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Volume 43, Issue 4
Displaying 1-15 of 15 articles from this issue
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  • A Strategy to Treat Neonatal Hypoxic Encephalopathy Using Glial Cell Line-Derived Neurotrophic Factor
    Shinji Katsuragi, Tomoaki Ikeda, Tsuyomu Ikenoue
    2011 Volume 43 Issue 4 Pages 265-272
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.265
    JOURNAL FREE ACCESS
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      Hypoxic-ischemic encephalopathy is one of the main causes of neurological disabilitles. It has been reported that the infarcted area can be reduced by injection of glial cell line-derived neurotrophic factor (GDNF) into the brain parenchyma after a hypoxic/ischemic insult in neonatal rats. We have shown that GDNF is expressed in neuronal and non-neuronal cells throughout all regions of the developing rat brain. We developed a system for the delivery of a constant supply of glial cell line-derived neurotrophic factor to the brain via implantation of GDNF secreting cells directly into the brain parenchyma. The aim of this study was to examine the neuroprotective effect of GDNF using this delivery system. We implanted a capsule containing GDNF secreting cells in 7 day old Wistar rats, and two days later, they underwent hypoxic-ischemic stress. The capsule provided strong neurological protection, as indicated by a reduction in the infarcted area and the severity of histological damage in the treated group compared with controls. We then investigated whether this new delivery method improved the long time learning and memory disability caused by hypoxic-ischemic stress. We examined the effect of implantation of the cells on three tasks: 1) eight arm radial maze task for short memory: 2) choice reaction time task for reference memory: and 3) water maze task for long term memory. In all of the three tasks, implantation of the GDNF capsule improved learning and memory disability. Glial cell line-derived neurotrophic factor treatment is effective not only in reducing brain damage but also in preventing learning and memory impairment following hypoxic-ischemic insult in neonatal rats.
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  • Efficacy of Botulinum Toxin Type A against Cervical Dystonia and Muscular Hypertonia in Persons with Severe Motor and Intellectual Disabilities with Respiratory Problems and/or Dysphagia
    Mina Yokoyama, Manami Honda, Yumi Okoshi, Tatsuo Masuyama, Yuji Iwasak ...
    2011 Volume 43 Issue 4 Pages 273-276
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.273
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      We administered intramuscular injections of botulinum toxin type A (BTX-A) in 11 persons with cervical dystonia (CD) and muscular hypertonia (MH). All patients had severe motor and intellectual disabilities (SMID). Furthermore, in 10 patients, SMID was accompanied by respiratory problems and/or dysphagia. Three patients received night nasal intermittent positive pressure ventilation and 3 had undergone tracheotomy: 5 patients had upper respiratory problems. Because of these complications, BTX-A dose was gradually increased in those patients until the desired effect was obtained (mean last dose, 6.8 u/kg/dose). All patients were clinically assessed with the Tsui scale before treatment with BTX-A. At 1, 2, 4, and 8 weeks after BTX-A injections, responses to the injections were assessed with the Tsui scale repetitively in all patients. Significant or mild improvements in the Tsui scale scores were observed in 8 patients without any severe adverse effects. In addition, some improvements in respiration and body weight gain were observed. We observed a reduction in the number of oral medications in 10 cases. Administration of BTX-A for the treatment of SMID has numerous benefits, not all of which can be explained by Tsui scale scores. BTX-A is safe and has potential for use in the treatment of CD and MH with respiratory problems and/or dysphagia.
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  • Postoperative Course of a Patient Undergoing Selective Dorsal Rhizotomy for Cerebral Palsy
    Mayumi Touyama, Jun Touyama, Atsuo Aguni
    2011 Volume 43 Issue 4 Pages 277-281
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.277
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      Selective dorsal rhizotomy (SDR) is a surgical technique for reducing spasticity associated with cerebral palsy (CP). In the present study, we investigated the changes of clinical symptoms before and after SDR in a child with CP undergoing functional training at the Okinawa Child Development Center.
      Total score on the Gross Motor Function Measure significantly improved compared to preoperative values at approximately six months, one year, and two years postoperatively. The level of spasticity also significantly decreased postoperatively compared to preoperative levels according to evaluation using the Ashworth scale and the modified Ashworth scale. Based on these findings, SDR was considered effective for reducing spasticity associated with CP. In addition, orthopedic surgery was performed after SDR in 47% of patients, indicating the need to further investigate the timing of SDR.
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  • Analysis of the Blood and Serum Biochemistry Findings in Patients Demonstrating Convulsion with Mild Gastroenteritis
    Yuki Tsujita, Hiroshi Matsumoto, Yasuko Nakamura, Shigeaki Nonoyama
    2011 Volume 43 Issue 4 Pages 282-284
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.282
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      We analyzed the blood cell count and serum biochemistry findings in patients demonstrating convulsion with mild gastroenteritis (CwG). As a control group, age matched patients presenting with only gastroenteritis during the same period were compared. The results showed significant differences between the two groups regarding such factors as the sex ratio, serum uric acid, and serum chloride levels. All CwG patients showed hyperuricemia (10.0±2.2 mg/dL, mean±SD). The patients in both groups showed similar levels of metabolic acidosis. The patients with CwG therefore have both hyperuricemia and metabolic acidosis, which may contribute to the pathogenic mechanism of CwG.
