The roles of electroencephalography and neuroimaging in children with holoprosencephaly.

We analyze the respective roles of neuro-imaging and EEG in the assessment of 11 children with holoprosencephaly and epilepsy. Seizures were present in seven patients (64%); six were treated with antiepileptic drugs; five had intractable epilepsy. Two of the patients with intractable epilepsy became seizure-free under polytherapy. Fourteen EEG recordings were performed in eight patients. The abnormal EEG findings included slow waves, focal epileptiform discharges, slow spike-and-wave complexes, hypsarrhythmia, frontal fast activity, fronto-occipital gradients of amplitudes (posterior amplitude attenuation), lack of photic driving, periodic discharges, and extremely large amplitudes. A fronto-occipital gradient was found only in alobar and semilobar holoprosencephaly (HPE), while hypsarrhythmia only in lobar HPE. Lack of photic driving was found only in alobar HPE. All EEGs showed diffuse slow waves, and all patients had severe developmental delay. The Deep Gray Score(DGS) in neuroimaging studies, thought to predict clinical outcome, was irrelevant given the presence and intractability of the epilepsies. Patients with higher DGS, nonetheless, tended to have higher mortality rate. In conclusion, EEG evaluation provides additional functional information to neuroimaging studies in the assessment of neurological outcome in patients with HPE. With a more mature and well-formed cerebrum, as found in the lobar and semilobar types, the possibility of hypsarrhythmia and photic driving increased, while that of fronto-occipital gradients decreased.

Hyperthermia as an early sign of intracranial hemorrhage in full-term neonates.

BACKGROUND: Intracranial hemorrhage (ICH) in full-term infants is uncommon and is a rare cause of neonatal fever. This study was conducted to estimate the incidences of ICH and fever in a hospital-based population, and to determine if the clinical features of neonatal ICH with and without hyperthermia differ. METHODS: We selected 315 afebrile neonates who received cranial ultrasonography screening from 2003 to 2004 as the control group, and 153 neonates diagnosed with fever from 1998 to 2004 as the study group. During the same period, 28 full-term neonates with birth weights >2500g and ICH in the first week of life were enrolled retrospectively. They were divided into hyperthermia (n=11) and nonhyperthermia (n=17) groups. RESULTS: Three babies in the control group and 11 in the study group had ICH; the incidence of ICH in the hyperthermia group was significantly higher (3/315 vs. 11/153; p < 0.001). Compared with the nonhyperthermia group, the hyperthermia group had Less cyanosis (2/11 vs. 10/17; p = 0.04), Less lymphocyte predominance (33.7 vs. 80%; p = 0.032), higher neutrophil/lymphocyte ratio (1.9 vs. 0.3; p = 0.006), higher erythrocyte/leukocyte ratio (425 vs. 79.5; p = 0.05) in cerebrospinal fluid and an increased incidence of subarachnoid hemorrhage accompanied by intracerebral parenchymal hemorrhage (4/11 vs. 0/17; p = 0.016). CONCLUSION: The incidence of ICH was higher in febrile than in afebrile neonates. ICH presenting with hyperthermia might go unrecognized, because its other symptoms are subtle and a neutrophil predominance in the cerebrospinal fluid might result in a misdiagnosis of meningitis. ICH in the interior brain tended to manifest more commonly as hyperthermia than did ICH in the superficial brain. A cranial image examination should be considered in the evaluation of neonatal fever.

Spinal Burkitt's lymphoma manifesting as nocturnal abdominal pain and constipation: a case report.

A 3-year-old boy presented at the Taiwan Adventist Hospital in Taipei with nocturnal epigastric pain and constipation. Abdominal X-ray showed colonic faecal impaction. Abdominal sonography showed gastric stasis with thickened pyloric wall and dilated rectosigmoid colons. The mouth-to-anus transit time (MATT) was prolonged. Endoscopy showed pale gastric mucosa, atony of pylorus and widening of the duodenal bulb. Three weeks after the onset of abdominal pain, he developed urinary incontinence and rapidly deteriorating paraplegia of lower limbs. Magnetic resonance imaging (MRI) showed an extradural intraspinal mass of T5-T8 and a soft tissue mass in the right superior mediastinum. After a laminectomy and tumour excision, the patient's symptoms improved quickly. The pathology revealed Burkitt's lymphoma. This is the first report of nocturnal abdominal pain and constipation as the initial manifestation of spinal Burkitt's lymphoma.

Chronological changes on magnetic resonance images in a case of desmoplastic infantile ganglioglioma.

Desmoplastic infantile gangliogliomas (DIGs) are rare tumors during infancy. They often occur as huge cystic tumors in the frontal and parietal lobes, with their solid component being adjacent to the leptomeningeal membrane. This report presents a patient with DIG and intractable epilepsy. Due to hesitation by the patient's family in allowing surgery, this is the first time that the chronological changes in DIG have been observed on imaging studies. During the follow-up, the tumor changed from a pure solid tumor to a cystic one, which is a typical picture of DIG. Surgical pathology confirmed the diagnosis of DIG. However, for a patient with epilepsy, it is recommended that this condition should be treated immediately with surgery.