Mariko Maezawa

Steroid-responsive painful ophthalmoplegia in childhood: sphenoid sinusitis presenting as Tolosa-Hunt syndrome.

A childhood case of painful ophthalmoplegia which responded well to steroids was described. Although the clinical features suggested Tolosa-Hunt syndrome (THS), serial magnetic resonance imagings (MRI) revealed ethmoid and sphenoid sinusitis in this 7-year-old boy. It was considered that multiple factors such as allergy, viral infection and swimming predisposed him to develop severe sinusitis and gave rise to his painful ophthalmoplegia.

Magnetic resonance signal intensity ratio of gray/white matter in children: Quantitative assessment in developing brain

Magnetic resonance imaging (MRI) findings in 87 children with various clinical entities were used to determine the signal intensity ratio of gray/white matter in T1- and T2-weighted images using a 1.5 T MR Scanner. Signal intensity ratio changes in both T1- and T2-weighted images correlated well with advancing age (y = 0.9349-0.001575, r = -0.584, P < 0.0001 in T1-weighted images; y = 0.9798 + 0.002854, r = 0.723, P < 0.0001 in T2-weighted images), but the correlation was more linear when we included only normally developed (34) children (y = 0.9689-0.001967, r = -0.654, P < 0.0001 in T1-weighted images; y = 0.9882 + 0.002965, r = 0.747, P < 0.0001 in T2-weighted images). Abnormal ratios were observed in patients with congenital hydrocephalus, inherited metabolic diseases and cerebral palsy. Although the gray/white matter differentiation would not delineate the myelination itself, measurement of the signal intensity ratio of gray/white matter is a practical way to evaluate delayed myelination in a busy MR center.

Aberrant pancreatic tissue causing pyloric obstruction

Only six cases of aberrant pancreatic tissue causing pyloric obstruction have been reported [1–6] thus far. We report a 27-day-old female infant with aberrant pancreas at the pylorus who showed signs similar to those of hypertrophic pyloric stenosis. Initial pyloroplasty did not relieve the obstruction, which required antrectomy and reanstomosis 8 days later.

A clinical study of the effectivenss of vitamin B6 for the prevention of repeated convulsion during one febrile episode

and nonrecurrence groups. Seizures recurred at least once in six the potential clinical applications of vitamin B6. Our study was patients (11.5%) receiving diazepam and in 17 patients (48.0%) designed to assess the clinical effectiveness of vitamin B6 for the receiving chloral hydrate (P < 0.005). prevention of repeated convulsions during one febrile episode. Conclusion. Intermittent rectal diazepam for the prevention of febrile convulsion recurrence was more effective than rectal chloral hydrate.

Rubella encephalitis demonstrated by magnetic resonance imaging

mg/day (27.6 mg/kg/day) and the serum level, 2 h after the morning dose, was 10.0 pg/dl. Other laboratory findings included: serum total cholesterol, 246 mg/dl (normal range for infants, 45-182); triglyceride, 180 mg/dl (32-99); LDL, 806 mg/dl (165-465 for adults); total ketone bodies, 1917 pmol/l; 3-OHBA, 1551/2 pmol/l; ketone body ratio, 4.23; T3, 106 ng/dl (lOO-260): T4,6.2 pg/dl(7-15); and TSH, 4.0 pU/ml (0.2-4.0 in adults). Ketonuria was not remarkable. No clinical manifestation commonly associated with ketonuria, such as anorexia, vomiting or lassitude, was present. There were slight increases in GOT and GPT and the ketonemia returned to the normal range with the tapering of CBZ. Discussion: The epileptogenic focus in this case lies in the left fronto-temporal area. CBZ did not work at all even at a high dosage, the seizures being aggravated conversely. A few recent papers reported the effect of CBZ on the serum cholesterol level. The degrees of increase in serum cholesterol and lipids in our case were very great. It is clear that hyperlipidemia and ketosis of great degree were repeatedly observed at the time of the administration of a high dose of CBZ, and gradually decreased and eventually normalized along with the tapering of CBZ.

Guidelines for Discontinuation of Antiepileptic Drugs for Childhood Absence Epilepsy and Rolandic Epilepsy

It is generally considered that most patients with childhood absence epilepsy (CAE) and rolandic epilepsy (RE) have outgrown epilepsy by the age of 20. However, there are a few reports about a rational approach to the treatment of these epilepsies. We studied 56 patients with CAE and 60 patients with RE who were followed up for more than five years and were over 14 years of age at the last visit to make some guidelines when to discontinue antiepileptic drugs (AEDs).

Long-Term Prognosis of Childhood Epilepsy–Follow-Up Until Adulthood in Patients Whose Age of Onset Is Under 10

In patients suffering from epilepsy, the symptoms usually start in childhood and last until adulthood. During the long period of treatment, patients usually change their clinics due to transference and various other reasons. So it is difficult to follow-up these patients in the same clinic for a long time, making it difficult for us to carry out detailed investigations of a long-term prognosis in patients with epilepsy. To overcome this problem, only adult patients who have been followed-up at our neuropediatric outpatient clinic since childhood have been selected for this investigation of the long-term prognosis in patients with epilepsy.

Long-term prognosis of childhood epilepsy - Follow-up until adulthood in patients whose age of onset is under 10 years.

当院およびその関連施設の小児科外来に受診中の患者で, (1) 10歳未満てんかん発症, (2) 10年以上外来通院, (3) 現在20歳以上, の3条件のすべてを満足する例を社会適応良好 (A) 群63例, 社会適応障害 (B) 群36例に分け, てんかんの長期予後を検討した。A, B群の発作 残存率はそれぞれ11%, 56%である。大学に在学中のものはA群22例, B群1例, 就職しているものは, A群37例 (59%), B群23例 (64%) で, 8群は全例単純な作業に従事している。社会適応の予後に関連する因子は, 初発年齢, 基礎疾患, 神経・発達異常, てんかん類型, 発作頻度であり, けいれんの予後に関連する因子もほぼ同様である。

Prognosis of nonfebrile generalized convulsions in children. With special reference to the factors related to their prognosis.

小児期における無熱性全般けいれん発作71例の予後を検討し, 以下の結果を得た。1) 発作の消失したものは, 総数71例中50例 (70.4%), 正常な精神運動発達・精神発達を示したものは, 64例 (90.1%) であった。2) 経過中, 他の発作型を認めたものは, 10例 (14.1%) で, 内訳は, 単純部分発作3例, 複雑部分発作6例, 複雑部分発作+非定型欠神発作1例であった。3) 発作の予後に関与する因子として,i) 精神運動発達遅滞・精神遅滞の合併 (p<0.01)ii) 治療開始までの期間 (p<0.05)iii) 30分以上持続した無熱性全般けいれん発作の既往 (p<0.05) 3つが指摘された。