Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0003635 (apraxia)
 2,817 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Subcortical dementia is a clinical syndrome characterized by slowness of mental processing, forgetfulness, impaired cognition, apathy, and depression. First recognized in progressive supranuclear palsy and Huntington's disease, the concept has been extended to account for the intellectual impairment of Parkinson's disease, Wilson's disease, spinocerebellar degenerations, idiopathic basal ganglia calcification, the lacunar state, and the dementia syndrome of depression. Disorders manifesting subcortical dementia have pathologic changes that involve primarily the thalamus, basal ganglia, and related brain-stem nuclei with relative sparing of the cerebral cortex. Recent studies of neuropsychologic deficits following focal subcortical lesions also support a role for these structures in arousal, attention, mood, motivation, language, memory, abstraction, and visuospatial skills. The clinical characteristics of subcortical dementia differ from those of dementia of Alzheimer's type where prominent cerebral cortical involvement produces aphasia, amnesia, agnosia, and apraxia.
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PMID:Subcortical dementia. Review of an emerging concept. 623 97

This work is to study the ultrastructure of the neuronal lipofuscin that occurred in the brain and the spinal cord of an autopsy case of familial Alzheimer's disease and to compare with those in several other diseases. The patient was a 46-year-old male, whose father and elder brother were diagnosed as Alzheimer's disease and died at the age of 42, respectively. He became afflicted with forgetfulness and disorientation at the age of 36. He developed a grand mal seizure at the age of 39 and thereafter, his clinical course was characterized by pyramidal signs, dysarthria and the symptoms of Gerstmann's syndrome, visuo-spatial agnosia, apraxia for dressing and constructive apraxia. He became bedridden at 45 years old and died of general prostration. The brain weighed 1,250 g, and the cerebral cortex showed mild atrophy. The neuronal loss, senile plaques and Alzheimer's neurofibrillary tangles were found throughout the cerebral cortex. The senile plaques were also found in the basal ganglia, the cerebellar medulla and cortex. There was severe amyloid angiopathy in the occipital and cerebellar cortices. The specimens for electron microscopy were taken from the cerebral cortex, the basal ganglia, the thalamus, the midbrain, the medulla oblongata, the cerebellum and the spinal cord. The ultrastructural study revealed three different types of the neuronal lipofuscin, though different stainability between these lipofuscin granules could not be manifested by several histochemical methods. Their morphological differences seemed to be based on the sites of the central nervous system.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Ultrastructural study of the neuronal lipofuscin--an autopsy case of familial Alzheimer's disease]. 648 35

This article, basing on experimental analysis and clinical observations, focuses on the role of subcortical structures in memory processes. It explained terminological problems and defined terms of memory: immediate, delayed, recent, remote, declarative and procedural. The present article pointed out functional hemispheric specialization as a predicator of material-specific forms of memory. Neuroanatomical basis was revealed, especially limbic system with its connections to prefrontal, cortical and brain stem regions. Amnesic Korsakoff and Wernicke syndromes, transient global amnesia, memory loss after bilateral damage of temporal lobes and after anterior communicating artery aneurysm rupture, were also discussed. Next part exhibited current knowledge about definition of dementia which may be caused by many multi-focal brain diseases like multiinfarct (vascular) dementia, Parkinson disease, Huntington disease, and sclerosis multiplex, and compared to Alzheimer disease. Term of dementia was defined, according to Cummings and Benson, as syndrome of acquired intellectual dysfunction when three of the following mental functions are impaired: language, memory, visuospatial skills, emotion, and cognition (abstraction, calculation, judgement). There is little doubt that various subcortical diseases are characterised by similar, no specific dysfunctions of cognitive processes including: disturbed attention and concentration, slowness of mental processing, forgetfulness, personality alterations and mood disturbances as well as motivational impairment, visuospatial disturbances, absence of symptoms of cortical dysfunction such as aphasia, agnosia and apraxia and associated motor disorder. Review of the literature suggests that rapid forgetting and retrieval deficits are most often symptoms of memory deficits observed after subcortical brain injuries.
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PMID:[Neuropsychological description of memory impairment following cortical and subcortical brain injuries]. 756 22

