An autopsy case of dementia with motor neuron disease accompanying Alzheimer's disease lesion]

We report the case of a 60-year-old man with autopsy-proven dementia with motor neuron disease (D-MND) and Alzheimer's disease lesion. The patient presented with clumsiness of his right hand at the age of 55 years old and subsequently developed dysarthria, weakness and atrophy of his upper limbs. He was unaffectionate towards his family, repeated the same phrase, and showed severe disorientation of time and place. Neurological examination on admission showed not only diffuse lower motor neuron signs, such as weakness, atrophy, fasciculation and areflexia in both upper limbs, but also dementia (HDS-R 9/30). He died of respiratory insufficiency. Neuropathological examination showed mild atrophy of the frontal and temporal lobes and anterior spinal roots. Microscopic examination of cortical sections revealed degenerative changes with simple atrophy and gliosis, and these changes were predominant in layers 1 and 2 of the frontal and temporal cortices. Using immunohistochemical staining, ubiquitin-positive but tau-negative inclusions were frequently found in neurons of the hippocampal granular cell layers and temporal lobes. Many senile plaques and neurofibrillary tangles were present in all sections of the brain. Our final diagnosis was dementia with motor neuron disease accompanying Alzheimer's disease lesion, because of hypoperfusion in the parietal lobe as well as the frontal lobe demonstrated by SPECT, and the presence of many senile plaques and neurofibrillary tangles in the cerebral cortex. Overlapping of pathologically-proven D-MND and Alzheimer's disease lesion is extremely rare, and this case may improve our understanding of the process of neurodegeneration.     

An autopsied case of subacute progressive dementia with flactuated disturbance of consciousness, myoclonus and periodic synchronous discharges on EEGs]

We present an autopsied case which developed progressive dementia at the age of 45. EEGs showed periodic synchronous discharges (PSD) when he showed fluctuated cloudy consciousness with myoclonus. Cerebrospinal fluid examination showed mild to moderate increase in protein and monocytes for duration of illness, but antivirotics and antibiotics were not effective. He was not apallic even at the terminal stage when computed tomography (CT) revealed marked cerebral atrophy, and died of pneumonia 27 months after onset. He was clinically diagnosed as having Creutzfeldt-Jakob disease (CJD) because of subacute progressive dementia, myoclonus and PSD. Neuropathological examination revealed marked brain atrophy with severe neuronal loss and gliosis in the cerebral cortex and subcortical nuclei, although no spongiform degeneration was found. Senile plaques, neurofibrillaly tangles, argyrophilic glial inclusions and glial nodules were not detected. Prion protein immunoreactivity was negative. Therefore, this case can not be neuropathologically diagnosed as having CJD, Alzheimer's disease, non-Alzheimer-type degenerative dementias or any encephalitis. This case might have suffered from an unknown new disease.     

An autopsy case of dementia lacking distinctive histology showing semantic dementia]

We report an autopsy case of dementia lacking distinctive histology (DLDH) showing semantic dementia. At age 47, a Japanese man developed aspontaneity, followed by semantic dementia a few months after the onset. Thereafter he developed disinhibition and the language disturbance, which progressed transcortical sensory aphasia and terminally mixed transcortical aphasia. At age 48, about 10 months after the disease onset, neurological examination revealed frontal signs and hyperreflexia in the four extremities and 4 months later, the patient presented with mild rigidity in the right upper and lower extremities. At age 49, 1 year and 8 months after the onset of the disease, he could not walk by himself. At age 50, 2 years and 8 months after the onset, he died of pneumonia. The brain weighed 1350 g. Macroscopically, atrophy of the frontal lobes and temporal lobes, predominant in the left, was evident. The caudate nucleus was severely atrophic, in addition to the depigmentation of the substantia nigra. Neuronal loss and astrocytosis was obvious in the cerebral cortex, prominently in the frontotemporal lobes, amygdala, caudate nucleus, putamen, pallidum, thalamus, and substantia nigra. In the caudate nucleus, prominent neuronal loss with fibrillary gliosis was obvious. Senile plaques, neurofibrillary tangles, Pick bodies, astrocytic plaques, and tufted astrocytes were not found by Gallyas and tau staining. Ubiquitin-immunoreactive intracytoplasmic inclusions were not encountered in the hippocampal dentate gyrus and superficial layers in the frontotemporal cortex. On the basis of meticulous perusal of the literature, we believe that our case is the first autopsy case of DLDH reported in Japan.     

