Infantile spasms (IS) is a type of epileptic encephalopathy with poor developmental outcomes. The goal of treatment is complete control of spasms, improvement of hypsarrhythmia, and rapid reversal of delayed development. Improvements in diagnosis can result in the selection of appropriate therapy, which can improve a patient's developmental outcomes. However, there is no consensus on the best initial treatment for IS. Here, we describe 6-month-old boy with severe periventricular leukomalacia, developmental regression, and hypsarrhythmic pattern on electroencephalography, who was successfully treated with intravenous methylprednisolone.
Consensus ; Electroencephalography ; Infant ; Infant, Newborn ; Leukomalacia, Periventricular ; Methylprednisolone ; Spasm ; Spasms, Infantile

Various anatomical defects have been described in the surviving twin who had a stillborn, macerated monozygotic co-twin with Disseminated Intravascular Coargulation. The etiology is thought to be placental transfer of emboli or thromboplastic material through placental vascular anastomoses. We experienced a case of monozygotic twin with deceased co-twin at 30 weeks of gestation and confirmed to have antenatal periventricular germinal matrix and intraventricular hemorrhage, multicystic periventricular leukomalacia and diffuse encephalomalacia by neurosonography on first day of life despite of no clinical evidence of brain damage. The pathologic findings of placenta revealed infarct with massive fibrin deposition. A brief review of related literature is presented.
Brain* ; Encephalomalacia ; Fibrin ; Hemorrhage ; Humans ; Infant, Newborn ; Leukomalacia, Periventricular ; Placenta ; Pregnancy ; Twins ; Twins, Monozygotic*

OBJECTIVE: To evaluate the role of cranial sonography and computed tomography in the diagnosis of neonatal intracranial hemorrhage and hypoxic-ischemic injury in an Indian set-up. METHODS: The study included 100 neonates who underwent cranial sonography and computed tomography (CT) in the first month of life for suspected intracranial ischemia and hemorrhage. Two observers rated the images for possible intracranial lesions and a kappa statistic for interobserver agreement was calculated. RESULTS: There was no significant difference in the kappa values of CT and ultrasonography (USG) for the diagnosis of germinal matrix hemorrhage/intraventricular hemorrhage (GMH/IVH) and periventricular leucomalacia (PVL) and both showed good interobserver agreement. USG, however detected more cases of GMH/IVH (24 cases) and PVL (19) cases than CT (22 cases and 16 cases of IVH and PVL, respectively). CT had significantly better interobserver agreement for the diagnosis of hypoxic ischemic injury (HII) in term infants and also detected more cases (33) as compared to USG (18). CT also detected 6 cases of extraaxial hemorrhages as compared to 1 detected by USG. CONCLUSION: USG is better modality for imaging preterm neonates with suspected IVH or PVL. However, USG is unreliable in the imaging of term newborns with suspected HII where CT or magnetic resonance image scan is a better modality.
Hemorrhage ; Humans ; Infant ; Infant, Newborn ; Intracranial Hemorrhages ; Ischemia ; Leukomalacia, Periventricular ; Magnetic Resonance Spectroscopy

PURPOSE: To compare the findings of early periventricular leukomalacia on MR imaging and on US. MATERIALS AND METHODS: MR imaging was performed in 17 neonates in whom well-demarcated increased periventricular echogenicity was seen on sonography. One more patient was included during the same period because MR imaging of this patient showed a periventricular lesion not suspected on previous US. Initial sonography was performed within 6 days of birth and was followed up between one week and one month later. MR images were obtained within the first month of life. RESULTS: Twelve of 17 neonates showed abnormal periventricular signal intensities on MR imaging. Follow up sonography revealed cystic changes in two cases and heterogeneous hyperechogenicities in three. All patients except the two with cystic changes showed normal periventricular echogenicity on final sonography, On MR imaging,11 cases showed multifocal periventricular increased signals on T1-weighted images, and two cases showed mainly decreased signals representing cysts. Positive findings were more evident on T1-weighted than on T2-weighted image. CONCLUSION: on T1-weighted imaging, the characteristic finding of early periventricular leukomalacia was multifocal periventricular hyper or hypointensities, and hyperintense lesions were more common than hypointense. In the diagnosis of early noncystic periventricular leukomalacia, MR imaging was more objective than US.
Diagnosis ; Follow-Up Studies ; Humans ; Infant, Newborn ; Leukomalacia, Periventricular* ; Magnetic Resonance Imaging* ; Parturition

PURPOSE: To analyse the sequential sonographic findings of cystic PVL and to evaluate relationship between sonographic grading of PVL and patterns of neurologic outcomes. MATERIALS AND METHODS: Authors have retrospectively analysed the sequential sonographic findings of 36 cases of PVL in the preterm neonates. Initial sonographic features done within 3 days of life were divided into 3 patterns such as normal, localized, and diffuse hyperechogenic flare. Grading of PVL confirmed by follow-up studies was classified as involvement of one lobe (grade 1), two lobes (grade 2) and more than extent of grade 2(grade 3). The relationship between sonographic grading of leukomalacia and later neurologic outcomes were also analysed. RESULTS: Initial sonographic patterns according to grading of PVL were normal pattern in seven of nine (77.8%) of grade 1, diffuse hyperechogenic flares in five of eight cases of grade 2 and in 13 of 16 cases of grade 3. There was a significant difference bewteen the grades and frequency of pattern of diffuse hyperechoic flare (p=0.021). Average detection timing of cystic PVL was 38.4+/-18.9 days in grade 1, 29.8+/-14 days in grade 2, and 19.1+/-5.6 days in grade 3 with a significant statistical difference between the detection time and grades (p=0.037). Cerebral palsy has occurred in 62.5% of grade 1 and 100% of grade 2 and grade 3 (p=0.043). Frequency of spastic quadriplegia was higher in grade 3 (76.5%) than in grade 1 (25%) and grade 2 (12.5%) (p=0.001). CONCLUSION: Most of grade 1 cystic PVL revealed normal pattern of white matter echogenicity in initial ultrasonography and needed follow up examination over one month period. Spastic quadriplegia occured mainly in patients with grade 3 cystic PVL.
Cerebral Palsy ; Follow-Up Studies ; Humans ; Infant, Newborn ; Infant, Newborn* ; Leukomalacia, Periventricular* ; Quadriplegia ; Retrospective Studies ; Ultrasonography*

