Publications (37)49.35 Total impact
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Article: Hyperosmolar hyperglycemic state in advanced amyotrophic lateral sclerosis.
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ABSTRACT: Our objective was to describe cases of hyperosmolar hyperglycemic state (HHS) in advanced amyotrophic lateral sclerosis (ALS) patients and discuss its pathophysiology. Five ventilator-dependent patients with ALS, with no previous history of diabetes, showed development of marked hyperglycemia (plasma glucose levels of 755-1544 mg/dl) after preceding infectious episodes. All patients had severe generalized muscle wasting and tetraplegia. The initial manifestations of HHS were fever, drowsiness, or polyuria. Hydration and intravenous insulin therapy were markedly effective, resulting in favorable recovery without the necessity of chronic medication for hyperglycemia in all cases. Seventy-five grams oral glucose tolerance tests performed via feeding tubes in two patients after the successful treatment of HHS revealed increased insulin resistance and diminished early-phase insulin secretion with preserved total insulin secretion. In conclusion, a marked loss of skeletal muscle, the largest glucose consumer of the human body, with background abnormality of early-phase insulin secretion, might be a causative factor of HHS in advanced ALS.Amyotrophic Lateral Sclerosis 12/2010; 12(5):379-81. · 3.40 Impact Factor -
Article: Electrophysiological assessment of corticorespiratory pathway function in amyotrophic lateral sclerosis.
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ABSTRACT: Respiratory muscle paralysis is inevitable in the clinical course of amyotrophic lateral sclerosis (ALS). Our objective was to electrophysiologically assess the function of the phrenic nerve and diaphragm motor cortex in ALS. Phrenic nerve M waves, diaphragm motor evoked potentials (MEPs) induced by transcranial magnetic stimulation, and their clinical correlations were analyzed in 29 ALS patients. The M wave amplitude was significantly lower in patients than in healthy control subjects (p<0.001). The MEP amplitudes both in the expiratory and inspiratory phases were significantly decreased in patients (p<0.01). In particular, 15 patients showed no MEPs in the expiratory phase, six of whom also showed no MEPs in the inspiratory phase. Five of them had no respiratory complaints. There was a weak, non-significant correlation between the inspiratory MEP amplitude and forced vital capacity (p=0.052). We conclude that the loss of MEP might reflect the subclinical involvement of the voluntary respiratory drive from the diaphragm motor cortex, potentially leading to further respiratory deterioration.Amyotrophic Lateral Sclerosis 09/2009; 11(1-2):57-62. · 3.40 Impact Factor -
Article: Mixed lineage kinase 2 and hippocalcin are localized in Lewy bodies of Parkinson's disease.
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ABSTRACT: The expression of hippocalcin, a calcium-sensor protein of the recoverin family, and mixed lineage kinase 2 (MLK2) in Lewy bodies (LBs) was immunohistochemically examined in patients with Parkinson's disease (PD). Hippocalcin and MLK2 were colocalized in the halo of LBs, and neither protein was detected in normal pigmented neurons. Since hippocalcin binds to the C-terminal region of MLK2 [Nagata K., Puls A, Futter C, Aspenstrom P, Schaefer E, Nakata T et al., The MAP kinase kinase kinase MLK2 co-localizes with activated JNK along microtubules and associates with kinesin superfamily motor KIF3. EMBO J 1998;17:149-1588.], it may constitutively activate MLK2. Both hippocalcin and MLK2 may be associated with the pathogenesis of PD.Journal of the neurological sciences 04/2009; 281(1-2):51-4. · 2.32 Impact Factor -
Article: Glycogen synthase kinase-3beta is associated with Parkinson's disease.
