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Masaki Ikeda,
Kimie Yonemura,
Satoko Kakuda,
Yuichi Tashiro,
Yukio Fujita,
Eriko Takai,
Yukiko Hashimoto,
Kouki Makioka,
Natsumi Furuta,
Koichi Ishiguro, [......],
Jun'ichi Yoshida,
Osamu Miyaguchi,
Tamao Tsukie,
Ryouzou Kuwano,
Tsuneo Yamazaki,
Haruyasu Yamaguchi,
Masakuni Amari,
Masamitsu Takatama,
Yasuo Harigaya, Koichi Okamoto
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ABSTRACT: Abstract We studied seven cases of Alzheimer's disease (AD). Six of the patients had presenilin 1 (PS1) mutations (PS1AD). Three novel PS1 mutations (T99A, H131R and L219R) and three other missense mutations (M233L, H163R and V272A) were found in the PS1AD group. We measured the levels of phosphorylated tau (ptau-181, ptau-199) and Aβ (Aβ1-42, Aβ1-40 and Aβ1-38) in the cerebrospinal fluid (CSF) of PS1AD patients, early-onset sporadic AD (EOSAD), late-onset sporadic AD (LOSAD) and non-demented subjects (ND). The CSF levels of Aβ1-42 in the three AD groups were significantly lower than those of the ND group (p < 0.0001). CSF levels of Aβ1-42 in the PS1AD group were significantly lower than those in the two sporadic AD groups. The Aβ1-40 and Aβ1-38 levels in the CSF of the PS1AD group were significantly lower than those of the three other groups (p < 0.0001, respectively). The levels of Aβ1-40, Aβ1-38 and Aβ1-42 in the CSF of the PS1AD group remained lower than those of the ND group for 4 years. Not only CSF Aβ1-42, but also Aβ1-40 and Aβ1-38 decreased in the advanced stages of PS1AD.
Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 05/2013; · 2.12 Impact Factor
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ABSTRACT: Here, we report a case of Cockayne syndrome (CS) in a Japanese man who displayed a unique pathology of phosphorylated trans-activation response (TAR) DNA-binding protein 43 (pTDP-43) with abundant Rosenthal fibers. Many round pTDP-43-positive structures were detected throughout the CNS; however, most of them were located in two regions that also exhibited neuronal depletion: the cerebellar cortex and the inferior olivary nucleus. To a lesser extent, these aggregates were also present in the cerebellar white matter, around the subependymal regions in the brain stem, and in the spinal cord. Intraneuronal pTDP-43 inclusions were only observed in a small number of neurons in the inferior olivary nucleus. Double-label immunofluorescence revealed that many of the aggregates were localized to astrocytes. The observed distribution and the morphology of the pTDP-43-positive structures were unique and have not yet been reported. Therefore, a pTDP-43-related pathology may be implicated in CS as well as in other neurodegenerative diseases such as frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Whether the pathology of these diseases reflects a primary neurodegenerative process or a secondary reaction is not known.
Neuropathology 04/2013; · 2.02 Impact Factor
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ABSTRACT: Overexpression of BTBD10 (BTB/POZ domain-containing protein 10) suppresses G93A-superoxide dismutase 1 (SOD1)-induced motor neuron death in a cell-based amyotrophic lateral sclerosis (ALS) model. In the present study, paraffin sections of spinal cords from 13 patients with sporadic ALS and 10 with non-ALS disorders were immunostained using a polyclonal anti-BTBD10 antibody. Reduced BTBD10 expression in the anterior horn cells was more frequent in spinal cords from ALS patients than in cords from patients with non-ALS disorders. We further investigated the relationship between the level of BTBD10 immunoreactivity and the morphology of the Golgi apparatus (GA) and the presence of phosphorylated TAR-DNA-binding protein 43 (pTDP-43). Mirror sections of spinal cords from five sporadic ALS cases were immunostained with antibodies against BTBD10 and trans-Golgi-network (TGN)-46 or pTDP-43. Whereas 89.7-96.5% of the neurons with normal BTBD10 immunoreactivity showed normal GA morphology and no pTDP-43 cytoplasmic aggregates, 86.2-94.3% of the neurons with reduced BTBD10 expression showed GA fragmentation and abnormal pTDP-43 aggregates. These findings suggest that reduced BTBD10 expression is closely linked to the pathogenesis of sporadic ALS.
