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Shimizu T.,Tokyo Metropolitan Neurological Hospital
European Neurological Review | Year: 2013

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with progressive loss of upper and lower motor neurons. Autonomic nervous abnormalities, including sympathetic hyperactivity and sympathovagal imbalance, have been found in both early and advanced stages of ALS. In early stage, the dysfunction may be subclinical. Occasionally, elevated blood pressure or heart rate and increased sweating may be observed. In advanced stage when ventilators are required, the sympathetic hyperactivity may lead to hypertensive crisis without counter-regulation of heart rate, followed by the consecutive circulatory collapse, known as the 'autonomic storm'. The symptoms of 'autonomic storm' are similar to that of 'baroreflex failure', and 'autonomic storm' indicates poor prognosis and may result in sudden death. Careful evaluation and individual treatment are strongly suggested, although appropriate therapeutic approaches have not been established. Causative central nervous lesions remain to be elucidated, although the limbic system may be involved. The autonomic dysfunction further supports the concept that ALS is a multisystem-degenerative disease. © TOUCH MEDICAL MEDIA 2013. Source

Kawai K.,University of Tokyo | Morino M.,Tokyo Metropolitan Neurological Hospital | Iwasaki M.,Tohoku University
Brain and Development | Year: 2014

Delalande's vertical hemispherotomy is an innovative evolution of hemispherectomy in minimizing brain resection. We report our modification for this surgical procedure. We modified the original procedure in two aspects for the purpose of less brain resection and confirmation of the complete disconnection. Firstly, all procedures were done via an interhemispheric route instead of a transcortical route. Secondly, we set the anterior disconnection plane as the one that connects the anterior end of the choroidal fissure to the anterior end of the foramen of Monro, instead of the former to the subcallosal area. We applied this modified vertical hemispherotomy to 7 cases. Four cases were children with hemimegalencephaly and other 3 were adults with ulegyric hemisphere. Surgical procedure was completed without complication in all cases. There was no case that required CSF shunting. Seizure outcome was Engel's class I in 6 and class IV in 1. Postoperative MRI revealed complete disconnection of the affected hemisphere in all patients. We reported our modification of vertical hemispherotomy. Although these are minor modifications, they further minimized brain resection and may serve for less invasiveness of procedure and improvement in completeness of disconnection and its confirmation during surgery. © 2013 The Japanese Society of Child Neurology. Source

Sugaya K.,Tokyo Metropolitan Neurological Hospital
Sub-cellular biochemistry | Year: 2012

Huntington's disease (HD) is among the polyglutamine (polyQ) disorders, which are caused by expansion of CAG-trinucleotide repeats. These disorders share common characteristics, and have thus long been thought to have a unifying pathogenic mechanism resulting from polyQ expansion. However, this scenario has recently become more complex, as studies have found multiple pathways for the assembly of disease-related polyQ protein aggregates that differ in both structure and toxicity. There are fascinating disease-specific aspects of the polyQ disorders, including the repeat-length dependence of both clinical features and the propensity of the expanded polyQ protein to aggregate. Such aggregation kinetics have proven useful in explaining the disease process. This chapter describes two risk-based stochastic kinetic models, the cumulative-damage and one-hit models, that describe genotype-phenotype correlations in patients with polyQ diseases and reflect alternative pathways of polyQ aggregation. Using repeat-length as an index, several models explore the quantitative connection between aggregation kinetics and clinical data from HD patients. The correlations between CAG repeat-length and age-of-onset are re-evaluated, and the rate of disease progression (as assessed by clinical measures and longitudinal imaging studies of brain structure) are surveyed. Finally, I present a mathematical model by which the time course of neurodegeneration in HD can be precisely predicted, and discuss the association of the models with the major controversies about HD pathogenesis. Source

Takai K.,Tokyo Metropolitan Neurological Hospital | Taniguchi M.,Tokyo Metropolitan Neurological Hospital
Neurosurgical Focus | Year: 2012

Object: Spinal arteriovenous malformations (AVMs) are classified into types according to anatomical characteristics: dural arteriovenous fistulas (AVFs), intramedullary AVMs, perimedullary AVFs, and extradural AVFs. Spinal extradural AVFs are much rarer than other types of spinal AVMs, and the available literature on this clinical entity has been based only on case reports or small case series. To investigate the clinical characteristics of patients with spinal extradural AVFs, the authors systematically reviewed the associated literature in the MRI era. Methods: The PubMed database was searched for all relevant English-language case reports and case series published from 1990 to 2011. The clinical differences between Type A with and Type B without intradural venous drainage were statistically compared, especially regarding clinical features and angiographic and MRI findings. Results: Forty-five cases of spinal extradural AVFs were found. Type A spinal extradural AVFs were diagnosed in patients with a significantly older age (mean 63.5 years) as compared with Type B AVFs (mean 34.3 years, p < 0.0001). Most cases of Type A spinal extradural AVFs exhibited a diffuse high signal intensity of the spinal cord on T2-weighted MR images and no mass effect (p < 0.0001), and they commonly occurred in the thoracolumbar and lumbar regions (p < 0.0001). On the other hand, cases of Type B lesions exhibited a normal signal intensity of the cord with severe mass effect due to an enlarged extradural venous plexus, and they commonly occurred in the cervical and upper thoracic regions (p < 0.0001), frequently in patients with neurofibromatosis Type 1 (p = 0.049). Because Type B AVFs consisted of high-flow, multiple complex anastomoses between arteries and the epidural venous plexus, patients with these lesions tended to undergo multisession treatments, and the rate of partial AVF occlusion was significantly higher than for Type A AVFs (p = 0.018), although there was no difference in symptom outcomes between the 2 groups. Conclusions: To the best of the authors' knowledge, a comparative analysis of the clinical differences in patients with extradural AVFs with or without intradural venous drainage has yet to be described in the literature. They concluded that in the diagnosis of spinal extradural AVF, evaluation of intradural venous drainage is important because the cause of myelopathy determines the treatment goals. Source

Takai K.,Tokyo Metropolitan Neurological Hospital | Taniguchi M.,Tokyo Metropolitan Neurological Hospital
Acta Neurochirurgica | Year: 2015

Background: Spinal intramedullary arteriovenous malformations (AVMs) fed by an anterior spinal artery are surgically challenging vascular lesions. Method: We herein presented microsurgical resection techniques for an intramedullary glomus AVM located in the lateral part of the high cervical spinal cord with an operative video. These techniques included (1) a lateral suboccipital approach via cervical hemilaminectomy in the lateral position; (2) retrograde dissection of the AVM located between the spinal tracts; (3) coagulation and division of multiple narrow sulcal branches of the anterior spinal artery. Conclusion: Patients who underwent these techniques achieved good outcomes with minimal bleeding and morbidity. © 2015, Springer-Verlag Wien. Source

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