ABSTRACT
Cranial Ultrasound (cUS) may not be sensitive enough to detect subtle white matter (WM) injuries. Our study compared serial cUS with MRI at term equivalent age (TEA) to determine if it is possible to identify an ultrasound representation of subtle diffuse WM injuries such as punctate lesions (PWMLs) and diffuse excessive high signal intensity (DEHSI). Fifty-six very preterm infants were scanned sequentially from birth to TEA, an MRI was performed at TEA. Each echodensity found on cUS was classified as absent, transient (≤7 days), or prolonged (>7 days). A transient periventricular echodensity was detected in seven infants (12.5%), and a prolonged echodensity in 15 (26.8%). MRI examinations were performed in all 56 infants. No altered signal intensity was found in 18 infants (32.1%). DEHSI was detected in 14 infants (25%), and PWMLs were detected in eight babies (14.3%). Both abnormalities were found in 16 infants (28.6%). The positive predictive values of the prolonged echodensity for DEHSI and PWMLs were 86.7% and 46.7% respectively. However, a significant statistical correspondence (p=0.002, Odds Ratio 11.9) was found comparing DEHSI with cUS abnormal echodensities. Serial cUS during the neonatal period in preterm infants is essential and cannot be replaced with MRI at TEA. MRI seems to be more reliable in detecting mild or moderate WM abnormalities. However, serial cUS performed by an experienced neonatologist can provide valuable information on early WM changes such as prolonged echodensities that could potentially lead to a diffuse injury.
Subject(s)
Leukoencephalopathies/diagnostic imaging , Leukoencephalopathies/pathology , Ultrasonography, Doppler, Transcranial , Female , Gestational Age , Humans , Infant, Newborn , Infant, Premature , Magnetic Resonance Imaging , Male , Nerve Fibers, Myelinated/diagnostic imaging , Nerve Fibers, Myelinated/pathology , Retrospective Studies , Sensitivity and SpecificityABSTRACT
The clinical onset of hereditary neuropathy with liability to pressure palsy (HNPP) in childhood is rarely reported. On the basis of a 5-year-old affected patient, we reviewed the cases reported in the literature to evaluate the clinical and genetic characteristics of patients with an early onset (<10 years) of HNPP.
Subject(s)
Hereditary Sensory and Motor Neuropathy/genetics , Hereditary Sensory and Motor Neuropathy/physiopathology , Age of Onset , Child, Preschool , Hereditary Sensory and Motor Neuropathy/epidemiology , Humans , Male , Myelin Proteins/genetics , Neural Conduction/physiology , Paralysis/epidemiology , Paralysis/genetics , Paralysis/physiopathologyABSTRACT
To assess the efficacy, tolerability and safety of Levetiracetam (LEV) therapy, we identified 21 (15 male; 6 female) patients with a history of benign epilepsy with centrotemporal spikes (BECTS), with and without secondarily generalization in children and adolescents aged between 5.0 and 12.1 years. LEV was administered as a first drug (number of patients=9) or converted after previous treatment with other AEDs (number of patients=12). The patients were subdivided into two groups: "newly diagnosed" patients and "converted" patients. Patients were followed up for 12 months and all patients were able to continue on LEV treatment. At the end of follow-up (12 months), all patients were seizure free or showed a reduction of seizures >50%. LEV dosage ranged from 1000 to 2500mg/daily. Overall, 100% of patients completed the 12 months study, without any important side effect. Somnolence and irritability occurred in two (9.5%) patients. Our results support findings that LEV monotherapy is effective and well tolerated in children with BECTS. Prospective, large, long-term double-blind studies are needed to confirm these findings.
Subject(s)
Anticonvulsants/therapeutic use , Epilepsy, Rolandic/drug therapy , Piracetam/analogs & derivatives , Adolescent , Anticonvulsants/administration & dosage , Child , Electroencephalography , Female , Follow-Up Studies , Humans , Levetiracetam , Male , Piracetam/administration & dosage , Piracetam/therapeutic use , Prospective Studies , Treatment OutcomeABSTRACT
BACKGROUND: Frontal lobe epilepsy (FLE) is a type of epilepsy that is difficult to treat and there are few studies about the use of topiramate (TPM). AIM OF THE STUDY: To evaluate the efficacy and tolerability of TPM monotherapy in FLE. METHODS: The study group consisted of 55 (33 male; 22 female) patients. TPM was administered as a first drug (n = 16) or converted after previous treatment (n = 39). All patients were followed every 3 months for at least 1 year. The patients were subdivided into two groups: 'newly diagnosed' patients and 'difficult-to-treat' patients. RESULTS: Overall, all patients completed the 1-year study. At the end of follow-up, 10 patients showed disappearance of seizures and 33 patients showed improvement in seizure frequency. In particular, among the newly diagnosed patients 6/16 patients showed complete cessation of seizures and 5/16 patients showed very good response; in the other group, 4/39 patients showed complete cessation and 4/39 patients showed a very good response. No patients of both groups had worsening of seizures. No treatment-limiting adverse events associated with TPM were reported. CONCLUSIONS: TPM is effective in newly diagnosed patients with FLE; TPM can be considered for the treatment of FLE.