Neuropathology 01/2013; · 2.02 Impact Factor
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ABSTRACT: Aim: To examine whether the Frontal Assessment Battery is associated with the immediate effects of physical therapy on gait disturbance in patients with Parkinson's disease. Methods: A total of 18 patients with idiopathic Parkinson's disease (Hoehn and Yahr stage range 3-4) who were able to ambulate independently and who were not demented were included. Patients were divided into two groups on the basis of Frontal Assessment Battery scores: the high score group (score ≥13, n = 11) and the low score group (score ≤12, n = 7). A 3-D motion analysis system was used to acquire gait parameter data before and after a 30-min physical therapy program. Stride length, step length, cadence, walking velocity, single support time and double support time were measured. The range of motion of the hip, knee and ankle joint, and maximal trunk displacement on the horizontal plane were measured. Results: In the high-score group, significant improvement was observed in walking velocity, stride length and step length, and in the range of motion of the hip and knee joint. Maximal trunk displacement decreased significantly. In contrast, no significant improvement was observed in the low-score group. Multivariate logistic regression analysis showed that Frontal Assessment Battery scores were a predictor of improvement in the range of motion of bilateral hip and knee joints, and maximal trunk displacement. Conclusions: We showed that the subtests of motor learning of the Frontal Assessment Battery might be associated with the immediate effects of physical therapy on gait disturbance in Parkinson's disease. Geriatr Gerontol Int 2012; ••: ••-••.
Geriatrics & Gerontology International 10/2012;
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Masaki Ikeda,
Kimitoshi Hirayanagi,
Motonobu Arai,
Satoko Kakuda,
Kouki Makioka,
Natsumi Furuta,
Eriko Takai,
Hiroo Kasahara,
Setsuki Tsukagoshi,
Yukio Fujita,
Masakuni Amari,
Masamitsu Takatama,
Yoichi Nakazato, Koichi Okamoto
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ABSTRACT: A middle-aged male suffering from encephalopathy with cerebral amyloid angiopathy (CAA) with amyloid beta (Aβ) presented with initial symptoms of transient consciousness disturbance and left visual field photophobia. Lesions with aberrantly high signal on T2-weighted magnetic resonance imaging (MRI) of the brain appeared in the right temporal lobe posterior to the occipital lobe and spread to other areas. Brain biopsy revealed Aβ deposits in vascular walls and numerous diffuse plaques in parenchymal areas. Based on MRI findings, Initial corticosteroid therapy with beta methasone effectively improved the neurological symptoms of consciousness disturbance and motor deficits. After corticosteroid therapy was stopped at 4 weeks, recurrence occurred. Additional corticosteroids did not improve clinical symptoms and the patient progressed to a bed-ridden state with a severe consciousness disturbance. Notably, CSF Aβ1-42 and CSF Aβ1-40 decreased while the recurrent encephalopathy worsened. After intense deterioration, the patient became stable. CSF Aβ1-42 increased but remained at a very low level. This case of CAA encephalopathy with apolipoprotein E ϵ4/ϵ4 homozygosity showed Aβ deposits in vascular walls and numerous diffuse plaques in parenchymal areas. The clinical course suggests that reduction of CSF Aβ1-42 and Aβ1-40 might be related to clinical deterioration in cases of encephalopathy.
Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 09/2012; · 2.12 Impact Factor
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ABSTRACT: To characterize the phenotype of spinocerebellar ataxia type 36 (SCA36), a novel dominant disorder (nicknamed "Asidan") caused by a hexanucleotide GGCCTG repeat expansion in intron 1 of the NOP56 gene.
We investigated the clinical, genetic, and neuropathologic characteristics of 18 patients with SCA36. We performed histologic evaluation of a muscle biopsy specimen from 1 patient with SCA36, and neuropathologic evaluation of an autopsied brain from another patient with SCA36.
The (GGCCTG)n expansion was found in 18 ataxic patients from 9 families. The age at onset of ataxia was 53.1 ± 3.4 years, with the most frequent symptoms being truncal ataxia (100% of patients), ataxic dysarthria (100%), limb ataxia (93%), and hyperreflexia (79%). Tongue fasciculation and subsequent atrophy were found in 71% of cases, particularly in those of long duration. Skeletal muscle fasciculation and atrophy of the limbs and trunk were found in 57% of cases. Lower motor involvement was confirmed by EMG and muscle biopsy. The neuropathologic study revealed significant cerebellar Purkinje cell degeneration with obvious loss of lower motor neurons. Immunohistochemical analysis showed that NOP56 was localized to the nuclei of various neurons. Cytoplasmic or intranuclear inclusion staining of NOP56, TDP-43, and ataxin-2 was not observed in the remaining neurons.
