ABSTRACT
PURPOSE: The purpose of the study was to evaluate the effects of mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) on corpus callosum (CC) morphometry in large and pathologically proven MTLE-HS patients. METHODS: We measured certain CC dimensions in 103 patients and 33 healthy controls using in vivo magnetic resonance imaging. In particular, we compared the two groups in relation to the clinical (localization of the HS, duration of epilepsy, frequency of seizures and length of seizures) and demographical (age, gender, handedness) features. Students' t test, two-way ANOVA and Spearman test were used for statistical analysis. RESULTS: There was no significant difference between CC morphometry with respect to age and handedness among patients. The differences between the genders, however, were significant favouring longer diameters in males. We found significant decrease in the dimensions of the genu, body, isthmus and splenium of the CC in the MTLE-HS group, but there was no reduction in the size of the rostrum. CONCLUSIONS: This general reduction in the size of the CC except for the rostrum was thought to be the result of cortical atrophy secondary to the disease. Concerning the preserved rostral part of the CC, it was thought that the fibers of the frontal lobe pass through different pathways than the tracts in the rostrum.
Subject(s)
Corpus Callosum/pathology , Epilepsy, Temporal Lobe/pathology , Hippocampus/pathology , Adult , Case-Control Studies , Female , Humans , Magnetic Resonance Imaging , Male , Sclerosis , Young AdultABSTRACT
We report a case of partial trisomy 22q with de novo duplication of chromosomal region 22q11.1-22q13.1, also confirmed by microarray comparative genomic hybridization (Array-CGH) analysis. The fetus had interhemispheric cyst and corpus callosum agenesis diagnosed by MRI which has not been reported in the literature. This novel phenotype differs from the reported cat eye syndromes by the absence of heart defects and the presence of brain anomalies.
Subject(s)
Abnormalities, Multiple/genetics , Brain/abnormalities , Chromosomes, Human, Pair 22 , Eye Abnormalities/genetics , Gene Duplication , Trisomy/genetics , Abnormalities, Multiple/diagnosis , Agenesis of Corpus Callosum , Arachnoid Cysts , Comparative Genomic Hybridization , Eye Abnormalities/diagnosis , Fatal Outcome , Female , Humans , Infant, Newborn , Magnetic Resonance Imaging , Male , Oligonucleotide Array Sequence Analysis , Pregnancy , Prenatal Diagnosis , Syndrome , Trisomy/diagnosisABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the immune system that is associated with frequent involvement of the central nervous system (CNS). The MR imaging and CT findings of the CNS infiltration have been reported in the past; however, the diffusion-weighted imaging (DWI) findings have not been previously described. We present MR imaging findings in a case of secondary HLH with CNS involvement, with an emphasis on the DWI findings.
Subject(s)
Brain Diseases/diagnosis , Brain/pathology , Diffusion Magnetic Resonance Imaging , Lymphohistiocytosis, Hemophagocytic/diagnosis , Adolescent , Brain Diseases/pathology , Female , Humans , Leukemia-Lymphoma, Adult T-Cell/complications , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/pathologyABSTRACT
BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is the most common demyelinating disorder of childhood. Its clinical features, prognosis and treatment vary in different reports. OBJECTIVES: To examine a series of children with ADEM for clinical findings, course, recurrences, and possible variables affecting outcome. METHODS: Multicentric data collected from 7 tertiary referral centers were registered and evaluated in a central database in 1990 - 2001 for clinical, laboratory, and MRI features. Course and prognosis were assessed in patients with at least 12 months' follow-up. RESULTS: Forty-six patients were evaluated. Median age at onset was 8 years, M/F ratio, 1.7/1. Most common symptoms and signs pertained to the motor system and consciousness. Of 39 children with 12 months' follow-up, 71 % recovered completely. Thirteen (33 %) children had relapses. Patients who had more than one relapse (n = 4) presented with new symptoms at each attack. Treatment with high-dose methylprednisolone was associated with complete recovery, and tapering over more than 3 weeks, with a lower rate of relapses. MRI lesions could persist even in asymptomatic patients; in particular, periventricular lesions tended to disappear later than others. CONCLUSIONS: Complete clinical recovery is common and serious complications are rare in childhood ADEM, but the rate of relapses is considerable. Clinical picture at first relapse may help to identify patients likely to experience multiple relapses. The timing and duration of steroid treatment affects outcome.
