1/9. 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.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
2/9. Sporadic Creutzfeldt-Jakob disease presenting with nonconvulsive status epilepticus.Creutzfeldt-Jakob disease (CJD) is a rare prion disease characterized by a spongiform encephalopathy in humans. Although the characteristic triad of myoclonus, dementia, and periodic EEG activity is easy to recognize, unusual manifestations of the disease may be challenging and create a diagnostic dilemma. We report a case of CJD that occurred in a 26-year-old patient who presented with a receptive (Wernicke's) aphasia secondary to nonconvulsive status epilepticus.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
3/9. Creutzfeldt-Jakob disease in venezuela. A case report.A case of Creutzfeldt-Jakob disease (CJD) in a 32 year old man is presented. The clinical picture included a rapid progressive dementia associated with ataxia, global aphasia, myoclonus and pyramidal signs, death occurred after 13 months. The diagnosis of CJD was confirmed by CT and neuropathological studies. This is the first report of CJD occurring in venezuela.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
4/9. Creutzfeldt-Jakob disease presenting as isolated aphasia.Progressive aphasia without dementia (primary progressive aphasia) is increasingly recognized as an important neurobehavioral syndrome. Clinical diagnosis of progressive aphasia is difficult early in its course, and the differential diagnosis is usually said to include Alzheimer's and Pick's diseases. We report a 61-year-old man with autopsy-proven Creutzfeldt-Jakob disease (CJD) whose major initial manifestation was a progressive, fluent aphasia. myoclonus was absent, and characteristic EEG abnormalities appeared relatively late. We believe that this case of CJD is unique in its presentation of profound and isolated aphasia. CJD should be considered in the differential diagnosis of the progressive aphasia syndrome.- - - - - - - - - - ranking = 10keywords = aphasia (Clic here for more details about this article) |
5/9. Unilateral Creutzfeldt-Jakob disease.A 73-year-old woman had progressive right hemiparesis, aphasia, and focal motor seizures. EEG showed periodic discharges on the left. She died 8 weeks after onset. At autopsy, there was marked spongiform change, neuronal loss, and severe proliferation of astrocytes predominantly on the left and most prominently in the insular and centroparietal cortex. The changes were consistent with Creutzfeldt-Jakob disease (CJD), but pathology was slight or absent on the right side. This case appears as the first report of what might be called unilateral CJD. Such a condition should be included within the differential diagnosis of progressive unilateral cerebral disorders.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
6/9. Unilateral Creutzfeldt-Jakob disease presenting as rapidly progressive aphasia.A 64-year-old man presented with a three day history of progressive Broca's aphasia, followed within 3 weeks by exclusively right-sided myoclonus, rigidity, and dystonia. Within 4 weeks he was globally aphasic. He died within 7 weeks of onset. In the final week, rigidity and myoclonus became bilateral. CT and MRI were normal. SPECT showed diminished perfusion of the left hemisphere. EEG showed periodic discharges on the left. At autopsy, there were marked cortical spongiform change, neuronal loss, and gliosis throughout the left hemisphere and in the right occipital cortex. Elsewhere in the right hemisphere, spongiform change was non-existent to minimal. There was moderate spongiform change in the molecular layer of the cerebellar cortex, much more marked on the left. Clinical and pathological unilateral cerebral predominance extended to the ipsilateral cerebellum. Creutzfeldt-Jakob disease is an important consideration in patients with rapidly progressive unilateral cerebral signs associated with a movement disorder.- - - - - - - - - - ranking = 5keywords = aphasia (Clic here for more details about this article) |
7/9. 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.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
8/9. aphasia or dementia: the cautionary tale of Dr. JJ.This case study involves the differential diagnosis of aphasia versus dementia. However, the case of Dr. JJ is instructive from a number of aspects, presented here as "cautionary notes" which are intended to remind clinicians of sometimes-forgotten interpersonal issues in the diagnosis of disorders of speech and language. This case also is intended to illustrate that merely distinguishing between dementia and aphasia is sometimes inadequate, particularly when potentially transmittable (and in some cases, curable) dementias might be involved.- - - - - - - - - - ranking = 2keywords = aphasia (Clic here for more details about this article) |
9/9. Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: molecular analysis of one case.A 49-year-old woman presented with isolated aphasia followed by dementia and ataxia with a duration of 4 years. Histopathologically there was panencephalic involvement, status spongiosus, and kuru-type plaques. Molecular analysis showed heterozygosity at codon 129 in the prion protein (PrP) gene, and type 2 protease-resistant PrP. The comparison between this case and those previously reported suggests that the panencephalopathic variant of Creutzfeldt-Jakob disease (CJD) is an aspecific end-stage condition displayed by most if not all CJD variants in individual patients with an unusually prolonged course.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |