1/301. Hereditary sensory neuropathy with deafness and dementia: a clinical and neuroimaging study.We describe three sibling patients with autosomal dominantly inherited sensory neuropathy, sensorineural hearing loss and dementia. The features of cognitive-behavioral deficits in the patients, including executive dysfunction, apathy, indifference and inattention, were consistent with a frontal lobe dysfunction. magnetic resonance imaging showed a diffuse brain atrophy. A fluorodeoxyglucose positron emission tomography in one patient and a single photon emission computed tomography in another demonstrated a glucose hypometabolism or a hypoperfusion in the medial frontal and thalamic regions. Primary frontal involvement or frontal dysfunction secondary to thalamic lesions may contribute to the nature of dementia in these patients.- - - - - - - - - - ranking = 1keywords = frontal lobe, frontal, lobe (Clic here for more details about this article) |
2/301. early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests?OBJECTIVE: To examine the role of structural (magnetic resonance imaging [MRI]) and functional (single photon emission computed tomography [SPECT]) imaging and neuropsychologic evaluation in the early diagnosis of frontal variant frontotemporal dementia (fvFTD). BACKGROUND: Current criteria for FTD stress the need for neuropsychologic and functional neuroimaging abnormalities, yet caregivers report lengthy histories of behavioral change. It is not known when, in the course of the disease, these investigations become abnormal, because few longitudinal studies have been reported. METHOD: Longitudinal study of two patients with serial neuropsychologic evaluation and MRI and HMPAO-SPECT scanning. RESULTS: Both patients, men aged 49 and 50, had major changes in personality, behavior, and social conduct that progressed over 5 to 6 years in a way that conformed to the clinical picture of fvFTD. There was remarkably little abnormality on neuropsychologic testing, and MRI and HMPAO-SPECT findings initially were normal. Over time, however, abnormalities on SPECT, frontal atrophy on MRI, or a neuropsychologic profile more typical of fvFTD developed in both patients. CONCLUSIONS: Standard neuropsychologic tests and conventional brain imaging techniques (MRI and SPECT) may not be sensitive to the early changes in fvFTD that occur in the ventromedial frontal cortex, and better methods of accurate early detection are required. These findings are relevant to the diagnostic criteria for FTD.- - - - - - - - - - ranking = 1.1203732385442keywords = frontal (Clic here for more details about this article) |
3/301. amyotrophic lateral sclerosis with dementia. Case report.A patient is described in whom a profound and rapidly progressive dementia occurred in association with clinical features of amyotrophic lateral sclerosis. A magnetic resonance imaging showed signs of frontal and especially left temporal atrophy. The pattern of dementia indicated impaired frontotemporal lobe functions, evidenced by reduced tracer uptake in the frontotemporal lobes on brain single photon emission computed tomography. Neuropathological examination in this patient revealed mild frontotemporal atrophy with spongiform changes and neuronal loss affecting mainly layers II and III of the frontotemporal cortices. There was atrophy of the hypoglossal nuclei. The spinal cord changes were consistent with motor neuron disease. The patient showed an irreversible and progressive course. A review of the relevant literature was made.- - - - - - - - - - ranking = 0.1753578883152keywords = frontal, lobe (Clic here for more details about this article) |
4/301. motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration.The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities. The predominant frontal-temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease-inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. His premorbid diagnosis was cortical-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.- - - - - - - - - - ranking = 0.16005331979203keywords = frontal (Clic here for more details about this article) |
5/301. frontotemporal dementia and Alzheimer's disease: differential diagnosis.This report addresses the clinical differentiation of Alzheimer's disease (AD) from frontotemporal dementia (FTD), including Pick's disease. The accuracy of a clinical diagnosis of a dementing disorder is determined in part by the prior probability (base rates) of the disorder, which predicts an overwhelming likelihood of a diagnosis of AD, because the prevalence of AD is much greater than FTD. The clinical features of the disorder also determine the accuracy of diagnosis. Recent studies have reported an improvement in the differential diagnosis of FTD, utilizing the Lund-Manchester criteria. patients with FTD typically have early noncognitive behavioral changes with relatively spared cognition, frontal atrophy and enlargement of the Sylvian fissures on CT and MRI scans, and frontal-temporal deficits on SPECT or PET scans. In contrast, AD patients have early cognitive changes with relatively preserved personality and behavior, hippocampal and medial-temporal lobe atrophy on CT or MRI scans, and parietotemporal SPECT or PET deficits.- - - - - - - - - - ranking = 0.32775892384564keywords = frontal, lobe (Clic here for more details about this article) |
