Serial MRI and MRS studies with unusual findings in Rasmussen's encephalitis.

Rasmussen's syndrome is characterized by intractable seizures and progressive neuropsychiatric deterioration secondary to unilateral cortical inflammation and tissue destruction. Diagnosis of Rasmussen's syndrome in the early phase depends mainly on the clinical features. Neuroimaging and histopathologic examinations may not be specific during this period. We report a case of Rasmussen's syndrome followed by serial MRI and magnetic resonance spectroscopy (MRS) studies over a 3- to 16-month period. A healthy 6-year-old boy presented with focal motor seizures. An MRI study demonstrated prominent enlargement and T2 hyperintensity of the left mesial temporal lobe and perisylvian region. This early finding evolved to volume loss and later progressive atrophy of the ipsilateral hemisphere when epilepsia partialis continua occurred. Being aware of those early MRI features in a patent with increasing frequency of focal motor seizures should suggest Rasmussen's syndrome. In addition, we found prominently increased myoinositol concentration in atrophic cortex which might reflect increased gliosis in the late period of the disease.

Normative data of sympathetic skin response and RR interval variation in Turkish children.

Sympathetic skin response (SSR) and RR interval variation (RRIV) are used commonly for the assessment of sympathetic and parasympathetic nervous system function, respectively. We determined the normal values of SSR and RRIV in 23 (14 females, nine males) Turkish children aged 5 to 14 (mean 9.86, SD 2.48) years. SSR was recorded on the hands and feet during the electrical stimulation of both median and posterior tibial nerves, respectively. Similar response was elicited on both feet during the stimulation of the right median nerve. RRIV testing was performed during rest on the supine position and deep inspiration at a frequency of 6 times/min. The SSR was elicited in all children. The mean SSR latencies recorded on the feet during the stimulation of median or posterior tibial nerve were significantly more prolonged than those recorded at the hands (P < 0.001). There was no significant difference between the mean latencies of SSR recorded at the ipsilateral and contralateral palms or soles. The mean latencies recorded at the sole during stimulation of the median nerve were not significantly different compared to those that recorded at the sole during the posterior tibial nerve (P > 0.05). The SSR amplitudes were not assessed because of great variability and rapid habituation. The mean RRIV (46.54+/-11.29%) during deep breathing was significantly increased as compared to that (35.90+/-10.63%) during rest (P < 0.003). As a result, SSR and RRIV are preferred non-invasive tests for evaluation of autonomic nervous system in children. The SSR is useful and reliable if it is obtained in the optimum technical conditions. Further research is necessary to establish strict criteria for abnormality.

Hemimegalencephaly and Hirschsprung's disease: a unique association.

A 2-year-old boy with hemimegalencephaly and Hirschsprung's disease is reported. The unique association of these two entities is considered to be the presence of a common insult or insults that affect the innervation of the bowel and the formation of the cerebral cortex. Short-segment subtype of Hirschsprung's disease may suggest that this effect occurred between the eighth and twelfth weeks of gestation. Although there is a well-known coexistence of Hirschsprung's disease with the malformations that share a common neurocristopathic origin (abnormalities of neural crest cell growth, migration, or differentiation), a few extremely rare cases, as in this case, might reflect the coexistence of Hirschsprung's disease with a cerebral malformation (i.e., hemimegalencephaly) that is a nonneurocristopathic entity by itself.