Improved neurologic prognosis for a patient with propionic acidemia who received early living donor liver transplantation.

Despite medical therapy, patients with propionic academia (PA) still display a tendency to develop epilepsy. Patients with neonatal-onset PA who have received early living donor liver transplantation (LDLT) are limited in number, and the effect on neurologic prognosis, including epilepsy, is not clear. We report a patient with PA whose EEG findings improved dramatically after undergoing LDLT at age 7 months. The patient's neurologic development and brain MRI findings were quite satisfactory at age 2 years and 3 months. LDLT is effective not only in preventing metabolic decompensation, but also in improving neurologic function to ensure better quality of life.

[A case of oligodendroglioma with temporal lobe epilepsy initially suspected as having paroxymal tachycardia].

A 4-year-old girl had repetitive attacks of chest pain, palpitation and loss of consciousness, which lasted for a few minutes and occurred several times a day. Interictal and ictal EEGs revealed that these episodes were complex partial seizures with autonomic symptoms originating from the right antero-temporal area. Brain MRI depicted a tumor in the right temporal lobe, the suspected etiology of the seizures. The tumor and its surrounding area were carefully resected using electrocorticogram. Pathologically, the tumor was diagnosed as an oligodendroglioma. MR spectrometry demonstrated a definite reduction of NAA/creatine ratio in the tumor. It is reported that a probability of developing seizures in cases of oligodendroglioma is about 80-90%. The occurrence rate of seizures in oligodendroglioma is much higher than that in other brain tumors. The cause of the epileptogenesis in oligodendroglioma might be due to the pathological tissue of satellitosis and secondary degeneration of neurons.

Gelastic seizure with hypothalamic hamartoma: proton magnetic resonance spectrometry and ictal electroencephalographic findings in a 4-year-old girl.

Gelastic seizure is a rare symptom often associated with hypothalamic hamartoma. We present here a 4-year-old girl with gelastic epilepsy caused by hypothalamic hamartoma and report the magnetic resonance spectrometry and electroencephalographic (EEG) findings. At the age of 2 1/2 years, she developed brief, repetitive laughing attacks or mixed attacks with laughing and crying, which were refractory to carbamazepine. An interictal EEG showed intermittent slow waves in the left frontocentral region and sporadic positive sharp waves in the left centroparietal area. Ictal EEG demonstrated dysrhythmic theta activity in the left central area 3 seconds after the onset of laughing. Brain magnetic resonance imaging demonstrated a large sessile mass, isointense to gray matter, in the region of the hypothalamus, suggesting hypothalamic hamartoma. Proton magnetic resonance spectrometry of the hypothalamic hamartoma revealed a significant reduction of the N-acetylaspartate/serum creatinine ratio. The altered chemical shift imaging with magnetic resonance spectrometry in our patient suggests a biochemical abnormality in the tissue of the hypothalamic hamartoma. Moreover, this abnormal function of the hamartoma tissue might be closely related to epileptogenesis because the time difference between the ictal laughter and the subsequent EEG changes in the ictal EEG does not support the idea that the activated cortex is the epileptogenic focus.