[Three pediatric cases of group A coxsackievirus-associated encephalitis/encephalopathy].

We report three pediatric cases of group A coxsackievirus-associated encephalitis/encephalopathy. A 4-year-old girl with coxsackievirus A6 infection presented with an exanthem on her upper body on the fourth day of fever. The following day, she developed an episode of convulsions, and subsequently experienced sudden cardiopulmonary arrest. A head computed tomography scan revealed severe brain edema. Two patients with neurological sequelae had initially presented with status epilepticus that required intravenous barbiturate treatment. Both cases had high-signal-intensity lesions on their diffusion-weighted brain magnetic resonance images. A 5-year-old girl with subcortical white matter lesions was diagnosed with acute encephalopathy with febrile convulsive status epilepticus, and a 2-year-old boy with bilateral hippocampal lesions was diagnosed with parainfectious limbic encephalitis. These findings indicate that group A coxsackievirus is a causative agent of pediatric encephalitis/encephalopathy; moreover, the prognosis may be poor in some cases.

[Case of acute brainstem and striatal encephalopathy associated with Mycoplasma pneumoniae infection].

Acute encephalopathy with bilateral striatal necrosis (EBSN) is a rare reversible neurological disease characterized by an abrupt onset following an acute infectious disease and by severe extra-pyramidal signs associated with striatal lesions. Brainstem involvement is rarely observed in this disease. We report a 10-year-old boy who had EBSN associated with Mycoplasma pneumoniae. He became lethargic after acute bronchitis. A few days later, he showed extra-pyramidal signs, pyramidal signs and overactive urinary bladder symptoms. Cranial T2-weighted and diffusion-weighted magnetic resonance imaging (MRI) demonstrated high-signal intensity in the bilateral striatum and substantia nigra. These symptoms improved soon after the administration of L-dopa in the acute phase. The effects of corticosteroids were not apparent in the acute phase. The serum particle agglutination titers against Mycoplasma pneumoniae determined on admission, the 12th hospital day and 2 months later were 1:2,560, 1:2,560 and 1:320, respectively. Two years later, a mild tic was observed. A mild atrophy was noted in the bilateral basal ganglia, but not in the substantia nigra on cranial MRI. This case is the first reported one with EBSN with the presence of an overactive urinary bladder, which could possibly caused by loss of dopaminergic inhibition.

Early infantile manifestations of incontinentia pigmenti mimicking acute encephalopathy.

OBJECTIVE: We retrospectively reviewed six patients with incontinentia pigmenti (IP) who had encephalopathic manifestations during early infancy. METHODS: We enrolled six patients who met the following criteria from the mailing list of the Annual Zao Conference: (1) diagnosis of IP; (2) encephalopathic manifestations with reduced consciousness and clusters of seizures by 6 months of age; and (3) no evidence of central nervous system infection or metabolic derangement. RESULTS: The onset of the encephalopathic events was within the first 2 months of life in all but one patient. All had clusters of focal clonic seizures. The duration of seizures was typically 5 min. The seizures ceased within 5 days in all patients. Various degrees of reduced consciousness were observed in association with the frequent seizures. Diffusion-weighted imaging during the acute phase showed reduced water diffusion in the subcortical white matter, corpus callosum, basal ganglia, thalami, and internal capsule in two patients. Scattered subcortical white matter lesions were observed on fluid-attenuated inversion-recovery images in two patients. CONCLUSIONS: The encephalopathic manifestations in patients with incontinentia pigmenti were characterized by seizure clusters and reduced consciousness, albeit of relatively short duration. Magnetic resonance imaging abnormalities were predominant in the subcortical areas in most patients.

[A case of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), diagnosed in the age of puberty].

We report a 13-year-old boy with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) at an early stage. He showed migraine, cognitive deficits, depressive episodes and areas of white matter hyperintensity on MRI. There were no first-degree relatives accompanied with similar symptoms. T2-and fluid-attenuated inversion recovery (FLAIR)--weighted brain MRI revealed areas of apparently symmetric high intensity in the deep white matter and periventicular caps. On ultrastructural studies of the biopsied skin, there were free granular osmiophilic materials (GOM) between vascular smooth muscle cells in the cutaneous vessels. But there were no excavations in the cell membranes that contained GOM. On immunostaining with Notch3 monoclonal antibodies, granular staining was not observed in vessels of the skin. No mutation was detected on DNA analysis of the Notch3 gene (exon 4 and part of exon 5) in peripheral leukocytes. Although the frequencies of migraine episodes and depressive episodes decreased with amitriptyline and ibuprofen, the cognitive deficits (delayed-recall impairment) and areas of white matter hyperintensity on MRI have been unchanged for the past four