Susan Beckwitt Turkel

Cerebellar mutism in children: Report of six cases and potential mechanisms ☆

Cerebellar mutism is a rare finding associated with resection of posterior fossa tumors or cerebellar hemorrhages. We reviewed the medical records of six children, aged 6 to 12 years, who developed cerebellar mutism after resection of a posterior fossa mass or as a result of posterior fossa trauma. From 1989 to 1994, 210 children underwent posterior fossa resection at our institution, and four developed mutism (an incidence of 1.6%). All four patients had primitive neuroectodermal tumors. The fifth patient experienced trauma, and another patient had an arteriovenous malformation (AVM). In four children, hydrocephalus developed as a result of their tumor or AVM. Four developed cerebellar mutism 24 to 48 hours after surgery or trauma, and one developed cerebellar mutism 5 days after surgery, coincident with hydrocephalus. In one, mutism occurred after a second resection was performed for a recurrence of his posterior fossa tumor. Cerebellar mutism lasted 10 days in one patient and 2 to 8 weeks in the other four. Dysarthria was apparent in four patients during the recovery phase. We suggest trauma to the dentate nucleus and/or its outflow tract, the superior cerebellar peduncle, as a cause of reversible mutism. Because posterior fossa tumors are common in children, mutism should be recognized as an important side effect of surgery.

Brain injury and intrauterine death

Many of the stresses contributing to brain injury in the intrapartum or neonatal period may also exist prenatally. To define this problem, we reviewed the clinical features and neuropathologic findings in 433 consecutive stillbirth autopsies. Twenty-five had evidence of periventricular/intraventricular hemorrhage or gliosis. Twenty infants had died in utero before active labor and five during delivery. Nine stillbirths were infected. Ten cases had periventricular/intraventricular hemorrhage alone, five had the hemorrhage with parenchymal hemorrhage, five had parenchymal hemorrhage only, and five had gliosis. This group of 25 cases establishes that intrauterine brain injury is not rare. Separation of antepartum events occurring in utero from those imposed during labor, delivery, resuscitation, and the neonatal period is very important and has significant medical and legal implications.

Case Series: Neuropsychiatric Symptoms With Pediatric Systemic Lupus Erythematosus

OBJECTIVE To describe the psychiatric presentation, serologic findings, and neuroimaging patterns in children and adolescents with central nervous system involvement with systemic lupus erythematosus (CNS-SLE). METHOD Pediatric patients with psychiatric symptoms who fulfilled the 1997 revised diagnostic American College of Rheumatology criteria for SLE were studied. Complement levels, and anti-double-stranded DNA, anti-Smith, anti-phospholipid, and anti-neuronal antibodies were evaluated. Computed tomography, magnetic resonance imaging, and single photon emission computed tomography (SPECT) neuroimaging studies were reviewed. RESULTS The 10 patients with CNS-SLE presented with psychosis, mood disturbance, or confusion, and 8 patients had concomitant neurologic symptoms. The 8 girls and 2 boys ranged in age from 7.5 to 17 years. Serum anti-neuronal antibodies were positive with onset of symptoms and declined with improvement. Initial SPECT was abnormal in all 10 patients and remained abnormal. CONCLUSION SPECT and anti-neuronal antibodies help confirm CNS involvement in patients with SLE and neuropsychiatric symptoms.

Atypical Antipsychotic Medications to Control Symptoms of Delirium in Children and Adolescents

BACKGROUND Atypical antipsychotics have been documented to be effective in the management of delirium in adults, but despite considerable need, their use has been less studied in pediatric patients. OBJECTIVE A retrospective chart review was done to describe the use of atypical antipsychotics in controlling symptoms of delirium in children and adolescents. METHODS Pharmacy records at Childrens Hospital Los Angeles were reviewed to identify patients to whom antipsychotic agents were dispensed over a 24-month period. Psychiatric inpatient consultations during the same 24-month period were reviewed. Patients 1-18 years old diagnosed with delirium given antipsychotics constituted the study population. Delirium Rating Scale-Revised-98 (DRS-R98) scores were retrospectively calculated, when possible, at time antipsychotic was started to confirm the initial diagnosis of delirium and evaluate symptom severity, and again when antipsychotic was stopped, to assess symptom response. RESULTS Olanzapine (n=78), risperidone (n=13), and quetiapine (n=19) were used during the 2 years of the study. Mean patient age, length of treatment, and response were comparable for the three medications. For patients with two DRS-R98 scores available (n=75/110), mean DRS-R98 scores decreased significantly (p<0.001) with antipsychotic without significant adverse side effects. CONCLUSION Although randomized placebo-controlled studies are needed, atypical antipsychotic medications appeared to be effective and safe for managing delirium symptoms in pediatric patients while underlying etiology was addressed.

Fetal injury prior to labor: Does it happen?

