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Of approximately 2,000 pathologically confirmed symptomatic CVD patients, atherothrombotic infarctions were found in 23%, lacunar infarctions in 18%, cardioembolic infarctions in 17%, hypertensive cerebral hemorrhages in 16%, lobar type hemorrhages in 3%, subarachnoid hemorrhages in 4%, progressive subcortical vascular encephalopathy of the Binswanger type (PSVE) in 8%, and others. Among 3 periods from 1975-1984, 1985-1994, 1995-2004, PSVE cases decreased during the last period, but there was no significant difference in the relative proportions of the other types of CVD during these 30 years. History of hypertension was recorded in 2/3-3/4 of the atherothrombotic infarction, in 3/4-4/5 of the lacunar infarction, and in 3/4-4/5 of the cerebral hemorrhage. Severe atherosclerosis in the main stem of cerebral arteries was found in about 3/4 of the atherothrombotic infarction, in about half of the lacunar infarction. Most frequent cardiogenic embolic source was nonvalvular atrial fibrillation showing about 3/4 of the embolic infarctions. The incidence of cerebral arterial aneurysm and of subarachnoid hemorrhage was higher in females than in males.

Responsible locus for dementia in Parkinson disease (PD) was investigated. Serial 1,395 autopsy cases were studied for the combined pathology of PD and Alzheimer disease (AD). Following the one-year rule by the first Consensus Guidelines, definite AD pathology was quite rare in PD with dementia (PDD) but common in dementia with Lewy bodies (DLB) . Plaque-dominant senile changes apparently enhanced neocortical Lewy-body pathology in both the conditions. About the hypometabolism in the visual cortex of PDD, a 66-year old man presented with fluctuation in hallucination commensurate with fluctuating hypometabolism. Considering the paucity in pathological changes of the visual cortex, this hypometabolism may represent functional impairment in the fiber connection. Comparative pathological studies with PD and PDD were carried out. Only one case of a 48-year-old woman, who unexpectedly died of heart failure, was free from cognitive decline, and did not show limbic and neocortical involvement. Another case of a 75-year old man with MCI presented with the similar pathology. All other cases showed clinical documentation of cognitive impairment and limbic and neocortical pathological involvement. Thus, the combination of prospective clinical and radiological studies and retrospective pathological studies (dynamic neuropathology) may be essential to investigate a role of the basal-forebrain cholinergic system.

An 80-year-old man who had was administered antihypertensive medication showed repeated transient unresponsiveness during standing and/or walking. Neurological examination showed no focal neurological signs except mild dementia. Head-up tilting examination induced unresponsiveness and a decrease of systolic blood pressure from 111 mmHg to 86 mmHg. This unresponsiveness disappeared 3 minutes later when the blood pressure recovered to 102/64 mmHg. Cerebral angiography demonstrated occlusion in the right internal carotid artery and the right vertebral artery. Severe stenosis was observed at the left internal carotid artery and the left vertebral artery. Discontinuance of the antihypertensive medication reduced the frequency of orthostatic transient unresponsiveness. Elastic stockings in the lower extremities completely prevented the attack; head-up tilting did not induce hypotension with elastic stockings. The transient unresponsiveness in the present case was probably correlated to hemodynamic failure under the condition of severe cerebral arteriosclerosis.

We report a 68-year-old man who have had only slowly progressive micrographia in both hands over the past 5 years without rigidity, tremor, bradykinesia, and festination or freezing in walking. The fine finger movements were normal. PET studies suggested the diagnosis of progressive supranuclear palsy or pure akinesia. Administration of L-DOPA and L-threo-DOPS did not improve his micrographia. Festination and freezing in writing may contribute to the genesis of micrographia in this case. We postulate that "pure micrographia" is an early atypical sign of pure akinesia.

From an electron microscopic study I indicated that the number of nerve fibres per unit area in the frontal white matter was significantly less in PSVE than that in controls. The white matter pallor in PSVE is mainly based on the loss of nerve fibres. The dementia in PSVE is probably related to the loss of nerve fibres in the cerebral white matter. The extent of frontal white matter pallor tended to be less broad in vascular parkinsonism (VP) than in PSVE without parkinsonism. The relatively slight damage of the white matter may contribute to VP observed in PSVE. The number of oligodendrocytes and of astrocytes was decreased in PSVE showing half of that in the deep white matter of the controls. This suggests that the loss of oligodendrocytes and astrocytes can play a role to the process of nerve fibres loss in PSVE. Not only the severe hyalinotic changes in the white matter arterioles, but also the diffuse atherosclerotic lesions in the main stem of cerebral arteries were the characteristic arterial lesions in PSVE. On the basis of these arteriosclerotic lesions, we speculated that the repeated hypotension or the greater variability of casual blood pressure could induce PSVE in the elderly hypertensives. However, the retrospective analysis of casual blood pressure did not support our hypothesis.

FDG-PET activation study was performed in two pure akinesia (PA) patients with silent pattern in surface electromyography (EMG) frozen gait analysis. Three patients (2 PA and 1 PD with reciprocal co-contraction pattern) and 7 normal subjects underwent FDG-PET study in two different condition of "rest" and "walk", in order to evaluate the change in regional cerebral activity during gait. In a PD patient, the activated pattern was no different from that of normal individuals; the cerebellar vermis was solely activated significantly. In contrast, 2 PA patients showed characteristic activation in the dorsolateral frontal area in addition to the areas found in normals. These results suggest that the dorsolateral frontal cortex may play an inhibiting role to the motor cortex resulting in the silent pattern of frozen gait in PA. Combined study of surface EMG and FDG-PET during gait may be useful for analyzing the pathophysiological mechanism of gait disturbance.

It remains controversial whether agraphia can coexist in a case with apraxia of speech, and whether an apraxia of speech can be classified into a category of aphasia. We examined the presence of agraphia in 2 right-handed patients of apraxia of speech. Case 1 of mild agraphia showed an infarcted lesion in the left precentral gyrus extending to the neighboring white matter, which involving the arcuate fasciculus on MRI. Positron emission tomography (PET) indicated decrease of cerebral blood flow (CBF) and cerebral metabolic rate of oxygen (CMRO2) in the infarcted lesion, but no decrease of CBF and CMRO2 in the Broca's area. In this case, agraphia was more conspicuous in "kanji" than in "kana" and severity of the agraphia was not correlated to that of speech disturbance on naming test. Case 2 without agraphia showed a small infarcted lesion in the left precentral gyrus, which did not extend to the deep white matter on MRI. Agraphia can coexist with apraxia of speech in a case with the lesion in the left precentral gyrus, in which the cortical lesion is relatively widespread or extends to the deep white matter. However, lack of etiological connection between the agraphia and the apraxia of speech was suggested. We could not confirm the location in the left precentral gyrus which is responsible for the agraphia.

We reported a familial case of parkinsonism in which clinical features are different between a mother and her daughter. The mother developed rigidity and bradykinesia at age 76. Her symptoms were slowly progressive, and were responsive to levodopa. Thus, she was diagnosed as having Parkinson's disease. In contrast, her daughter developed postural instability as well as rigidity and bradykinesia at age 53 in rapid progression. In addition, she also developed pyramidal signs and autonomic failures, including orthostatic hypotension and urinary incontinence. The disease phenotype of the daughter resembled that of striatonigral degeneration (SND). However, PET studies showed normal binding of 11C-N-methyl-spiperone (NMSP) in the striatum, suggesting that the daughter was also affected with Parkinson's disease.

