Recurrent bacteremia with different strains of Streptococcus pyogenes in an immunocompromised child.

We report an immunocompromised child who experienced two episodes of bacteremia due to Streptococcus pyogenes. Random amplification of polymorphic DNA profiles, emm genotypes, superantigen profiles, antimicrobial susceptibility, and resistance-related genes were investigated, and the results showed different profiles between the two isolates. This is the first report describing recurrent bacteremia caused by different strains of S. pyogenes.

[Acute encephalitis/encephalopathy associated with transient involuntary movement and limbic dysfunction in the recovery phase].

We report 3 patients aged 3 - 4 years who presented with impaired consciousness and status epilepticus or a cluster of seizures, followed by transient involuntary movements and limbic dysfunction in the recovery phase. In all the patients, the involuntary movements were observed mainly on the left side and consisted of dystonia, athetosis, chorea, facial myoclonus, and oral dyskinesia. The patients also showed stereotypic movement, oral tendency, visual agnosia, and emotional disturbance, which suggested limbic dysfunction resembling Klüver-Bucy syndrome. Single-photon emission computed tomography (SPECT) revealed hypoperfusion of the unilateral basal ganglia and the adjacent frontal and temporal lobes. No obvious lesions were observed on brain MRI in the acute phase in 2 patients, who recovered completely during the follow-up period. However, the last patient, who had abnormalities with regard to the limbic system and subcortical white matter on diffusion-weighted image in the acute phase, exhibited mental retardation, epilepsy, and persistent oral tendency during the follow-up period.

Effect of corticosteroids in a twin child with idiopathic localization-related epilepsy.

Corticosteroids have been used only in the treatment of special epileptic syndromes or epileptic encephalopathies, such as infantile spasms. We report an antiepileptic effect of corticosteroids that were used for treatment of nephropathy in a monozygotic twin child with idiopathic localization-related epilepsy (I-LRE). The patient and her monozygotic twin sister exhibited repeated partial seizures at two years of age and electroencephalogram (EEG) showed focal spikes in the occipital area and, on other occasions, the centro-parietal areas. After oral antiepileptic drugs were started, the twins still exhibited occasional seizures. The patient had IgA nephropathy at four years of age and intravenous methylprednisolone and oral prednisolone were administered. Her seizures and epileptiform discharges on EEG disappeared, while her sister continued to have seizures and EEG abnormalities. When the dose of oral predonisone was reduced, the seizures relapsed and EEG again revealed focal spikes. We conclude that corticosteroids exhibit efficacy towards seizures and epileptiform discharges on EEG in patients with I-LRE without epileptic encephalopathies.

Subacute encephalopathy: clinical features, laboratory data, neuroimaging, and outcomes.

We sought to clarify the clinical, laboratory, neuroradiologic, and neurophysiologic features of the "subacute" subtype of encephalopathy. We retrospectively identified nine patients with subacute encephalopathy out of 97 patients diagnosed as manifesting acute encephalopathy. Neurologic symptoms, clinical course, laboratory data, neuroradiologic and electroencephalographic findings, and outcomes were reviewed through medical records. The median age of patients was 44 months (range, 28-156 months). The initial neurologic sign was a brief seizure in 4, a prolonged seizure in 3, delirious behavior in 1, and a loss of consciousness in 1. Loss of consciousness the next day was subtle in 4, and mild in 5. However, a worsening of consciousness was observed 3-7 days after onset. Laboratory data were unremarkable, and electroencephalography during the early phase found abnormalities in 4 of 7 patients. Magnetic resonance imaging revealed no abnormalities during the early phase, and mild cortical atrophy during the late phase. All but one patient had various degrees of neurologic sequelae. Subacute encephalopathy was characterized by a delayed worsening of neurologic symptoms, mild cortical atrophy on late magnetic resonance imaging, and poor neurologic outcomes. Recognition of this type of acute encephalopathy is important, and a method to promote early diagnosis is desirable.

Phenytoin desensitization in a child with symptomatic localization-related epilepsy.

We reported a child with refractory partial seizures successfully managed by clinical desensitization to phenytoin. The patient had ischemic brain lesions due to cardiopulmonary arrest at 39 weeks of corrected age. He had complex partial seizures refractory to several antiepileptic drugs since 4 years of age. At 8 years 1 month of age, phenytoin was first administered. Fever and maculopapular rashes appeared at 10 days after phenytoin initiation, and then the drug was discontinued. At 8 years 8 months of age, desensitization was attempted because of refractoriness of seizures to drugs other than phenytoin. Desensitization was started at 1mg daily, and then the dose was doubled every week. His seizures were controlled by 150mg/day of phenytoin in combination with primidone. No problems have been observed during desensitization.