An 81-year-old man with personality change, dementia, and gait disturbance with diffuse leukoaraiosis of the cerebral white matter]

We report an 81-year-old patient with progressive dementia, disinhibition, and gait disturbance. He showed visuospacial disorientation, apathy, and gait disturbance at 76 years of age. When he was 77 years old, he was diagnosed Parkinson's disease and treated with the 1-dopa, the dopamine agonist, the amantadin, and the anti-cholinergic drug. These treatments didn't improve his motor disturbances. His motor disturbances, apathy, and abnormal behavior progressed gradually. He was admitted to the hospital at the age of 77. He was severely demented and akinetic. Sometime, violent behavior and hallucination were seen. The brain MRI showed frontotemporal lobe atrophy and severe leukoaraiosis of the frontal white matter. At 79 years of age, he became mute and bedridden. When he was 80 years old, large infarction occurred in his occipital lobe. He died due to renal failure and respiratory suppression at 81 years of age. His brain was examined pathologically. At the neurological CPC, the chief discussant arrived at the conclusion that his diagnosis was Binswanger's disease. Other possibilities discussed were FTD, CBD, and progressive subcortical gliosis. The post-mortem examination revealed diffuse white matter degeneration due to atherosclerotic change of the small artery, many lacunar infarctions, and severe infarction of the occipital lobe. These findings led the diagnosis of Binswanger's disease and cerebral infarction.     

A clinicopathological study of young-onset dementia: report of 2 autopsied cases

We retrospectively examined the clinical features and the neuroradiological findings on autopsy of 2 cases of young-onset dementia. The patient in case 1 was a 43-year-old woman who was unable to determine the time on the clock and who made frivolous remarks. Neuropsychological test batteries demonstrated memory impairment and frontal lobe dysfunction. T2-weighted magnetic resonance imaging (MRI) of the head revealed abnormal high-intensity signals around the lateral ventricles and thinning of the corpus callosum. Single photon emission computed tomography (SPECT) revealed patchy reduction in the accumulation of tracers in both the frontal lobes. Her neurological condition gradually deteriorated, and she died 13 years after the onset of the disease. She was clinically diagnosed with atypical Alzheimer's disease on the basis of visual cognitive impairment and memory impairment observed in the initial phase. However, the neuropathological diagnosis was adult-onset leukodystrophy with axonal spheroids. The patient in case 2 was a 43-year-old man who had gradually started behaving selfishly and had become ill-tempered and apathetic. He was admitted to a hospital. He was anosognosic and showed frontal lobe dysfunction. T2-weighted MRI scan of the brain showed abnormal high-intensity signals around the lateral ventricles; atrophy of the frontal and temporal lobes, hippocampus, and brainstem; and thinning of the corpus callosum. SPECT revealed patchy reduction in the accumulation of tracers in both the frontal lobes and the cerebellum. His neurological condition gradually deteriorated, and he died after being clinically ill for 7 years. The patient was clinically diagnosed with frontotemporal dementia on the basis of the clinical features and MRI findings. However, the neuropathological diagnosis was chronic meningoencephalitis. The frequency of neurological metabolic and inflammatory diseases is significantly high although it is not as high as that of degenerative diseases in young-onset dementia. Since such diseases may respond to therapy, they should be considered in the differential diagnosis of young-onset dementia, especially in patients presenting with atypical clinical features. Neuroradiological examination may contribute to the differential diagnosis of atypical dementia at young age.     

Frontotemporal dementia with ubiquitinated neuronal inclusions presenting with primary lateral sclerosis and parkinsonism: clinicopathological report of an autopsy case

We report a case displaying upper motor sign, parkinsonism, and behavioral abnormality, with marked degeneration of the precentral cortex, neostriatum and frontotemporal lobes, as well as ubiquitinated neuronal inclusions. The patient was a 66-year-old male at the time of death. At age 57, he noticed progressive difficulties in speaking and swallowing. At age 60, he was severely anarthric and displayed emotional lability and incontinence. Neurologically, very poor movement of tongue was observed, but without atrophy or fasciculation. Deep tendon reflexes were hyperactive. Grasp reflex and snout reflex were also positive. Needle electromyography revealed no abnormalities. A diagnosis of primary lateral sclerosis and character change was made. At age 62, he developed bradykinesia and rigidity of the neck and all extremities. Treatment with carbidopa-levodopa was initiated, but resulted in minimal improvement. At age 65, he was bed-ridden, and had repeated occurrences of aspiration pneumonia; he died of pneumonia. Neuropathological examination revealed marked atrophy of the frontal and temporal lobes with Betz cells completely absent and moderate atrophy of the neostriatum. The spinal cord and nerve roots appeared normal. Immunohistochemically, ubiquitin-positive but tau-negative intraneuronal inclusions were found in the frontal and temporal cortices, including the precentral cortex and the hippocampal dentate gyrus, and the neostriatum. This case could be included with inclusion-associated disorders such as frontotemporal dementia or amyotrophic lateral sclerosis with dementia, and furthermore, predominant upper motor sign and parkinsonism could represent phenotypes of clinical manifestations with such inclusions.     

