Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Horner’s Syndrome Secondary to Mycobacterium Avium Complex Infection Abstract Clinical examination of a 65-year old female demonstrates a left Horner’s syndrome. Chest CT reveals a large cavitary lesion of the left lung apex secondary to a Mycobacterium avium complex infection. Case History 65 year-old female presents to the neuro-ophthalmic disease department for evaluation of ptosis and anisocoria She reports a ptosis of her left eyelid for about 9 months, but did not seek care due to personal issues at home. The ptosis has been stable since the onset. Systemic history is remarkable for a recent diagnosis of a Mycobacterium avuim complex (MAC) lung infection attributed to Adalimumab (Humira® AbbVie Inc., North Chicago IL) use, Crohn’s disease, asthma and hypertension. Ocular history is remarkable for a laser peripheral iridotomy OU secondary to narrow anatomical angles, pre-surgical cataracts OU and compound hyperopic astigmatism with presbyopia OU. Current medications include azithromycin, ethambutolol, rifampin, hydrochlorothizide, and albuterol. She was previously being treated with Adalimumab (Humira® AbbVie Inc., North Chicago IL) for Crohn’s disease, but this was stopped after she was diagnosed with the MAC infection. She is currently under the care of pulmonology She is a current every day smoker for the past 40 years Pertinent Exam Findings Best-corrected visual acuities: o OD: 20/20-1, OS: 20/20 Extraocular motilites: o Full and smooth OU Confrontation fields: o Full to finger count OU Palpebral apertures o OD: 11mm, OS: 9mm Pupillary testing: o Bright illumination OD: 3.25mm, OS: 2.75mm o Dim illumination OD: 4.25mm, OS: 3.25mm o (-)RAPD Diagnostic Pupil Testing with 0.5% Apraclonidine (images will be included) o After 30 minutes Bright illumination OD: 3.00mm, OS: 3.00mm Dim illumination OD: 4.25mm, OS: 3.75mm o After 1 hour Bright illumination OD: 3.0mm, OS: 3.75mm Dim illumination OD: 3.75mm, OS: 4.5mm Reversal of anisocoria confirms left Horner’s Syndrome Anterior Segment o Patent LPI OU o 2+ nuclear sclerosis, 1+ anterior cortical spoking OU Posterior Segment o Optic nerve: 0.45/0.45 perfused, healthy, distinct OU o Macula: flat and intact OU o Vasculature: normal course and caliber OU Differential Diagnosis: Horner’s syndrome: damage along the long course of the sympathetic nerve from the hypothalamus to the eye o Inflammation at the lung apex secondary to diagnosis of MAC infection versus mycobacterium tuberculosis History of weight loss, chronic productive cough and arm/shoulder pain o Pancoast tumor History of arm/shoulder pain History of smoking o Trauma to the head/neck o Osteoarthritis of the neck with bony spurs o Aortic aneurysm o Stroke o Space-occupying mass o Multiple sclerosis o Vasculopathic o Cluster headache o Carotid cavernous fistula o Carotid artery dissection o Idiopathic Argyll-Robertson Ciliary spasm Myasthenia gravis Partial cranial nerve III palsy Dermatochalasis Congenital ptosis Additional Testing: Bloodwork: o Elevated glucose (135 mg/dL) o Elevated globulin (3.8 g/dL) o Elevated RDW (15.2%) o Elevated platelet count (408 thousand/uL) o Elevated absolute neutrophils (8127 cells/uL) Chest CT: (images will be included) o Large cavitary lesion of left lung apex. Left parahilar mass or adenopathy. Interstitial nodular disease bilaterally (left greater than right). Diagnosis and Discussion: Based on the location of the lung lesion on the CT scan, the left-sided Horner’s syndrome can be confirmed to be secondary to the MAC infection of the left lung. A Horner’s syndrome is classically defined as a triad of ipsilateral ptosis, pupillary miosis and anhydrosis. A Horner’s syndrome can be confirmed with diagnostic pupil testing in office with 0.5% or 1.0% topical apraclonidine. It is important that no other topical medications are instilled prior to the testing as it is a measure of suprasensitivity Apraclonidine is a direct alpha α2 against with weak α1 activity, which is why it does not dilate a normal eye. In an eye with