SOLVED5

What is the diagnosis of the case below? Support your answer. A 48-Year-Old Man with Transient Vision Loss Presentation of Case Dr. Naomi J. Serling-Boyd (Medicine): A 48-year-old man was evaluated at this hospital because of transient vision loss. The patient had been in his usual state of good health until 1 hour before evaluation, when he noticed white “sparkles” flickering in the vision of the left eye, followed by sudden blurriness. He had no floaters or eye pain. During the next 2 minutes, the blurry vision worsened and became “dark,” such that the patient could not see a hand in front of his face when he covered the right eye. He presented to this hospital for evaluation, arriving 1 hour after the onset of vision symptoms. On presentation, the patient noted that the vision in the left eye was improving but remained blurry. The temperature was 36.7°C, the blood pressure 120/65 mm Hg, the heart rate 92 beats per minute, and the body-mass index (the weight in kilograms divided by the square of the height in meters) 24.2. Visual acuity was 20/20 in the right eye and was limited to hand motions at 0.9 m (3.0 ft) in the left eye; 2 hours later, visual acuity in the left eye was 20/40 for distance vision and 20/30 with correction for near vision. Results of the Ishihara color test were normal. The pupils were equal, round, and reactive to light, with no relative afferent pupillary defect. Extraocular movements were intact; visual fields were full in response to confrontation. Intraocular pressure and results of a slit-lamp examination of the anterior and posterior segments of the eye were normal bilaterally. There was severe ptosis of the left upper eyelid (margin reflex distance, 4 mm in the right eye and 1 mm in the left eye). A dilated fundus examination revealed normal optic disks, maculae, and vessels. A nontender, irregular mass was palpable under the left superior orbital rim, but there was no proptosis. The temporal arteries were prominent bilaterally, more so on the right side than on the left side, with normal pulsations and no tenderness. There was no tenderness of the scalp or temporomandibular joints. The remainder of the physical examination was normal. A review of systems was notable for fatigue and weight loss of 4.5 kg in the past year, which the patient attributed to inactivity and decreased appetite. There was a 2-month history of bilateral jaw pain with chewing of firm foods but not with soft foods. There was lateral hip soreness in both hips after walking but no stiffness. There was a sensation of locking when flexing or extending the right first finger. Two days before evaluation, the patient had a temporal headache on the right side that resolved. He reported no numbness or weakness. The patient had a history of presbyopia. He had not undergone any surgeries, was not currently taking any medications, and had no known allergies. He was born and raised in a country in Eurasia and had lived in the United States for 20 years. He traveled internationally for his work as a university professor. He was divorced and lived with his two children in a house in New England. He had smoked a half pack of cigarettes for 5 years but had quit smoking 20 years earlier. He drank alcohol rarely and did not use illicit drugs. His parents were alive and well, both of his grandmothers had had breast cancer, and there was no family history of ocular or autoimmune disease. Laboratory evaluation revealed normocytic anemia and elevated levels of alanine aminotransferase, aspartate aminotransferase, and alkaline phosphatase. Results of tests of kidney and thyroid function were normal, as were blood levels of electrolytes, calcium, glucose, glycated hemoglobin, creatine kinase, homocysteine, and lipoprotein(a). Testing for antinuclear antibodies was negative, and levels of IgG subclasses were normal. Other laboratory test results are shown in Table 1. Aspirin was administered, along with empirical treatment with intravenous methylprednisolone, and the patient was admitted to the hospital. Dr. Hillary R. Kelly: Magnetic resonance imaging (MRI) of the head and orbits (Figure 1), performed before and after the administration of intravenous contrast material, revealed enlargement of the right medial rectus muscle with stranding of the adjacent extraconal fat, as well as an enhancing masslike lesion in the left anterior superior orbit, abutting the left lacrimal gland. The masslike lesion showed a hypointense signal on short-tau inversion recovery imaging. The brain parenchyma was normal. Magnetic resonance angiography of the head and neck was normal, as was ultrasonography of the temporal arteries. Ultrasonography of the abdomen revealed markedly heterogeneous liver parenchyma and an enlarged periportal lymph node, measuring 2 cm in largest dimension. Computed tomography (CT) of the chest, abdomen, and pelvis (Figure 1), performed after the administration of intravenous contrast material, revealed multiple punctate calcifications throughout the liver and hypoattenuation of the right hepatic lobe, as well as diffuse coalescent mesenteric and retroperitoneal lymphadenopathy with coarse calcifications. There was a prominent right hilar lymph node, measuring 10 mm in diameter. The lung parenchyma was unremarkable. Dr. Serling-Boyd: On the second hospital day, diplopia occurred when the patient looked to the far right. On examination, there was mild limitation of abduction of the right eye. After a second dose of intravenous methylprednisolone was administered, the patient chose to leave the hospital against medical advice. After discharge, he took prednisone daily. During the next week, additional laboratory test results became available. Testing for antineutrophil cytoplasmic antibodies (ANCAs) was negative. Blood testing for blastomyces antigen, coccidioides antibodies, and histoplasma antibodies was negative, as was an interferon-γ release assay for Mycobacterium tuberculosis. MRI of the head and orbits was performed. A coronal short-tau inversion recovery image (Panel A) and a coronal T1-weighted fat-saturated image obtained after the administration of intravenous gadolinium (Panel B) show enlargement of the right medial rectus muscle (arrows), which is associated with marked T2-hypointense signal (Panel A) but shows enhancement similar to that of the other extraocular muscles (Panel B). Mild enhancement and fat stranding are present in the adjacent extraconal fat. An axial three-dimensional fast spoiled gradient echo image (Panel C), obtained after the administration of intravenous gadolinium, shows an enhancing masslike lesion (arrow) in the left anterior superior orbit, abutting the left lacrimal gland. CT of abdomen and pelvis was also performed. Axial and coronal images (Panels D and E, respectively), obtained after the administration of intravenous contrast material, show diffuse coalescent mesenteric and retroperitoneal lymphadenopathy (Panel D, arrows). An additional axial image (Panel F) shows geographic areas of heterogeneous hypoattenuation in the right lobe of the liver. Multiple small calcifications of varying shapes and sizes are shown throughout the liver (Panels E and F, arrows). In addition, coarse calcifications are shown within some of the mesenteric and retroperitoneal lymph nodes (Panels D and E, arrowheads).

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Table 1. Laboratory Data. Table 1. Laboratory Data.* Variable Reference Ranger On Admission White-cell count (per
ul) 4500-11,000 6310 Differential count (per ul) Neutrophils 1800-7700 4520 Lymphocytes 1000-4800 1160
Monocytes 200-1200 440 Eosinophils 0-900 170 Basophils 0-300 20 Hemoglobin (g/dl) 13.5-17.5 11.6 … Show more

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