Rinsho Shinkeigaku
Online ISSN : 1882-0654
Print ISSN : 0009-918X
ISSN-L : 0009-918X
Volume 50 , Issue 3
Showing 1-9 articles out of 9 articles from the selected issue
Original Articles
  • Takayoshi Shimohata, Kaori Yanagawa, Keiko Tanaka, Masatoyo Nishizawa
    2010 Volume 50 Issue 3 Pages 137-140
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    We retrospectively investigated prognostic factors of 27 consecutive patients with bacterial meningitis seen at Niigata University Medical and Dental Hospital between 1980 and 2006. Patients were divided into the two categories using the Glasgow outcome scale (GOS) at discharge; the poor outcome group (GOS=1 to 4; n=15) and the good outcome group (GOS=5; n=12). Poor outcome was significantly associated with the severe consciousness disturbance, and the presence of intracranial (brain swelling, seizure, cerebral hemorrhage) or systemic (pneumonia) complications. The clinical features of patients who died of bacterial meningitis (GOS=1) were almost identical to those of patients with poor outcome (GOS=1 to 4); however, the protein and sugar content in the cerebrospinal fluid in the patients who died were significant compared to the patients with good outcome (GOS=5). Pneumococcal infection was also considered to be a poor prognostic factor. Future prospective studies should be performed on a larger group of patients for establishing the prognostic factors of adult bacterial meningitis.
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  • Utako Nagaoka, Toshio Shimizu, Tokiko Matsukura, Mayumi Takeda
    2010 Volume 50 Issue 3 Pages 141-146
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    We investigated nutritional states of 28 patients with multiple system atrophy (MSA) by measuring body mass index (BMI), arm muscle circumference (%AMC) and triceps skin fold thickness (%TSF). We also analyzed retrospectively chronological changes of nutritional status in 13 MSA patients surviving more than 10 years. BMI and %AMC were significantly reduced in patients having tube feeding compared with patients who had oral intake, whereas %TSF was increased in some patients with tube feeding. From the chronological study, patients at the stage of respiratory or swallowing deterioration showed marked malnutrition, whereas patients during the advanced, but stable stages with tracheostomy and gastrostomy showed much fat accumulation even under low calorie intake less than 1,000kcal/day. Daily amount of calorie intake should be sufficient during respiratory or swallowing deterioration, but it should be restricted at the advanced stable stage to avoid fat accumulation.
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Case Reports
  • Noriko Hasegawa, Ken-ichi Shimada, Yasuji Yamamoto, Kiyoshi Maeda
    2010 Volume 50 Issue 3 Pages 147-150
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    We reported a patient with probable dementia with Lewy bodies (DLB) showing cervical dystonia during treatment with donepezil. A 78-year-old female had been treated with donepezil 5mg/day for 18 months. The patient admitted to our hospital because of severe antecollis. Antecollis disappeard three weeks after discontinuation of donepezil.
    Five months later the patient received donepezil 3-5mg/day for disease progression. The patient showed laterocollis again after a month-treatment with donepezil. Physical examination and labolatory tests were nomal. Magnetic resonance imaging of the neck showed no abnormal finding, but electromyography revealed dystonic changes in the neck muscles. Three weeks after discontinuation of donepezil, laterocollis disappeared. These findings suggest that treatment with donepezil induced cervical dystona in a patient with DLB.
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  • Yusuke Hazama, Yasuhiro Asou, Kenichiro Nakamura, Noriyuki Kimura, Tos ...