a Horner’s, there is an up-regulation of α1 receptors, causing dilation, resulting in a reversal of the anisocoria. The clinical signs of a Horner’s syndrome occur due to interruption of the sympathetic nerve along its long course from the hypothalamus to the eye. The sympathetic nerve originates in the hypothalamus and descends through the reticular formation in the brainstem to the ciliospinal center of budge in the spinal cord. The preganglionic fibers then exit the spinal cord and pass over the apex of the lung to enter the sympathetic chain in the neck to synapse in the superior cervical ganglion. Here, arise the post-ganglionic axons, which travel with the carotid artery up the neck and into the cranium to eventually form the long and short posterior ciliary nerves to the eye. Since pre-ganglionic fibers cross over the apex of the lung, any mass or infection here can cause damage. Mycobacterium avium complex (MAC) is a non-tuberculous mycobacterial (NTM) pulmonary infection with increasing incidence worldwide. Although the disease is most commonly seen in association with immunosuppressive diseases like HIV, the increasing therapeutic use of tumor necrosis factor-alpha (TNF-α) medications has been associated with an increase in NTM infections. The clinical symptoms of the infection include a chronic or recurrent cough with expectoration of mucoid sputum, generalized weakness, malaise, low energy, low-grade fevers and weight loss. A chest CT in patients with active MAC infections commonly demonstrates fibrocavitary lesions involving the upper lobe of the lung, which can predispose a patient to a Horner’s syndrome. To the best of our knowledge, this is the first case report of a Horner’s syndrome secondary to a Mycobacterium avium complex infection. Treatment and Management When a Horner’s syndrome is suspected, a thorough history can help localize the underlying pathology. If the patient is asymptomatic, a normal work-up will include neuro-imaging of the entire course of the sympathetic nerve including an MRI/MRA of the head and neck and CT scan of the chest. Treatment varies based on the underlying pathology found. In this case, the treatment includes a 1-year course of azithromycin 250mg, ethambutolol 400mg and rifampin 300mg We are interested to see if the Horner’s syndrome resolves with treatment of the MAC infection, as this has been demonstrated in patients with Mycobacterium tuberculosis infections Conclusion If a Horner’s syndrome is suspected, pharmacologic testing with 0.5% or 1.0% apraclonidine should be performed to confirm the diagnosis prior to proceeding with an extensive work-up. The course of the sympathetic nerve is long, so a targeted history should be obtained to tailor the neuro-imaging towards the underlying pathology. It is important to consider all patients on anti-TNF-α as immunocompromised since these medications increase their risk of secondary infections, including tuberculosis. Although Mycobacterium tuberculosis infections must always be ruled out in patients with a chronic cough, malaise, weight-loss and a Horner’s syndrome, a non-tuberculous infection, like MAC, may also be the culprit. References: 1. Bacal, D.A., Levy, S.R. “The use of apraclonidine in the diagnosis of horner syndrome in pediatric patients.” Arch Ophthalmol 2004; 122(2):276-279 2. Davagnanam, I., Fraser, C.L., Miszkiel, K., Daniel, C.S., and Plant G.T. “Adult Horner’s syndrome: a combined clinical, pharmacological, and imaging algorithm.” Eye 2013; 27:291-298 3. Freercks, R., Sonderup, M.W. “Tuberculous lymphadenitis and Horner’s syndrome. SAMJ 2011; 101(6):381-382 4. Graff, J.M., Lee, A.G. “Horner’s syndrome (due to cluster headache): 46-year-old male presenting with HA and ptosis. February 21, 2005; Available from: http://www.EyeRounds.org/cases/case22.htm. 5. Johnson, M.M., Odell, J.A. “Nontuberculous myocbacterial pulmonary infections.” J Thorac Dis 2014; 6(3):210-220 6. Zheng, C., Fanta, C.H. “Non-tuberculous mycobacteriaul pulmonary infection in the immunocompetent host.” Q J Med 2013; 106:307-315