This is the first description of the unique clinical features of SCA36, a relatively pure cerebellar ataxia with progressive motor neuron involvement. Thus, SCA36 is a disease that stands at the crossroads of SCA and motor neuron disease.
Neurology 06/2012; 79(4):333-41. · 8.31 Impact Factor
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ABSTRACT: Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder. Its histopathological features include glial cytoplasmic inclusions that contain α-synuclein as the main component. Recently, multiple lines of evidence have suggested a role for lysosomes in the pathogenesis of many neurodegenerative diseases. To elucidate whether lysosomes are also implicated in the pathology of MSA, we carried out an immunohistochemical study using antibodies against lysosomal proteins in the brains of patients with MSA and in control brains. A robust increase in the expression and an alteration in the morphology and distribution of lysosomal-protein-positive structures were observed in MSA brains. Double immunohistochemistry demonstrated that lysosomal markers did not colocalize mainly with glial cytoplasmic inclusions, but colocalized with a microglial marker. These immunohistochemical signatures suggest that lysosomes are activated in microglia during the disease process, and play a pivotal role in the pathology of MSA.
Neuroreport 03/2012; 23(5):270-6. · 1.66 Impact Factor
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ABSTRACT: TDP-43 has been identified as a major component of the pathological inclusions in most forms of frontotemporal lobar degeneration with ubiquitin-positive inclusions and in amyotrophic lateral sclerosis (ALS). In the present study, paraffin sections of the midbrain in 112 patients with various non-ALS disorders and 27 patients with sporadic ALS were immunostained with antibody against phosphorylated TDP-43 (pTDP-43). pTDP-43-positive inclusions in oculomotor neurons were detected in 18 of 112 patients with non-ALS disorders (16.1%). The appearance of the inclusions showed fine filamentous structures rather than the skein-like inclusions seen in the anterior horn cells of ALS spinal cords. The incidence was increased in the age range of 80-89 years old (10/37 cases; 27.0%), in which 6 of 10 cases demonstrated AD pathology in the temporal lobes. Twenty-seven ALS patients were examined and the findings were compared with those of non-ALS patients. There were 13 cases demonstrating pTDP-43-positive inclusions (48.1%) which showed stronger immunoreactivities in ALS cases. This is the first report demonstrating fine filamentous pTDP-43-positive inclusions in oculomotor neurons in non-ALS disorders. Although the mechanisms underlying pTDP-43 in oculomotor neurons are currently unknown, its detection is of interest, and the expression may occur not only in ALS but also during the aging process.
Journal of the neurological sciences 01/2012; 315(1-2):20-5. · 2.32 Impact Factor
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ABSTRACT: Oculodentodigital dysplasia (ODDD) is a rare autosomal dominant inherited disorder mainly affecting the development of the face, eyes, dentition, limbs, hair and heart. GJA1 (the gap junction protein α-1) has been determined to be a causative gene of ODDD, mapped to chromosome 6q22-24 identified as the connexin 43 gene (Cx43). We found a novel GJA1 mutation (W25C) as the possible causative gene in this sporadic ODDD patient with neurological features of motor deficits by pyramidal tract signs, and sensory deficits due to peripheral nerve disturbance. It is also notable that the MRI of this patient demonstrated widespread aberrant signal lesions in the brain and brainstem.
Internal Medicine 01/2012; 51(1):93-8. · 0.94 Impact Factor
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ABSTRACT: This report deals with a 46-year-old male with Waldenström's macroglobulinemia (WM), who developed POEMS syndrome four years after diagnosis. The patient was diagnosed with WM, based on the presence of IgM-κ type monoclonal (M) protein and infiltration of lymphoplasmacytic cells identified in bone marrow aspirates. Four years later, the patient presented with progressive weakness and paresthesia of the limb extremities, and he was admitted to our hospital. Physical and neurological examination on admission revealed polyneuropathy, hepatosplenomegaly, hypothyroidism, IgM-κ M protein, leg edema, and cutaneous hyperpigmentation. He fulfilled the diagnostic criteria for POEMS syndrome. Laboratory tests showed normocytic normochromic anemia, elevated erythrocyte sedimentation rate, and increased levels of soluble IL-2 receptor, IL-6 and plasma vascular endothelial growth factor (VEGF). He was started on lenalidomide. After therapy, the leg edema and limb dysesthesia improved, and the VEGF level decreased from 608 pg/ml to 380 pg/ml. This is a very rare case of POEMS syndrome associated with WM, and is the first case treated with lenalidomide in Japan. VEGF presumably producted WM may be associated with development of POEMS syndrome.