An autopsied case of corticobasal degeneration with onset of nonfluent aphasia revealing symmetrical cerebral involvement

Herein we describe a patient with established corticobasal degeneration with onset of nonfluent aphasia and showing symmetrical cerebral involvement. A 64-year-old man with a speech disorder for 2 years visited our hospital. He had nonfluent aphasia (reduced spontaneous speech, loss of intonation, anomia, repetition disorder, and difficulty in speaking short sentences). He also showed right-sided motor neglect, hypertonus of the left lower limb, a mask-like facial expression, and difficulty in closing his eyes. He was restless and walked around even during examination, suggesting frontotemporal dementia (FTD). Single-photon emission computed tomography (SPECT) revealed symmetrical reduction of cerebral blood flow in the bilateral fronto-temporo-parietal lobes. His neurological condition deteriorated gradually and a year later he could not speak comprehensive sentences. Magnetic resonance imaging (MRI) of the head at age 70 showed symmetrical atrophy of the bilateral fronto-temporal lobes. He died of respiratory failure after clinical problems lasting ten years. On pathological examination, the fixed brain weighed 1,010 g and showed bilateral symmetrical atrophy of the frontal lobes. Histopathological examination revealed neuronal loss and gliosis in the frontal lobes, especially in the frontal convexity, superior frontal gyrus and precentral gyrus. Gallyas-Braak silver staining showed astrocytic plaques, argyrophilic threads and coiled bodies mainly in the frontal lobes. The substantia nigra showed severe neuronal loss on both sides and presence of free melanin. Pathological diagnosis was corticobasal degeneration (CBD). We believe that the patient had nonfluent aphasia and FTD reflected in bilateral degeneration of the frontal lobes. Some cases of CBD may present with symmetrical degeneration of the brain, even though left-hemisphere symptoms such as aphasia reveal themselves at an early stage.     

A progressive neurodegenerative disorder with cortical spongiform change and white matter damage presenting with pseudobulbar palsy

An autopsy case of spongiform encephalopathy with severe white matter degeneration, beginning with slowly progressive pseudobulbar palsy was reported. A 58-year-old man with no family history of neurologic disease noticed speech disturbance at age 38, which gradually deteriorated. By the age of 41, he became totally aphonic, but he could write and understand the meaning of writing. At age 42, he showed pseudobulbar palsy. At age 43, he gradually developed parkinsonism and generalized hyper-reflexia. At age 46, he showed dementia, aphonia, rigidity, generalized hyper-reflexia, left grasping, and quadriplegia in flexion. He died of respiratory failure at age 58, in the state of akinetic mutism, 20 years after the onset of the symptoms. Neuropathologically, the brain showed marked diffuse atrophy. Microscopic examination revealed spongiform encephalopathy with severe neuronal loss and profound white matter degeneration throughout the cerebrum. This case was difficult to categorize in any known neurodegenerative disease, both clinically and neuropathologically, suggesting a new entity of neurodegenerative disease.     

Post–traumatic Alzheimer''s disease: preponderance of a single plaque type

The cause of Alzheimer's disease is unknown. Several factors have been proposed including head trauma. At present, the link between head injury and a subsequent neurodegenerative process is largely circumstantial, except in the case of dementia pugilistica (punch drunk syndrome) found in boxers. Recent studies have shown that the brains of boxers with this syndrome contain large numbers of 'diffuse' beta-protein immunoreactive plaques. We supposed that this plaque type might be associated with trauma induced Alzheimer-like degeneration. In order to test this hypothesis we have re-investigated a previously reported case of post-traumatic premature Alzheimer's disease. Immunocytochemistry using antibodies to amyloid beta-protein revealed large numbers of 'diffuse' non-Congophilic plaques with little or no neuritic component. A similar preponderance of this plaque type is present in the brains of boxers with dementia pugilistica. Our observations support the idea of a trauma induced Alzheimer-like degenerative process and indicate that such a condition is associated with a marked preponderance of 'diffuse' plaques.     

