Use of gabapentin in the treatment of childhood reflex sympathetic dystrophy.

Reflex sympathetic dystrophy, a painful syndrome involving an extremity after trauma or injury, is increasingly reported in the pediatric population. Although no clear pathophysiologic mechanism for this disorder has been identified, the role of central serotonin activity seems important. Gabapentin, a new antiepileptic medication, has been demonstrated to be effective in adults with reflex sympathetic dystrophy. The first reported case of a child with a diagnosis of reflex sympathetic dystrophy who was treated successfully with gabapentin is presented.

Gabapentin for self-injurious behavior in Lesch-Nyhan syndrome.

Self-injurious behavior is a common clinical problem in children with Lesch-Nyhan syndrome, an X-linked disorder of purine metabolism. This behavior is not observed in other conditions associated with increased serum concentrations of uric acid, hypoxanthine, and xanthine. Various neurotransmitters appear to play a pivotal role in self-injurious behavior. The authors present a patient with Lesch-Nyhan syndrome, whose self-injurious behavior was effectively treated with gabapentin, and discuss possible mechanisms of action.

Hydrocephalus as a possible early symptom in a child with a spinal cord tumor.

Spinal cord tumors are relatively uncommon in children. These tumors have been associated with increased intracranial pressure in both children and adults. An infant presented initially with hydrocephalus and subsequently developed symptoms consistent with spinal cord abnormalities. Various proposed etiologies for increased intracranial pressure in spinal cord tumors are presented. Spinal cord tumors should be considered in the presence of childhood hydrocephalus and increased intracranial pressure without a clearly defined etiology.

MRI findings in a patient with partial monosomy 10p.

Partial monosomy 10p is a rare chromosomal disorder characterised by frontal bossing, micrognathia, congenital heart defects, vesicoureteral abnormalities, and developmental delay. This is the first report to describe seizures not associated with hypocalcaemia, as well as cortical atrophy and decreased white matter volume on magnetic resonance imaging, in a patient with documented partial monosomy 10p. The neuroradiographic abnormalities found in this patient provide a first step towards understanding the aetiology of the developmental delay and ventriculomegaly associated with this chromosomal abnormality.

Breath-holding spells in a patient with transient erythroblastopenia of childhood.

We describe a child with transient erythroblastopenia of childhood and breath-holding spells. The spells resolved after oral iron supplementation but before the anemia resolved. The physiologic role of iron in autonomic nervous system regulation and its potential role in the treatment of breath-holding spells are discussed.

Protein C and protein S activity in sickle cell disease and stroke.

Stroke is a significant complication of sickle cell anemia in the pediatric population. The pathophysiology of stroke in sickle cell anemia remains unclear. Protein C and protein S activities were measured in children with sickle cell anemia and stroke, and compared to those with sickle cell anemia who were neurologically normal. Results showed significantly decreased levels of both protein C and protein S activities in children with sickle cell anemia who have had a stroke. This pilot study suggests that a possible coagulopathic state in children with sickle cell anemia may be associated with an increased risk for cerebrovascular disease. Further research in this area is indicated.

Neutropenia in a patient receiving intravenous immune globulin.

A child with Guillain-Barre syndrome treated with intravenous immune globulin (IVIG) developed neutropenia (absolute neutrophil count = 390), which resolved 3 days after completion of the therapy. Potential mechanisms for the development of neutropenia during the use of IVIG therapy are discussed. In this case, testing of the IVIG used revealed the presence of a high concentration of anti-neutrophil antibodies compared to other samples. It is recommended that white blood cell and neutrophil counts be monitored daily during the use of such therapy.

Stroke in purine nucleoside phosphorylase deficiency.

The first documented case of cerebrovascular disease occurring in a 13-year-old girl with purine nucleoside phosphorylase deficiency is reported. This patient, the oldest known survivor with purine nucleoside phosphorylase deficiency, had previously experienced multiple sequential neurologic problems. She presented with episodes of transient left hemiparesis, followed shortly thereafter by dense left hemiplegia. Magnetic resonance imaging revealed a right internal capsule infarct; cerebral angiography revealed vasculopathy of the proximal vessels. Proposed mechanisms for neurologic dysfunction and cerebrovascular disease in purine nucleoside phosphorylase deficiency are discussed.