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S Ishiai,
Y Koyama,
K Seki,
S Orimo,
N Sodeyama,
E Ozawa,
E Y Lee,
M Takahashi, S Watabiki,
R Okiyama,
T Ohtake,
M Hiroki
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ABSTRACT: Unilateral spatial neglect has been rarely reported in patients with AD, although they often have right and left asymmetry of temporoparietal dysfunction.
To investigate if patients with AD would show unilateral spatial neglect in the line bisection test, and to reveal the relationship between their neglect and the area of cerebral dysfunction. Method: Thirty-two patients with mild to moderate AD and 32 age-matched healthy control subjects underwent an extensive line bisection test. SPECT was also obtained for the patients.
Rightward bisection errors exceeded the normal range in 25% of patients with AD. They exhibited greater rightward errors for the longer lines in the left hemispace than in the right hemispace, and with the right hand than with the left hand; this corresponds to the characteristics of neglect seen after right hemisphere lesions. All patients who bisected 200 mm lines with errors over 10 mm showed disproportionate lowering of performance IQ and asymmetric right hemisphere hypoperfusion, especially in the temporoparietal region. Seventy-five percent of the patients performed normally in the center presentation but erred slightly toward the body midline in the right and left hemispaces.
Left unilateral spatial neglect in mild to moderate AD may be rather common if tested with the line bisection test. Rightward errors over 10 mm suggest right temporoparietal dysfunction. In AD, three or more bisections of 200 mm lines in the center presentation are recommended for detection of neglect. Patients with AD but without neglect may have difficulty in shifting attention into the peripheral sector of the egocentric space.
Neurology 09/2000; 55(3):364-70. · 8.31 Impact Factor
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ABSTRACT: We investigated clinicopathologically the pyramidal signs, including spasticity, hyperreflexia, and Babinski's sign, and the involvement of the pyramidal tract and primary motor cortex, in seven Japanese autopsy cases of multiple system atrophy (MSA). Pyramidal signs were observed in six (86%) of the seven autopsy cases. Hyperreflexia and Babinski's sign were each evident in five patients, but spasticity was observed in only one patient. Loss of Betz cells and presence of glial cytoplasmic inclusions in the primary motor cortex were noticed in all seven cases. Astrocytosis in the fifth layer of the primary motor cortex was noticed in five cases, but its presence was not related to the duration of the disease. Involvement of the pyramidal tract in the spinal cord, particularly of the small myelinated fibers, was observed in all seven cases, but no involvement of the pyramidal tract in the midbrain was evident in any of the six cases in which this structure was examined. In MSA, pyramidal signs were shown to be present more frequently than believed before, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells in MSA has not been reported. Our clinicopathological findings may also make a contribution to the understanding of the clinicopathological hallmarks of MSA.
Acta Neuropathologica 07/2000; 99(6):628-36. · 9.32 Impact Factor
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K Tsuchiya,
K Ishikawa, S Watabiki,
O Tone,
K Taki,
C Haga,
M Takashima,
U Ito,
R Okeda,
H Mizusawa,
K Ikeda
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ABSTRACT: This report concerns a Japanese family with genetically confirmed SCA 6, including an autopsy case, and a review of Japanese autopsy cases of autosomal dominant cortical cerebellar atrophy (ADCCA). The proband (Case 1) was a Japanese woman. She developed gait disturbance at age 62. The father and younger sister (Case 2) had the same disorder. She died at age 67 due to subarachnoid hemorrhage. Neuropathological examination revealed severe loss of Purkinje cells in the cerebellum, prominently in the dorsal vermis, and absence of neuronal loss in the inferior olives. Molecular genetic study showed the CAG-repeat expansion of SCA 6 gene. The younger sister (Case 2) developed gait disturbance at age 62. Neurological examination at age 66 revealed cerebellar signs without sensory disturbance. Neuroimaging at this time showed cerebellar atrophy, prominently in the vermis. She died of multiple myeloma at age 66. A neuropathological review of Japanese autopsy cases of ADCCA showed that there are two patterns in the distribution of cerebellar cortical lesions of Japanese patients with ADCCA. The distribution of cerebellar cortical lesions in genetically confirmed Japanese patients with SCA 6 is more prominent in the vermis than in the hemisphere.
