Eye Amaurosis Research Articles

Case of Amaurosis Fugax in the Setting of a Persistent Primitive Hypoglossal Artery Requiring Carotid Endarterectomy with Regional Anesthesia.

BACKGROUND A persistent primitive hypoglossal artery (PPHA) is a rare congenital anomaly leading to persistent carotid-basilar anastomosis. This is a report of an 83-year-old man with a PPHA presenting with amaurosis fugax of the left eye requiring carotid endarterectomy under regional anesthesia. CASE REPORT An 83-year-old man presented with 2 weeks of intermittent self-resolving visual disturbances, followed by an episode of left eye amaurosis fugax. The patient had been referred to the hospital for further investigation of symptoms 1 day following the amaurosis fugax event. Carotid Doppler ultrasound demonstrated a greater than 90% stenosis of the left internal carotid artery. Computed tomography carotid and Circle of Willis angiography confirmed a mixed, ulcerated plaque and revealed a persistent left hypoglossal artery originating from the left internal carotid artery and continuing as the basilar artery. On day 3 of admission, left carotid endarterectomy was performed under conscious sedation and regional anesthesia to permit continuous monitoring of neurological status and avoid the need for intraoperative shunting. "Permissive hypertension" by targeting a systolic blood pressure of 190 to 200 mmHg was sought for the duration of clamp time. There was no deterioration of neurological function during clamping of the carotid vessels. The patient recovered well and was discharged 2 days after surgery, with no residual neurology. CONCLUSIONS This report has presented a rare case of PPHA to highlight awareness of this congenital vascular anomaly when undertaking carotid endarterectomy.

Extensive bone erosion caused by pseudotumoral aneurysm growth

Carotid ophthalmic aneurysms constitute 0.9–6.5% of the aneurysms of the ICA with up to 20% of the cases presenting with visual symptoms. We report a case of an adult woman, presented with chronic headaches and protracted visual alterations progressing to left eye amaurosis. Neuroradiological exams, revealed a giant partially thrombosed carotid ophthalmic aneurysm extending anteriorly, causing pseudotumoral spheno-orbital bone erosion. The patient underwent surgical clipping, evacuation of the thrombotic mass and decompression of the optic pathways with rapid recovery of the vision. This unusual case, contributes to the available body of evidence on aneurysms growth.

Granular cell tumor (GCT) mimicking a nonsecreting anterior pituitary adenoma

A 33-year-old male with right eye amaurosis and panhypopituitarism previously submitted to surgery and radiotherapy was admitted with a presumptive recurrence of a nonsecreting pituitary macroadenoma (the first diagnosis, in another service). Magnetic Resonance Imaging (MRI) confirmed an expansive solid isointense lesion in the posterior region of the sella with suprasellar component (). After a new surgery, histological revealed granular cell tumor (GCT) (). […] Granular cell tumor (GCT) mimicking a nonsecreting anterior pituitary adenoma

Serial carotid MRI identifies rupture of a vulnerable plaque resulting in amaurosis fugax

A 66-year-old man with cryptogenic ischemic stroke and multiple acute ischemic lesions in the right middle cerebral artery territory was enrolled in the CAPIAS trial (Carotid Plaque Imaging in Acute Stroke; [NCT01284933][1]). Baseline carotid MRI demonstrated a right-sided nonstenotic American Heart Association type-VI plaque with a large lipid/necrotic core and intraplaque hemorrhage (figure 1). Eleven months later, the patient presented again after an episode of right eye amaurosis fugax. Repeat carotid MRI revealed a new ulceration on the right side with large parts of the former lipid/necrotic core missing (figure 2). We hypothesize that plaque rupture had caused embolization into the right retinal artery. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT01284933&atom=%2Fneurology%2F80%2F12%2F1171.atom

Acute Central Retinal Artery Occlusion Treated with Intravenous Recombinant Tissue Plasminogen Activator