    2010 Volume 50 Issue 3 Pages 151-155
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    A 71-year-old man was diagnosed as Parkinson disease at age 59, and levodopa therapy was started. Eleven years after the beginning of treatment, he noticed high fever (38.0°C∼39.0°C) in July, but hyperthermia spontaneously disappeared three months later. In early July of the following year, he was re-admitted to our hospital because of continuous high fever, despite no any inflammation. Neurological examination revealed flexion posture of trunk and limbs and short step gait. He also presented limb rigidity, akinesia, and resting tremor during off period. Routine laboratory examinations and radiological examinations showed no remarkable findings. Autonomic testing revealed orthostatic hypotension and anhidrosis below trunk and lower limbs. By controlling the room temperature at 26°C, hyperthermia showed a marked decline. In despite of no reports found associations between heat retention and Parkinson disease, in this case we speculate hyperthermia was caused by heat retention.
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  • Harumi S. Yomono, Hiroshi Kurisaki, Akira Hebisawa, Yoshio Sakiyama, Y ...
    2010 Volume 50 Issue 3 Pages 156-162
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    This is the first autopsy case of SCA2 with parkinsonian phenotype. At the age of 46, the patient got symptoms of parkinsonism to which anti-parkinsonian drugs were effective. He had mosaic 38, 40 CAG repeat expansions on chromosome 12q23-24, being diagnosed as SCA2, and his mother and his son also had CAG expansions on the same locus. In addition to parkinsonism, he also exhibited autonomic disturbance, dementia, and mild cerebellar ataxia. Brain images revealed severe atrophy of pons and medulla oblongata, resembling MSA-C. HVA and 5-HIAA were reduced in the cerebrospinal fluid, and the heart-mediastinum (H/M) ratio in myocardial 123I-MIBG cintigram was decreased, which suggested Lewy body pathology. He died at the age of 75 and the autopsy revealed atrophy of the olivo-ponto-cerebellar (OPC) system and substantia nigra which was compatible to SCA2, although the OPC system atrophy was less severe than formerly reported SCA2 cases. The degrees of atrophy of the OPC system and substantia nigra might explain the predominancy of clinical symptoms. Anti-1C2 positive inclusions in the pontine nuclei, inferior olive nuclei, cerebellum and substantia nigra confirmed a polyglutamine disease. In addition, there were the anti-phosphorylated α-synuclein positive, Lewy body related pathological changes in the substantia nigra, the locus ceruleus, the dorsal motor nuclei of vagus, and the sympathetic nerve in the myocardium. Major genetic abnormalities related to Parkinson disease were not detected. As another case of SCA2 with Lewy body pathology was reported in Japan, the coexistence of SCA2 and Lewy body pathology may not be accidental. Since myocardial MIBG scincigram can predict Lewy body pathology, we should seek more clinical cases of SCA2 with Lewy body pathology.
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  • Tomo Iwashima, Takahisa Tateishi, Ryo Yamasaki, Kyoko Motomura, Yasuma ...
    2010 Volume 50 Issue 3 Pages 163-167
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    An 80-year-old man (patient 1) was admitted to our hospital with numbness of the right leg and weakness of the lower extremities, predominantly in the right leg, for 1 year previously. Neurological examination revealed moderate weakness without atrophy, and fasciculation in the bilateral lower extremities. No hyperreflexia was noted, and the plantar response was flexor. Neither bulbar palsy nor sensory disturbance was observed.
    Electromyography (EMG) showed a chronic neurogenic pattern, including giant motor unit potentials and reduced interference in all four limb muscles. MRI images of the cervical and lumbar spines showed severe age-related spondylosis. The clinical and laboratory findings led to a diagnosis of spinal progressive muscular atrophy. Motor paralysis progressed slowly for the following four years, culminating in respiratory failure.
    A 79-year-man, the younger brother of patient 1 (patient 2), suffered from gait disturbance for 3 years before the admission to our hospital. During the following 3 years, bilateral leg weakness developed, causing difficulty walking. Neurological examination revealed a diffuse mild weakness with atrophy and fasciculation in the bilateral lower extremities; the right deltoid muscle was also mildly weak. Mild hyperreflexia was also noted on the left side, and the plantar response was extensor on the left. EMG showed acute and chronic neurogenic patterns in the four limb muscles. Because the missense mutation c.377 T>C; p.L126S was found on exon 5 of the superoxide dismutase (SOD) 1 gene in this patient, a diagnosis of familial ALS was made.
