No to hattatsu. Brain and development
-
Objective: To elucidate the pathophysiology of West syndrome and mechanism of immunoglobulin therapy for this syndrome, we investigated serum and cerebrospinal fluid (CSF) cytokine levels before and after high-dose intravenous immunoglobulin (IVIG) therapy in patients with West syndrome. Methods: We measured serum and CSF cytokine levels of 11 patients with West syndrome who was referred to Saitama Children’s Medical Center from April 2010 to May 2014. All patients received IVIG, ranging from 200 to 500 mg/kg/day for 3 consecutive days (initial IVIG treatment), before adrenocorticotrophic hormone therapy. ⋯ Before IVIG treatment, the level of IL-8 in CSF was significantly higher than that in serum, while the serum IL-18 level in the seizure-free group was significantly lower than that in the seizure-persisting group. Alterations of serum IL-18 level and CSF IL-8 level were different between the seizure-free and seizure-persisting groups. Conclusions: Serum IL-18 and CSF IL-8 may be important factors for elucidating the pathophysiology of West syndrome and mechanism of IVIG therapy.
-
We report the case of a 4-year-old girl who presented with paroxysmal sympathetic hyperactivity (PSH), after developing severe hypoxic-ischemic-encephalopathy because of cardiopulmonary arrest. She showed dramatic paroxysmal sympathetic activity with dystonia. She was treated with wide variety of medications against PSH, which were found to be effective in previous studies. ⋯ One is that PSH among children have been rarely reported, and the other is that this case of prolonged PSH delineated the transition of plasma catecholamines during the treatment. The excitatory: inhibitory ratio (EIR) model proposed by Baguley was considered while dis- cussing drug sensitivity in this case. Accumulation of similar case studies will help establish more effective treatment strategies and elucidate the pathophysiology of PSH.
-
Case Reports
[A girl with dyslexia suspected to have Irlen syndrome, completely relieved by wearing tinted lenses].
Irlen syndrome is a proposed perceptual processing disorder characterized by visual distortions while reading. Patients with this syndrome may experience light sensitivity, visual stress, and other related problems such as dyslexia. Tinted lenses and colored overlays have been designed to help individuals with the symptoms of Irlen syndrome. ⋯ These ambiguous criteria may be responsible for the criticism over the validity of this condition. Although this was only an anecdotal case, our patient exhibited the core functional deficit described in Irlen syndrome and showed a dramatic improvement with tinted lenses; therefore, this case may facilitate investigations into the mechanism underlying Irlen syndrome, if it actually exists. Although further studies are required to confirm the validity of this syndrome and the treatment approach, Irlen syndrome should be recognized as a disorder since its symptoms can be easily relieved by wearing tinted lenses or color filters.
-
Topiramate (TPM), lamotrigine (LTG), and levetiracetam (LEV) are three new-generation antiepileptic drugs (AEDs) which have recently come into use in add-on therapy for refractory childhood epilepsy in Japan. The aim of this study was to evaluate their efficacy and tolerability, and to clarify the role of these three AEDs in childhood epilepsy therapy. ⋯ LEV was the most effective of the three add-on therapies in refractory childhood epilepsy with partial and generalized onset. Regarding seizure-free, TPM was more effective than the other therapies, but it had many side effects. LTG tended to be more effective for generalized epilepsy, particularly idiopathic epilepsy, than partial epilepsy. We conclude that it is necessary to develop a treatment plan for pediatric epilepsies after considering the advantages and disadvantage of these new AEDs.
-
Review Case Reports
[A case of Guillain-Barré syndrome with severe pain successfully controlled with acetaminophen, gabapentin, and parenterally infused fentanyl].
We presented a case of an 8-year-old boy with Guillain-Barré syndrome characterized by severe intractable pain in the soma and lower extremities, which appeared 2 weeks after a febrile cold. At his first visit to our hospital, he could not stand or walk because of the severe pain, and muscle weakness and absence of deep tendon reflexes were observed. ⋯ The pain was successfully controlled with parenterally infused fentanyl. It is suspected that opioid analgesics are useful for severe pain control in patients with Guillain-Barré syndrome.