Pediatric neurology
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Pediatric neurology · Oct 2007
Case ReportsAcute dyskinesia on starting methylphenidate after risperidone withdrawal.
We report on acute and transient dyskinesia occurring within hours of taking modified-release methylphenidate (Concerta XL) in a stimulant-naïve 7-year-old boy who had recently stopped taking risperidone. We propose that the mechanism of this adverse drug reaction is likely to be an interaction between supersensitive dopamine receptors and acute exposure to an indirect dopamine agonist. Clinicians need to be aware of this potential side effect, and to use the combination of psychostimulants and neuroleptic drugs with caution.
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Pediatric neurology · Jul 2007
Pediatric epilepsy surgery in focal lesions and generalized electroencephalogram abnormalities.
Generalized abnormalities on scalp electroencephalograms (EEG) are not uncommon in children with partial epilepsy in whom a dominant focus of interictal and ictal abnormalities concordant to the brain lesion usually clarifies surgical candidacy. Children with exclusively generalized or multiregional EEG abnormalities and mental retardation are usually not considered surgical candidates, even when brain lesions are seen on imaging. Of 176 pediatric epilepsy surgeries at our center, we describe 10 children with exclusively generalized and multiregional interictal and ictal EEG abnormalities who had resection of a focal lesion seen on brain MRI. ⋯ We conclude that generalized and multiregional EEG abnormalities in the absence of dominant focus may not preclude epilepsy surgery in children with a congenital or acquired lesion seen on MRI. Generalized EEG abnormalities are likely secondary phenomena that resolve after surgery. Maladaptive neural plasticity and secondary epileptogenesis are potential mechanisms that mask an epileptogenic lesion with generalized EEG abnormalities.
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Pediatric neurology · Jun 2007
Infantile onset of hereditary spastic paraplegia poorly predicts the genotype.
Age of symptom onset of hereditary spastic paraplegia varies from infancy to the eighth decade. Infantile onset of hereditary spastic paraplegia without a positive family history may cause difficulties in reaching the correct diagnosis and misdiagnosis as a diplegic form of cerebral palsy is particularly common. Infantile onset of hereditary spastic paraplegia caused by mutations in the spastin gene (SPAST) is very rare and previously was mostly associated with codominant mutations in this gene. ⋯ Several family members were previously diagnosed as having cerebral palsy. Infantile onset of hereditary spastic paraplegia may be caused by mutations in multiple genes, and this phenotype does not reliably predict the genotype. Pediatric neurologists need to be aware of relatively frequent de novo mutations in hereditary spastic paraplegia genes and a possibility that this condition presents in infancy without a positive family history.
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Pediatric neurology · May 2007
Phase I trial of pirfenidone in children with neurofibromatosis 1 and plexiform neurofibromas.
We aimed to define the dose of pirfenidone in children and adolescents with neurofibromatosis 1 and plexiform neurofibromas that is pharmacokinetically comparable to the active adult dose. Pirfenidone was administered orally on a continuous dosing schedule. The starting dose level was 250 mg/m2/dose. ⋯ Dose-limiting toxicities were observed in 2 of 12 patients receiving 500 mg/m2 three times a day. The plasma pharmacokinetics of pirfenidone were highly variable, but not age dependent. The second dose level was the pharmacokinetically comparable dose and is being used in an ongoing phase II trial of pirfenidone for children with neurofibromatosis 1 and progressive plexiform neurofibroma.
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Pediatric neurology · May 2007
Case ReportsLupus anticoagulant and thrombosis following Henoch-Schonlein purpura.
A male adolescent developed a sinovenous thrombosis 4 weeks following a Henoch-Schonlein purpura episode. A hypercoagulation evaluation revealed a positive lupus anticoagulant. This suggests an association between Henoch-Schonlein purpura and antiphospholipid antibody syndrome and is the first report of sinovenous thrombosis after Henoch-Schonlein purpura that was likely due to elevated antiphospholipids. Children who develop Henoch-Schonlein purpura with neurologic features including headache should be evaluated for sinovenous thrombosis and a hypercoagulable state.