Acute Encephalopathy with Unilateral Cortical-Subcortical Lesions in Two Unrelated Kindreds Treated with Glucocorticoids Prenatally for Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency: Established Facts and Novel Insight

Background: Prenatal glucocorticoid (GC) treatment of the female fetus with 21-hydroxylase deficiency (21-OHD) may prevent genital virilization and androgen effects on the brain, but prenatal GC therapy is controversial because of possible adverse effects on fetal programming, the cardiovascular system and the brain. Case Reports: We report 2 patients with congenital adrenal hyperplasia (CAH) due to 21-OHD who were treated prenatally with dexamethasone, suffered from an acute encephalopathy and showed focal and multifocal cortical and subcortical diffusion restrictions in early MRI and signs of permanent alterations in the follow-up neuroimaging studies. Both patients recovered from the acute episode. Whereas the first patient recovered without neurological sequelae the second patient showed hemianopsia and spastic hemiplegia in the neurological follow-up examination. Conclusion: These are 2 children with CAH, both treated prenatally with high doses of dexamethasone to prevent virilization. The question arises whether prenatal high-dose GC treatment in patients with CAH might represent a risk factor for brain lesions in later life. Adverse effects/events should be reported systematically in patients undergoing prenatal GC treatment and long-term follow-up studies involving risk factors for cerebrovascular disease should be performed.

Is surgery in acute aortic dissection type A still contraindicated in the presence of preoperative neurological symptoms?

OBJECTIVES Severe neurological deficit (ND) due to acute aortic dissection type A (AADA) was considered a contraindication for surgery because of poor prognosis. Recently, more aggressive indication for surgery despite neurological symptoms has shown acceptable postoperative clinical results. The aim of this study was to evaluate early and mid-term outcomes of patients with AADA presenting with acute ND. METHODS Data from 53 patients with new-onset ND who received surgical repair for AADA between 2005 and 2012 at our institution were retrospectively reviewed. ND was defined as focal motor or sensory deficit, hemiplegia, paraplegia, convulsions or coma. Neurological symptoms were evaluated preoperatively using the Glasgow Coma Scale (GCS) and modified Rankin Scale (mRS), and at discharge as well as 3-6 months postoperatively using the mRS and National Institutes of Health Stroke Scale. Involvement of carotid arteries was assessed in the pre- and postoperative computed tomography. Logistic regression analysis was performed to detect predictive factors for recovery of ND. RESULTS Of the 53 patients, 29 (54.7%) showed complete recovery from focal ND at follow-up. Neurological symptoms persisted in 24 (45.3%) patients, of which 8 (33%) died without neurological assessment at follow-up. Between the two groups (patients with recovery and those with persisting ND), there was no significant difference regarding the duration of hypothermic circulatory arrest (28 ± 14 vs 36 ± 20 min) or severely reduced consciousness (GCS <8). Multivariate analysis showed significant differences for the preoperative mRS between the two groups (P < 0.007). A high preoperative mRS was associated with persistence of neurological symptoms (P < 0.02). Cardiovascular risk factors, age or involvement of supra-aortic branches were not predictive for persistence of ND. CONCLUSION More than half of our patients recovered completely from ND due to AADA after surgery. Severity of clinical symptoms had a predictive value. Patients suffering from AADA and presenting with ND before surgery should not be excluded from emergency surgery.

Is surgery in aCute aortiC disseCtion type A still ContraindiCated in the presenCe of preoperative neurologiCal symptoms?

Gebiet: Kardiologie Abstract: OBJECTIVES: Severe neurologiCal defiCit (ND) due to aCute aortiC disseCtion type A (AADA) was Considered a ContraindiCation for surgery beCause of poor prognosis. ReCently, more aggressive indiCation for surgery despite neurologiCal symptoms has shown aCCeptable – postoperative CliniCal results. The aim of this study was to evaluate early and mid-term outComes of patients with AADA presenting with aCute ND. – – METHODS: Data from 53 patients with new-onset ND who reCeived surgiCal repair for AADA between 2005 and 2012 at our institution were retrospeCtively reviewed. ND was defined as foCal motor or sensory defiCit, hemiplegia, paraplegia, Convulsions or Coma. NeurologiCal symptoms were evaluated preoperatively using the Glasgow Coma SCale (GCS) and modified Rankin SCale (mRS), and at disCharge as well as 3–6 months postoperatively using the mRS and National Institutes of Health Stroke SCale. Involvement of Carotid arteries was assessed in the pre- and postoperative Computed tomography. LogistiC regression analysis was performed to deteCt prediCtive faCtors for reCovery of ND. – – RESULTS: Of the 53 patients, 29 (54.7%) showed Complete reCovery from foCal ND at follow-up. NeurologiCal symptoms persisted in 24 (45.3%) patients, of whiCh 8 (33%) died without neurologiCal assessment at follow-up. Between the two groups (patients with reCovery and – those with persisting ND), there was no signifiCant differenCe regarding the duration of hypothermiC CirCulatory arrest (28 ± 14 vs 36 ± 20 min) or severely reduCed ConsCiousness (GCS <8). Multivariate analysis showed signifiCant differenCes for the preoperative mRS between the two groups (P < 0.007). A high preoperative mRS was assoCiated with persistenCe of neurologiCal symptoms (P < 0.02). CardiovasCular risk faCtors, age or involvement of supra-aortiC branChes were not prediCtive for persistenCe of ND. – – CONCLUSION: More than half of our patients reCovered Completely from ND due to AADA after surgery. Severity of CliniCal symptoms had a prediCtive value. Patients suffering from AADA and presenting with ND before surgery should not be exCluded from emergenCy surgery.