Rinsho Shinkeigaku
Online ISSN : 1882-0654
Print ISSN : 0009-918X
ISSN-L : 0009-918X
Volume 50, Issue 9
Displaying 1-9 of 9 articles from this issue
Reviews
  • Hideki Mochizuki
    2010 Volume 50 Issue 9 Pages 623-627
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    Parkinson's disease (PD) is one of the most common chronic neurodegenerative diseases. Levodopa provides the best symptomatic benefit with the fewest short-term adverse effects, but its use is associated with the development of motor fluctuations and dyskinesias. The introduction of dopamine agonists early in the treatment of PD leads to a delay of these motor complications, but all available dopamine agonists may cause profound adverse effects in some patients. The objective of this manuscript is to review the initial treatment, the neurotoxicity of levodopa, the swallowing disturbance and QOL of PD. Levodopa still is the gold standard for PD. However, the treatment decisions should be based on considerations of risks versus benefits for individual patients.
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  • Takashi Nagamine
    2010 Volume 50 Issue 9 Pages 628-633
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    The idea that each brain function is undertaken by a small part of focal brain was established in 19th century. Since then, trials to reveal an individual function in each area have been continued. Although intraoperative electric stimulation/suppression has been considered to be the most secure way of exploration of brain function, this method has several limitations due to its invasive nature. On the other hand, recent advancement of explorative measure with high sensitivity has enabled measurement of physical phenomenon accompanied with change of brain function from outside of the skull. These methods can be applied to normal subjects using their capability of repetitive and multiple measurements and have provided new findings. Combination of several techniques knowing characteristics and limitation of each method will provide new insight into the brain.
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Case Reports
  • Akira Inukai, Taiji Katayama, Masakuni Kenjo, Yuki Yokokawa, Ikuko Aib ...
    2010 Volume 50 Issue 9 Pages 634-640
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    A previously healthy 55-year-old woman developed abnormal sensation on the right occipital region. It expanded for the following three weeks. On admission, examination revealed abnormal and decreased sensation in touch and pinprick at right C2 to C6 dermatome without skin lesion. There was no muscle weakness. Deep tendon reflexes were more active in the right than in the left. MRI demonstrated a lesion of isointensity on T1-weighted, hyperintensity on T2-weighted, which was enhanced with contrast material on gadolinium-enhanced T1-weighted image at the upper cervical spinal cord corresponding to C2. Laboratory studies showed no immunosuppression and autoantibodies. The antibody index to varicella-zoster virus (VZV) was elevated in the cerebrospinal fluid (CSF). This finding prompted us to a diagnosis of myelitis of zoster sine herpete.
    VZV is thought to be a causative agent in cases of CNS infections of unknown etiology such as myelitis, even in the absence of skin manifestations. Amplification of VZV DNA by PCR in the CSF and the detection of an intrathecal production of anti-VZV antibodies have important diagnostic value, although their presence depends on the timing of the CSF sampling. The percentage of PCR-positive cases drops after seven or ten days, whereas that of specific antibodies-positive cases elevates. Because VZV myelitis are usually protracted, PCR does not always provide an exquisite sensitivity. Thus, the evaluation of antibody index provides the evidence of intrathecal production of anti-VZV antibodies. That is expressed as CSF antibody titer/serum antibody titer/CSF IgG/serum IgG. This quotient superior to 1.5 or 2.0 suggests CNS synthesis. As the sample of our patient was taken relatively late, this value was diagnostic.
    We would like to emphasize the importance of making precise diagnosis and adequate initial treatment in patients with myelitis of unknown etiology even if there is no skin lesions.
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  • Yosuke Miyaji, Hiroshi Doi, Shigeru Koyano, Yasuhisa Baba, Yume Suzuki ...
    2010 Volume 50 Issue 9 Pages 641-644
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    We report a 50-year-old woman with an unremarkable birth and developmental history, and with no family history of neurological disorders. The patient had a 6-year history of progressive cervical dystonia, oral dyskinesia, and hyperreflexia. She was initially considered to have spastic paraparesis of unknown cause. Because brain MRI showed mild atrophy of the cerebellar vermis, genetic analysis for spinocerebellar ataxia types 1, 2, 3, 6, 7, 8, 12, and 17, and dentatorubral-pallidoluysian atrophy was performed. The results revealed an abnormal expansion of CAG repeats (38 repeats) in one allele of ATXN2, and the patient was diagnosed with spinocerebellar ataxia type 2 (SCA2). She had no major clinical features of SCA2 such as cerebellar ataxia, slow saccade, or hyporeflexia. Recent reports have shown the CAG repeat expansion in ATXN2 to be detected in patients with familial L-dopa-responsive parkinsonism. The present case suggests that CAG repeat expansion in ATXN2 may be detected in some patients with spastic paraparesis, and that wide variations of clinical manifestations exist in SCA2.
