A 39-year-old woman with sudden onset of dysarthria and left hemiparesis; no history of underlying systemic disease; symptoms resolved spontaneously in less than 24 hours.
What is your diagnosis?
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The answer is Developmental Venous Anomaly Associated with Unilateral Dystrophic Calcification of the Basal Ganglia and Thalamus
Background:
DVA is the most common cerebral vascular abnormality (3%) and generally follows a benign clinical course.
In rare cases, chronic venous hypertensive changes in the territory drained by a DVA may lead to dystrophic calcification of the drainage territory.
This may be related to stenosis of the DVA or may be related to wall thickening and increasing resistance in the absence of frank stenosis.
Clinical Presentation:
DVAs are generally incidental findings and are only very rarely symptomatic.
In this case, the patient’s symptoms were not thought to be related to the imaging appearance. The patient had ipsilateral weakness, resolving spontaneously, which was thought to represent either a TIA or a psychosomatic presentation.
Key Diagnostic Feature:
Unilateral calcification of the basal ganglia, without mass effect, associated with a developmental venous anomaly
Differential Diagnoses:
Prior unilateral injury such as infection or neoplasm (lymphoma, toxoplasmosis, neurocysticercosis, tuberculosis and other granulomatous diseases, HIV-related infection, chemo or radiotherapy)
nonketotic hyperglycemic hemichorea-hemiballismus
Treatment:
No treatment is required.
中文版：
女性，39歲，突發構音困難，左側偏癱，既往無系統性疾病，症狀於24消失內自行消失。
診斷：發育性靜脈畸形合併單側基底節、丘腦營養不良性鈣化
背景：
發育性靜脈畸形（DVA）為最常見的腦血管畸形（3%），臨床常表現為良性過程。
極少數病例，因DVA引流區域靜脈壓力長期增高，可導致引流區域營養不良性鈣化。
這可能與畸形靜脈管腔閉塞有關，或與血管壁增厚，管腔內血流阻力增加有關。
臨床表現：
DVAs常偶然發現，極少出現臨床症狀。
本病例中，患者症狀可能與影像表現無關。患者單側肢體無力，自發緩解，可能為TIA表現或受心裡影響而出現的症狀。
關鍵診斷點：
單側基底節區鈣化，無佔位效應，合併發育性靜脈畸形。
鑑別診斷：
單側腦損傷繼發改變，如感染，腫瘤（淋巴瘤，弓形體病，腦囊蟲，結核及其他的肉芽腫性病變，HIV相關性感染，化療或放療）。
非酮症高血糖合併偏側舞蹈病
治療：
無需治療。
From AJNR Case of the week