Diffuse cortical lesions with hemorrhage in cerebral Whipple's disease

A 30-year-old Chinese male with a history of diarrhea and arthralgia presented for evaluation of progressive dementia, epilepsy, and increased intracranial pressure. Imaging of the brain showed progressive cortical and subcortical lesions with hemorrhage involving the bilateral temporal and occipital lobes, the posterior parietal lobes, and the left frontal lobe. "Foamy" periodic acid-Schiff (PAS)-positive macrophages were demonstrated on brain biopsy. The patient showed clinical improvement following treatment with chloromycetin and sulfadiazine for 2 months. This constitutes the first reported case of cerebral Whipple's disease with diffuse cortical lesions with hemorrhage reported in a Chinese individual. Further, this case points out the significance of early recognition and treatment of cerebral Whipple's disease, especially in those cases with unusual manifestations.     

额叶型痴呆的临床和病理

目的　证实一种少见的伴有痴呆的神经系统变性疾病———额叶型痴呆。方法　对 1例因并发肺炎而死亡的 46岁进行性痴呆患者 ,进行脑部剖检 ,经系列的组织染色及PrP ,tau蛋白等免疫组织化学染色。结果　该例患者有 :(1)进行性神经、精神症状 ,病程为 3年 ;(2 )头部CT示双侧额叶灰质萎缩 ,脑电图呈阵发性全导联 ,长间歇期 (>2s)的高波幅慢波 ;(3)脑重 10 5 0g ,脑萎缩仅限于额叶 ,未累及颞叶 ;(4)额叶灰质从第二层开始神经细胞大量脱失伴明显胶质增生 ,而锥体细胞相对完好。Beilschowsky及Gallyas染色无异常发现 ;(5 )神经细胞及胶质细胞内未发现任何包涵体 ;(6 )PrP、tau蛋白免疫组织化学染色呈阴性反应。结论　该病例为典型额叶型痴呆 ,今后在分析伴有痴呆的神经系统变性疾病时 ,应想到此类型痴呆。     

Case with probable dementia with Lewy bodies, who shows reduplicative paramnesia and Capgras syndrome]

We report a case of probable dementia with Lewy bodies (DLB), showing reduplicative paramnesia (RP) and Capgras syndrome (CS). The patient, a right-handed 60 year-old male, began to show progressive dementia. At the age of 65, he showed fluctuating cognitive impairment and recurrent visual hallucinations. His SPECT demonstrated hypoperfusion not in the medial temporal cortices, but in the parieto-occipital lobes, where the right hemisphere was dominantly hypoperfused. He was diagnosed with probable DLB. In addition to recurrent visual hallucinations, he showed a sense of self- (or others) transfiguration, consciousness of something non-existent (Leibhaftige Bewusstheit; Jaspers, K.), and fluctuating visuo-spacial impairment. At the age of 67, he gradually complained of his duplicative wives "sosie". Finally he went so far as to talk about a nameless phantom boarder. We considered that RP and CS of this case comprised a sense of self-(or others) transfiguration, misidentification of important persons and places, and productive symptoms such as consciousness of something non-existent (Leibhaftige Bewusstheit) and visual hallucinations. The above mentioned symptoms might be originated not only from the disturbance of visuospacial recognition, which involves the limbic system (especially amygdala), medial frontal cortex, and right hemisphere of the brain, but also from the disturbance of recursive consciousness, due to diffusely damaged brain regions with Lewy body pathology. (Authors' abstract)     

Severe anterograde amnesia with extensive hippocampal degeneration in a case of rapidly progressive frontotemporal dementia

Frontotemporal dementia (FTD) is usually characterized as a spectrum of relatively slowly progressive disorders with largely focal frontal or temporal presentations. The development of clinical and research criteria for discriminating FTD from Alzheimer's disease has relied, in part, on the relative preservation of episodic memory in FTD. We present a patient with FTD who, in addition to the more typical behavioural and language deficits, had a profound anterograde amnesia at the time of diagnosis. Neuroimaging confirmed atrophy of frontal and temporal lobes bilaterally, most marked in the anterior left temporal region. At post-mortem, non-Alzheimer pathology resulting in devastating cell loss was revealed in the hippocampi, as well as in the frontal and temporal cortex, thus providing neuroanatomical corroboration of the episodic memory deficit. Progression of the disease was extraordinarily rapid, with just 2 years between reported onset and time of death. This case demonstrates that the pattern of FTD may include severe anterograde amnesia as a prominent and early consequence of the disease.     