Journal of the Neurological Sciences 10/1998; 160(1):54-9. · 2.35 Impact Factor
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ABSTRACT: We investigated neuropathologically the distribution of the cerebellar cortical lesions in three Japanese autopsy cases of multiple system atrophy (MSA) using hemisphere specimens. The lesions were classified as mild, moderate or severe. The distribution of cerebellar cortical lesions in all three cases were uniform: the cerebellar cortical lesions were more conspicuous in the vermis than in the hemisphere. These neuropathological findings differ from the established theory that cerebellar lesions of MSA are more pronounced in the hemisphere than in the vermis. The degree of cerebellar cortical lesions in our cases increased in relation to the duration of the disease. Our pathological data may contribute to the morphological differential diagnosis in various neurodegenerative disorders including late cortical cerebellar atrophy. Our neuropathological findings may also make a contribution to the neuroradiological progress in the differential diagnosis of spinocerebellar disease.
Journal of the Neurological Sciences 03/1998; 155(1):80-5. · 2.35 Impact Factor
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ABSTRACT: This report concerns serial brain computed tomography (CT) images in two patients with corticobasal degeneration (CBD). The diagnosis of CBD was confirmed by neuropathological examination. In one case, we obtained serial brain CT images for one year and five months, and in the other, for three years and four months. The CT images showed that the anterior portions of the cerebrum atrophied progressively with the length of the disease. This was also seen in the CT images with respect to the caudate nucleus. Regarding radiological and pathological correlations, we consider that the progressive atrophy of the anterior portions of the cerebrum, seen in brain CT images, is ascribed to neuronal loss and gliosis in the anterior portions of the cerebrum. We also believe that the observed caudate nucleus atrophy is due to loss of neurons and gliosis in this structure, and also to the fibrillary gliosis of the cerebral white matter. The progressive atrophy of the caudate nucleus detected in brain CT is a valuable feature for the neuroradiological diagnosis of CBD.
Journal of the Neurological Sciences 12/1997; 152(1):23-9. · 2.35 Impact Factor
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ABSTRACT: We report an unusual autopsy case of corticobasal degeneration. The patient was male who was 67 years old at the time of his death. He developed clumsiness of his right hand at age 65. Neurological examination at age 66 revealed supranuclear ophthalmoplegia of upward gaze and parkinsonism. Progressive supranuclear palsy was suspected. The patient's clinical course was rapidly progressive. Dementia developed nine months after the onset of the disease, and the patient manifested apallic syndrome at 21 months and died of pneumonia at about 25 months. At autopsy the brain weighed 1370g. Macroscopic examination revealed prominent atrophy of the anterior and parietal lobes, particularly of the pars opercularis in the left inferior frontal gyrus, and there was marked depigmentation of the substantia nigra. Histological examination showed neuronal loss, glial proliferation, spongy state, and ballooned neurons in the frontal and parietal lobes. Neuronal loss with glial proliferation was conspicuous in the striatum, pallidum, thalamus, and substantia nigra. There was slight neuronal loss in the dentate nucleus and locus ceruleus. Staining by Gallyas-Braak method revealed massive appearance of argyrophilic threads. This case is important in terms of the clinical differentiation from progressive supranuclear palsy, and is also interesting in regard to the pathological correlation with slowly progressive aphasia.
Nō to shinkei = Brain and nerve 07/1996; 48(6):559-65.
