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Chloramphenicol (CP) is recently one of the rarely-used antibiotics. In this study, we present four patients with intractable bacterial meningitis, who were successfully treated with CP and discuss the therapeutic indications of CP in these pediatric cases. The patients were diagnosed as bacterial meningitis at the ages ranging from 2 months to 1 year and 4 months. The causative organisms found in three of the patients were H. influenzae and in the fourth patient, S. pneumoniae. According to the microbial sensitivity tests, these organisms were highly sensitive to antibiotics including ceftriaxone, meropenem and/or panipenem/betamipron. Treatment with these antibiotics was initially effective; however, recurrences of meningitis appeared in all patients. Administration of CP (100 mg/kg/day) started between the 11th and the 58th days, and was continued for 9 days up to 19 days. Their fever had disappeared within four days after the administration of CP, and it was confirmed that all patients completely recovered from meningitis. Two of the patients developed a mild degree of anemia, but soon recovered after the discontinuation of CP. None of them had neurological sequela. We recommend CP as one of the choices for the treatment of intractable bacterial meningitis.

A newborn baby boy presented with giant melanocytic nevi on the face, trunk and extremities, and focal cortical dysplasia on MRI. At 3 months of age, he developed intractable epilepsy, and MRI at 2 years of age revealed a high-intensity area in the bilateral cerebellum on T1-weighted images, indicative of melanosis. Based on the findings of the skin and MRI, we diagnosed the boy with neurocutaneous melanosis. Cytodiagnosis of cerebrospinal fluid showed no malignancies. EEG, magnetoencephalogram and ECD-SPECT indicated that the clonic seizures originated from a focus in the right focal cortical dysplasia. Complications also included sebaceous nevus of the head and face, which was characteristic of sebaceous nevus syndrome, lipoma of the face and cauda equina, and limbal dermoid. Sebaceous nevus syndrome may have been due to certain allelic defects that were independent of those for neurocutaneous melanosis.

To explore the frequency and prognosis of neurological complications of acute lymphocytic leukemia, retrospective studies were made of patients with acute lymphocytic leukemia. Neurological complications were found in 13 of 100 patients during and after treatment. They were caused by chemotherapy in 8 patients, irradiation therapy in 2, vitamin B1 deficiency in 1, and unknown in 2. Medications primarily relevant to these complications were methotrexate in 5 patients, L-asparaginase in 2, cytosin arabinoside in 1. The patients were diagnosed as having leukoencephalopathy (8), cerebrovascular injury (4), and Wernicke's encephalopathy (1). Symptomatic epilepsy was found in one patient, and mental retardation was seen in three patients during a 2-year course of treatment. We conclude that careful management is required in the treatment of acute lymphocytic leukemia, because of the persistence of neurological complications, although their severity is decreasing with advances in treatment.

Etiology and management of neonatal seizures was retrospectively studied in 39 patients, who were admitted in our neonatal intensive care unit. Midazolam was administered to 22 patients by intravenous bolus injection (0.15 +/- 0.08 mg/kg) and/or continuous intravenous infusion (0.33 mg +/- 0.11 mg/kg/hr), both of which were judged as effective in 71% and 76% of the patients, respectively. Seven patients showed decrease of blood pressure possibly related with administration of midazolam, but five of them were managed by decreasing dose of the drug or observation of clinical pictures. We consider that midazolam is effective and relatively safe for the management of neonatal seizures.

We encountered a 5-month-old baby with shaken baby syndrome, which had been caused by being thrown up about 50 cm in the air several times by his father. Intracranial hemorrhage, bleeding in the eye grounds in both eyes, and convulsion were noted on admission. Shaken baby syndrome was diagnosed from the history, and the patient was given necessary treatment, which led to favorable prognosis. Father was not aware of the danger of handling his baby in that way, and no sign of maltreatment was noted on the body surface of the baby. An attention should be payed that the danger leading into syndrome could be hidden among this kind of caring in the everyday life.

