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Results for “Rapidly progressive dementia”

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At least 19 recordsLinked to original sources

PubMed1993

[A 61-year-old man with rapidly progressing dementia and gait disturbance].

We report a 61-year-old male with rapidly progressive dementia and gait disturbance. He was well until spring 1990 as a postmaster, when there was an onset of memory disturbance and mistakes in his job. In May 1990, his wife noted slurring of his speech. In August, there was an onset of gait disturbance. He fell down frequently. In October, he was seen by a neurologist, who found moderate dementia, small step gait, retropulsion, freezing, paratonic rigidity, bradykinesia and a restriction in the vertical gaze on him. His dementia and gait disturbance progressed rapidly and in May 1991, he developed fever and dyspnea and was admitted to Juntendo University Urayasu Hospital. On admission, he was chronically ill and wheezing rale was heard on both lung fields. Neurologically, he was awake but without response to the simplest examiner's command. Cranial nerves appeared intact except for a restriction in the upward gaze. His posture was opisthotonic with a decorticated posture. Marked rigidity was present in all four limbs. He could not sit or stand. Deep reflexes were diminished symmetrically. He was treated by supportive cares, however, he expired 12 days after his admission. In no time myoclonus was observed, nor PSD recorded in his EEG. Cranial CT scans revealed moderate cortical atrophy. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that he had Creutzfeld-Jakob disease despite the absence of myoclonus and PSD. Postmortem examination revealed diffuse spongy state of the cerebral hemisphere as well as striatum.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗

PubMed2005

[Sporadic cerebral amyloid angiopathy with ischemic leukoencephalopathy and microbleeds. Cause for a rapidly progressive dementia syndrome].

We describe a 72-year-old patient with rapidly progressive dementia and a complex focal seizure. Magnetic resonance (MR) imaging revealed leukoencephalopathy with the involvement of the U-fibers as well as cortical and subcortical microbleeds. Brain biopsy confirmed the diagnosis of cerebral Abeta amyloid angiopathy (CAA). The presented case illustrates the significance of CAA as a cause of rapidly progressive dementia and leukoencephalopathy and points out the importance of T2-weighted MR imaging in the evaluation of dementia.

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PubMed1991

Angiotropic lymphoma (malignant angioendotheliomatosis) presenting with rapidly progressive dementia.

An 87-year-old male presented with rapidly progressive dementia, disorder of consciousness and myoclonus, suggesting Creutzfeldt-Jakob disease, but with nonspecific EEG and negative CT findings. Autopsy revealed a malignant angiotropic lymphoma with accumulation of tumor cells within small vessels of the brain and visceral organs. Immunohistochemistry disclosed a large cell lymphoma of B cell type.

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PubMed2003

14-3-3 protein in the CSF of patients with rapidly progressive dementia.

BACKGROUND: The presence of 14-3-3 protein in the CSF has been described to have high sensitivity and specificity for Creutzfeldt-Jakob disease (CJD). OBJECTIVE: To relate 14-3-3 protein in the CSF with the clinical diagnoses of diseases causing rapidly progressive dementia. METHODS: The authors studied 46 patients with rapidly progressive dementia that was classified into three diagnostic groups: definitive or probable CJD, possible CJD, and other diagnoses. The definitive or probable CJD group comprised 17 patients (3 definitive sporadic, 1 probable iatrogenic, 3 familial, and 10 probable sporadic CJD cases), the possible CJD group was composed of 7 patients, and the group with other diagnoses had 22 patients. Detection of the 14-3-3 protein was done by the immunoblotting method. RESULTS: In the definitive or probable CJD group, the test for 14-3-3 protein in CSF was positive in 14 (82%) cases, whereas 3 patients (1 probable sporadic and 2 familial cases) had negative results. CSF was positive for 14-3-3 protein in three of seven cases with possible CJD (42%). In the group with other diagnoses, three individuals had false-positive results (13%). Their diagnoses were definitive Alzheimer's disease, hypercalcemia, and multiple intracerebral hemorrhages. CONCLUSIONS: The detection of 14-3-3 protein in CSF is a useful in vivo diagnostic test for CJD and, when used in the appropriate clinical context, shows a good correlation to CJD. The presence of the 14-3-3 protein in the CSF reinforces the CJD clinical diagnosis but may not be able to differentiate CJD from other causes of rapidly progressive dementia in everyday clinical practice.

