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Summary:The prognosis and evolutional changes of 77 patients with West syndrome (WS) were studied after patients were classified into four groups op the basis of their magnetic resonance imaging (MRI) findings: anomaly, perinatal injury, normal, and the other groups. The average age at onset of spasms was earliest in the patients with anomalies and latest in patients with normal MRI findings. Patients with normal MFU findings had the shortest duration of spasms, and patients with anomalies had the longest duration of spasms. Antecedent seizures were observed in 6 patients (3 patients with anomalies, 1 patient with normal MRI findings, and 2 patients with other abnormalities). Thirty-five patients had subsequent seizures.Patients with anomalies often had partial seizures and patients with perinatal injuries often had generalized seizures. Seizures were infrequent in patients with normal MRI findings. Developmental outcome was best in the patients with normal MRI findings and worst in patients with perinatal injuries. Various types of epileptic syndromes occurred subsequent to WS in patients with anomalies, although nonspecific symptomatic generalized epilepsy was common in patients with perinatal injuries. These results suggest that seizure prognosis, evolutional changes in seizures, and developmental outcome are different among the types of brain lesions. Key Words: West syndrome-Magnetic resonance imaging.West syndrome (WS) is an age-dependent epileptic syndrome caused by various brain disorders. WS has been frequently classified into two groups: cryptogenic and symptomatic. The prognosis and evolutional changes of WS have usually been discussed according to this classification. Because the symptomatic WS group consists of patients with numerous types of brain lesions, the prognosis and evolutional changes may be different among the types of brain lesions. Magnetic resonance imaging (MRI) provides us with detailed information about brain lesions (1,2). In the present study, we studied the prognosis and evolutional changes of WS after classifying the patients with WS into four major groups and several subgroups on the basis of their MRI findings. SUBJECTS AND METHODSPatients with WS, who were treated in Department of Pediatrics, Nagoya University Hospital, and three related institutions were evaluated. In this study, patients who met the following criteria were studied: (a) onset of WS between 1985 and 1994, (b) >2 years follow-up observation, and (c) performance of at least one cranial MRI. Eventually, 77 patients (46 boys and 3 1 girls) were studied. Twenty-three of them were born prematurely, and the corrected age was applied to the subsequent analysis of these patients. Seizures and epileptic syndromes were classified on the basis of their clinical manifestations and EEG according to the Classification of the International League Against Epilepsy (ILAE). WS was diagnosed when a patient had a series of spasms and showed hypsarrhythmia on their interictal EEG. Severe epilepsy with multiple independent spike f...

Summary: Purpose: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS-ES).Methods: We examined 45 patients with West syndrome whose epileptic spasms were recorded with simultaneous video-electroencephalography (EEG) monitoring between 1982 and 1996. We investigated the patients who had PS-ES and compared the PS-ES patients with the 37 patients without Results: Of the 45 patients who had epileptic spasms in clusters (ES) and hypsarrhythmia on the interictal EEG, eight (1 7%) had ES preceded by partial seizures (PS) with a close temporal association. Seven of these eight were female patients. The underlying disorders were tuberous sclerosis (one patient), Aicardi syndrome (one), nonketotic hyperglycinemia PS-ES.(one), and focal cortical dysplasia (one). The etiology was unknown in the remaining four patients, but was suspected to be of prenatal origin in three. Three types of seizure sequence were identified: PS followed several seconds later by ES (two patients), alternating PS and ES starting with PS (three), and PS gradually replaced by ES with overlapping of the two (three). PS-ES disappeared or was replaced by other types of seizures in 1-34 months. Six patients could not walk, and all patients could not speak any sentences at age 3 years.Conclusions: Compared with patients without PS-ES, those with PS-ES more often had organic brain lesions of prenatal origin, other types of seizures before the onset of ES, asymmetric hypsarrhythmia on the EEG, and poor psychomotor outcome. Key Words: West syndrome-Partial seizureEpileptic spasms-Development-Infant.West syndrome is characterized by certain types of seizures [i.e., epileptic spasms (ESs)], which often occur in clusters. However, some patients with this epileptic syndrome have other types of seizures, especially partial seizures (PSs), either dependent on or independent of ES (1-13). An increasing number of reports on the temporal association of ESs and PSs have been published (4-6,8-10,12,13). Hrachovy et al. (1 1) stated that the coupling of PSs with ESs occurs rarely and in some instances is explained by chance coincidence. Other investigators, however, reported much higher incidences of ES, which were closely associated with PS in West syndrome patients (1 0,12,13). To elucidate this seizure sequence and the relation between ESs and PSs in West syndrome, we retrospectively reviewed patients who had hypsarrhythmia on interictal EEG and ESs evidenced by simultaneous video-EEG monitoring (SVEM).Accepted April 12, 1999. Address correspondence and reprint requests to Dr. T. Kubota at Department of Pediatrics, Nagoya University School of Medicine, 65 Tsurumai, Showa-ku, Nagoya, Japan, 466-8550. METHODS AND SUBJECTSBetween 1982 and 1996, ES was recorded at least once in 45 patients with SVEM at Nagoya University Hospital. They all had hypsarrhythmia on the interictal EEG and were admitted for the treatment of the epileptic seizures. After admission, all of the patients underwent a full ...

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Meningitis is not a common complication of chronic granulomatous disease (CGD). Here, we present details of a 3-year-old boy with X-linked CGD, who suffered from fungal meningitis. While 19 samplings using conventional cerebrospinal fluid (CSF) cultures failed to detect any organisms, fungal DNA was identified in the CSF by a new polymerase chain reaction (PCR)-based method. The patient recovered without any aequelae after treatment with a combination of antifungal agents, interferon-y and granulocyte infusions. Thih case report demonstrates that fungal meningitis must be included in the differential diagnosis of infections in CGD patients and that the PCR-based detection of fungal DNA is a powerful tool for diagnosis. Key wordschronic granulomatous disease. fungal meningitis, PCR-based diagnosis Chronic granulomatous disease (CGD) is an uncommon inherited disease characterized by severe recurrent pyogenic infections that begin in childhood.' Phagocytes from patients with CGD exhibit defective killing of ingested microorganisms because of deficient production of superoxide and related inicrobicidal oxidants by the NADPH oxidase. The active oxidase is composed of multiple components that catalyze the electron transfer from NADPH to oxygen and it is now recognized that CGD can result from genetic defects in at least four different oxidase polypeptides. The most common form of CGD is X-linked and is due to mutations in the gene for a 91 kDa membrane glycoprotein (gp91-phox).' Patients with CGD have frequent bacterial and fungal infections that are often life-threatening. Although Staphylococciis aureu.s and Aspergillus species are major pathogens, the spectrum of infecting organisms is wide, and includes those generally considered to be commensals. Continuous prophylaxis with trimethoprim-sulfamethoxazole has been shown to be effective in reducing the frequency of bacterial infections. but the efficacy of prophylactic use of anti-fungal agents is not established.'