Central retinal artery occlusion (CRAO) causes ischemic stroke of the eye. We report a case of CRAO that was successfully treated with intravenous recombinant tissue plasminogen activator (rt-PA) and review the current literature. A 64-year-old right-handed man presented to the emergency department with acute left eye amaurosis. An ophthalmologic assessment revealed a left afferent pupillary defect, minimal visual acuity, macular edema with a cherry red spot, and multiple emboli in the inferotemporal arcade of the left eye. A neurologic examination was otherwise nonfocal; neuroimaging was normal. Acute CRAO was diagnosed, and rt-PA was administered intravenously 185 minutes after symptom onset. A repeat examination 4.5 hours after treatment found improved vision, reduced macular edema, and no emboli. An ophthalmologic evaluation 10 days later found a visual acuity of 20/200 in the left eye and bilateral arterial sclerosis without evidence of retinal emboli or macular edema. This case illustrates that intravenous rt-PA may be an effective therapeutic option for CRAO in select patients. Given the current literature and the recommended established safety window for thrombolytics in acute ischemic cerebral stroke, it is reasonable to administer intravenous treatment for CRAO within 4.5 hours after symptom onset. Nevertheless, it is critical that a prospective clinical trial confirm the efficacy, safety, and time window for treatment.

Internal carotid artery thrombosis and its evolution documented by computed tomography angiography.

A case of a 79-year-old man, affected by persistent right eye blindness after multiple episodes of transient left hemiparesis and right eye amaurosis. Brain computed tomography (Angio CT) well documented right internal carotid artery (ICA) plaque with a 8-mm thrombus extending from ICA origin to the ophthalmic artery, in progressive lysis in the next days.

Localized nasal cavity, sinus, and massive bilateral orbital involvement by human T cell leukemia virus 1 adult T cell lymphoma, with epidermal hypertrophy due to mite infestation

HTLV1 adult T cell lymphoma occurs tends to be widely disseminated and aggressive, with only brief responses to chemotherapy. Aside from cervical adenopathy, involvement of head and neck structures is uncommon and orbital involvement rare.We report a case of nasal cavity HTLV lymphoma with massive bilateral orbital involvement and proptosis, resulting in complete left and partial right eye amaurosis. No other sites of disease were found. Response to chemotherapy was rapid and complete, with almost complete restoration of vision and oculo-motor function; the patient has remained in remission for one year. An associated problem was striking bilateral hypertrophic, hyperkeratotic eyelid and breast lesions due to mite infestation.

Pituitary Metastases from Follicular Thyroid Carcinoma

Metastatic carcinomas to the pituitary gland are uncommon, occurring in only 1% of the pituitary masses. They often originate from breast or lung carcinomas and may resemble a nonfunctioning pituitary adenoma both clinically and radiologically. Here we describe a patient with pituitary metastasis from follicular thyroid carcinoma and discuss the unique features of these lesions. A 45-year-old woman was admitted to the emergency rescue room of our hospital with a 2-month history of progressive headache and blurred vision. Evaluation revealed right eye amaurosis, with a mild abducens and oculomotor palsy. Pituitary magnetic resonance imaging showed a mass that was hypo-intense in T1-weighted and hyper-intense in T2-weighted-images, located from the sphenoid sinus up to chiasmatic cisterns, raising and deflecting the optic chiasm, down to hypopharynx region, and distorting the cavernous sinuses. No evidence of anterior or posterior hypopituitarism was recorded. The immediate trans-sphenoidal surgery was uncomplicated with partial improvement of the visual fields and headache. Histopathology revealed a metastasis with well-differentiated follicular thyroid architecture. Total thyroidectomy and lymph node dissection was performed with a final histopathological diagnosis of follicular thyroid carcinoma. Subsequently, her headache became more severe. 131-I ablation treatments were performed 15 days and 12 months after thyroidectomy with decrease in headache and a decline in serum thyroglobulin levels. Pituitary metastases from thyroid carcinoma are very uncommon. As this patient illustrates, they tend to produce symptoms relating to space-occupying expansion in the parasellar region rather than to those due to destruction of the pituitary gland. Although rare, pituitary metastases caused by thyroid malignancy should be considered in patients with expanding parasellar lesions if they have thyroid cancer or uncharacterized thyroid diseases. They are unlikely to be amenable to complete resection and should be considered for 131-I treatment, perhaps avoiding the need for extensive neurological surgery.

