Rinshō shinkeigaku = Clinical neurology
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We report a patient with cerebral air embolism in whom we could perform serial brain magnetic resonance images (MRIs). A 78-year-old man was admitted to our hospital because of recurrent empyema after surgery for esophageal cancer. He suddenly demonstrated left hemiparesis in the middle of pleural lavage. ⋯ He didn't receive hyperbaric oxygen therapy because he had status epilepticus. The hypointense spots on the T2*WI became smaller and spread after 7 hours from onset and almost disappeared after 53 days. It is important to recognize that cerebral arterial air embolism is similar to multiple microbleeds and cerebral arteriovenous fistula in hypointense spots on the T2*WI.
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Case Reports
[A case of myasthenia gravis presenting solely with bulbar palsy unassociated with easy fatigability].
A 69-year-old Japanese female was admitted because of progressive nasal voice and dysphagia. Neurological examination revealed paresis of the soft palate with marked dysphagia and rhinolalia. Otherwise there was no weakness or easy fatigability in extraocular muscles and extremities. ⋯ The present case shows that it is important to consider MG even in cases presenting solely with progressive bulbar palsy without easy fatigability. So far, cases of bulbar myasthenia gravis with positive anti-MuSK Ab have often been reported. As shown in the present case, bulbar myasthenia gravis can also be associated with positive anti-ACh-R Ab but negative anti-MuSK Ab.
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Paraneoplastic limbic encephalitis is a rare neurological disorder that frequently precedes the detection of malignancy. We report the case of a 68-year-old male with small-cell lung cancer who developed paraneoplastic limbic encephalitis associated with presence of the anti-Hu antibody, after achieving complete remission of the tumor by chemotherapy. The patient visited our hospital because of progressive sensory disturbance of the distal extremities at 65 years of age. ⋯ To our knowledge, this is the first report of a patient presenting with paraneoplastic neurological syndrome in which limbic encephalitis developed after tumor disappearance. So we must recognize the possibility of neurological symptoms occurring during remission. As the mechanism of pathogenesis, delayed neuronal cell damage due to immune responses against the tumor is implicated.
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Case Reports
[A case of sensory ataxic neuropathy, dysarthria, and ophthalmoparesis with multiple mitochondrial DNA deletions].
We report the case of a 62-year-old man with sensory ataxic neuropathy, dysarthria, and ophthalmoparesis (SANDO). He developed gait disturbance at 54 years of age, muscle weakness at 56 years, and difficulty hearing at 58 years. His brother had muscle weakness in both legs from age 20 years, and was diagnosed with Charcot-Marie-Tooth disease because he had muscle weakness of the four extremities, decreased CMAP and SNAP amplitudes on peripheral nerve conduction tests, and loss of large myelinated fibers and onion-bulb formations on sural nerve biopsy. ⋯ A1217V). We diagnosed SANDO. This is the first case of SANDO with large mitochondrial DNA deletions in Japanese.
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In Japan, prion diseases are classified into 76.6％ with sporadic Creutzfeldt-Jakob disease (sCJD), 19.6％ with genetic prion diseases, and 4.9％ with acquired prion disease. In sCJD, MM1 type sCJD, which show typical clinical course of CJD, is the most common. Among atypical sCJD cases, MM2 type sCJD is the most common. ⋯ All cases with acquired prion diseases except for one case of variant CJD are dura mater graft-associated CJD (dCJD), and the number of dCJD in Japan is account for more than 60％ of all patients with dCJD all over the world. The remarkable frequency of dura mater graft use in Japan might contribute to the elevated incidence of dCJD, but the possible reasons for the disproportionate use of this procedure in Japan remain unclear. Our recent study revealed that differences in the medical conditions precipitating dura mater graft might contribute to the frequent use of cadaveric dura mater and the higher incidence of dCJD in Japan.