1.Multiple system atrophy in a patient with primary ciliary dyskinesia
Hideya Sakaguchi ; Satoshi Yamashita ; Tomohiro Suga ; En Kimura ; Yasushi Maeda ; Teruyuki Hirano ; Makoto Uchino
Neurology Asia 2013;18(1):103-105
We present the case of a patient with primary ciliary dyskinesia who later developed clinically probable
multiple system atrophy. Multiple system atrophy is a sporadic neurodegenerative disorder clinically
characterised by various combinations of parkinsonism, cerebellar ataxia, autonomic failure, and
pyramidal sign. Primary ciliary dyskinesia is a genetically heterogeneous disorder of motile cilia and
results in chronic bronchitis, bronchiectasis, chronic rhinosinusitis, chronic otitis media, situs inversus,
and male infertility. Most of the causative genes for primary ciliary dyskinesia encode proteins that
are part of the heavy or intermediate chain of axonemal dynein in ciliary outer dynein arms. We
hypothesised that axonemal dynein dysfunction in primary ciliary dyskinesia results in reduced autophagy,
accompanied by impaired cytoplasmic dynein function, which in turn accelerates -synucleinopathy in
multiple system atrophy. Furthermore, we contemplated a potential association between primary cilia
and neuronal function. Although it is not yet clear if a causal link between multiple system atrophy
and primary ciliary dyskinesia exists, further investigation into the relationship between axonemal
dynein dysfunction in primary ciliary dyskinesia and α-synucleinopathy should be conducted.
2.Drastic Therapy for Listerial Brain Abscess Involving Combined Hyperbaric Oxygen Therapy and Antimicrobial Agents.
Keiichi NAKAHARA ; Satoshi YAMASHITA ; Katsumasa IDEO ; Seigo SHINDO ; Tomohiro SUGA ; Akihiko UEDA ; Shoji HONDA ; Tomoo HIRAHARA ; Masaki WATANABE ; Taro YAMASHITA ; Yasushi MAEDA ; Yasuhiro YONEMOCHI ; Tomohiro TAKITA ; Yukio ANDO
Journal of Clinical Neurology 2014;10(4):358-362
BACKGROUND: Listeria monocytogenes (L. monocytogenes) is a rare causative pathogen of brain abscess that is often found in immunocompromised patients. Although patients with supratentorial listerial abscesses showed a longer survival with surgical drainage, the standard therapy for patients with subtentorial lesions has not been established. CASE REPORT: We report herein a patient with supra- and subtentorial brain abscesses caused by L. monocytogenes infection. These abscesses did not respond to antibiotics, and his symptoms gradually worsened. Drainage was not indicated for subtentorial lesions, and the patient was additionally treated with hyperbaric oxygen therapy, which dramatically reduced the volume of abscesses and improved the symptoms. CONCLUSIONS: This is the first report of drastic therapy for a patient with listerial brain abscesses involving combined antibiotics and hyperbaric oxygen therapy. The findings suggest that hyperbaric oxygen therapy is a good option for treating patients with deep-seated listerial abscesses and for who surgical drainage is not indicated.
Abscess
;
Anti-Bacterial Agents
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Anti-Infective Agents*
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Brain Abscess*
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Drainage
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Humans
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Hyperbaric Oxygenation*
;
Immunocompromised Host
;
Listeria monocytogenes