A Fatal Case of Acute Encephalopathy in a 8-year-old girl from a Pandemic of Influenza A( H1N1) in 2009

We report on a case in which a child died from acute encephalopathy associated with Influenza A( H1N1)pdm. The case pertains to an 8-year-old girl, who was diagnosed with influenza A on the previous day andwas prescribed zanamivir hydrate. She experienced abdominal pain and delirium the next morning, so shewas referred to our hospital. Her level of consciousness at the time of consultation was JCS 200. In the brainCT scan, a swollen brain stem and bilateral middle cerebral artery were depicted. She was in a state of respiratoryfailure, and was admitted to the intensive care unit. She was diagnosed with acute encephalopathycaused by Influenza A, and therapeutic brain hypothermia as well as steroid pulse therapy were performed;however, she died on the third day after hospitalization from multiple organ failure

Image Analysis with the Brain Easy Analysis Tool (BEAT) Method in Cases of Encephalomalacia Following Shaken Baby Syndrome

Brain easy analysis tool( BEAT) is newly released software to calculate composite images both MRI andSPECT on computer graphics. At first, we herein report two cases with shaken baby syndrome associatedwith multicystic encephalomalasia diagnosed based on MRI. Next, we created fusion MRI-SPECT imagesusing BEAT. The result of composited images was not only well recognized in anatomical visually but alsoeasy to explain data to patients. This report is the second case report with this software called BEAT

Clinically Mild form of Joubert Syndrome-related Disorder in a 7-year-old Female:A case report

Joubert syndrome-related disorders (JSRD) is a very rare syndrome observed with agenesis of the vermis,episodic hyperpnea, abnormal eye movements, and cerebellar ataxia and mental retardation. There have been many reports on the image-based diagnosis using MRI/CT, but not many clinical reports on the intelligence of subjects with JSRD. We herein report a clinically mild form of JSRD in a 7-year-old female. The patient did not have any clinical abnormalities in the neonatal period.She initially visited the neurology outpatient department at 6 months of age because her neck was still unstable. A brain MRI was conducted to assess symptoms of abnormal eye movements and body trunk cerebella rataxia, and she was diagnosed with JSRD based on the observation of agenesis of the vermis and characteristic molar tooth signs. Regarding her motor development, she was able to hold her head up at 7months of age, and was able to sit up at 1 year and 2 months old. She underwent rehabilitation, and a wide base cerebellar gait was observed when she was 5 years old. In addition, at 5 years of age, she could intermittently speak two-word sentences, draw pictures with an understanding of color, and was able to engage in actions such as throwing a ball. At 6 years of age, her conversation became better. She entered elementary school at 7 years of age, was able to go up and down stairs, read words, do single-digit addition, and write many Chinese characters with a pencil. The intellectual dysfunction of subjects with JSRD is generally moderate to severe. Thus, this case was diagnosed to be a clinically mild form of JSRD, because the patient exhibited limited effects on her intelligence

Anterior Spinal Artery Syndrome in 13-year Old boy:A Case Report

We herein report a rare case of a child patient with anterior spinal artery syndrome. The patient presentedwith mobility impairment in the lower limbs after suffering from a common cold. Temperature perceptionand pain perception were lost in both sides below the level of the fourth thoracic spine. In a thoracicspine MRI, abnormal signals were detected in the ventral side of the spinal cord below the Th3 level, thusleading to our diagnosis of anterior spinal artery syndrome. Gamma globulin therapy and steroid pulse therapywere applied concomitantly but no obvious effects were obtained. Continuing treatment with rehabilitationstarted for one month. Four months later, he was able to maintain a standing position and walk using awalking stick. The loss of temperature perception and pain perception in both sides below the Th4 remained.In the present case, movements in daily life became possible through the effects of rehabilitation. However,an impairment in the patient\u27s temperature and pain perception remained, and thus close attention is requiredfor various disorders and in daily life

Lennox-Gastaut Syndrome Associated with Unilateral Hemispheric Porencephaly

We report an 18 year-old male with a hemispheric large porencephaly who demonstrated symptomaticWest syndrome and then developed into Lennox-Gastaut syndrome. An electroencephalogram showed anasymmetrical high voltage slow spike and wave discharges only on the right side as a side of porencephalichemisphere. Interestingly, on the opposite side of the porencephalic hemisphere, an electroencephalogramshowed a low amplitude background activity in general. In this unique features both right side hemisphericporencephaly and asymmetric high voltage slow spike and waves due to Lennox-Gastaut syndrome, theporencephalic hemisphere due to cortical parenchymal loss may be more affected than the other hemisphereby epileptiform discharge in this patient

Long survival case of trisomy 13 mosaicism in a 7-year-old male

Trisomy 13 is a complication of various congenital abnormalities of the heart, brain, etc. Regarding the vitalprognosis, many die within a year from birth. We herein report on the case of a 7-year 1-month-old boywith mosaicism trisomy 13 with the two considerations mentioned below as the cause for long-term survivalin this case. The first is that there were no serious associated abnormalities to the heart, brain, or otherorgans, and the second is that a tracheotomy was carried out on a repeated respiratory infection with respiratoryfailure. Long-term in-home care was possible for the child and he was observed playing with toys bytouching them. Trisomy 13 has a poor vital prognosis, so some argue that active treatment should be restrained.However, for cases with no severe associated abnormalities, long-term survival may be possiblewith active treatment