14-3-3 Proteins↗

PubMed1997

A 66-year-old woman with a rapidly progressing dementia and basal ganglia involvement.

A 66-year-old woman presented with a rapidly progressive dementia initially characterized by an auditory agnosia. She experienced a rapid progression of her aphasia and developed ataxia and myoclonus. An initial neurological evaluation suggested a left parieto-temporal lesion, however, neuroimaging did not reveal any. An MRI of her brain demonstrated highly focal T2 hyperintensities in her basal ganglia. The patient was diagnosed with Creutzfeldt-Jacob Disease (CJD) on the basis of the presence of two proteins in the CSF that are highly sensitive and specific for CJD. Pathological examination confirmed the diagnosis. The differential diagnosis and utility of MRI in patients with CJD is discussed.

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PubMed1996

Rapidly progressive dementia caused by nonenhancing primary lymphoma of the central nervous system.

A 76-year-old woman had rapidly progressive dementia over 4 months. Proton density- and T2-weighted MR images of the head showed increased signal in the periventricular and subcortical white matter of both cerebral hemispheres and the brain stem. Enhancement was not seen after administration of contrast material. Because of the rapid progression of the dementia and increasing signal abnormalities within the cerebral white matter, the patient underwent a craniotomy with biopsies of the right frontal lobe. Pathologic specimens were positive for malignant large-cell lymphoma, B-cell phenotype. The MR appearance is atypical for primary lymphoma of the central nervous system.

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PubMed2005

Miliary brain metastases presenting as rapidly progressive dementia.

We report the case of a 79-year-old woman who developed a rapidly progressive dementia (RPD) with severe memory impairment, early visual hallucinations and extrapyramidal signs. Symptoms started suddenly after hip replacement surgery following an accidental fall. Motor epileptic seizures appeared at the end of the illness. Dementia worsened gradually leading to akinetic mutism. She died five and a half months after the onset of symptoms. MRI showed cerebral atrophy but failed to detect any other lesion. Results of all laboratory tests performed were negative. After the most frequent treatable diseases were excluded, the diagnosis of dementia with Lewy bodies was initially considered. CJD was also suggested based on the rapid evolution of the disease and the positivity of 14-3-3 protein in CSF. Neuropathological examination revealed an extensive miliary metastatic dissemination from an unknown primary adenocarcinoma. Pulmonary origin was suggested according to the immunohistochemical profile. Histopathological changes of Alzheimer's disease were also observed in the cerebral cortex and hippocampus. Neither Lewy bodies nor PrP deposits were found. The sudden onset of the dementia just after the hip replacement surgery raises the possibility of a pathological fracture with secondary tumoral microembolic dissemination. Despite its rarity, this entity should be included in the differential diagnosis of RPD. This case illustrates the definite importance of neuropathological post-mortem examination in order to elucidate the different types of dementia.

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PubMedDate not supplied

Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings.

Primary central nervous system lymphoma (PCNSL) usually presents with clinical and neuroimaging findings consistent with single or multiple intracranial mass lesions. On cranial magnetic resonance imaging (MRI), such lesions are nearly always contrast enhancing, reflecting disruption of the blood-brain barrier at the site of tumor nodules. We describe 2 cases from the UCLA Medical Center who developed a rapidly progressive dementia due to extensive gray and white matter cerebral lesions involving much of the brain. In the patient who came to autopsy, widely infiltrating, focally necrotic B-cell plasmacytoid lymphoma was noted throughout the cerebral neuraxis. MRI findings in case 2 were consistent with diffuse lymphomatous brain infiltration without mass lesions, which was biopsy proven. We conclude that PCNSL may occur in a diffusely infiltrating form which may occur without MRI evidence of mass lesions or blood-brain barrier compromise. We refer to this entity as 'lymphomatosis cerebri' and add it to the differential diagnosis of a rapidly progressive dementia.

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PubMedDate not supplied

Neuropsychological features of rapidly progressive dementia in a patient with an atypical presentation of Creutzfeldt-Jakob Disease.