We investigated the existence, clinical symptoms, and brain pathology of cerebrovascular parkinsonism. In this study, clinicopathological criteria of cerebrovascular parkinsonism was defined as follows: 1) at least 2 or more symptoms from the following 4 symptoms: tremor, rigidity, akinesia or bradykinesia, and short stepped gait or freezing, 2) no depigmentation, no Lewy body at the substantia nigra, no other degenerative disease, and 3) existence of cerebrovascular lesions. Among consecutive 4,000 autopsy series in the elderly, clinicopathologically confirmed cerebrovascular parkinsonism was found in 24 patients with mean age of 80 years. Cerebrovascular parkinsonism was characterized by the short-stepped gait as initial symptoms, absence of the resting tremor, lead-pipe rigidity, the symmetry of findings, and negative response to levodopa. Pseudobulbar palsy was observed in 54%, pyramidal findings in 63% of the cases. Most cases had multiple vascular lesions of the basal ganglia, but the distribution of lesions was not different from that in the cases of progressive subcortical vascular encephalopathy of Binswanger type without parkinsonism. Diffuse pallor and the loss of oligodendrocytes in the frontal white matter observed in the cerebrovascular parkinsonism suggested that the symptoms of parkinsonism resulted from the white matter damages in the frontal lobe.

We report an autopsy case of 66-year-old woman with closed head injury due to a fall from stairs. She had recovered after a half day period of coma following the fall. Severe right peripheral oculomotor nerve palsy was noted from the beginning. MRI demonstrated lesions in the corpus callosum and right midbrain. She had died of suffocation due to sputum impaction after 50 hours after the fall. Autopsy examination revealed traumatic subarachnoidal hemorrhage, partial tearing and petechial hemorrhage of the corpus callosum, and many axonal retraction balls scattered in the cerebral white matter. Avulsion of the right oculomotor nerve root and fresh midbrain infarct with hemorrhage in the territory of right paramedian artery were observed. Axonal retraction balls were densely distributed in the periphery of midbrain infarct. The distribution pattern of axonal retraction balls in the cerebrum was similar to that of damage due to shear strain in Holbourn's gelatin model of closed head injury. The midbrain infarct and oculomotor nerve avulsion may be due to both mechanical and ischemic damage during and after the fall. All of the lesions described above may be closely related to the rotatory force from the right forehead.

An old woman had stroke at age 67. Cerebral CT scan disclosed the low-density lesion with high-density area in the left temporal lobe, but no empty delta sign in the superior sagittal sinus. Thereafter, she had sometimes panic attacks. At the age of 70, she was admitted because of loss of consciousness probably caused by the same attack. On admission, Brain CT scan showed no fresh lesions. During the stay in the hospital, she had an episode of deep vein thrombosis of her left leg. The patient was found to have not only reduced biochemical activity but also low immunological level of AT III. Her nephew had also the low plasma AT III antigen concentration. Transesophageal echocardiography showed no abnormality, but atherosclerotic change at the aortic arch. We speculated that hemorrhagic cerebral infarction in the territory of the branch of middle cerebral artery could be induced by the arterial thrombus which was related to the hypercoagulable state associated with familial AT III deficiency, although the possibility of cardiogenic embolic infarction or of cerebral vein thrombosis could not be ruled out. Familial AT III deficiency is one of the causes of cerebral infarction even in the elderly.

We described an autopsy case of 79-year-old woman with clinically unclassified senile dementia. The patient developed forgetfulness at the age of 73, and later, persecution mania, apathy and episodic stupor, but no extrapyramidal symptoms. Neuropathological examination revealed severe neuronal loss and gliosis of substantia nigra, moderate neuronal loss and marked grumose degeneration of dentate nucleus, and mild astrocytosis of subthalamic nucleus. Abundant neurofibrillary tangles (NFT) were observed in subthalamic nucleus, globus pallidus, substantia nigra, locus ceruleus, tegmentum of brain stem, pontine nucleus, inferior olive, and dentate nucleus. Gallyas silver impregnation method showed a wide distribution of argyrophilic grains and threads in cerebrum, brain stem and cerebellum. Although absence of clinical and neuropathological hallmarks excluded the diagnosis of progressive supranuclear palsy (PSP), the distribution of NFT and argyrophilic grains in this patient resembled PSP.

