Abstract
year-old gentleman with a previous medical history characterized by recurrent bi-frontal and bi-temporal headaches, mild hypertension, hypercholesterolemia and recurrent renal calculi. 54-year-old gentleman with a previous medical history consistent of recurrent headaches, hypertension, dilated cardiomyopathy, hypercholesterolemia and recurrent renal calculi. Over a one-year period, he gradually developed worsening headaches associated with intermittent blurred vision, generalized aches and pains, mild cognitive impairment, and several episodes of focal upper and lower limb weakness. These symptoms led to several evaluations in the AE however, due to an rather atypical 'resolution' of previous ischemic events, this led to the suspicion of a possible case of multiple sclerosis, leading to an lumbar puncture which ultimately was slightly abnormal but was not positive for oligoconal bands. In the meantime, all serologic investigations were negative for infectious, autoimmune and neoplastic causes. Finally, a CT-angiogram followed by a Digital Subtraction Angiogram (DSA) revealed - in comparison with a previous CTA - a considerable narrowing in both MCAs, leading to the diagnosis of primary angiitis of the central nervous system (PACNS). After IV methylprednisolone, the patient stabilized for a short period of time, only to have a large L-MCA stroke which required a decompressive hemicraniectomy; he remains globally aphasic with mild right upper limb weakness.
Highlights
List of Abbreviations: primary angiitis of the central nervous system (PACNS): Primary Angiitis of the Central Nervous System; CTA: Computed Tomography Angiogram; DSA: Digital Subtraction Angiogram; DCMP: Dilated Cardiomyopathy; S-ECHO: Stress Echocardiogram; TTE: Transthoracic Echocardiogram; RBBB: Right Bundle Branch Block; middle cerebral artery (MCA): Middle Cerebral Artery; oligoclonal bands (OCB): Oligoclonal Bands; TIA: Transient Ischemic Attack; GSA: Giant Cell Arteritis
A CT-angiogram followed by a Digital Subtraction Angiogram (DSA) revealed – in comparison with a previous CTA – a considerable narrowing in both MCAs, leading to the diagnosis of primary angiitis of the central nervous system (PACNS)
54-year-old gentleman with a previous medical history characterized by recurrent bi-frontal and bi-temporal headaches, mild hypertension, hypercholesterolemia and recurrent renal calculi
Summary
List of Abbreviations: PACNS: Primary Angiitis of the Central Nervous System; CTA: Computed Tomography Angiogram; DSA: Digital Subtraction Angiogram; DCMP: Dilated Cardiomyopathy; S-ECHO: Stress Echocardiogram; TTE: Transthoracic Echocardiogram; RBBB: Right Bundle Branch Block; MCA: Middle Cerebral Artery; OCB: Oligoclonal Bands; TIA: Transient Ischemic Attack; GSA: Giant Cell Arteritis. One year before his final diagnosis, he started to present a gradual onset of shortness of breath, mainly exacerbated by moderate exertion, accompanied by occasional chest pain As these symptoms worsened over the following weeks, a Transthoracic Echocardiogram (TTE) was requested, revealing signs of a Dilated Cardiomyopathy (DCMP). A Stress Echocardiogram (S-ECHO) was subsequently performed without signs of reversible ischemia and with good contractile reserve in lateral, inferior, anterior and posterior walls He was given treatment with Bisoprolol and GTN spray to use as required. A CT head and CT-angiogram (CTA) was done without signs neither of ischemia, haemorrhage nor of carotid or middle cerebral artery (MCA)/anterior cerebral artery (ACA) stenosis (Figure 1A and B) He was discharged and evaluated the following day in the TIA clinic, with a discharge diagnosis of possible giant cell arteritis. The temporal artery biopsy was later found to be negative
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