Severe Dementia Associated with Bilateral Symmetrical Degeneration of the Thalamus

Stern, Karl

doi:10.1097/00005053-194002000-00039

Cited by 15 publications

(19 citation statements)

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“…and McMencmy et al [12]. The cases of Poursines et al [IS] and McMenemy et al [12] showed a marked spongy state or glial prolifera tion with patchy neuronal loss, while the 3 cases of Stern [21]. Schul man [20], and Garcin et al [3] showed thalamic degeneration similar in histopathological nature and distribution to that of the present cases.…”

Section: Nosological Questionssupporting

confidence: 68%

“…Thalamic lesions are often observed in this disease and their distribution resembles those of the present cases [9. 17], Kirschbauni [5] described a 'thalamic type' of C-J disease, and in cluded 5 cases from the literature with a short clinical course, namely the cases of Stern [21], P oursines et al [18], Schulman [20]. Garcin et al [3] .…”

Section: Nosological Questionsmentioning

confidence: 99%

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Thalamus Degeneration in Japan

Oda¹

1976

Stereotact Funct Neurosurg

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12 Japanese cases with marked thalamus degeneration are reviewed. They are divided into 3 groups. The 4 cases of the first group showed a relatively short clinical course. Neuropathologically, the lesions of this group are selectively severe in the thalamus, and olivocerebellar atrophy is also observed. Because of the significance of the thalamic changes, this group is called ‘primary thalamus degeneration’. The cases of combined system degeneration and presenile dementia, such as Pick''s disease, are described in the 2nd and 3rd groups. In the thalamus, the developmentally younger, and medially and dorsolaterally located nuclei are most severely affected in all the cases. The substantia nigra, globus pallidus and cerebellar afferent systems, and occasionally the dentate nucleus, subthalamic nucleus and reticular formation, are also involved. In the cases of presenile dementia, in which the frontal and posterior parietal lobes are damaged, degenerated areas in the cerebrum and thalamus have a close relationship with each other genetically as well as in fiber connections. The clinicopathological relationship is discussed on the basis of those neurophathological findings.

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Section: Nosological Questionssupporting

confidence: 68%

Section: Nosological Questionsmentioning

confidence: 99%

Thalamus Degeneration in Japan

Oda¹

1976

Stereotact Funct Neurosurg

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“…Primary thalamic degeneration (PTD) causing dementia was first described in 1939 by Stern 13 in a subject who presented severe atrophy of ventral anterior and dorsomedial thalamic nuclei, and a variable degree of gliosis in the cerebral cortex. Martin, in 1975 14 , classified the thalamic degenerations into: preferential thalamic degeneration, thalamic degeneration associated with multi-system atrophies and the thalamic form of Creutzfeldt-Jakob disease.…”

Section: Discussionmentioning

confidence: 99%

Frontotemporal dementia with severe thalamic involvement: a clinical and neuropathological study

Radanovic

Rosemberg

Adas

et al. 2003

Arq. Neuro-Psiquiatr.

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-Frontotemporal dementia (FTD) is the third-leading cause of cortical dementia after Alzheimer's disease and Lewy body dementia, and is characterized by a dementia where behavioral disturbances are prominent and appear early in the course of the disease. We report the case of a 58 year-old man affected by dementia with behavioral disturbances, in addition to rigid-hypokinetic and a lower motor neuron syndrome that were present at later stages of the illness. Neuroimaging studies showed frontotemporal atrophy. Neuropathological studies revealed intense thalamic neuronal loss and astrocytic gliosis, as well as moderate frontotemporal neuronal loss, astrocytosis and spongiform degeneration. Thalamic degeneration has previously been described among the wide group of neuropathological features of FTD. The aim of the present study is to show the clinical and neuropathological aspects of thalamic degeneration in FTD, along with its role in behavioral disturbances, a common finding in this condition.KEY WORDS: frontotemporal dementia, thalamic degeneration, neuropathological findings. Demência frontotemporal com intenso envolvimento talâmico: estudo clínico e neuropatológicoRESUMO -Demência frontotemporal (DFT) é a terceira causa de demência cortical, após doença de Alzheimer e demencia dos corpos de Lewy, caracterizando-se por ser uma síndrome demencial em que as alterações de comportamento são proeminentes e aparecem precocemente. Descrevemos o caso de um homem de 58 anos apresentando demência com expressiva alteração de comportamento, somadas a síndrome rígido-hipocinética e de neurônio motor inferior. Os exames de neuroimagem mostraram atrofia frontotemporal. O exame neuropatológico revelou intensa perda neuronal e astrocitose talâmica, bem como moderada depleção neuronal frontotemporal, com astrocitose e degeneração espongiforme. Degeneração talâmica já foi descrita entre os possíveis achados neuropatológicos na DFT. O objetivo deste estudo é contribuir com uma descrição dos aspectos clínicos e neuropatológicos da degeneração talâmica na DFT, bem como seu envolvimento nos transtornos de comportamento, tão freqüentes nesta condição clínica. PALAVRAS-CHAVE: demência frontotemporal, degeneração talâmica, achados neuropatológicos. Frontotemporal dementia (FTD) represents 10-20% of all dementias (being the third-leading cause of cortical dementia after Alzheimer's disease and Lewy body dementia) 1,2 . Loss of personal awareness, impulsiveness, apathy, lack of initiative, perseverative behavior, hyperorality, progressive reduction of speech and preserved spatial abilities, together with frontotemporal atrophy on CT or MRI scans and hypoperfusion / hypometabolism in frontal, temporal and frontotemporal regions in SPECT and PET studies, are findings that make for a very probable diagnosis of FTD. Family history is described in 20-40 % of such cases. A rigid-hypokinetic and lower motor syndromes have also been described in later stages of FTD 3,4 . The Lund and Manchester Groups 5 defined three neuropathological...

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“…In these regions nerve cell loss, gliosis, neurofibrillary tangles, and granulovacuolar changes have been noted (Steele et al, 1964 Stern (1939) described a dementing syndrome associated with bilateral symmetrical degeneration of the thalamus. This patient's symptoms were 'lack of memory and orientation, a tendency to perseveration, and changes in personality such as inertia and complete lack of initiative'.…”

Section: Dementia Of Progressive Supranuclear Palsymentioning

confidence: 99%

The subcortical dementia' of progressive supranuclear palsy

Albert¹

1998

Neurocase

105

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Dementia is a term used to describe a constellation of behavioural signs and symptoms of intellectual dysfunction due to widely varying aetiologies and dependent on separate neurophysiological mechanisms. Analyses of the patterns of disordered behaviour in demented patients may lead to an understanding of the underlying pathophysiological processes and may indicate potentially useful forms of treatment for the dementias.This communication describes the particular pattern of dementia observed in five patients with progressive supranuclear palsy, a disease entity characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia, and dementia. The pattern of intellectual deterioration described in this report has many features in common with that of other reported cases2. An analysis of our data and of those from the literature suggests that a behaviour pattern

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Severe Dementia Associated with Bilateral Symmetrical Degeneration of the Thalamus

Cited by 15 publications

References 0 publications

Thalamus Degeneration in Japan

Thalamus Degeneration in Japan

Frontotemporal dementia with severe thalamic involvement: a clinical and neuropathological study

The subcortical dementia' of progressive supranuclear palsy

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