6/301. An autopsy case of spinocerebellar ataxia type 6 with mental symptoms of schizophrenia and dementia.We herein report the findings of an autopsy case of spinocerebellar ataxia type 6 (SCA6) which revealed a mild CAG-repeat expansion in the alpha1A voltage-dependent calcium channel (CACNL1A4) gene on chromosome 19p13. A 39-year-old man who showed slowly progressive mental disorders and gait ataxia was clinically diagnosed to have cortical cerebellar atrophy (CCA) and schizophrenia. None of his relatives revealed any symptoms such as spinocerebellar disease, however, his younger brother had shown some mental disorders. The patient eventually died at 52 years of age, and an autopsy was thus performed. The main histopathological findings included a severe neuronal cell loss of purkinje cells and inferior olivary nuclei. The number of purkinje cells in our case had decreased severely in comparison to that in either OPCA or age-matched control cases, and the purkinje cells in the cerebellar hemisphere were more affected than those in the cerebellar vermis. The neurons of the dentate nucleus and pontine nuclei were well-preserved, and no pathological changes were seen in cerebral cortices or basal ganglia. The clinicopathological findings were similar to those of late cortical cerebellar atrophy (LCCA), Holmes' cortical cerebellar atrophy (Holmes type) or SCA6 cases reported previously. Using genomic dna extracted from archival paraffin-embedded sections in the frontal lobe, cerebral basal ganglia and cerebellum, the identical mild CAG-repeat expansions in the CACNL1A4/SCA6 gene were revealed in all samples examined. These findings suggest that in cases with LCCA or Holmes type atrophy, we should thus examine the CAG-repeat expansions in the SCA6 gene, and the genomic dna extracted from paraffin-embedded sections was thus found to be useful in diagnosing SCA6 retrospectively.- - - - - - - - - - ranking = 0.51984004062392keywords = frontal lobe, frontal, lobe (Clic here for more details about this article) |
7/301. adult-type metachromatic leukodystrophy with a compound heterozygote mutation showing character change and dementia.A 26-year-old Japanese woman slowly developed a change of character such as hypospontaneity and blunted affect, followed by obvious mental deterioration. She was diagnosed as having a disorganized type of schizophrenia at the first examination. brain magnetic resonance imaging demonstrated diffuse high intensity in the cerebral white matter, particularly in the frontal lobes. The single photon emission computed tomography images using 123I-IMP disclosed diffuse cerebral hypofusion, especially in the frontal lobes. Electroencephalogram showed a moderate amount of 5-6Hz theta waves on the background of alpha activity. Nerve conduction velocities in the extremities were delayed. The level of leucocyte arylsulphatase was low. In the arylsulphatase A gene analysis, a compound heterozygote having the 99Gly-->Asp and 409Thr-->Ile mutations was confirmed. The patient was diagnosed as having metachromatic leukodystrophy. She gradually showed obvious dementing symptoms such as memory disturbance and disorientation. The characteristics of the psychiatric symptoms in the leukodystrophy are discussed.- - - - - - - - - - ranking = 1.0396800812478keywords = frontal lobe, frontal, lobe (Clic here for more details about this article) |
8/301. Intractable epilepsy as the initial manifestation of neurosyphilis.A 29-year-old man experienced intractable partial seizures as the initial manifestation of neurosyphilis. The diagnosis was made after the onset of dementia 9 months later. Both the epilepsy and dementia resolved with penicillin therapy. Syphilis should be considered in patients with adult-onset focal epilepsy, particularly if there is associated dementia. Treatment may be successful even when the diagnosis is delayed.- - - - - - - - - - ranking = 0.061560335413201keywords = epilepsy (Clic here for more details about this article) |
9/301. Fronto-temporal dementia and motor neuron disease: a neuropsychological study.The neuropsychological follow-up study of a 58-year-old man suffering from Motor Neuron Disease (ALS/MND) and Fronto-Temporal Dementia (FTD) is reported. Neuromuscular signs first appeared at the age of 51 and slowly progressed to late bulbar involvement; behavioural symptoms of the frontal type first appeared around age 53; lastly, several neuropsychological symptoms suggestive of worsening temporal involvement supervened at age 57. Our patient died at 59 of respiratory failure with the classic clinical and neuroradiological picture of FTD. A short discussion addresses the controversial issue of the coupling of ALS/MND with Dementia and its possible interpretation as the expression of a chance association of relatively common diseases, versus that of a single multifaceted disease. The role of a detailed neuropsychological assessment is highlighted, within the context of increasingly specific diagnostic criteria for FTD.- - - - - - - - - - ranking = 0.16005331979203keywords = frontal (Clic here for more details about this article) |
10/301. Early thalamic and cortical hypometabolism in adult-onset dementia due to metachromatic leukodystrophy.A case of early-onset adult dementia with family history of dementia is reported, characterised by neuropsychological deficits, suggesting frontal involvement, with mild non specific white matter abnormalities on CT scan. Familial Alzheimer's disease was suspected but the neuropathological diagnosis on brain biopsy was metachromatic leukodystrophy. 18FDG-PET revealed a very peculiar pattern of metabolic impairment in thalamic areas, in medial and frontopolar regions, and in occipital lobes. Neuropsychological follow-up showed relatively stable difficulties of long-term memory and signs of frontal lobe dysfunction, similar to those observed in subcortical dementias. MRI subsequently showed periventricular leukoencephalopathy. The brain metabolic pattern observed in that case of metachromatic leukodystrophy was quite different from that reported in other types of dementia.- - - - - - - - - - ranking = 0.68754564467753keywords = frontal lobe, frontal, lobe (Clic here for more details about this article) |
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