Rinsho Shinkeigaku
Online ISSN : 1882-0654
Print ISSN : 0009-918X
ISSN-L : 0009-918X
Volume 57, Issue 5
Displaying 1-13 of 13 articles from this issue
Review
  • Teruyuki Hirano
    2017 Volume 57 Issue 5 Pages 203-207
    Published: 2017
    Released on J-STAGE: May 27, 2017
    Advance online publication: April 27, 2017
    JOURNAL FREE ACCESS

    Outcome of acute stroke patients with embolic large vessel occlusion can be improved dramatically, if the effective and rapid reperfusion of the occluded vessel achieved. Since 2015, when the evidence of acute endovascular therapy established its efficacy, such scenario became no longer uncommon. Along with introduction of direct oral anticoagulant (DOAC), many options of antithrombotic therapy for acute ischemic stroke also became available. This review summarize the recent advances on acute ischemic stroke therapy.

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Case Reports
  • Shunsuke Takai, Asako Tagawa, Tomoko Ogawa, Hiroyuki Kato, Noriko Sait ...
    2017 Volume 57 Issue 5 Pages 208-213
    Published: 2017
    Released on J-STAGE: May 27, 2017
    Advance online publication: April 27, 2017
    JOURNAL FREE ACCESS

    Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia; it is characterized by repeated respiratory or systemic infections caused by bacteria, viruses, and fungi, as well as with various autoimmune disorders such as pure red cell aplasia. A 65-year-old woman was admitted to our hospital with ptosis and abdominal muscle weakness. Based on the presence of anti-acetylcholine receptor (Ach-R) antibodies, she was diagnosed with myasthenia gravis (MG). At that time, invasive thymoma of Masaoka stage IVa was also detected. Regression of thymoma and clinical remission of MG was achieved by chemotherapy followed by high-dose corticosteroid. However, several months later, the patient started developing repeated bacterial respiratory tract infections, cytomegalovirus infections, and esophageal and systemic candidiasis. Laboratory tests revealed a marked decrease of serum gamma-globulin levels (IgG 586 mg/dl, IgA 32 mg/dl, IgM 29 mg/dl) and severe reduction in the B cells ratio, as well as a decrease in the CD4+CD25+T cell to CD4+CD25−T cell ratio indicative of deregulation of CD4+T cell activation. These results suggested that the patient impaired humoral and cell-mediated immune responses. We continued the treatment with antibiotics and regular immunoglobulin supplementation through intravenous injections. Although autoimmune disorders are often observed in Good syndrome, the association with MG is quite rare. The case report is followed by the review of literature.

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  • Hidehiro Ishikawa, Yuichiro Ii, Atsushi Niwa, Keita Matsuura, Masayuki ...
    2017 Volume 57 Issue 5 Pages 214-219
    Published: 2017
    Released on J-STAGE: May 27, 2017
    Advance online publication: April 27, 2017
    JOURNAL FREE ACCESS

    A 55-year-old man with no mental retardation had presented a history of frequent transient clumsiness of his right upper and lower extremities for about 20 years. He was admitted to a general hospital with weakness of right side of the body, and first-ever generalized seizure attack occurred the next day. Brain CT showed calcification in the left cerebral cortices. So he was referred to our hospital. On neurological examination, he had mild clumsiness of his right upper limb and right pyramidal tract sign. He had neither facial port-wine stain nor glaucoma. The blood test and cerebrospinal fluid analysis were unremarkable. Electroencephalogram showed slowing and reduction of activity at the left frontal and parietal areas with no epileptic activities. Brain CT showed “tram–track calcification” and lobar atrophy in the left fronto-parietal cortices. Susceptibility weighted imaging (SWI) on MRI revealed enlarged transmedullary veins in the left periventricular white matter and low intensity lesions along the cortical gyri. Post gadolinium fluid-attenuated inversion recovery imaging (FLAIR-Gd) showed leptomeningeal enhancement in the left fronto-parietal lobes more extensively than those by post gadolinium T1-weighted image. Brain perfusion single photon emission computed tomography with a technetium-99m-ethyl cysteinate dimer (99mTc-ECD SPECT) revealed hypoperfusion in the fronto-parietal lobes. These clinical and neuroimaging findings were compatible with type III Sturge-Weber syndrome (SWS). His condition was improved after treatment with oral levetiracetam (1,000 mg daily). Although adult-onset type III SWS is very rare, it is important to perform SWI and post-contrast FLAIR for assessing leptomeningeal angioma in patients with seizure with focal cortical calcification even if they have no facial nevus.

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  • Takashi Isobe, Madoka Mori-Yoshimura, Yasushi Oya, Yuko Saito, Miho Mu ...
    2017 Volume 57 Issue 5 Pages 220-224
    Published: 2017
    Released on J-STAGE: May 27, 2017
    Advance online publication: April 27, 2017
    JOURNAL FREE ACCESS

    We report a 62-year-old woman with a history of Basedow’s disease and Sjogren’s syndrome who presented with slowly progressive limb muscle weakness over the course of ten years. On physical examination, she had dry eye and mouth, but was otherwise normal. Neurological examination revealed symmetrical proximal dominant muscle weakness. Polymyositis was suspected at initial diagnosis due to her clinical course, physical examination, and autoimmune disease. However, the final diagnosis based on a muscle biopsy was the chronic myopathic type of sarcoid myopathy. Among 25 definite sarcoid myopathy cases in the National Center of Neurology and Psychiatry muscle repository from 2010 to 2015, 6/25 had autoimmune diseases. All 6 patients were female and had the chronic myopathic type of sarcoid myopathy. The number of patients with Sjogren’s syndrome, thyroid disease, autoimmune hepatitis, and idiopathic thrombocytopenia were 4, 1, 1, and 1, respectively. Only the present case had both thyroid disease and Sjogren’s syndrome. In conclusion, the chronic myopathic type of sarcoid myopathy is one possibility to consider in patients who present with progressive myopathy together with autoimmune diseases.