BACKGROUND: Creutzfeldt-Jakob Disease (CJD) is a degenerative disease of the brain, characterized by rapid and irreversible decline, with dementia, ataxia, myoclonus, and other neurological and neurobehavioral disorders associated with rapidly progressive spongiform encephalopathy. The mode of transmission and basic pathomechanism remain unclear. The clinical picture of CJD is highly diverse, producing a number of variants. MATERIAL AND METHODS: The patient to be described is a 68-year-old Polish female, JR, clinically diagnosed with CJD. The article presents the case history in detail, with particular emphasis on neuropsychological testing, which was initiated when the patient was still lucid and capable of cooperation. The first presenting symptom was agraphia, followed by hemianopsia and other vision disorders, culminating in visual hallucinations. As the progress of the disease accelerated there was rapidly progressive dementia, aphasia developing to organic mutism, myoclonus, hyperkinesia, ultimately loss of all verbal contact or voluntary movement. RESULTS: JR's neuropsychological parameters declined in a period of less than 3 months from near normal to levels characteristic of severe dementia. CONCLUSIONS: The clinical picture here presented is consistent with that of the Heidenhain variant of CJD, with spongiform encephalopathy beginning in the right occipital lobe. Several features of the case remain atypical, however, including the absence of the most common genetic mutation and the patient's long survival after onset.

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PubMed1997

Autoimmune thyroiditis and a rapidly progressive dementia: global hypoperfusion on SPECT scanning suggests a possible mechanism.

We report the clinical, laboratory, EEG, and SPECT findings in a 59-year-old euthyroid woman with previously undiagnosed autoimmune thyroiditis, subclinical hypothyroidism, and rapidly progressive dementia. We made a diagnosis of Hashimoto's encephalopathy based on elevated thyrotropin, abnormal EEG, and clinical improvement after thyroid hormone replacement. SPECT demonstrated global hypoperfusion with normalization on clinical recovery, suggesting a possible mechanism for the pathogenesis of Hashimoto's encephalopathy.

Brain↗

PubMed2002

Rapidly progressive dementia syndrome associated with a novel four extra repeat mutation in the prion protein gene.

OBJECTIVE: To gain a better understanding of how mutations of the prion protein (PrP) gene are responsible for progressive dementia syndrome and to clarify the correlation between genotype and phenotype, which should help to explain how the prion promotes neurological symptoms. BACKGROUND: Genetic prion diseases are associated with point or insertional mutations in the PrP gene. The insertional mutations described so far consist of one to nine extra octapeptide repeats, except three repeats. Insertions of one to four extra octapeptide repeats cause Creutzfeldt-Jakob disease (CJD) in patients without a family history of neurological disorders. CJD generally presents as progressive dementia. METHODS: Routine clinical assessment and sequence analysis of the PrP gene of DNA from a 56 year old Japanese man with progressive dementia syndrome. RESULTS: Sequence analysis disclosed a novel four octapeptide repeat insertion within the PrP gene. The patient was initially affected by progressive cerebellar and brainstem signs; a few months later myoclonus and rapidly progressive dementia appeared. These symptoms were similar to those of sporadic CJD. CONCLUSION: Taken together with previous investigations of CJD patients with insertional mutations, the current observation strengthens the notion that small octapeptide insertions from one to four extra repeats within the PrP gene cause CJD, which is characterised by late onset after the sixth decade, rapid progression, death within a few months, and lack of a family history of neurological disorders, the latter suggesting incomplete penetrance. Different patients with four extra octapeptide repeats have different patterns of extra insertions, suggesting that progression of the disease depends on the number of extra repeats.

Creutzfeldt-Jakob Syndrome↗

PubMed1996

Rapidly progressive dementia: a clinicopathologic correlation.

We evaluated a 66-year-old man with a rapidly progressive, akinetic-rigid dementia syndrome. Despite extensive testing, which included magnetic resonance imaging (MRI), we were unable to make the correct antemortem diagnosis. Autopsy demonstrated spontaneous progressive multifocal leukoencephalopathy. This report illustrates that even in the absence of characteristic MRI findings, this uncommon cause of dementia should be considered in the differential diagnosis of rapidly progressive, akinetic-rigid syndromes with dementia.

