Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity☆
Introduction
Bithalamic pathological processes are often characterized by significant impairment of awareness and intellect, leading to profound dementia [1]. The differential diagnosis of dementia with associated bilateral thalamic lesions includes infarctions [2], [3], vascular malformations [4], [5], tumors [6], [7], infections [8], fatal familial insomnia [9], [10] and conditions concurrently affecting the cerebral cortex, such as Creutzfeldt–Jakob disease [11]. In addition, there are reports of bilateral thalamic disease resulting in dementia that cannot be classified under any of the above categories [12]. The pathological features of this ‘primary’ thalamic dementia include neuronal loss and gliosis without evidence of parenchymal necrosis and a normal cerebral vasculature [13].
DeGirolami et al. described a 60 year old male with exclusively bilateral thalamic involvement, fatal outcome and pathological characteristics consisting of vascular thickening, parenchymal necrosis and gliosis. They suggested the term ‘subacute diencephalic angioencephalopathy’ (SDAE) for this hitherto undescribed entity [1]. We were able to identify three subsequent reports that are consistent with their original description [14], [15], [16]. The diagnosis of SDAE was established post-mortem in all three of these cases. The radiological features of SDAE are unclear, since only one of the previous studies included a brief description of the computerized tomographic findings [15].
We report two patients with SDAE who presented with dementia and died within months after admission to the hospital. In both cases, the diagnosis was suggested by the pathological findings in stereotactic biopsy specimens and was confirmed at autopsy. The pathological features as well as MRI findings of these unusual cases are presented.
Section snippets
Case 1
A 69 year old, right-handed male was admitted to the emergency room after a motor vehicle accident. He had sustained sternal and lumbar fractures, but there was no direct injury to the head. He was hospitalized for 12 days after which he was discharged home in a stable condition. A few days after discharge he began complaining of somnolence, followed by urinary incontinence and confusion. There was significant weight loss and change in his personality, but he did not suffer from weakness or
Discussion
This study describes the radiological and pathological features of two patients with SDAE, a rare disorder of unknown etiology. The features in both cases were progressive mental deterioration, occurrence in adult males, bilateral parenchymal and vascular changes within the diencephalon. The disease has proven fatal in all cases (Table 1) [1], [14], [15], [16], [17]. Radiologically, lesions in both of our patients were bilateral, irregular, low-density areas on CT scan and variable contrast
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Expanding the spectrum of subacute diencephalic angioencephalopathy
2016, Journal of Clinical NeuroscienceCitation Excerpt :However, it is characteristically limited to the thalamus, and our patient demonstrated frank cortical and brainstem involvement [4,5]. Further, as Tihan et al. described, the vasculopathy and parenchymal necrosis observed in all reported SDAE patients, including our own, is sufficiently distinct from the degenerative processes in primary thalamic dementia to distinguish SDAE [5]. Another essential entity to consider is SBAE, which shares the full spectrum of pathologic and clinical features that are associated with SDAE, and thus far has been distinguished only by localization [7].
Subacute brainstem angioencephalopathy: Favorable outcome with anticoagulation therapy
2008, Journal of the Neurological SciencesCitation Excerpt :Foix–Alajouanine syndrome consists of subacute myelopathy secondary to thrombosis of abnormal vascular channels in which venous congestion causes progressive subacute myelopathy [1]. A cranial variant of this syndrome has been described in 4 cases with lesions in a symmetrical diencephalic distribution and typical histopathological findings [2–5]. Characteristically, all patients were in the older age groups, suffered from progressive neurological deficit which eventually lead to death in the time course of 2–3 months.
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Presented in part at the 76th Annual Meeting of the American Association of Neuropathologists, Atlanta, GA, June 2000.