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  • Ryuji Yajima, Kensaku Kasuga, Tomoe Sato, Takeshi Ikeuchi, Masatoyo Ni ...
    2010 Volume 50 Issue 9 Pages 645-650
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    We report the case of a 76-year-old man with apraxia of eyelid opening (AEO) and amyotrophic lateral sclerosis with dementia (ALS-D)/frontotemporal lobar degeneration with motor neuron disease (FTLD-MND). The initial symptom was AEO. Neurological examination revealed mainly bulbar symptoms with a neurogenic pattern on needle electromyograms of the tongue muscles and the biceps muscles. Furthermore, he developed severe dementia with frontal lobe dysfunction and progressive non-fluent aphasia. Brain magnetic resonance imaging revealed severe cerebral atrophy and leukoaraiosis in the bilateral frontal lobes and the anterior part of the bilateral temporal lobes; brain 99mTc ethyl cysteinate dimer single photon emission computed tomography (ECD SPECT) showed hypoperfusion in the same areas.
    The patient showed improvement in stereotyped behavior and AEO after treatment with 50mg/day of fluvoxamine maleate (the initial dose was 25mg/day). Because serotonin receptors are markedly reduced in the frontal and temporal cortexes of patients with FTLD, we considered that dysfunction of the serotonergic system in the frontotemporal lobe caused AEO.
    Considering the findings of this case along with those of previous reports, we propose that there is a relatively homogeneous development of ALS-D/FTLD-MND with AEO.
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  • Yasushi Iwasaki, Keiko Mori, Masumi Ito, Akira Deguchi, Taizo Shiraish ...
    2010 Volume 50 Issue 9 Pages 651-655
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    A Japanese male showed gradually progressing dementia with psychiatric symptoms including abnormal behavior, night and day reversal, nocturnal delirium, loud shouting, agitation, resistance to care, and loud soliloquy. The patient had a history of right cerebral embolism due to atrial fibrillation 1 month before the onset of dementia. Head CT revealed widespread cerebral infarction in the right cerebral hemisphere with bilateral lateral ventricular dilatation. The patient died at the age of 83, 10 months after the onset of cerebral embolism. The clinical diagnosis was mixed-type dementia.
    On autopsy the brain weighed 1,160g. Widespread cerebral amyloid angiopathy (CAA) was observed, with distribution most severe in the cerebral cortical vessels and slightly milder in the leptomeningial and subarachnoid vessels. The artery, arteriole, and capillary walls were thickened by the deposition of amorphous, eosinophilic and β-protein immunopositive amyloid. Aβ-deposition was more severe in capillaries and CAA tended to be more severe in the occipital regions. Multiple cortical microinfarcts were found, particularly in the crests of the cerebral gyri of watershed zones. Cerebral white matter, basal ganglia, thalamus, brainstem and spinal cord were relatively preserved from CAA. Infarction was not apparent, except for an embolic lesion in the right cerebral hemisphere and the cortical microinfarcts. We did not observe fibrinoid necrosis, granulomatous angiitis or giant cell reaction associated with CAA-vasculopathies. Rare instances were observed of neurofibrillary tangles and senile plaques corresponding to Braak stages II and A, respectively.
    We thought the multiple cortical microinfarcts occurred due to chronic hypoperfusion associated with CAA-associated vasculopathies of capillaries in the cerebral cortex. We suspected that the dementia was influenced by the CAA with multiple cortical microinfarcts. Pathologic findings of the patient suggest that CAA without AD-related Aβ-deposition might exist and that capillary Aβ-deposition might be an important factor of hemodynamic perturbation.
