+ Site Statistics
References:
52,654,530
Abstracts:
29,560,856
PMIDs:
28,072,755
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn

+ Translate
+ Recently Requested

Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity



Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity



Clinical Neurology and Neurosurgery 103(3): 160-167



Subacute diencephalic angioencephalopathy (SDAE) is a rare and fatal disease of unknown etiology that involves the thalami bilaterally. To date, there have been four cases reported, in which the diagnosis was established only after post mortem examination of the brain. We report two male patients, ages 69 and 41 years, who presented with progressive dementia and somnolence. Radiological evaluation revealed enhancing lesions involving both thalami. The differential diagnosis included a number of neoplastic, inflammatory and vascular processes. In both cases, pathological evaluation of biopsy specimens suggested the diagnosis of SDAE. Despite supportive care, the disease progressed rapidly and both patients died within weeks after initial presentation. The diagnosis was confirmed at autopsy in both cases. SDAE is a rare cause of bithalamic disease that can be mistaken for a neoplasm as well as a number of conditions that necessitate different treatment choices. The histopathological findings can establish the diagnosis when combined with radiological and clinical information. This report emphasizes the utility of stereotactic biopsy in early diagnosis of SDAE.

(PDF emailed within 0-6 h: $19.90)

Accession: 047469258

Download citation: RISBibTeXText

PMID: 11532556

DOI: 10.1016/s0303-8467(01)00131-7



Related references

Subacute diencephalic angioencephalopathy: an entity similar to angiodysgenetic necrotizing encephalopathy and Foix-Alajouanine disease. Journal of Neurology 239(7): 379-381, 1992

Expanding the spectrum of subacute diencephalic angioencephalopathy. Journal of Clinical Neuroscience 23: 8-13, 2016

Subacute diencephalic angioencephalopathy--report of an additional case. Journal of the Neurological Sciences 45(1): 73-81, 1980

Subacute diencephalic angioencephalopathy. A clinicopathological case study. Journal of the Neurological Sciences 22(2): 197-210, 1974

Subacute diencephalic angioencephalopathy: Report of an additional case. Journal of the Neurological Sciences 45(1): 73-81, 1980

Prostatic Ductal Adenocarcinoma-A Rare Entity With Radiological Dilemma Demanding Endoscopic Biopsy. Urology 108: E1-E2, 2017

Cleidocranial Dysplasia: Presentation of Clinical and Radiological Features of a Rare Syndromic Entity. Mymensingh Medical Journal 27(2): 424-428, 2018

A rare presentation of subacute sclerosing panencephalitis with acute fulminant course and atypical radiological features. Annals of Indian Academy of Neurology 16(4): 732-733, 2013

Subacute brainstem angioencephalopathy: favorable outcome with anticoagulation therapy. Journal of the Neurological Sciences 275(1-2): 167-169, 2008

Subacute brainstem angioencephalopathy: A case report and review of the literature. Journal of the Neurological Sciences 208(1-2): 101-104, April 15, 2003

Case Report of Two Cases of Patella Subacute Osteomyelitis in Diabetic Women A Rare Entity. Journal of Orthopaedic Case Reports 6(2): 13-15, 2016

Radiological intervention for heamoptysis in a case of acute pancreatitis a rare entity. Hpb 18: E535-E536, 2016

Ossified dysraphic hamartoma with lipomyelocoele/lipomyelomeningocoele-A rare radiological entity. Clinical Imaging 43: 60-63, 2017

Myopericytoma of the Base of the Finger: Radiological and Pathological Description of a Rare Benign Entity. Journal of Hand Surgery, 2018

Subacute thyroiditis, a neglected entity in the diagnosis of fever of unknown origin. Amb 28(2): 67-68, 1982