We report our experience using lidocaine to determine the epileptic focus in a case of refractory status epilepticus associated with an aberrant intracranial shunt tube. Pentobarbital anesthesia rapidly suppressed convulsions. However, whenever the pentobarbital was decreased, the status epilepticus was resumed. The seizure looked primarily generalized both clinically and electroencephalographically, but EEG monitoring with intravenous administration of lidocaine demonstrated that the ictal waves began from the right anterotemporal area, where the shunt valve and tube were placed. Removal of the shunt tube and the surrounding scar tissue eliminated status epilepticus. Our result suggests the excellent efficacy of lidocaine to distinguish secondarily generalized status epilepticus from primarily generalized one.

Three cases with petit mal absence attacks with secondary bilateral synchrony were presented. The interictal EEG showed unilateral focal epileptic discharges in the frontal area with normal background activity. The ictal EEG disclosed 3-Hz generalized symmetrical spike-wave patterns preceded by unilateral focal discharges, which were elicited by hyperventilation. None of the three cases had abnormal neurological findings or abnormal CT findings. All three cases had normal psychomotor development. One case had a history of febrile convulsions. The ictal manifestations were as follows; impairement of consciousness without any other signs in one case, with decreased posture tone in the second, and automatism in the third. The authors suggest that there can be petit mal absence with 3-Hz generalized spike-wave discharges induced by secondary bilateral synchrony.

Two cases of ictal automatisms of frontal lobe origin are reported. Both two cases had automatisms characterized by abrupt onset and ending, short duration, preservation of consciousness and frequent attacks in cluster. The ictal manifestations included rocking of body and violent thrashing of limbs in prone position with loud screaming. No initial staring or postictal confusion was noted. Interictal EEGs showed spikes in the frontal area. Ictal EEGs began with attenuation of background activity and/or fast rhythmic waves dominant in the frontal area. Just after clinical seizures, high voltage slow waves in bilateral frontal areas and rhythmic alpha waves in the occipital areas were recorded. The EEG findings suggested that the paroxysmal activities were localized in bilateral frontal lobes, not spreading to the temporal lobe.

In the series of our studies of positron emission tomography (PET), we had some cases whose cerebral blood flow was reduced in the cerebrum, cerebellum and brain stem, and was preserved only in the basal ganglia region. We studied their clinical features and electrophysiological findings of these cases. These 5 cases included neuronal ceroid lipofuscinosis, Krabbe disease, Tay-Sachs disease, progressive myoclonus epilepsy and subacute sclerosing panencephalitis. Clinically they showed symptoms associated with diffuse cerebral and brain stem involvements. Electrophysiological studies also revealed the involvements of cerebrum and brain stem. These 5 cases were classified to persistent vegetative state clinically. Vegetative state was considered to be heterogeneous concerning about cerebral metabolism. There may be one group presenting a peculiar cerebral metabolic condition described here in vegetative states. And this condition may be specific to some neurodegenerative or metabolic disorders that involve cerebellum and brain stem as well as cerebrum.

Negative myoclonus in a 4-year-old boy was analyzed by polygraphic study, which lead to the diagnosis of simple partial seizure. He had irregularly trembling movements of right upper limb when he held some objects with his right hand. There were also coarse trembling movements in his body and lower limbs while sitting or standing. Polygraphic recording showed that the involuntary movements were caused by intermittent inhibition of muscle contraction associated with electrical silence on EMG and spikes in the left central area on EEG.

We reported two cases of brain infarction. They were cousins. Case 1 was a 12-year-old girl, who complained of aphasia, dyscalculia, right-left disorientation and right homonymous hemianopsia. CT showed low density areas in left superior and middle temporal gyri. Case 2 was a 15-year-old boy, who had left hemiplegia and hypesthesia to pain, temperature and touch on the left side of the body. CT showed low density areas from the genu of the internal capsule to the corona radiata, and from the posterior portion of putamen to the posterior limb of the internal capsule on the right side. Both cases had hypertriglyceridemia which might be associated with the etiology of infarction.

In 12-month-old girl with Krabbe's disease, serial CT and MRI examinations showed characteristic findings, which were symmetric high densities in globus pallidus and thalami before and during the course of brain atrophy and decreased attenuation in white matter. The low densities in white matter were not so remarkable as other degenerative diseases affecting primarily white matter. The high densities in the globus pallidus changed into low densities with progression of the white matter hypodensities and brain atrophy. These findings on CT in Krabbe's disease may assist neurologists in narrowing the differential diagnosis and requesting appropriate laboratory tests.