[Drug-induced hypersensitivity due to phenytoin].

This study aimed to clarify factors associated with intravenous administered phenytoin-induced hypersensitivity reaction. The incidence of hypersensitivity was significantly more frequent in boys than in girls (P < 0.05). Patients with hypersensitivity were relatively younger than those without hypersensitivity, although the difference was not statistically significant. There was no relation between the initial dose or maximum blood level of phenytoin and the occurrence of hypersensitivity. The initial serum level of phenytoin was significantly lower in patients with hypersensitivity than in those without hypersensitivity (P < 0.05), whereas the total dose of phenytoin was relatively larger in patients with hypersensitivity than those without. Reactivation of human herpes virus-6 was not recognized in all 3 patients in whom virological examination was performed using real-time polymerase chain reaction.

Diffusion-weighted MRI for early diagnosis of neonatal herpes simplex encephalitis.

AIM: To determine the early changes and evolutions of brain diffusion-weighted imaging (DWI), and analyze prognostic factors of the early changes among patients with neonatal herpes simplex encephalitis (NHSE). METHOD: We selected patients who developed encephalitis by 28 d after birth; had herpes simplex infection; and who underwent magnetic resonance imaging, including DWI, ⩽7 d of symptom onset. Thirty-two DWI scans between 0 and 28 d after onset in 13 patients and the clinical data were recruited. The distribution, evolution of the lesions, and neurological outcome were analyzed. RESULTS: DWI frequently showed multiple cortical lesions in both hemispheres in the early period and both hemispheres on DWI (8/9 scans at ⩽48 h, 7/7 patients). As time from onset increased, the cortical lesions tended to coincide with subcortical white matter lesions beneath the initial cortical lesions (p<0.01). Lesions from the cortex extended to the subcortical white matter in 7 patients. Deep cerebral lesions, involving basal ganglia, internal capsules, thalamus, were also found in 9 patients ⩽7 d of onset. The distributions of deep cerebral lesions (none/unilateral/bilateral) ⩽7 d of onset showed significant correlations with neurological prognoses (gross motor functions: p<0.01; developmental or intellectual quotient scores: p<0.01). INTERPRETATION: Cortical lesions were main findings of DWI in NHSE in the early period. Bilateral deep cerebral lesions ⩽7 d were highly indicative of poor motor and cognitive outcomes.

Allergen-specific T-cell response in patients with phenytoin hypersensitivity; simultaneous analysis of proliferation and cytokine production by carboxyfluorescein succinimidyl ester (CFSE) dilution assay.

BACKGROUND: Phenytoin can induce diversified adverse reactions including generalized eruptions and the hypersensitivity syndrome. Delayed-type allergic mechanisms have been postulated to underlie these reactions. The tests most widely used to detect T-cell sensitization to drugs are the patch test and the lymphocyte transformation test (LTT), but their sensitivity is not sufficient. Simultaneous assessment of both the frequencies and the cytokine-producing phenotypes of allergen-specific T cells has become possible with the recently introduced carboxyfluorescein succinimidyl ester (CFSE) assay. METHODS: Seven patients who presented with phenytoin-induced maculopapular exanthema with and without fever were included in this study. Peripheral blood mononuclear cells (PBMCs) were labeled with CFSE and cultured with phenytoin for seven days. The cells were stained with anti-CD4 and cytokine-specific monoclonal antibodies (MoAbs), and analyzed with FACSCalibur. RESULTS: The phenytoin-specific proliferation of CD4+ cells in patients was significantly higher than in the four controls exposed to phenytoin, and in seven healthy children with no previous phenytoin intake. A significant difference in the percentages of CD4+ IFN-gamma+ cells between patients and the seven healthy children was observed. The sensitivity and specificity of proliferation were 100% and 90.9%, and those of IFN-gamma secretion were 71.4% and 100%, respectively. CONCLUSIONS: Phenytoin-specific proliferation may be detected with greater sensitivity by the CFSE dilution assay than the conventional LTT. The assay revealed that both CD4+ and CD4- T cells proliferated and produced IFN-gamma and TNF-alpha after stimulation with phenytoin. The CFSE dilution assay might be useful for the diagnosis and understanding of drug hypersensitivity.