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  • Prognoses of Acute Encephalopathy
    Mana Kurihara, Toshitaka Kohagizawa, Manabu Yoshihashi, Chieko Iino, R ...
    2011 Volume 43 Issue 4 Pages 285-290
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.285
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      We investigated the prognoses of 103 children with acute encephalopathy at more than one year from the onset. The patients were divided into five groups according to the clinical courses during the acute stage: group 1: 1 case with metabolic disorder, group, 2: 24 with cytokine storms, group, 3: 68 with prolonged convulsion more than 30 minutes, group, 4: 5 with severe refractory status epilepticus, and group, 5: 5 with the main symptom of impaired consciousness. We checked the past histories, etiologies, severities of consciousness loss, complications and disabilities including higher cortical dysfunction in their medical charts. The average age of onset in all cases was 3 years, with the highest age of 6 years 5 months in group 4. Regarding the past histories, febrile seizures, asthma and theophylline medication were prominent though they were not significantly different. Regarding etiologies, influenza infection, 36 cases, and HHV-6 infection, 7 cases, were prominent though they were not significantly different. Complicating disabilities comprised mental retardation, 89.3%, higher cortical dysfunction, 77.7%, epilepsies, 68.9%, and motor disturbance, 27.2%. The severity of disabilities increased in the order of 1, 2, 3, 4, 5. Attention deficit and visiospacial disturbance were the main symptoms of higher cortical dysfunction.
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  • Paroxysmal Automatic Movements Mimicking Neonatal Seizures Induced by Midazolam
    Yumiko Ishizaki, Shinichi Watabe, Nobuyoshi Mimaki, Yoshio Arakaki, Yo ...
    2011 Volume 43 Issue 4 Pages 291-294
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.291
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      We have observed paroxysmal automatic movements including drum-beating and pedaling motions in three full-term neonates following intravenous bolus injections (0.1-0.3mg/kg/dose) or drip infusions (0.2mg/kg/h) of midazolam used for sedation. In one patient, abnormal movements were also induced by a bolus injection of midazolam during the EEG recording, and no change was revealed in the EEG during the episode. In another patient, abnormal movements were further worsened by an injection of diazepam. Interictal EEGs of all patients were normal. The clinical manifestations of these paroxysmal automatic movements and the mode of their appearance were quite similar in all patients. It is quite likely that abnormal movements in the patient without ictal EEG change do not have epileptic origin but brainstem release phenomenon induced by midazolam. Because the abnormal movements in the other two cases had similar clinical manifestations and mode of appearance, we suspected that these movements were also non-epileptic though ictal EEGs were not recorded in theses cases.
      When we encounter paroxysmal automatic movements mimicking neonatal seizures following intravenous midazolam administra-tion, ictal EEG recordings are recommended. If there are no ictal changes, we should avoid treatment with anticonvulsant drugs for these movements. Since midazolam is frequently used in neonates for sedation during various examinations, future investigations on the selection of appropriate drugs and dosage for sedation in neonates, including the usage of midazolam, are necessary.
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  • Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion with Visual Disturbance and Higher Brain Dysfunction
    Saeko Morino, Tomohide Goto, Ushio Ohtaki, Sahoko Miyama
    2011 Volume 43 Issue 4 Pages 295-299
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.295
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      Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a recently described clinicoradiologic syndrome. Clinically, a prolonged febrile seizure is followed by subsequent seizures which occur several days after the initial seizure. On MRI, reduced diffusion appears predominantly in the frontoparietal subcortical white matter at the time of the subsequent seizures. The main symptom between the initial and subsequent seizures is disturbance of consciousness.
      We report a case with AESD who presented 1) reduced diffusion on MRI which was dominant in the occipital lobe, and 2) reversible visual disturbance followed by higher brain dysfunction such as a cognitive deficit and disturbed speech. A 2-year-old Japanese girl was admitted because of visual disturbance which appeared 4 days after a generalized tonic-clonic seizure associated with fever. Two days later, she had another seizure when MRI revealed reduced diffusion in the subcortical white matter. The MRI finding was not typical of AESD in that reduced diffusion appeared dominantly in the occipital lobe. Normal ophthalmologic findings and abnormal visual evoked potential results suggested that her visual disturbance was due to an impaired visual pathway in the subcortical white matter in the occipital lobe. The present case indicates that there is a subgroup of AESD in which the subcortical lesion seen on MRI is dominant in the occipital lobe.
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  • A Case of Neurocutaneous Melanosis Associated with Focal Cortical Dysplasia
    Tomoko Mizuno, Eiji Nakagawa, Yoshiaki Saito, Hirofumi Komaki, Kenji S ...