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  • Miho Shinohara, Masahiro Nagao, Shiro Matsubara
    2010 Volume 50 Issue 9 Pages 656-660
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    A 62-year-old immunocompetent woman presented with 11 days of headache, 2 days of right eye ache and 1 day of fever and lethargy. Neurological examination revealed nuchal stiffness, right proptosis, bilateral ptosis, and right abducens palsy. Cerebrospinal fluid (CSF) examination revealed elevated white cell count (164/μl) and protein level (115mg/dl). Cranial MRI showed sphenoid sinusitis, thromboses of the right superior ophthalmic vein, bilateral cavernous sinuses, left sphenoparietal sinus and left sigmoid sinus, and enhanced meninges. Purulent meningitis and multiple mycotic cerebral venous sinus thromboses were diagnosed. After empirical therapy with meropenem, fever persisted and CSF cell count further elevated (668/μl on day 3). Additional treatment with liposomal amphotericin B (L-AMB) and low-dose heparin from day 3 ameliorated her symptoms and lowered her CSF cell count. Laboratory test on admission later revealed elevated serum aspergillus antigen (index=3.6) and positive blood culture for streptococcus viridans. L-AMB was replaced by voriconazole due to skin rash, and the latter was changed to itraconazole due to drug-induced hepatitis. She was discharged without complication and has been free of recurrence for 7 months. Aspergillus has a propensity to invade cerebral vessels and meninges, causing local thrombosis and meningitis with high mortality and morbidity. Direct penetration from adjacent sphenoid sinus can be a cause of cavernous sinus thrombosis, due to extreme thinness of the wall of sphenoid sinus. Cerebral venous sinuses lack valves, and this may facilitate the spread of mycotic thrombus to the other sinuses. Early preemptive treatment with antimycotic agents brought a favorable outcome to our patient.
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  • Yoshiyuki Kondo, Madoka Mori-Yoshimura, Yukiko K. Hayashi, Yasushi Oya ...
    2010 Volume 50 Issue 9 Pages 661-665
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    We report a 29-year-old man with limb-girdle muscular dystrophy type 2M (LGMD2M) caused by a compound heterozygous mutation of 3-kb insertion in the 3'-untranslated region and c.1073A>C (p.Q358P) mutation in exon 9 in FKTN. He had been diagnosed since childhood as having Becker muscular dystrophy based on limb-girdle muscle weakness and calf muscle hypertrophy. Loss of ambulation occurred at age 26 years and cardiomyopathy was noted one year later. Muscle biopsy at age 29 revealed dystrophic changes with loss of immunoreactivity to α-dystroglycan (α-DG), which prompted us to analyze FKTN and subsequent establishment of the diagnosis of LGMD2M. Brain MRI revealed hypoplasia of the right cerebellar hemisphere and tonsil. Dysplastic part was present in the lower medial part of the hypoplastic hemisphare, which was bordered by a deep cleft. Previously reported LGMD2M patients had mild or minimal muscle weakness in addition to dilated cardiomyopathy. In contrast, our patient had more severe skeletal muscle weakness and loss of ambulation. Treatment with β-blockers or angiotensin II converting enzyme blockers has been reported to be efficacious for cardiomyopathy in patients with muscular dystrophy. The precise diagnosis should be established early in patients with muscular dystrophy complicated with cardiomyopathy.
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Brief Clinical Note
  • Kanako Kakinuma, Masashi Nakajima, Soutarou Hieda, Hiroo Ichikawa, Mit ...
    2010 Volume 50 Issue 9 Pages 666-668
    Published: 2010
    Released on J-STAGE: October 04, 2010
    JOURNAL FREE ACCESS
    A 63-year-old man with hypercholesterolemia developed sensory and motor disturbances in the ulnar side of the right hand, and over three days the weakness evolved to entire right arm. Examination on the 6th day after onset showed mild lower facial palsy in addition to the upper limb weakness on the right. The weakness involved entire right arm sparing shoulder girdle muscles, which was worse in the 4th and 5th digits with claw hand deformity of the hand. Magnetic resonance imaging showed multiple small infracts in the centrum semiovale as well as in the medial side of the precentral knob on the left. Magnetic resonance angiography, ultrasonography, and 3D-CT angiography of the neck showed severe stenosis associated with unstable plaque of the left internal carotid artery. Hemodynamic mechanisms including microemboli and hypoperfusion associated with severe internal carotid artery stenosis are likely to cause stroke in evolution after initial presentation of pseudo-ulnar palsy in the present case.
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