Posterior fossa neoplasm and PHACES syndrome: a case report.

A 4-year-old girl with PHACES syndrome (posterior fossa brain malformations, hemangiomas, arterial anomalies, cardiac anomalies/coarctation of the aorta, eye abnormalities, and sternal clefting/supraumbilical raphe) developed a cerebellar pilocytic astrocytoma 18 months after resolution of her neck, ear, and thoracic hemangiomas. Because cutaneous hemangiomas may have involuted by the time a patient is diagnosed with a central nervous system neoplasm, it seems possible that in other such patients the association may have gone unrecognized. Cerebellar pilocytic astrocytoma may be a rare manifestation of the posterior fossa malformations of PHACES.

Suppression of pilocarpine-induced ictal oscillations in the hippocampal slice.

Activation of muscarinic cholinergic receptors produces oscillations in the hippocampal slice that resemble the theta rhythm, but also may produce abnormal synchronous activity that is more characteristic of epileptiform activity. We used pilocarpine, a muscarinic agonist and convulsant, and an elevation in extracellular potassium (5-7.5 mM) to produce synchronous neuronal activity that was prolonged (>2 s) and mimicked synchronization noted during seizures in vivo (ictal activity). In the CA3 region of adult rat hippocampal slices, prolonged ictal oscillations consisted of rhythmic field potentials occurring at 4-10 Hz for up to 30 s (ictal duration) that occurred in a regular periodic pattern every 12-166 s (ictal interval). The duration and interval between ictal oscillations were measured before and after application of drugs to define determinants of ictal occurrence. High threshold calcium channel antagonists (nifedipine and verapamil) blocked ictal activity. Release of calcium from intracellular stores also appeared to be important for ictal synchronization because ictal activity was blocked by dantrolene, an inhibitor of calcium release from intracellular stores, and by thapsigargin which blocks the ATPase that maintains intracellular calcium stores. These suppressive effects appeared to be postsynaptic because nifedipine, dantrolene, and thapsigargin had no effect on evoked fEPSPs. Enhancement of presynaptic inhibition by activation of GABA(B) or adenosine A(1) receptors suppressed ictal activity and depressed the amplitude of evoked population synaptic potentials. The results point to an important role for high threshold calcium channels and release of calcium from intracellular stores in addition to strength of synaptic connections in generation of prolonged oscillations that underlie seizure activity.

Surgery for hemispheric malformations of cortical development.

The treatment of patients with hemispheric malformations of cortical development presents a challenging clinical problem that often requires surgical intervention. The development of a safe and effective treatment strategy for these patients requires an extensive work-up with a great deal of attention to a variety of multidisciplinary testing methods. In this article, the authors describe the presurgical evaluation of patients with hemispheric malformations of cortical development and epilepsy and review the surgical techniques currently available. They also detail some of the controversial issues regarding surgical treatment of these patients.

Cost effectiveness of a clinical pharmacist on a neurosurgical team.

OBJECTIVE: In 1999, the Society of Critical Care Medicine formally recognized that pharmacists were essential for the provision of high quality care to the critically ill population. This study is a brief quantitative analysis of the benefit provided by a clinical pharmacist in a multidisciplinary neurosurgical setting. METHODS: Patients admitted to the neurosurgical service in the 2 years before and 2 years after the implementation of dedicated neurosurgical pharmacy services were retrospectively reviewed. The clinical pharmacist was responsible for monitoring and evaluating all adult patients on the service and rounding with the team 6 days a week. RESULTS: A total of 2156 patients were admitted during the study period. No significant differences were noted among severity of illness scores between the 2 groups. During this time, 11 250 interventions were recorded by the pharmacist. The average pharmacy and intravenous therapy cost per patient between the pre- and postimplementation groups decreased from $4833 to $3239, resulting in a total savings of $1,718,260 over the duration of the study period. The average hospital stay decreased from 8.56 to 7.24 days (P = 0.003). Early hospital mortality also decreased from 3.34% to 1.95% (P = 0.06). For those patients who were discharged from the hospital, there was a significant decrease in readmission rates between the 2 groups (P < 0.05) CONCLUSION: Having a dedicated clinical pharmacist with critical care training rounding routinely with a neurosurgical team significantly reduced hospital stay, readmission rates, and pharmacy costs. Clinical pharmacists can have a significant effect on clinical and economic measures in the intensive care unit, and their participation on a multidisciplinary critical care team should be a standard of care.

Relative adrenal insufficiency in patients with acute spinal cord injury.

INTRODUCTION: Relative adrenal insufficiency has been shown to occur in the settings of critical illness and septic shock, impairing the body's ability to respond to stress. Studies have demonstrated that the treatment of adrenal insufficiency (AI) results in shock reversal, hemodynamic stability, and a subsequent decrease in mortality. Endocrine changes and AI have been reported in patients with spinal cord injuries during their extended courses of rehabilitation. DISCUSSION: We describe two cases of patients with cervical spine injuries who presented with acute adrenal insufficiency following their injuries. With the addition of low-dose corticosteroids, each patient had symptom resolution and demonstrated clinical improvement. CONCLUSION: Patients with spinal cord injuries are at risk for AI, both in the acute and chronic settings following injury prompting the need for an increased awareness of this condition. Although variability exists in the exact criteria for the diagnosis of AI, the combination of clinical symptoms, depressed serum cortisol concentrations, and responsiveness to exogenous steroid therapy should all contribute to the diagnosis of this condition.