A 55-year-old woman was admitted to our hospital with abrupt onset of chorea-ballism in the right arm and both legs. She had thirst before admission. The blood glucose level was 976 mg/dl, the serum osmolality was 335 mOsm/l, and there was slight ketonuria on admission. Even after normalization of the blood glucose level choreiform movement of the right arm and leg continued. Brain CT showed bilateral and symmetrical high density of the putamen on admission, which diminished at 32 days leaving an area of low density. This suggested that the high density was not calcification. There was no mass affect. Symmetrical high intensity of the bilateral putamen on MR T1-weighted images which was slight on admission increased in intensity after 2 months, but disappeared 4 months after the onset in association with the disappearance of choreiform movement. The abnormality in the putamen was considered to be the cause of her involuntary movements. In nonketotic hyperglycemia, high intensity basal ganglia lesions on T1-weighted MR images have previously not been reported. High intensity in the putamen on T1-weighted MR images in the present case might represent multiple petechial hemorrhages which occurred in association with metabolic disorders including nonketotic hyperglycemia.

A 66-year-old man was admitted to Tokyo Metropolitan Geriatric Hospital because of progressive speech disturbance and ataxic gait. He had no history of abdominal surgery, liver disease, heavy drinking nor blood transfusion. One day before the admission, he showed confusional behavior for 6 hours, but he had no other history of consciousness disturbance. Neurological examination revealed slurred speech, fixation nystagmus, limb and gait ataxia, and hyperreflexia with pathological reflexes. Extrapyramidal signs were not identified. Laboratory examination showed marked hyperammonemia (118-292 micrograms/dl) with poor ICG excretion (ICG15 min = 30.8%). Percutaneous portography showed a large shunt vessel between the portal vein and the left hepatic vein. The plasma ammonium level of the right hepatic vein is normal (15 micrograms/dl), but that of the left one is very high (298 micrograms/dl). Therefore we concluded that hyperammonemia of the systemic circulation was resulted from portal-systemic shunt. A T2 weighted MRI image demonstrated symmetrical high signal intensity in the deep cerebral white matter. Unique lesions were observed in the bilateral middle cerebellar peduncles, and shown as low signal intensity in T1 weighted image and as high signal intensity in T2 weighted image. Hyperammonemia and neurological impairments of this patient did not improve with medical treatment. Three months after occlusion of the shunt vessel, fixation nystagmus and extensor plantar responses abolished and unsteadiness gait improved. Hyperammonemia might cause the cerebellar ataxia in the present case.

A 68-year-old right-handed woman was admitted to Tokyo Metropolitan Geriatric Hospital because of slowly progressive dysarthria and writing disability over 2-year period. On admission, severe dysarthria was observed, but no dysphagia. The dysarthria mostly resembled a type of pseudobulbar palsy, although it was associated with effortful speech production. An oro-facial apraxia was also found. She could name objects, and could understand spoken words correctly. Examination using the Western Aphasia Battery showed diminution of word fluency, impaired repetition and perseveration and writing errors. On the Wechsler Adult Intelligence Scale-R verbal IQ was 100 and performance IQ was 87. These scores did not suggest any significant degree of general intellectual deterioration. Wisconsin card sorting test disclosed mild frontal dysfunction. Magnetic resonance imaging showed cortical atrophy in the bilateral frontal and temporal lobes. Measurements of regional cerebral metabolic rate by 18F-FDG-PET demonstrated decreased uptake in the latero-dorso-inferior area of the bilateral frontal lobes, especially on the left side. The present case showed slowly progressive dysarthria and progressive aphasia without generalized dementia, and without typical aphasia. These symptoms are speculated to be related to the atrophy in the bilateral frontal and temporal lobes shown by MRI and the decreased metabolic rate in the left dominant bilateral frontal lobes on PET study. The pathologic process responsible for these lesions remains obscure.