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  • Shinichi Takeshima, Shuichiro Neshige, Naoyuki Hara, Tomoshi Kubo, Tak ...
    2017 Volume 57 Issue 5 Pages 225-229
    Published: 2017
    Released on J-STAGE: May 27, 2017
    Advance online publication: April 27, 2017
    JOURNAL FREE ACCESS

    We report two cases (a married couple) of intoxication due to angel’s trumpet ingestion. Case 1: A 71-year-old woman was found lying unconscious on the sofa at home and was brought to our hospital by ambulance. She showed mydriatic anisocoria, and an intracerebral lesion was suspected. However, the brain magnetic resonance imaging showed no abnormal lesion and acute encephalopathy of unknown cause was diagnosed. Case 2: A 68-year-old man (husband of the patient of Case 1) showed alteration of consciousness with agitation and was admitted to our hospital on the next day. He also had slight mydriasis. As his manifestations were similar to those of his wife, we studied their medical history again. We found that they mistook the roots of angel’s trumpet for burdock and cooked and ate them. This intoxication causes characteristic encephalopathy with altered consciousness and mydriasis. In the case of anisocoria or mild mydriasis, the diagnosis is difficult sometimes. The intoxication occurred within a family; this was a clue to the correct diagnosis. Severe cases exhibit pyramidal signs and symptoms or convulsion, and deaths have been reported. Angel’s trumpet intoxication is an important neurological emergency.

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Brief Clinical Notes
  • Haruchika Ogura, Jiro Fukae, Satoshi Kimura, Mikiko Aoki, Kazuki Nabes ...
    2017 Volume 57 Issue 5 Pages 230-233
    Published: 2017
    Released on J-STAGE: May 27, 2017
    Advance online publication: April 28, 2017
    JOURNAL FREE ACCESS

    A 55-year-old man was admitted to our hospital for investigation of high fever, decreased consciousness and bilateral visual impairment. His cerebrospinal fluid analysis revealed pleocytosis of mononuclear cells and an increased protein concentration. FLAIR images revealed multiple high-intensity lesions in the frontal lobe, part of which was enhanced with gadolinium. Despite initiating treatment with acyclovir and corticosteroids, his consciousness and visual acuity deteriorated. Immunopathological examination of brain biopsies showed numerous herpes simplex virus type 2-positive neurons and macrophages, leading to a diagnosis of herpes simplex encephalitis (HSE). Fundoscopic examination revealed multiple foci of retinitis with vasculopathies, and inflammation in the anterior chamber and vitreous, indicating acute retinal necrosis (ARN). Foscarnet treatment was initiated in place of acyclovir and his consciousness improved, with a slight improvement in visual acuity. ARN is typically caused by a herpes virus infection limited to the eyeball, and rarely in combination with HSE. In such cases, there is a latency of approximately 2–4 weeks between ARN and the onset of encephalitis. Our case is unique in that HSE and ARN developed simultaneously, and it highlights that there may not always be a latency between the onsets of the two disorders. Finally, foscarnet should be considered in cases of HSE and ARN with acyclovir resistance.

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  • Motoyuki Umekawa, Keiko Hatano, Hideyuki Matsumoto, Takahiro Shimizu, ...
    2017 Volume 57 Issue 5 Pages 234-237
    Published: 2017
    Released on J-STAGE: May 27, 2017
    Advance online publication: April 27, 2017
    JOURNAL FREE ACCESS

    The patient was a 47-year-old man who presented with diplopia and gait instability with a gradual onset over the course of three days. Neurological examinations showed ophthalmoplegia, diminished tendon reflexes, and truncal ataxia. Tests for anti-GQ1b antibodies and several other antibodies to ganglioside complex were positive. We made a diagnosis of Fisher syndrome. After administration of intravenous immunoglobulin, the patient’s symptoms gradually improved. However, bilateral facial palsy appeared during the recovery phase. Brain MRI showed intensive contrast enhancement of bilateral facial nerves. During the onset phase of facial palsy, the amplitude of the compound muscle action potential (CMAP) in the facial nerves was preserved. During the peak phase, the facial CMAP amplitude was within the lower limit of normal values, or mildly decreased. During the recovery phase, the CMAP amplitude was normalized, and the R1 and R2 responses of the blink reflex were prolonged. The delayed facial nerve palsy improved spontaneously, and the enhancement on brain MRI disappeared. Serial neurophysiological and neuroradiological examinations suggested that the main lesions existed in the proximal part of the facial nerves and the mild lesions existed in the facial nerve terminals, probably due to reversible conduction failure.

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