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ABSTRACT: We immunohistochemically examined the expression of glycogen synthase kinase-3beta (GSK-3beta) in the brains of Parkinson's disease (PD) patients. GSK-3beta was localized in punctate structures in the cytosol of subsets of neurons in the midbrain and upper pons. GSK-3beta was also localized in Lewy bodies (LBs) as was phosphorylated GSK-3beta (Ser9) (pGSK-3beta (Ser9)). Both GSK-3beta and pGSK-3beta (Ser9) were localized specifically in the halo of LBs. The core of LBs was negative for GSK-3beta, while pGSK-3beta (Ser9) was present in only a small number of LB cores. Cortical LBs were positive for pGSK-3beta (Ser9) but not for GSK-3beta. Neither GSK-3beta nor pGSK-3beta (Ser9) was present in glial cytoplasmic inclusions (GCIs) in the brains of multiple system atrophy (MSA) patients. Our results suggest that GSK-3beta plays a role in the pathogenesis of PD but not in that of MSA.Neuroscience Letters 12/2008; 449(2):103-7. · 2.11 Impact Factor -
Article: [Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis--relation of respiratory function at the time of PEG to survival length].
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ABSTRACT: We investigated retrospectively 35 patients with amyotrophic lateral sclerosis who underwent percutaneous endoscopic gastrostomy (PEG), focusing on their respiratory function at the time of PEG. There were statistically significant correlations between the forced vital capacity and arterial carbon dioxide pressure, and the duration from the PEG to death or initiation of ventilatory support. In comparison between the 20 patients who died or was placed on ventilator within 6 months after PEG (group A) and the 15 patients who survived more than 6 months without ventilatory support after PEG (group B), the arterial carbon dioxide pressure showed a significantly high level in the group A (mean 47.5 +/- S.D. 5.4 mmHg) than in the group B (42.2 +/- 5.2 mmHg) (P < 0.05). PEG should be performed in ALS patients while respiratory function is well preserved, especially before arterial carbon dioxide pressure is increased.Rinsho shinkeigaku = Clinical neurology 10/2008; 48(10):721-6. -
Article: [A nationwide survey of ALS patients on trachoestomy positive pressure ventilation (TPPV) who developed a totally locked-in state (TLS) in Japan].
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ABSTRACT: We conducted a nationwide survey of ALS patients on tracheostomy positive pressure ventilation (TPPV) who had developed a totally locked-in state (TLS) during the period from August to November 2006, in Japan. TLS occurred in 89 of 709 (13%) ALS patients on TPPV. On the second investigation, 29 of 41 patients with TLS showed complete palsy of more than two voluntary motor systems out of 4 motor systems [respiratory, pontine and medullar (bulbar), limb and external ocular motor systems] successively during a certain six months. The conventional classification of ALS based on the initial symptoms (bulbar, upper limb and lower limb type) was not found to be useful for predicting the onset of TLS. Seventy percent the patients developed TLS within 5 years after the start of TPPV. Thirty-seven (90%) patients finally developed total ophthalmoplegia at the onset of TLS, while one patient eventually developed complete bulbar palsy. One of 11 ALS patients with TLS, whom we experienced at Tokyo Metropolitan Neurological Hospital, also eventually showed complete palsy of the pontine (bulbar) motor system (inability to pull back the jaw). Due to the fact that TLS is a state of complete palsy of the voluntary motor systems for communication, which may occur during the course of ALS in around 15% of patients, further clinical investigation of TLS including cognition is thus considered to be essential for improving the palliative care of ALS patients on TPPV.Rinsho shinkeigaku = Clinical neurology 08/2008; 48(7):476-80. -
Article: High cognitive function of an ALS patient in the totally locked-in state.