We report a patient with a ten-year history of slowly progressive recent memory decline without additional cognitive impairment in presenility. A right-handed Japanese barber first experienced forgetfulness at the age of 59. Neurological examination at age 64 revealed no abnormality except for severe impairment in memorizing. Brain CT-scans were normal. In spite of gradually deteriorating memory, he was capable of organizing his work until the age of 67 years. At age 69, he showed intense recent memory defect and disorientation in time, but immediate memory span and remote memory beyond the retrograde gap were better preserved. Intelligence quotient (IQ) on the Wechsler adult intelligence scale-R remained 85 (verbal IQ, 88: performance IQ, 83). Neurological examination was negative. He showed no signs of aphasia, agnosia, or apraxia. Minimal organic personality changes were noticed. Brain CT-scans and MRI revealed mild atrophy of the temporal lobes and hippocampal formation on both sides. I123-IMP single photon emission computed tomography (SPECT) disclosed a decrease of cerebral blood flow in both the inferior temporal and superior frontal regions. SPECT after acetazolamide administration showed augmented accumulation in areas with decreased accumulation at baseline. Despite progressive memory impairment, the absence of aphasia, agnosia, or apraxia differentiates our case from more common Alzheimer's disease. A degenerative disorder of focal cerebral atrophy or "simple senile dementia" of presenile onset was suspected of causing the underlying pathophysiological changes.
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PMID:[Slowly progressive memory impairment without generalized dementia--a clinical and radiological study]. 766 20

We report a 65-year-old female who have suffered from progressive gait disturbance for 3 years, followed by disorientation and forgetfulness. Neurological examination revealed dementia, constructional disability, limb kinetic apraxia, supranuclear gaze palsy, especially on downward gaze, symmetrical muscle rigidity and bradykinesia. Involuntary movements were undetectable. Brain MRI showed significant brain atrophy in the left fronto-parietal lobe. The three-dimensional surface display with 131I-IMP demonstrated decreased cerebral blood flow in the left frontoparietal cortex. The diagnosis of this case is discussed with regard to either progressive supranuclear palsy or corticobasal degeneration or both.
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PMID:[A case with clinical features of progressive supranuclear palsy with apraxia--corticobasal degeneration?]. 772

The cognitive disturbances in progressive supranuclear palsy (PSP) gave rise to the term "subcortical dementia." PSP patients demonstrate prominent recall deficits and moderate forgetfulness although their short-term and implicit perceptual memory processes are intact. PSP patients have both slowed motor responses and dramatically slowed information processing speed. Executive dysfunction appears early in the course of the disease and is relatively severe. The combination of severely slowed information processing and marked executive dysfunction are characteristic of PSP and differentiates it from other dementias. In their landmark description of progressive supranuclear palsy (PSP) as a clinicopathological entity, Steele et al. (1964) reported that cognitive disturbances were present in seven out of their nine patients. Ten years later, Albert et al. (1974) characterized these changes to be part of a "subcortical dementia," They analyzed 5 of their own PSP cases and also reviewed the published literature; they found a common cluster of symptoms, including the presence of forgetfulness, slowness of thought process, emotional or personality changes, and impaired ability to manipulate acquired knowledge. Albert et al. analysis was qualitative, but in the authors' view, clearly differentiated PSP patients from patients with "cortical dementia" who presented with aphasia, apraxia, and/or agnosia. They also suggested that the symptoms found in PSP were similar to those that had previously been described in patients with frontal lobe lesions.
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PMID:Cognitive disturbances in progressive supranuclear palsy. 796 98

We present a 81-year old male who developed dementia, gait disturbance and right hemiparesis. He was well until the age of 74 when he developed a hemorrhagic infarction in the right occipital region, which left him left homonymous hemianopsia. One year later he had one TIA attack consisting of dizziness, headache, and some clouding of consciousness. At that time, atrial fibrillation was found. At age 79, he was attacked by right hemiparesis. Cranial CT scans revealed a lesion consistent with a hemorrhagic infarct in the left middle cerebral artery territory. Two months prior to his final admission, he had a gradual onset of forgetfulness, labile affect, nocturnal agitation and hallucination which were followed by gait disturbance and urinary incontinence. On admission, he was alert but moderately demented. In addition he showed difficulty in repetition, limb kinetic and ideomotor apraxia of the left hand indicative of sympathetic apraxia, and constructional apraxia bilaterally. Granial nerves appeared intact except for left homonymous hemianopsia. His gait was wide-based and small stepped. No weakness or ataxia was noted. Deep reflexes were diminished on the left side. Plantar reflex was equivocally extensor of the left. Light touch and pain was slightly diminished on the right side. Cranial CT scans revealed a large low density area in the left fronto-temporo-parietal region. Also ventricular dilatation, diffuse low density change in the subcortical white matter, and diffuse cortical atrophy were seen. His clinical course was complicated by melena, anemia, pneumonia, cardiac failure and renal failure. He expired 2 months after his admission.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A 81-year-old man with dementia, gait disturbance, hemiparesis, and sympathetic apraxia]. 833 25