Specificity of changes in cerebral blood flow in patients with frontal lobe dementia.

Eight patients with a clinical diagnosis of probable Alzheimer's disease, eight patients with the clinical diagnosis of frontal lobe dementia, and eight controls were examined with single photon emission tomography (SPECT) using 99Tc-HMPAO. Patients with Alzheimer's disease and those with frontal lobe dementia met DSM-III-R criteria for mild dementia and were in the early stages of the illness. Compared with patients with Alzheimer's disease, the group with frontal lobe dementia had significantly lower blood flow in the frontal lobes (dorsolateral and orbital), the anterior temporal cortex, and the basal ganglia. Within the frontal lobe dementia group, blood flow was significantly lower in the orbital than in the dorsal frontal cortex, and in the anterior temporal than in the dorsal temporal cortex. The present study shows the specificity of changes in regional cerebral blood flow in the diagnosis of different types of dementia, and supports the importance of orbitofrontal, anterior temporal, and basal ganglia dysfunction in the production of the psychiatric syndrome of frontal lobe dementia.     

Progressive supranuclear palsy accompanied with progressive subcortical vascular encephalopathy of Binswanger type--a case report

We report the clinical and neuropathological findings in an autopsy case of progressive dementia, Parkinsonism, pseudobulbar palsy and supranuclear ophthalmoplegia. Since 70 years old, this hypertensive patient developed forgetfulness, unsteady gait and festination. These symptoms rapidly worsened and he was admitted in October 1983, at age 71. He had severe dementia and showed stiff face. Voluntary vertical movement of the eyes was severely disturbed, but reflex vertical movement by the doll's head eye maneuver was not affected. Muscle tone in the limbs increased slightly, and deep tendon reflexes were hyperactive in jaw and the upper extremities. Babinski sign was negative bilaterally. Sensation and coordination remained normal. Although he was not paretic or ataxic, his movements were very slow. He could not stand by himself and easily fell backward. These clinical features resembled those of progressive supranuclear palsy except for severe dementia and rapid progression. Brain CT scan revealed marked dilatation of the lateral ventricles, prominent periventicular lucency and atrophy of brainstem and cerebral cortex. Treatment with levodopa slightly improved his movement, but difficulty in swallowing worsened increasingly. He died of hypoglycemic coma and aspiration pneumonia in September 1984, about two years after the initial symptoms. General autopsy showed severe pneumonia and atrophy of the liver. The brain weighed 1,210g. Atherosclerotic change in the cerebral arteries were mild. Coronal sections of the cerebral hemispheres revealed diffuse ischemic change and multiple small infarctions in the bilateral cerebral white matter. Cortical atrophy was observed in the cerebral hemispheres. The basal ganglia, thalamus, and pons showed status lacunaris. Atrophy of midbrain and depigmentation of the substantia nigra were observed macroscopically.     

New V272A presenilin 1 mutation with very early onset subcortical dementia and parkinsonism

In this report, we present the clinical and pathological details of a kindred of four individuals with a novel missense mutation (V272A) of the presenilin 1 gene (PSEN1) that experienced a subcortical dementia. The age of onset of symptoms ranged 26-36-year old, with an age at death of 36-46 years. Initial symptom was a marked mood disorder, with prominent parkinsonism in one case. The neuropsychological study, as well as the neuroimaging and PET in the proband were concordant with a subcortical dementia. The cerebral pathology showed in this patient, aside from the classical lesions of Alzheimer disease, Lewy bodies in cortex and substantia nigra, and widespread subcortical neuritic lesions. This clinical pattern and pathology expands the clinical spectrum of familial Alzheimer's disease and compel to include mutations of PSEN1 gene in the genetic study of subcortical dementia.     

Is the immobility of advanced dementia a form of lorazepam-responsive catatonia?

Patients with end-stage dementia typically are very immobilized. Could this state actually be a form of lorazepam-responsive catatonia? Catatonia has been documented following cerebrovascular accidents, head injury, HIV encephalitis, brain tumors, and multiple sclerosis. Identified anatomical substrates include frontal lobes, parietal lobes, limbic system, diencephalon, and basal ganglia. Given that Alzheimer's disease, vascular dementia, Lewy body dementia, corticobasal degeneration, frontotemporal dementia, and Parkinsonian dementia often have degeneration in some of the same areas, dramatic awakenings might be possible by giving lorazepam challenges to locked-in dementia patients. If even a small percentage were lorazepam responders, the impact worldwide would be tremendous. Serious consideration should be given to undertaking large-scale clinical trials.     