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ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a motor neuron disease characterized by progressive degeneration of upper and lower motor neurons. Patients with ALS progressively lose their ability to control voluntary movements and occasionally enter the totally locked-in state (TLS), in which they cannot move any part of their bodies including the eyes. In this study, we clarified the preserved abilities and reorganization of the motor system of a 73-year-old patient with ALS in the TLS using optical topography, a recently developed extension of near-infrared spectroscopy. The patient performed four cognitive tasks: dichotic listening, covert singing, word fluency, and motor imagery. The bilateral prefrontal and bilateral sensorimotor areas were activated during the two language-related tasks (dichotic listening task and the word fluency), the right prefrontal and sensorimotor areas during the covert singing task, and the right prefrontal and dorsal sensorimotor areas during the motor imagery task. Contralateral sensorimotor activation was not observed in the motor imagery task. These results suggest that cognitive functions can be preserved in ALS in the TLS, with sensorimotor areas playing an important role.Neuroscience Letters 05/2008; 435(2):85-9. · 2.11 Impact Factor -
Article: Neuropathology in Amyotrophic Lateral Sclerosis Patients on Respirators: Uniformity and Diversity in 13 Cases
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ABSTRACT: We performed a neuropathological study on 13 patients with amyotrophic lateral sclerosis (ALS) who had been on respirator support. Twelve patients had developed upper and lower motor neuron signs, exclusively, before being placed on respirators. One patient was diagnosed as having the pseudoneuritic type of ALS. The main pathological findings in the 13 cases were the simultaneous involvement of both motor neurons and the presence of Bunina bodies and ubiquitinated filaments in the anterior horn cells. While on the respirator, two rapidly progressing patients developed supranuclear ophthalmoplegia and ended in totally locked-in state. These two cases showed widespread systemic degenerative changes in the central nervous system (CNS) that involved the frontoparietal cortices adjacent to the precentral gryrus, the pallido-luysio-nigral system, thalamus, hypothalamus, amygdala, brain stem tegmentum, dentate-rubral system, Clarke's spinocerebellar-middle root zone and Onuf's nucleus. Eight normally or rapidly progressing patients developed supranuclear ophthalmoplegia, with or without emotional disinhibition and blood pressure lability. Some of the CNS structures affected in the two patients described above, were also altered in these eight cases. We attribute the ophthalmoplegia to the involvement of the brain stem tegmentum and frontal cortices, which are concerned with eye movement. In addition, the emotional and autonomic labilities of some of the patients may be related to alterations of the limbic system. By contrast, the clinicopathological features of the remaining three, slowly progressing patients were confined to both motor neurons. On the basis of these findings we address the intrinsic uniformity of ALS and some of its diversitiesNeuropathology 10/2007; 13(3):229 - 236. · 2.02 Impact Factor -
Article: [Percutaneous endoscopic gastrostomy in patients with intractable neurological diseases--retrospective study of the indication, complication and prognosis].
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ABSTRACT: We investigated retrospectively 157 neurological patients who underwent percutaneous endoscopic gastrostomy (PEG) since 2003 May to 2006 January, focusing on their neurological diagnosis, complication, relation to ventilatory support and survival prognosis. Among 157 patients, 42 patients had amyotrophic lateral sclerosis, 39 Parkinson's disease, and 27 multiple system atrophy. The percentage for the neurodegenerative diseases was 68.0%. The most frequent complication during PEG procedure was transient hypoxia (8 patients), three of whom needed oxygen therapy, but no patients received emergent artificial ventilation. After PEG. six patients experienced accidental self-removal of the catheter, but there was no subsequent peritonitis. Seven patients were on tracheostomy positive-pressure ventilation during PEG. and two on non-invasive intermittent positive-pressure ventilation around the PEG period, in all of whom the PEG was carried out without any trouble. Investigation on survival after PEG showed that nine ALS patients died within six months because of respiratory failure. We conclude that PEG for neurological patients was generally performed safely. For ALS patients, however, respiratory function should be carefully monitored, and PEG should be performed in ALS patients before their respiratory function becomes worse.Rinsho shinkeigaku = Clinical neurology 10/2007; 47(9):565-70. -
Article: [Autopsy case of atypical type of alzheimer's disease clinically diagnosed as corticobasal degeneration].