Three Cases of Shaken Baby Syndrome without a History of Shaking

Three cases of Shaken Baby Syndrome (SBS) without a history of abusive shaking have been reported.The reason why SBS without intentional shaking as follows;case 1was throwing, case 2 was dropping, case3 was unknown. For all 3 reported cases, attending physicians suspected the SBS from the presence of subduralhematoma and fundus hemorrhage. All 3 cases occurred at home, and the parents had no knowledgeof SBS. After a detailed interview, the diagnosis of SBS without a history of intentional shaking was made.Although the mechanism in detail was unclear in 3 cases, these SBS may happen by the difference betweenacceleration and the deceleration during the throwing and dropping movement;similar to intentional shakingthat causes of common SBS.For subdural hematoma and fundus hemorrhage of unknown cause, it is important to conduct an interviewwith the possibility of unintentional SBS in mind, without the occurrence of abusive shaking. In addition,attention should be paid to both pediatrician and parents about the risk of SBS in the care of infant inthe everyday life

Stress in Children with Pervasive Developmental Disorders

The purpose of this study is to examine urinary 17-hydroxycorticosteroid (OHCS) excretion as a measureof stress response in controls and in children with pervasive developmental disorders (PDDs) accompaniedby mental retardation( MR, intelligence quotient[ IQ]<70)( low-functioning PDD:LFPDD) or notaccompanied by MR( IQ≧70)( high-functioning PDD:HFPDD). Prospective, non-randomized study of 24children with LFPDD ─ 17 with autism and seven with PDDs not otherwise specified( PDDs-NOS) ─ and14 children with HFPDD ─ 11 with PDDs-NOS and three with Asperger\u27s disorders. Urinary 17-OHCSwas measured between June 2002 and June 2004. In 21 of 24 LFPDD subjects and 8 of 14 HFPDD subjectsa second measurement was taken 6 to 12-months after the first, after the subjects had received interventionfor developmental disorders. Baseline urinary 17-OHCS levels in both LFPDD( p<0.0005) and HFPDD(p<0.005) were significantly higher than in controls. LFPDD subjects had significantly greater (p<0.05)levels of this stress hormone than did HFPDD subjects. Levels of 17-OHCS in both LFPDD (p<0.05) andHFPDD (p<0.05) decreased significantly after 6 to 12 months of intervention. PDD subjects showed higherlevels of 17-OHCS than controls and showed a significant reduction in the level of stress indicator after theyhad received intervention for developmental disorders

ドッキョウ イカ ダイガク ショウニカ ニオケル ザイタク ジンコウ コキュウキ リョウホウ ノ ゲンジョウ

獨協医科大学小児科学における2005年6月から2008年6月の3年1 ヶ月間の在宅人工呼吸療法の現状について報告した.導入された在宅人工呼吸器の延べ台数は合計10台で,稼働台数は毎年増加傾向を認めた.当院で本療法を導入した児の基礎疾患では,重度の中枢神経疾患に慢性呼吸不全を併発した例が多いことが特徴的であった.院内で在宅人工呼吸療法を用いた在宅管理のトレーニングを導入した後,実際に在宅治療に移行できた症例は10例中6例であり,今後の課題が残された.今後,在宅医療件数は増加することが見込まれている.円滑な在宅医療の実現のためには患児はもとより介護する家族に対する早急な社会的な支援体制の構築が望まれる.We herein report on the current state of home-care typeof mechanical respirator treatment implemented over 3years and 1 month from June 2005 to June 2008 in the pediatricdepartment of the Dokkyo University School of Medicine.A total 10 home-care mechanical respirators were introduced,and there was a trend involving an increaseevery year. The underlying diseases of the children forwhom this treatment was introduced were characterized bymany cases in which a severe central neurological diseaseoccurred in combination with chronic respiratory failure.Since introducing this treatment in our hospital, there havebeen few cases that could be smoothly transferred to hometreatment, and further investigations therefore need to beconducted.In the future, it is expected that number of home treatmentwill increase, and the development of immediate socialsupport system for, not only the affected infants, but alsothe families taking care of them is desired

ショウノウ テイケイセイ オ ミトメタ チョウ テイシュッセイ タイジュウジ ノ ジョジ

小脳低形成は失調を起こす先天的な脳形成異常である.近年,小脳低形成と超低出生体重児の関連性が指摘されている.我々は運動発達の遅れを契機に小脳低形成と診断した小児例を報告する.症例は1 歳8ヶ月の女児.在胎23 週5 日,出生体重555 g の超低出生体重の分娩歴がある.出生後,呼吸障害にて日齢99 まで人工呼吸器管理を要した.日齡173,後遺症なく退院.1 歳6 ヶ月で座位と立位保持が不安定性であった.脳MRI を行い小脳低形成と診断した.児はその後リハビリテーションを開始し歩行を獲得した.低出生体重児で座位保持困難などの運動発達遅滞が見られた場合,正期産よりも小脳低形成の合併が多いことを考慮し,早期にMRI を施行することが望ましい.Congental cereballer hypoplasia is a well known cause oftruncal ataxia. In recent years, several report showed a correlationbetween very low birth weight infant and hypoplasiaof cerebellum. Here we report a patient with cerebellarhypoplasia who was born with 23 weeks gestational age,weighting 555 g. She was immediately treated with artificialventilation until 99th day of her age due to respiratory failure.At the 173th day, she was discharged from the neonatalintensive care unit with no apparent sequela. At oneyear and 3month-old, she could not walk and keep sittingalone. Brain MRI showed hypoplasia of both cereballarhemisphere and vermis. The diagnosis of congenital cerebellarhypoplasia was made and rehabilitation therapy wasstated. Physicians should recognize the high prevalence ofcerebellar hypoplasia when examine very low birth weightinfant with developmental motor delay, and brain MRIstudy is useful to detect this condition