CNS Changes in DRPLA with Dementia and Personality Changes: CT, MR and SPECT Findings

Abstract: CNS changes in a case of DRPLA associated with dementia and personality changes were observed by CT, MR and SPECT. Brain CT and MR of the patient revealed progressive cortical atrophy which was recognized in parallel with the clinical course of the progression of dementia and personality changes. SPECT using ¹²³I-iodoamphetamine (IMP) disclosed a diffuse low perfusion of the cerebral cortex, especially in the frontal and temporal lobes. These findings suggest that the dementia and personality changes in this case might be concerned with a dysfunction of the cerebral cortex.     

A Case of Traumatic Brain Injury Developing Frontal Lobe Syndrome after a Long Incubation Period

Abstract : A 55‐year‐old man, who was a heavy drinker for about 30 years, had experienced a heavy blow on the right posterior temporal region on account of a traffic accident at the age of 16. He recovered without any sequelae. However, he began to make mistakes and had trouble with his job about 40 years after the accident. His symptoms and neuropsychological examination suggested frontal lobe syndrome. Cranial magnetic resonance imaging (MRI) showed that the frontal and temporal lobes seemed to be predominantly atrophic and that there was great enlargement of the lateral and third ventricles, and a post‐contusion lesion in the left frontal lobe. Brain single photon emission computer tomography (SPECT) demonstrated diffuse cerebral hypoperfusion. Cranial MRI suggested that the contusion was proportionate to contre coup injury resulting from the accident. We supposed that the extensive brain damage induced edema and ischemia soon after the accident. Consequently, the circulatory disturbance might have caused cerebral atrophy and enlargement of the ventricles. Since he was relatively young at the time of the accident, the plasticity of his brain compensated for the injury intensively, and obvious symptoms suggesting frontal lobe syndrome were latent for about 40 years after the accident. Continuous drinking may have induced recent dysfunction of the compensated brain. However, such severe atrophy and focal damaged lesion could not be solely accounted for by heavy alcohol consumption. On the other hand, organic changes due to aging and brain circulatory insufficiency caused by hypertension or hyperlipidemia may also disclose the cerebral dysfunction. His cranial MRI and SPECT seemed not to be consistent with frontotemporal dementia. Furthermore, we considered that his case was also different from Alzheimer's disease and normal pressure hydrocephalus. However, it is necessary to follow up neuro‐imaging. Conclusively, he developed frontal lobe syndrome based on traumatic brain injury and induced by continuous drinking, aging, and brain circulatory insufficiency.     

Multi-infarct dementia clinically simulating dementia of Alzheimer type. A comparison with angular gyrus syndrome

A 74-year-old right-handed man with multiple cerebral infarction who presented with dementia simulating dementia of Alzheimer type (DAT) is reported. He had been well until April 20, 1987 when he developed transient right hand palsy lasting overnight. Eleven days later, he became confused, disorientated, and amnestic. He was admitted to this hospital on June 8. Physical examination revealed hypertension (170/90mmHg). On neurological examination, his consciousness was clear but he was demented. He showed disorientation, amnesia, and urinary incontinence. His most prominent symptom was disturbance of speech, including fluent aphasia and alexia with agraphia. Additionally, he showed ideomotor apraxia, construction apraxia, right-left agnosia, finger agnosia, and acalculia. On July 9, he had a transient attack of right hemiplegia with confusion. The brain CT scan performed on admission was unremarkable except for cavum septi pellucidum and a small low density area in the right basal ganglia. However, single photon emission computed tomography (SPECT) by 123I-labeled N-isopropyl-p-iodoamphetamine disclosed hypoperfusion of the cerebral blood flow in the border zones of the temporoparietal and frontal lobes on the left. A follow-up brain CT scan taken one month later demonstrated low density in the new areas corresponding to hypoperfusion shown by SPECT. Although the clinical features of the present case resembled those of DAT, dementia in this case was regarded as the result of multiple cerebral infarction since it occurred acutely with mild motor deficits, and brain CT scans and SPECT showed lesions indicating focal cerebral ischemia.(ABSTRACT TRUNCATED AT 250 WORDS)