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ABSTRACT: We report a pedigree of autosomal dominant spinocerebellar degeneration associated with pigmental retinopathy. The proband is a 75-year-old man. He noticed night blindness at the age of 10 years and a diagnosis of bilateral pigmentary retinopathy was made at age 63. At the age of 65 years, he developed dysarthria and difficulty in walking. At age 69, neurological examination revealed cerebellar signs, and brain CT scans showed mild atrophy of the brain stem and cerebellum. Repeated brain CT scans revealed slight progression of the brain stem and cerebellar atrophy. Molecular genetic studies showed the absence of any mitochondrial DNA mutation at 8993. The father of the proband exhibited cerebellar signs and pigmentary retinopathy. One older brother had cerebellar signs and another had pigmentary retinopathy. To our knowledge, hereditary spinocerebellar degeneration with retinal degeneration is rare in Japan. This study is the first full report on hereditary spinocerebellar degeneration with pigmentary retinopathy in Japan, although an abstract was published by Konishi et al. We also discuss the neuropathological discordance on hereditary olivoponto-cerebellar atrophy with retinal degeneration.
Nō to shinkei = Brain and nerve 12/1994; 46(11):1081-6.
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ABSTRACT: Three patients with left unilateral spatial neglect after predominantly frontal lobe lesions were asked to extend a horizontal line leftwards to double its original length. In this line extension task, they readily executed movements in or towards the contralesional left space. They performed the task in the left and right hemispaces as well as in the midline. The mean extension lengths did not differ significantly among these three spatial conditions. These results suggest that directional hypokinesia takes little part in left unilateral spatial neglect due to frontal lobe lesions. It is considered that the patients could execute leftward movements as the task oriented their attention sufficiently to the left. Two of the three patients, like reported cases with frontal neglect, showed a typical exploratory deficit for the left space in the line cancellation test. Such a deficit found in the traditional tasks, however, does not mean the presence of directional hypokinesia. All three patients showed visual extinction on double simultaneous stimulation. An attentional mechanism seems to play a predominant part in unilateral spatial neglect due to frontal lesions.
Journal of Neurology Neurosurgery & Psychiatry 10/1994; 57(9):1085-90. · 4.76 Impact Factor
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ABSTRACT: We report two patients with Pick's disease in senescence. Patient 1 is a 78-year-old woman. She developed abnormal behavior at the age of 76 years. Neurological examination at age 76 revealed poor rapport, easy angriness, "Denkfaulheit", oral tendency, and slight dementia [WAIS (Wechsler Adult intelligence Scale) total IQ 62]. Cranial CT scan and MRI showed bilateral atrophy of the frontal and temporal lobe, especially of the temporal lobe. Patient 2 is a 73-year-old man. He developped sexual abnormal behavior and "triebhafte Hemmungslossigkeit" at the age of 71 years. Neurological examination at age 72 revealed poor rapport, lack of spontaneity, easy angriness, "Denkfaulheit", and slight dementia [WAIS total IQ 91]. Transient "stehende Redensarten" was noticed. Cranial CT scan and MRI showed bilateral atrophy of the frontal and temporal lobe, especially of the frontal lobe. To our knowledge, Pick's disease with an onset in the senescence is very rare. Pick's disease should be included in the differential diagnosis of abnormal behavior in the senescence.
Nō to shinkei = Brain and nerve 09/1994; 46(8):787-92.
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ABSTRACT: Patients with left unilateral spatial neglect following lesions that mainly involved the right parietal lobe performed a line extension task, extending a horizontal line leftward to double its original length. We examined line extension performances in the left and right hemispaces, as well as in the midline, to assess whether spatial conditions affected these performances. Whatever the severity of neglect found in the line bisection test, the line extension performances of the patients were almost accurate and comparable with those of normal controls across the three spatial conditions. The neglect patients executed movements in or toward the contralesional space as the task oriented their attention sufficiently to the left. The results suggest that the motor component, ie, directional hypokinesia, has little part in left unilateral spatial neglect due to right parietal lesions.
