Journal of child neurology
-
Implantation of an intrathecal baclofen pump is recommended for children with cerebral palsy as a means to improve care and comfort when other options fail to control severe hypertonia. Making an assessment of a child's spasticity-related limitations in both routine care and activity is a necessary component of selection of intrathecal baclofen candidates. ⋯ Questionnaires completed by caregivers and patients at a pediatric physiatry spasticity clinic over an 11-year period were reviewed to evaluate whether the Rehabilitation Institute of Chicago Care and Comfort Caregiver Questionnaire captured improved caregiving and comfort of children with cerebral palsy and severe spasticity following intrathecal baclofen pump implantation. The Questionnaire scores showed improvement after intrathecal baclofen pump implantation, consistent with subjective reports of patient and caregiver satisfaction.
-
Review Case Reports
Fatal outcome in hemiconvulsion-hemiplegia syndrome.
Hemiplegia-hemiconvulsion-epilepsy syndrome is characterized by prolonged unilateral clonic seizures in a child followed by the development of hemiplegia. Focal status epilepticus results in unilateral cerebral edema of the epileptic hemisphere in the acute phase followed by cerebral hemiatrophy. Literature in the last 5 years does not describe malignant cerebral edema or resultant death. ⋯ The first indication of worsening of clinical status after being seizure free was voltage suppression on continuous electroencephalography (EEG). We describe neuroimaging, EEG findings, and neuropathologic findings at autopsy and review pertinent literature. We also evaluate the evolving role of continuous EEG monitoring in the pediatric intensive care unit.
-
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation syndrome (ROHHADS) is a rare, but potentially lethal, pediatric disorder. To date, nearly 80 patients have been reported in the literature; however, the etiopathogenesis is still unclear and debated. ⋯ Aiming to establish the immune-mediated pathogenesis, we report 2 children with a clinical picture consistent with ROHHADS and whose cerebrospinal fluid analysis disclosed an intrathecal synthesis of oligoclonal bands. Even if many aspects remain to be explained, this finding suggests that ROHHADS could share similar pathogenetic mechanisms with other immune-mediated central nervous system disorders, and even more important, it might pave the way to a therapeutic chance for these patients by means of immunotherapy.