    2011 Volume 43 Issue 4 Pages 300-303
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.300
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      A newborn baby boy presented with giant melanocytic nevi on the face, trunk and extremities, and focal cortical dysplasia on MRI. At 3 months of age, he developed intractable epilepsy, and MRI at 2 years of age revealed a high-intensity area in the bilateral cerebellum on T1-weighted images, indicative of melanosis. Based on the findings of the skin and MRI, we diagnosed the boy with neurocutaneous melanosis. Cytodiagnosis of cerebrospinal fluid showed no malignancies. EEG, magnetoencephalogram and ECD-SPECT indicated that the clonic seizures originated from a focus in the right focal cortical dysplasia. Complications also included sebaceous nevus of the head and face, which was characteristic of sebaceous nevus syndrome, lipoma of the face and cauda equina, and limbal dermoid. Sebaceous nevus syndrome may have been due to certain allelic defects that were independent of those for neurocutaneous melanosis.
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  • Remarkable Effect of a Modified Ketogenic Diet in a Boy with Focal Seizures Followed by Epileptic Spasms in a Cluster
    Hiroka Takahashi, Katsumi Imai, Rumiko Takayama, Jun Mine, Sanae Ohtan ...
    2011 Volume 43 Issue 4 Pages 305-308
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.305
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      A modified ketogenic diet was demonstrated to be remarkably effective in a child with intractable symptomatic focal epilepsy with combined seizures of focal seizures and epileptic spasms (ES) in a cluster (ESC). ES started at 8 months of age and disappeared with ACTH therapy. At the age of 13 months, the child began to have intractable focal seizures that, later, were followed by ESC 10 times a day. Brain MRI showed only a non-specific diffuse cerebral atrophy. Interictal EEG showed high amplitude diffuse disorganized slow waves with prominent sharp waves predominant over the bilateral occipital region. We started a modified ketogenic diet (mKD) treatment without fasting or a water/calorie limitation. Since the 20th day of mKD, the patient has been seizure free (6 months) without adverse effects. EEG showed remarkable improvement and he has some improvement in the developmental milestones. A modified ketogenic diet is easier to start and continue compared to the classic ketogenic diet, and should be tried in intractable epilepsies that are not treatable surgically early in life from the developmental prognosis point of view.
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  • Effective Cyclophosphamide Pulse Therapy for an Young Infant with Severe Dermatomyositis
    Ayako Senju, Masayuki Shimono, Naoki Shiota, Masahiro Ishii, Kenichi T ...
    2011 Volume 43 Issue 4 Pages 309-312
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.309
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      We herein report a 3 year-old boy, who showed proximal muscle weakness and pain at the age of one and a-half years. When he visited our hospital at the age of 1 year and 11 months, he could hardly move by himself. He also had difficulty in swallowing and suffered from multiple dermal ulcers. His blood test showed slightly elevated muscle enzyme activity, and magnetic resonance imaging suggested severe inflammation of the muscles. Radiological examination proved hypoperistalsis of the esophagus. With additional skin and muscle biopsies, we diagnosed him with juvenile dermatomyositis (JDM). Methyl-prednisolone pulse therapy was not effective enough, thus oral methotrexate, cyclosporine A and monthly cyclophosphamide pulse therapy were added. After the fourth cyclophosphamide pulse therapy, his muscular strength was restored, and the ulcers healed dramatically. Due to scarcity of severe cases, neither standardized classification nor grading system for severity in JDM has ever been established, which perplexes physicians in finding the best therapeutic strategy. Further investigation, experience and efforts are necessary to standardize an evaluating system and therapeutic strategy against JDM.
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  • A Case of Sudden Unexpected Death in Epilepsy 3 Years after the Onset of Acute Encephalitis with Refractory, Repetitive Partial Seizures
    Tetsuhiro Fukuyama, Yuji Inaba, Tsukasa Higuchi, Yukio Sekiguchi, Shui ...
    2011 Volume 43 Issue 4 Pages 313-316
    Published: 2011
    Released on J-STAGE: December 25, 2014
    DOIhttps://doi.org/10.11251/ojjscn.43.313
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      Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is a peculiar form of encephalitis mainly affecting children. Although not usually lethal, we report a case of sudden unexpected death in epilepsy (SUDEP) 3 years after the onset of AERRPS.
      A 6-year-old boy was admitted to our hospital because of fever and extremely refractory partial and secondary generalized seizures with delirium and psychiatric change. The seizures were highly resistant to anticonvulsants and suppressed only by large dose intravenous administration of midazolam. Seven months after the onset, the seizures were ameliorated by treatment with potassium bromide and clorazepate. After the acute phase, the patient developed complex partial seizures that tended to present with cyanosis. At the age of 10, he was found lying prone in respiratory arrest with facial pallor. Although he regained cardiac function after being taken to our emergency room, the patient died 12 days later. Six SUDEP cases after the onset of AERRPS, including this one, have been reported to date. Since epilepsy following AERRPS is one of the risk factors of SUDEP, clinicians should consider SUDEP to be a rare but high risk syndrome in AERRPS-afflicted children.
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