A 68-year-old man was admitted because of sudden onset gait disturbance and bradykinesia. He experienced left putaminal bleeding following mild right hemiparesis and emotional incontinence without any difficulties in his daily life since three years before admission. On neurological examination the patient was alert and oriented. He showed forced crying. Myerson's sign was positive. There were no abnormal findings in ocular movements, pupillary reflexes and other cranial nerves. Muscle tone was increased with cog-wheel phenomenon in bilateral upper extremities. Coordination was preserved. He showed severe akinesia and small steppage gait with stooped posture. Freezing phenomenon was observed in initiation of gait and turning. The deep tendon reflexes were increased in the right side with bilateral pathological reflexes. There was no definite weakness and sensory disturbance in all extremities. Brain CT revealed a small high density lesion in the medial side of right cerebral peduncle and a lens-shaped low density lesion in the left putamen. On T1 and T2 weighted images of MRI, right peduncular lesion showed low signal. It extended to the substantia nigra which was partially destructed. His parkinsonism was rapidly improved and completely disappeared within following two weeks. High density lesion of right peduncle on CT also disappeared. We discussed the mechanisms of parkinsonism following unilateral mesencephalic hemorrhage in this patient.

An 80-year-old right-handed man suddenly became impossible to recognize any color 7 years prior to admission. He complained that everything looked like of the same color, monochromatic. On admission, he could not discriminate any color and any familial face. Left homonymous hemianopsia associated with right lower partial visual filed defect was observed, but visual acuity of both eyes was well preserved. Visual-visual color tasks (Ishihara, matching, Hue test, Panel-D15) disclosed the disturbances in color perception. However, he could roughly distinguished red or brown from the other colors. The color test was also impaired regarding the visual-verbal color tasks (naming, pointing). However, verbal description of the color concept, which was shown by the verbal-verbal color tasks, was well preserved. In addition, we observed left hemispatial neglect, disturbance of face recognition and topographical disorientation. MRI revealed old hemorrhagic infarcts in the bilateral occipital and temporal lobes, including the bilateral lateral and medial occipito-temporal gyri. Disturbance of color recognition in this case was diagnosed as cerebral achromatopsia on the basis of clinical characteristics and MRI findings. This is the first case of cerebral achromatopsia of which lesions were detected by MRI in detail.

An 85-year-old housewife was admitted owing to the sudden onset of amnestic syndrome on June 27, 1986. There was no history of abulia or somnolence. Though she showed severe amnesia, her understanding was not impaired. There was no aphasia, no dysarthria or other focal sings. The CT showed a low density area in the genu of the left internal capsule. The patient's amnestic syndrome did not improve during the following four years and thus she was readmitted for further examination in July, 1990. Although her WAIS scores were fairly good and intelligence was considered normal, she showed very poor performance on the Wechsler memory scale-R and Benton visual retention test. MRI of the brain showed infarction which extended from the genu to the anterior limb of the left internal capsule. The longstanding amnesia in the present case was induced probably by the infarction of the genu of the left internal capsule, where some fibers of memory pathways, such as the anterior thalamic peduncle, ansa peduncularis, and stria terminalis, may pass.

We report a 77-year-old woman with Guillain-Barré syndrome following Campylobacter jejuni infection. She was admitted complaining of mild weakness in the left leg. Seven days before, she had severe diarrhea, which continued several days. After admission, the weakness soon worsened resulting in tetraparesis, and the respiration became so impaired that she was supported by the artificial ventilator. Deep tendon reflexes were absent in four limbs, and no sensory disturbance was noted. CSF contained the protein as low as 20.4 mg/dl in concentration; no pleocytosis was seen. Muscle action potentials evoked by the stimulation of nerves were markedly reduced in amplitude, but conduction velocity along the nerve was decreased mildly. Campylobacter jejuni was detected by bacterial culture of the stool; the antibody was positively raised against this bacteria in serum. By 90 hospitals days, she was restored to spontaneous respiration.