Rinshō shinkeigaku = Clinical neurology. 01/2012; 52(3):186-9.
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Haruyasu Yamaguchi,
Satoshi Takahashi,
Kenji Kosaka, Koichi Okamoto,
Tsuneo Yamazaki,
Masaki Ikeda,
Makoto Osawa,
Masakuni Amari,
Yasuo Harigaya,
Shuichi Awata,
Yohko Maki
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ABSTRACT: In out-patient clinics, having simple procedures to check for signs of dementia is invaluable. In the present study, we evaluated the imitation of hand gestures to detect visuomotor deficits in dementia in clinical practice.
In all, 1219 subjects were enrolled in the present study, including 497 with Alzheimer's disease (AD), 98 with dementia with Lewy bodies (DLB), 71 with other types of dementia diseases, 175 with a Clinical Dementia Rating (CDR) of 0.5, and 378 normal controls. All subjects were aged 65 years or older. Subjects were recruited from 10 clinics and two communities. Visuomotor function was evaluated by the Yamaguchi fox-pigeon imitation test (YFPIT), which consists of a simple one-handed sign for 'fox' and a complex two-handed sign for 'pigeon', a rapid, game-like test with low psychological burden.
The success rate (successful/total) for imitating the 'pigeon' hand gesture was reduced as the severity of the dementia increased: 85.7% in normal controls, 60.6% in CDR 0.5 (mild cognitive impairment), 39.2% in CDR 1 (mild dementia), 21.2% in CDR 2 (moderate dementia), and 5.7% in CDR 3 (severe dementia). The success rate for imitating the 'pigeon' hand gesture was higher in patients with DLB than AD within the CDR 1 group (51.2% vs 35.4%, respectively), but lower for patients with DLB than AD within the CDR 2 group (12.5% vs 24.4%, respectively). The success of imitating the hand gesture for 'fox' was similar for patients with AD and DLB. Of those subjects who failed to imitate the hand gesture for 'pigeon', 49.5% of those with AD showed the palm-palm pattern (both palms facing outward), suggesting deficits of perspective conversion from the first-person to the third-person. Conversely, 52.8% of patients with DLB showed a dorsum-dorsum pattern (both dorsa facing outwards), suggesting deterioration of visual attention and recognition.
In conclusion, the YFPIT is a useful test to detect visuomotor deficits in dementia that can differentiate between AD and DLB.
Psychogeriatrics 12/2011; 11(4):221-6. · 1.21 Impact Factor
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ABSTRACT: A previously healthy 34-year-old man sustained multiple skull fractures in a traffic accident. Radiological findings and visual field examination did not detect any abnormality. Shortly after the accident, he noticed blurred vision in both eyes. Six months after the accident, he gradually developed disturbance of visual acuity in the right eye. His best corrected visual acuity (BCVA) was 0.8 OD and 1.2 OS and brain MRI did not show any abnormality, while Humphrey visual field analysis demonstrated right homonymous hemianopsia. Two months after the initial presentation, his BCVA showed 0.1 OD and 0.08 OS. Visual field examination suggested that both right homonymous hemianopsia and left blind spot had become enlarged. Mitochondrial DNA analysis demonstrated G11,778A mutation and a diagnosis of Leber's hereditary optic neuropathy (LHON) was made. A few reports have documented mild acute insult to the head or blunt optic trauma as triggers of optic neuropathy in subjects with LHON. Although, the precise mechanism of LHON following trauma remains unknown, it appears that an acute insult may be sufficient to precipitate neuropathy in the optic nerve already compromised by mitochondrial dysfunction. Asymptomatic carriers should be advised to avoid possible precipitating factors such as head trauma.
Rinsho shinkeigaku = Clinical neurology 10/2011; 51(10):781-3.
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Nippon rinsho. Japanese journal of clinical medicine 10/2011; 69 Suppl 8:88-92.