Major advances have been made in the identification and prevention of perinatal factors that lead to long-term handicap or neurologic deficits. When the infant or child exhibits a major handicap, scrutiny of the pregnancy management often occurs in an attempt to define the causal factors. The medical goal of this inquiry is to prevent injuries and, when possible, to eliminate these factors. In the litigious sense, any deviation from optimal, ideal care or any unusual observations, such as unusual or atypical fetal heart rate patterns, are often causally linked to the adverse outcome. There are at least four categories of major fetal injury that probably occur prior to labor. An awareness of, and a diligent search for, details will no doubt clarify the legitimate origins of many so-called birth injuries. Hence the common tendency to fixate on minor deviations and/or deficiencies of labor and delivery management as causing catastrophic injuries will be successfully challenged.

The diagnosis and management of delirium in infancy.

OBJECTIVE Atypical antipsychotics have been documented to be effective in the management of delirium in adults and older children, but despite considerable need, their use has been less studied in the very young. A retrospective chart review was undertaken to describe the use of atypical antipsychotics in controlling symptoms of delirium in infants and toddlers. METHODS All psychiatric inpatient consultations performed during a 3 year period were reviewed to identify children <36 months old diagnosed with delirium. Delirium Rating Scale (DRS) scores were retrospectively calculated when the antipsychotic was initiated and discontinued, to confirm the diagnosis of delirium and evaluate symptom severity, and then to assess symptom response to pharmacologic intervention. RESULTS There were 10 boys and 9 girls in the study population (ages 7-30 months, mean 20.5 months). Olanzapine (n=16) and risperidone (n=3) were used, and length of treatment and response were comparable for both medications. Mean DRS scores decreased significantly (p<0.001) with antipsychotic administration, without significant adverse side effects. CONCLUSIONS Although randomized placebo controlled studies are needed to better characterize the indications, risks, and benefits, these atypical antipsychotic medications appeared to be effective and safe for managing delirium symptoms in very young pediatric patients.

A Fetus With Recombinant of Chromosome 8 Inherited From her Carrier Father

SummaryA pericentric inversion of chromosome 8, inv(8)(p23q22), in a male carrier resulted in an unbalanced recombinant, rec(8)dup q, inv(8)(p23q22), which was diagnosed prenatally. The features seen in the aborted fetus resembled the features seen in a previously affected child who received the identical recombinant from her carrier mother. In this particular inversion involving chromosome 8, both male and female carriers risk producing an unbalanced progeny. Different familial pericentric inversions are reviewed for the presence or absence of unbalanced recombinants.

The Pharmacologic Management of Delirium in Children and Adolescents

Delirium is a serious and common problem in severely medically ill patients of all ages. It has been less addressed in children and adolescents. Treatment of delirium is predicated on addressing its underlying cause. The management of its symptoms depends on the off-label use of antipsychotics, while avoiding agents that precipitate or worsen delirium. Olanzapine, quetiapine, and risperidone are presently considered first-line drugs, usually replacing haloperidol. Other agents have shown promise, including melatonin to address the sleep disturbance characteristic of delirium, and dexmedetomidine, an α2-agonist, that may facilitate lower doses of benzodiazepines and opioids that may worsen delirium.

Symptoms before and after posterior fossa surgery in pediatric patients.

The posterior fossa syndrome (PFS) is common after cerebellar tumor resection in pediatric patients. It is characterized by postoperative mutism and ataxia and associated with persistent abnormalities in mood and cognition. Method: A 2-year prospective study of children and adolescents with cerebellar tumors identified by neuroimaging was performed at the Children’s Hospital Los Angeles. Results: There were 8 girls and 14 boys in the study, aged 14 months to 17 years. The tumor sizes ranged from 2 to 6.5 cm in diameter. The patients presented with ataxia, headache, vomiting, depressed or irritable mood and inattention. Symptoms of PFS were present postoperatively in all except for the 2 patients with lateral tumors. The symptoms began before resection, were most prominent immediately after surgery, and improved over time. Neuropsychological assessment of 10 patients documented a persistent cognitive decrement. Conclusion: This small, descriptive study provides information on the natural history of pediatric posterior fossa tumors from before surgery through the postoperative period.

Recurrent delirium after surgery for congenital heart disease in an infant

Objective: The objective of this article is to describe a case of recurrent delirium after cardiac surgery in an infant. Design: Case report. The institutional review board at Childrens Hospital Los Angeles waived the need for informed consent. Setting: Cardiothoracic intensive care unit in a freestanding childrens hospital. Patient: A male infant with hypoplastic left heart syndrome who developed delirium on consecutive admissions to the cardiothoracic intensive care unit after cardiac surgery. Intervention: Pharmacologic intervention using the atypical antipsychotic olanzapine. Measurements and Main Results: The symptoms of delirium resolved with the initiation and continuation of olanzapine on both occasions. Conclusion: Delirium is a common, but often unrecognized, diagnosis in the intensive care unit. Its early recognition and treatment may prevent unnecessary use of narcotics and benzodiazepines, decrease length of stay and may improve long-term neurocognitive function. This case report describes an infant who developed discrete, consecutive episodes of delirium following surgery for congenital heart disease. Both episodes were treated effectively with olanzapine.