To investigate the histopathological basis of white matter pallor on myelin staining in PSVE, the frontal white matter was examined by electron microscopy in 7 cases with PSVE. The number of nerve fibres per unit area of the white matter was significantly less in PSVE compared with that in the control group or in senile dementia of Alzheimer's type (SDAT). Nerve fibers in PSVE had a tendency to have thinner myelin sheaths than in the control group or in SDAT, but the difference was not significant. The white matter pallor in PSVE is mainly based on the loss of nerve fibres. The dementia in PSVE is probably related to the loss of nerve fibres in the cerebral white matter. A sum of oligodendrocytes and astrocytes per unit area decreased in the area with white matter pallor. Main stem of cerebral arteries, leptomeningeal arteries, medullary arteries in the cortex, and small arteries or arterioles in the cerebral white matter were studied pathologically in 22 elderly patients with PSVE. Not only the severe hyalinotic changes of arterioles in the cerebral white matter, but also the diffuse atherosclerotic lesions in main stem of cerebral arteries and/or the stenotic lesions in leptomeningeal arteries were the characteristic arterial changes of PSVE. Angionecrosis was observed in 70% of the PSVE patients. To substantiate my hypothesis that PSVE in the elderly can be induced by the repeated hypotension or the greater variability of the casual blood pressure in the hypertensives, I studied retrospectively the casual blood pressures in PSVE.(ABSTRACT TRUNCATED AT 250 WORDS)

A case with non-paralytic pontine exotropia (NPPE) due to brainstem infarction is reported. A 77-year-old hypertensive man suddenly developed dizziness, double vision, dysarthria, and right ataxic hemiparesis. Oculomotor findings on admission consisted of: (1) full right exotropia in the primary position; (2) complete adductive paralysis of the left eye with slight preservation of convergence; (3) tonic deviation of the right eye to the full abducting position with right-beating nystagmus after an immediate forward gaze. The leftward saccades showed multiple saccades with slow velocity on electronystagmography (ENG). The right exotropia disappeared and the slight adductive paresis of the left eye remained with right monocular nystagmus seven weeks after the onset. Magnetic resonance imaging (MRI), which was performed nine weeks after the onset, disclosed a small lesion with high intensity involving the left medial longitudinal fasciculus (MLF) on T2-weighted spin echo image. The leftward saccades showed multiple saccades with normal velocity eleven weeks after the onset. The hypofunction of unilateral PPRF with ipsilateral MLF lesion probably causes the contralateral NPPE.

It has been reported that most of pure motor monoparesis were induced by the superficial mass lesions in the cerebral hemisphere. However, pure motor monoparesis due to lacune is rare. We reported a 81 year-old woman of pure motor monoparesis caused by the lacunar infarction in the parasagittal subcortical white matter. That lesion could not be detected by CT scan performed at onset, and 18 days later after the stroke, but was detected by MRI performed 5 days after the onset. The coronal section of MRI showed the stick-like shaped small infarction in the white matter, which runs along the corticospinal tract, just under the precentral gyrus. The coronal section of MRI is useful for the demonstration of the anatomical localization of lacune.