A developing central nervous system is vulnerable to various insults such as infection and ischemia. While increased understanding of the dynamic nature of brain development allows a deeper insight into the pathophysiology of perinatal brain injury, the precise nature of specific fetal and neonatal brain injuries and their short- and long-term clinical consequences need special attention and further elucidation. The current review will describe the pathophysiological aspects and clinical significance of white matter injury of prematurity, a main form of perinatal brain injury in premature newborns, with a particular emphasis on its potential antenatal components.
Brain ; Brain Injuries ; Central Nervous System ; Humans ; Infant, Newborn ; Ischemia ; Leukomalacia, Periventricular ; White Matter*

OBJECTIVETo study the relationship between the degree of white matter damage and changes in brain function in premature infants early after birth according to amplitude-integrated electroencephalogram (aEEG) and raw EEG (with burst-suppression patterns).

METHODSThirty-eight premature infants of less than 32 weeks' gestational age and with white matter damage, including 20 cases of mild white matter damage and 18 cases of severe white matter damage, were included in the study. Forty-two premature infants without white matter damage were selected as a control group. After birth, they were examined using aEEG and brain ultrasound once a week until four weeks after birth or a corrected gestational age of 32 weeks. The white matter damage and control groups were compared in terms of aEEG patterns and amplitudes and burst suppression ratio (BSR) on EEG.

RESULTSThe white matter damage and control groups had highly discontinuous patterns and had no complete sleep cycles. The lower amplitude was significantly smaller in the severe white matter damage subgroup than in the mild white matter damage subgroup and control group. There was alternating burst-suppression activity on the raw EEG in the white matter damage and control groups; and the severe white matter damage subgroup had a significantly longer suppression time and a significantly higher BSR on EEG compared with the mild white matter damage subgroup and control group.

CONCLUSIONSBrain function monitoring should be performed in premature infants with white matter damage early after birth so as to detect cases of severe white matter damage in time.

OBJECTIVESponsored by the Subspecialty Group of Neonatology of Pediatric Society, China Medical Association, more than 10 large-scale hospitals participated in the near two-year multicenter investigation for brain injuries in premature infants in China. This study presented the investigation result for the incidence of periventricular leukomalacia (PVL) in premature infants from 10 Third Class A Level hospitals.

METHODSThe premature infants with a gestation age<37 weeks in the 10 hospitals were given routine cranial ultrasound scanning within seven days after birth, and then repeated every 3-7 days until discharge from January 2005 to August 2006. The severity of PVL was graded based on de Vries classification.

RESULTSA total of 4 933 premature infants were enrolled. The total incidence of PVL and the incidence of cystic PVL were 2.3% (112/4 933) and 0.3% (16/4 933), respectively. Of the 112 PVL cases, 96 (85.7%) were with grade I, 14 (12.5%) with grade II, and 2 (1.8%) with grade III. The incidence of PVL in 4 maternal and child health care hospitals were significantly lower than that in 6 general or children's hospitals (1.4% vs 2.8%) (X2=10.284, P<0.01). Vaginal delivery and mechanical ventilation were possible high-risk factors for the development of cystic PVL.

CONCLUSIONSThe data of the multicenter investigation can basically reflect the situation about the occurrence of PVL in premature infants in major big cities of China. It is important to improve the ability to recognize the sonogram of non-cystic periventricular white matter injury.

Steroid use in perinatal period consists of the prenatal betamethasone or dexamethasone, the hydrocortisone treatment for transient adrenal insufficiency, and the dexamethasone treatment for bronchopulmonary dysplasia. Antenatal steroid treatment is a standard therapy for impending preterm delivery. Antenatal steroid reduced mortality and severe morbidities including intraventricular hemorrhage and periventricular leukomalacia. Usually betamethasone was superior to dexamethasone. Postnatal hydrocortisone can be administered if hypotension cannot be recovered by volume challenge and maximal inotropics support. High-dose dexamethasone cannot be recommended for the treatment of bronchopulmonary dysplasia because of increased cerebral palsy. Further randomized controlled trials should be performed for the low-dose dexamethasone therapy for bronchopulmonary dysplasia.
Adrenal Insufficiency ; Betamethasone ; Bronchopulmonary Dysplasia ; Cerebral Palsy ; Dexamethasone ; Hemorrhage ; Humans ; Hydrocortisone ; Hypotension ; Infant, Newborn ; Leukomalacia, Periventricular

Disease Progression ; Humans ; Infant, Newborn ; Infant, Premature ; Infant, Premature, Diseases ; pathology ; Leukomalacia, Periventricular ; pathology