We reported an autopsy case with recent memory disturbance, characterized by localized atrophy of parahippocampal gyrus, subiculum and amygdala. This patient initially exhibited recent memory disturbance at the age of 73. She was disoriented to time and place and immediately forgot having had a meal. At the age of 75, she was hospitalized because of progressive forgetfulness and congestive heart failure. One year later, she was admitted to our medical center. On admission, she was alert, but showed severe recent memory disturbance and disorientation to time and place. By contrast, she had neither aphasia nor apraxia. No other neurological symptoms were found. Brain CT showed localized atrophy of the medial part of bilateral temporal lobes and brain SPECT (123I-IMP) revealed a decrease of cerebral blood flow in the same regions. We considered her as early stage of Alzheimer type dementia (ATD) clinically. She died of pneumonia and DIC at the age of 78. Her illness lasted about 5 years. General autopsy showed prolapse of mitral valves, bronchopneumonia and DIC. The brain weighed 1,150 gm. Coronal sections of the brain revealed locarized atrophy of bilateral mediobasal part of the temporal lobes including the rostral parahippocampal gyrus, subiculum and amygdala. There were severe neuronal loss with astrogliosis and a few neurofibrillary tangles (NFT) in the rostral para-hippocampus, CA1 of the hippocampal formation, prosubiculum and amygdala. There were neither senile plaques (SP) nor NFT in the cerebral neocortex. This case lacked neocortical SP and NFT and showed bilateral localized atrophy of rostral parahippocampal gyrus, CA1, subiculum and related structure of the ventromedial temporal lobe with severe neuronal loss and astrogliosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[An autopsy case with recent memory disturbance, characterized by localized atrophy of parahippocampal gyrus, subiculum and amygdala]. 833 75

A 65-year-old woman was admitted to our hospital for forgetfulness, depression and eccentric behavior that had been first noticed 2 years prior to admission. She showed memory impairment, perseveration and repeated violent actions, but no limb-kinetic apraxia. She died 12 years after the onset of symptoms. At autopsy, the unfixed brain weighed 820 g. Atrophy was circumscribed in the frontal lobe on both sides. The globus pallidus and the caudate nucleus were markedly atrophic and gold yellow in color, and the substantia nigra was strikingly pale. The cortical area showed neuronal loss and status spongiosus of the second and third cortical layers with ballooned neurons. Marked neuronal loss was observed in the dorsomedial nucleus of the thalamus, Meynert basal nucleus and substantia nigra. With Holzer stain, fibrillary gliosis was found to be severe in the frontal lobe, globus pallidus, subthalamic nucleus, hippocampus, dorsomedial nucleus of thalamus, substantia nigra, pontine tegmentum and inferior olivary nucleus. With Bielschowsky-Hirano stain, neurofibrillary tangles were observed in the cortex, hippocampus, substantia nigra, dentate nucleus, subthalamic nucleus, pontine nucleus, the inferior olivary nucleus, dorsomedial nucleus of the thalamus and, to a lesser extent, the neostriatum. Strikingly numerous argyrophilic and tau-positive threads were present in the cerebral white matter. These neuropathological findings corresponded to corticobasal degeneration, but lesions characteristic of progressive supranuclear palsy were also found. Moreover, widespread iron deposition throughout the central nervous system was the most striking finding of the present case. To our knowledge, such a case has not been reported in the literature to date.
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PMID:A case of clinically and neuropathologically atypical corticobasal degeneration with widespread iron deposition. 1190 10

Corticobasal syndrome (CBS) is characterised by asymmetrical parkinsonism and cognitive impairment. The underlying pathology varies between corticobasal degeneration, progressive supranuclear palsy, Alzheimer's disease, Creutzfeldt-Jakob disease and frontotemporal lobar degeneration sometimes in association with GRN mutations. A 61-year-old male underwent neurological examination, neuropsychological assessment, MRI, and HMPAO-SPECT at our medical centre. After his death at the age of 63, brain autopsy, genetic screening and mRNA expression analysis were performed. The patient presented with slow progressive walking disabilities, non-fluent language problems, behavioural changes and forgetfulness. His family history was negative. He had primitive reflexes, rigidity of his arms and postural instability. Later in the disease course he developed dystonia of his left leg, pathological crying, mutism and dysphagia. Neuropsychological assessment revealed prominent ideomotor and ideational apraxia, executive dysfunction, non-fluent aphasia and memory deficits. Neuroimaging showed symmetrical predominant frontoparietal atrophy and hypoperfusion. Frontotemporal lobar degeneration (FTLD)-TDP type 3 pathology was found at autopsy. GRN sequencing revealed a novel frameshift mutation c.314dup, p.Cys105fs and GRN mRNA levels showed a 50% decrease. We found a novel GRN mutation in a patient with an atypical (CBS) presentation with symmetric neuroimaging findings. GRN mutations are an important cause of CBS associated with FTLD-TDP type 3 pathology, sometimes in sporadic cases. Screening for GRN mutations should also be considered in CBS patients without a positive family history.
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PMID:Symmetrical corticobasal syndrome caused by a novel C.314dup progranulin mutation. 2186 16


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