Improvement of stiff-person syndrome symptoms in pregnancy: Case series and literature review.

OBJECTIVE: To describe 2 cases from a single academic institution of improvement in stiff-person syndrome (SPS) symptoms during pregnancy and to review the clinical outcomes of SPS in 6 additional pregnancies described in the literature. METHODS: Evaluation of clinical symptoms and treatment changes of disease state during pregnancy. RESULTS: Seven patients with 9 pregnancies are described in women with a diagnosis of SPS. Six of 7 (86%) women were positive for glutamic acid decarboxylase (GAD65) antibody. In 5 of 9 (56%) pregnancies, symptomatic medications (antispasmodics) were significantly reduced with stabilization or improvement in symptoms through pregnancy. Nine live, healthy pregnancies resulted. All 7 (100%) women experienced worsening of symptoms after the birth of their children, and symptomatic therapies were resumed and/or increased. CONCLUSIONS: The immune pathogenesis of SPS continues to be explored. Immunomodulatory shifts during pregnancy may influence changes of clinical SPS symptoms and provide insight into the unique pathogenesis of SPS. Some women with SPS may be able to reduce symptomatic medications related to clinical improvement during pregnancy. Women with SPS may safely carry pregnancies to term, delivering healthy and unaffected babies.

Diagnosis influences response of cerebral near infrared spectroscopy to intracranial hypertension in children.

OBJECTIVE: To describe cerebral regional oxygen saturation measured by near infrared spectroscopy in the setting of normal and increased intracranial pressure in children to evaluate the association between cerebral regional oxygen saturation and intracranial pressure in comparison with other clinical variables. DESIGN: Prospective observational cohort study. SETTING: Two academic tertiary care centers' pediatric intensive care units. PATIENTS: Thirty patients with intracranial pressure and near infrared spectroscopy monitoring (median age, 11.5 yrs; interquartile range, 5.2-13 yrs) for a range of neurologic diagnoses, including brain tumor, trauma, intracerebral hemorrhage, and hydrocephalus. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Temporally correlated cerebral regional oxygen saturation with hematologic (hematocrit), biochemical (pH), and physiological (intracranial pressure, mean arterial pressure, cerebral perfusion pressure, temperature, heart rate, pulse oximetry and end-tidal carbon dioxide) variables. Cerebral regional oxygen saturation during episodes of increased intracranial pressure was lower than with normal intracranial pressure (mean +/- sd intracranial pressure >20 = 71% +/- 13% vs. intracranial pressure <20 = 75% +/- 10%), although the mean difference (-4%) is small compared with variability in the measurement. Neither isolated values nor change in cerebral regional oxygen saturation were significantly associated with intracranial pressure or cerebral perfusion pressure in the overall population. Isolated values and change in end-tidal CO2 were significantly correlated with cerebral regional oxygen saturation and change in cerebral regional oxygen saturation (all p < 0.01). In exploratory analyses, the diagnostic group significantly modified the effect of intracranial hypertension on regional oxygen saturation: regional oxygen saturation decreased during intracranial hypertension in patients with brain tumors (p = .05) and hydrocephalus (p < .001) but increased during intracranial hypertension in those with intracranial hemorrhage (p < .001). CONCLUSIONS: These data suggest that cerebral regional oxygen saturation is lower with intracranial hypertension. However, the relationship between cerebral regional oxygen saturation and intracranial pressure is strongly influenced by diagnosis.