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ABSTRACT: A 60-year-old, right-handed man developed gait disturbance. He also had difficulty in dressing and demonstrated depressive status, but did not have memory impairment. He was diagnosed as having corticobasal degeneration (CBD) because of right cortical atrophy and contralateral movement disorder. He died at the age of 69. The pathological diagnosis was not CBD but Alzheimer's disease. The brain showed severe atrophy, especially in the right superior parietal lobe and postcentral gyrus. The cortical lesion was most marked in the right parietal lobe within the neocortex, and was more severe than that in the limbic area. Clinically this patient was highly suspected to have CBD. This case, however, is categorized into atypical Alzheimer's disease with a focal cortical syndrome.Rinsho shinkeigaku = Clinical neurology 10/2007; 47(9):581-4. -
Article: [Case of ocular neuromyotonia with transient and gaze-induced diplopia].
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ABSTRACT: A 71-year-old man had recurrent spells of transient diplopia for about four years. Vertical diplopia were induced by downward gaze, mostly to the lower-left, and lasted for about ten seconds. During the spells, limitation in elevation of the left eye was observed. Other neurological examinations were normal except for a minor paresis of the left trochlear nerve revealed only by detailed neuroophthalmological examination. The patient was treated with carbamazepine 200 mg daily, which abolished the spells. He was diagnosed to have idiopathic ocular neuromyotonia of the left third or fourth nerve. Ocular neuromyotonia is a rare disturbance of ocular motility, characterized by paroxysmal monocular involuntary spasms of one or more extraocular muscles supplied by the third, fourth or sixth cranial nerves. Past studies showed that most patients had radiation therapy to the parasellar or sellar regions. Slight injury to the peripheral nerves such as one caused by radiation may have an important role in the development of ocular neuromyotonia. Furthermore, patients with ocular neuromyotonia often benefit from the use of carbamazepine, a membrane-stabilizing agent. We report a case of idiopathic ocular neuromyotonia without history of damage to the cranial nerves. It is the first report in Japan.Rinsho shinkeigaku = Clinical neurology 07/2007; 47(6):344-7. -
Article: Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination.
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ABSTRACT: To evaluate the effect of interferon beta-1b (IFNB-1b) on multiple sclerosis (MS) with severe optic nerve and spinal cord demyelination, we examined the relationship between IFNB-1b treatment outcome and the clinical and genetic characteristics of three types of demyelinating diseases of the central nervous system, i.e., neuromyelitis optica (NMO), MS and MS with severe optic-spinal demyelination. Japanese MS frequently carried HLA DPB1*0501, which is associated with NMO. MS with DPB1*0501 showed severe optic-spinal demyelination represented by longitudinally extensive spinal cord lesion, blindness and CSF pleocytosis. IFNB-1b treatment did not succeed in these patients because of the increase of optic nerve and spinal cord relapse and other severe side effects. IFNB-1b should not be administered to demyelinating patients with genetic and clinical characteristics mimicking NMO such as HLA DPB1*0501 allele, longitudinally extensive spinal cord lesion, blindness and CSF pleocytosis even if they have symptomatic cerebral lesions as typically seen in MS. The present study strongly suggests that these patients should be diagnosed as having NMO.Journal of the Neurological Sciences 02/2007; 252(1):57-61. · 2.35 Impact Factor -
Article: Polyglutamine expansion mutation yields a pathological epitope linked to nucleation of protein aggregate: determinant of Huntington's disease onset.