Incidental Findings during Extracranial Carotid Duplex: Two Cases of Carotid Body Tumors

In Case 1, A 65-year-old man with a family history of carotid body paraganglioma and no other risk factors was referred to the noninvasive vascular laboratory for a carotid duplex with complaints of left eye amaurosis fugax. In Case 2, an 84-year-old woman with a history of hypertension and hyperlipidemia presented to the noninvasive vascular laboratory for carotid duplex under the suspicion of a right hemisphere transient ischemic attack. Duplex sonography of both cases recorded minimal <49% stenosis of both internal carotids. However, in Case 1, a homogenous mass was noted at the left carotid bifurcation, apparently fed by a branch of the external carotid artery, that later was confirmed by angiography to be a branch of the pharyngeal artery.

Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínico

Wegener's granulomatosis is a systemic necrotizing vasculitis that affects medium size and small vessels. Neurological involvement occurs in 22% to 54% of patients, mainly in the form of mononeuritis multiplex. Central nervous system involvement is reported in only 2% to 8% of the cases. We report a 42-year-old male who presented with headache, diplopia, third and sixth cranial nerve palsies and left eye amaurosis associated to mass located in the Meckel cavum and diffuse meningeal involvement. A biopsy of the mass disclosed a chronic granulomatous necrotizing inflammation with Langhans giant cells. A chest CAT scan showed three cavitated lung nodules and ANCA antibodies were positive in a titer of 1:80. Treatment with steroid and cyclophosphamide was started and cranial nerve palsies resolved and the number and size of lung nodules decreased. The patient was lost from follow up.

Infected carotid pseudoaneurysm and carotid-cutaneous fistula as a late complication of carotid artery stenting

Carotid stenosis after neck irradiation is a well-described entity. A 78-year-old man presented with left eye amaurosis fugax 11 years after radical neck dissection and neck irradiation for mucoepidermoid carcinoma. The patient underwent carotid artery stenting for a high-grade stenosis of the left internal carotid artery. Twenty months after the procedure, a pulsatile neck mass developed with intermittent arterial bleeding. After control of the bleeding, he underwent resection of the left carotid bifurcation, including the indwelling stent and reconstruction with a saphenous vein interposition graft. A pectoralis myocutaneous flap was used for wound closure. In this case, localized infection of the stented carotid artery led to mycotic degeneration, pseudoaneurysm formation, and erosion to the skin surface. As carotid artery stenting becomes more widely used, previously unreported late complications associated with this procedure are likely to become apparent, and continued close follow-up is warranted.

A FAMILIAL SYNDROME OF PROGRESSIVE CONE DYSTROPHY, DEGENERATIVE LIVER DISEASE, ENDOCRINE DYSFUNCTION AND HEARING DEFECT

Seven patients, 6 females and one male, with progressive cone dystrophy are reported. One patient developed amaurosis in one eye and fere amaurosis in the other. The least affected patient (13 years of age) had fairly good central cone vision, but a rod response only outside the central area. Attenuated retinal vessels, disc pallor and general atrophic appearance without pigmentation were typical findings. Six of the patients originated from 2 sibships. Increasing impairment of vision during pregnancy was seen in two patients. Pathological glucose tolerance, diabetes, liver disease, endocrinological disturbances, and hearing defects were recorded. Thus, this cone dystrophy appears to be part of a disease affecting several organs. The familial occurrence suggests that this disorder is inherited.