    Eight patients reported as familial ALS with the SOD1L126S mutation, including the present cases, all developed an onset of weakness in the lower extremities, but showed few upper motor neuron signs. It is important to consider the possibility of this type of familial ALS in a case of spinal progressive muscular atrophy with late-onset and mild progression, if family history is positive.
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Brief Clinical Notes
  • Hisashi Imbe, Hideto Nakajima, Takumi Ito, Haruko Kitaoka
    2010 Volume 50 Issue 3 Pages 168-171
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    We report the case of a 31-year-old woman who presented with neuromyelitis optica (NMO) associated with Sjögren syndrome and distal renal tubular acidosis. She was hospitalized because of cervical transverse myelopathy and right optic neuritis. She had been clinically diagnosed with Sjögren syndrome, with a high titer of anti-SS-A antibody (1:500) and anti-SS-B antibody (1:498). She also showed hypokalemia, metabolic acidosis, and nephrocalcinosis caused by distal renal tubular acidosis associated with Sjögren syndrome. T2-weighted magnetic resonance imaging (MRI) revealed long lesions extending from the medulla oblongata to the lower thoracic cord. In addition, gadolinium-enhanced MRI revealed a right optic nerve lesion in the optic canal. High titer of anti-aquaporin-4 (AQP4) antibody was detected in the patient's serum (1:131,072). A combination therapy comprising steroid pulse therapy and plasmapheresis improved her clinical symptoms, and the administration of oral prednisolone (20mg/day) was effective in preventing the recurrence of NMO. In patients with myelitis/transverse myelopathy associated with autoimmune disorders such as Sjögren syndrome, examining the titer values of anti-AQP4 antibody is indispensable in determining the appropriate therapy.
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  • Hiroyo Haruki, Motoharu Kawai, Jun-ichi Ogasawara, Michiaki Koga, Kiyo ...
    2010 Volume 50 Issue 3 Pages 172-174
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    We report a novel regimen of nutritional management in 22-year-old woman with myopathic form of very-long-chain acyl-CoA dehydrogenase deficiency. This regimen is based on avoidance of fasting by frequent intake of carbohydrates and substitution of medium chain triglyceride for long- and very long-chain fatty acids. Oral intake of medium amount of long-chain fatty acid (300kcal daily) was allowed, to facilitate compliance and to escape pigmentary retinopathy. After this nutritional management and lifestyle guidance about prevention of fatigue and starvation, the patient was free from severe rhabdomyolysis for more than three years, which had forced her to hospital management nine times in seven years.
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  • Megumi Bessho, Hideto Nakajima, Takumi Ito, Haruko Kitaoka
    2010 Volume 50 Issue 3 Pages 175-177
    Published: 2010
    Released: March 25, 2010
    JOURNALS FREE ACCESS
    We report an 87-year-old woman who presented with incomplete Brown-Séquard syndrome after reactivation of varicella-zoster virus (VZV). Two days after herpes zoster in the right side of the chest, she developed weakness of the right lower limb. Neurological examination revealed a spastic palsy in the right lower limb and left side loss of pain and temperature sense to T6. However, vibration and position sense was not impaired in both sides. Spinal T2-weighted MR images showed a high-intensity lesion in the right side of the spinal cord except posterior funiculus at the level of T2. Cerebrospinal fluid analysis showed 109 leukocytes/mm3, 79mg/dl protein, negative VZV PCR, elevated titer of anti-VZV IgM and IgG, and increase of IgG index. Although she was treated with a combination of acyclovir and steroid pulse therapy, her weakness in the right lower limb was not improved. In this case, since the posterior funiculus circulated from the posterior spinal artery was not involved, the incomplete Brown-Séquard syndrome may be caused by spinal cord infarction due to VZV vasculitis of the anterior spinal artery.
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