Publications (20)67.65 Total impact
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Article: A family with IVIg-responsive Charcot-Marie-Tooth disease.
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ABSTRACT: We report a family of intravenous immunoglobulin (IVIg)-responsive X-linked Charcot-Marie-Tooth disease Type 1 (CMT1X) with a novel gap junction protein 1 mutation. Two of three siblings in the family complained of subacute motor and sensory impairment, and their symptoms improved after the administration of IVIg. Additional IVIg treatment also resulted in similar improvement. The other also showed a mild improvement on IVIg. It has been suggested that an immune-mediated process is involved in the progression of neuropathy in CMT1X. The finding in our report provides evidence of susceptibility to immune-mediated demyelinating neuropathy in some form of CMT1X. Superimposed demyelinating neuropathy as well as a gradual deterioration of neuropathy over decades can be a clinical manifestation of CMT1X.Journal of Neurology 12/2012; · 3.47 Impact Factor -
Article: Middle Cerebellar Peduncles and Pontine T2 Hypointensities in ARSACS.
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ABSTRACT: BACKGROUND AND PURPOSE: Magnetic resonance imaging (MRI) of autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) cases in Quebec and Europe was reported to show linear hypointensities in T2-weighted and Fluid Attenuated Inversion Recovery (FLAIR) images of the pons. We attempted to clarify the characteristics of the brain MRI findings in ARSACS cases. METHODS: Eight Japanese early-onset ataxia patients with ARSACS confirmed molecularly were investigated. We performed neurological examination, SACS gene analysis, and MRI in the patients. RESULTS: Hypointensity lesions in the middle cerebellar peduncles in addition to the pons were observed in T2-weighted and FLAIR images in all eight cases. Although superior cerebellar atrophy was seen in all cases, this MRI finding might not be specific for ARSACS. Upper cervical cord and medulla oblongata atrophy was not observed in 3 of the 7 patients examined. CONCLUSION: Not only pontine but also middle cerebellar peduncle hypointensity lesions observed in T2-weighted and FLAIR images could be specific findings for ARSACS even in cases with variable clinical phenotypes. J Neuroimaging 2012;XX:1-4.Journal of neuroimaging: official journal of the American Society of Neuroimaging 01/2012; · 1.72 Impact Factor -
Article: Fatal subarachnoid hemorrhage caused by Aspergillus arteritis without angiographic abnormalities.
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ABSTRACT: No source of bleeding is detected by angiogram in 15-20% of patients with nonaneurysmal subarachnoid hemorrhage (SAH). This negative angiographic finding might suggest a benign prognosis. We describe a case of fatal SAH caused by Aspergillus arteritis without formation of fusiform dilatation or aneurysms. A 76-year-old man with a 2-month history of progressive visual loss due to pachymeningitis around the optic nerves suffered from SAH in the bilateral sylvian fissures. Repetitive serum galactomannan assay and angiography showed no abnormality. Post mortem examination revealed marked proliferation of Aspergillus in the granulomas of the frontal base dura mater. In addition, major trunks and several branches of the bilateral middle cerebral arteries were invaded by Aspergillus hyphae, which destroyed the walls in the absence of dilatation and aneurysms. Invasive aspergillosis of the CNS often forms a mycotic aneurysm. However, four autopsy cases of nonaneurysmal SAH due to invasive aspergillosis have been reported. The present case is the second autopsy case of Aspergillus arteritis without angiographic abnormality, resulting in fatal SAH. Aggressive and continuous antifungal therapy is absolutely necessary in suspected cases of invasive aspergillosis of the CNS, even if angiography is negative and therapeutic markers of aspergillosis are normal.Neuropathology 01/2012; 32(5):566-9. · 2.02 Impact Factor -
Article: Amyotrophic lateral sclerosis with demyelinating neuropathy.
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ABSTRACT: Amyotrophic lateral sclerosis (ALS) with demyelinating polyneuropathy is a rare condition. We describe two ALS patients with demyelinating neuropathy. Immunomodulatory therapies brought slight symptomatic benefits to the patients, but the treatments could not halt the progression of ALS. Chance coincidence of the two diseases is unlikely in view of the low prevalence. ALS, mainly consisting of progressive axonal degeneration, might show temporal demyelinating features of peripheral nerves both electrophysiologically and pathologically. The pathomechanism for the demyelination in ALS remains to be elucidated.Internal Medicine 01/2012; 51(14):1917-21. · 0.94 Impact Factor -
Article: Radicular myoclonus in a patient with Guillain-Barré Syndrome.