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ABSTRACT: We report an autopsy case of a 75-year-old Japanese woman with motor neuron disease (MND) showing numerous neuronal and glial inclusions immunostained with anti-fused in sarcoma (FUS) antibody. At 73 years, she received a diagnosis of MND and died of respiratory insufficiency 2 years later. No mutation was found in all exons of the FUS gene. Neuropathological examination revealed a reduced number of anterior horn cells and degeneration of the pyramidal tracts. Neither Bunina bodies nor inclusions positive for ubiquitin/phosphorylated TAR DNA binding protein of 43 kD (pTDP-43), such as skein-like or round inclusions, were observed. However, basophilic inclusions (BIs) were frequently observed in the remaining neurons of the anterior horns, facial nuclei, hypoglossal nuclei, vestibular nuclei, dentate nuclei and inferior olivary nuclei. In an immunohistochemical analysis, the BIs showed strong immunoreactivity with anti-FUS and anti-ubiquitin-binding protein p62 (p62) antibodies. The nuclear staining of FUS was preserved in some neurons with FUS-positive inclusions, and a few FUS-positive glial inclusions were found. FUS-positive inclusions were more common than p62-positive inclusions in some anatomical regions, and in some neurons, p62 immunoreactivity was observed in only parts of the BIs. These results suggest that BI formation and TDP-43 aggregation have different pathogenic mechanisms, and FUS may play an important role in the pathogenesis of MND with BIs. This patient has the oldest reported age of onset for MND with BIs, and clinical features observed in this patient were indistinguishable from those of classic sporadic MND. Therefore, we consider that the age of onset and clinical features of FUS-related disorders may be variable.
Neuropathology 04/2011; 31(2):170-6. · 2.02 Impact Factor
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ABSTRACT: Peripherin is a type III intermediate filament protein expressed with low levels in spinal motor neurons. Amyotrophic lateral sclerosis (ALS) is characterized by the presence of Bunina bodies, skein-like inclusions, and Lewy body-like inclusions (LBLIs) in the remaining anterior horn cells, where the first and third structures are detected by Hematoxylin-Eosin (H & E) staining. We examined paraffin sections of lumbar spinal cords from six ALS patients, using H & E staining and immunostaining for human peripherin. The results demonstrated that there were a total of 73 anterior horn cells containing one or more Bunina bodies, and that twelve of these cells (approximately 16.4%) demonstrated peripherin-positive Bunina bodies. In fact, some part of chain-like Bunina bodies showed peripherin-positive reaction, although there were a much higher number of non-immunoreacitive Bunina bodies in each neuron. LBLIs were clearly immunostained for peripherin corresponding to the core, while some of them showed different types of immunoreactivities due to oblique cutting of inclusions. Our findings suggest that although the mechanisms underlying peripherin co-localization in Bunina bodies are unknown, peripherin could be involved in forming these inclusions. Furthermore, following cystatin C and transferrin, peripherin is the third most prevalent protein that partially localizes in Bunina bodies.
Journal of the neurological sciences 03/2011; 302(1-2):14-8. · 2.32 Impact Factor
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ABSTRACT: Optineurin is a gene associated with normal tension glaucoma and primary open-angle glaucoma, one of the major causes of irreversible bilateral blindness. Recently, mutations in the gene encoding optineurin were found in patients with amyotrophic lateral sclerosis (ALS). Immunohistochemical analysis showed aggregation of optineurin in skein-like inclusions and round hyaline inclusions in the spinal cord, suggesting that optineurin appears to be a more general marker for ALS. However, our detailed examinations demonstrated that optineurin was found not only in ALS-associated pathological structures, but also in ubiquitin-positive intraneuronal inclusions in ALS with dementia, basophilic inclusions in the basophilic type of ALS, neurofibrillary tangles and dystrophic neurites in Alzheimer's disease, Lewy bodies and Lewy neurites in Parkinson's disease, ballooned neurons in Creutzfeldt-Jakob disease, glial cytoplasmic inclusions in multiple system atrophy, and Pick bodies in Pick disease. With respect to optineurin-positive basophilic inclusions, these structures showed variable immunoreactivities for ubiquitin; some structures were obviously ubiquitin-positive, while others were negative for the protein, suggesting that optineurin expression was not always associated with the expression of ubiquitin. This study indicates that optineurin is widely distributed in neurodegenerative conditions; however, its significance is obscure.