A 89-year-old man was admitted because of slowly progressive gait disturbance during these several years. Neurological examination revealed paraparesis with bilateral sensory disturbance in the lower extremities, more severely on the left side. Vibration sense was almost completely disturbed under the level of bilateral crista iliaca. Deep tendon reflexes decreased in the lower extremities. No urinary incontinence was observed. The narrow spinal canal and cervical spondylosis were seen at the C 5-6 level, showing the anterior-posterior distance of 10 mm. Computed tomography of the lumbar spine showed spondylolisthesis between the level 4 and 5. CSF showed high protein concentration (300 mg/dl), whereas normal cell counts. These findings suggested the following two possibilities; cervical myelopathy associated with the disturbance of the cauda equina due to lumbar spondylolisthesis, or the disturbance at the lower thoracic or upper lumbar level of spinal cord. The spinal MRI revealed the irregular mass lesion in the lower spinal cord at the level of spine Th 11-12 on T2 weighted images, with enhancement by Gd-DTPA on T1 weighted images. These MRI findings suggested the intradural extramedullary tumor, such as benign neurinoma or meningioma. No clear cut lesions were found at the cervical spinal cord or at the cauda equina. MRI was useful for the diagnosis of sites and lesions of spinal cord in the present case, whose neurological signs and symptoms could be explained by the coexistent cervical spondylosis and lumbar spondylolisthesis.

We report two males with progressive post-poliomyelitis muscular atrophy (PPMA) who developed slowly progressive weakness and atrophy in the previously unaffected muscles 50 years after acute poliomyelitis. Case 1 is a 61-year-old male who had suffered from poliomyelitis at the age of 3 years. After recovery from acute symptoms, paralysis remained in the right face, left upper extremity and right lower extremity. Paralysis had remained unchanged until the age of 55 years, when he felt increased fatigue and progressive muscle weakness of all extremities. Neurological examination revealed muscle atrophy of the left upper and right lower extremities, and muscle hypertrophy of the left lower extremity. Deep tendon reflexes were decreased or absent. Babinski sign was negative. Reduction of his physical level and administration of anticholinesterase agents improved his feeling of fatigue. Case 2 is a 66-year-old male who suffered from poliomyelitis in his infancy, and paralysis remained in the left hand and right lower extremity. At the age of 50 years, he felt fatigue, muscle weakness of the previously unaffected legs and joint pain. Neurological examination revealed proximal muscle atrophy and weakness of lower extremities. Deep tendon reflexes were decreased and Babinski sign was negative. Needle electromyography of both cases showed typical neurogenic pattern or high amplitude and polyphasic long duration MMUs. Muscle biopsy of them from quadriceps femoris showed variability in fiber size, moderate increase of internal nuclei, fiber splitting, type grouping, grouped atrophy and scattered small angulated fibers, indicating chronic denervation and reinnervation. The clinical features and laboratory findings of the present two cases are consistent with those of PPMA.(ABSTRACT TRUNCATED AT 250 WORDS)

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)

Flunarizine hydrochloride (FZ), a calcium entry blockade, has been used nationwide in Japan as a cerebral active vasodilator since October, 1984. The present paper reports 31 cases of FZ-induced Parkinsonism, depression and akathisia, referred to our hospital between October 1986 and September 1988. Out of the 31 patients, four including two with Parkinson's disease and one each with progressive supranuclear palsy and olivopontocerebellar atrophy showed worsening of their parkinsonian symptoms within a few months after FZ administration. The remaining 27 patients (7 males and 20 females) newly developed Parkinsonism after treatment with FZ. Symptoms appeared one week to two years (mean: 6.1 months) after starting FZ of a daily dose of 10 mg. FZ had been used in 6 patients for cerebrovascular episodes confirmed by clinical history or brain CT, and in the remainder, for dizziness, light-headedness, hypertension, amnesia or hypochondric neurotic complaints. Akinesia and bradykinesia progressed rather rapidly after onset, and patients became unambulatory within several months. Symptoms had worsened, and L-dopa, anticholinergic drugs, and bromocriptine had been ineffective until FZ was discontinued. Their Parkinsonism was characterized by marked akinesia, bradykinesia, and moderate rigidity. Masked face was seen in most of them. Tremor was absent at rest, and induced in 12 patients by posture and/or action. Sixteen patients were accompanied by depression, and five, by akathisia. Improvement began several weeks after withdrawal of FZ, and most patients recovered almost completely within a few months although mild rigidity and bradykinesia remained in some.(ABSTRACT TRUNCATED AT 250 WORDS)

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.