A slower rate of progression of disease characterized EOMS patients, suggesting more plasticity to recover in developing CNS, but the early clinical manifestation cannot be considered a positive prognostic factor.

(J Neurol Neurosurg Psychiatry 1998;64:516-523)

Summary: Purpose: To assess the role of 'H-magnetic resonance spectroscopy (MRS) in detecting biochemical abnormalities in neuronal migration disorders (NMDs).Methods: We performed 'H-MRS studies on 17 brain NMD areas [five polymicrogyria, eight subcortical heterotopia, and four cortical dysplasia on magnetic resonance imaging (MRI)]. The study group consisted of I5 patients, all but one affected by partial epileptic seizures. Spectra were acquired from volumes of interest localized on NMDs and contralateral sides and compared with those obtained on gray and white matter of 18 neurologic controls.Results: NMD lesions were characterized by lower Nacetylaspartate to creatine (NAAICr) and choline to Cr (Chot Cr) ratios than those of the white (p = 0.002 and p = 0.004) and gray matter (p = 0.03 and p = 0.06) of neurologic controls. In addition, the nonnal-appearing contralateral sides to the NMD lesions showed a significant decrease of Cho/Cr ratio when compared with those of white (p = 0.003) and gray matter (p = 0.05) of neurologic controls. No relation was found between NAAlCr decrease, EEG abnormalities, and NMD sides, or between NANCr ratios, duration of epilepsy, and frequency of seizures. Lactate signal was detected in the spectra of four patients who had an epileptic seizure a short time before MR examination.Conclusions: NAA/Cr decrease may be related more to structural and functional alteration of the NMD sides than to epileptic activity in these lesions. Low Cho/Cr may be related to a more extensive diffuse hypomyelination than suggested by the MRI findings. An activation of anerobic glycolysis during and after seizures could account for the presence of lactate. These data confirm that 'H-MRS is an advanced technique that may provide useful biochemical information in vivo on neurobiologic processes underlying NMDs. Key Words: Neuronal migration disorders-Epilepsy-Proton magnetic resonance spectroscopy-Magnetic resonance imaging.Neuronal migration disorders (NMDs) are a broad group of brain illnesses characterized by anomalies of site, thickness, shape, and organization of cerebral cortex due to a complete or partial failure of the neuroblast migration process, which may occur between the gestational weeks 8 and 15 (1,2). The etiology of NMD is extremely varied and may include genetic and chromosomal factors or maternal and environmental causes (for example, toxic, infectious, and hypoxichschemic) (3).Several clinical studies reported that frequently NMDs are associated with severe partial epilepsy, suggesting a combination of morphologic and functional abnormali-

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Multiple sclerosis (MS) onset is usually in adult life (age 20-40 years). Discordant data have been reported concerning the frequency of early onset MS (EOMS) that ranges from 2.7% to 5%, whereas there is a general agreement on prevalence of female sex, particularly after puberty. The initial symptoms in EOMS are frequently characterized by visual loss whereas the other functional systems are involved with a variable frequency. Literature data show that EOMS tends to have a relapsing-remitting course, a high rate of recovery from the initial attack, a long time interval between the first and second attacks and a slow progression rate. A poor prognosis is reported in a few cases of EOMS and seems to be related to number of relapses and to the delay between the first and second attacks.