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ABSTRACT: Polyglutamine (polyQ) expansion mutation causes conformational, neurodegenerative diseases, such as Alzheimer's and Parkinson's diseases. These diseases are characterized by the aggregation of misfolded proteins, such as amyloid fibrils, which are toxic to cells. Amyloid fibrils are formed by a nucleated growth polymerization reaction. Unexpectedly, the critical nucleus of polyQ aggregation was found to be a monomer, suggesting that the rate-limiting nucleation process of polyQ aggregation involves the folding of mutated protein monomers. The monoclonal antibody 1C2 selectively recognizes expanded pathogenic and aggregate-prone glutamine repeats in polyQ diseases, including Huntington's disease (HD), as well as binding to polyleucine. We have therefore assayed the in vitro and in vivo aggregation kinetics of these monomeric proteins. We found that the repeat-length-dependent differences in aggregation lag times of variable lengths of polyQ and polyleucine tracts were consistently related to the integration of the length-dependent intensity of anti-1C2 signal on soluble monomers of these proteins. Surprisingly, the correlation between the aggregation lag times of polyQ tracts and the intensity of anti-1C2 signal on soluble monomers of huntingtin precisely reflected the repeat-length dependent age-of-onset of HD patients. These data suggest that the alterations in protein surface structure due to polyQ expansion mutation in soluble monomers of the mutated proteins act as an amyloid-precursor epitope. This, in turn, leads to nucleation, a key process in protein aggregation, thereby determining HD onset. These findings provide new insight into the gain-of-function mechanisms of polyQ diseases, in which polyQ expansion leads to nucleation rather than having toxic effects on the cells.PLoS ONE 02/2007; 2(7):e635. · 4.09 Impact Factor -
Article: Trigger finger as an initial manifestation of familial amyloid polyneuropathy in a patient with Ile107Val TTR.
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ABSTRACT: We describe a Japanese family with transthyretin Val107-related familial amyloid polyneuropathy (FAP). The clinical features were high-aged onset, sensorimotor polyneuropathy, carpal tunnel syndrome (CTS) and trigger finger. In addition, the proband showed cardiac conduction block and amyloid deposition in the sural nerve and dermis. Trigger finger may be a so far unknown clinical manifestation of Val107 FAP due to amyloid deposition in the connective tissue like CTS.Internal Medicine 02/2007; 46(8):501-4. · 0.94 Impact Factor -
Article: Characterization of the T cell receptor repertoire in the Japanese neuromyelitis optica: T cell activity is up-regulated compared to multiple sclerosis.
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ABSTRACT: To characterize T cell immunity in Japanese neuromyelitis optica (NMO), we examined the T cell receptor (TCR) repertoire in NMO patients with complementarity-determining region 3 (CDR3) spectratyping and compared the results with those from multiple sclerosis (MS) patients and healthy subjects. Both NMO and MS patients had a larger number of clonally expanded Vbeta genes than healthy subjects. Moreover, NMO patients had a significantly larger number of expanded Vbetas than MS patients. The detailed analysis revealed that Vbeta1 and Vbeta13 were significantly activated in NMO than MS. These results reflected unique pathophysiology of Japanese NMO, which is distinguishable from that of MS. Furthermore, longitudinal examinations of the TCR repertoire demonstrated that the number of clonally expanded Vbetas in NMO correlates with the Kurtzke Expanded disability status scale (EDSS). Although the activation pattern of the TCR repertoire in relapsing-remitting MS (RRMS) was similar to that in NMO, secondary progressive MS (SPMS) patients with longer disease durations and higher EDSS scores consistently had a smaller number of clonally expanded Vbetas than RRMS patients. Detailed TCR investigations will provide useful information to evaluate the clinical and immunological status of NMO and MS and to develop effective immunotherapies.Journal of the Neurological Sciences 12/2006; 249(2):145-52. · 2.35 Impact Factor -
Article: [Transcortical sensory aphasia due to extensive infarction of left cerebral hemisphere].