Neurology 03/1994; 44(2):294-8. · 8.31 Impact Factor
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ABSTRACT: Two cases of presenile dementia with marked recent memory disturbance were reported. Patient 1 is a 54-year-old woman. She noticed forgetfulness at the age of 51 years. Neurological examination at aged 52 revealed marked recent memory disturbance, but examination by WAIS (Wechsler Adult Intelligence Scale) showed good results (verbal IQ 106, performance IQ 104, total IQ 106). There was neither disorientation in place nor character change. Cranial CT scan and MRI revealed the absence of brain atrophy. About 3 years after the onset of the disease, the degree of dementia is slight and disorientation in place does not appear. Patient 2 is a 67-year-old man. He noticed forgetfulness at the age of 63 years. Neurological examination at aged 66 revealed marked recent memory disturbance, but examination by WAIS-R showed moderate results (verbal IQ 89, performance IQ 87, total IQ 88). There was neither disorientation in place nor character change. Cranial CT scan and MRI revealed slight dilatation of the inferior horns of the lateral ventricle and slight cortical atrophy. About 4 years after the onset of the disease, the degree of dementia was slight and disorientation in place did not appear. We can not rule out the possibility that our cases belong to Alzheimer's disease, but the clinical course of our cases is peculiar. In the relation of responsible lesion in pure amnestic syndrome, hippocampal dementia, and simple senile dementia, our cases are interesting and important.
Nō to shinkei = Brain and nerve 02/1994; 46(1):77-83.
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ABSTRACT: Two cases of alcoholic cerebellar degeneration with pyramidal sign were reported. Patient 1 with alcohol dependence syndrome was a 46-year-old woman. After the alcohol abuse of about eight years, she complained of gait disturbance. The gait disturbance progressively worsened in about two months and she could not ambulate freely by herself. Neurological examination revealed nystagmus, ataxic and spastic gait, slight weakness and spasticity of the lower extremities, hyperreflexia of the extremities, bilateral Babinski's signs, and incoordination of the lower extremities. Examination of liver function and serum B12 was normal. Cranial CT scan and MRI revealed atrophy of the cerebellar vermis and dorsal part of the cerebellum. Though neurological signs slightly improved after the admission to our hospital and the abstinence from alcohol abuse, ataxic gait and hyperreflexia of the extremities have continued. Patient 2 was a 58-year-old man. He was a heavy drinker, but was not a patient with alcohol dependence syndrome. After the heavy drinking of about 40 years, he complained of gait disturbance. The gait disturbance had progressively worsened in about four months. Neurological examination revealed ataxic gait, hyperreflexia of the lower extremities, and bilateral Babinski's signs. Laboratory examination revealed slight liver dysfunction with minimal GPT and moderate gamma-GTP elevation. Examination of serum B12 was normal. Cranial CT scan and MRI revealed atrophy of the cerebellar vermis. Though bilateral Babinski's signs disappeared after the abstinence from heavy drinking, ataxic gait and hyperreflexia of the lower extremities have continued. Alcoholic myelopathy without hepatic cirrhosis was rarely reported. In the relation of alcoholic cerebellar degeneration to alcoholic myelopathy, our cases are interesting and important.
Nō to shinkei = Brain and nerve 03/1993; 45(2):169-75.
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ABSTRACT: We investigated the ability of 25 patients with left unilateral spatial neglect to make a clock face by putting numbers inside a printed circle. Impairment seen in this clock-drawing test did not parallel neglect severity as judged by results of the line-cancellation and line-bisection tests, as well as the copying of a daisy. The score for clock drawing correlated highly with the verbal WAIS score. Most neglect patients with a verbal IQ of 87 or more could draw a clock face fairly well and used planning in placing the numbers 12, 3, 6, and 9 before the others. In clock drawing, verbal intelligence may compensate for left unilateral spatial neglect. We therefore recommend use of the line-cancellation and line-bisection tests, as well as the copying test, but do not recommend use of the clock-drawing test in the diagnosis of left unilateral spatial neglect.
