total number of children = 14). Aetiology and outcome of alpha coma patterns and other rhythmic coma patterns were similar. In five children, one type of rhythmic pattern changed to another. Records with reactive rhythmic coma 66.7% (6/9), were associated with favourable outcome. Sixty percent of the records (6/10 records in seven children) with non-reactive pattern were associated with unfavourable outcome. This clinically significant difference did not reach statistical significance (lower Paediatric Cerebral and Overall Performance Category Scale score p= 0.14; favourable outcome p=0. 19). CONCLUSION: Rhythmic coma patterns in comatose children are not uncommon. Aetiology, reactivity and outcome of individual patterns are similar and thus make the rhythmic coma patterns distinct EEG signatures in comatose children. There was a clinically significant better outcome with reactive rhythmic coma patterns.

Adolescent↗

Childhood acute disseminated encephalomyelitis presenting as Foix-Chavany-Marie syndrome.

A 10-year-old boy presented with anarthria and bilateral central faciolinguovelopharyngeomasticatory paralysis with "automatic voluntary dissociation" suggestive of Foix-Chavany-Marie syndrome or anterior opercular syndrome following a nonspecific upper respiratory infection. Brain magnetic resonance imaging revealed bilateral subcortical perisylvian demyelination in addition to multiple subcortical white-matter demyelination. The clinicoradiologic picture suggested acute disseminated encephalomyelitis. The child recovered completely following intravenous methylprednisolone and intravenous immunoglobulin therapy. This is the first report of anterior opercular syndrome in childhood acute disseminated encephalomyelitis.

Child↗

Plasmapheresis in childhood acute disseminated encephalomyelitis.

Accepted modes of therapy in acute disseminated encephalomyelitis include intravenous methylprednisolone, intravenous immunoglobulin or a combination of both. Effectiveness of plasmapheresis has been demonstrated by many case reports. We used plasmapheresis to treat steroid non-responsive acute disseminated encephalomyelitis in two children. There was complete clinical and radiological recovery in both the cases.

Adolescent↗

Partial seizures triggering infantile spasms in the presence of a basal ganglia glioma.

Infantile spasms associated with brain tumors have been reported. A focal cortical lesion can induce infantile spasms by triggering the brainstem and basal ganglia in this vulnerable age group. We report the case of a female infant with a low-grade glioma in the right basal ganglia, spreading to the cortical area. She presented at the age of five months with left hemiparesis and partial seizures. She developed infantile spasms at the age of 12 months. This is the first video clip report of partial seizures triggering symmetrical spasms in series, secondary to a basal ganglia glioma extending to the cortex. [Published with video sequences].

Anticonvulsants↗