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ABSTRACT: We report a case of transcortical sensory aphasia occurred after extensive infarction of left cerebral hemisphere. A 68-year-old, right-handed man with atrial fibrillation suddenly developed cerebral embolism of left middle cerebral artery. He was treated conservatively, and the right hemiplegia, aphasia, apraxia in a slight degree and right hemispatial neglect in a slight degree consequently existed. MRI showed a large cortical and subcortical infarct lesion including the left Broca's area, central region, perisylvian area with Wernicke's area and temporal lobe. In contrast, neuropsychological evaluation using the Western Aphasia Battery (WAB) demonstrated transcortical sensory aphasia, e.g., fluency 8, auditory comprehension 1. repetition 10 and object naming 2.4. In addition to preserved repetition, both linguistic prosody and affective prosody were well preserved. Most cases with transcortical sensory aphasia are known to occur with the lesion including temporo-parieto-occipital junction of dominant hemisphere. Our patient and a few other reported cases of transcortical sensory aphasia had a lesion in perisylvian area including Wernicke's area. Therefore, it is possible that their minor hemisphere worked selectively for repetition. Furthermore, we suggest that this patient presented dissociative aphasia that all the process of repetition and the function of linguistic and emotional prosody were represented in the right hemisphere and the other functions including comprehension of word meanings were existed in the left hemisphere. We believe that our case of transcortical sensory aphasia with dissociative aphasia gives a suggestion about the mechanism and localization of repetition and prosody in the whole system of language.Rinsho shinkeigaku = Clinical neurology 06/2006; 46(5):317-21. -
Article: Acute cervical motor radiculopathy induced by neck and limb immobilization in a patient with Parkinson disease.
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ABSTRACT: A 68-year-old woman with Parkinson disease (PD) presented with acute monoplegia of her left upper extremity after the neck and limb immobilization for several hours. Her sensory function was normal, and the chest X-ray showed left phrenic nerve palsy. Electrophysiological studies showed multi-segment muscle involvement (C3 to T1) including denervation potentials and reduced interference of motor units in needle electromyography. M wave amplitude in peripheral nerve stimulation was preserved except for the ulnar nerve, suggesting both axonal injury and conduction block at the anterior spinal roots. The patient showed fair recovery in several months, suggesting sufficient reinnervation and recovery of conduction block. Incomplete root avulsion was thought to be the pathomechanism of acute cervical motor radiculopathy.Internal Medicine 02/2006; 45(12):789-93. · 0.94 Impact Factor -
Article: A variant form of nasogastric tube syndrome.
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ABSTRACT: Nasogastric tube syndrome named by Sofferman et al in 1981 is a laryngeal complication presenting with life-threatening vocal cord abductor paralysis derived from peroforation of the NG tube-induced esophageal ulcer. As compared with the previously reported cases of this syndrome, our 4 autopsied patients were so peculiar in the following two points that vocal cord abductor paralysis developed repeatedly and no esophageal ulcer was present in spite of the presence of the laryngeal abductor muscle injury. We hypothesized that the etiology of such a variant form was circulatory injury of the laryngeal abductor muscle which was caused by the compression of the postcricoid blood vessels perfusing this muscle. Nasogastric tube syndrome, which is treatable by decannulation, cannot be ruled out even if no esophageal ulcer is detected by fiberoptic laryngoscopy.Internal Medicine 01/2006; 44(12):1286-90. · 0.94 Impact Factor -
Article: [An autopsy case of dementia with Lewy bodies which was clinically suspected to be Parkinson's disease complicated with Alzheimer-type dementia].
Nō to shinkei = Brain and nerve 01/2005; 56(12):1058-68. -
Article: [A case of sensory ataxic neuropathy and polymyositis of perivascular type associated with Sjögren's syndrome].
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ABSTRACT: A 53-year old woman was admitted with of sensory disturbance and weakness of lower limbs which had progressed slowly in the previous ten years. A diagnosis of sensory ataxic neuropathy associated with Sjögren's syndrome was made. A sural nerve biopsy showed marked loss of myelinated fibers. A muscle biopsy revealed atrophy of muscle fibers along with perivascular cellular infiltration. The dorsal root ganglia have been considered to be the main site affected in the ataxic neuropathy in Sjögren's syndrome. However, the evidence for that was meager. The perivascular inflammatory change observed in the muscle may also have existed in the peripheral nervous system including the dorsal root ganglia.Nō to shinkei = Brain and nerve 11/2004; 56(10):877-80.
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2007–2008
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Tokyo Metropolitan Komagome Hospital
Tokyo, Tokyo-to, Japan
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