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ABSTRACT: A 53-year-old Japanese woman presented with myoclonus during the course of Guillain-Barré syndrome. The myoclonus was characterized by relatively regular involuntary movements, starting from proximal muscles of the right lower leg, and moving almost simultaneously towards the left lower leg and upper trunk. Surface electromyography revealed rhythmic synchronous discharges with 100-200 ms duration in the agonist and antagonist muscles at approximately 4 Hz. The jerk-locked back averaging, long latency reflexes, and somatosensory evoked potentials studies were normal. We report myoclonus due to radiculitis in a patient with Guillain-Barré syndrome.Internal Medicine 01/2012; 51(15):2021-3. · 0.94 Impact Factor -
Article: Novel compound heterozygous mutations of the SACS gene in autosomal recessive spastic ataxia of Charlevoix-Saguenay.
Clinical neurology and neurosurgery 12/2011; 114(6):746-7. · 1.30 Impact Factor -
Article: Fever of unknown origin.
The Lancet 11/2011; 378(9804):1756. · 38.28 Impact Factor -
Article: Taste impairment in Guillain-Barré syndrome: more frequent than thought?
Journal of the Peripheral Nervous System 09/2011; 16(3):270-1. · 2.80 Impact Factor -
Article: POEMS syndrome with a giant pancreas cyst.
Neurological Sciences 08/2011; 32(5):987-8. · 1.32 Impact Factor -
Article: Ulnar Nerve Palsy-like Motor and Sensory Loss Caused by A Small Cortical Infarct.
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ABSTRACT: A 56-year-old man with a small infarct in the left precentral knob area induced both motor and sensory impairments that were similar to right ulnar nerve palsy. The only difference from ulnar nerve palsy was that the patient showed sensory disturbance not only on the ulnar side but also on the radial side of the right ring finger.Journal of stroke and cerebrovascular diseases: the official journal of National Stroke Association 03/2011; -
Article: Clinical availability of skin biopsy in the diagnosis of Parkinson's disease.
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ABSTRACT: To determine whether skin biopsy is practically useful in the premortem diagnosis for Parkinson's disease (PD), we examined Lewy pathology in the skin of the chest wall and leg, obtained from 6-mm punch biopsies, using phosphorylated alpha-synuclein antibody in 20 patients with clinically diagnosed PD. Abnormal accumulation of alpha-synuclein was found in the chest skin of two (10%) of 20 patients, but not in the leg. Although skin biopsy combined with a conventional immunohistochemistry for alpha-synuclein is not sufficient as a diagnostic tool, we could firstly demonstrate Lewy pathology in premortem tissue. The skin remains to be a promising tissue to be examined for the premortem diagnosis of PD.Neuroscience Letters 12/2009; 469(3):357-9. · 2.11 Impact Factor -
Article: Cerebral venous thrombosis with dural arteriovenous fistulas and antiphospholipid syndrome: a case report.
Neurological Sciences 10/2009; 31(2):237-8. · 1.32 Impact Factor -
Article: An unusual case of chronic relapsing tetanus associated with mandibular osteomyelitis.
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ABSTRACT: A 55-year-old man underwent radiation therapy due to malignant lymphoma of the neck. Eight years after the therapy he developed tetanus. It appears that the radiation therapy resulted in mandibular necrosis, and that this lesion may have been the infectious focus of tetanus. Treatment with penicillin G was very effective in the acute stage, and chronic administration of metronidazole prevented relapse of the disease. However in spite of injections of tetanus toxoid, symptoms of tetanus returned when the administration of metronidazole was discontinued because the infectious focus could not be completely removed. This is the first report of chronic relapsing tetanus associated with radiation-induced mandibular osteomyelitis, and demonstrates that tetanus can occur due to mandibular focus but the chronic administration of metronidazole can prevent relapse.Internal Medicine 02/2009; 48(15):1311-3. · 0.94 Impact Factor -
Article: A novel mutation in the arylsulfatase A gene associated with adult-onset metachromatic leukodystrophy without clinical evidence of neuropathy.
European Neurology 11/2008; 60(6):310-1. · 1.81 Impact Factor -
Article: Reuptake of L-DOPA-derived extracellular DA in the striatum of a rodent model of Parkinson's disease via norepinephrine transporter.