Neurology 02/1993; 43(1):106-10. · 8.31 Impact Factor
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ABSTRACT: Three cases (case 1, female, aged 30; case 2, male, aged 32; case 3, male, aged 34) of benign brainstem encephalopathy with truncal ataxia were reported. Two patients had prodromal symptoms Neurological examination revealed truncal ataxia in all cases. As additional neurological signs, anisocoria, mydriasis, nystagmus, ptosis, transient opsoclonus, and facial palsy were seen. There was neither drowsiness nor myoclonus in the three cases. On laboratory examinations, cold agglutination test revealed significant elevation in two cases. The examination of cerebrospinal fluid showed a moderate rise of proteins in one case, but did not revealed pleocytosis in any of the cases. Magnetic resonance imaging of one patient revealed an area of high intensity in the left pontine tegmentum by T2-weighed imaging. The prognosis for all these cases was good, and the reappearance of neurological signs was not present until now. Our cases were different from brainstem encephalitis (Bickerstaff's encephalitis) because of an absence of disturbed consciousness and no pleocytosis in the cerebrospinal fluid. Our cases were also different from "myoclonus-opsoclonus syndrome" because of an absence of myoclonus. We discussed a possibility of a new clinical syndrome which we call "benign brainstem encephalopathy with truncal ataxia".
Nō to shinkei = Brain and nerve 11/1992; 44(10):893-8.
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ABSTRACT: A case of multiple cranial nerve palsy by herpes zoster was reported. A 79-year-old man showed fever, sore throat, and dysphagia. No vesicle was noted at ear and pharynx. The patient developed, later, left peripheral facial nerve palsy. The cerebrospinal fluid revealed pleocytosis with increased protein. The viral antibody titer of herpes zoster was significantly elevated both in cerebrospinal fluid and in serum. The left facial palsy was slightly improved. But his dysphagia didn't improve during at least 10 months after the onset. Among the cranial nerves, trigeminal and facial nerves are the most commonly affected by herpes zoster. But there are a few cases of the 9th and 10th cranial nerve involvement in the literature. However, dysphagia has rarely been reported in these previous cases, only four cases developed severe dysphagia like the present patient. All of these cases including our case were over sixty years old, while cases with slight dysphagia were under sixty years old. No other differentiating factor is noted between these two groups with regard to sites of vesicles, findings of cerebrospinal fluid and mode of therapy.
Rinsho shinkeigaku = Clinical neurology 06/1992; 32(5):524-6.
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The American Journal of Medicine 12/1988; 85(5):738-9. · 5.43 Impact Factor
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ABSTRACT: A 62-year-old man and his maternal uncle had a selective vitamin E deficiency without generalized fat malabsorption. A progressive neurological disorder comprising ataxia, areflexia, and loss of proprioception developed in their sixth and seventh decades. The vitamin E deficiency is thought to be due to abnormally accelerated utilization, excretion, or degradation of the vitamin. This adult-onset spinocerebellar syndrome is due to vitamin E deficiency not caused by malabsorption.
Annals of Neurology 08/1987; 22(1):84-7. · 11.09 Impact Factor
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Rinsho shinkeigaku = Clinical neurology 04/1986; 26(3):237-40.
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Rinsho shinkeigaku = Clinical neurology 08/1985; 25(7):760-6.
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ABSTRACT: This article argues against the general idea that the patient described by Gowers as distal myopathy in 1902 may have suffered from myotonic dystrophy. A case of distal myopathy with markedly atrophic sternocleidomastoids is presented. Patients of distal myopathy with absence of sternocleidomastoids, sometimes accompanied by facial weakness, are recorded in the literature. All these cases are sporadic and may be called distal myopathy of Gowers, which is different from Welander's cases.
European Neurology 02/1984; 23(3):144-7. · 1.81 Impact Factor