Neuropathology 02/2011; 31(6):569-74. · 2.02 Impact Factor
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ABSTRACT: Multiple system atrophy (MSA) and Parkinson's disease (PD) are classified as synucleinopathies that exhibit α-synuclein deposition in the central nervous system. Recently, activation of the unfolded protein response (UPR), which is a cellular stress response triggered by endoplasmic reticulum (ER) stress, was reported in PD and involvement of ER stress was indicated for this disease. To elucidate whether ER stress is also implicated in the pathology of MSA, we performed a series of immunohistochemical studies using MSA brain sections. Here, we showed the presence of an activated UPR response in oligodendroglia of postmortem MSA brains. The UPR protein-positive structures were observed in lesions where glial cytoplasmic inclusions (GCIs) appeared and colocalized highly in cells showing oligodendrocytic characteristics in the presence of α-synuclein inclusions. The UPR protein-positive structures appeared as granular shapes that are morphologically similar to granulovacuolar degeneration (GVD) and colocalized with GVD marker proteins. Double immunohistochemistry demonstrated that some of the activated UPR protein-positive structures were localized in oligodendrocytes that contained GCIs with faint α-synuclein labeling, without ubiquitination, and showing a strong correlation with the relocation of the tubulin polymerization-promoting protein (TPPP/p25α). These findings suggest that activation of the UPR may be induced at the early stage of the disease process, thus playing a pivotal role in the pathology of MSA.
Journal of the neurological sciences 10/2010; 297(1-2):60-5. · 2.32 Impact Factor
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Yukiko Yamamoto-Watanabe,
Mitsunori Watanabe, Koichi Okamoto,
Yukio Fujita,
Mandy Jackson,
Masaki Ikeda,
Yoichi Nakazato,
Yoshio Ikeda,
Etsuro Matsubara,
Takeshi Kawarabayashi,
Mikio Shoji
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ABSTRACT: Here we report a Japanese family with amyotrophic lateral sclerosis (ALS) characterized by very rapid progression, high penetrance and an autosomal dominant mode of inheritance. The phenotype includes atrophy of sternocleidomastoideus muscles, bulbar involvement, weakness of neck muscles and proximal muscle atrophy. These clinical symptoms are reminiscent of myopathy. All patients examined had similar clinical symptoms, age at onset and disease duration. The proband was found to have mutation R521C in the FUS/TLS gene, and was diagnosed as having ALS6. Autopsy material was available from the mother of the proband and FUS-immunoreactive neuronal and glial cytoplasmic inclusions were observed in the anterior horn of the spinal cord. While atrophy and weakness of the sternocleidomastoideus muscle is not emphasized in previous reports, this symptom may be a clinical hallmark of ALS6.
Journal of the neurological sciences 09/2010; 296(1-2):59-63. · 2.32 Impact Factor
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ABSTRACT: We report a 47-year-old woman with relapsed delayed radiation myelopathy (DRM), occurring 5 years and 10 years after radiation therapy for nasopharyngeal carcinoma at 37 years old. Sensations of pain and temperature had been disturbed in the right leg since 42 years old. MRI showed Gadolinium-enhanced lesion as a ring-like-enhancement of the spinal cord at C1-2 on T1-weighted image (T1WI), with high signal area and swelling of the spinal cord at the upper C1 to C6 areas on T2-weighted image. We diagnosed her as having DRM after considering the differential diagnosis, e.g., multiple sclerosis, spinal tumor and other neurological diseases. Her sensory symptoms quickly improved following therapy with prednisolone and warfarin. Although she remained healthy for a few years, dysesthesia of the neck on the right side appeared 5 years later after the first clinical occurrence. At this time, MRI demonstrated Gadolinium-enhanced lesion as a ring-like enhancement of the spinal cord at C2 on T1WI. but the area also differed from that of previous lesion; a high signal area and swelling of the spinal cord was also seen on FLAIR image of the medulla and upper C1 to C6. For recurrence of DRM, we administered prednisolone and warfarin. Thereafter, the patient recovered and the spinal cord lesion on MRI decreased markedly. The clinical course demonstrated that administration of prednisolone and warfarin might be effective for relapsed DRM.
Rinsho shinkeigaku = Clinical neurology 06/2010; 50(6):393-8.
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Koichi Okamoto
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ABSTRACT: We first reported ubiquitin-positive tau-negative intraneuronal inclusions in the hippocampal granular cell layer and entorhinal cortices in patients with amyotrophic lateral sclerosis (ALS). We then found that those inclusions occur frequently in patients with presenile dementia and motor neuron disease. The ultrastructure of the inclusions consists mainly of granules with a few filaments. In 2006, TDP-43 was identified as a major component of the inclusions specific for frontotemporal lobar degeneration and ALS. Here, we review the current knowledge regarding ubiquitin-positive tau-negative intraneuronal inclusions.
Neuropathology 05/2010; · 2.02 Impact Factor
