Haung-Chi Lin

Intracranial arachnoid cysts in children: related signs and associated anomalies

Intracranial arachnoid cysts are benign development anomalies that may be clinically asymptomatic. The authors describe 30 children with intracranial arachnoid cysts in terms of clinical manifestations and relations to the associated brain anomalies or lesions. The mean age at onset of clinical manifestations was 4 years, 7 months (range 1 day to 14 years). The mean age at diagnosis was 6 years, 2 months (range 10 days to 16 years). Most patients with nonprogressive symptoms, such as seizures and headache, had focal epileptiform discharges on electroencephalogram, and they benefited from antiepileptic drugs. Surgery resulted in only partial reduction in both cyst size and seizure frequency in patients with intractable seizures, and it also failed to improve some neurologic signs, such as sexual precocity or cranial neuropathy resulting from long-term compression of arachnoid cysts. We conclude that the only absolute indication for surgery is the presence of progressive hydrocephalus or intracranial hypertension. The associated anomalies or lesions include brain tumors, giant nevocellular nevi, achondroplasia, microphthalmia, intracystic hemorrhage, dysgenesis of the corpus callosum, and heterotopia.

Paroxysmal nocturnal hemoglobinuria presenting as moyamoya syndrome

We report an 11-year-old girl who has paroxysmal nocturnal hemoglobinuria (PNH) and was admitted because of recurrent cerebrovascular accidents (CVA) and intermittent hemoglobinuria. Internal carotid angiography revealed bilateral typical moyamoya patterns. Although CVA due to arterial thrombosis may occur in PNH, the basal moyamoya vessels were never mentioned in case reports yet. The moyamoya syndrome has been reported in a variety of diseases and represents the nonspecific response to an impairment of arterial flow at specific sites in the brain. Our case discloses that PNH may present as moyamoya syndrome.

Estimation of prevalence and incidence of infantile spasms in Taiwan using capture–recapture method

PURPOSE To estimate the prevalence, incidence, and case-fatality of infantile spasms (IS) in Taiwan. METHODS A retrospective cohort of patients with IS was obtained from one medical center to identify 69 IS cases from 1985 to 1997. This cohort, in conjunction with the claimed data from the National Health Insurance, was used to estimate the prevalence and incidence of IS by capture-recapture design, taking the case-fatality of IS into account. RESULTS The prevalence rate of IS for aged 0-9 years was 0.046 per thousand. The incidence rate was estimated as 6 over 100,000 per year in Taiwan. Of the 69 IS cases, 8 deaths were ascertained. The case-fatality rate was 11.6%. The leading cause of death was status epilepticus. CONCLUSIONS We have demonstrated an efficient method to estimate the incidence and prevalence rates of IS in Taiwan. Our results help to make a clear understanding of the disease burden of IS in this society.

Risk factors associated with infantile spasms: a hospital-based case-control study in Taiwan

We investigated the risk factors associated with infantile spasms (IS) by a hospital-based case-control study in Taiwan. Twenty-five patients with IS were recruited from one medical center (National Taiwan University Hospital) between 1990 and 1997. Based on a close-structured questionnaire, standardized interviews were carried out to obtain information on risk factors associated with IS. Two comparison groups are used, including a total of 106 subjects in the Disease Control group, and 139 subjects in the Normal Control group. Unconditional logistic regression is used to calculate odds ratios (OR) and 95% confidence interval (CI). Univariate analysis revealed gestational age, congenital cerebral anomalies, tuberous sclerosis (TS), asphyxia, febrile seizure, and developmental delay (before onset of spasm) were at increased risk of IS. After adjustment of multiple risk factors through unconditional logistic regression, significant risk factors for IS include congenital cerebral anomalies, TS, asphyxia, postterm, and developmental delay were highly associated with IS. The risk factors of IS may closely relate to underlying neurological abnormalities. Our results are consistent with the previous findings.

Strokes in Children: A Medical Center-based Study

Stroke is an important cause of mortality and morbidity in children. Cases of pediatric stroke admitted to National Taiwan University Hospital from January 1985 to December 1995 were reviewed. Patients whose stroke was obviously caused by premature birth, birth trauma or head injury were excluded. Totally 65 patients were enrolled, including 37 boys and 28 girls. Their ages ranged from birth to 18 years old. They were classified into two groups: ischemic stroke (38 patients) and hemorrhagic stroke (27 patients), according to the pathogenesis. The ages of onset, clinical manifestation, underlying diseases and treatment of these two groups were systematically analyzed. The major presenting symptoms of both ischemic and hemorrhagic strokes were motor deficit (65.8%) and consciousness disturbance (55.6%). A wide variety of diseases predisposing to strokes was identified. The major causes of hemorrhagic stroke were vascular malformation and oncologic conditions, with the latter, the most frequently encountered underlying diseases associated with childhood ischemic stroke. The mortality rate for hemorrhagic stroke was 37% and, for ischemic stroke, 21.1%. There was male predominance in pediatric stroke. Although the clinical symptoms and signs might provide some guidelines to differentiate between hemorrhagic and ischemic strokes, neuroimaging studies were crucial to more exact diagnosis. A variety of diseases may contribute to pediatric stroke. Early diagnosis determine treatability, then aggressive treatment are important.

Transplantation for Adrenoleukodystrophy with HLA-A and B Nonidentical Paternal Marrow: Report of One Case

We report the result of allogeneic bone marrow transplantation (BMT) in a 14-year-old boy who was neurologically severely involved with the childhood form of adrenoleukodystrophy (ALD) and received marrow from his HLA-A and B nonidentical, MLC-nonreactive paternal donor without T-cell depletion processing. Bone marrow transplantation corrected the excess content of very long chain fatty acid in plasma but did not arrest the deterioration of the neurological status during 3.5-year post-transplant follow-up period. Since partially matched or unrelated donors have been applied to clinical BMT successfully with current new techniques, ALD patients will have a better prognosis when they are transplanted in status of mild and early involvement. Our first experience may be helpful in more trials of BMT for genetic leukodystrophy in Taiwan.