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ABSTRACT: To determine the role of norepinephrine transporter in reuptake of L-DOPA-derived extracellular DA in the DA-denervated Parkinsonian striatum, we examined extracellular DA levels in the striatum of 6-hydroxyDA-lesioned rats that received L-DOPA (50 mg/kg with 12.5 mg/kg of benserazide) and L-DOPA plus desipramine (25 mg/kg), a selective norepinephrine reuptake inhibitor, using in vivo microdialysis. The pretreatment with desipramine increased levels of extracellular DA derived from administrated L-DOPA in the DA-denervated striatum. This study provides evidence that L-DOPA-derived DA is taken up by the norepinephrine transporter, instead of the dopamine transporter, in the striatum with dopaminergic denervation. This result suggests that the norepinephrine transporter could be a promising target in the treatment for Parkinson's disease.Synapse 09/2008; 62(8):632-5. · 2.94 Impact Factor -
Article: A small trinucleotide expansion in the TBP gene gives rise to a sporadic case of SCA17 with abnormal putaminal findings on MRI.
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ABSTRACT: A Japanese woman developed gait disturbances at 25 years of age, and subsequently underwent gradual changes in her personality. By the age of 42, she showed clear signs of dementia and cerebellar ataxia, and displayed behavioral abnormalities, choreic movements and hyperreflexia. The findings of MRI not only showed cerebellar and cerebral atrophy, but also revealed putaminal rim hyperintensity on T2-weighted images. We identified a heterozygously expanded CAG/CAA repeat (45/36) within the TATA-binding protein gene, leading to a diagnosis of SCA17. These results show that a 45 CAG/CAA repeat is pathological, giving rise to early-onset SCA17.Internal Medicine 02/2008; 47(24):2179-82. · 0.94 Impact Factor -
Article: [A case of brain nocardiosis successfully treated with minocycline].
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ABSTRACT: A 60-year-old man with surgically treated nocardia pyothorax was referred to our hospital since he became drowsy. Brain MRI revealed multiple brain abscesses. His cerebrospinal fluid (CSF) showed increase in polymorphonuclear cells and decrease in glucose. Since he was allergic to sulfamethoxazole * trimethoprim, ceftriaxone and then minocycline were given. Minocycline resulted in dramatic improvement of neurological symtoms, MRI findings and CSF cell count. PCR analysis of 16S ribosomal DNA using his resected thoracic wall revealed that nocardia from his tissue was strain IFM0860. Strain IFM0860 nocardia was found to be sensitive to minocycline but not to sulfamethoxazole * trimethoprim and ceftriaxone. Intravenous administration of minocycline was followed by three-year per os administration of minocycline during which he had no recurrence of brain abscess. Thus, brain nocardiosis could be successfully treated with appropriate antibiotics. The lesson from the present case is that identification of the type of nocardia by PCR analysis of 16S ribosomal DNA could help accomplish tailor-made antibiotic therapy.Nō to shinkei = Brain and nerve 07/2006; 58(6):505-8. -
Article: Reversible multifocal conduction block in sarcoid neuropathy.
Journal of the Peripheral Nervous System 04/2006; 11(1):93-5. · 2.80 Impact Factor -
Article: Disseminated intraparenchymal microgranulomas in the brainstem in central nervous system sarcoidosis.
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ABSTRACT: We report a 70-year-old woman with sarcoidosis and multiple cranial nerve palsy. The patient suffered from dysarthria, dysphagia and weakness of the upper and lower extremities and died of sepsis. No abnormalities were noted in brain MRI. At autopsy, numerous epithelioid granulomas with Langhans giant cells were present in the bilateral lungs, including the hilar lymph nodes. The brain had a normal external appearance. Histologically, there were brainstem parenchymal lesions consisting of many microgranulomas, lymphocytic infiltration, activated microglias and astrocytosis. Perivascular lympocytic cuffing was also seen. Neither granulomas nor lymphocytic infiltration were seen in the leptomeninges. The present case was considered to be a peculiar type of neurosarcoidosis, that is, "sarcoid brainstem encephalitis".Neuropathology 01/2006; 25(4):361-4. · 2.02 Impact Factor -
Article: Venous cerebral infarction in a patient with peripheral hemodialysis shunt and occlusion of the left brachiocephalic vein.
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ABSTRACT: Intracranial venous congestion is a rare condition in hemodialysis patients with central venous occlusion. We report a patient with cerebral venous infarction resulting from high reflex flow into the cranium induced by an arteriovenous hemodialysis shunt in the arm and occlusion of the brachiocephalic vein. This case illustrates that abnormal extracranial venous circulation should be considered when cerebral venous congestion is assumed to produce neurologic symptoms in patients with an arteriovenous shunt.Journal of stroke and cerebrovascular diseases: the official journal of National Stroke Association 20(4):381-3.
Top Journals
Institutions
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2009–2012
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Aomori Prefectural Central Hospital
Aomori-shi, Aomori-ken, Japan
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2008
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Hirosaki University
- Department of Neurology
Hirosaki, Aomori-ken, Japan
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