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PubMed1997

[Rapidly progressive dementia caused by tumor microembolism in the central nervous system].

Brain tumoral microembolism is a very unusual etiology of cerebral vascular disease in patients with cancer. The clinical course is characterized by diffuse encefalopathy with associated neurologic deficits. We report a 72-year-old male patient, who developed rapidly progressive dementia associated with diffuse myoclonic jerks, without neurologic deficit, whose brain biopsy showed microscopic emboli of adenocarcinoma of unknown origin in capillary vessels. We did not find metastasis in other organs. We suggest that this entity should be included in the differential diagnoses of the subacute dementias.

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PubMed2001

[Rapidly progressive dementia disclosing primary angiitis of the central nervous system].

Angiitis of the central nervous system involves a wide spectrum of clinical symptoms. Complementary exams (CSF, neuroradiological exams) are not very specific and sensitive. Diagnostic procedures often require cortical and leptomeningeal biopsy. Treatment, based on corticosteroids and cyclophosphamid, have greatly improved prognosis. We report a rapidly progress dementia syndrome that revealed angiitis of the central nervous system. Corticosteroid treatment did not prevent a fatal outcome. Autopsy findings confirmed the diagnosis of central nervous leucocytoclastic angiitis.

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PubMed2001

Two-octapeptide repeat deletion of prion protein associated with rapidly progressive dementia.

Insertions of integral numbers of an octapeptide repeat in the prion protein gene are pathogenic mutations associated with inherited prion diseases. Conversely, deletions of a single octapeptide repeat are found as normal polymorphisms in many populations and do not predispose individuals to prion disease. The authors report a two-octapeptide repeat deletion in an elderly woman with a rapidly progressive dementia consistent with Creutzfeldt-Jakob disease. This mutation was absent from more than 3,000 individuals and may be causally related to prion disease and represent a novel disease mechanism.

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PubMed2004

Rapidly progressive dementia due to Mycobacterium neoaurum meningoencephalitis.

Dementia developed in a patient with widespread neurologic manifestations; she died within 5 months. Pathologic findings showed granulomatous inflammation with caseation necrosis, foreign body-type giant cells, and proliferative endarteritis with vascular occlusions. Broad-range polymerase chain reaction identified Mycobacterium neoaurum as the possible pathogen. Central nervous system infection by M. neoaurum may result in rapidly progressive dementia.

Dementia↗

PubMed2004

Rapidly progressive dementia due to bilateral internal carotid artery occlusion with infarction of the total length of the corpus callosum.

The authors report a patient with rapidly progressive cognitive decline due to bilateral internal carotid artery occlusion (ICAO) resulting in multiple pathologically proven cerebral infarctions including the entire length of the corpus callosum. The gradual evolution of the deficits was suggestive of hemodynamic ischemia. Bilateral ICAO should be considered in the differential diagnosis of patients with rapidly cognitive decline. Although ICAO commonly spares the splenium, complete callosal infarction is possible in the presence of bilateral ICAO.

Carotid Stenosis↗

PubMed1999

[Brain SPECT using Tc-99m-bicisate (ECD) in rapidly progressive dementia syndrome].

We present a 61-year-old male patient with progressive dementia. A brain SPECT with Tc-99m-bicisate was performed for confirmation of clinically suspected Alzheimer-dementia. At the time of the SPECT-investigation marked apraxia and aphasia besides severe dementia were present. Electrophysiological as well as anatomical neuroimaging findings showed non-diagnostic alterations. SPECT revealed distinct perfusion defects, which made Alzheimer Dementia unlikely. The further course of the patient was determined by rapidly progressive deterioration with development of akinetic mutism. Thereafter, increased levels of neuron-specific enolase as well as 14-3-3 proteins were found in the cerebro-spinal fluid (CSF). The patient finally died with signs of cerebral decortication. Due to the clinical course and the CSF-findings the patient's final diagnosis was Creutzfeldt-Jakob-disease, nevertheless no autopsy was performed. The presented case report underscores the clinical utility of perfusion brain SPECT in the differential diagnosis of dementias.

Brain↗